January-April 2021

Clinico-Radiological Significance of Restoration of Radial Bow in Pediatric Forearm Fractures: An Indian Perspective

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 35-39|Aakash Mugalur1, Binoti A Sheth2


Authors :Aakash Mugalur[1], Binoti A Sheth[2]

[1] Narayani Hospital and Research Centre, Vellore, India
[2] Dept of Orthopaedics, LTMGH, Sion, Mumbai, India

Address of Correspondence
Dr Aakash Mugalur, #492, 9th Cross, 8th Main, T K Layout, Mysore, Karnataka-570009. India
Email: orthoaakash@gmail.com


Abstract

Background: The normal anatomical radial bow is of crucial importance to the normal range of motion of the forearm and to the strength generated by the muscles. The restoration of the normal amount and location of the radial bow is of crucial importance in functional outcome of forearm fractures. Few studies exist on characteristics of radial bow in Indians.
Materials and methods: In this prospective observational clinic-radiological study we aimed to establish the normal characteristics of the radial bow in the Indian population, and compare the radial bow of the injured limb with the normal contralateral side to establish a co-relation of the radial bow with rotational movements of forearm and to establish whether remodelling had any effect on the characteristics of radial bow. We used modified Schmetisch & Richards method to establish the characteristics of radial bow. The proposed sample size was 100 patients under the age of 14 years, but only 86 patients completed of the study.
Results: The mean of the site of maximum radial bow was at 61.68 % and that of the magnitude of radial bow was 7.62 % of the total radial length. The location and magnitude of radial bow have a significant correlation with forearm rotation .A negative correlation exists between the location of maximum radial bow and the magnitude of the radial bow but it is not statistically significant.
Conclusion: The characteristics of radial bow in our are comparable to that of the western literature. In forearm fractures every effort must be made to restore the radial bow close to the values of the normal limb for better functional outcome. Multicentric studies with larger sample size and a longer follow-up might add value to the available data.
Key Words: Radial bow, forearm, fracture, radius, ulna


Introduction
Forearm fractures are the very common fractures in children and account for about 40 percent of all pediatric fractures. [1,2] Despite their apparent simplicity they still continue to be a challenge to the treating orthopedician owing to the treatment complexity and the risk of complications.[3] Most patients in the pediatric age group need not be and should not be treated by open reduction and internal fixation.[4] The aim of the line of management, whether conservative or operative is to achieve optimal reduction of the fracture and to restore functionality to the injured limb. Despite enormous remodelling potential in the paediatric population, malunion is not uncommon and has a strong bearing on functional outcome. Pronation and supination movements add to the dexterity of the upper limb and are very important in the activities of daily living. These are complex movements influenced by numerous factors. Of the many factors the normal anatomical radial bow is of crucial importance to the normal range of motion of the forearm and to the strength generated by the muscles.[5] Alteration of the normal bowing may result from forearm fractures. While axial deformities are easily recognised, changes of bowing may be subtle and minor fractures which may influence it are frequently missed. Apart from appropriate reduction and rotational alignment, it is equally important to restore the radial bow comparable to the normal side for optimal treatment of forearm fractures. Studies show that restoration of the normal radial bow is related to the functional outcome. A good functional result is associated with restoration of the normal amount and location of the radial bow.[6] Estimation of the radial bow of the unaffected forearm and comparison of the injured limb with it can be useful for optimising reduction by non operative or operative management. To the best of our knowledge we did not find any literature delineating the characteristics of the radial bow in the Indian population. In our study we aimed to establish the characteristics of the radial bow with respect to the Indian population, compare the radial bow of the reduced injured limb with the normal side and follow it up to establish a co-relation with remodelling, to measure rotational movements of the forearm and establish a co-relation between the radial bow and rotational movements. We also wanted to evaluate the effect of remodelling of the isolated ulna fractures on the radial bow.
Materials and methods: The study was a prospective observational study with the intended sample size of 100 patients in the paediatric age group( 0 – 14 yrs ) with forearm fractures .The study was conducted at our institute and parents of the patients gave a formal consent and were free to withdraw their children from the study at any given point of time. Clearance from the institutional ethics committee was obtained before starting with the study. The first 100 Patients aged less than 14 years with radius or ulna or both forearm bones fractures with no previous history of fractures in either forearm bones were included in the study. Patients aged more than 14 years, history of fracture of either bones of either forearm, patients with pre-existing deformity and co-existing elbow and wrist injuries, with neurological deficit affecting the forearms were primarily excluded from the study. The primary line of management was closed reduction and casting. The patients were followed up clinico-radiologically on weekly basis in the first one month to assess for loss of reduction and cast related complications. Since eight patients showed loss of reduction beyond the acceptable criteria, requiring surgical intervention they were secondarily excluded from the study to negate the effect of the possible stiffness arising of surgery. Six patients were lost to follow up over the period of time. The remaining Eighty six patients were followed up clinico-radiologically at 1, 3, 9 monthly intervals. At every visit a standardised anteroposterior view of the forearm was obtained. The point of maximum radial bow and maximum radial bow were measured as percentage of the total radial length for comparability using the modified Schemitsch and Richards method.[6] Two measurements were taken independently by two observers and a mean of the values were taken to avoid inter observer bias. The pronation–supination movements and deformity were also assessed clinically at follow up. The data was tabulated and analysed using SPSS13.0 software.
Results: We had 71 % male and 29 % female patients with 24% of the fractures occurring in the age group of four to six years. Fall on outstretched hand was the mode of trauma in majority of the patients ( 91 %). The characteristics of the radial bow and the pronation and supination movements in our study are enlisted in Table 1. There is a statistically significant positive co-relation between the maximum radial bow and the prono-supination movements (Table 2 & 3). There is a statistically significant negative co-relation between the point of the maximum radial bow and the prono-supination movements (Table 4). We found a negative co-relation between the maximum radial bow and the point of the maximum radial bow but it was statistically insignificant.(Table 5)
Discussion: In our study the male to female ratio was 2.448 to 1 with a significant male preponderance. The increased male preponderance in our study could be associated with increased outdoor and sports activity in male children in our subcontinent and hence increased risk and vulnerability for the fractures. 24 % of the fractures occurred in the age group of 4-6 years with no significant predilection to fracture in any specific age group in the rest of the study group. Forearm shaft fractures have been shown to occur most commonly in the 12 to 16 year old age group which are a challenging group to treat.[1] The difference in the distribution of the fractures when compared to other studies could be attributed to the low sample size and the sampling method of including the first hundred patients as the study group. In our study the predominant mode of trauma was fall on outstretched hands (91 %) while the rest were due to direct impact. This is in acceptance with the published data on the mechanism of injury in forearm fractures where the indirect transmitted force is implicated in most of the forearm fractures. The primary mechanism of injury associated with radial and ulnar shaft fractures is a fall on outstretched hand that transmits indirect force to the bones of the forearm.[7]With respect to the location and magnitude of the radial bow, our results are comparable with the results of M Firl[8] and Schemitsch.[6] The minor differences in values could be due to the differences in the study group. The study group of M Firl although had pediatric population they didn’t represent a normal population as they had suspected injury to forearm. The other contributing factor could be the difference in the race. Both the papers analysed Western population. We couldn’t find any published data on normal radial bow in the Indian population. We used the modified method of Schemitsch & Richards[6, 8] to measure the radial bow as we could express it as a percentage of the total radial length and the results could be compared. Measurement of the radial bow in a singular plane, although representing a simplification, gives clinically significant information[8] and can be applied in the clinical scenario owing to the simplicity and reproducibility. Measurement of the bow on the normal side and comparing it with the affected side can help to diagnose plastic deformation of the radius in the absence of obvious fracture of the radius and serve as a guide for optimum reduction in the treatment of the fractures of the forearm.( Figure 1 and 2) The mean supination in our study was 93 degrees and the mean pronation was 89.97 degrees. The mean values of pronation and supination is not comparable in various studies. The average arc of normal forearm motion for the Mayo[9] group was 68 degrees of pronation to 74 degrees of supination. In a study by Rickert et al[10] the arc of motion was from 75 degrees of pronation to about 100 degrees of supination. In yet another study by Boone and Azen[11] it ranged from 77 degrees of pronation to 83 degrees of supination. Though it has been traditionally taught that 50 degrees of pronation and supination represent adequate forearm motion [9], it has been observed that losing 20 to 30 degrees of either pronation or supination carries the potential for significant functional impact upon important activities of daily living [12]. Prono-supination movements assume even greater importance in the Indian scenario. An “all or none” phenomenon exists whereby a small deviation from the norm of the radial bow, a good functional result could be expected in terms of rotational movements of the forearm, but once a certain point was reached there was a rapid decline in the outcome.[6] Statistical analysis has shown that a change in the location of the maximum radial bow is of greater functional significance than a change in the value of the maximum radial bow.[7] We opine that both the location and magnitude of the radial bow have a significant correlation with forearm rotation and hence has a significant effect on functional outcome. We compared isolated ulna fractures with radius-ulna & radius fractures to evaluate the effect of isolated ulna fractures on radial bow if any. In our study we observed that there is no statistically significant difference between both the groups. We conclude that isolated ulna fractures do not affect the magnitude and location of the radial bow in a statistically significant manner. We in our study also analysed the relation between the magnitude and location of the radial bow. We observed a negative correlation between the location of maximum radial bow and the magnitude of the radial bow but it was not statistically significant. Even earlier studies have failed to establish a significant correlation between the magnitude and location of the maximum radial bow. However it must be noted that they are related to functional outcome and to each other to differing degrees.[6] We followed up the maximum radial bow and the location of maximum radial bow for a period of nine months. We did not find any significant change in the value of maximum radial bow or its location during this observation period either on the fractured side or on the normal side. Despite our short follow up of nine months we opine it is important to achieve the normal radial bow on the fractured side at the initial setting itself as the remodelling process does not alter the location or the maximum value of the bow significantly. M Firl et al[8] state that the length of the radius and the value of maximum radial bow clearly increase with age but the location of maximum radial bow remained a constant. One of the reasons which could be responsible for this discrepancy between our study and the available literature could be the short follow up period of nine months in our study. We found a negative correlation between the location of the maximum radial bow and range of supination. The corelation was statistically significant throughout the duration of the study. We also established a negative co-relation between the location of maximum radial bow and pronation. The correlation was negative at every follow up, but it was statistically significant at three and nine months. In our study we found a positive co-relation between the maximum radial bow and rotational movements of the forearm. The co-relation was positive at one, three and nine monthly intervals and it was statistically significant. To evaluate the effect of radial bow in further detail on forearm rotation requires further follow up and linear regression analysis. But that was beyond the scope of our present study. But it has been found that the relation between the radial bow and rotational movements is not linear.[6] Although we have established a correlation between the radial bow and rotational movements of forearm it must be noted that radial bow is not the only factor rotational movements of the forearm are dependent on. There are many other contributory factors which are difficult to be excluded completely in clinical scenario. Small sample size and a short follow-up were among the drawbacks of the study. Further we evaluated patients in a particular subset of Indian patients. Large multicentric studies with a larger sample size and a longer follow up are necessary to further delineate the characteristics of radial bow in Indian population.

Conclusion:
The characteristics of the radial bow of the Indian population match that of the western population. Restoration of the radial bow characteristics is very important in the management of forearm fractures. Every effort must be made to restore the characteristics of radial bow in forearm fractures for a better functional outcome.

References 

1. Cheng JC, Ng BK, Ying SY, et al. A 10 year study of the changes in the pattern and treatment of 6,493 fractures. J Pediatr Orthop 1999;19: 344-350.
2. Chung KC, Spilson SV. The frequency and epidemiology of hand and forearm fractures in the United States. J Hand Surg Am 2001; 26:908-915.
3. Davis DR, Green DP. Forearm fractures in children: pitfalls and complications. Clin Orthop Relat Res 1976; 120: 172-184.
4. Canale ST. Fractures and Dislocations in Children:Canale ST, Beaty JH. Campbell’s Operative Orthopaedics, vol 2, 11th edition, Mosby Elsevier;2008. p.1545-1556.
5. Richards RR. Chronic disorders of the forearm. J Bone Joint SurgAm. 1996; 78-A: 916-30.
6. Schemitsch EH, Richards RR. The effect of malunion on functional outcome after plate fixation of fractures of both bones of the forearm in adults. J Bone Joint SurgAm. 1992; 74-A:1068-78.
7. Atkas S, Saridogan K, Moralar U, et al. Patterns of single segment nonphyseal extremity fractures in children. Int Orthop 1999;23:345-347
8. Firl M, Wünsch L. Measurement of bowing of the radius. J Bone Joint SurgBr. 2004;86-B:1047-9.
9. Morrey BF, Askew LJ, Chao EY. A biomechanical study of normal elbow motion. J Bone Joint Surg Am 1981; 63: 872-877.
10. Rickert M, Burger A, Gunther CM, et al. Forearm rotation in healthy adults of all ages and both sexes. J Shoulder Elbow Surg 2008; 17: 271-275.
11. Boone DC, Azen SP. Normal range of motion of joints in male subjects. J Bone Joint Surg Am 1979; 61: 756-759.
12. Mehlman CT, Wall EJ. Injuries to the shafts of the radius and ulna: Beaty JH, Kasser JR et al. Rockwood and Wilkin’ Fractures in Children, 7th edition, Lippincott Williams & Wilkins; 2010. p.347-404.


How to Cite this Article:Mugalur A, Sheth BA. Clinico-radiological significance of restoration of radial bow in pediatric forearm fractures: An Indian perspective. International Journal of Paediatric Orthopaedics Jan-April 2016;2(1):35-39.

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Prevalence and Degree of Consanguinity in Idiopathic Clubfoot in India

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 33-34|Sanika Milind Kulkarni 1, Shekhar Malve 2, Rajiv Negandhi 2


Authors :Sanika Milind Kulkarni [1], Shekhar Malve [2], Rajiv Negandhi [2]

 [1]Bharati Vidyapeeth Deemed University Medical College and Hospital, Sangli, India.
[2]Dept of Orthopaedics, Post Graduate Institute of Swasthiyog Pratishthan, Miraj, Maharashtra, India.

Address of Correspondence
Ms Sanika Kulkarni
Post Graduate Institute of Swasthiyog Pratishthan, Miraj, Maharashtra, India.
Email: sani0209@gmail.com


Abstract

Background: In India, clubfoot is quite commonly seen. Consanguineous marriage is one of the etiological factors associated with clubfoot deformity. Consanguineous marriage is a fairly common sight, especially in rural areas in India. As there is no study from India which mentions relationship of consanguinity and clubfoot, the present study was conducted.
Aim: The aim of the study was to find if there was any significant correlation between relationship of consanguinity and clubfoot, and divide them based on degree of consanguinity.
Material and Method: The study was conducted from 2010 to 2015 at tertiary institute. The total number of cases which were considered of idiopathic variety of clubfoot was 406. Out of these, the cases were divided into presence or absence of consanguinity, and also the degree of consanguinity. A detailed history about consanguinity was taken.
Result: According to the data and classification, 140 patients were born out of consanguineous marriage. This states that about 34.4% of the cases had consanguineous marriage. After classification into degrees, it was found that there were no cases of first degree consanguinity, 25 cases of second degree and maximum number of cases i.e. 115 cases with third degree consanguinity.
Conclusion: Thus it can be concluded that consanguinity is significantly seen in cases with idiopathic clubfoot. So, we need to create awareness regarding consequences of consanguineous marriages, particularly in rural and developing areas.
Keywords: Consanguinity, clubfoot


Introduction
India, being a culturally diverse nation, with various religions and castes, is a land with people following different customs. With various backgrounds, follow various belief systems and mind-sets. People from rural places, tribes, and low socio-economic areas, believe that marriage of their son/daughter within the family would make them safer and secure in life and thus getting them married to their relatives.
Clubfoot is one of the commonest congenital anomalies seen in orthopaedic practice. Prevalence of Clubfoot in Asian countries is about 1.5/100 children.
According to a study from India, in Sindhudurg District of Maharashtra, India, the prevalence of Clubfoot is 1.8/1000 new borns. (1) One of the etiologies mentioned in the literature of Clubfoot, is consanguineous marriage. Although Clubfoot is quite common in India, there are very few studies mentioning the prevalence of consanguinity in Clubfoot cases.

Aims
To determine the prevalence and degree of consanguinity in patients with idiopathic congenital talipes equinovarus.

Material and Method
This study was done in a tertiary care hospital, with a dedicated Pediatric Orthopaedic unit. We do see large number of cases here, of clubfoot, coming from backward areas, rural places and low socio-economic statuses, where the literacy and education is low.

We collected data from the year 2010 to 2015, of clubfoot cases that came to the hospital for advice and treatment. In all these cases, we selected the cases of idiopathic clubfoot. They consisted of treated and untreated cases of idiopathic clubfoot. At the time of consultation, detailed history of child with perinatal period, and also marital history of parents was taken. Any consanguineous marriage found in the history was noted and mentioned in detail.

