What’s New in Pediatric Orthopaedics: Hand and Upper Extremity Update Donald S. Bae, MD* and Charles A. Goldfarb, MDw

Key Words: pediatric, hand, congenital (J Pediatr Orthop 2014;34:S63–S67)

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s clinical care and scientific investigation have advanced, there has been increasing subspecialization within pediatric orthopaedic surgery. In recognition of this, the first Hand and Upper Extremity Subspecialty Program was held at the 2013 Annual Meeting of the Pediatric Orthopaedic Society of North America. Featuring a distinguished faculty of experts within the field of pediatric hand surgery, didactic presentations, faculty debates, and free papers were presented on a host of posttraumatic, congenital, and neuromuscular conditions. The purpose of this review is to summarize some of the highlights of these presentations.

POSTTRAUMATIC RECONSTRUCTION OF THE PEDIATRIC UPPER LIMB Elbow Humeral lateral condyle fractures are the second most common injury of the skeletally immature elbow. Principles of acute fracture management are well established and based primarily upon fracture displacement.1–4 Careful attention to these principles is essential to avoid the complications of nonunion and malunion. Nonunions of the lateral condyle do occur, even in initially nondisplaced fractures treated with cast immobilization, and may lead to pain, stiffness, progressive cubitus valgus, tardy ulnar nerve palsy, and arthrosis. Traditional treatment has focused upon attaining bony union through reduction, fixation, and bone grafting of the metaphyseal bone.5 These techniques provide high union rates, although nonanatomic reductions are often accepted due to the difficulty in achieving anatomic alignment and the risks associated with excessive disFrom the *Department of Orthopaedic Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA; and wDepartment of Orthopaedic Surgery, Washington University School of Medicine, St Louis Childrens Hospital, St. Louis, MO. The authors declare no conflicts of interest. Reprints: Donald S. Bae, MD, Department of Orthopaedic Surgery, Boston Children’s Hospital, Harvard Medical School, 300 Longwood Avenue, Hunnewell 2, Boston, MA 02115. E-mail: donald. [email protected]. Copyright r 2014 by Lippincott Williams & Wilkins

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section and nonunion fragment mobilization. More recently, combined lateral column compression fixation with supracondylar dome osteotomy has been proposed, with increased union and deformity correction.6 Intra-articular malunions are rare but challenging complications of lateral condyle fractures. Patients often present with pain, loss of elbow motion, and/or deformity. Corrective osteotomy of established intra-articular malunion is challenging given the difficulty in restoring articular congruity and attendant risks of osteonecrosis. In symptomatic patients with functionally limiting pain or loss of motion, intra-articular corrective osteotomy may improve radiographic alignment and elbow motion.7 In this study, Milch type 1 injuries achieved better clinical results with surgery compared to Milch type 2 injuries. Posttraumatic elbow contractures are also an unusual but challenging complication of pediatric elbow trauma. The cause of posttraumatic stiffness may be intrinsic or extrinsic to the elbow and pediatric patients with functionally limiting loss of elbow motion may benefit from surgical release. Surgery is typically performed via an extensile lateral or medial approach, with ulnar nerve decompression/transposition, removal of heterotopic bone, and selective capsulectomy.8–11 Postoperative care is rigorous and critical to successful results. Adequate analgesia, early range-of-motion exercises (including continuous-passive-motion machines immediately postoperatively), and diligent postoperative therapy is needed. With meticulous surgical technique and attention to these guiding principles, meaningful improvements of elbow flexion-extension motion may be achieved.12,13

Forearm Monteggia fracture dislocations refer to ulnar fractures with associated proximal radioulnar joint dissociation and radiocapitellar dislocation. Although the principles of acute Monteggia care are well established, chronic Monteggia lesions do occur with a missed initial diagnoses or loss of reduction.14 In patients with pain or functional limitations, surgical reconstruction may be considered, provided the articular surfaces of the proximal radius and capitellum are still congruent. Although a host of techniques have been advocated, the principles of chronic Monteggia reconstruction are well established: restoration of ulnar length and alignment with congruent and stable radiocapitellar reduction.15–22 Many authors have advocated a single-stage osteotomy of the

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ulna via a posterolateral extensile approach, protection of the radial and posterior interosseous nerves, corrective osteotomy of the ulnar malunion, and open radiocapitellar reduction. The need for annular ligament reconstruction remains debated. Although initial reports of Monteggia reconstruction were fraught with complications and suboptimal outcomes,23 more recent reports have demonstrated that with appropriate patient selection, adherence to surgical principles, and meticulous surgical technique, improvement in motion and function with preserved elbow stability may be achieved in approximately 80% of cases.15–22 Patients and families should be counseled, however, regarding the risk of recurrent instability and stiffness.

