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 

1. Louri GM,Linse RE: Radial longitudinal deficiency:A review and update Hand Clin. 1998;14(1):85-99.
2.Heikel H.A.:Aplasia and hypoplasia of the radius Acta Orthop Scand Suppl 39:1,1959
3. Bayne LG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg Am. 1987 Mar;12(2):169-79.
4. 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 Dec;87(12):2639-48.
5.James MA, McCarroll HR Jr, Manske PR: The spectrum of radial longitudinal deficiency: a modified classification J Hand Surg Am. 1999 Nov.24(6):1145-55
6. Riordan DC. Congenital absence of the radius. J Bone Joint Surg Am. 1955
Dec;37-A(6):1129-39
7.Lamb DW. Radial club hand. A continuing study of sixty-eight patients with one hundred and seventeen club hands. J Bone Joint Surg Am. 1977 Jan;59(1):1-13..
8. Kotwal PP, Varshney MK, Soral A. Comparison of surgical treatment and nonoperative management for radial longitudinal deficiency. J Hand Surg Eur Vol. 2012 Feb;37(2):161-9.
9.Albee FH:Formation of radius congenitally absent:condition seven years after implantation of bone graft, Ann. Surg.87:105,1928
10.Sayre RH. A contribution to the study of club hand, Trans Am Ortho Assoc 6:208, 1893
11. Lidge RT. Congenital radial deficient club hand, J Bone Joint Surg 51-A 1041, 1969
12. Watson HK, Beebe RD, Cruz NI. A centralization procedure for radial clubhand. J Hand Surg Am. 1984 Jul;9(4):541-7.
13.Manske PR, McCarroll HR Jr, Swanson K. Centralization of the radial club hand: an ulnar surgical approach. J Hand Surg Am. 1981 Sep;6(5):423-33
14. Lamb DW. The treatment of radial club hand. Absent radius, aplasia of the radius, hypoplasia of the radius, radial paraxial hemimelia. Hand. 1972 Feb;4(1):22-30.
15.DeLorme TL:Treatment of congenital absence of radius by transepiphyseal fixation J Bone Joint Surg 51-A:117,1969
16.Bora FW, Osterman AL, Kaneda RR:Radial club hand deformity:long term follow up J Bone Joint Surg 63-A: 741,1987
17.Kessler I:Centralization of the radial club hand by gradual distraction, J Hand Surg 14-B:37,1989
18. Nanchahal J. and Tonkin M.A. : Pre-operative distraction lengthening for radial longitudinal deficiency J. Hand Surg. Br. 1996:21:pp 103-10.
19. Kanojia R.K., Sharma N, and Kapoor S.K.: Preliminary soft tissue distraction using external fixator in radial club hand. J. Hand Surg, Eur. Vol. 2008; 33:pp 622-627.
20.Sabharwal S et al.: Pre-centralization soft tissue distraction for Bayne type 4 congenital radial deficiency in children J Paed Orth 2005, 25(3):377-81
21.Suneel Bhat et al.MAC system in the treatment of radial club hand J Child Ortho 2009 Dec 3(6):493
22.Prokopovich VS; Aligning of the length of the forearm bones in the congenital club hand in children Orthop Traumatol Protez 1:51,1980
23.Buck –Gramcko D: Radialization as a new treatment for radial club hand J Hand Surg 10-A(pt 2):964,1985
24.Farr S, Petje G, Sadoghi P: Radiographic early to mid term results of distraction osteogenesis in radial longitudinal deficiency J Hand Surg Am 2012: 37,2313-2319
25.Peterson BM, McCarroll HR,James MA:Distraction lengthening of the ulna in children with radial longitudinal deficiency J Hand Surg Am 2007,32:1402-07
26.Yoshida K,Kawabata H,Wada M:Growth of the ulna after repeated bone lengthening in radial longitudinal deficiency J Paed Ortho 2011 :31, 674-678
27.Sestero AM, Van Heest A,Agel J:Ulnar growth patterns in radial longitudinal deficiency J Hand Surg Am 2006 Jul- Aug,31(6):900-7
28. Hill RA, Ibrahim T, Mann HA, Siapkara A. Forearm lengthening by distraction
osteogenesis in children: a report of 22 cases. J Bone Joint Surg Br. 2011 Nov;93(11):1550-5.
29.Kawabata H, Shibata , Masotami T, Yasui N:Residual deformity in congenital radial club hands after previos centralization of the wrist, ulnar lengthening and correction by the Ilizarov method J Bone Joint Surg Br 1998 Sept 80(5):762-5
30.Matsuno T, Ishida O,Sunagawa T, Suzuki O:Radius lengthening for the treatment of Bayne and Klug type 2 and 3 radial longitudinal deficiency J Hand Surg Am 2006:31, 822-829
31. Vikki SK: Vascularised metatarsophalangeal joint transfer for radial hypoplasia Semin Plast Surg 2008:22, 195-212
32. Damore E; Kozin S.H. Thoder J.J. and Porter S : The recurrence of deformity after surgical centralization for radial clubhand J. Hand Surg. Am-2000; 25:pp 745-751.
33. Pike J.M., Manske P.R., Steffen J.A. and Goldfarb C.A. :Ulnocarpal epiphyseal arthrodesis for recurrent deformity after centralization for radial longitudinal deficiency J. Hand Surg. Am. 2010; 35:pp. 1755-61.
34. Geck MJ, Dorey F, Lawrence JF, Johnson MK. Congenital radius deficiency:
radiographic outcome and survivorship analysis. J Hand Surg Am. 1999 Nov;24(6):1132-44.