We omitted all the cases other than idiopathic variety, such as Arthrogryposis, Meningomyelocele, and Syndromic cases.
We included all the cases of treated or untreated cases of idiopathic clubfoot and divided them by presence or absence of consanguinity. Then the cases with consanguineous marriage were further classified into degrees.
For classification of degrees of consanguinity, we referred to the NHS National Genetics and Genomics Education Centre (4) through their website (www.geneticseduction.nhs.uk)
From this source, we found information about classification of consanguinity into degrees. Degree of consanguinity is determined by the degree of relationship between the two individuals. That in turn is determined by the shared amount of genes in the relatives. As half of the genes in a person are inherited from each parent, and shared with the sibling, marriage with parent, sibling or child, is first-degree consanguinity. Sharing one-quarter proportion of genes with aunt/uncle, niece/nephew, grandchild/grandparent, marriage with one of these relations is second-degree consanguinity. The third degree of consanguineous marriage is with the relations sharing one-eighth proportion of genes, i.e. with first cousins, or great grandparents.
Following is the classification of consanguinity into degrees:
First Degree: marriage with Parent, Child, or Sibling.
Second Degree: marriage with Aunt/Uncle, Niece/Nephew, Grandparent/Grandchild
Third Degree: marriage with First Cousins.

Result
From the years 2010 to 2015, at our institute we saw 457 cases of clubfoot. Out of these, 39 were cases of non-idiopathic variety. So we excluded them. Out of the remaining 418 cases, 240 cases had come at neonatal age and 178 cases had come at different ages, for treatment. Out of these 418 cases of idiopathic clubfoot, 12 parents were not willing to share their marital history. So, after excluding them, our final data comprised of 406 cases, which were then classified into degrees of consanguinity.
So with the final sample size of 406 cases, 230 cases were presented to us at neonatal ages while 168 were late presenters (Age 3 months to 10 years).
In this study, out of 406 cases, there were 272 males and 134 females. Out of these patients, 282 had unilateral clubfoot, and 124 cases had bilateral. With respect to consanguinity, there were 140 children born out of consanguineous marriage. This accounts to about 34.4% of patients with consanguineous marriage of parents. According to the above-mentioned classification, there were 0% cases of first-degree consanguinity, about 18% i.e. 25 cases with second-degree consanguinity. Majority of them 82% i.e. 115 cases had third-degree consanguinity. This way, the commonest degree was the third, and within the third degree of consanguinity, all the marriages were found to be between respective children of brother and sister.
Consanguineous marriages are more prevalent in rural areas than urban. These have been very commonly associated with low age at marriage, low educational level of mother, and low occupational status of the father.
Association of consanguinity and pediatric disorders is well established. Parental consanguinity has been linked with various risks including stillbirths, perinatal mortality, congenital birth defects [DDH, CTEV], malformations, mental retardation, and neonatal diabetes mellitus.
Parental consanguinity increases the autosomal recessive conditions through the expression of the recessive deleterious alleles, especially in the offspring of first-degree consanguineous marriages.
In our study, we have attempted to establish the correlation of clubfoot and consanguineous marriage. In previous study made by Shrinivas and Nataraj (3) they have established consanguineous marriage as one of the factors causing clubfoot.
Our study demonstrates, that almost one-third of the cases of CTEV that came to our outpatient clinic have consanguineous marriage of parents. These findings are similar to the study done by Sahin at al. (2), which demonstrated similar percentage of cases of clubfoot and consanguinity.
There is no direct relation of sex or number of feet affected (unilateral or bilateral), with consanguinity.

Conclusion
The result of the presented study reveals that there is a significantly high proportion of consanguinity in clubfoot patients. There is an urgent need to inform the people, properly about the anticipated deleterious effects of inbreeding in societies where inter-relation marriage is widely practiced. Further etiological studies that look into the association of consanguinity and clubfoot are needed to support the finding and clarify the significance of such an association.

References 

1. The Mode of Inheritance and Other Variables in Idiopathic Talipes Equinovarus.
Dr. R. S. Kulkarni. Civil Hospital Sindhudurg, Sindhudurgnagari, Maharashtra State, India. The Journal of Maharashtra Orthopaedic association, Vol 3-year 1, March 2006.
2. Consanguineousmarriageand increased risk of idiopathic congenital talipes equinovarus: a case-control study in a rural area. Sahin O, Yildirim C, Akgun RC, Haberal B, Yazici AC, Tuncay IC. J Pediatr Orthop. 2013 Apr-May;33(3):333-8. doi: 10.1097/BPO.0b013e3182784af4. PMID: 23482273.
3. Parental consanguinity and associated factors in congenital talipes equinovarus.
Sreenivas T, Nataraj AR. Foot (Edinb). 2012 Mar; 22(1):2-5. doi: 10.1016/j.foot.2011.08.001. Epub 2011 Sep 15. PMID: 21920728
4. NHS National Genetics and Genomics Education Centre website (www.geneticseduction.nhs.uk).


How to Cite this Article:Kulkarni SM, Malve S, Negandhi R. Prevalence and Degree of Consanguinity in Idiopathic Clubfoot in India.  International Journal of Paediatric Orthopaedics Jan-April 2016;2(1):33-34.

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Management Of Non-Idiopathic Clubfeet

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 27-32|Ishani P Shah1, Alvin H Crawford2, Junichi Tamai3, Shital N Parikh4*


Authors :Ishani P Shah [1], Alvin H Crawford [2], Junichi Tamai[3], Shital N Parikh[4*]

1 Ashirwad Nursing Home.Jambli Galli, Borivali West, Mumbai India.
2 Department of Orthopaedics, University of Cincinnati.
3 Division of Pediatric Orthopaedic Surgery, Cincinnati Children’s Hospital Medical Center

Address of Correspondence
Shital N Parikh, MD
Division of Pediatric Orthopaedic Surgery, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Av, Cincinnati, OH 45229
Email: shital.parikh@cchmc.org


Abstract

Background: The management of idiopathic clubfeet with Ponseti method of casting has been well outlined in literature. However management of non-idiopathic clubfeet, like the ones associated with arthrogryposis or myelomeningocele, has been challenging. Various treatment modalities including casting, soft tissue releases, bony procedures and talectomy have been attempted, either in isolation or in combination, with variable results. The aim of the current study was to perform a comprehensive review of the literature related to the classification and management of non-idiopathic clubfeet. Recent literature has shown benefits of a trial of Ponseti method of casting for all non-idiopathic clubfeet. Most of these clubfeet, however, would require surgical treatment due to either insufficient correction or recurrence. We attempt to provide, based on the review of literature and our experience, a treatment algorithm to help with the management of complex, non-idiopathic clubfeet.
Keywords:Non-idiopathic clubfoot, CTEV, Ponseti, arthrogryposis, myelomeningocele.


Introduction
With the success of Ponseti casting technique, the management of most congenital talipes equinovarus deformity or idiopathic clubfeet, has been standardised. The management of non-idiopathic clubfeet, however, continues to be challenging. These non-idiopathic clubfeet are associated with many neuromuscular and congenital syndromes, the most common of which are arthrogryposis and myelomeningocele (Table 1). The diagnosis of the underlying condition associated with clubfeet is important as it can affect management and help predict prognosis. These feet have been considered more rigid with a higher rate of recurrence compared to idiopathic clubfeet. In the past, they have been considered to be resistant to non-operative treatment and have been treated with multiple surgical procedures including extensive soft-tissue releases and radical bony surgeries  .
The purpose of this study is to perform a comprehensive review of the existing literature, review our experience and provide guidelines for management of non-idiopathic clubfeet. The article would first describe the distinct features of common conditions associated with non-idiopathic clubfeet and would discuss the need for special considerations, if any, in their orthopaedic management. This would be followed by general principles and treatment algorithm for management of non-idiopathic clubfeet.

ARTHROGRYPOSIS
Arthrogryposis is derived from the Greek words, joint (arthron) and hooked (grupon). It is a condition of non-progressive contractures of two or more joints often resulting from fetal akinesia . Arthrogryposis is not a specific diagnosis but a clinical finding characteristic of more than 300 disorders .
Clubfeet in arthrogryposis are usually stiffer and the deformities are more severe than idiopathic clubfeet. The skin is thin and smooth, muscles are pale and thin and often replaced by fat and fibrous tissue, ligaments and capsular tissues are thickened and tight, and articular surfaces are abnormally shaped with absent skin creases around the joint. Deformities are usually bilateral but rarely symmetrical. Deformities are not progressive but untreated deformities become more rigid with growth  . There is a resistance to correction and also a tendency to recur. Of the various types of arthrogryposis, clubfeet in amyoplasia have severe deformities, are stiffer, more rigid and more resistant to treatment than in distal arthrogryposis. .
Concomitant contractures of the knee and/or hip pose a unique problem to clubfeet management in arthrogryposis  . The knee could have a fixed deformity in extension (subluxated) or in flexion. During casting for clubfeet, a knee with an extension deformity can cause slippage of the cast and hence loss of correction. Simultaneous gradual correction of the knee and the foot in cast could be attempted. Failure of closed treatment of knee extension contracture may require surgical intervention. Simultaneous surgery of the knee (e.g. quadricepsplasty) and cast treatment of the clubfoot pose a challenge as well, as the knee would require early postoperative mobilization and this may compromise the correction of the foot. When knee has flexion contracture, soft tissue stretching/release or corrective extension osteotomy could be done after correction of clubfoot.
Hip joints in arthrogryposis are often subluxated or dislocated. These dislocations are often teratogenic and can rarely be treated by closed reduction. When both hips are affected, they are best left untreated and when unilateral, closed or open reduction should be attempted in the first few months.
Children with arthrogryposis often require multiple corrective surgeries and hence repeated anaesthesia. Airway access could be challenging and fibre optic intubation should be considered in such circumstances. Intravenous access may be difficult due to presence of tense skin and less subcutaneous tissue. Intra-operative positioning may be difficult due to multiple contractures. Perioperative respiratory issues and malignant hyperthermia should be considered in these children .

Diastrophic Dysplasia
Diastrophic dysplasia is an autosomal recessive disorder characterised by short stature, multiple joint contractures, cervical spine scoliosis, hitch hiker’s thumb, cauliflower ears and severe clubfoot deformity. Similar to arthrogryposis, clubfeet are usually rigid, difficult to treat and may be co-existent with hip and knee contractures. Surgical correction pose difficulty in these feet due to distorted anatomy. Recurrences after initial treatment are common and may require extensive releases and/or talectomy .

Freeman Sheldon Syndrome
This is a type of distal arthrogryposis characterised by ‘whistling mouth facies’, short stature, hand deformities in form of ulnar deviation and thumb in palm and rigid clubfeet.

Neurogenic Clubfoot
Clubfeet occur in 30-50% of spina bifida patients. It is most commonly seen in lumbar lesions at the level of L3-L4 and above.  . The level of lesion should be identified as it would affect the functional motor level and hence the ambulatory capacity. In a potential walker, the aim is to have a plantigrade foot that can weight bear; in a non-walker, the aim is to have a braceable foot to rest on the footpads of a wheelchair. Presence or absence of foot sensation should be assessed. The deformity in neurogenic feet can be attributed to varied causes including intrauterine malposition, muscle imbalance, spasticity and/or fibrotic contractures. These feet are rigid, difficult to treat and have a high risk of recurrence. There is an increased risk of pressure sores and skin breakdown due to altered or absent sensations. Physeal injury is not uncommon with manipulations. There is a high risk of fractures in these children undergoing manipulation which might not be diagnosed early due to absence of pain. There may be delayed healing of wound after surgery.
Casting in these children with myelomeningocele should be used only to maintain the correction and not to correct the deformity. Insensate feet make it difficult to identify pressure sores. Casting and manipulation should be stopped if swelling, sores or ulcers develop.

Mobius Syndrome
Mobius syndrome is a rare non progressive congenital neuromuscular disorder. It is characterised by palsy of the sixth/seventh cranial nerve and malformation of orofacial structures and limbs. Typically they have absent abduction of both eyes. Clubfoot is the most common deformity of the foot. Diagnosis should be suspected when multiple cranial nerve dysfunction exist with clubfeet.

Streeter’s Dysplasia
This is a disorder of fetal development in which the amnion separates from the chorion and forms bands which encircle limbs . Part of the limb distal to the band may be amputated, anomalous or normal. Incidence of clubfeet with constriction bands is 12-56%  . These children may or may not have a neurological deficit on the involved side. Neurologic deficit has been found more commonly with zone 2 bands (band between knee and ankle) and grade 3 severity i.e. (reaching up to the level of fascia compromising distal circulation)  . Clubfeet with constriction bands are rigid, respond poorly to conservative treatment and often require soft tissue release  . Feet with neurological deficit have been more resistant to treatment by earlier authors and require more number of surgeries than ones without a deficit  .
In case the constriction band causes circulatory compromise, emergent release should be done. All other bands may be released in a staged manner after correction of clubfoot. Paralytic feet may require tendon transfer depending on the involvement.
Down’s Syndrome
Down’s syndrome is the most frequent trisomy (chromosome 21). It is characterised by hypotonia and hyperlaxity, mental retardation, congenital heart disease, atlanto-axial instability, habitual dislocation of patella and hip and genu valgum. Though typical foot deformity due to hyperlaxity has been pes planus with metatarsus adductus, occasionally soft tissue contracture around the ankle, subtalar and mid-tarsal joints present as clubfoot  . In spite of generalised ligament laxity, these feet are resistant to non-operative treatment and often require surgical treatment . These feet, however have a tendency for overcorrection and calcaneovalgus deformity following treatment due to generalised hyperlaxity.
Pre-operative evaluation should include cardiac evaluation and cervical spine x-rays to screen for cervical spine instability.

Larsen’s Syndrome
Larsen’s syndrome is a rare congenital connective tissue disorder caused by mutation in gene encoding filamin B (FLNB). It has genetic heterogeneity with both autosomal dominant and autosomal recessive patterns. It is characterised by flat facies, cardiac defects and varied musculoskeletal Issues that include joint hypermobility, vertebral anomalies, recalcitrant dislocations of multiple major joints and clubfeet. Early detection of the syndrome is important for management decisions and prognostic counselling. Management of cervical spine instability should be a priority in these children. When hips, knees and feet are involved, treatment of the knee should be a priority. Clubfeet in these children should be treated by conservative method soon after birth and surgical treatment should be postponed until knee dislocation is corrected . Due to hyperlaxity, care should be taken to prevent overcorrection of these feet as they can often develop calcaneovalgus deformity. Hip dislocation, if bilateral, should be managed by careful neglect due to poor results of surgical treatment.
Preoperative assessment should include thorough respiratory, cardiac and neurologic evaluation. Lateral cervical spine x-rays in flexion and extension should be mandatory prior to anaesthesia to evaluate cervical spine instability which is common in this syndrome.

