Volume 2 | Issue 2 | May-Aug 2016 | Page 34-37|Dhurvas Ramlal Ramprasath, Vasudevan Thirunarayanan, Murugan Shanmuga Sundaram

Authors :Dhurvas Ramlal Ramprasath [1], Vasudevan Thirunarayanan [1], Murugan Shanmuga Sundaram [2]

[1] Senior Assistant Professor, Department of Orthopaedic Surgery, Government Royapettah Hospital,Westcott road,Royapettah,Chennai,India-600014.
[2] Junior Resident, Department of Orthopaedic Surgery, Government Royapettah Hospital, Chennai, India- 600014.

Address of Correspondence
Dr Ramprasath D.R.,
12/23, Murugappa street, Purasawakkam, Chennai, India. PIN- 600007.
Email ID: dhurvasramprasath@gmail.com

Abstract

Several osteotomies are available to correct cubitus varus and valgus deformities in children. The purpose of this study was to evaluate clinical and radiological outcome following Kim’s step cut translation osteotomy for such deformities.
Materials and Methods: We have instituted Kim’s step cut translational osteotomy in seven children having deformities of elbow (cubitus varus – 4 and cubitus valgus – 3). Patients were followed up for a period of 8 to 14 months during the period of August 2014 to October 2015. Clinically and radiologically, preoperative and postoperative Humerus-Elbow-Wrist angle, Range of motion of elbow, Lateral/Medial Prominence Index and neurological examination for ulnar nerve were determined. Results were evaluated according to modified Oppenheim et al criteria.
Results: The mean postoperative Humerus-Elbow-Wrist angle in patients with cubitus varus was 8.5±2.06 0 (range, 50 to 100). The mean improvement in Lateral Prominence Index was 7.4 ±1.28% (from –13.15% to -5.75% ). In cubitus valgus patients, mean
postoperative Humerus-Elbow-Wrist angle was 12.33±3.510(range, 80 to 150) . The mean improvement in MPI was 8.7±0.83% (from -15.5% to -6.8% ) . In all patients range of motion was comparable with normal side elbow. Bone union was achieved in all patients. According to Oppenheim’s criteria, six patients had excellent results and one patient had good result. .None of them had any complications.
Conclusion: Even though multiple procedures are available for correcting deformities of elbow, Kim’s step cut translational osteotomy provides good correction angle, lesser prominence of the condyle, better stability and three dimensional correction.
Key Words: Cubitus varus, Cubitus valgus, Kim’s osteotomy, Lateral/ Medial prominence Index.

Introduction
Cubitus varus and valgus deformities are complications of elbow fractures in children[1]. Cubitus varus has multiple components that include varus malalignment, hyperextension and internal malrotation[2,3]. The most important indication for osteotomy is to achieve a good cosmesis[2,4]. Many surgical techniques have been described to correct these deformities including closing wedge, opening wedge, dome and step cut osteotomies[5-12]. The closing wedge osteotomy has a tendency to produce prominent condyles after correction,often compromising the cosmetic outcome[2,13-16]. The inclusion of translation in the osteotomy improves cosmetic appearance by minimizing the persistent prominence of the medial or lateral condyle. This can be achieved by Kim’s osteotomy. The aim of our study was to evaluate clinically and radiologically, the preoperative and postoperative Humerus-Elbow-Wrist(HEW) angle, Lateral/Medial Prominence Index(LPI/MPI), Range of Motion(ROM), in children undergoing Kim’s step cut translation osteotomy for cubitus varus and valgus.

Materials and Methods
This is a retrospective study, from August 2014 to October 2015, involving seven paediatric patients in the age group 8 – 14 years with male:female ratio of 4:3. Those with cubitus varus deformity had sustained supracondylar humerus fracture and those with valgus deformity had fracture lateral condyle. All the patients had undergone native treatment immediately after injury, and presented to our department after a period of 14 months to 34 months after injury.
We have instituted step cut translational osteotomy of Hui Taek Kim [1] in all the seven patients. Preoperatively, radiological and clinical planning includes measurement of HEW angle, lateral/medial prominence index (using the method described by Wong et al [2,16]),range of motion of elbow, neurological examination for ulnar nerve and internal rotation malalignment (using the method described by Yamamoto[17,18]). Same radiological and clinical parameters were evaluated postoperatively.
HEW angle was measured by drawing two lines, one line along the anatomical axis of humerus ,and another line joining midpoints of two transverse lines(one proximal and one distal) across the forearm that connected the medial cortex of ulna and lateral cortex of radius (Fig-1). The Lateral/Medial Prominence Index was measured by using the formula shown in (Fig-2).
We determined the Correction Angle (CA) for patients with cubitus varus by adding varus HEW angle with normal side HEW angle, and for patients with cubitus valgus by subtracting normal side HEW angle from affected side valgus HEW angle. A template using X-ray film, was prepared preoperatively, to mark the site and size of osteotomy, using following technique(Fig-3).
The outline of the bone was drawn on a trace paper. A horizontal line was drawn perpendicular to anatomical axis of humerus at a level 0.5 to 1 cm proximal to the olecranon fossa. Now the trace paper was cut along the horizontal line and the distal fragment was rotated laterally and translated medially(in case of cubitus varus) so as to achieve HEW angle of normal side. Vice versa was done for cubitus valgus deformity. An inverted V was marked on the trace paper. We then cut out the triangular overlapping area from the paper and prepared X-ray film of same size and shape. This triangular X-ray film was sterilised for
use during osteotomy.

Figure 1: Humerus-Elbow-Wrist (HEW) Angle

Figure 2: Lateral/Medial Prominence Index % (LPI/MPI) LPI (%)=(AB-BC)/AC×100 LPI (%)=(AB-BC)/AC×100

Figure 3: Preop Templating

Method of Osteotomy
With the patient in lateral decubitus position,through posterior approach ,ulnar nerve was isolated and protected. The triceps aponeurosis was split. The triangular X-ray template (turned face downward because of posterior approach) was placed over the bone 1cm proximal to olecranon fossa and necessary osteotomy was done to remove an identical piece of bone. The distal fragment was rotated laterally for cubitus varus(and medially for cubitus valgus) and inserted into the inverted V shaped defect. The deformity correction was assessed clinically and then the fixation was done with distal radius T-Plate and 3.5mm cortical
screws. The ulnar nerve was transposed anteriorly in patients who had tardy ulnar nerve palsy due to cubitus valgus deformity.
Patient was immobilised in long arm slab for 2 weeks following which active and assisted mobilisation was done intermittently retaining the splint until radiological union was achieved.

Results
The HEW angle, LPI/MPI, ROM were measured and analysed (Table -1).

