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Pollicisation: Embryology, History, Technique and Results

Volume 2 | Issue 2 | May-Aug 2016 | Page 29-33|Amita S. Hiremath, Binita N. Raut, Amit A. Yadav, Mukund R. Thatte Authors :Amita S. Hiremath [2], Binita N. Raut [1], Amit A. Yadav [2], Mukund R. Thatte [3] [1] KLE University’s J.N.Medical College,Belgavi, [2] Bombay Hospital and Institute of Medical Sciences Mumbai. [3] Bombay Hospital […]

Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

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 […]

Pollicisation: Embryology, History, Technique and Results

Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

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Pollicisation: Embryology, History, Technique and Results

Volume 2 | Issue 2 | May-Aug 2016 | Page 29-33|Amita S. Hiremath, Binita N. Raut, Amit A. Yadav, Mukund R. Thatte

Authors :Amita S. Hiremath [2], Binita N. Raut [1], Amit A. Yadav [2], Mukund R. Thatte [3]

[1] KLE University’s J.N.Medical College,Belgavi,
[2] Bombay Hospital and Institute of Medical Sciences Mumbai.
[3] Bombay Hospital and Institute of Medical Sciences Bai Jerbai Wadia Hospital for Children, Shushrusha Citizens’ Co‑op. Hospital,Maharashtra, India

Address of Correspondence
Dr Mukund R. Thatte, Room 6, 2nd Floor, New Wing, Bombay Hospital, New Marine Lines, Mumbai ‑.400 020, Maharashtra, India.
E‑mail: mthatte@gmail.com

Abstract

Thumb hypoplasia/aplasia is one of the components of radial longitudinal deficiency. It can occur either alone or it may be associated with other components of radial longitudinal deficiency. Aim of thumb reconstruction is to provide a functional and stable thumb which will improve the grip and aesthetic appearance of hand. Pollicisation is the treatment for Type IIIB,IIIC, IV and V types of thumb hypoplasia as per Blauth’s Classification as Modified by Manske and Buck-Gramcko. In the opinion of the authors, pollicisation is one of the finest and most difficult operations in reconstructive hand surgery.
Keywords: Pollicisation: History, Principles and Surgical technique.

Introduction
Napier has said “The hand without a thumb is at worst, nothing but an animated fish-slice, and at best a pair of forceps whose points don’t meet properly”. [1]
Thumb hypoplasia is a congenital condition, which can range from slightly small digit to deficiency of some musculotendinous units or osseous components to complete absence of thumb.40% of the hand function relies on a strong thumb. [2]For proper functioning of the hand, thumb size, its position,mobility and the relation with other fingers is critical. Children with thumb hypoplasia can manipulate small objects using side to side pinch, between adjacent digits, ‘Chopstick’ action; but it is difficult to grasp large objects. [3]
Thumb hypoplasia/aplasia is one of the components of radial longitudinal deficiency. It can occur either alone or it may be associated with other components of radial longitudinal deficiency. A wide range of manifestations on the preaxial side of the upper limb is seen in radial longitudinal deficiency. Hence, in any case of thumb hypoplasia, one should examine proximal forearm and contralateral thumb.
Incidence
Thumb hypoplasia is a component of various congenital malformations like thumb duplication, transverse deficiencies, brachydactyly, symbrachydactyly, cleft hand complex, constriction ring syndrome etc., and hence it is very difficult to find out the true incidence of thumb hypoplasia alone. Tay SC et al in 2006, reported upto 16% incidence of thumb hypoplasia amongst congenital hand deformities. [4] There is equal distribution of cases between male and female. About 60% cases have bilateral involvement with often asymmetrical involvement. [5] In unilateral cases, right hand is more commonly affected. [6]
In a study by Abdel-Ghani and Amro, [7] 86% cases of thumb hypoplasia were associated with various conditions involving cardiovascular, gastrointestinal and genitourinary system. Various conditions commonly seen in association with thumb hypoplasia are Holt-Oram syndrome, VACTERL association, Fanconi’s anaemia, Thrombocytopenia absent radius(TAR) syndrome. [6] The severity of radial longitudinal deficiency is directly proportional to the degree of thumb hypoplasia according to James et al. [8]

Genetics
The upper limb bud appears at 4th week of embryonic development opposite the caudal cervical somites. Various signalling centres have been identified that are responsible for upper limb development. [4,8]
1. Apical Epidermal Ridge(AER) – responsible for proximal to distal development (shoulder to hand) and is mediated by number of fibroblast growth factors.
2. Zone of Polarizing Activity (ZPA) – responsible for anterior-posterior pattern formation(also called Pre and Post Axial) and is mediated by sonic hedgehog (Shh) and homeobox (HOX) gene products.
3. WNT7a signalling centre – responsible for dorsal ventral development.
The exact mechanism of embryologic insult that leads to thumb hypoplasia is unclear. Abnormalities in AER or ZPA can lead to thumb hypoplasia. Studies have proved that administration of teratogenic agents or inactivating fibroblastic growth factors in rats and mice can lead to radial longitudinal hypoplasia with thumb hypoplasia. [10]
The deletion of chromosome 22q11 can lead to thumb hypoplasia in radial longitudinal deficiency [5]. However, it is difficult to point out single gene or enzymatic defect as the cause for thumb hypoplasia [8].