Wrist Posttraumatic distal radial growth arrest is estimated to occur in 4% to 5% of patients following distal radius physeal fractures.24 Risk factors include late or repeated forceful reduction attempts25 as well as the presence of an intra-articular distal radius physeal fracture (eg, Salter-Harris type III and IV injuries) which increases the risk of arrest (35%).26 Growth disturbance may lead to changes in ulnar variance and thus wrist biomechanics, resulting in pain and functional limitations generally from ulnocarpal impaction, triangular fibrocartilage complex (TFCC) tears, distal radioulnar joint (DRUJ) instability. In patients with established distal radius growth arrest, surgical treatment may lead to improved wrist alignment and function.27 The reconstructive strategy depends upon a number of factors, including pattern of arrest, growth remaining, ulnar variance, radial inclination, and associated soft-tissue (TFCC, DRUJ) pathology. In general, the goals of treatment are to restore neutral ulnar variance and >10 degrees of radial inclination, to repair or reconstruct concomitant soft-tissue pathology, and to avoid recurrent deformity. Surgical treatment options include ulnar shortening osteotomy, distal ulnar and/or radial epiphysiodesis, radial osteotomy, radial lengthening, TFCC repair, DRUJ stabilization, or combinations thereof.

Hand Although the vast majority of hand fractures are amenable to nonoperative treatment, surgical reduction and stabilization is recommended for displaced phalangeal neck fractures. Displacement results in alterations of the normally volarly concave subcondylar fossa,



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resulting in a mechanical block to interphalangeal joint flexion. As these injuries are far from the phalangeal physis, bony remodeling potential is limited. Closed reduction and percutaneous pin fixation is recommended as initial management for acute displaced fractures.28 Late presentation is common, due to failure of acute diagnosis or false assumptions regarding the injury severity or remodeling potential. Treatment for the late-presenting phalangeal neck fracture depends upon a number of factors, including patient age, magnitude of deformity, functional limitations, and time from injury. Bony remodeling is most reliable in young patients with considerable growth remaining in whom the primary deformity is in the flexionextension plane.29–32 Remodeling may take years, and rotational and/or coronal plane malalignment will not reliably improve. In patients with nascent malunions, percutaneous osteoclasis, reduction, and pinning may be considered with the best results in the patient with persistent tenderness at the fracture site, radiographically visible fracture line, interphalangeal joint motion of 80% involve the EXT 1 or 2 gene mutations (EXT 1 is more concerning as it carries a higher risk of sarcoma transformation). Growth of these exostoses may cause pain but more problematic is the bony deformity, restricted motion, and asymmetric growth, especially in the forearm. Surgical excision may be sufficient for isolated, symptomatic exostoses from the scapula, humerus, forearm, or digits but avoidance of deformity and long-term disability is the primary goal. The most problematic presentation of forearm disease is shortening of the ulna which can lead to radius bowing, radial head dislocation, and ulnar translocation of the carpus. The ideal preventative strategy remains elusive but considerations include excision of problematic osteochondroma with hopeful restoration of ulna growth, ulna lengthening, radius osteotomy, partial or complete growth arrest of radius, and/or detethering of the radius and ulna. The ultimate goal is a balanced wrist and a reduced radiocapitellar joint/proximal radioulnar joint. Once the radial head is dislocated, reconstruction is challenging and salvage procedures are considered including a single-bone forearm.42–49 The indications for a single-bone forearm are pain in the setting of severe forearm deformity with radial head dislocation. Forearm rotation is typically poor and the wrist may be malaligned. Conceptually, the one bone forearm offers a permanent solution to a difficult problem with high patient and family satisfaction. Several technique pearls may be helpful. The distal ulna and proximal radius are excised in an extraperiosteal manner. The biceps insertion is either maintained or transferred to the proximal ulna. The posterior interosseous nerve is protected and prophylactic fasciotomies may be helpful to minimize complications. The forearm is aligned in slight pronation, intercalary bone graft (from excised radius and ulna) is placed between the bones, and the compression screws and/or a plate is used to stabilize the radius and ulna.50

