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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 […]

Radial Hemimelia Treatment – Decision Making and Management, Algorithm of Management, Operative Approaches, Results

Volume 2 | Issue 2 | May-Aug 2016 | Page 17-23| Rujuta Mehta Authors : Rujuta Mehta [1] [1 ] HOD .Dept of Paediatric Orthopaedics. B J Wadia hospital for Children, Parel, Mumbai [2] Dept of Paediatric Orthopaedics, Nanavati Superspeciality Hospital, Mumbai, India. Address of Correspondence Dr Rujuta Mehta HOD, Dept. of Paediatric Orthopaedics, B J […]

Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

Radial Hemimelia Treatment – Decision Making and Management, Algorithm of Management, Operative Approaches, Results

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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

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.

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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Radial Hemimelia Treatment – Decision Making and Management, Algorithm of Management, Operative Approaches, Results

Volume 2 | Issue 2 | May-Aug 2016 | Page 17-23| Rujuta Mehta

Authors : Rujuta Mehta [1]

[1 ] HOD .Dept of Paediatric Orthopaedics. B J Wadia hospital for Children, Parel, Mumbai
[2] Dept of Paediatric Orthopaedics, 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

Abstract

Radial hemimelia treatment has evolved a lot with many different types of surgical options; manifestations are myriad and presentations ages may vary from infancy to late childhood in most countries. A stepwise approach is needed for decision making and to manage this complex anomaly. The reader is presented here with a detailed clinical examination, decision making algorithm and an overview of surgical treatment options and the method preferred by the author with trends of interim and long term results and some other details in this review article.
Keywords: radial hemimelia, management, radial club hand

Introduction
Treatment for radial hemimelia has to be planned with a holistic picture in mind. The aim is to realign the carpus over the ulna in order to substitute for the missing radial pillar, as well as retain a good function, albeit without recurrence in an ideal world. The secondary issues of thumb aplasia and digital anomalies which invariably accompany the forearm deformity, need to be addressed separately: each on their own merit and severity. But since this anomaly involves multiple problems the whole balancing act is one of correct decision making and optimal clinical timing of each procedure.

Clinical examination
The first foundation for a proper decision making is a good clinical examination. The following typical features (as shown in Fig (1 and b) are often present and should be evaluated in detail

Figure 1 a: volar view of a radial club hand showing manus varus, severely bowed and shortened forearm ,mild thumb hypoplasia and symphallangic index and camptodactylous middle digits.

Figure 1 b: dorsal view of the same showing some amount of elbow hypoplasia clinically by lack of dimpling and flexion at the cubital fossa region, extremely short forearm and a pronounced volar subluxation at the wrist due to complete absence of the radial pillar

a) Mannus varus deformity of wrist
b) Shortened and or bowed forearm
c) With or without elbow synostosis or varying degrees of stiffness,
d) Thumb hypoplasia to aplasia,
e) Anomalies of the digits like symphallangism or camptodactyly,
f) Oligo dactyly or oligo-syndactyly.
The shoulder, elbow, wrist, and digital range of movement should be noted; in an infant often difficult; for active and passive motion. The ability to flex the elbow for hand-to-mouth function is examined, as this influences the treatment algorithm. Any procedure on the wrist is contra-indicated in the presence of an elbow synostosis. The position of the wrist with respect to the ulna and the ability to passively correct the radial deviation also are measured. The hand forearm angle (Fig 3a). as described by Manske and Goldfarb [1] should be carefully documented. The amount of ulnar bowing and angle of deviation from long axis should also be measured
The thumb is examined for hypoplasia and graded accordingly (elaborated in forthcoming part II of the symposium) Thumb deficiency contributes to functional impairment. Stiffness of the fingers is assessed, and the ability to grasp and release is determined via functional tasks. ‘Chop-stick ‘ grip between the index and middle finger or at times rarely even between ring and little finger is seen in late untreated children especially after 2 years of age . However the above features may vary with the degree of radial deficiency and the presence of associated anomalies. [2]. A thorough systemic evaluation is nevertheless mandated despite variations. The principal organ systems involved in these are the cardiac, renal, and hematological (though some of these may not manifest before 5 to 6 years of age), vertebral, occasionally foot and ankle, trachea-esophageal, and anal problems. Details of the various syndromes and their clinical relevance are explained in article by Chatterjee et al