Table-1: Preoperative and Postoperative Measurements.
HEW- Humerus-Elbow-Wrist angle, LPI /MPI – Lateral/Medial Prominence Index, CA- Correction Angle

In cubitus varus patients, mean postoperative HEW angle was 8.50(range, 50to 100),with mean correction of 21.50(range,190 to 250). The mean improvement in LPI was 7.4% (from -13.15% to -5.75% ) .In cubitus valgus patients(Fig-5), mean postoperative HEW angle was 12.330(range, 80 to 150),with mean correction of 21.660(range,200 to 230). The mean improvement in MPI was 8.7% (from -15.5% to -6.8% ).In all patients, range of motion was comparable with normal side elbow (Table 1). Pronation and supination movements were normal in all our cases. Bone union was achieved in all patients.

Figure 4: Osteotomy and Fixation

Figure 5: X-ray(Preop and Postop)

According to Oppenheim’s criteria [7], excellent result (Fig-6) was achieved in 6 patients and good result in one patient, and no patient had poor result.

Figure 6: Lateral/Medial Prominence Index % (LPI/MPI)

Discussion
In patients with cubitus varus/valgus, the following problems need to be addressed deformity correction in coronal plane(valgus/varus), sagittal plane(fixed flexion/hyperextension),horizontal plane(internal/external rotation deformity); ulnar nerve palsy, if any.
The deformity is better corrected during childhood. Correction, particularly in cubitus varus, in adult is challenging due to mature skeleton, inherent instability at osteotomy site, risk of delayed union/non union, implant failure, infection, stiffness and neurovascular complications [19]. A rough estimate will be around a year after original injury. Again patient demands, growth potential and status of physis should be taken into account while planning surgery[20].
Major types of osteotomies are – simple closing wedge [17,21,22],step cut translation [1,17,23], dome rotational osteotomy[13,14,17,24] and spike translation osteotomy[17]. Many of these osteotomies have got their own disadvantages, like lateral scar, medial and lateral condylar prominence and difficulty in correcting rotational deformities( due to contractures)[1,24,25,26].
Various method of fixation include use of K-wires, screws, plates and external fixators (Ilizarov technique) [5,13,25,27-29]. We have used Kim’s method of step cut translation Osteotomy, and fixed with distal radius T-plate.
This method has got multiple advantages. Adequate Correction Angle (CA) is achieved by moving the apex more medially (in cubitus varus) or laterally (in cubitus valgus). The stability of fixation is enhanced because the distal fragment is inserted into the inverted V shaped proximal fragment and fixation was done with plates. The prominence of condyles (lateral condyle in cubitus varus and medial condyle in cubitus valgus) is less with Kim’s osteotomy (when compared to other methods ) because distal fragment is translated. With Kim’s osteotomy, three dimensional correction is possible. The correction of internal rotation is recommended when the difference in rotational alignment in both sides is greater than 100 [1,17,18]. In our study ,we did not encounter patient with hyperextension, or internal rotation more than 100, when compared to normal side. Hence we have not attempted correction in sagittal/horizontal planes.
The limitations of our study is smaller sample size and follow up of only 14 months duration.

Conclusion
This simple step cut translation osteotomy(Kim’s) results in good cosmetic deformity correction,very firm fixation,earlier elbow movement and also avoids problems of condylar prominence and non union. Deformities in sagittal/horizontal plane can also be corrected.

References 

1. Hui taek kim, md, Jung sub lee, md, and Chong il yoo, md. Management of cubitus varus and valgus. The journal of bone & joint surgery.Volume 87-a , number 4, april 2005.
2. K. Bali , P. Sudesh, V. Krishnan, A. Sharma, S.R.R. Manoharan, A.k. Mootha. Modified step-cut osteotomy for post-traumatic cubitus varus: our experience with 14 children. Orthopaedics & traumatology: surgery & research (2011) 97, 741-749.
3. Takagi T, Takayama S, Nakamura T, Horiuchi Y, Toyama Y,Ikegami H. Supracondylar osteotomy of the humerus to correct cubitus varus: Do both internal rotation and extension deformities need to be corrected? J bone joint surg am 2010;92(7):1619-26.
4. Pankaj A, Dua A, Malhotra A, Bhan S. Dome osteotomy for posttraumatic Cubitus varus a surgical technique to avoid lateral condylar prominence. J pediatr orthop 2006;26(1):61-6.
5.Y.H. Yun,S.J. Shin,J.G. Moon. Reverse v osteotomy of the distal humerus For the correction of cubitus varus. J bone & joint surg [br].2007;89-b:527-31.
6.Derosa GP, Graziano GP. A new osteotomy for cubitus varus. Clin orthop 1988;236:160-5.
7. Kanaujia RR, Ikuta Y, Muneshige H, Higaki T, Shimogaki K. Dome osteotomy for cubitus varus in children. Acta orthop scand 1988;59:314-17.
8. Kim HS, Jahng JS, Han DY, et al. Modified step-cut osteotomy of the humerus. J Pediatr orthop b 1988;7:162-6.
9. Koch PP, Exner GU. Supracondylar medial open wedge osteotomy with external fixation For cubitus varus deformity. J pediatr orthop b 2003;12:116-22.
10. Laupattarakasem W, Mahaisavariya B, Kowsuwon W, Saengnipanthkul S. Pentalateral osteotomy for cubitus varus: clinical experiences of a new technique. J Bone joint surg [br] 1989;71-b:667-70.
11. Oppenheim WL, Clader TJ, Smith C, Bayer M. Supracondylar humeral osteotomy For traumatic childhood cubitus varus deformity. Clin orthop 1984;188:34-9.
12. Uchida Y, Ogata K, Sugioka Y. A new three-dimensional osteotomy for cubitus Varus deformity after supracondylar fracture of the humerus in children. J pediatr Orthop 1991;11:327-31.
13. Bellemore MC, Barrett IR, Middleton RW, Scougall JS, Whiteway DW. Supracondylar osteotomy of the humerus with correction of cubitus varus. J bone joint surg br 1984;66(4):566-72.
14. Matsushita t, Nagano a. Arc osteotomy of the humerus to correct cubitus varus. Clin orthop relat res 1997;336:111-5.
15. Tien YC, Chih HW, Lin GT, Lin SY. Dome corrective osteotomy for cubitus varus deformity. Clin orthop relat res 2000;380:158-66.
16.Wong HK, Lee EH, Balasubramaniam P.The lateral condylar prominence. A complication of supracondylar osteotomy for cubitus varus. J bone joint surg br 1990;72:859-61.
17. Ali Moradi MD, Ehsan Vahedi MD,Mohammad H.Ebrahimzadeh MD. Spike Translation: A New Modification in Step-cut Osteotomy for Cubitus Varus Deformity. Clin Orthop Relat Res (2013) 471:1564–1571.
18. Yamamoto I, Ishii S, Usui M, Ogino T, Kaneda K. Cubitus varus deformity following supracondylar fracture of the humerus: a method for measuring rotational deformity. Clin Orthop Relat Res. 1985;201:179–185.
19. S Pandey, A Shrestha, AP Regmi, A Prajapati, S Dhakal and G Neupane. Cubitus varus in young adults correction with lateral closing wedge osteotomy and screw, k-wire and ss-wire fixation.Journal of Chitwan Medical College; 2012, 1(2); 60-62.
20. Sandeep patwardhan , Ashok k shyam. Cubitus varus deformity – rationale of treatment and methods. International journal of paediatric orthopaedics | volume 1 | issue 1 | july-sep 2015 | page 26-29.
21. Graham B, Tredwell SJ, Beauchamp RD, Bell HM. Supracondylar osteotomy of the humerus for correction of cubitus varus. J Pediatr Orthop. 1990;10:228–231.
22. Voss FR, Kasser JR, Trepman E, Simmons E Jr, Hall JE. Uniplanar supracondylar humeral osteotomy with preset Kirschner wires for posttraumatic cubitus varus. J Pediatr Orthop. 1994;14:471–478.
23. Davids JR,Lamoreaux DC, Brooker RC,Tanner SL, Westberry DE.Translation step-cut osteotomy for the treatment of posttraumatic cubitus varus.J Pediatr Orthop. 2011;31:353-365
24. Labelle H, Bunnell WP, Duhaime M, Poitras B. Cubitus varus deformity following supracondylar fractures of the humerus in children. J Pediatr Orthop. 1982;2:539–546.
25. French PR. Varus deformity of the elbow following supracondylar fractures of the humerus in children. Lancet. 1959;2:439-41.
26. King D, Secor C. Bow elbow (cubitus varus). J Bone Joint Surg Am. 1951; 33:572-6.
27. Carlson CS Jr, Rosman MA. Cubitus varus: a new and simple technique for correction. J Pediatr Orthop 1982;2:199-201.
28. Levine MJ. Horn BD, Pizzutillo PD. Treatment of posttraumatic cubitus varus in pediatric population with humeral osteotomy and external fixation. J Pediatr Orthop 1996;16:597-601.
29. Karatosun V, Alekberov C, Alici E, Ardic CO, Aksu G. Treatment of cubitus varus using the Ilizarov technique of distraction osteogenesis. J Bone Joint Surg [Br]2000;82-B:1030-3.