Arthrogryposis Upper extremity manifestations of arthrogryposis vary by specific joint involvement and disease severity. The overall treatment goals include ambulation and independence with activities of daily living, including feeding and perineal care. Early therapy including passive stretching and splinting can be helpful but are typically insufficient to avoid surgery. Upper extremity manifestations vary from distal only finger involvement to a more www.pedorthopaedics.com |

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complete presentation with involvement of each joint from the shoulder to the fingers. The upper extremities are not considered in isolation as lower extremity manifestations affect upper extremity intervention, from timing of surgery to specific procedures considered. Psychosocial considerations are also vital in this population including the family’s ability to cooperate with therapy and the economic strain of multiple surgeries. The elbow is most commonly stiff in extension and thus limits feeding and self-care—it is a primary focus of intervention. Elbow flexibility and power are separate issues, but the elbow must be flexible before muscle transfer can be considered for active flexion.51,52 The hallmark operation to provide elbow flexion is posterior elbow capsulotomy with triceps lengthening.53 In arthrogryposis, the flexed wrist is often used for support, especially in children with severe lower extremity involvement, as locomotion on the ground can be assisted with pressure on the dorsal wrist. This not only exacerbates the flexion posture but dictates caution for procedures that correct wrist position. If the wrist is corrected to neutral but locomotion patterns are unchanged, recurrence of flexion posture is likely. In addition, finger flexibility and use patterns with the wrist flexed are considered before any corrective procedure to prevent any functional worsening. Nonetheless, a closing-wedge osteotomy through the midcarpal joint of the wrist can effectively improve wrist position and improve function. Extensor carpi ulnaris tendon transfer can accompany the osteotomy to maximize wrist extension power.54 This is a well-tolerated, reliable procedure with high patient and family satisfaction. Hand procedures can also be functionally helpful in selected patients. Most commonly, the clasped thumb posture is corrected with thumb-index webspace deepening, with or without concomitant thumb metacarpophalangeal chondrodesis and tendon transfer. Positioning the thumb out of the palm both increases thumb use for large object grasp and allows improved finger flexion by removing the thumb impediment to flexion.55 ACKNOWLEDGMENTS The authors wish to acknowledge the contributions of the Hand and Upper Extremity Specialty Day faculty: Andrea Bauer, MD; Lisa Lattanza, MD; Apurva Shah, MD MBA; Roger Cornwall, MD; Ann Van Heest, MD; Marybeth Ezaki, MD; Michelle James, MD; Dan Zlotolow, MD; Michael Garcia, MD; and Peter Waters, MD.

REFERENCES 1. Foster DE, Sullivan JA, Gross RH. Lateral humeral condylar fractures in children. J Pediatr Orthop. 1985;5:16–22. 2. Flynn JC, Richards JF Jr, Saltzman RI. Prevention and treatment of non-union of slightly displaced fractures of the lateral humeral condyle in children. An end-result study. J Bone Joint Surg Am. 1975;57:1087–1092. 3. Pirker ME, Weinberg AM, Hollwarth ME, et al. Subsequent displacement of initially nondisplaced and minimally displaced fractures of the lateral humeral condyle in children. J Trauma. 2005;58:1202–1207.