Principles of Treatment
Conventional non-operative treatment has been considered ineffective in cases of non-idiopathic clubfeet . Serial manipulations and casting of these feet often resulted in incomplete correction and frequent recurrences. Previous literature thus focused on the operative treatment of these feet, with most feet requiring multiple procedures. Soft tissue releases, however, also resulted in recurrences and required brace wear till skeletal maturity  . Due to high incidence of failure of soft tissue releases, talectomy has been considered to be the procedure of choice as a primary surgery as well as in cases of recurrence .
Ponseti popularised the method of serial casting to correct idiopathic clubfeet with excellent results. In non-idiopathic feet Ponseti method decreases the severity of deformity and hence decreases the need for extensive surgery. Trial of Ponseti method should be the first line of treatment for all clubfeet, irrespective of their etiology. In recent literature, short term results of Ponseti method for correction of non-idiopathic clubfeet have been encouraging (Table 2). Most of these feet were Dimeglio grade IV or Pirani grade 4 or above  . More number of casts were required than idiopathic feet, however good initial correction was achieved in most non-idiopathic feet . Arthrogrypotic feet being more rigid than idiopathic, complete correction as in idiopathic foot might not be achieved. Usually, 40-45 degree of abduction and 5 degree dorsiflexion could be achieved . The recurrence rate has been higher, but the deformity tends to improve with re-casting in most cases  . Moroney el al   reported that after Ponseti casting, the Pirani score in feet which required surgery reduced from 5.1 to 3.7. Thus casting, even if not successful, reduced the morbidity of future surgery.
Severity in non-idiopathic clubfeet has not been classified in literature probably because of low incidence. Most often these feet are either not graded or are graded as per Dimeglio score or Pirani scores which have been described for idiopathic clubfeet. Since most non-idiopathic clubfeet are rigid and a trial of Ponseti method has been justified for most patients, we have classified non-idiopathic feet on basis of response to Ponseti casting to standardize management protocols (Table 3).
A treatment algorithm for non-idiopathic clubfeet has been outlined (Fig 1). After initial correction with Ponseti method, the main factor leading to relapse has been non-compliance of brace  . The thick rigid capsule is incapable of stretching with growth and results in recurrence despite adequate bracing.  . Ill-fitting brace could contribute to non-compliance. In non-idiopathic clubfeet, it is important to maintain the correction obtained which might be less than that obtained in idiopathic feet. The brace may have to be modified to the degree of correction achieved i.e. 40 – 45 degrees of abduction and 5 degrees of dorsiflexion instead of 70 degrees and 20 degrees, respectively, as in idiopathic feet. The brace modification can improve compliance . As and when flexibility improves, more bend can be added to the brace as required. Often due to concomitant hip and knee contractures, Ankle Foot Orthosis (AFO) should be considered instead of Foot Abduction Brace (FAB) for better fit and compliance. Brace might have to be modified in case of a congenital constriction band with an amputation of the other leg – by using an AFO instead of an abduction bar. A brace in the form of an AFO should be generally worn till skeletal maturity.
If there has been partial correction with Ponseti method, surgery may be required. When no visible change is seen between two consecutive casts, casting should be stopped and surgery should be considered. The procedure of choice in such cases has been controversial. It would depend on the severity of the residual deformity, the age of child and ambulatory potential. Not all cases require extensive surgery. When only residual equinus and/or forefoot varus persists, a heel cord lengthening and anterior tibialis transfer can give good results (Fig 2). With more severe residual deformities, some authors prefer extensive soft tissue releases, reserving talectomy as a salvage procedure for the most severe of all deformities. Some favour talectomy primarily stating the higher failure of soft tissue releases  . Extensive soft releases i.e. posteromedial and lateral have shown good results when performed by 1-2 years of age to preserve articular congruity. If the child presents after 2 years of age or does not have ambulatory potential then a primary talectomy should be preferred to avoid recurrence and repeat surgery. Often only a talectomy is not enough to correct the deformity and should be accompanied by soft tissue release (Fig 3, 4). Cincinnati incision adequately provides the required extensive visualization for dissection medially, posteriorly and laterally  .
In very severe rigid clubfeet, correction might not be achieved with Ponseti casting. Primary talectomy with extensive soft tissue releases should be considered in these children for best results.
Soft tissue releases have shown good results when done at 7-8 months of age. When soft tissue releases are done, care should be taken to do as complete of a release as possible with excision of 1-2 cm of tendon to prevent recurrence. Soft tissue releases often correct the forefoot varus, though in cases of severe rigid equinus, the equinus is often not corrected even after release of tendo Achilles and posterior capsular releases. Complete excision of the talus provides enough laxity for correction of the equinus and varus deformity. Talectomy however might not correct the forefoot deformity and additional procedure in the form of an osteotomy might be required for such severe deformities. Failure of soft tissue releases could be either due to inadequate initial correction because of tight skin and soft tissues on the medial side or due to a relapse after cast removal due to the wide medial gap created secondary to the severe deformity  . However, soft tissue release is preferred in younger patients as later salvage in the form of talectomy can be done in cases of failure. When presenting at a later age, the adaptive changes in joints preferably warrant a primary bony surgery to prevent failure and recurrence.
Talectomy has shown satisfactory outcomes after short term as well as long term follow up  . In younger children, however, less radical surgery may be preferred before talectomy. If a child presents late, talectomy could be performed primarily with the best results achieved when done at 1-5 years of age. Failure of talectomy may be due to poor technique, due to either partial excision or incorrect placement of the talus under the calcaneus allowing for a later posterior drift and recurrence of equinus . A failure after poorly performed talectomy, could be difficult to treat and may require a corrective osteotomy.
Triple arthrodesis is recommended if child presents after 10- 12 years of age as earlier surgery may limit the growth of that foot.
Non-idiopathic feet have a higher rate of recurrence and hence could require secondary and tertiary procedures. Recurrence within six months of previous surgery suggest incomplete correction and hence complete correction at the initial surgery should be attempted. In case of recurrence after extensive soft tissue releases, talectomy should be considered instead of revision of soft tissue releases due to scar tissue. Ilizarov external fixator can also be used primarily or in case of secondary or tertiary recurrence.
Similar to idiopathic clubfeet, the primary aim of management of non-idiopathic clubfeet should be to achieve a painless, platigrade foot with minimal number of procedures as possible. When possible, this should be achieved before walking age to prevent adaptive changes in bone. The evaluation of result should be based on the final outcome as well as the number of procedures required to achieve it.

Summary
In recent literature, Ponseti method of casting has shown promising results in non-idiopathic feet albeit in short term. Although it may not avoid surgery in these patients in long term, it definitely reduces the severity of deformity and hence the magnitude of surgery required. It should be the first line of approach in all clubfeet, irrespective of their etiology. Later, depending on the amount of correction achieved, rigidity of the feet, walking potential and age of the patient, decision related to surgical treatment should be made. Attempt to ensure complete correction should be made in the first surgery itself to avoid recurrence. This often might require extensive soft tissue release with talectomy with or without midfoot osteotomy to correct forefoot varus. Patients should be followed long-term to identify and treat recurrence and family should be strongly counselled about the importance of such follow-up visits.

References 

1. Gibson, D.A. and N.D. Urs, Arthrogryposis multiplex congenita. J Bone Joint Surg Br, 1970. 52(3): p. 483-93.
2. Lloyd-Roberts, G. and A. Lettin, Arthrogryposis multiplex congenita. J Bone Joint Surg Br, 1970. 52: p. 494-508.
3. Tachdjian, M. and J. Herring, Pediaric Orthopaedics. 3rd ed. 2001, Philadelphia: W B Saunders.
4. Bamshad, M., A.E. Van Heest, and D. Pleasure, Arthrogryposis: a review and update. J Bone Joint Surg Am, 2009. 91 Suppl 4: p. 40-6.
5. Sodergard, J. and S. Ryoppy, Foot deformities in arthrogryposis multiplex congenita. J Pediatr Orthop, 1994. 14(6): p. 768-72.
6. Pujari, V.S., et al., Arthrogryposis multiplex congenita: An anesthetic challenge. Anesth Essays Res, 2012. 6(1): p. 78-80.
7. Al Kaissi, A., et al., Corrections of lower limb deformities in patients with diastrophic dysplasia. Orthop Surg, 2014. 6(4): p. 274-9.
8. Frischhut, B., et al., Foot deformities in adolescents and young adults with spina bifida. J Pediatr Orthop B, 2000. 9(3): p. 161-9.
9. Sharrard, W.J. and I. Grosfield, The management of deformity and paralysis of the foot in myelomeningocele. J Bone Joint Surg Br, 1968. 50(3): p. 456-65.
10. de Carvalho Neto, J., L.S. Dias, and A.P. Gabrieli, Congenital talipes equinovarus in spina bifida: treatment and results. J Pediatr Orthop, 1996. 16(6): p. 782-5.
11. Kino, Y., Clinical and experimental studies of the congenital constriction band syndrome, with an emphasis on its etiology. J Bone Joint Surg Am, 1975. 57(5): p. 636-43.
12. Allington, N.J., S.J. Kumar, and J.T. Guille, Clubfeet associated with congenital constriction bands of the ipsilateral lower extremity. J Pediatr Orthop, 1995. 15(5): p. 599-603.
13. Gomez, V.R., Clubfeet in congenital annular constricting bands. Clin Orthop Relat Res, 1996(323): p. 155-62.
14. Tada, K., K. Yonenobu, and A.B. Swanson, Congenital constriction band syndrome. J Pediatr Orthop, 1984. 4(6): p. 726-30.
15. Hennigan, S.P. and K.N. Kuo, Resistant talipes equinovarus associated with congenital constriction band syndrome. J Pediatr Orthop, 2000. 20(2): p. 240-5.
16. Chang, C.H. and S.C. Huang, Clubfoot deformity in congenital constriction band syndrome: manifestations and treatment. J Formos Med Assoc, 1998. 97(5): p. 328-34.
17. Livingstone, B. and P. Hirst, Orthopedic disorders in school children with Down’s syndrome with special reference to the incidence of joint laxity. Clin Orthop Relat Res, 1986(207): p. 74-6.
18. Miller, P.R., K.N. Kuo, and J.P. Lubicky, Clubfoot deformity in Down’s syndrome. Orthopedics, 1995. 18(5): p. 449-52.
19. Laville, J.M., P. Lakermance, and F. Limouzy, Larsen’s syndrome: review of the literature and analysis of thirty-eight cases. J Pediatr Orthop, 1994. 14(1): p. 63-73.
20. Kasser, J., Lovell and Winter’s Review of Paediatric Orthopaedics 2006.
21. Fisher, R.L., et al., Arthrogryposis multiplex congenita: a clinical investigation. J Pediatr, 1970. 76(2): p. 255-61.
22. Menelaus, M.B., Talectomy for equinovarus deformity in arthrogryposis and spina bifida. J Bone Joint Surg Br, 1971. 53(3): p. 468-73.
23. Boehm, S., et al., Early results of the Ponseti method for the treatment of clubfoot in distal arthrogryposis. J Bone Joint Surg Am, 2008. 90(7): p. 1501-7.
24. Gerlach, D.J., et al., Early results of the Ponseti method for the treatment of clubfoot associated with myelomeningocele. J Bone Joint Surg Am, 2009. 91(6): p. 1350-9.
25. Moroney, P.J., et al., A single-center prospective evaluation of the Ponseti method in nonidiopathic congenital talipes equinovarus. J Pediatr Orthop, 2012. 32(6): p. 636-40.
26. Morcuende, J.A., M.B. Dobbs, and S.L. Frick, Results of the Ponseti method in patients with clubfoot associated with arthrogryposis. Iowa Orthop J, 2008. 28: p. 22-6.
27. Janicki, J.A., et al., Treatment of neuromuscular and syndrome-associated (nonidiopathic) clubfeet using the Ponseti method. J Pediatr Orthop, 2009. 29(4): p. 393-7.
28. Dobbs, M.B., et al., Factors predictive of outcome after use of the Ponseti method for the treatment of idiopathic clubfeet. J Bone Joint Surg Am, 2004. 86-a(1): p. 22-7.
29. Drummond, D.S. and R.L. Cruess, The management of the foot and ankle in arthrogryposis multiplex congenita. J Bone Joint Surg Br, 1978. 60(1): p. 96-9.
30. Dias, L.S. and L.S. Stern, Talectomy in the treatment of resistant talipes equinovarus deformity in myelomeningocele and arthrogryposis. J Pediatr Orthop, 1987. 7(1): p. 39-41.
31. Green, A.D., J.A. Fixsen, and G.C. Lloyd-Roberts, Talectomy for arthrogryposis multiplex congenita. J Bone Joint Surg Br, 1984. 66(5): p. 697-9.
32. Crawford, A.H., J.L. Marxen, and D.L. Osterfeld, The Cincinnati incision: a comprehensive approach for surgical procedures of the foot and ankle in childhood. J Bone Joint Surg Am, 1982. 64(9): p. 1355-8.
33. Solund, K., S. Sonne-Holm, and J.E. Kjolbye, Talectomy for equinovarus deformity in arthrogryposis. A 13 (2-20) year review of 17 feet. Acta Orthop Scand, 1991. 62(4): p. 372-4


How to Cite this Article:Shah IP, Crawford AH, Tamai J, Parikh SN. Management of Non-Idiopathic Clubfeet.  International Journal of Paediatric Orthopaedics Jan-April 2016;2(1):27-32.

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Short Term Outcomes of Cuboid Closing Wedge Osteotomy with Percutaneous Soft Tissue Release in Resistant and Relapsed Cases of Clubfoot – A Prospective Study of Forty-four Feet

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 23-26| Sukalyan Dey1, Taral Nagda1, Jaideep Dhamele1, Chasnal Rathod1


Authors : Sukalyan Dey[1], Taral Nagda[1], Jaideep Dhamele[1], Chasnal Rathod[1]

Consultant Institute of Pediatric Orthopedic Disorders Mumbai India.
Fellow Institute of Pediatric Orthopedic Disorders Mumbai India

Address of Correspondence
Dr . Taral Nagda
Consultant Institute of Pediatric Orthopedic Disorders Mumbai India.
Email address: taralnagda@gmail.com


Abstract

Background: Recurrences and relapses are formidable aspects of clubfoot management. Most cases present with stiff, nonreducible feet which donot respond to conservative treatment. To treat these difficult cases, a procedure that addresses both soft tissue and bony restraints, provides sustained correction with minimal complications would be ideal. This prospective study was carried to evaluate the outcome of cuboid osteotomy with percutaneous soft tissue release as an effective procedure in difficult clubfeet in children younger than five years.
Material and Methods: The study cohort consisted of 44 feet in 29 children in whom the forefoot adduction and midfoot cavus relapsed or persisted after Ponseti method of casting or previous surgeries. In addition to clinical evaluation by modified Pirani scoring, comprehensive radiological evaluation was performed before after the surgery. Patients underwent a percutaneous five-point release of the posteromedial soft tissues followed by a mini-open lateral closing wedge cuboid osteotomy and fixation with K-wires. Patients were followed up for mean of 29 months (23-58 months).
Results: Mean age at the time of surgery was 42 months (8-59 months). In all cases Pirani score improved by minimum of two points, reaching full correction in 88% (n=37). Straightening of the lateral border of the foot was achieved in 91% feet (n=40). Marked improvements were also seen in radiological parameters viz. talus-1st MT angle in AP (by 20.5°, p<0.001), calcaneum-5th MT angle in AP (by 18.6°, p<0.001), talus-1st MT angle in lateral views (by 14.5°, p<0.001) and calcaneum-1st MT angle in lateral views (by 12.2°, p<0.001). There was no recurrence of the deformity. Only one case had superficial wound dehiscence which healed in one week with regular care.
Discussion and Conclusion: Percutaneous release of postero-medial soft tissues coupled with lateral column shortening has consistently provided instant, sustained correction in our study. Relative ease, safety and low rates of complications or recurrences make the procedure an attractive option for managing difficult clubfeet especially in those younger than five years.
Keywords: Clubfoot, Cuboid Osteotomy, Percutaneous Release.


Introduction
Clubfoot is the commonest congenital musculoskeletal disorder. With 1,50,000 children born with it each year, the global burden of the disease is tremendous because of its consequent disability with financial loss and social stigma if left untreated[1]. Fortunately, with the advent of the Ponseti Technique, the management of clubfoot has become effective, affordable and easy to accomplish [2]. However, the very cause of the disease being multifactorial, ranging from collagen abnormalities to neuromuscular disorders, all clubfeet do not respond uniformly to Ponseti protocol of serial casting, tenotomy and bracing. Some feet may initially show response, yet relapse as soon as the treatment is completed. A relapsed clubfoot is defined as any foot requiring further intervention following successful correction with the Ponseti technique. [3]. A foot is considered resistant when the de­formity shows no evidence of further improvement af­ter three months of adequate conservative treatment [4].
Ponseti himself said that regardless of the mode of treatment, the clubfoot has a strong tendency to relapse especially some feet which Ponseti labelled as “ severe and stiff with small calf size”[5]. Relapse rate in Ponseti method has been variously stated to range from 5 to 41% [6]. These relapsed clubfeet pose unique difficulties. Soft tissues adapt to the increasing body mass of the growing child and effect of weight bearing. Gradually bony changes take place including overgrowth of the lateral column of the foot. In such a stiff deformed foot, only casting is not likely to be entirely effective.
Various surgical procedures have been described for correction of deformities. Each technique has its own advantages and drawbacks.
Soft tissue releases have been described for clubfeet. They can bring the clubfoot to acceptable alignment so long as bony architecture maintains some degree of conformity. When columnar disparity occurs due to lateral overgrowth, which becomes clinically evident as forefoot adduction, midtarsal ostotomies are required. Combined procedure of lengthening of the medial column and shortening the lateral column would seem to be the optimum treatment. However, operating on both sides of the foot and forcing correction by stretching of the medial side puts contracted medial soft tissues at risk of dehiscence.
Hence we proposed that a percutaneous posteromedial soft tissue release would avoid wound related complications frequently associated with an open dissection and when combined with a trans-cuboid lateral column shortening osteotomy will bring about the full correction of all the deformities. So, with the primary objective of evaluation of outcome of the procedure we carried out the prospective study.