Dr Dhurvas Ramlal Ramprasath

Dr. Vasudevan Thirunarayanan

Dr. Murugan Shanmuga Sundaram

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Volume 2 | Issue 2 | May-Aug 2016 | Page 24-28|Donald S Bae

Authors : Donald S Bae [1]

[1 ] Dept of Orthopaedic Surgery 300 Longwood Avenue, Hunnewell 2 Boston, MA 02115.

Address of Correspondence
Dr Donald S. Bae
Associate Professor of Orthopaedic Surgery
Harvard Medical School
Department of Orthopaedic Surgery 300 Longwood Avenue, Hunnewell 2 Boston, MA 02115
Email: Donald.bae@childrens.harvard.edu

Abstract

Radial longitudinal dysplasia continues to challenge the pediatric hand and upper limb surgeon. Despite decades of clinical experience and research, surgical strategies to improve wrist alignment and upper limb function remain imperfect, with persistent concerns regarding recurrent deformity, stiffness, and distal ulnar growth disturbance. For these reasons, many authors have proposed alternative treatment paradigms to traditional centralization techniques. The purpose of this review is to explore the arguments for surgical treatment, to identify the potential sequelae of traditional centralization, and to present an “alternative treatment paradigm” for radial longitudinal dysplasia.

Key Words: Radial longitidinal dysplasia, centralization, bilobe flap

Introduction
Radial longitudinal dysplasia (RLD) refers to failure of formation of the radial aspects of the hand and forearm, and is currently classified as an abnormality of formation and differentiation of the antero-posterior axis of the developing upper limb by the Oberg-Manske-Tonkin classification system [1].The incidence is thought to be between 1 in 30,000 and 100,000 live births , and RLD has a slight male and right-sided predisposition. Over half of patients have bilateral upper limb involvement.
There is a broad spectrum of anatomic anomalies involving the skeletal, muscular, and neurovascular structures, and associated syndromes are common, including VACTERL association, Holt-Oram syndrome, Cornelia de Lange syndrome, thrombocytopenia-absent radius, Diamond-Blackfan anemia, Rothmund-Thomson syndrome, and Fanconi’s anemia [2,3,4,5]. A host of condition-specific schemes have been proposed, based mostly upon the severity of bony involvement, and to date the modified Bayne and Klug classification continues to be used by most providers [6,7].
Overall, the goals of treatment include maximizing upper limb and hand function, ideally by preserving wrist motion, improving wrist alignment, maintaining limb length and growth potential, and preserving the ability for subsequent hand reconstruction and pollicization. Furthermore, the aesthetic differences –and secondary effects thereof– imparted by RLD must be recognized and considered in an appropriate fashion.
A host of questions continue to be raised with the growing experience of RLD treatment, particularly with longer term follow-up and patient-derived functional outcome measures. What is the natural history of RLD? Is surgical treatment for deformity correction worthwhile? And if so, what is the best method of treatment to maximize function and minimize complications?
The purpose of this review will be to address these questions using the available information from India and the United States, and in doing so, present an alternative treatment algorithm for patients with RLD.

Natural History versus Surgical Reconstruction
With any congenital difference, the first fundamental task of the pediatric orthopaedic surgeon is to understand the natural history of the condition and the comparative outcomes of natural history versus surgical treatment. Kotwal et al. published an important paper seeking to address this fundamental question as it relates to RLD [8]. One hundred and three patients with RLD treated non-operatively were compared to 239 patients treated with surgical reconstruction. All patients had Bayne type 3 or 4 deficiency and were treated at a single center from 1985 to 2004. Patients were assessed according to standard clinical parameters, radiographic measurement of the hand-forearm angle (HFA), and visual analogue scores (VAS) for pain and aesthetic appearance. Grip strength was evaluated using dynamometry, though pinch strength was not measured due to the heterogeneity of hand/thumb involvement. The Prosthetic Upper Extremity Functional Index (PUFI), originally developed to evaluate function in patients with transverse upper limb deficiencies, was used to assess upper limb function.
Non-operative treatment consisted of early stretching, serial casting, splinting, and.or functional bracing. Centralization consisted of centralization (n=202) or radialization (n=107) via an ulnar incision, excising redundant ulnar skin, notching the carpus, reefing the wrist joint capsule, preserving the distal ulnar physis, and stabilizing with a 1.6mm Kirschner (K-) wire [9,10]. Tendon transfers were performed in selected cases. Radialization was performed in a similar fashion, though tendon transfers were more commonly performed and K-wire fixation was achieved through the index metacarpal [11].
Overall, patients with type 3 and 4 RLD treated with surgical reconstruction demonstrated better PUFI scores, had improved HFA, and reported improved aesthetics than non-operatively treated patients. However, surgical patients also had decreased wrist range of motion (ROM), and interestingly functional results as assessed by the PUFI correlated best with digital ROM, regardless of treatment type. Ekblom et al. similarly reported that digital and wrist ROM is more important for activity and participation than HFA in their analysis of 20 patients with RLD [12]. Holtslag et al. similarly found that only ROM of the didgits correlated with function, as assessed by the Sequential Occupational Dexterity Assessment [13]. No significant differences in activity and participation between patients with mild versus severe RLD. Thus, while there is published information suggesting that surgical treatment provides functional, alignment, and aesthetic improvements that are superior to natural history, it is important to remember the importance of wrist and digital ROM in upper extremity and hand function.