Surgical Decision making
Type I radial club-hands have minor radial deviation of the wrist, which creates less of a functional problem than types II, III, and IV. (for details of classification refer to article by Sheth and Kumar) In these severe grades of dysplasia particularly with considerable absence of the radius, the wrist assumes severe radial deviation that increases to 90° over time. This further compromises the flexor and extensor tendons range and length, creating functional difficulty. The goals of treatment besides improving function of the extremity are [3,4] :
• To correct radial deviation
• Balance the wrist on the forearm
• Maintain mobility
• Promote growth of the forearm
• Enhance limb appearance for better body image
Centralization is indicated in radial club-hand types II, III, and IV, in which there is severe radial wrist deviation (than 30 degrees or more) [5] and insufficient support of the carpus. The carpal ring is pivoted back into position over the carpus by releasing all tethers on radial aspect, open reduction of ulno –carpal joint and capsulorrhaphy and restoring wrist stability, which is highly essential for development of a good power grip and other reconstructions. This is quite similar to what is done in developmental dysplasia of the hip. The optimum timing for performing centralization surgery is ideally 6 months of age before the onset of hand to mouth reflex. [8]. Correction of deformity early allows the brain cells to recognize normalized wrist alignment and mechanics as part of body image in the developing brain a phenomenon known as cortical adaption.
Thumb hypoplasia also requires consideration when formulating a treatment plan for radial club-hand. An absent or deficient thumb inhibits use of the hand in the correct prehensile way that was designed for human anatomy. Thumb reconstruction is usually delayed until after forearm treatment. Individuals not treated thus in the correct sequence of forearm and thumb correction may develop erroneous compensatory patterns like “chopsitick” action shown in (Fig 2b) which are very difficult to eliminate after early childhood as they get permanently established in the brain .

Figure 2 a: Adult with untreated bilateral radial hemimelia.

Figure 2 b: same adult patient demonstrating chopstick action between index and middle finger.

Contra-Indications
Contraindications for surgical intervention are a limited life expectancy in a child and elbow extension contractures that prevent the hand from reaching the mouth if the deformity at the wrist is corrected [6]. Surgery is also contraindicated for adults who have adjusted to their deformity (Fig 2a).

Investigations
As a protocol ALL radial dysplasia patients should be fully investigated and referred to pediatric subspecialists [2]. The heart is evaluated by auscultation and echocardiography. The kidneys are examined by ultrasound, and the platelet status is assessed by blood count and peripheral blood smear.
Plain radiographs are obtained to evaluate the degree of radial aplasia (Fig 3a) and to assess associated abnormalities of the elbow, wrist, and hand and spine when indicated . In radial club-hand, ossification is delayed,(fig 3b) and final determination of complete aplasia of the radius or carpus must be deferred until later almost up to the age of 8 years ( Fig 3c). In some cases a frank synostosis of the ulno- humeral joint may also be seen early. X rays of the whole spine AP and lateral should also be done as a screening to detect subtle VATER anomalies and vertebral involvement. In established cases it is better to evaluate the spinal involvement with more focused and detailed x rays and higher imaging whenever necessary.
For details of further investigations particularly the hematological ones which may have a bearing on the life expectancy of the child please refer to chapter no 2. The visceral anomalies get priority over centralization if life threatening , but with modern day anesthesia techniques of regional anaesthesia even high risk cases can be operated maybe a few months later than expected but within infancy period.

Figure 3 a: Hand forearm angle .the angle between the line D : which is the line connecting the midpoint of the transverse axis of the ulna with the midpoint of the transverse axis of the 3rd metacarpal base which has to be parallel to the long axis of the distal ulna. The angle B is then formed between the long axis of the 3 rd metacarpal and the perpendicular joining the midpoint where the line D ends.

Figure 3 b: X ray of a radial club hand at 4 months of age showing seemingly complete absence of radius and a very light cartilaginous shadow of the anlage .

Figure 3 c: x ray of the same child shown in fig 3 a, at 8 years of age showing good ossification of radius changing the grade of classification.

Surgical Treatment
Correction of radial club hand especially the more severe grades (which are incidentally commoner) requires a combination of non-operative and operative management that begins shortly after birth.