Material and Method
Twenty nine consecutive children younger than five years with resistant or relapsed clubfoot were enrolled for the study. A total of forty four feet in these 29 subjects were thus included in the study. Inclusion criteria consisted of: presence of forefoot adduction or mid foot cavus deformities have recurred due to any reason after full correction, or b) deformities were not corrected by any conservative treatment by Ponseti technique or operative techniques. Such previous intervention on the study cohort consisted of Ponseti casting & tenotomy (n=19), operative techniques, which consisted of soft tissue release (n=12), JESS fixation (n=2), bony procedures (n=2) and tibialis anterior transfer (n=2). We also included 7 feet of neglected cases which were not treated previously by any of the methods. These feet were clinically evaluated using the Pirani Scoring. A comprehensive radiological evaluation was performed before and after the surgery. This consisted of talus-first metatarsal angle (TFMA-AP) and calcameum-fifth metatarsal angle (CFMA-AP) in standing AP views and talus-first metatarsal angle (TFMA-L) and calcaneum-first metatarsal angle (CFMA-L) in weight-bearing lateral radiographs.
The patients were operated under general anaesthesia with a caudal block for postoperative pain control. We used tourniquet for a blood less field during dissection. The first stage consisted of percutaneuos “Five Point Release” of the tight postermedial structures. These structures which impede correction are namely the Tendo Achilles, tibiallis posterior, plantar fascia, abductor hallucis , and the long toe flexors. This was followed by a transverse incision along the dorsolateral aspect of the foot, centring over the cuboid. The extensor digitorum brevis was separated from its origin on the lateral side of the calcaneus and retracted towards the top of the foot, thus giving exposure of the cuboid, anterior portion of the calcaneus, and the neck of the talus. Two K wires were passed through the cuboid to assess the amount of wedge to be resected. The proximal K wire was perpendicular to the axis of the hindfoot and the distal one perpendicular to that of the forefoot. A dorsolaterally based wedge from the cuboid was resected using the K wires as the guide. When the gap was closed, the k wires essentially became parallel and the normal axis of the foot was achieved. The closure of the lateral cuboid wedge was also accompanied by the rotation of the forefoot, which corrected adduction and supination. The osteotomy site was secured with two k wires passing through the osteotomy site. Wound was closed and the limb was kept in an above knee plaster slab for initial two weeks. At two weeks sutures were removed and below knee plaster was applied and kept for subsequent four weeks. At six weeks, K wires were removed as the osteotomy site had healed by then. Gait training and progressive full weight bearing with ankle foot orthoses were encouraged. Orthoses were gradually weaned off and patients continued with regular footwear. For radiological evaluation, serial radiographs starting from six weeks postoperative and every three months thereafter were taken to assess the same parameters as that of the preoperative evaluation. Patients were followed up for a mean period of 29 months (23-58 months)
Along with Pirani Score, pain or difficulty in walking if any, were noted. Ease of wearing normal foot wear was assessed. Parents’ satisfaction with the appearance and function of the foot was also evaluated.
For statistical analysis was performed using Chi Square test, paired t test & Student t tests. Statistical significance was set at 99% confidence interval (p=0.01).

Observation and Results
Amongst the study cohort, there were twenty one boys eight girls. Mean age at the time of surgery was 42 months (range 8-59 months). Fifteen cases were bilateral and the rest unilateral.
Pirani score in all cases improved by minimum of two points , the mean improvement being 2.4±1.2 points which was statistically significant (p<0.001)(Table 1). Full correction was achieved in 88% cases. Straightening of the lateral border of the foot was achieved in 91% feet (n=40). Marked improvements were also seen in radiological parameters viz. talus-1st MT angle in AP (by a mean of 20.5°±13.5, p<0.001), calcaneum-5th MT angle in AP (by 18.6°±13.4, p<0.001), talus-1st MT angle in lateral views (by 14.5°±11.4, p<0.001) and calcaneum-1st MT angle in lateral views (by 12.2°±10.1, p<0.001). There was no recurrence of the deformity. Al patients were able to bear weight on a painless plantigrade feet and could use regular shoes.

Complications
None of the patients suffered from any major complications like infection, loss of fixation, displacement of the osteotomy or non-union at the osteotomy site. Only one case had superficial wound dehiscence which healed in one week with regular care. One case had residual equinus deformity of 10° which was found to be due to flat topped talus, unrelated to the surgery.

Discussion
The ultimate goal of treatment of a clubfoot is to achieve a functional , pain-free plantigrade foot with good mobility. However in recurrent or resistant cases, achieving this goal becomes progressively challenging. The effectiveness of Ponseti method diminishes with increasing age of the child. [7]. Penny has described how the effect of weight bearing exaggerates the existing equinus , varus and cavus deformities eventually rendering it non-reducible.[8]. This happens because the lateral column of the foot (calcaneus plus cuboid) elongates more than the medial one(talus, navicular, and cuneiform) and in that process the bones lose their natural reciprocal conformity. [9,10] After finding the obvious limitations of conservative methods to treat relapsed and resistant clubfeet, many surgeons devised soft-tissue release procedures[11,12]. But it was found that although STR can be used to correct some cases of resisitant clubfoot [12-14], in patients with bony deformity and columnar disparity, it can at most, provide only partial correction. Extensive STRs may have short-term complications, and as much as 47% of patients may undergo additional surgery[15,16].
Bony procedures have the ability to restore the normal foot alignment through equalisation of the columnar length and thus provide a definitive solution to resistant deformities. This involves shortening the lateral column and/or lengthening of the medial column. Combination of these midfoot osteotomies and soft tissue releases can correct the hindfoot deformities also. This is possible by elongation of medial flexors, invertors and by direct reduction of the navicular bone over the head of the talus.[17]
The complex deformities of the foot can be corrected by number of ways. Shortening the lateral column by resecting wedge of bone from the midtarsal region is effective in aligning the foot. Bone can be removed by resecting distal part of calcaneum (Lichtblau procedure), cuboid enucleation or by a formal dorsolaterally based wedge subtraction osteotomy of cuboid. Conversely, the medial column can be lengthened by opening wedge osteotomy of the medial cuneiform bone. These two procedures can be combined together whereby the wedge removed from the cuboid is grafted into the distracted osteotomy gap of the medial cuneiform.
Whatever procedure in performed, the resultant foot should be plantigrade, articular surfaces should be preserved to prevent further stiffness and the growth potential of the bone should be left undisturbed. In addition the procedure should provide one-time correction so that no further surgeries are required later in life. Medial cuneiform opening osteotomies are more appropriate for children older than four years as the ossific nucleus does not appear till then and the bone remains soft, leading to higher incidence of graft extrusion and reoperation rates[18]. According to Gordon et al. [19], this procedure should be reserved for patients aged 5 years or older because the medial osteotomy is technically difficult in a small, partially ossified cuneiform . Lourenco et al. [20] treated 39 feet with a closing who achieved good results with this dual column osteotomy. He stressed the importance of a well formed medial cuneiform ossification centre for which appears when the child is at least four years old.
Another difficulty with acute lengthening of the medial column is that the procedure is likely to stretch the skin and soft tissues which have so long remained contracted, to an extent beyond their elastic limit. Results from the arteriography study by Greider et al. [21] in children with clubfoot showed there is only one major vessel supplying the foot: the posterior tibial artery. While performing surgery on the medial side the vessel may get injured unless protected. Also , undue stretching of the medial side at attempt to correct a severely deformed foot may result in segemental thrombosis of the artery. This can seriously affect the outcome of the surgery. Following the correction of severe deformities, problems with skin closure can also occur [18].
Much of these potential difficulties can be avoided if posteromedial releases are performed using percutaneous technique to make the foot more mobile and then performing a formal lateral closing wedge osteotomy to accomplish columnar alignment. No patient in our study suffered from any medial soft tissue related problems. The present study reflects that correction of deformities can be achieved by percutaneous releases even without opening the critical medial aspect of the foot.
At the end of the study period, all our patients walked with a painless plantigrade and supple foot. There was no recurrence or persistence of deformity and hence none of the cases had to undergo surgery. Ninety-one percent feet achieved a straight lateral border. In the rest nine percent cases, the calcaneus-5th metatarsal angle was less than 10 degrees and hence were acceptable in appearance and function.
In the series published by Loza where double column osteotomy was performed, the mean post-operative radiographic angles were : talus first metatarsal angle in AP view (TFMA-AP)-7º (–10º to 25º), Calcaneo Fifth Metatarsal angle in AP view (CFMA-AP) was 9º (10–20º), lateral talo-first metatarsal angle (TFMA-L) was 11º (0–29º) [22]. The corresponding values of the parameters in our study were : TFMA-AP was 4.7º ( –5º-17º), CFMA-AP was 3.1º (-8-18º), TFMA-L was 8.5º (-2º-28º), which are comparable to that of Loza et al. It means that even with a single column osteotomy with a selective medial release, a correction comparable to double column osteotomy can be achieved.
Arjandas et al performed a retrospective analysis of 14 feet in children younger than five years having a persistent “bean shape” deformity despite treatment by Ponseti method [23]. Each was operated with using a combination of a closing wedge cuboidal osteotomy and trans-midfoot osteotomy without a medial opening wedge osteotomy. At the end of the last follow up (mean 2.6 years) the mean TFMA-AP was 12, CFMA-AP was 10, TFMA-L was 8 and CFMA-L was 13. These figures are comparable to those of our study. Marginally more correction (especially talus-first metatarsal angle in AP view, which reflects extent of adduction deformity), achieved in our study may be the result of Percutaneous medial release performed by us.
Similar results were also shown by Cesare et al (2012) in their study with 53 neglected rigid non-reducible clubfeet in children 6-9 years in whom, selective medial soft tissue release with lateral closing wedge cuboid osteotomy was performed [17].
The study however had some limitations. It was a study from a single institute. Another shortcoming of the study was short duration of follow up. The long term outcome of the study remains to be assessed. Pohl and Nicol reported one case of recurrence at 2 years [24], Schaefer and Hefti observed a tendency to adduction deformity with longer followup. Hence, following these patients for a longer duration would be more desirable [25].

Conclusion
Thus the procedure of lateral closed wedge osteotomy of the cuboid with medial percuateneus soft tissue release has shown consistently good overall results in terms of deformity correction, painfree weight bearing and preserving range of movement. Ease of the procedure, short period of convalescence, exceedingly low recurrence rates and complications are the attractive attributes of the procedure which make it ideal for children younger than five years with resistant or recurrent clubfoot.

Clinical Message
The findings of the present study reveal that surgical procedure of percutaneous soft tissue release with lateral closing wedge osteotomy of the cuboid is effective in managing recurrent and relapsed cases of clubfoot, while minimising the complications and rates of recurrence or reoperation. This could be the optimal answer to the entity of difficult clubfoot which has so long been a challenge to the orthopaedic surgeons.

References 

1. Dobbs MB, Gurnett CA. Update on clubfoot: etiology and treatment. ClinOrthopRelat Res. 2009;467:1146–1153.
2. Cesare Faldini MD, Francesco Traina MD, Alberto Di Martino MD, PhD, MatteoNanni MD, Francesco Acri MD , Can Selective Soft Tissue Release and Cuboid Osteotomy Correct Neglected Clubfoot? ClinOrthopRelat Res. 2013; 471:2658–2665.
3. Abdelgawad AA, Lehman WB, van Bosse HJ, Scher DM, Sala DA. Treatment of idiopathic clubfoot using the Ponseti method: Minimum 2-year follow-up. J PediatrOrthop B. 2007;16:98–105.
4. Dobbs MB, Rudzki JR, Purcell DB, Walton T, Porter KR, Gurnett CA. Factors predictive of outcome after use of the Ponseti method for the treatment of idiopathic clubfeet. J Bone Joint Surg Am. 2004;86-A:22–7.
5.Parsa A, Moghadam MH, Mohammad HJ. Relapsing and residual clubfoot deformities after the application of the Ponseti method: a contemporary review. Arch Bone Jt Surg 2014; 2:7–10.
6. Ponseti IV. Relapsing clubfoot: Causes, prevention and treatment. Iowa Orthop J. 2000;22:55–7.
7. Bhaskar A, Patni P. Classification of relapse pattern in clubfoot treated with Ponseti technique. Indian J Orthop. 2013;47:370–6.
8. Yagmurlu MF, Ermis MN, Akdeniz HE, Kesin E, Karakas ES. Ponseti management of clubfoot after walking age . Pediatr Int. 2011;53(1):85–9.
9. Penny JN. The neglected clubfoot. Tech Orthop. 2005;20:153–166.
10. Lavy CB, Mannion SJ, Mkandawire NC, Tindall A, Steinlechner C, Chimangeni S, Chipofya E. Club foot treatment in Malawi: a public health approach. Disabil Rehabil. 2007;29:857–862.
11. Pirani S, Naddumba E, Mathias R, Konde-Lule J, Penny JN, Beyeza T, Mbonye B, Amone J, Franceschi F. Towards effective Ponseti clubfoot care: the Uganda Sustainable Clubfoot Care Project. Clin Orthop Relat Res. 2009;467:1154–1163.
12. Codivilla A. The classic: Tendon transplant in orthopedic practice by A Codvilla. Clin Orthop Relat Res. 1976;118:2–6.
13.Turco VJ. Surgical correction of the resistant club foot: one-stage posteromedial release with internal fixation: a preliminary report. J Bone Joint Surg Am. 1971;53:477–497.
14. Zadek I, Barnett EL. The importance of the ligaments of the ankle in correction of congenital clubfoot. JAMA. 1917;69:1057.
15. Huang YT, Lei W, Zhao L, Wang J. The treatment of congenital club foot by operation to correct deformity and achieve dynamic muscle balance. J Bone Joint Surg Br. 1999;81: 858–862.
16. Kruse L, Gurnett CA, Hootnick D, Dobbs MB. Magnetic resonance angiography in clubfoot and vertical talus: a feasibility study. Clin Orthop Relat Res. 2009;467:1250–1255.
17. Dobbs MB, Nunley R, Schoenecker PL. Long-term follow-up of patients with clubfeet treated with extensive soft-tissue release. J Bone Joint Surg Am. 2006;88:986–996.
18. Faldini C, Traina F, Di Martino A, Nanni M, Acri F. Can Selective Soft Tissue Release and Cuboid Osteotomy Correct Neglected Clubfoot? ClinOrthopRelat Res 2013; 471:2658–2665.
19. Ettl V, Kirschner S, Krauspe R, Raab P. Midterm results following revision surgery in clubfeet. IntOrthop 2009;33:515–520.
20. Gordon JE, Luhmann SJ, Dobbs MB, Szymanski DA, Rich M, Anderson DJ, Schoenecker PL. Combined midfoot osteotomy for severe forefoot adductus. J Pediatr Orthop. 2003; 23(1):74–78
21. Lourenco AF, Dias LS, Zoellick DM, Sodre H. Treatment of residual adduction deformity in clubfoot: the double osteotomy. J Pediatr Orthop. 2001; 21(6):713–718
22. Greider TD, Siff SJ, Gerson P, Donovan MM. Arteriography in club foot. J Bone Joint Surg Am. 1982;64:837–840.
23. Loza ME, Bishay SN, El-Barbary HM, Hanna AA, Tarraf YN, Lotfy AA. Double column osteotomy for correction of residual adduction deformity in idiopathic clubfoot. Ann R CollSurgEngl 2010; 92: 673–679).
24. ArjandasMahadev MD, Ismail Munajat MD, AzuraMansor MD, James H. P. Hui MD Combined Lateral and Transcuneiform without MedialOsteotomy for Residual Clubfoot for Children, ClinOrthopRelat Res. 2009; 467:1319–1325.
25. Pohl M, Nicol RO. Transcuneiform and opening wedge medial cuneiform osteotomy with closing wedge cuboid osteotomy in relapsed clubfoot. J Pediatr Orthop. 2003;23:70–73.
26. Schaefer D, Hefti F. Combined cuboid/cuneiform osteotomy for correction of residual adductus deformity in idiopathic and secondary club feet. J Bone Joint Surg Br. 2000;82:881–884.


How to Cite this Article:Dey S, Nagda T, Dhamele J, Rathod C. Short Term Outcomes of Cuboid Closing Wedge Osteotomy with Percutaneous Soft Tissue Release in Resistant and Relapsed Cases of Clubfoot- A Prospective Study of Forty-four Feet. International Journal of Paediatric Orthopaedics Jan-Apr 2016;2(1):23-26.

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Dorso-Lateral Wedge correction osteotomy: Is this the way forward?

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 19-22|Viraj U Shingade1


Authors :Viraj U Shingade[1]

Children Orthopaedic Care Institute and Pravira Hospital Daya chambers, Wardha road , Nagpur, Maharashtra.