Dr. Binoti Sheth

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Volume 2 | Issue 1 | May-Aug 2016 | Page 8| Rujuta Mehta1

Authors :Rujuta Mehta[1]

[1] B J Wadia Hospital For Children, Parel, Mumbai India
[2]Nanavati Superspeciality Hospital, Mumbai, India.

Address of Correspondence
Dr Rujuta Mehta
HOD, Dept. of Paediatric Orthopaedics, B J Wadia Hospital,
Nanavati Hospital, Jaslok Hospital & Shushrusha Hospital.
Email: rujutabos@gmail.com

The evolution of treating this anomaly is a journey of learning; learning to reform or substitute for nature’s glitch: one of changing paradigms in treatment with a constant reality check at every juncture in terms of complications and recurrence.
The story about the deformity radial hemimelia, radial dysplasia, radial dysmelia or radial club hand as we popularly refer to it is neither new nor too contrasting if we study it carefully looking both backwards and forwards. Paediatric orthopods, hand and plastic surgeons alike have been laboring painstakingly to better their own efforts at recreating the missing elements over the centuries to its contemporary garb (Fig. 1). It is one of those complex congenital anomalies the exact genesis of which is not completely clear but the treatment of which sees a confluence of three major branches of reconstructive surgery.

Figure 1: temoral evolution of Surgical Approaches to reconstruct the missing elements in Radial Hemimelia

Hence we have put together a special anniversary issue, the best known thinkers and experienced surgeons on the Indian scenario as well as extra special contribution from Boston children’s hospital as bonus: masters who are adept at their techniques and have penned down words of wisdom in great detail in the forthcoming chapters. Since the subject is multifaceted, vast and complex we have divided it into two issues.
Part 1 which deals with the evolution ,treatment and preliminary surgeries in infancy and toddler age group and part 2 which deals with the early and late childhood treatment of radial hemimelia and other issues. I would urge the reader to pay attention to the structuring of both these issues and delve into the depths of each sub topic ,as it has been put together with a great deal of thought .
One of the major milestones surely has been the rational concept of radialisation, early surgery, retaining growing cartilage and early pollicisation and that is why it has withstood the test of time. Distraction techniques have been another value addition to the changing face of modern day radial club hand treatment. I congratulate Prof Bhaskaranand and Dr Binoti Sheth for an exhaustive chapter about natural history, treatment and long term outcomes. The focused section on etiology and pathogenesis is albeit a little narrow in scope but by no means trivial and both the lead authors Dr Anirbaan Chaterjee and co-authors have done a commendable job of the same.
The chapter on pollicisation is both a brilliant compilation of literature review as well as lucid technique guide for the senior and junior surgeons alike; a task not as simple as it seems to achieve. Dr Thatte takes the reader elegantly through a surgical technique that truly defines the word reconstruction. The succinct expose by Dr Don Bae offers a thought provoking fresh perspective paving the way for yet another change in the management of this multi focal deformity. Our issue would have been incomplete without these two astute writers.
Before signing off I would ponder on the thought –whether in future genetic engineering and the much hyped concept of stem cells can bring about a dramatic change? My humble submission is that if it has taken centuries for surgeons to a arrive at as complete an approach as possible to the surgical management ,it would be wishful thinking that one can start playing God so soon in the near future.

People forget how fast you did a job—but they remember how well you did it.
–Howard W. Newton
And finally as the guest editor in chief in my chapter, I have endeavored to recount in a simplistic fashion the approach and technique that works best in my hands as that is the bottom line and to my mind a decision that matters the most clinically.

Dr Rujuta Mehta
Guest Editor

Dr. Rujuta Mehta

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