Early ( 0 to 3 months of age )
Treatment begins with counseling about nature and prognosis, when the child presents at birth to ensure a less drop- out rate and eradication of emotional and social stigma. Passive stretching of the taut radial structures is started at the initial visit. Stretching is performed at each diaper change and at bedtime. A stiff elbow with limited motion also is stretched during this time. Splint fabrication should be done with heat mouldable materials .Serial plasters are challenging until 3 months of age due to the extremely short working length of the forearm. When started at around 3 to 4 months of age however are certainly beneficial in maintain the suppleness and preventing wound complications post op.
Soft tissue distraction techniques [7], various fixator –distractor assemblies have been used with great enthusiasm for primary distraction both for late cases [13] and early in severe deformities. The author also prefers to use distraction with the mini ex fix for gr 3 and 4 radial club hands with angulation over 450. The principles, technique and results of the same have been highlighted in chapter [6].

Operative Methods
Early intervention takes advantage of the ability of the immature brain to adjust. Children with bilateral deficiencies that affect both the forearm and thumb require staged treatment to gain maximal use of the reconstructed limbs. Centralization of the wrist on the ulna to date remains a popular treatment to correct radial deviation. This procedure is performed in patients aged ideally 6 months of age before the onset of hand to mouth reflex vide Buck Gramko [8]. Surgery at this time allows for improvement in forearm length and provides a foundation for the development of motor function within the hand and for better cortical adaption in the growing immature brain.
Buck –Gramko [ 8] in his classical technique of Radialisation improved the ulnar lever arm by transferring the radial flexor and extensor to the ulnar aspect reversing the imbalance of the tight and strong radial musculo-tendinous units; which gained wide acceptance. This technique of tendon transfer is applicable and adequate to treat, grade 1 and 2 radial hemimelia cases where the deforming forces need to be converted to corrective forces in order to restore soft tissue balance at the wrist (Fig 4 a, b,c,d,e) . In an ideal world all radial club hands thus treated would not recur –but very often one encounters missing or short tendons especially in grade 3 and 4 radial dysplasia, and hence not available or adequate for a perfect rebalancing the deforming forces. As a pioneer in this field Prof. Buck Gramko also showed that excision of carpal bones and ulnar epiphyseal cartilage for grade 3 and 4 radial club hands ; is not only unnecessary, but its preservation leads to better range of wrist motion post surgery

Figure 4 a: X ray of Gr 2 radial club hand.

Figure 4 b: FCR identification for tendon transfer volar aspect grade 2 radial club hand.

Figure 4 c: ECR identification on dorsal aspect grade 2 radial club hand.

Figure 4 d: ECU identification on volar aspect in grade 2 radial club hand.

Figure 4 e: result after tendon transfer in grade 2 radial club hand showing complete correction.

Surgical Approaches
The skin incision and approach for open surgery has varied from author to author. Buck-Gramcko [8] and Lamb [3] favored the dorsal lazy S shaped approach (Fig 5a) this incision is not very aesthetic and runs opposite to the lines of langer and natural healing. Hence if there a wound dehiscence or hypertrophy it becomes a very unsightly scar .The disadvantages being a lack of radial skin in late and severe deformities and redundancy of the ulnar skin after correction, which needs unnecessary excision of good skin. Others have recommended a Z plasty (Fig 5b) along the concave radial border, and adding a longitudinal incision along the ulnar border overlying the ulno-carpal joint; the skin bridge between the two incisions however impedes access. Manske [1] used a single ulnar transverse ellipse incision (Fig. 5c) and so did Flatt but invariably both methods have been unable to solve the problem of unnecessary excision of ulnar skin.

Figure 5 a: dorsal incision as described by buck Gramcko

Figure 5 b: Z plasty incision radial aspect.

Figure 5 c: manske incision ulnar transverse ellipse.

Evans [9] in 1995 published his bilobed flap .He and his co authors have described a bilobed incision in which skin from the dorsum of the hand is transposed to the radial side to cater to the shortage, and the redundant excess is utilised to cover the defect now created on the dorsum.(Fig 6a,b).

Figure 6 a: Bilobed flaps raised , note forceps pointing over the ulnar redundancy flap.