Classification
Thumb hypoplasia can present with wide range of functional and aesthetic differences. Hence careful inspection and examination is required. One should carefully examine the size, position, skeletal components, intrinsic and extrinsic muscles, joint stability, and first web space and associated conditions. One should correlate soft tissue and skeletal deficiencies while taking decision about the treatment.
Classification helps in taking decision about the treatment. In 1937, Muller first classified thumb hypoplasia. It is currently the Blauth’s classification which is widely accepted in classifying and as a guide for surgical planning. Manske further classified type 3 hypoplasia in A and B depending on stability of CMC joint. [4, 11]

Blauth’s Classification as Modified by Manske and Buck-Gramcko:

Type I: Mild hypoplasia
Slight decrease in thumb size
Slender phalanges and metacarpals
Normal intrinsic muscles
Normal trapezium, scaphoid, distal radius and styloid process

Type II: Moderate hypoplasia
Underdeveloped or absent thenar muscles
Adducted posture- first web space narrowing
Ulnar Collateral ligament (UCL) insufficiency at MP joint
CMC stable

Type III: Severe hypoplasia
All the features of type II plus
Radial carpal aplasia
Extrinsic muscle and tendon abnormalities
Type III A: Stable CMC joint
Type III B: Unstable CMC joint
Type IIIC: Buck Gramcko has added this variation in which thumb has only metacarpal with no musculotendinous unit and a wider skin bridge
Type IV: Pouce floutant (French Floating thumb)
Thumb is small and has rudimentary skeletal elements. It is an unstable digit, connected to the hand by only a skin bridge containing the neurovascular bundle.
Type V: Aplasia i.e.Absence of all thumb structures and radial carpal bones

Figure 1: Preoperative picture of Type IV thumb hypoplasia (volar view)

Figure 2: Preoperative picture of Type IV thumb hypoplasia (dorsal view)

Figure 3: Preoperative xray showing rudimentary skeletal elements

Evaluation
Thumb hypoplasia can be the only presentation of radial longitudinal deficiency or it may be associated with other anomalies. Plain bilateral radiographs will help to assess thumb phalanges, metacarpal and carpometacarpal joint. One should assess wrist and forearm to rule out radial longitudinal deficiency.
As thumb hypoplasia can be associated with other syndromes, thorough cardiac, renal, haematological and musculoskeletal evaluation is essential. Common investigations include a complete blood count, 2D Echo Cardiogram and sonography abdomen for solid organs. This is important as the treatment of these conditions may take priority over thumb hypoplasia management. [12]Any positive findings may also impact anaesthesia for surgery.

Figure 4: Buck Gramcko type markings for dorsal side

Figure 5: Buck Gramcko type markings for palmar side

Figure 6: Dorsal flaps raised preserving dorsal venous network

Treatment
Aim of thumb reconstruction is to provide a functional and stable thumb which will improve the grip and aesthetic appearance of hand.
Typically Type I is more or less a functional thumb,hence no treatment is required.
Type II: depending on findings; first web space release, opponensplasty using Abductor Digiti Minimi (ADM) or other suitable donors and/or UCL reconstruction
Type III A: same procedures like type II and in addition may require further additional extrinsic tendon transfers to improve the function, which transfers and how needs to be decided on a case by case basis, depending on need.
Type IIIB,IIIC, IV and V: Pollicisation

Timing of surgery:
Surgery at an early age is recommended for better functional results as it will allow cognitive development and physiological adaptation of the reconstructed thumb before corticalisation which happens around 18 months of age. [13]
We are basically going to discuss about pollicisation in advanced hypoplasia IIIB onwards or Aplasia in this paper.
Pollicisation is a Latin word; Pollex means thumb and Pollicisation is defined as reconstruction of thumb using the adjacent radial finger.

History of pollicisation
In 1885,Guermonprez seems to be the first person to report a case of pollicisation. Before the second world war, many surgeons Dunlop (1923), Bunnell (1931) have reported doing pollicisation. However, follow up study of these cases had unsatisfactory results.
The major modification in the procedure was seen after the Second World War. Gosset (1949) [14] described index finger and ring finger pollicisation on neurovascular pedicle without skin attachments. Middle finger pollicisation was described by Hilgenfeldt (1950) [15]. Further contributions were by Bunnell (1952), Littler (1953). [16.17]
In 1971, Buck Gramcko published an article about pollicisation [18] which is the landmark article in establishing the technique of pollicisation in our opinion. Buck Gramcko’s major work on pollicisation evolved after the thalidomide tragedy in Europe which had led to many congenital hand and upper limb anomalies. Manske, Foucher et all have modified the technique but Buck Gramcko’s technique is the gold standard method still commonly used. The authors use the Buck Gramcko technique.