Concerns with centralization
Surgical treatment in the form of centralization or radialization is not without concerns. First the longevity of correction and functional improvement continues to be debated. In their longer-term follow-up study of 25 wrists treated with centralization, Goldfarb et al. reported recurrent deformity (HFA of 25 degrees), limited wrist ROM (mean arc 31 degrees) and forearm shortening (ulnar length 54%), perhaps due to iatrogenic distal ulnar physeal disturbance [14].
In efforts to minimize the soft-tissue tension and thus the risks of recurrent deformity, stiffness, and growth arrest, many authors have advocated pre-centralization distraction using a host of external fixation constructs. In theory, pre-centralization distraction reduces soft tissue tension, facilitates centralization, obviates the need for carpal notching or prolonged implant placement which may contribute to distal physeal disturbance. Thatte and Mehta reported on a series of 29 wrists treated with distraction and subsequent radialization at a young age [15]. HFA improved from 74 to 10 degrees, ulnar length was maintained at over 70%, and correction was maintained at mean 6.5 year follow-up. These results are excellent by all accounts, but have not been consistently reproduced in all centers.
Manske et al. compared 13 wrists treated with centralization alone to 13 wrists treated with centralization preceeded by distraction [16]. With centralization alone, HFA improved from 53 degrees pre-operatively to 13 degrees immediately post-operatively to 27 degrees at most recent follow-up. With pre-operative distraction, HFA improved from 53 degrees pre-operatively to 21 degrees immediately post-operatively to 36 degrees at last follow-up. Based upon the similar amount of recurrence, the authors concluded that distraction may facilitate surgical centralization but does not necessarily minimize recurrence. Similar findings were noted by Dana et al. in a series of 8 patients with over two-year follow-up, as well as Lamb et al, McCarthy et al., Shariatzadeh et al, and others [17,18,19,20].
The second concern with centralization techniques has been the risk of iatrogenic ulnar growth disturbance, resulting in upper limb length discrepancy and shortening of an already dysplastic forearm. Potential factors contributing to the risk of ulnar physeal arrest include devascularization from soft tissue dissection around the distal ulna, carpal notching and increased pressure imparted on the distal ulna during centralization, as well as the placement and retention of an intramedullary implant crossing the ulnar physis. Sestero et al. evaluated ulnar growth patterns in 124 limbs of 90 patients and found decreasing ulnar growth in patients treated with non-notching and notching centralization techniques [21]. Indeed, non-operatively treated limbs retained 64% of “normal” length, whereas those patients who underwent non-notching and notching techniques of centralization had ulnar lengths of 58% and 48%, respectively.
To counteract and address both recurrent radial deviation deformity as well as ulnar shortening, several authors have proposed late reconstruction consistent of ulnar lengthening with or without subsequent ulnocarpal arthrodesis. Farr et al. reported on 8 forearm lengthenings performed in 6 patients at mean age of 10 years [22]. At almost 5 year followup, a mean of 7cm of lengthening was achieved. HFA improved from 25 degrees pre-operatively to 11 degrees post-operatively, though recurrent deformity was again observed, with a final follow-up HFA of 23 degrees.
Arthrodesis –or chondrodesis in the skeletally immature—allows for correction of wrist deformity at the expense of ulnocarpal motion. Pike et al. reported on 12 patients with recurrent radial deviation of greater than 45 degrees who underwent ulnocarpal chondrodesis with Kirschner (K-) wire fixation [23]. Patients had a mean post-oeprative DASH score of 24.5, with visual analog scores (VAS) of 7 and 8 for appearance and function, respectively.
Based on these and many other reports, the “classic” or traditional treatment algorithm in the United States and around the world has been (Fig. 1) early splinting, stretching and/or casting, followed by centralization with or without pre-operatively distraction. For those with recurrent deformity and/or excessive limb length discrepancy, ulnar lengthening and ulnocarpal chondrodesis or arthrodesis may be considered.

Figure 1: “Traditional” treatment algorithm for radial longitudinal dysplasia, utilizing centralization or radialization.

Is there another way?
While commonly utilized, the drawbacks of recurrent deformity, additional limb length discrepancy, and loss of wrist motion despite multiple surgeries have motivated many providers to consider alternative treatment options. Ideally, surgical treatment should preserve wrist motion and forearm length, and in doing so maximize hand and upper limb function. To this end, several authors from the United States and around the world have proposed an alternative treatment algorithm consisting of two stages (Fig. 2).

Figure 2: An alternative treatment paradigm for radial longitudinal dysplasia, emphasizing soft-tissue releases via bilobe flap reconstruction, which acknowledges risks of recurrent deformity after centralization and prioritizes preservation of wrist motion and distal ulnar physeal integrity

The first surgical step in this algorithm is addressing radial deviation of the wrist through soft tissue releases, capsulorraphy, and tendon transfers via bilobe flap reconstruction [24,25]. Philosophically, this represents a departure from traditional tenets of RLD surgery; the focus of releases is to improve wrist alignment while judiciously preserving the distal ulnar physis. Wrist motion is prioritized over alignment, given the evidence that function is most directly correlated to wrist and digital motion and that typical surgical interventions result in recurrence.
Technically, a bilobed flap may be performed either dorsally or volarly, depending upon surgeon preference and awareness of the aesthetic implications of dorsal scars and/or color mismatch between the dorsal and volar skin (Fig. 3). A proximally based flap is designed longitudinally which will be rotated or transposed to provide skin and soft tissue to the tight, often deficient radial wrist. A second lobe is based proximally and incorporating the excessive ulnar soft tissues, which will be rotated to cover the dorsal donor area. Careful flap elevation is performed to maintain vascularity to these random patterned flaps, with care to preserve subcutaneous nerve and vein, when possible. After the flaps have been elevated and mobilized, near circumferential exposure of the deeper structures is possible. Tight radial-sided structures are release, the ulnar capsule may be incised and imbricated, and tendon transfers (particular flexors to ulnar extensors) may be performed to achieve dynamic rebalancing across the wrist. Great care is made to preserve all the soft tissues around the distal ulna in efforts to preserve its vascularity and integrity of the distal ulnar physis. Temporary K-wire stabilization may be performed from the ulnar metaphysis to the carpus, avoiding wire passage across the distal ulnar physis. Splinting, therapy, and ROM exercises are begun at 4-6 weeks post-operatively.