Figure 6 b: Bilobed flaps moved , ulnar flap moved into dorsal and dorsal moved into radial defect , note forcep is pointing to the ulnar defect which closes by primary intention.

There is a significant learning curve associated with this flap. In experienced hands the incision may heal as beautifully as shown in fig (Fig 6c, d) and may not even be seen if covered with a bracelet or watch or shirt sleeve (Fig 6e,f)

Figure 6 c: Ulna flap perforator marked by arrow.

Figure 6 d: closure of moved dorsal flap over radial defect.

Figure 6 e: closure of moved ulna flap over dorsal defect.

Figure 6 f: healing of moved dorsal flap.

Figure 6 g: healing of dorsal flap and ulna defect.

This too is fraught with a pitfall of superficial necrosis if the adipo-venous plane is violated. (Fig 7 a,b) The trick however is to keep the flap harvest as glabrous as possible taking care to maintain the perforator vessel feeding the flap as shown in (Fig 6c) and not skeletonising the ulna head as otherwise it may contribute towards physeal growth arrest of distal ulna [10]. The final closure too must be without tension. The advantages of the overall 360 degree exposure of the upper end ulna and carpus achieved with this flap and the ease of reduction, far outweigh its disadvantages, which can be easily managed with conservative treatment.
Pilz murdin [11] compared the 5 diffirent incisions described above .according to them the preferred practice in each region has by and large varied a lot however , now the bilobed flap is gaining popularity . The author personally prefers to have the best of both worlds , i.e. combine the advantages of distraction with a good surgical exposure and aesthetics and hence has been practicing the below highlighted method since almost 17 years now the mid term results of which are already published [16]

Figure 7 a: superficial to moderate necrosis of dorsally moved ulna flap.

Figure 7 b: superficial necrosis of moved dorsal flap over radial defect

Pre-Operative protocol
The main preoperative emphasis is placed on the status of soft tissues. Stretching and splinting of the taut radial structures is required prior to surgery. Failure to elongate the tight radial side limits the ability to centralize the wrist on the ulna. Preliminary soft-tissue lengthening with an external fixator is a option in cases recalcitrant to stretching, such as in older children or patients with a recurrent deformity.[7, 16]
Preoperative measurements of the degree of active and passive motion of the digits and wrist are recorded. Radiographs in the anteroposterior and lateral projection, including the elbow and hand, are obtained. The degree of ulnar bow is calculated from the lateral radiograph as the angle between the proximal and distal ulna. Angulation of more than 45° usually requires corrective ulnar osteotomy at the time of centralization to realign the forearm. Some authors however delay the ulna osteotomy as a second stage. This too is well accepted and is a prerequisite before limb lengthening .The sites, method and technique with fixation of ulnar osteotomy and results have been eluded to in detail in part II of the symposia