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4. Mintzer CM, Waters PM, Brown DJ, et al. Percutaneous pinning in the treatment of displaced lateral condyle fractures. J Pediatr Orthop. 1994;14:462–465. 5. Wattenbarger JM, Gerardi J, Johnston CE. Late open reduction internal fixation of lateral condyle fractures. J Pediatr Orthop. 2002;22:394–398. 6. Tien YC, Chen JC, Fu YC, et al. Supracondylar dome osteotomy for cubitus valgus deformity associated with a lateral condylar nonunion in children. J Bone Joint Surg Am. 2005;87:1456–1463. 7. Bauer AS, Bae DS, Brustowicz KA, et al. Intra-articular corrective osteotomy of humeral lateral condyle malunions in children: early clinical and radiographic results. J Pediatr Orthop. 2013;33:20–25. 8. Cohen MS, Hastings H II. Post-traumatic contracture of the elbow. Operative release using a lateral collateral ligament sparing approach. J Bone Joint Surg Br. 1998;80:805–812. 9. Mansat P, Morrey BF. The column procedure: a limited lateral approach for extrinsic contracture of the elbow. J Bone Joint Surg Am. 1998;80:1603–1615. 10. Urbaniak JR, Hansen PE, Beissinger SF, et al. Correction of posttraumatic flexion contracture of the elbow by anterior capsulotomy. J Bone Joint Surg Am. 1985;67:1160–1164. 11. Hotchkiss RN. Treatment of the stiff elbow. In: Green D, Hotchkiss RN, Pederson WC, Wolfe SW, eds. Green’s Operative Hand Surgery. 5th ed. New York: Churchill Livingstone; 1999:939–958. 12. Bae DS, Waters PM. Surgical treatment of posttraumatic elbow contracture in adolescents. J Pediatr Orthop. 2001;21:580–584. 13. Darlis NA, Kaufmann RW, Sotereanos DG. Open surgical treatment of post-traumatic elbow contractures in adolescent patients. J Shoulder Elbow Surg. 2006;15:709–715. 14. Ring D, Waters PM. Operative fixation of Monteggia fractures in children. J Bone Joint Surg Br. 1996;78:734–739. 15. Bae DS, Waters PM. Surgical treatment of acute and chronic Monteggia fracture-dislocations. Oper Techn Orthop. 2005;15:308–314. 16. Bell Tawse AJ. The treatment of malunited anterior Monteggia fractures in children. J Bone Joint Surg Br. 1965;47:718–723. 17. Exner GU. Missed chronic anterior Monteggia lesion. Closed reduction by gradual lengthening and angulation of the ulna. J Bone Joint Surg Br. 2001;83:547–550. 18. Gyr BM, Stevens PM, Smith JT. Chronic Monteggia fractures in children: outcome after treatment with the Bell-Tawse procedure. J Pediatr Orthop B. 2004;13:402–406. 19. Inoue G, Shionoya K. Corrective ulnar osteotomy for malunited anterior Monteggia lesions in children. 12 patients followed for 1-12 years. Acta Orthop Scand. 1998;69:73–76. 20. Hui JH, Sulaiman AR, Lee HC, et al. Open reduction and annular ligament reconstruction with fascia of the forearm in chronic Monteggia lesions in children. J Pediatr Orthop. 2005;25:501–506. 21. Degreef I, De Smet L. Missed radial head dislocations in children associated with ulnar deformation: treatment by open reduction and ulnar osteotomy. J Orthop Trauma. 2004;18:375–378. 22. Kim HT, Conjares JN, Suh JT, et al. Chronic radial head dislocation in children, part 1: pathologic changes preventing stable reduction and surgical correction. J Pediatr Orthop. 2002;22:583–590. 23. Rodgers WB, Waters PM, Hall JE. Chronic Monteggia lesions in children. Complications and results of reconstruction. J Bone Joint Surg Am. 1996;78:1322–1329. 24. Cannata G, De Maio F, Mancini F, et al. Physeal fractures of the distal radius and ulna: long-term prognosis. J Orthop Trauma. 2003; 17:172–179; discussion 179-180. 25. Lee BS, Esterhai JL Jr, Das M. Fracture of the distal radial epiphysis. Characteristics and surgical treatment of premature, post-traumatic epiphyseal closure. Clin Orthop Relat Res. 1984;185:90–96. 26. Fu ED, Shah AS, Waters PM, et al. Growth Disturbance Following Intraarticular Distal Radius Fractures in the Skeletally Immature Patient. Toronto, Canada: Annual Meeting of the Pediatric Orthopaedic Society of North America; 2013. 27. Waters PM, Bae DS, Montgomery KD. Surgical management of posttraumatic distal radial growth arrest in adolescents. J Pediatr Orthop. 2002;22:717–724. 28. Al-Qattan MM. Phalangeal neck fractures in children: classification and outcome in 66 cases. J Hand Surg Br. 2001;26:112–121. r