Principles of Pollicisation:
In 1990 Littler had said:
“It is not the full length of the thumb, nor its great strength and movement, but rather its strategic position relative to the fingers and the integrity of the specialized terminal pulp tissue which determines the prehensile status.”

Littler has classified requirements of the procedure as follows [19]
Mechanical
1. Correct length
2. Strategic position
3. Stability

Physiological
1. Movement
2. Sensibility
3. Absence of pain
4. Integration

Satisfactory appearance
In the opinion of the authors, pollicisation is one of the finest and most difficult operations in reconstructive hand surgery. Patient’s parents have a tough time deciding about amputing the useless thumb and using index finger for thumb reconstruction. Authors feel it is simultaneously rewarding to and taxing on the surgeon performing it. [20]

TECHNIQUE Figs. 1- 17
Fig. 1,2 and 3 represent pre operative photos and X-Ray.
Markings as per Buck-Gramcko (Fig. 4,5) Senior author (MRT) starts the surgery by raising the dorsal flaps taking care to preserve dorsal venous network. (Fig. 6)
Once the dorsal flaps are raised preserving dorsal venous network, palmar incisions are made and pedicles are dissected on the palmar side. Sometimes it may be required to open the neural ring on the ulnar side pedicle,if it is present.(Fig.7)

Figure 7: Dissection of vascular pedicles of Index finger

After dissecting and safeguarding the pedicle, first dorsal interosseous and first palmar interosseous with lateral band extension is identified and cut. To identify the distal lateral band at the time of final suturing, a 6-0 nylon suture is used for tagging the remaining lateral band (Fig. 8,9,10)

Figure 8: First dorsal interroseous raised with tendon

Figure 9: Lateral band on finger tagged with 6/0 nylon loose stitch for later identification

Figure 10: First palmar interroseous raised with tendon

Next step is to identify the two extensors – extensor indicis proprius and extensor digitorum communis of index finger and cut them. In more severe variants of Radial dysplasia only one tendon is present.(Fig. 11)

Figure 11: Extensor digitorum communis and Extensor Indicis proprious seen

Figure 12: Extensor Indicis proprious (EIP) cut and raised off MP joint so as to act on distal interphalangeal as future extensor pollicis longus

For setting the index completely free, the deep transverse metacarpal ligament between index and middle finger has to be divided. (Fig. 13)

Figure 13: Dividing deep transverse metacarpal ligament between index and middle finger

The line of division of metacarpal is through the epiphysis at the level of the head. The shaft is disarticulated at the CMC joint and is excised extra-periosteally.(Fig. 14,15)

Figure 14: Division of index finger metacarpal at the level of head of metacarpal

Figure 15: Metacarpal shaft disarticulated at carpometacarpal joint

At every step, one has to be careful about the neurovascular pedicle of the index finger.
The metacarpal head has to be rotated by 900 in extension and fixed with 4-0 nylon to prevent hyperextension of the new CMC joint.(Fig 16)

Figure 16: Rotating metacarpal head dorsally by 900

Index finger is now rotated 150 degrees, abducted 40-50 degrees and fixed at the CMC level with a 4-0 nylon suture which goes thru the CMC joint- head of metacarpal-CMC other lip.(Fig 17)

Figure 17: Index finger is fixed at CMC level

The Extensor/s are sutured first. The EIP becomes the EPL and the EDC becomes the APL
The Metacarpal level attachment of the EIP is dissected off; this enables the EIP to act on the DIP as an extensor. (Fig 12)
The EDC is kept attached at the base of the PPx of the IF so that once the tendon is reattached it acts as the APL.
The First dorsal interosseous is attached next to the lateral band to become the new abductor – opponens complex.(Fig. 18)

Figure 18: Suturing first dorsal interosseous to lateral band as the new abductor opponens complex

Dorsal flaps are sown.
The Palmar Interosseous is now attached to the other lateral band to become the Adductor Pollicis.
Skin flaps are now adjusted and closed to get the new thumb(Pollex) in opposition and pronation to achieve pulp to pulp pinch.(Fig. 19)
Fig. 20,21 show two views of the completed operation

Figure 19: Adjusting skin flaps to get new thumb in opposition and pronation

Figure 20: Final position palmar view

Figure 21: Final position dorsal view

A bulky padded dressing is given to protect the new thumb and tourniquet released and vascularity of both arteries and veins is checked.(Fig. 22)
Fig. 19 shows a long term result