Surgical technique /Essential Steps
1. Incision and exposure of radial side defect with incision and approach. The author is well versed with the bilobed Evan’s flap (Fig 8) for grade 4and prefers to use the same in majority of cases. for grades 2 and 3 where a significant amount of dissection is required on the ulnar side, the author prefers the Manske ulnar approach (Fig 5 c) .For grade 1 and 2 radial club –hands where the skin shortage is not there and hence rebalancing is not required; the author usually uses bilateral Z plasties if the skin is tight or the Manske approach coupled with a mirrored radial transverse half incision like a hemi cincinnati incision used in club feet.
2. Identification of median nerve which may be tented over the defect and retraction out of harm’s way.
3. Identification and isolation of flexor carpi radialis along with the muscle belly and harvest over a stay suture :(if present) or hypoplastic and short especially where the thumb is well developed and the grade of dysplasia is mild.
4. Identification of extensor carpi radialis along with muscle belly and freeing its excursion by releasing the tight sheaths (differentiation of longus and brevis or two slips maybe deficient), and harvest over a stay suture.
5. Identification and excision of radial anlage, freeing the carpus from underlying fibrotic tissue.
6. Mobilizing the carpus to open up the radial side soft tissue upto neutral.
7. Proceeding with identification of extensor carpi ulnaris which is usually thick, redundant and curved over the head of the ulna and retracting it away.
8. Identification of the flexor carpi ulnaris which may be short and stubby but hypertrophied.
9. Open reduction of wrist joint at the cleavage of ulno-carpal junction by a sharp dissection. The recognition of this plane is aided either by a sharp needle or with performing wrist dorsiflexion and palmar flexion.
10. Passing a kirshner wire retrograde through the 2nd metacarpal and then back into the carpus after moving the carpus ulnar ward, to centre the wrist over 2nd metacarpal base where possible. Some authors resort to creating a notch in the carpal bed and the tapering edge of ulna to fit the mismatched sizes into each other. This was practiced and probably still is by a large section of orthopaedic surgeons still doing a single stage procedure in late and difficult cases. The argument for this being that the stability that this affords to the reduction is very much needed for preventing recurrence. However long term studies on ulna growth patterns have shown that this further reduces the growth potential of the hypoplastic limb.
The author strongly advices against the notch plasty and personally prefers to use pre-op distraction to aid with the reduction and preserve the growing cartilage at the wrist.
11. Careful reefing of the wrist joint capsule, particularly over the dorsal carpal area, and double breasting where required to prevent future volar subluxation. Do not proceed with the completion of tendon transfer unless wrist capsule closure is secured.
12. Completion of tendon transfer of flexor carpi radialis to flexor carpi ulnaris on volar aspect, and extensor carpi radialis to extensor carpi ulnaris dorsally. If the tendons falls short periosteal sleeves form the base of the metacarpals may be used or simple the tethering force on radial side may be released ad attached as ulnar ward as possible.
13. Skin and soft tissue closure without tension and above elbow slab over a well double padded dressing.

Figure 8: Direction of flap movement , B transposes with rotation to A, and A transposes to radial defect.

Post Operative Protocol
Immediate active and passive digital motion is initiated, along with measures to reduce swelling. The timing of K-wire removal is largely need based. At least 6 to 8 weeks of fixation is desirable till the carpus stabilizes. Some authors however recommend 12 weeks prior to removal; in practical terms it is usually difficult to maintain for this long and may invite complications like loosening and infection. Following K-wire extraction, a splint is made and removed for exercises, with gradual weaning from the splint over the next 4-6 weeks. A night-time splint regimen is encouraged until skeletal maturity is reached.
At some centers the tendon and/or bony procedures are performed simultaneously to better align the forearm and to balance the wrist. The extensor carpi ulnaris is used to increase its moment arm for ulnar deviation. Other less common options are discussed in part II of the symposia. Unfortunately, no method reliably and permanently corrects the radial deviation, balances the wrist, and allows continued growth of the forearm [12, 13]. Recurrence ,complications and long term problems are discussed in part II of the symposia. The maintenance of the carpus at the end of the ulna without sacrificing wrist mobility or stunting forearm growth remains a daunting task.[12, 13]

Follow-up and Results
Patients with radial deficiencies require follow-up into adulthood. The evaluation should include not only the status of the centralization but also any additional operative and non-operative needs [14,15]. The shortened extremity with diminished motion may not be able to accomplish certain functions. These tasks often can be carried out with the use of assistive devices. In addition, as the child ages, distraction osteogenesis may be an option to increase forearm length. As this review is being published , the authors personal experience with results of long term follow up with solutions to tackle the bowstring effect causing residual radial deviation and especially volar re-subluxation with follow up results of ulna growth and breadth are being analysed . The trends show much higher percentage of ulna growth almost above 70% and the diameter hypertrophy is way beyond expectation (Fig 9 and 10). This goes to prove that the earlier the procedure is done, the realigned carpus remodels the ulna dome to form a stable receptacle and support. Thus the growing cartilage is retained and it adapts to the function of the missing radius well.

Figure 9: Clinical mid term result.

