SCIENTIFIC ARTICLE

Osteosarcoma of the Distal Radius Treated With Segmental Forearm Resection, Hand Replantation, and Subsequent Limb Lengthening: Case Report Hiroshi Hatano, MD, Tetsuro Morita, MD, Hiroto Kobayashi, MD, Yasuhiro Iwabuchi, MD

A 9-year-old girl with osteosarcoma of the radius was treated with segmental forearm resection and replantation followed by forearm lengthening of 11 cm. At 9-year follow-up, she had recovered sensory function, and her pinch and grasp were sufficient for performing daily activities. Functional outcomes evaluated by the Disabilities of the Arm, Shoulder, and Hand questionnaire and International Society of Limb Salvage functional score system were 4/100 and 23/30, respectively. (J Hand Surg Am. 2014;-:-e-. Copyright Ó 2014 by the American Society for Surgery of the Hand. All rights reserved.) Key words Distal radius, limb lengthening, limb salvage, resection-replantation.

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therapy for malignant bone and soft tissue tumors. Tumor resection leaves massive bone defects; therefore, several reconstructive techniques have been used for forearm reconstruction.1,2 However, amputation is occasionally necessary to achieve curative resection when the tumors have massive neurovascular involvement. Amputation of the upper extremity results in permanent physical disability and may have profound psychosocial implications. Resection-replantation, reported by Windhager et al,3 is a surgical method of partial limb salvage in patients with upper extremity malignancies that otherwise would be unresectable. This procedure comprises segmental resection of the tumorbearing region of the forearm and replantation of the distal segment of the limb with necessary shortening.3e5 This partially preserves limb function and avoids IMB SALVAGE SURGERY IS A STANDARD

From the Department of Orthopedic Surgery, Niigata Cancer Center Hospital, Kawagishicho, Chuoku, Niigata; and the Iwabuchi Orthopedic Clinic, Yoshida, Tsubame, Niigata Pref, Japan. Received for publication December 12, 2013; accepted in revised form March 24, 2014. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Hiroshi Hatano, MD, Department of Orthopedic Surgery, Niigata Cancer Center Hospital, 2-15-3, Kawagishicho, Chuoku, Niigata, Niigata Pref, 951-8566, Japan; e-mail: [email protected]. 0363-5023/14/---0001$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2014.03.030

amputation. However, the unusual appearance of a shortened upper extremity remains an unsolved issue. Distraction lengthening has been used in the management of deformity and shortening of forearm skeletal deficiencies.6e8 We present long-term results of resection-replantation with subsequent limb lengthening in a 9-year-old girl with an osteosarcoma of the distal radius. CASE REPORT A right-handed 9-year-old girl presented with a hard mass in her left forearm. Radiographs showed a massive osteoblastic lesion of the left distal radius with a circumferential extraosseous mass (Fig. 1). Open biopsy revealed a conventional osteosarcoma. She received neoadjuvant chemotherapy involving methotrexate, doxorubicin, cisplatin, and ifosfamide. Restaging magnetic resonance imaging showed progressive disease with massive enlargement of the tumor (Fig. 2). Because the tumor invaded the median, radial, and ulnar nerves and the radial and ulnar arteries, amputation was recommended in order to achieve complete resection. The patient and her parents refused amputation and desired a limb-sparing option. We therefore suggested resection-replantation as a means to allow for wide tumor resection and avoid complete amputation. The potential limitations in hand function after this reconstruction and the unusual appearance after shortening of the limb were discussed carefully with her and her parents. We also

Ó 2014 ASSH

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Published by Elsevier, Inc. All rights reserved.

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FOREARM LENGTHENING AFTER RESECTION-REPLANTATION

FIGURE 1: A Anteroposterior and B lateral radiographs on presentation show an osteosarcoma of the left distal radius. Abundant periosteal new bone formation extended from the middle of the diaphysis to the distal radius.

FIGURE 2: Restaging axial magnetic resonance image shows circumferential involvement of the forearm. All of the critical neurovascular structures as well as the ulna (arrow) were invaded by the tumor.

planned distraction lengthening of the forearm after completion of postoperative chemotherapy. During surgery, the tumor-bearing region of the distal forearm was completely resected as a segment including the radius (15 cm), lunate, ulna (13 cm), all neurovascular structures, soft tissue, and skin (Fig. 3). The hand was covered with gauze sponges soaked in saline at room temperature until replanted. The ulna was impacted into the residual carpals; then, total wrist arthrodesis was secured with 3 Kirschner wires. Because we chose the ulna to create a 1-bone forearm and to use for subsequent lengthening, the residual radius was left unfixed (Fig. 4). The radial artery, 1 adjacent vein, and 2 subcutaneous veins were anastomosed end to end. The ulnar artery was not repaired because a sufficient blood supply from the radial artery was observed. The limb ischemia time was 1 hour (time from clamp to perfusion). Neurorrhaphy of both the median and the ulnar nerves was performed microsurgically. Because the amount of resection of the radial side was larger than that of the ulnar side, the superficial radial nerve could not be coapted. The long digital flexor and extensor tendons were fixed to their corresponding muscles except for the flexor pollicis longus tendon, which was transferred to the brachioradialis. The postoperative course was uneventful, and chemotherapy was resumed 2 weeks later. Methotrexate, J Hand Surg Am.