Address of Correspondence
Dr Viraj U Shingade
Children Orthopaedic Care Institute and Pravira Hospital Daya chambers, Beside Haldiram Building, Ajani square, Wardha road. Nagpur, Maharashtra-440015.
Email: virajshingade@rediffmail.com


Abstract

 In the present era of more and more advancements, when we are talking about mini invasive procedures, developing countries are still facing the major burden of neglected clubfoot deformities; where children present to orthopaedic surgeon at 5-18 years of age, not treated in the past. The orthopaedic surgeon to whom they present is in great dilemma about how to treat these cases. Majority of children cannot afford surgical procedures like Ilizarov which need expensive implants, and frequent visits to the specialised centres. Patients usually ask for one-time procedure which will give them the best chance of cure since they are brought for the treatment by charitable agencies or social workers. Dilemma in choosing correct method of treatment for these neglected or relapsed feet led to the evolution of a new single-staged, simpler and effective technique of dorso-lateral wedge corrective osteotomy. The procedure has been extensively studied in neglected as well as relapsed cases of club feet with long term follow up of more than 10 years. This review briefly describes the technical details of the procedure.
Keywords: Clubfoot, Club feet, Neglected, Relapsed, Osteotomy, Ponseti.


Introduction
The Need for new surgical technique: In the present era of more and more advancements, when we are talking about mini invasive procedures, developing countries are still facing the major burden of neglected clubfoot deformities; where children present to orthopaedic surgeon at 5-18 years of age, not treated in the past. These children walk on dorsum or dorso-lateral aspect of foot (Figure 1). They cannot use normal shoe-wear and are prone for injuries while playing games and walking long distances. They get repeated wounds at callosities, formed over dorsum of foot. They also have difficulty in squatting, which is the commonest position, used for day-to-day activities including toileting. Social stigma of having reversed feet is an important factor as such children are avoided by friends and girls experience problems in getting married.
The orthopaedic surgeon to whom they present is in great dilemma about how to treat these cases. Majority of children cannot afford surgical procedures like Ilizarov[1] which need expensive implants, and frequent visits to the specialised centres. Patients usually ask for one-time procedure which will give them the best chance of cure since they are brought for the treatment by charitable agencies or social workers.
Extensive soft tissue releases in these cases, pose problems of skin approximation, wound healing and scarring[1, 2, 3]. Conventional bony procedures cannot fully correct these severe deformities and combination of multiple procedures is required[1,2,3,4,5,6]. Triple arthrodesis[5] is associated with stiffness and ankle arthritis. Ponseti’s method for managing neglected cases have problems of compliance, frequent visits for plastering, expenses involved and subsequent need of osteotomies[7,8,9] to get complete correction.
Dilemma in choosing correct method of treatment for these neglected or relapsed feet due to above reasons led to the evolution of a new single-staged, simpler and effective technique of dorso-lateral wedge corrective osteotomy. The aim was not to obtain a normal anatomical foot; but to obtain a foot which is more or less plantigrade and able to fit shoes. A longitudinal follow up study was conducted by our institute to measure the outcome of this procedure in neglected as well as relapsed clubfoot deformities. The study was published [10] in the year 2012 with good follow up of cases and in fact now we have follow up more than 10 years of majority of our cases. Subsequently we found that the procedure was equally useful for neglected and relapsed cases of clubfoot associated with myelomeningocele and it was published[11] in the year 2014.

Operative Technique
The details of the operative technique are described in the published article in Journal Current Orthopaedic Practice [10] in the year 2012. The technique in brief is as follows: Under epidural anesthesia, the patient is placed supine; after painting and draping, the tourniquet is inflated. For correction of the cavus deformity, a percutaneous plantar fasciotomy is done (Figure 2A) from the medial aspect of the sole. The foot is then stretched manually to confirm complete release of plantar fascia. To correct the equinus, percutaneous Achilles complete transverse tenotomy (Figure 2B) is done and foot is stretched (dorsiflexed) manually. The Achilles tenotomy unlocks the heel, correcting equinus as well as heel varus. In rare cases of relapsed deformity if equinus is not corrected completely, the posterior ankle capsular release can be done through same incision. Attention is then directed to the dorsal closing wedge osteotomy. An elliptical skin incision is made on the dorso-lateral aspect at the apex of the deformed foot (Figure 2C).The incision extends from the lateral border of the foot to just short of the medial border. The elliptical skin and preformed bursa are excised. All dorsal musculature is separated and preserved with a periosteal elevator and chisel and then retracted. The dorsal neurovascular bundles are protected. The osteotomy is marked with an osteotome at the apex of the deformed foot. The wedge should be wider dorsally (1-2 cm) and taper towards the sole (Figure 2D). The amount of bony wedge required depends on the severity of the deformity. A rough clinical guide that can be used is that children who walk on the lateral border of the foot usually do not require more than 1-1.5cm of wedge; those who walk on the dorsum of foot may need more than 1.5cm of wedge. One can always remove additional bone if the correction is found to be inadequate. This also prevents the over-correction. The bones included in wedge are cuboid and all three cuneiforms. Occasionally, the navicular or distal calcaneus or base of the metatarsals may require osteotomy, depending on the severity of the deformity. Occasionally impingement of the navicular against the head of the talus occurs, not allowing full correction of the deformity, even with cuboid and cuneiform osteotomy. In those patients, naviculectomy should be done, and sometimes an additional terminal portion of the talar head may need to be excised, taking care not to disturb the subtalar joint. After wedge removal, care is taken to remove cartilage from the osteotomy bed and approximating edges to prevent nonunion and pseudarthrosis. No attempt is made to touch the subtalar joint. The deformity is then corrected by closing the space manually by everting and dorsiflexing the foot and by approximating the bony surfaces, as if closing an open book (Figures 2E and F). The forefoot is stabilized to the hindfoot with three Kirschner-wires passed from the metatarsals to the calcaneus(Figure 2G). Cancellous bone from the resected wedge is grafted at the osteotomy site. No attempt is made to look for radiographic correction because judgement is clinical. The wound is closed after placing a drain (Figure 2G) and the tourniquet is deflated. An above-knee cast was applied. A window was made in the cast (Figure 2I), and the limb was kept elevated on pillows.
Three days of intravenous antibiotics are given along with an epidural for pain relief. On the third postoperative day, the wound is inspected through the window in the cast
(Figure 2I). Hematoma, if present, should be removed along with the drain. Patients are discharged on the fourth postoperative day and non-weight bearing is advised for 6 weeks. After 6 weeks, the Kirschner-wires and cast are removed, and a plastic molded ankle-foot orthosis is applied to be worn for 6 months. Squatting is encouraged at home.
The foot deformities were graded according to classification system described by Dimeglio [12] and the results of the surgical procedure were studied extensively using evaluation system described by International Clubfoot Study group and Bensahel [13].

Discussion
Here we are not discussing about Dimeglio grade I or II deformities. We are discussing the neglected cases who have Dimeglio-grade III/IV deformities and who have came for the treatment at 5 yrs of age or later. Also we are discussing about patients with relapsed clubfeet in developing countries who present late to the hospital and may have been operated on at hospitals where no experts were available. Hence, at the time of presentation, they usually have severe (Dimeglio-grade III/IV) deformities that cannot be corrected with conventional procedures such as those described by Dwyer or Dillwyn Evans alone, and a combination of procedures[14,15,16] is required.
With the described technique, excellent and good results were achieved in 89.6% feet at our centre; indicating that the technique is effective and extremely useful in both relapsed as well as neglected clubfeet (Figure 3 and 4). When we compared the results of neglected feet and relapsed feet we found that the results were better in neglected feet. The lower proportion of excellent and good results in relapsed feet compared with neglected feet could be from scarring of previous surgeries with resultant stiffness at the ankle, subtalar, or mid tarsal joints. This was consistent [16,17,18] with other studies. We also found that the results were better in younger (<10 years) children due to less bony incongruency and better ability to remodel.
Bones in these feet are so deformed that they cannot achieve normal radiographic relationships. Our results were independent of radiographic findings and were consistent with other [18,19] studies. Only one child with poor results (bilateral relapsed feet) had complete correction of the deformity intraoperatively but was lost to follow-up for 59 months. The patient neither performed squatting exercises nor used splints, leading to recurrence. This suggests that squatting has an important role to play. In spite of poor results, he maintained good function.
No feet (including those with poor results) had clinical or radiographic evidence of arthritis at the ankle or any other joint in last 10 years of follow-up. A possible reason could be that our procedure preserves the subtalar joint. This is an advantage over triple5 arthrodesis.
Patients usually have foot discomfort and plantar pain during the initial few months after cast removal. This is due to sensitivity of the sole and disuse osteoporosis of the calcaneus and metatarsals. None of the patients complained pain during follow-up, which might be attributed to good fusion at the osteotomy site with the use of cancellous grafting.
Clubfoot is a three-dimensional deformity, with a contracted tendo-Achilles being the main deforming force. Achilles tenotomy unlocks the heel and corrects equines and heel varus. No separate procedure or calcaneal osteotomy is necessary for varus correction (Figures 2 H).
With the described technique one can achieve good correction of all components intra-operatively (Figures 2 G, H) and can have varus-valgus movements on the table. Meticulous preservation of dorsal structures preserves function and prevents wound complications.
In bilateral cases, we advice surgery on both feet under a single anesthesia; which reduces the rehabilitation time, saves resources, and provides a unique opportunity for correction. This single-stage surgical procedure is easy, safe, and reproducible, and it can be performed with basic orthopaedic instruments.
There are no rigid criteria about specific bones to be osteotomized. If the surgeon performs an osteotomy at the apex of a deformed foot and takes out a wedge dorsally, tapering towards the sole, no error can occur.
There is no risk of neurovascular damage. By removing a dorsal wedge, tension in the system is released. Problems of medial scarring and wound breakdown are avoided because the technique is percutaneous medially. No iliac crest graft or major implants are required. Fewer follow-ups are required than with other techniques. We believe that the technique is cost effective, requiring only 10% of the cost involved in the Ilizarov or other techniques. The only disadvantage is possible shortening of foot length in unilateral cases. We observed an average shortening of 1.18cm which is comparable to other series of bony procedures and even those feet treated[19,20,21] conservatively.
We agree that the best approach in developing countries to reduce the load of neglected cases and to avoid long-term problems associated with extensive soft-tissue releases is early[21] intervention during infancy. This can be achieved by seeking help from paramedical persons like nurses or physiotherapists to use the Ponseti technique[21,22] at outlying hospitals, but until that time this load of neglected cases persists;the described technique provides a good alternative procedure to surgeons of the developing world. It is a good alternative to conventional procedures for management of neglected or relapsed, late presenting clubfoot deformities. We do not claim that this is the only best alternative, but it may suit the needs of developing countries, particularly in settings like ours, where patients mostly of low socioeconomic means come from remote villages and are unable to comply in terms of follow-up

References 

1. Penny JN. The neglected clubfoot. Techniques in Orthopaedics Vol 20. Philadelphia: Lippincott Williams & Wilkins Inc.; 2005: 153–166.
2. Kumar PNH, Laing PW, Klenerman L. Medial calcaneal osteotomy for relapsed equinovarus deformity: long term study of the results of Frederick Dwyer. J Bone Joint Surg. 1993; 75-B: 967–971.
3. Yamamoto H, Muneta T, Ishibashi T, et al. Posteromedial release of congenital club foot in children over five years of age. J Bone Joint Surg. 1994; 76-B:555–558.
4. Uglow MG, Clarke NMP. Relapse in staged surgery for congenital talipes equinovarus. J Bone Joint Surg. 2000; 82-B: 739–743.
5. Pell RF, Myerson MS, Schon LC. Clinical outcome after primary triple arthrodesis. J Bone Joint Surg. 2000; 82-A:47–57.
6. Legaspi J, Li YH, Chow W, et al. Talectomy in patients with recurrent deformity in club foot: a long-term follow-up study. J Bone Joint Surg. 2001; 83-B:384–387.
7. Lourenco AF, Morcuende JA. Correction of neglected idiopathic club foot by the Ponseti method. J Bone Joint Surg. 2007; 89- B:378–381.
8. Nogueira MP, Batlle AME, Alves CM. Is it possible to treat recurrent clubfoot with the Ponseti technique after posteromedial release?: a preliminary study. Clin Orthop Relat Res. 2009; 467:1298–1305.
9. Culverwell AD, Tapping CR. Congenital talipes equinovarus in Papua New Guinea: a difficult yet potentially manageable situation. Int Orthop. 2007; 33:521–526.
10. Shingade V, Shingade R, Ughade S.Correction of neglected or relapsed clubfoot deformity in an older child by single staged procedure-early results-Curr. Orthop. Practice 2012; 23(2),122-129.
11. Shingade V, Shingade R, Ughade S Single-stage correction for clubfoot associated withmyelomeningocele in older children: early results Curr. Orthop. Practice 2014;25(1),64-70.
12. Dimeglio A, Bensahel H, Souchet P, et al. Classification of clubfoot. J Pediatr Orthop B. 1995; 4-B:129–136.
13. Bensahel H, Kuo K, Duhaime MInternational Clubfoot Study Group Outcome evaluation of the treatment of clubfoot: the international language of clubfoot. J Pediatr Orthop B. 2003; 12- B:269–271.
14. Wilson HA. Bone operations for the correction of club-foot, based upon an analysis of 435 operations by 108 operators. J Bone Joint Surg. 1894; S1-6:159–194.
15. Dekel S, Weissman SL. Osteotomy of the calcaneus and concomitant plantar stripping in children with talipes cavovarus. J Bone Joint Surg. 1973; 55-B:802–808.
16. Tayton K, Thompson P. Relapsing club feet: late results of delayed operation. J Bone Joint Surg. 1979; 61-B:474–480.
17. Addison A, Fixsen JA, Lloyd-Roberts GC. A review of the Dillwyn Evans type collateral operation in severe club feet. J Bone Joint Surg. 1983; 65-B:12–14.
18. Graham GP, Dent CM. Dillwyn Evans operation for relapsed club foot: long term results. J Bone Joint Surg. 1992; 74-B: 445–448.
19. Laaveg SJ, Ponseti IV. Long term results of treatment of club foot. J Bone Joint Surg. 1980; 62-A:23–31.
20. Wynne-Davies R. Talipes equinovarus: a review of eighty—four cases after completion of treatment. J Bone Joint Surg. 1964; 46- B:464–476.
21. Dobbs MB, Nunley R, Schoenecker PL. Long-term follow-up of patients with clubfeet treated with extensive soft-tissue release. J Bone Joint Surg. 2006; 88-A:986–996.
22. Janicki JA, Narayanan UG, Harvey BJ, et al. Comparison of surgeon and physiotherapist-directed Ponseti treatment of idiopathic clubfoot. J Bone Joint Surg. 2009; 91-A:1101–1108.


How to Cite this Article:Shingade VU. “Dorso-Lateral Wedge correction osteotomy: Is this the way forward?”. International Journal of Paediatric Orthopaedics Jan-April 2016;2(1):19-22.

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Use of Illizarov Technique in correction of complex Clubfoot

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 10-18|Ruta Kulkarni1, Rajiv Negandhi1


Authors :Ruta Kulkarni[1], Rajiv Negandhi[1]

 Dept of Orthopaedics, Post Graduate Institute of Swasthiyog Pratishthan, Miraj, Maharashtra.

Address of Correspondence
Dr Ruta Kulkarni
Dept of Orthopaedics, Post Graduate Institute of Swasthiyog Pratishthan, Miraj, Maharashtra, India
Email: rutam@gmail.com


Abstract

Neglected and recurrent clubfoot present with two primary types of deformities namely dynamic deformities and rigid deformities. Soft tissue tightness cause dynamic deformities while bony elements leads to rigid deformities. Deformity correction in cases of clubfoot with ilizarov frame works well if principles of deformity correction are followed well. Dynamic deformity can be stretched out well with soft tissue distraction, while rigid deformity will require bony procedure. Ring fixators can achieve painless, functional plantigrade foot with minimal complications, however patient compliance and acceptability are practical issues. This article reviews various clinical scenarios and provides a detailed management plan using ring fixators.
Keywords: Ilizarov ring fixator, clubfoot, osteotomy, distraction histiogenesis


Introduction
In most children conventional surgical management of idiopathic clubfoot gives good results, but a recurrent deformity requiring further operation may occur. In 10-15 % of children, its treatment depends upon the nature and severity of the foot. A foot which is supple may require recasting or dynamic transfer of tibialis anterior. A rigid foot is likely to require repeated soft tissue procedures and sometimes bony procedures in form of lateral column shortening and calcaneal osteotomy .This can make foot more stiff and shorten already short foot. An alternative is to use the Illizarov technique. Application of this external fixator allows gradual distraction of joints and correction of all aspects of the deformity.
Foot deformities can be corrected by: (1) Soft tissue distraction, and (2) osteotomy distraction by Ilizarov method. Ilizarov apparatus is best suited for complex three-dimensional correction of foot deformities. The foot has multiple joints functioning in different directions with different axis of rotation. When the osteotomy is distracted wedge type of new regenerate bone is formed, correcting the deformity.