Figure 3: Clinical photographs depicting elements of soft-tissue release via a volar bilobed flap. (a) Pre-operative resting position, depicting radial deviation of the wrist and a hypoplastic thumb. (b) After pre-operative stretching and splinting, the wrist may be passively stretched to neutral position. (c, d, e) Intra-operative photographs depicting the (c) radial, (d) volar, and (e) ulnar aspects of the incision. A volar approach was chosen given the aesthetic advantages of leaving the dorsal wrist free of scars. (f) Intra-operative photograph after flap elevation, allowing for access to the musculotendinous units for release and/or transfer. Note the soft-tissues about the distal ulnar physis are carefully preserved. (g) Intra-operative depiction of flap rotation, providing tissue to the previously taught radial wrist. (h, i) Clinical photographs after wound closure, demonstrating improved alignment of the wrist. (j) At 3 months post-operatively, incisions are well healed, flaps remain viable, and the wrist is supple with improved resting alignment. (All images courtesy of Children’s Orthopaedic Surgery Foundation, copyright 2016)

Vuillermin et al. published their series of 18 wrists in 16 patients treated with soft tissue release and bilobe flap reconstruction [24]. A t mean 9 year follow-up, HFA improved modestly from 88 degrees pre-operatively to 64 degrees at most recent follow-up. However, patients maintained a mean of 73 degree wrist flexion-extension and had Disabilities of the Arm, Shoulder, and Hand (DASH) scores and global Pediatric Outcomes Data Collection Instrument (PODCI) scores of 27 and 88, respectively. VAS scores for overall satisfaction were quite high (mean 1.2 on a 10 point scale). These patient-derived measures of outcome compare favorably to other published information regarding the results of centralization. Importantly, there were no physeal growth disturbances, and no patients went on to secondary ulnocarpal chondrodeses or other salvage procedures.
After soft-tissue releases via bilobe flap reconstruction, subsequent soft-tissue distraction and free vascularized metatarsophalangeal (MTP) joint transfer may be performed in a secondary staged fashion [26,27]. Pioneered by Dr. Vilkki, free MTP joint transfer provide an attractive, albeit technically demanding, option to provide more durable carpal support and maintain more normal HFA.
As originally described, distraction lengthening is performed across the ulnar aspect of the wrist, with half pin or smooth wire fixation into both the proximal ulna and ulnar metacarpals [27]. Once adequate distraction has been achieved and more normal wrist alignment restored, free second MTP joint transfer is performed. Arterial inflow is typically achieved from the radial artery to the plantar metatarsal artery. Local veins are used for venous outflow. More extensive radial soft tissues are released, with the native FCR split and reattached to the donor toe flexor and extensor apparatus. K-wire fixation is typically utilized to fix the proximal ulna to the donor metatarsal as well as the radial metacarpals to the donor proximal phalanx; the previously applied fixator is retained for additional support during the early postoperative period. The transferred joint forms a Y-shaped “strut” to support the radial wrist and hand, thereby maintaining HFA while still allowing for wrist motion.
Vilkki previously reported on 19 wrists in 18 patients treated with distraction and MTP joint transfer. At mean 11 year follow-up, HFA was 28 degrees and wrist flexion-extension arc averaged 83 degrees [27,28]. While there was increased radial deviation over time, this change averaged 12 degrees over 10 to 15 years follow-up. Ulnar length is typically preserved, with length approximating two-thirds of normal.
While the author has no personal experience with free vascularized joint transfer, it should be noted that this procedure is generally delayed until later in childhood, given the technical challenges of the procedure. Interestingly, multiple authors have reported that patients often decline secondary MTP joint transfers, given their satisfaction and function following soft-tissue releases via bilobed flap reconstructions [25].
While early reports highlight the theoretical advantages in preserving motion and growth, the merits of this “alternative algorithm” bear further investigation. At present, there is little published information to compare the relative efficacy, durability, and long-term patient-reported functional outcomes of traditional centralization versus soft-tissue releases with or without free MTP joint transfer. In the United States, a multicenter prospective longitudinal cohort study is underway to address this, and other, important congenital questions [29].

References 

1. Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and classificationof congenital anomalies of the hand and upper extremity. J Hand Surg Am 2010; 35: 2066-2076.
2. Goldfarb CA, Manske PR, Busa R, Mills J, Carter P, Ezaki M. Upper-extremity phocomelia reexamined: a longitudinal dysplasia. J Bone Joint Surg Am 2005; 87: 2639-2648.
3. James MA, McCarroll HR, Manske PR. Characteristics of patients with hypoplastic thumbs. J Hand Surg Am 1996; 21: 104-113.
4. Shimamura A. Inherited bone marrow failure syndromes: molecular features. Heamtology Am Soc Hematol Educ Program 2006: 63-71.
5. Waters PM, Bae DS. Radial Longitudinal Deficiency. In: Pediatric Hand and Upper Limb Surgery: A Practical Approach. Philadelphia: Lippincott Williams and Wilkins, 2012, pp 121-131.
6. Bayne LG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg Am 1987; 12: 169-179.
7. James MA, McCarroll HR, James MA, Manske PR. The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg Am 1999; 24: 1145-1155.
8. Kotwal PP, Varshney MK, Soral A. Comparison of surgical treatment and nonoperative management for radial longitudinal deficiency. J Hand Surg Eur 2012; 37: 161-169.
9. Bora FW, Osterman AL, Kaneda RR, Esterhai J. Radial club-hand deformity Long-term follow-up. J Bone Joint Surg Am. 1981, 63: 741–5.
10. Manske PR, McCarroll HR, Swanson K. Centralization of the radial club hand: An ulnar surgical approach. J Hand Surg Am. 1981, 6: 423–33.
11. Buck-Gramcko D. Radialization as a new treatment for radial club hand. J Hand Surg Am. 1985, 10: 964–68.
12. Ekblom AG, Dahlin LB, Rosberg HE, Wiig M, Werner M, Arner M. Hand function in children with radial longitudinal deficiency. BMC Musculoskeletal Disorders 2013; 14: 116-130.
13. Holtslag I, van Wijk I, Hartog H, van der Molen AM, van der Sluis C. Long-term functional outcome of patients with longitudinal radial deficiency: cross-sectional evaluation of function, activity, and participation. Disabil Rehab 2013; 35: 1401-1407.
14. Goldfarb CA, Klepps SJ, Dailey LA, Manske PR. Functional outcome after centralization for radius dysplasia. J Hand Surg Am 2002; 27: 118-124.
15. Thatte MR, Mehta R. Treatment of radial dysplasia by a combination of distraction, radialisation, and a bilobed flap- the results at 5-year follow-up. J Hand Surg Eur 2008; 33: 616-621.
16. Manske MC, Wall LB, Steffen JA, Goldfarb CA. The effect of soft tissue distraction on deformity recurrence after centralization for radial longitudinal deficiency. J Hand Surg Am 2014; 39: 895-901.
17. Dana C, Auregan JC, Salon A, Cuero S, Glorion C, Pannier S. Recurrence of radial bowing after soft tissue distraction and subsequent radialization for radial longitudinal deficiency. J Hand Surg Am 2012; 37: 2082-2087.
18. Lamb DW. Radial club hand: a continuing study of sixty-eight patients with one hundred and seventeen club hands. J Bone Joint Surg 1977; 59: 1-13.
19. McCarthy JJ, Kozin SH, Tuohy C, Cheung E, Davidson RS, Noonan K. External fixation and centralization versus external fixation and ulnar osteotomy: the treatment of radial dysplasia using the resolved total angle of deformity. J Pediatr Orthop 2009; 29: 797-803.
20. Shariatzadeh H, Jafari D, Taheri H, Mazhar FN. Recurrence rate after radial club hand surgery in long term follow up. J Res Med Sci 2009; 14: 179-186.
21. Sestero AM, Van Heest A, Agel J. Ulnar growth patterns in radial longitudinal deficiency. J Hand Surg Am 2006; 31: 960-967.
22. Farr S, Petje G, Sadoghi P, Ganger R, Grill F, Girsch W. Radiographic early to midterm results of distraction osteogenesis in radial longitudinal deficiency. J Hand Surg Am 2012; 37: 2313-2319.
23. Pike JM, Manske PR, Steffen JA, Goldfarb CA. Ulnocarpal epiphyseal arthrodesis for recurrent deformity after centralization for radial longitudinal deficiency. J Hand Surg Am 2010; 35: 1755-1761.
24. Vuillermin C, Wall L, Mills J, Wheeler L, Rose R, Ezaki M, Oishi S. Soft tissue release and bilobed flap for severe radial longitudinal deficiency. J Hand Surg 2015; 40: 894-899.
25. Wall LB, Ezaki M, Oishi SN. Management of congential radial longitudinal deficiency: controversies and current concepts. Plast Reconstr Surg 2013; 132: 122-128.
26. De Jong, JP, Moran SL, Vilkki SK. Changing paradigms in the treatment of radial club hand: microvascular joint transfer for correction of radial deviation and preservation of long-term growth. Clin Orthop Surg 2012; 4: 36-44.
27. Vilkki SK. Distraction and microvascular epiphysis transfer for radial club hand. J Hand Surg Br, 1998; 23: 445-452.
28. Vilkke SK. Vascularized metatarsophalangeal joint transfer for radial hypoplasia. Semin Plast Surg 2008; 22: 195-212.
29. Bae DS, Canizares MF, Miller PE, Roberts S, VUillermin C, Wall LB, Waters PM, Goldfarb CA. Intraobserver and interobserver reliability of the Oberg-Manske-Tonkin (OMT) classification: establishing a registry on congenital upper limb differences. J Pediatr Orthop 2016 Feb 2 [epub ahead of print].PMID 26840275.
26. De Jong, JP, Moran SL, Vilkki SK. Changing paradigms in the treatment of radial club hand: microvascular joint transfer for correction of radial deviation and preservation of long-term growth. Clin Orthop Surg 2012; 4: 36-44.
27. Vilkki SK. Distraction and microvascular epiphysis transfer for radial club hand. J Hand Surg Br, 1998; 23: 445-452.
28. Vilkke SK. Vascularized metatarsophalangeal joint transfer for radial hypoplasia. Semin Plast Surg 2008; 22: 195-212.
29. Bae DS, Canizares MF, Miller PE, Roberts S, VUillermin C, Wall LB, Waters PM, Goldfarb CA. Intraobserver and interobserver reliability of the Oberg-Manske-Tonkin (OMT) classification: establishing a registry on congenital upper limb differences. J Pediatr Orthop 2016 Feb 2 [epub ahead of print].PMID 26840275.