Figure 22: Final dressing

Figure 23: showing 7 years follow up results

Complications of procedure
Losing the finger—very rare but possible
Skin necrosis
Hyperextension at CMC joint
Too long length
Functional Result may not be as good in severe radial longitudinal deficiency. There are chances of abnormalities of muscles of index finger and stiffness of index finger PIP joint in these cases (Symphalangism) which leads to relative functional impairment following pollicisation.
In our opinion it is still better than a four fingered hand at all times

Conclusion
Pollicisation is the optimum method of thumb reconstruction in thumb hypoplasia/aplasia

References

1. The rule of thumb by Tom Tyler JAC 30.3.4(2010) ,711-32.http://www.cyberchimp.co.uk/research/thumb.html
2. Lightdale-Miric N, Mueske NM, Lawrence EL, Loiselle J, Berggren J, Dayanidhi S, Stevanovic M, Valero-Cuevas FJ, Wren TA. Long term functional outcomes after early childhood pollicization. J Hand Ther. 2015 Apr-Jun;28(2):158-65
3. MahmutKömürcü, SerdarYüce et al. Index finger pollicization for treating a congenitally nonfunctioning thumb in patients with radial longitudinal deficiency. Eastern Journal of Medicine 2014;19: 175-181.
4. Tay SC, Moran SL, Shin AY, Cooney WC III. The hypoplastic thumb. J Am AcadOrthopSurg 2006;14: 354 –366.
5. Thumb hypoplasia .Scott A. Riley,Ronald C. Burgess. Hand Surg 2009;34A: 1564–1573.
6. James MA, McCarroll HR Jr, Manske PR. Characteristics of patients with hypoplastic thumbs. J Hand Surg 1996;21A: 104–113.
7. Abdel-Ghani B, Amro S. Characteristics of patients with hypoplastic thumb: a prospective study of 51 patients with the results of surgical treatment. J PediatrOrthop B 2004;13:127–138.
8. James MA, Green HD, McCarroll HR Jr, Manske PR. The association of radial deficiency with thumb hypoplasia. J Bone Joint Surg 2004;86A: 2196–2205.
9. Tickle C. Experimental embryology as applied to the upper limb. J Hand Surg 1987;12B: 294 –300.
10. Kato H, Ogino T, Minami A, Oshio I. Experimental study of radial ray deficiency. J Hand Surg 1990;15B:470–476.
11. Kozin SH. Deformities of the Thumb. Green’s Operative Hand Surgery.6 thed.ScottW.Wolfe, Robert N., William C., Scott K. editors. Philadelphia: Churchill Livingstone, Elsevier: 2011.p 1371.
12. Riley SA. An overview of radial longitudinal deficiency. CurrOrthop Prac2008;9:655–659.
13. Joseph Upton III and Amir Taghinia. Congenital hand III: Disorders of formation- thumb hypoplasia.James Chang and Peter C. Neliganeditors.Plastic Surgery- Hand and Upper Extremity.Elsevier Saunders,2013.p 572-602.
14. Gosset I: La pollicisation de I ‘index . J Chir.1949;65:403.
15. Bhaskaranand Kumar, Ashwath Acharya, and Anil K. Bhat. A relook at pollicization. Indian J Plast Surg. 2011 MayAug;44(2): 266–275.
16. Paul Binhammer and Graham Lister.Pollicization. Reconstructive Surgery in Hand Mutilation. Edited by Guy Foucher, Martin Dunitz: 1997.pg 29-40.
17. J W Littler, The neurovascular pedicle method of digital transposition for reconstruction of the thumb. Plast. Reconstr. Surg. 1953, 12:303-14
18. BuckGramcko D. Pollicization of the index finger. Method and results in aplasia and hypoplasia of the thumb. J Bone Joint Surg Am 1971;53:1605-17.
19. Yves Allieu, Michel Chammas and Jean Luc Roux. Considertions concerning pollicization. Reconstructive Surgery in Hand Mutilation. Edited by Guy Foucher, Martin Dunitz: 1997.pg41-52.
20. Thatte MR, Nehete S, Garude K, Mehta R. Unfavourable results in pollicisation. Indian J Plast Surg. 2013 May;46(2):303-11..

How to Cite this Article: Hiremath AS, Raut BN, Yadav AA, Thatte MR. Pollicisation: Embryology, History, Technique and Results. International Journal of Paediatric Orthopaedics May-Aug 2016;2(2):29-33.

Dr Mukund R. Thatte

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Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

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

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How to Cite this Article: Bae DS. Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm. International Journal of Paediatric Orthopaedics May-Aug 2016;2(2):24-28.

Dr. Donald S. Bae

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Pollicisation: Embryology, History, Technique and Results

Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

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Pollicisation: Embryology, History, Technique and Results

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Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

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