Principle of Deformity Correction
Paley has laid down the principles of deformity correction in the book “Principles of Deformity Correction” Springer-Verlag[1,2]. The deformities of the foot are corrected on two principles as promulgated by Ilizarov. Principle of tension stress which states that gradual traction on living tissues stimulates tissue genesis and growth throughout the distraction period. The second principle is the shape forming processes acting upon bone tissue are dependent upon the magnitude of the applied load and the adequacy of blood supply. An increase in the pressure load on a supply to that region results in bone atrophy. If however, the increased load is accompanied by adequate blood supply, the bone hypertrophies according to Wolf’s law remains normal[3,4].

Conventional Surgery
Conventional surgery in most cases has given excellent results and is definitely indicated in uncomplicated cases of foot deformities. The conventional procedures for correction of deformities are: (1) tendon transfers, (2) tendon lengthening, (3) arthrodesis, (4) osteotomies, and (5) soft tissue release.
There are many complications of the conventional operations: (1) the surgery may cause shortening of the foot which is already shorter because of the paralysis, deformity or lack of blood supply, (2) infection may occur which may cause further deformity, (3) pseudarthrosis may occur, (4) the osteotomy may not unite and may cause further stiffness of an already stiff joint, (5) if there is infection, conventional surgery may be associated with complications of osteomyelitis, arthrodesis may occur, and (6) recurrence of deformity is known[5,6].

The Advantages of Ilizarov Method
It is a minimally invasive procedure with minimal dissection, and therefore, decreased risk of neurovascular and soft tissue injury and infection.
This is particularly advantageous in the multiply operated foot.
The Ilizarov method is also not limited by the magnitude of the deformity. Even very severe deformities can be treated by this method.
It allows a comprehensive approach to foot deformity correction by treating not only the foot deformity, but also the associated tibial deformities; leg and foot length discrepancies and even the thin calf can be widened.
Another important advantage is any residual deformity after surgery can be corrected during the postoperative period. It is adjustable even after an acute correction is performed. Achieving a perfectly plantigrade foot in the operating room, whether with an osteotomy or an arthrodesis, is difficult. With the circular external fixator it is possible to obtain the desired correction either acutely in the operation room or gradually after operation.
Non-osteotomy treatment may still be considered in the presence of fixed bony deformity if limited arthrodesis are planned to maintain the correction that is obtained by joint distraction. This reduces the amount of bone that needs to be resected at the time of arthrodesis.4

Disadvantages of Ilizarov Method
Ilizarov method is associated with many complications especially in the foot, because foot has a large number of small joints and axes. Axis of rotation of each joint is different from other joints,
The pain factor: Most of the patients do complain of pain.
The treatment period is lengthy with prolonged joint immobilization. Functional loading, however, including full weight bearing as tolerated, is permitted during treatment. This helps to counteract effects of the prolonged joint immobilization.

Strategies
There are two strategies of Ilizarov method to correct the foot deformity: (1) soft tissue distraction with or without surgical release of the soft tissue. (2) Bony distraction by osteotomy. In this strategy, the distraction occurs through osteotomies, regenerating new bone and eliminating deformities by opening wedge-type corrections.7 The joints remain undisturbed with osteotomy distraction techniques.
The strategy depends on: (1) the age of the patient, (2) the presence of bony deformities—here the deformity is corrected by distracting across joints in an attempt to bring them into a new congruous relationship to a plantigrade position, and (3) the stiffness of the foot.(4) Presence of arthrodesis.

Indications for Soft Tissue and Osteotomy Distraction (Fig 1. & 2)
Paley has given the following guidelines:
Age is an important consideration in deciding non-osteotomy or osteotomy treatment.
According to Paley, non-osteotomy should be done in children below 8 years of age. The deformed foot can be corrected by a soft tissue distraction in children below the age of 8 years. In this group, shape of the foot bones can get remodeled. Soft tissue distraction relies on biologic plasticity of cartilaginous bones. This capability is unreliable in older patients. Cartilage fills the incongruities. During the postoperative period, distraction induces reshaping of bones by activation of the circumferential physis of these bones, leading to a new congruous alignment of the foot bones. The bones adapt to the new position
Adolescents and adults: An older patient with no bony deformity but a soft tissue contracture leading to cavus, equinus, varus, etc. is a good candidate for the soft tissue distraction. If the bones of the foot are congruous only soft tissue distraction can correct the deformity, irrespective of age.
Stiffness of the joint is an important consideration in decision making. When soft tissue distraction is to be done, if the joint is very stiff, there is significant risk of physeal disruption rather than joint distraction. In these cases osteotomy is preferable. Therefore, it is better to grade the stiffness. The author has graded the stiffness into three types: Mild, moderate and severe. Mild grade has a mobile foot, which can be brought to normal foot position from the deformed position. In moderate stiffness, deformed foot is corrected from the deformed position to 30° away from the normal position. In severe stiffness, foot is fixed at 30° or more. Mild and moderate stiffness can be corrected by a soft tissue distraction. Severe degree of stiffness needs osteotomy.
In very stiff deformities, especially as a result of multiple previous surgical attempts, osteotomy should be considered, despite the young age. One contraindication to soft tissue distraction is the presence of limited or extensive arthrodesis. These cases obviously require osteotomy. The soft tissue technique depends on the ability to distract them through multiple joints simultaneously. If these joints are already arthrodesed, this is no longer possible. Thus, the contraindications for soft tissue distraction are: (1) incongruity of the joints after the age of eight, (2) severe stiffness of the foot, and (3) arthrodesis of joints.
Recurrence of a deformity after Ilizarov frame removal is rare in bony corrections (osteotomy), but is common in soft tissue distraction technique, usually due to neurovascular imbalance. An osteotomy in such patients provides a lasting correction through bone instead of joints. Therefore, it is important to consider judicious use of adjunctive muscle balancing surgery (tendon lengthening or transfer) to maintain the correction obtained by the Ilizarov soft tissue method. In many cases, tendon lengthening is done at the time of the Ilizarov frame application.8 Casts are applied immediately on removal of the apparatus, and orthotics is often used long-term to maintain correction.
There are two systems of ilizarov construct to correct the foot deformities: (1) constrained system, and (2) unconstrained system.

In the consConstrained System ( Fig 3A & B)(Fig 4 A&B)trained system, hinges are used so that the movement occurs in one direction only, in the plane of the hinges. It is necessary to find the instant center of rotation of the joint and to perform the correction around this single center of rotation. The advantage in the constrained system is one can mobilize the joint, e.g. in an equinus deformity, the ankle joint can be mobilized with a hinge at the center of rotation of ankle joint. The center of the rotation of ankle is in the lateral facet of the talus in line with the sinus tarsi. While doing the ankle movements, the posterior distraction rod between tibia and hindfoot is removed. This system is particularly applicable to joints such as elbow, knee, ankle and wrist.
According to Grant the constrained system, which is the rule in most other areas of the body, is less applicable to the foot and ankle. When used, it usually corrects a deformity in one plane. The motions of the foot and ankle, however, are usually more complex, most occur through multiple joints and are three dimensional. Thus, a less constrained system has been developed in which the joints of the foot and ankle become the hinges used for correction. Universal hinges are placed on one side of the deformity, and a pulling or pushing device (the motor) is placed on the opposite side. The correction occurs through the joints between the hinge and the motor. If it is desired that the correction of a particular deformity should occur through a specific joint or point, constraints are placed in the system. This is done by positioning olive wires to force a motion to occur on one side or the other of the olive, thus, the place that the movement occurs is controlled.
The constrained system, on the other hand, has to be very precise, and the hinges must be aligned to the joint axis within a narrow range of tolerance to avoid jumping of the joints. In the unconstrained system, it allows the contracture to correct itself around soft tissue hinges and natural axes of rotation of joints. Incorrect hinge placement can also inadvertently lead to joint compression or subluxation or even dislocation. The unconstrained method is advantageous for the treatment of the multiple foot joints that do not have a known simple single axis of rotation and is less advantageous for the treatment of joints such as the ankle, which do have an easy-to-locate axis.

Unconstrained System (Fig.5)
In the unconstrained system, one allows the contracture to correct itself around soft tissue hinges and natural axes of rotation of joints. The advantage of the unconstrained system is that it is simpler to apply and more forgiving.
Treatment of Equinus Deformity
Equinus deformity can be treated by constrained or unconstrained method. Axis of rotation of the ankle lies approximately at the level of the lateral process of the talus. Its axis extends laterally through the tip of the lateral malleolus, and medially below the tip of the medial malleolus.

Constrained Method
The image intensifier is used to locate the axis of rotation of the ankle. Preoperatively, Mose circles are applied to a true lateral image of the ankle to identify the level of the axis of rotation. The center is usually within the lateral process of the talus. The image intensifier is used to obtain a true lateral image of the ankle such that the lateral malleolus is centered over the midlateral tibia. A wire is used to point to the center of rotation. Once the wire overlaps the region of the lateral process, this spot is marked on the skin. The same process should be repeated for both the medial and lateral sides. The image intensifier must be perpendicular to the tibia.
Step 1: Apply a preconstructed two-level frame to the tibia. Use four wires to fix the tibial frame to the leg. The author uses one half pin at 90° to the medial face wire.
Step 2: Suspend hinges from threaded rods off the distal tibial ring. Overlap the hinge with the center of rotation of the ankle joint.
Step 3: Apply the foot frame to the hinges. Adjust the foot frame so that it is parallel to the plantar aspect of the foot. This can be done by placing a board on the plantar aspect of the foot and making sure the foot frame is parallel to the board. A distraction rod off two pivot points such as twisted plate is connected posteriorly in the central hole between the two hinges. Wing nuts are used to connect the posts at either end of the distraction rod. This allows quick application and removal. The patient can combine distraction with removal of the distraction rod for exercise and rehabilitation.

Treatment of Equinus Deformity
Unconstrained Method (Technique—Paley9)
The same tibial base of fixation is used for the unconstrained method as for constrained method, but the foot frame is much simpler. This consists of a half-ring suspended off three threaded rods that are locked by a nut at their proximal end. The maximum posterior tilt of these washers is 7.5°. The half-ring is locked in place at that angle. Two smooth wires are inserted through the heel and fixed and tensioned to the half-ring. Deformity correction is performed by distraction on all three rods in order to pull the heel distally. The reason for the posterior tilt of these rods is that the ankle capsule in equinus runs in a straight line from the back of the talus to the posterior lip of the tibia. When the foot is in the plantigrade position, the line of the ankle capsule is tilted 5–7° posteriorly. This is because the posterior lip of the talus protrudes posterior to that of the tibia. If the rods were not tilted back but were parallel to the tibia, distraction along, that line would pull the ankle capsule directly distally. This would force the talus forward, out of the mortise. When the rods are tilted posteriorly, the talus is pulled back into the mortise.

Varus Deformity: (Technique-Paley)9(Fig 6 A & B)
Heel varus deformity is corrected by the same type of construct as that used in an unconstrained correction of equinus deformity. The difference is that an olive is used on the medial side. The threaded rods are connected via hinges. The posterior threaded rods are connected to a two, three, or four-hole hinge so that the hinge point is proximal to the level of the heel wire. In this way, as the medial side is distracted, because it has to pivot around the hinge, it will translate laterally, forcing the heel out of varus. The rods medially and laterally are connected with a hinge distally and conical washers proximally, or with twisted plates that have pivot points at both ends, or with a mixture of the two. The choice depends on the degree of deformity. Conical washers can adapt only to a 7.5° tilt in either direction. The correction is produced by asymmetrical distraction of all three rods. The medial rod is lengthened at five 0.25 mm adjustments per day, the middle rod at three 0.25 mm adjustments per day, and the lateral rod at one 0.25 mm adjustment per day. In this manner, there is no risk of crushing of the joint surface.

Correction of Foot Deformity by Soft Tissue Distraction 10,11
The Standard Frame
The standard foot assembly consists of the tibial component, the calcaneal component and the forefoot component. The tibial assembly usually consists of two rings. When two rings are used, it becomes stronger support assembly. The level of its attachment depends on the size and complexity of the rest of the frame, the more complex the forefoot and hindfoot components, the higher the level of the supporting components is attached.
The calcaneal component consists of half-ring surrounding the heel. In most cases, both “legs” of this half-ring must be made longer by the firm attachment of the connecting plates. Two cross wires are inserted through the calcaneus and are connected to the half-ring. Only 50–60 kg tension is given. A half-pin passed in the calcaneus posteriorly adds to the stability. The forefoot ring consists of half-ring across the dorsum of the foot. Two wires are passed through the neck of the metatarsals. There are three ways to pass wires through the metatarsals: (1) usually the 1st and 5th matatarsals are used to keep the metatarsal arch, (2) the wires are passed through all the metatarsal pressing 2nd, 3rd, 4th metatarsals plantarwards, bringing all the metatarsals in one line. This gives better stability, and (3) wires are passed through 2nd or 3rd metatarsals and from lateral 5th, 4th or 3rd metatarsals. This is suggested by BB Joshi in JESS system. To do this the 1st and 5th metatarsals are squeezed, while the wire is being inserted. When the wire passes through the cortices of the first metatarsal, drilling is stopped as the wire comes out of the far cortex of the 1st metatarsal. The wire is tapped till it reaches the 5th metatarsal. Then again the wire is drilled through the 5th metatarsal neck as decided and is connected to the second or ring and tensed. Another wire may be passed through the 1st or 2nd metatarsal or through the 5th, 4th and 3rd metatarsals and connected to the ring. This wire maintains the metatarsal arch and gives more strength to the forefoot half-ring.
The forefoot and hindfoot components are connected to the tibial ring using hinges. In some cases, the forefoot component is connected to the calcaneal component by two long plates or threaded rods. The forefoot and hindfoot component may or may not be connected to each other depending on the situation. The connection between the forefoot and hindfoot assemblies is flexible in most cases by using hinges. In some cases, another wire is passed through the mid-tarsal bones, either through the cuboid and navicular or through the talar head. This wire is connected tibial to the distal ring by rods, alter natively this wire is connected to the posts on a plate which is connected to the forefoot half-ring, according to the situation.

Correction of Foot Deformities by Distraction of Osteotomy
Osteotomies around the foot and ankle for distraction are devised by Ilizarov12,13. Paley has classified Ilizarov osteotomies for foot correction into two groups, osteotomies along the long axis of tibia and those along the long axis of foot.
Osteotomies in the long axis of tibia are:
Supramalleolar at metaphysis
Supramalleolar juxta-articular
U-osteotomy through calcaneus and talar neck.
Osteotomies in the long axis of foot are:
V-osteotomy
Posterior calcaneal osteotomy
Talocalcaneal osteotomy
Through talonavicular and calcaneal cuboid joints
Through metatarsals.
Osteotomy in the long axis of tibia will correct all deformities except the deformities between the hind and forefoot such as cavus or the rocker bottom foot and cannot lengthen foot. Therefore, the anatomic relation of hindfoot and forefoot must be normal. Osteotomy in the long axis of foot corrects all deformities of the hind or forefoot, but will not correct deformity at ankle or above, and limb length discrepancy.
U-osteotomy is made through a lateral approach to the hindfoot. U-osteotomy starts behind the subtalar joint, passes under this joint through superior part of the calcaneus across the sinus tarsi and neck of the talus. This is specially indicated when there is a flat top talus or very long-standing equinus deformity. In this situation, the talus is incongruous in the ankle joint and will not enter the ankle mortise because the anterior broad end will not be accommodated in the joint. This osteotomy is able to correct equinus, calcaneus, varus, valgus, and foot height. It is unable to correct deformities between the hindfoot and forefoot like cavus and rocker bottom foot (Fig.7)

V-osteotomy (Fig.8 & 9)
V-osteotomy is a double osteotomy, one osteotomy across the body of the calcaneus posterior to the subtalar joint and one osteotomy across the neck of the talus. The V-osteotomy is used to correct the relation of the hindfoot, midfoot and forefoot, one to the other. The hindfoot with the tuberosity and the Achilles lies posteriorly and the midfoot and forefoot lies anteriorly. This permits angular and rotational correction of the anterior and posterior segments in relation to the middle segment, the leg, and the ground, i.e. varus, valgus, adduction, supination, and pronation.
The V-osteotomy is indicated when there are deformities between the hind and forefoot. A prerequisite for this osteotomy is stiff subtalar joint. Essentially, all foot deformities can be corrected through the V-osteotomy, including hindfoot and forefoot equinus or calcaneus, rocker bottom deformities, cavus deformities, abductus and adductus deformities, and even deformities of length and bony deficiencies of the hindfoot or forefoot.