Dr. Donald S. Bae

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Volume 2 | Issue 2 | May-Aug 2016 | Page 12-16|Binoti Sheth1, Bhaskaranand Kumar2

Authors :Binoti Sheth[1], Bhaskaranand Kumar[2]

[1]Lokmanya Tilak Medical College & Sion Hospital, Mumbai. India
[2] Hand Surgical Services, Kasturba Medical College & Hospital, Manipal – 576 119, Karnataka, India.

Address of Correspondence
Dr Binoti Sheth
Department of Orthopaedics, Lokmanya Tilak
Medical College & Sion Hospital, Mumbai. India
Email: binotisheth@yahoo.com

Abstract

Clinical presentation of radial club hand deformity varies from mild hypoplasia to complete absence of radius. Various classification systems are described for radial club hand deformity as the treatment is based on the severity of affection. Treatment of radial club hand has progressed over the years from no treatment to conservative treatment to aggressive surgical correction. Recurrence after the surgical treatment is one of the challenging problems in this anomaly. History of treatment, advances and modifications of treatment, natural history and recurrence with respect to treatment have been described here.
Key words: Radial club hand, classification, treatment, recurrence

Introduction
In 1733, Petit first described radial club hand in an autopsy of a neonate with bilateral club hands and absent radii. If untreated, the deformity does not appear to worsen over time, but there is limitation in prehension and the hand is used primarily to trap objects between it and the forearm. Lamb found that the activities of daily living are not significantly affected in unilateral involvement, but bilateral involvement reduces the activities by one third. In radial club hand, ulna length at birth is known to be approximately 50-75% of the normal ulna length. This limb length discrepancy normally persists throughout growth. According to the literature, the ulna may finally be shortened by as much as 40% of its normal length.[1] There is general agreement favouring surgical correction at 3-6 months of age in children with inadequate radial support of the carpus.

Classifications:
Various classification systems have been described for radial club hand deformity. Most useful and currently accepted classification is modification of that proposed by Heikel.[2] In this classification system, four types are described.
Type 1: (short distal radius)
In this type, the distal radius epiphysis is present but is delayed in appearance. The proximal radial epiphysis is normal. The overall length of the radius is slightly less ,but the ulna is not bowed. (Fig.1)

Figure 1: Type 1 radial club hand

Type 2 : (hypoplastic radius)In this type both proximal and distal radial epiphysis are present, but are delayed in appearance. Due to this there is moderate shortening of the radius and thickening and bowing of the ulna.(Fig. 2)

Figure 2: Type 2 radial club hand

Type 3:(Partial absence of radius) Here , there is partial absence of proximal, middle or distal third of radius, distal being most common. Ulna is thickened and bowed. The carpus is usually radially deviated and unsupported. (Fig. 3)

Figure 3: Type 3 radial club hand

Type 4 : (total absence of radius)
This is the most common type of radial club hand. Here ,there is radial deviation of the carpus, palmar and proximal subluxation, frequent pseudoarticulation with the radial border of distal ulna.There is shortening and bowing of the ulna. (Fig. 4)

Figure 4: Type 4 radial club hand

Bayne and Klug [3] have described a radiological classification system with four types.
Type 1: Deficient distal radial epiphysis
Type 2 : Deficient distal and proximal radial epiphysis.
Type 3 :Partial aplasia of radius(present proximally)
Type 4 :Total aplasia of radius(complete absence)
Type 4 is the most common type of radial club hand.
A fifth type was added by Goldfarb et al [4] describing a radial dysplasia with participation of the humerus. James and colleagues expanded the classification by including deficiency of the carpal bones with a normal distal radius length as type 0 and isolated thumb anomalies as type N.
Variable degrees of thumb deficiencies are frequent with all patterns of radial club hand.(Fig. 5) Manske et al.[5] have devised a global classification system of radial longitudinal deficiency.(Table 1) They have included thumb and carpal abnormalities also in the classification. Carpal anomaly implies hypoplasia, coalition, absence or bipartite carpal bones .Hypoplasia and absence are more common on radial side of carpus and coalitions are more common on ulnar side. X rays must be taken beyond 8 years to allow for ossification of carpal bones.