Complications are common following centralization and can occur at the time of surgery or during the post-operative or follow-up period. Intra-operatively damage to the median nerve should be meticulously avoided by the use of careful blunt dissection and magnification loops during the surgery. The most important complication is compartment syndrome in the initial post op period and must be guarded against, by avoiding water tight skin closure, using loose well-padded dressing. Most other complications with our technique are minor and do not impact the overall outcome. These include pin-tract irritation and a transient diminution in finger motion. When using the distraction techniques instances of physeal distraction have been reported. These can be managed by immediate removal of fixator and immobilization in POP cast.
Recurrence is the most common source of failure after operative treatment, and the cause appears to be multi-factorial. Operative causes of failure include the inability to obtain complete correction at surgery, inadequate radial soft-tissue release, and failure to balance the radial deforming force, early k wire removal, poor postoperative splint use, and the natural tendency of the shortened forearm and hand to deviate as a natural consequence of the bow string effect of the long flexor tendons.
The application of sophisticated techniques, such as distraction osteogenesis and microsurgery [13], to the treatment of radial club-hand introduces additional potential complications, such as fracture of the regenerate bone, digital stiffness from lengthening, and vascular thrombosis of the microsurgical anastomosis.

Chart 1: Algorithm for Management as per grade of Hemimelia

References

1. Manske PR, McCarroll HR Jr, Swanson K. Centralization of the radial club hand: an ulnar surgical approach. J Hand Surg [Am]. Sep 1981;6(5):423-33.
2. Goldfarb CA, Wall L, Manske PR. Radial longitudinal deficiency: the incidence of associated medical and musculoskeletal conditions. J Hand Surg [Am]. Sep 2006;31(7):1176-82.
3. Lamb DW. Radial club hand. A continuing study of sixty-eight patients with one hundred and seventeen club hands. J Bone Joint Surg Am. Jan 1977;59(1):1-3. [
4. Goldfarb CA, Klepps SJ, Dailey LA, Manske PR. Functional outcome after centralization for radius dysplasia. J Hand Surg Am. 2002 Jan;27(1):118-24
5. McCarroll HR. Congenital anomalies: a 25-year overview. J Hand Surg [Am]. Nov 2000;25(6):1007-37.
6. Bayne LG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg [Am]. Mar 1987;12(2):169-79.
7. Nanchahal J, Tonkin MA (1996). Pre-operative distraction lengthening for radial longitudinal deficiency. Journal of Hand Surg [Br] 21:103-7.
8. Buck-Gramcko D. Radialization as a new treatment for radial club hand. J Hand Surg [Am]. Nov 1985;10(6 Pt 2):964-8.
9. Evans DM, Gately DR, and Lewis JS.The use of bilobed flap in the correction of Radial Club Hand .Journal of Hand surgery, 1995; 20B:333-337,
10. Heikel HV. Aplasia and hypoplasia of the radius: studies on 64 cases and on epiphyseal transplantation in rabbits with the imitated defect. Acta Orthop Scand Suppl. 1959;39:1-155.
11. Pilz SM, Muradin MS, Van der Meule JJ, Hovius SE. Evaluation of five different incisions for correction of radial dysplasia. Journal of Hand Surg 1998;23B:183-5.
12. Sestero AM, Van Heest A, Agel J. Ulnar growth patterns in radial longitudinal deficiency. J Hand Surg[Am]. Jul-Aug 2006;31(6):960-7.
13. Vilkki SK. Distraction and microvascular epiphysis transfer for radial club hand. J Hand Surg [Br]. Aug 1998; 23(4):445-52.
14. Damore E, Kozin SH, Thoder JJ, et al. The recurrence of deformity after surgical centralization for radial clubhand. J Hand Surg [Am]. Jul 2000; 25(4):745-51.
15. Kozin SH. Upper-extremity congenital anomalies. J Bone Joint Surg Am. Aug 2003; 85-A (8):1564-76.
16. 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 Vol. 2008 Oct;33(5):616-21.

How to Cite this Article: Mehta R. Radial Hemimelia Treatment – Decision Making and Management, Algorithm Of Management, Operative Approaches, Results. International Journal of Paediatric Orthopaedics May-Aug 2016;2(2):17-23.

Dr. Rujuta Mehta

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Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

Radial Hemimelia Treatment – Decision Making and Management, Algorithm of Management, Operative Approaches, Results

Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

Radial Hemimelia Treatment – Decision Making and Management, Algorithm of Management, Operative Approaches, Results

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Radial Longitudinal Dysplasia: An Alternative Treatment Paradigm

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Radial Hemimelia Treatment – Decision Making and Management, Algorithm of Management, Operative Approaches, Results

Authors : Rujuta Mehta [1]

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