Supramalleolar Osteotomy (Paley) (Fig.10)
Supramalleolar osteotomies can correct equinus, calcaneus, varus and valgus deformities. The relationship for the hindfoot to the forefoot must be normal if this is to be the sole treatment Indication.
Supramalleolar osteotomies are indicated in the following conditions: (1) Deformities of the metaphyseal and juxta-articular region of the distal tibia, (2) deformity at the ankle level. Ankle may have previous arthrodesis. Deformities at the talus or subtalar joint with ankylosis of the ankle joint. Equinus, calcaneus, varus, valgus, tibial torsion, and leg-length discrepancy can be corrected by this osteotomy.
Advantages
Rotational deformity of the tibia can be corrected.
If the tibia is short, it can be lengthened by distracting this osteotomy.
The supramalleolar osteotomy is technically the easiest of the Ilizarov foot osteotomies as this is a cancellous part.
The supramalleolar osteotomy is particularly useful in the multiply operated foot with poor skin, when the deformity is below the level of the ankle joint. One more advantage of the supramalleolar osteotomy is that is does not compromise motion in the hindfoot joints. It avoids operating on a multiply operated foot in cases where the deformity is below the level of the ankle joint deformity is below the level of the ankle joint.

Disadvantages
It cannot correct the deformity between the hindfoot and forefoot. The most common problem of this osteotomy is the translation of the distal fragment. This is because osteotomy is not done at the true apex of the deformity. This occurs when an angular deformity at one level is corrected at another level. For example, if a distal tibial deformity is at the level of the plafond (juxta-articular) rather than in the metaphysis, a metaphyseal osteotomy leads to a translational deformity. It is necessary to translate the metaphyseal osteotomy in addition to the angular correction.
Paley states that, it is preferable to use the supramalleolar osteotomy to correct only malalignment of the distal tibial articular surface. It can be used to correct deformities at the level of the talus when the ankle joint is very stiff. This leads to a tilt of the plafond that is insignificant when the ankle is very stiff. Because the apex of the deformity is distal to the osteotomy, the supramalleolar osteotomy must be translated, as mentioned previously.

Midfoot Osteotomy14 (Fig. 11 & 12)
Recently since year 2011 we have been doing midfoot and forefoot deformity correction by doing percutaneous midfoot osteotomy. This osteotomy is indicated for complex midfoot and forefoot deformities which are rigid. A percutaneous osteotomy is done at the level of cubid (midfoot). This osteotomy is a joint sparing osteotomy as it does not pass from any joint. A wedge is planned according to nature of deformity (varus /equines/cavus). A wedge is marked with help of 2 k-wires and osteotomy is done between them. After removing the wedge correction is almost complete. Remaining deformity can be corrected with regular non constrained Ilizarov frame. Lengthening of foot can also be achieved through same osteotomy. If the hind foot varus remains uncorrected then a calcaneus slide osteotomy is also added. This is rarely necessary. We find this joint spearing option easier and betterthan U or V osteotomy.

Conclusion
Deformity correction in cases of CTEV with ilizarov frame works well if principles of deformity correction are followed well. Dynamic deformity can be stretched out well with soft tissue distraction, while rigid deformity will require bony procedure. With advent of illizarov Painless, functional plantigrade foot can be achieved.


References

1. Paley D. Compensatory mechanisms and deformity. 2003. p. 596.
2. Paley D. Principles of deformity correction. In: Paley D (Ed). Springer; 2002. pp. 571-645.
3. Frant AD, Atar D, Lehman WB. Ilizarov technique in correction of foot deformities—a preliminary report. Foot and Ankle. 1990;11:1-5.
4. Paley D. The correction of complex foot deformities using Ilizarov’s distraction osteotomies. Clin Orthop. 1993;280.
5. Atar D, Lehman WB, Grant AD. Revision clubfoot surgery. In: Jahss M (Ed). Disorders of the Foot and Ankle. Philadelphia: WB Saunders; 1991. p. 40.
6. Caroll N. Clubfoot. In: Morrissy R (Ed). Lovell and Winter’s Pediatric Orthopaedics. Philadelphia: JB Lippinocott; 1990. pp. 927-56.
7. Ilizarov GA. Clinical application of the tension stress effect for the limb lengthening. Clin Orthop. 1990;250:8.
8. Paley D. Problems, obstacles and complications of limb lengthening by the Ilizarov technique. Clin Orthop. 1990;250:81-104.
9. Paley D. The Principles of deformity correction by the Ilizarov technique technical aspects. Tech Orthop. 1989;4:15-29.
10. Grill F, Franke JL. The Ilizarov distractor for the correction of relapsed or neglected clubfoot. J Bone Joint Surg Br. 1987;69B:593.
11. Herold HZ, Torok G. Surgical correction of neglected clubfoot in the older child and adult. J Bone Joint Surg Am. 1973;55A:1385-95.
12. Ilizarov GA, Shevtosov VI. Treatment of Equino-excavato-varus deformation of the feet in the adults by the Ilizarov transosseous osteosynthesis. Methodological recommendation Book Jurgan Internal Publication, 1987.
13. Lehman WB, Grant AD, Atar D. The use of distraction osteogenesis (Ilizarov) in complex foot deformities. In: Jahss M (Ed). Disorder of the foot and ankle. Philadelphia: WB Saunders, 1991. pp. 2735-45.
14. Ruta M Kulkarni , Anurag Rathore , Rajeev Negandhi , Milind G Kulkarni , Sunil G Kulkarni , Arpit Sekhri. Treatment of Neglected and Relapsed Clubfoot with Midfoot Osteotomy: A Retrospective Study. International Journal of Paediatric Orthopaedics 2015 July-Sep;1(1):38-43.


How to Cite this Article:Kulkarni R, Negandhi R. Use of Ilizarov Technique in correction of complex Clubfoot.  International Journal of Paediatric Orthopaedics Jan-April 2016;2(1):10-18.

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Treatment with Mini External Fixator for Correction of Clubfoot

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 6-9|Sandeep Patwardhan1, Chintan Doshi1


Authors :Sandeep Patwardhan[1], Chintan Doshi[1]

 Sancheti Institute for Orthopaedics and Rehabilitation, Shivaji nagar, Pune, India

Address of Correspondence
Dr Sandeep Patwardhan
Sancheti Institute for Orthopaedics and Rehabilitation, Shivaji nagar, Pune, India
Email: sandappa@gmail.com


Abstract

Background: Clubfoot is one of the oldest and commonest congenital deformities of mankind since man has adopted erect posture [1]. The ideal treatment of clubfoot still remains controversial, because its cause remains unknown, its pathological anatomy is uncertain and its behavior is unpredictable [2]. Few authors concluded that there are different etiological factors responsible for resistance to correction or recurrence after correction. The goal of any type of CTEV management is to reduce, if not to eliminate all elements of the clubfoot deformity, hence achieving a functional, pain free, normal looking plantigrade, mobile, callous free and normally shoeable foot [3]. Treatment of the idiopathic clubfoot by Ponseti method is accepted as a standard treatment when patient presents early [4]. Methods available to correct a clubfoot deformity follow a sequence of treatment which includes manipulation of soft tissues, repositioning of foot, holding the position in POP or by tape. This sequence leads to dynamic functional correction. However it is not always possible to use manipulation by Ponseti for neglected, late presenters and syndromic cases. These deformities can be corrected with the use of external device in the form of universal mini external fixator (UMEX) or a JESS fixator
Keywords: Congenital talipes equino varus, mini fixator, distraction histogenesis


Introduction
Clubfoot is one of the oldest and commonest congenital deformities of mankind since man has adopted erect posture [1]. The ideal treatment of clubfoot still remains controversial, because its cause remains unknown, its pathological anatomy is uncertain and its behavior is unpredictable [2]. Few authors concluded that there are different etiological factors responsible for resistance to correction or recurrence after correction. The goal of any type of CTEV management is to reduce, if not to eliminate all elements of the clubfoot deformity, hence achieving a functional, pain free, normal looking plantigrade, mobile, callous free and normally shoeable foot [3]. Treatment of the idiopathic clubfoot by Ponseti method is accepted as a standard treatment when patient presents early [4]. Methods available to correct a clubfoot deformity follow a sequence of treatment which includes manipulation of soft tissues, repositioning of foot, holding the position in POP or by tape. This sequence leads to dynamic functional correction. However it is not always possible to use manipulation by Ponseti for neglected, late presenters and syndromic cases. These deformities can be corrected with the use of external device in the form of universal mini external fixator (UMEX) or a JESS fixator.

What is a Mini Fixator?
Dr. B. B. JOSHI in 1990 developed a plain unconstrained simple, versatile, cheaper and light fixator system on the basis of biologic law of tissue histiogenesis of all tissues when they are put under gradual stretch. This system is termed as JESS, Joshi’s External Stabilization System. Universal mini external fixator (UMEX) was designed on similar principle. This fixator had a different design of the clamp to enhance stability and fixation. The concept of controlled differential distraction prevents crushing of tissues on the convex lateral side and limb lengthening along with correction of deformity takes place gradually and effectively to achieve supple foot [5].

Where can Mini external fixator be used in CTEV?
Mini external fixator is used for instrumented manipulation in practically almost all cases with CTEV [5]. However with the other non invasive methods like Ponseti method with similar results, mini fixator is now mostly used in late presenters, non idiopathic rigid feet (syndromes) and in cases with post surgical relapses.
Mini external fixator is useful method as the stretching is done four times in a day, repositioning is required once a week, position is hold with use of fixator and application of brace after correction is achieved to maintain correction.

What are the advantages of using mini external fixator?
It is a semi invasive procedure.
Gradual differential distraction allowing simultaneous correction of all the deformities.
Allows for three dimensional control and correction of deformity.
Because of distraction the corrected foot achieved is longer in length.
Excessive cartilage compression and chondrolysis of lateral growing bony structures caused by forceful manipulations is avoided.
It is possible to correct rigid, severe, relapsed clubfoot without shortening of foot.
It has direct purchase over distorted bony anatomy and hence better correction of bony alignment and remodeling.
It adds to tissues by distraction histogenesis as opposed to open surgery which leads to fibrosis and shortening.
Allows for scope of revision and rethinking.

What are the principles of use of universal mini external fixator in CTEV?
The basic principle of universal mini external fixator is the same as advocated by Ilizarov [6]. Physiological tension and stress applied to the tissue stimulates histogenesis of tissues, while controlled differential distraction gradually corrects the deformities and realigns the bones. Correction using mini external fixator is based on understanding that clubfoot deformity has 3 components, the leg, the hindfoot and the forefoot.
Thus it is essential to achieve skeletal hold in each component thus mini fixator system in CTEV correction involves use of 3 blocks the forefoot block, the hindfoot block and the leg block.
Distraction corrects only 1 axis. Differential distraction can correct 2 axis deformity. However to correct a 3 dimentional deformity in CTEV it is necessary to uncouple the distracters from the frame leaving the three blocks intact and manipulate the foot weekly to achieve manual derotation.
Following this the blocks are reconnected using the distracters and distraction protocol is continued over a week. This process is continued till over correction.

Technique of universal mini external fixator application –
The procedure is carried out under general anesthesia with the patient in supine position. The procedure consists of important steps of insertion of pins and formation of blocks and attachment of distracters between the blocks.
Insertion of Pins –
Technique of forefoot pins (Fig 1)–
One transfixing K-wire was passed through the necks of first and fifth metatarsal from lateral to medial side in such a way that the K-wire engaged the two metatarsals. Two additional wires were passed parallel to and 10 to 12 mm apart from either side, one engaging the first and second metatarsals and another engaging the fifth, fourth and third metatarsal. Take precaution that third metatarsal is not transfixed from both sides.

Fig 1. Insertion of forefoot pins. One pin transfixing the 1st and 5th metatarsal heads. Another pin engages 1st and 2nd metatarsals. Third pin engages 5th to 3rd Metatarsals.

Technique of hind foot pins (Fig 2)–
Two parallel K-wires were passed through the tuber of calcaneum from medial to lateral side taking care that they were well away from the course of the neurovascular structures on the medial side. Pins should exactly mimic the deformity. One additional half pin K-wire was passed from the posterior aspect of the calcaneum along the long axis. The entry point was below the insertion of the tendo-achilles in the midline using distractor as the guide.

Fig 2 – Hind foot pin and block. 2 Pins passed from medial to lateral aspect in calcaneum in a direction that mimic the deformity. One axial calcaneal pin from posterior aspect. These pins are connected to form foot block.

Technique of leg pins (Fig 3)–
With the patient in supine position and extended limb, two parallel K-wires were passed in the proximal tibial diaphyses from the lateral to the medial side. The wires were about 3 to 4 cm apart and run parallel to the axis of the knee joint at safe distance distal to tibial tuberosity. The K wires are passed using Z rod as a guide. In older children 3 wires were passed to increase the stability. Additional pin in saggital plane prevents rocking and loosening.

Attaching Connecting rods to complete fixation blocks
Two ‘Z’ rods were attached to the tibial pins, one on either side. The wires were prestressed before the link joints were tightened. Two transverse bars were attached to the ‘Z’ rods, one anteriorly and one posteriorly. Calcaneo-metatarsal distractors were then attached to the K-wires. Two ‘L’ rods were attached to calcaneal K-wires and two other ‘L’ rods were attached to the metatarsal K-wires one on either side with the arms of the ‘L’ rods facing posteriorly and inferiorly. One posterior transverse bar was attached to the posterior calcaneal half pin and the posterior arms of the ‘L’ rods. Two additional transverse rods were attached to the inferior arms of the ‘L’ rods which took the toe sling which provided dynamic traction to prevent flexion contracture of the toes as the deformity was being corrected.

Attach paired distracters (Fig 4) –
Paired distracters were attached between the forefoot block and the hindfoot block. Also another pair of distracter was attached between the hindfoot block and leg block.

Fig 4 – Placement of paired distracters. 2 distractors connecting leg block to hind foot block and 2 distractors connecting hindfoot block to forefoot block

Attach anterior spacer rods –
The transverse anterior rod of the tibial block and metatarsal block was connected on either side with anterior static spacer connecting rod. This provided tension force and kept the anterior portion of the joint open. It also prevented crushing of the articular cartilage and provided better glidage to the talus while correcting the hindfoot deformity of equinus.

Protocol of distraction and correction of deformity –
Distraction phase –
Medial distraction is carried out at a rate of 1/4th turn (0.25mm) four times a day (cumulative of one turn in a day which is 1mm) and lateral distraction is carried out at a rate of 1/4th turn(0.25mm) twice a day (cumulative of half a turn in a day which is 0.5mm)
Manual Repositioning –
Distraction is continued for 1 week following which patient is called for manual repositioning. Manual repositioning is carried out on OPD basis weekly occasionally with sedation if required.
During manual repositioning the distracters are uncoupled from the frame leaving the three blocks intact and the foot manipulated to achieve derotation. Following this the blocks are reconnected using the distracters and distraction protocol is continued over a week. This process is continued till over correction.
Holding phase –
It is important at the end of correction and achieved functional position to stop distraction and hold the corrected position. Holding mode is to continue frame for 6 to 8 weeks after completion of distraction phase

Bracing period –
Following the removal of mini external fixator system at the end of holding phase, the child is put in a brace. Bracing is continued to maintain the corrected position.

The illustration video demonstrates the process of differential distraction and correction of deformity
Fig 5 – Flowchart explaining the method of correction

Problems and Complications [7,8, 16] –
The method of differential distraction using universal mini external fixator also encounters certain problems and difficulties during the procedure. The conditions which need attention during the method are described here.
Flexion or clawing of the toe is seen during the distraction phase due to shortened and stretching of the flexor tendons. This can be managed during the distraction phase by use of straps or footplate. However after removal of the distracters the clawing is markedly reduced.
Acute over distraction needs urgent attention as it causes necrosis. Thus it is mandatory to observe the child at regular intervals.
Another important issue with use of mini external fixator is possibility of pin tract infection. Pin tract infection is managed by observing the foot at regular intervals with periodic pin tract dressings with betadine, tightening of loose screw, use of short course oral antibiotics and in rare cases revision of pin if needed.
Loosening of components is frequently seen when patient is coming on regular follow up. This can be managed by periodic retightening when they come for repositioning.
Compliance is a problem for any type of management in CTEV. The non compliance in relation to distraction protocol, bracing after complete correction will lead to recurrence of the deformity.

Discussion
The goal of any club foot surgery is to obtain a cosmetically acceptable foot, pliable, functional, painless, plant grade foot and to spare the parent and the child from frequent hospitalization and years of treatment with casts and braces [1, 9, and 10]. Physiological tension and stress applied to the tissues stimulates histoneogenesis, while controlled differential distraction gradually corrects the deformities and realigns the bones [11, 15]. External fixators are a versatile method of correcting complex three-dimensional deformities of the foot such as clubfoot. The major difference between the mini fixator or JESS fixators and circular fixators described by Ilizarov was that the wires in this study were not tensioned but only prestressed to prevent them from cutting through the soft bones. Mini external fixators are also lighter in weight, shorter, cheaper, and have an easier application than Ilizarov’s fixators. The absence of hinges also fails to correct rotational deformities [5]. Thus it is required to remove the distractors at regular intervals of distraction and manually reposition the foot and reattach the distractors. This continues till complete correction is achieved.
Correction by distraction has distinct advantage of histoneogenesis, lack of scar tissue formation and the absence of further shortening of the foot. There are many reports of the fixator assisted distractor correction of clubfoot with variations in the technique with good outcome (5 – 8). Suresh et al found JESS to be ideal for correction of residual and relapse clubfoot in their study involving 26 children with 44 clubfeet (7). Similar results were found by Oganesian and Istomina (14). Short-term assessment of results of clubfeet correction with JESS distractor by Anwar and Arun showed excellent and good results in 59.7% of cases (8).
Thus the evidence from various studies show that correction by mini external fixator is a useful method for the management of clubfoot in neglected and resistant cases.