Table 1: Global classification system of radial club hand

Figure 5: Variable degrees of thumb deficiencies

History of Treatment:
The goal of treatment in radial club hand is to correct the wrist deformity, to maintain the corrected position, to provide wrist-like mobility, to preserve longitudinal growth capacity of ulna and to achieve an acceptable cosmetic result.
Non Surgical Treatment
Riordan [6] recommends applying a long arm cast as soon after birth as possible. The cast is applied in three stages using a technique similar to that used in clubfoot casting. The hand and wrist are corrected first and then the elbow is corrected as much as possible. The correction may be achieved in the infant but according to Milford, casting and splinting is often impractical in a child of age less than 3 months. Lamb [7] reported that elbow extension contracture can be improved by splinting with hand and wrist in neutral position. Current recommendation of treatment is to start from birth with stretching and splinting in order to lengthen the soft tissues and facilitate the reduction of the deformity. A large retrospective review of 446 patients including 137 patients managed non surgically and 309 patients managed with either a centralization or radialization procedure, found that those managed surgically had improvement in appearance and function, including improved alignment, range of motion and strength [8].

Surgical Treatment
Initial surgical treatment described by Bardenheuer was ulnar osteotomy to correct bowing and splitting of distal ulnar for insertion of carpus. Hoffa in 1890 performed a distal transverse osteotomy of ulna to simply realign the ulna. Albee [9] in 1919 attempted to create a radius with a free tibial graft. These surgeries did not give satisfactory result.
Centralization:
In 1894, Sayre [10] described the centralization technique that has now become the treatment of choice across the world. Sayre had described shaping of the distal ulna and notching of the carpus.
Although maintaining alignment, this procedure resulted in a high incidence of spontaneous ulnocarpal fusion with inherent loss of motion and reduced growth potential of the ulna. Many authors have subsequently modified this procedure in an attempt to decrease the potential morbidity while maintaining wrist position. Lidge [11] in 1969 introduced an improved method where ulnar epiphysis was preserved at the expense of central carpal bones that had to be resected. Although early results were good, late results were less satisfactory. Lamb recommended creation of carpal notch to stabilize the carpus on the ulna. He suggested depth of the notch to be equal to the transverse diameter of the distal ulna and this required removal of all of the lunate and most of the capitate. As against this, Watson et al.[12] suggested not to excise any of the carpus because of the possibility of affecting growth of the forearm. Buck-Gramcko (1985) and Bayne and Klug also suggested not to remove any of the carpus.
Different incisions and surgical approaches for centralization procedure have been described due to the problems of significant skin tension on radial side following ulna repositioning. Manske and McCarroll [13] preferred transverse ulnar incision, as described by Riordan, removing an ellipse of skin (Fig. 6)

Figure 6: Transverse ulnar incision for centralization

Figure 7: S- shaped incision for centralization

Figure 8: Dorsal bilobed skin incision as described by Evans

Watson, Beebe and Cruz [12] preferred ulnar and radial z-plasty incisions to allow removal of the distal radial anlage which they believe is essential. Lamb (1972) [14] and Buck Gramcko (1985) described S-Shaped incision for better exposure. (Fig. 7)
Evans described dorsal bilobed skin flap to provide additional skin on radial side and take up excess on the ulna side of the wrist. This gives good access to wrist joint and surrounding soft tissue structures.(Fig. 8 )
DeLorme (1969) [15] first suggested intramedullary fixation of carpus on the ulna. Most authors now prefer to use K wire to secure alignment of long or index metacarpal with the ulna for at least 6 weeks. Still the recurrence rates are high. To overcome the recurrence tendency, additional procedures were introduced including collateral ligament reconstruction, soft tissue balancing by tendon transfer and leaving K wire for as long as possible (preferably several years) Bora et al [16]. recommended adjunctive tendon transfer. FDS from central digits was transferred around the postaxial side of the forearm into the dorsal aspect of metacarpal shaft. Hypothenar muscles were transferred proximally along ulnar shaft. ECU was transferred distally along the shaft of the metacarpal of little finger. However, this procedure failed to prevent 25-35 degrees of recurrent radial deviation. Bayne and Klug recommended transfer of FCU into the distally advanced ECU to help prevent radiovolar deformity.
Pre-centralization distraction:
Another recommendation to prevent recurrence was to use soft-tissue distraction before centralization (Fig. 9). Kessler [17](1989) described a technique to passively distract the soft tissue with an external fixation before centralization. Goldfarb et al reported on soft tissue distraction precentralization and noted that the centralization could be performed effectively and without tension after distraction, which averaged 6mm. Nanchahal and Tonkin [18] reported a similar experience, noting that carpal realignment was possible in 5 of 6 patients who underwent preoperative distraction, but only in 1 of 6 patients treated without distraction. Kanojia et al [19] reported similar experience with an external fixator and noted that early application can lead to completion of surgical treatment by 10 months of age in the vast majority of patients. Sabharwal et al[20] used Ilizarov for soft tissue distraction prior to centralization, considering it to be more comprehensive achieving physiologically sound distraction. Ilizarov has certain advantages over external fixator, as there is option of placing multiple bony anchors in different planes, application of translational hinges corresponding to the apex of the deformity. Bhat et al.[21] used MAC system (Multi axial correction) for correction of triplanar angulation, translation, rotation and limb length discrepancy. They found it useful especially in adolescent age group with previous failed soft tissue procedures. Dana et al.as well as Prokopovitch [22] and Kessler have reported small series of radial club hand deformities treated by external fixator application to gradually stretch the soft tissues to facilitate centralization.

Figure 9: Pre-centralization distraction using external fixator

Radialization:
Even with soft tissue distraction prior to centralization, there is high incidence of unsatisfactory results. Buck-Gramcko in 1985 developed a modified technique to improve the outcome. It is called ‘radialization’ [23]. In this technique, the hand is brought with its radial carpal bones over the head of the ulna and fixed with a K wire in moderate ulnar deviation to produce some overcorrection(Fig.10). Also ECR and FCR muscles are transposed to weaken the forces for radial deviation, thereby preventing recurrence while a better muscle balance is established. Both modifications tend to improve mechanical conditions which are prerequisites to maintain hand and forearm in proper relation. Also, in this technique, the resection of carpal bones can be avoided to help increase total arm length and improve range of motion. Buck Gramcko used this technique in 30 cases and found satisfactory results. With improved function and further growth, the distal end of the ulna became broad like a radius.