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29. Puckett BN, Gaston RG, Peljovich AE, et al. Remodeling potential of phalangeal distal condylar malunions in children. J Hand Surg Am. 2012;37:34–41. 30. Cornwall R, Waters PM. Remodeling of phalangeal neck fracture malunions in children: case report. J Hand Surg Am. 2004;29:458–461. 31. Hennrikus WL, Cohen MR. Complete remodelling of displaced fractures of the neck of the phalanx. J Bone Joint Surg Br. 2003;85:273–274. 32. Mintzer CM, Waters PM, Brown DJ. Remodelling of a displaced phalangeal neck fracture. J Hand Surg Br. 1994;19:594–596. 33. Waters PM, Taylor BA, Kuo AY. Percutaneous reduction of incipient malunion of phalangeal neck fractures in children. J Hand Surg Am. 2004;29:707–711. 34. Simmons BP, Peters TT. Subcondylar fossa reconstruction for malunion of fractures of the proximal phalanx in children. J Hand Surg Am. 1987;12:1079–1082. 35. Oberg KC, Feenstra JM, Manske PR, et al. Developmental biology and classification of congenital anomalies of the hand and upper extremity. J Hand Surg Am. 2010;35:2066–2076. 36. Kotwal PP, Varshney MK, Soral A. Comparison of surgical treatment and nonoperative management for radial longitudinal deficiency. J Hand Surg Eur Vol. 2012;37:161–169. 37. Sestero AM, Van Heest A, Agel J. Ulnar growth patterns in radial longitudinal deficiency. J Hand Surg Am. 2006;31:960–967. 38. Dana C, Auregan JC, Salon A, et al. Recurrence of radial bowing after soft tissue distraction and subsequent radialization for radial longitudinal deficiency. J Hand Surg Am. 2012;37:2082–2087. 39. Oishi SN, Carter P, Bidwell T, et al. Thrombocytopenia absent radius syndrome: presence of brachiocarpalis muscle and its importance. J Hand Surg Am. 2009;34:1696–1699. 40. VanHeest A, Grierson Y. Dorsal rotation flap for centralization in radial longitudinal deficiency. J Hand Surg Am. 2007;32:871–875. 41. Pike JM, Manske PR, Steffen JA, et al. Ulnocarpal epiphyseal arthrodesis for recurrent deformity after centralization for radial longitudinal deficiency. J Hand Surg Am. 2010;35:1755–1761. 42. Noonan KJ, Levenda A, Snead J, et al. Evaluation of the forearm in untreated adult subjects with multiple hereditary osteochondromatosis. J Bone Joint Surg Am. 2002;84-A:397–403.

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43. Darilek S, Wicklund C, Novy D, et al. Hereditary multiple exostosis and pain. J Pediatr Orthop. 2005;25:369–376. 44. Jager M, Westhoff B, Portier S, et al. Clinical outcome and genotype in patients with hereditary multiple exostoses. J Orthop Res. 2007;25:1541–1551. 45. Ishikawa J, Kato H, Fujioka F, et al. Tumor location affects the results of simple excision for multiple osteochondromas in the forearm. J Bone Joint Surg Am. 2007;89:1238–1247. 46. Shin EK, Jones NF, Lawrence JF. Treatment of multiple hereditary osteochondromas of the forearm in children: a study of surgical procedures. J Bone Joint Surg Br. 2006;88:255–260. 47. Bottner F, Rodl R, Kordish I, et al. Surgical treatment of symptomatic osteochondroma. A three- to eight-year followup study. J Bone Joint Surg Br. 2003;85:1161–1165. 48. Waters PM, Van Heest AE, Emans J. Acute forearm lengthenings. J Pediatr Orthop. 1997;17:444–449. 49. Rodgers WB, Hall JE. One-bone forearm as a salvage procedure for recalcitrant forearm deformity in hereditary multiple exostoses. J Pediatr Orthop. 1993;13:587–591. 50. Waters PM. Forearm rebalancing in osteochondromatosis by radioulnar fusion. Tech Hand Up Extrem Surg. 2007;11: 236–240. 51. Gogola GR, Ezaki M, Oishi SN, et al. Long head of the triceps muscle transfer for active elbow flexion in arthrogryposis. Tech Hand Up Extrem Surg. 2010;14:121–124. 52. Goldfarb CA, Burke MS, Strecker WB, et al. The Steindler flexorplasty for the arthrogrypotic elbow. J Hand Surg Am. 2004;29:462–469. 53. Van Heest A, James MA, Lewica A, et al. Posterior elbow capsulotomy with triceps lengthening for treatment of elbow extension contracture in children with arthrogryposis. J Bone Joint Surg Am. 2008;90:1517–1523. 54. Ezaki M, Carter PR. Carpal wedge osteotomy for the arthrogrypotic wrist. Tech Hand Up Extrem Surg. 2004;8: 224–228. 55. Ezaki M, Oishi SN. Index rotation flap for palmar thumb release in arthrogryposis. Tech Hand Up Extrem Surg. 2010;14:38–40.

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What's new in pediatric orthopaedics: hand and upper extremity update.

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