References

1. Ajai Singh , Evaluation of Neglected Idiopathic Ctev Managed by Ligamentotaxis Using Jess: A Long-Term Followup SAGE-Hindawi Access to Research Advances in Orthopedics 2011 :218489 ,6.
2. J. J. Gartland, “Posterior Tibial Transplant in the Surgical Treatment of Recurrent Club Foot,” The Journal of Bone & Joint Surgery, Vol. 46, No. 6, 1964, pp. 1217-1225.
3. K. Ikeda, “Conservative treatment of idiopathic clubfoot,” Journal of Pediatric Orthopaedics, vol. 12, no. 2, pp. 217–223, 1992.
4. I. V. Ponseti and E.N. Smoley, “Congenital clubfoot-the results of treatment,” The Journal of Bone and Joint Surgery, vol. 45, no. 2, pp. 134–141, 1963.
5. Joshi BB, Laud NS, Warrier S, Kanaji BG, Joshi AP, Dabake H. Treatment of CTEV by Joshi’s External Stabilization System (JESS). In: Kulkarni GS, editor. Textbook of Orthopaedics and Trauma. 1st ed. New Delhi: Jaypee Brothers Medical Publishers; 1999.
6. Bradish CF, Noor S. The Ilizarov method in the management of relapsed club feet. J Bone Joint Surg Br. 2000; 82:387-91.
7. Suresh S, Ahmed A, Sharma VK. Role of Joshi’s external stabilisation system fixator in the management of idiopathic clubfoot. J Orthop Surg (Hong Kong) 2003; 11:194-201.
8. Anwar MH, Arun B. Short term results of Correction of CTEV with JESS Distractor. J.Orthopaedics 2004;1:e3
9. Jason A. Freedman, Hugh Watts, and Norman Y. Otsuka , The Ilizarov Method for the Treatment of Resistant Clubfoot: Is It an Effective Solution J Pediatric Orthop 2006; 26:432-437 .
10. Grant AD, Atar D, Lehman WB. The Ilizrov technique in correction of complex foot deformities. Clin Orthop 1992; 280:94-103.
11. Galante VN, Molfetta L, Simone C. The treatment of club foot with external fixation: a review of results – Current Orthopaedics 1995; 9.
12. Wallander H, Hansson G, Tjernström B. Correction of persistent clubfoot deformities with the Ilizarov external fixator. Experience in 10 previously operated feet followed for 2-5 years. Acta Orthop Scand 1996; 67:283-7.
13. Ferreira RC, Costa MT, Frizzo GG, Santin RA. Correction of severe recurrent clubfoot using a simplified setting of the Ilizarov device. Foot Ankle Int 2007;28:557-68.
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How to Cite this Article:Patwardhan S, Doshi C. Mini Fixator for Correction of Neglected Clubfoot.  International Journal of Paediatric Orthopaedics Jan-April 2016;2(1):6-9.

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Neglected clubfoot: Patho-anatomy and clinical features

 Volume 2 | Issue 1 | Jan-Apr 2016 | Page 2-5|Mandar Agashe


Authors : Mandar Agashe [1*]

Director, Centre for Paediatric Orthopaedic Care (CPOC) at Dr. Agashe’s Hospital, Kurla, Maharashtra, India

Address of Correspondence
Dr Mandar Agashe
Centre for Paediatric Orthopaedic Care (CPOC) at Dr. Agashe’s Hospital, Kurla, Mumbai, India.
Email: mandarortho@gmail.com


Abstract

 Neglected clubfoot still remains a great problem in developing countries like India especially in the rural population with limited access to modern healthcare. It is the most common congenital problem leading to locomotor disability, so much so that the government of India has, in recent years added congenital clubfoot in one the eight notifiable conditions at birth with the hope of eliminating the scrouge of neglected clubfoot. With the popularity of the Ponseti method, more and more cases of congenital clubfoot are being treated at the time when they should be ideally treated- ie neonatal period and early infancy. However there are still some lacunae in the healthcare delivery system in India, leading to persistence of these neglected cases in rural population. The obstacles of poverty, lack of awareness and lack of medical facilities in accessible locations means that treatment is either not initiated or incompletely performed.
Since the deformity in neglected cases is a complex three-dimensional one, it is essential to understand the pathoanatomy of the same before embarking on the treatment. In this article, we deal with the detailed pathoanatomy as well as the clinical features related to neglected clubfeet.
Keywords: Congenital talipes equino varus, clubfeet, neglected


Introduction:
Neglected clubfoot still remains a great problem in developing countries like India especially in the rural population with limited access to modern healthcare[1,2]. It is the most common congenital problem leading to locomotor disability, so much so that the government of India has, in recent years added congenital clubfoot in one the eight notifiable conditions at birth with the hope of eliminating the scrouge of neglected clubfoot. With the popularity of the Ponseti method, more and more cases of congenital clubfoot are being treated at the time when they should be ideally treated- ie neonatal period and early infancy[1]. However there are still some lacunae in the healthcare delivery system in India, leading to persistence of these neglected cases in rural population. The obstacles of poverty, lack of awareness and lack of medical facilities in accessible locations means that treatment is either not initiated or incompletely performed[3,4].
Since the deformity in neglected cases is a complex three-dimensional one, it is essential to understand the pathoanatomy of the same before embarking on the treatment. In this article, we deal with the detailed pathoanatomy as well as the clinical features related to neglected clubfeet.

Pathoanatomy
The pathological anatomy of the neglected clubfoot can be divided into that related to bone and joints and that related to soft tissues (ligaments, muscles and tendons).

Bones and joints[5,6] [Figures 1,2,3]
The talus is the bone which is most affected in neglected clubfoot [blue arrow]. The talus is severely plantarflexed. The body is small and altered in shape. The talus becomes severely inverted in the ankle mortice. The trochlea is shorter than normal. Only the posterior portion of the talus articulates with the tibia in the ankle joint while the anterior portion is just covered with thin and stretched out ankle joint capsule. Normally, the posterior-most portion of the talus is extra-articular. However in neglected clubfeet with severe planter-flexion, the posterior portion of the talus becomes intra-articular and is covered by joint capsule. The neck of the talus develops significant medial angulation and the head becomes wedge shaped. There are two surfaces of the talus- the anterolateral and anteromedial. The anterolateral surface of the talus is left uncovered by the medially displaced navicular and is now covered by just thin joint capsule which is stretched out, and the skin. This is the part which is palpable just underneath the skin. The anteromedial surface now articulates with the navicular which is displaced medially and proximally.
The navicular is also very severely affected in neglected clubfeet [white arrow]. It is flattened or wedge-shaped and medially displaced, adducted and inverted. It articulates with the anteromedial surface of the talus and in some severe cases, even has a pseudo-articulation with the medial malleolus. The enlarged tibialis posterior is attached to a wide area on the navicular along with the medial malleolus.
The calcaneus also undergoes a significant alteration [black arrow]. The body of the calcaneus is severely plantarflexed and medially bowed. It is adducted and the lateral process of the calcaneum is under the talus, rather than being lateral to it. In severe neglected cases, the body of the calcaneus also undergoes severe changes, leading to it being bean-shaped rather than rectangular on the axial plane. The axes of the calcaneum and talus are parallel to each other. The cuboid is also medially displaced at the calcaneo-cuboid joint so that only the medial surface of the anterior process of the calcaneum articulates with the cuboid.
The cuneiforms and metatarsals also undergo secondary changes though to a lesser extent than the talus, calcaneum and navicular. The cuneiforms are medially displaced and have very haphazard articulations with the navicular and the cuboid. The metatarsals are shortened.
The talonavicular joint is entirely medially displaced. This articulation is between the anteromedial surface of the talus and the proximal surface of the navicular. There develop pseudoarticulations between the navicular and the tibia (at the medial malleolus) and also between the navicular and the calcaneum. In some cases, there is a fibrous band which is formed between the navicular and the calcaneum, almost like a fibrous calcaneonavicular bar.
The three talcalcaneal joints (anterior, middle and posterior) are also very abnormal. The anterior facet remains very narrow while the middle facet is variable. The posterior facet is short. The sustentaculum tali is left uncovered to a large extent. In very severe neglected clubfeet, the talo-calcaneal joints are almost in the weight bearing axis due to the severe inversion so that the talus assumes the weightbearing position.
The calcaneocuboid joint orientation is very important for the treatment of neglected clubfeet. The calcaneocuboid joint is very obliquely placed with the cuboid being subluxed anteriorly and medially while the lateral aspect of the calcaneum is left uncovered. The other joints of the foot (ie the intercuneiforms and intermetatarsals) follow the course of the main joints of the midfoot and hindfoot. The point to remember is that the first two metatarsals are severely pronated while the lateral three metatarsals are supinated. The degree of pronation will determine the cavus, which in turn will determine the fate of the foot in terms of whether the child walks on the lateral border or the dorsal surface.

Pathoanatomy of soft tissues (ligaments, muscles and tendons)[5-7]
The soft tissues in clubfeet also undergo a significant amount of secondary changes. They are subclassified as:
1) Changes in muscles:
The triceps surae and the tendoachilles is the most severely affected amongst all muscle tendon units in neglected clubfeet. The severity of neglected clubfoot depends mainly on the amount of shortening of the tendoachilles.Both the triceps surae and the tendoachilles are foreshortened and the tendon is significantly longer than the muscle. The tendoachilles is inserted medially on the calcaneus and is one of the deforming forces to pull the heel in varus.
Amongst the other muscle tendon units affected, the tibialis posterior is very important. The tibialis posterior becomes very broad and hypertrophied and is inserted over a very broad area on the inferomedial surface of the navicular and the medial cuneiform. This is the most important cause of supination of the foot. In some very severe cases, the tibialis posterior also seems to have an additional thick fibrous band which is attached to the cuboid, which pulls the cuboid medially. This itself leads to the significant displacement of the calcaneocuboid joint, which is seen in some cases.
The tibialis anterior undergoes some amount of hypertrophy. This is more apparently seen in incompletely treated clubfeet, in which the tibialis anterior causes a dynamic supination deformity during the swing phase of gait. The other muscles of the flexor compartment of the foot also undergo foreshortening with resultant clawing.
2) Changes in ligaments and other soft tissues:
The plantar fascia is very tight and results in the cavus component of the deformity. The amount of tightness of the plantar fascia and the cavus, decide how the child walks in neglected clubfeet[4-6]. If the cavus is moderately severe, the child walks on the lateral border of the foot. However if the cavus is exceedingly severe, the child starts walking on the dorsal surface of the foot, making normal ambulation exceedingly difficult.
In the ligaments, the deep layer of the deltoid ligament is very thick and forms a part of the pseudoarticulation between the medial malleolus and the medially displaced navicular. The tibianavicular and the calcaneonavicular ligaments are thickened and shortened. The ligaments on the medial, plantar and the posterior aspect of the foot undergo severe thickening with shortening and are the main tethers against effective closed manipulative treatment of neglected feet. Amongst them, the medial talo-calcaneal ligament and the plantar calcaneonavicular ligaments are very important. The eponymous “knot of Henry” becomes a thick fibrous band which extends from the undersurface of the navicular till the plantar surface of the medial cuneiform and the talus in neglected clubfeet.
Thus to conclude, neglected clubfoot is a three dimensional deformity, with severe secondary changes in bones, muscles, tendons and ligaments, all of whom have to be corrected for effective clinical management.

Clinical features
The neglected clubfoot, by definition is a foot which has experienced no or minimal surgical or non-surgical management[6] [figure 4]. In this case, the deformity starts increasing after the child starts weightbearing. This is because the structures which are never meant to bear the weight of the body are now in a weight bearing position, the weightbearing starts happening on the side and dorsum of the foot and secondary contractures start developing on the plantar-medial and posterior side of the foot. There is usually a large bursa or callosity at the lateral aspect of the foot, which on prolonged weight-bearing leads to skin breakdowns, ulcerations and infections. It is often difficult or impossible to wear normal shoes for ambulation.

Patterns of deformity[6]
Just like clubfoot deformity in the neonatal and infantile period, there are different patterns of deformity in a neglected clubfoot. Though all the major components of the deformity, ie cavus, forefoot adductus, heel varus and equinus are present in all patients, the exact combination is the main causative factor for a particular type of deformity. As a result, there are various feet with different grades of stiffness, mobility and deformity. This led a few investigators to subclassifiy the deformities in neglected clubfeet into three patterns of deformity since the treatment modality followed and prognosis depends on each of these patterns.
1) Moderately flexible: The foot can be considerably corrected to neutral position
2) Moderately stiff: There is some correctability but not to neutral position and with moderate deformity persisting
3) Rigid: There is almost no correction of deformity.
The classification can be applied either to the entire foot, or separately to the midfoot and hindfoot. Of course, the standard classifications of Pirani[8] and Dimeglio[9] can still be used additionally.
As explained previously, the degree of cavus determines whether the child walks on the lateral border of the foot or the dorsum of the foot. In lesser degree of cavus, the child walks on the lateral border while in greater degree of cavus the child walks on the dorsal surface itself. In the presence of this severe cavus, it is very difficult to appreciate the amount of equinus actually present. This usually gets unmasked once the cavus and adductus gets corrected.

Socio-economic factors
The kids with neglected clubfeet eventually learn to walk with no or some modified footwear[6]. However, it is a condition which is fraught with numerous struggles. This deformity poses a significant disability in young children, preventing access to education and social activity[10]. Many of them are outcast or even deemed as “cursed” with very little social interaction due to the obvious grotesque deformity[6]. Social stigma of having “reverse feet” is also very great, and many girls find it difficult to get married due to this[4]. They are unable to squat for toileting purposes which is an essential function especially in rural population[3,4]. There is significant pain, difficulty in locomotion over long distance, with frequent skin breakdowns, infections and callosities. The skin breakdowns and infections can sometimes be so severe that it may lead to amputations.

Conclusion
Congenital clubfoot is a complex problem which requires proper understanding of the anatomy as well as realistic goals for treatment before embarking on attempting to treat it.


References

1. Gadhok K, Belthur MV, Aroojis AJ, Cook T, Oprescu F, Ranade AS, Morcuende JA. Qualitative assessment of the challenges to the treatment of idiopathic clubfoot by the Ponseti method in urban India. Iowa Orthop J. 2012;32:135-40.
2. Lohia LK, Meena S, Kanojia RK Comparative study of complete subtalar release and Joshi’s external stabilization system in the management of neglected and resistant idiopathic clubfoot..Foot Ankle Surg. 2015 Mar;21(1):16-21.
3. Shingade V, Shingade R, Ughade S Single-stage correction for clubfoot associated With myelomeningocele in older children: early results Curr. Orthop. Practice 2014;25(1),64-70.
4. Shingade V, Shingade R, Ughade S. Correction of neglected or relapsed clubfoot deformity in an older child with single stage procedure: Early results. Curr. Orthop Practice. 2012; 23(2), 122-129.
5. Ponseti IV. Congenital clubfoot: Fundamentals for treatment. Oxford: Oxford University Press: 1996.
6. Penny JN. The neglected clubfoot. Techniques in Orthopaedics Vol 20. Philadelphia: Lippincott Williams & Wilkins Inc.; 2005: 153–166.
7. Scott WA, Hosking SW, Caterall A. Clubfoot. Observations on the surgical anatomy of dorsiflexion. J Bone Joint Surg (Br) 1984;66: 71-6.
8. Pirani S. A reliable and valid method of assessing the amount of deformity in the congenital clubfoot. Presented at the Pediatric Orthopaedic society of North America, May 2004; St. Louis.
9. Demiglio A, Bensahel H, Souchet P, et al. Classification of clubfoot. J Pediatr Orthop (B) 1996;4:129-136.
10. Owen RM, Penny JN, Mayo A, Morcuende J, Lavy CB. A collaborative public health approach to clubfoot intervention in 10 low-income and middle-income countries: 2-year outcomes and lessons learnt. J Pediatr Orthop B. 2012 Jul;21(4):361-5.
11. Sengupta A. The management of congenital talipes equinovarus in developing countries. Int Orthop 1987;11: 183-187.


How to Cite this Article: Agashe M. Neglected clubfoot: Patho-anatomy and clinical features. International Journal of Paediatric Orthopaedics Jan-Apr 2016;1(2):2-5.          

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