Figure 10: Radialization technique

Ulnar lengthening:
Ulnar lengthening was another technique used to increase forearm length, typically with the aid of a circular frame such that correction of the radial deviation can occur simultaneously. Farr et al [24] recommended lengthening procedure to be performed in school age children (8-10 years) to gain sufficient limb length for performance of ADL including perineal care in adolescence and adulthood. A second lengthening step may follow after growth cessation, depending on the functional outcome of the first procedure. They reported on 8 cases in 6 patients and noted that initial post surgical gains were not maintained and there was recurrence of radial deviation at an average 4 years follow up. Two major complications occurred, including an ulnar fracture after frame removal and insufficient regenerate during lengthening. Peterson et al [25] described 9 children who underwent 13 lengthenings after previous centralization procedures. The average gain in length was 4.4 cm (range 1.8-8.0 cm). All patients had at least one pin site infection that was treated with antibiotics. 4 patients required additional procedures including internal fixation and bone grafting for delayed union in 3 patients and wrist arthrodesis for recurrence in 1 patient. Yoshida et al[26] investigated the growth of the ulna after repeated lengthenings. After the initial lengthening, the average length improved from 57% to 89% of the normal side, but then regressed to 70% whereas after the second lengthening, the average length was 102% but regressed to 83%. Bone growth was markedly decreased after the second lengthening. Hence, when multiple lengthenings are performed the second one should be done after skeletal maturity (Fig. 11). Sestero et al [27] assessed the ulnar length and growth of 72 limbs treated either surgically or non surgically over 80 years. They found that non surgically treated extremities attained 64% of normal length, whereas patients who underwent surgery achieved 48% (notched centralization) to 58% (non notched centralization) of normal ulnar length. They also calculated an average annual ulnar growth of 2.6 cm after surgery, compared with 3.6 cm for patients treated non surgically. However, a recent study of 446 affected hands reported that surgical treatment was superior to conservative treatment in terms of appearance and function. Hill et al.[28] have suggested that full restoration of length is not necessary for this procedure to have a successful outcome. Recurrence of deformity after forearm lengthening has not been well described. Kawabata et al.[29] observed a tendency towards recurrence of ulna bowing and radial deviation in 4 of 7 patients, showing a final angular deformity of 19 degrees.

Figure 11: Ulnar lengthening using external fixator

Radial lengthening:
Matsuno et al [30] described 4 cases of mild (Bayne and Klug type I and II) radial club hand treated with radial lengthening and simultaneous soft tissue distraction between ulna and 3rd metacarpal. Three of the four patients required several lengthenings to correct the recurring discrepancy between the radius and ulna. Only two of the four patients had acceptable function and appearance after the multiple procedures. In one patient, lengthening was abandoned owing to severe bone absorption at the distal end of the radius.

Vascularised metastarsophalangeal transfer:
In 1920, an attempt was made to reconstruct the radius with bone graft to support the carpus. In 1945, non vascular epiphyseal transfer was attempted for treatment of radial club hand, but the results were disappointing, with disrupted ulnar growth plate and increase in limb length discrepancy. Also, there was inadvertent ankylosis / arthrodesis of wrist and loss of radial support. Vikki [30] reported the results of vascularised second metatarsophalangeal joint on 24 limbs with an average follow up of 11 years. The average radial deviation at final follow up was 280, the average active wrist total arc of motion was 830 and the average length of the ulna was 67% of the contralateral side. But complications were present in more than 50% of patients, including failure of the transfer and subluxation of the joint.

Post-operative assessment:
The outcome of various surgical procedures can be assessed by cosmetic appearance, total wrist arc of motion and certain radiological measurements as described by Manske et al.[13] HFA (Hand-FA angle) is the intersection angle between long axis of the long metacarpal and distal ulna, as measured on AP x-ray. Minus’ HFA refers to the hand and long axis of long finger metacarpal distal axis directed in ulnar deviation with respect to distal ulna. This is the more desired position. HFP (Hand FA position) refers to the distance in mm between base of little finger metacarpal and distal ulna. If proximal pole of little finger metacarpal was ulnar to the axis of distal ulna, the position (HFP) recorded as positive mm, a change in ulnar direction was considered an improvement in the position of hand. Ulnar bowing was defined as the angle between the intersection of the proximal and distal ulnar bi sector lines. The goal of treatment in radial club hand is to achieve slight overcorrection with ulnar deviation of hand and ulnar translation of hand and wrist at the end of treatment.(Fig. 12)

Figure 12: Follow up case of treated radial club hand

Recurrence:
Despite various advances in the surgical treatment of radial club hand, recurrence of radial angulation and ulna bowing is common. A possible cause may be the soft tissue, which remains pathologic after centralization or radialization. After consecutive lengthening and relative realignment of the forearm, the altered fibrous muscles and connective tissue lead to progressive rebowing of the ulna and a radial shift of the wrist. Precentralization distraction aids in soft tissue balancing at the time of centralization, but it is unknown whether the technique will improve maintenance of alignment with longer follow-up. Deformities noted to be more severe preoperatively were more likely to recur. Also, recurrence was associated with young age (less than 12 months) at index procedure and immediate post operative radial deviation position. It is advisable to inform the parents (and patients) at the time of centralization, that the deformity may recur. Lamb reported 3 recurrences of volar angulation after 31 centralization procedures [14]. Damore et al[32] reported that the mean total angulation (radial deviation and ulnar bow) improved from 830 before centralization to 380 after, but recurred to 630 radial deviation at a mean 6.5 years after centralization. Geck et al [34] performed survivorship analysis of 29 radial club hand treated with centralization or radialization, with an end point of revision surgery for recurrence. At 5 years, one third of patients presented with a recurrent deformity that was subsequently treated by revision. According to Pike et al.[33] post centralization recurrence of radial angulation to greater than 450, an inability to actively extend the wrist to within 250 of neutral (i.e. 250 of flexion) or both, are considered as indications for re-surgery. Treatment of recurrent deformity after centralization is problematic; repeated centralization is likely to fail again because of the instability of the hand and carpus at the end of a single forearm bone. The preferred treatment after a failed centralization procedure is ulnocarpal arthrodesis in skeletally mature or an epiphyseal arthrodesis when the distal ulna physis is still open. Epiphyseal arthrodesis showed no disturbance in extremity growth when performed in skeletally immature extremities when the surgical principles for preserving the epiphysis were carefully followed. There is always a concern about the irreversible feature of a wrist fusion in a young patient. To address this concern, a temporary arthrodesis is offered to patients to allow the patient to make an informed decision, the wrist is stabilized in surgery with percutaneous K wires holding the ulno-carpal joint in the anticipated position of fusion. If the patient likes the position, epiphyseal arthrodesis is performed in 1 to 2 weeks, if the patient does not like the position, K wires are removed and the wrist returns to its former position.
There are several treatment options for radial club hand, including non surgical management; centralization, radialization or ulnarization; ulnocarpal arthrodesis; soft tissue procedures including distraction; ulnar lengthening; and vascularized second metatarsophalangeal joint transfer. The most common procedure performed currently is soft tissue distraction followed by a wrist realignment procedure such as centralization, or radialization. Ultimately, a procedure is needed that can correct and maintain the deformity, yet permit growth with a low complication rate.

References 

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Dr. Binoti Sheth

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