FIGURE 3: The forearm just before completion of the segmental resection of the tumor-bearing region. To reduce ischemic damage during resection-replantation, continuity of the major vessels was maintained until just before complete resection.

doxorubicin, cisplatin, and ifosfamide were discontinued 10 months after surgery. For callus distraction of the ulna, corticotomy and application of an Orthofix external fixator (MiniRail Long Lengthener, Orthofix, Lewisville, TX) were performed 4 weeks after completion of chemotherapy and confirmation of recovery from myelosuppression. Distraction lengthening was started 11 days after corticotomy at a rate of 0.5 mm/d. The forearm was lengthened for 194 days, and 9 cm of length was gained. During the distraction lengthening of the ulna, the residual radius gradually migrated distally, and this led to a cubitus valgus r

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FOREARM LENGTHENING AFTER RESECTION-REPLANTATION

FIGURE 4: Radiographs show the distraction-lengthening process. A Before distraction lengthening (3 mo after resection-replantation). Bone union between the ulna and the carpals had occurred. B Radiograph obtained 6 months after starting distraction lengthening. The residual radius (arrow) had gradually migrated distally. C Nine years after the initial surgery. Although the ulna showed posterior bowing, distraction lengthening gained 11 cm in ulna length.

deformity (Fig. 4). Although a 7-cm shortening of the forearm remained, the device was removed 7 months after the discontinuation of lengthening and confirmation of consolidation of bone regeneration. Six months after removal of the device, the radius was surgically repositioned to a point where the radiocapitellar joint was stable. To prevent retranslocation, the radius was fixed to the ulna with 2 cancellous screws. A second distraction lengthening was then started using the same type of external fixator. However, bone regeneration was poor, and digital stiffness developed. Therefore, the second lengthening was terminated after 6 months with 2 cm of elongation. Six years after the initial surgery, the patient presented with a solitary metastasis in the right lung. She

J Hand Surg Am.

TABLE 1. The ROM (Extension/Flexion [ ]) and 2-Point Discrimination Distance of the Finger Joints of the Replanted Hand DIP

2-PD (mm)

MCP

PIP

Thumb

10/35

10/40*

Index

15/35

10/45

15/35

4

Middle

15/35

10/50

15/35

3

Ring

15/35

10/50

15/35

3

Little

20/35

10/50

15/35

3

3

DIP, distal interphalangeal joint; MCP, metacarpophalangeal joint; PIP, proximal interphalangeal joint. *ROM of the interphalangeal joint.

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FIGURE 5: A The clinical appearance at 9 years after the surgery. B, C Function at follow-up. The patient can grasp a 500-mL plastic bottle full of liquid against gravity B and hold a key with opposition of the thumb C.

underwent partial resection of the right lung followed by postoperative chemotherapy. Nine years after the original surgery, further tumors were not evident. A pulp-to-pulp pinch strength was present only between the thumb and the middle finger and measured 1.4 kgf; 44% of the opposite side. Holding an object, such as a 500-ml plastic bottle full of liquid, against gravity with a whole-hand grasp was possible. Grip strength was 6.1 kgf; 25% of the opposite hand. Opposition of the thumb was present and intrinsic thenar muscles were functional and of practical use. For instance, she could hold a smart phone and use the touchscreen with her left thumb. The other intrinsic muscles, however, were not functional. The range of motion (ROM) and 2-point discrimination of each finger of the left hand are shown in Table 1. The ROM of the left elbow was from 0 to 100 with her forearm fixed in 10 to 20 of supination. Even though posterior bowing of the ulna with a 7-cm J Hand Surg Am.

shortening remained (Fig. 4), the patient and her parents were satisfied with the function and appearance of the upper extremity (Fig. 5) and did not want further corrective surgery. Functional outcomes evaluated by the Disabilities of the Arm, Shoulder, and Hand questionnaire9 and International Society of Limb Salvage functional scoring system10 were 4/100 and 23/30, respectively. DISCUSSION Amputation has been the primary option to achieve complete resection of tumors in the upper extremity when there is massive neurovascular involvement. However, amputation through the forearm results in a functional deficit and also causes detrimental psychological effects. Resection-replantation is a partial limb salvage surgery that can achieve a wide tumor resection margin and retain limb function and appearance to r

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a certain extent; therefore, in selected cases, it may be superior to complete forearm amputation.3e5 Following Windhager et al,3 several authors have described the results of resection-replantation for tumors in the upper extremity.4,5 However, few patients underwent surgery at a level between the elbow and the wrist.3e5 Unlike the present case, at least 1 of the major nerves in the forearm was preserved in most reported cases3e5; therefore, specific details regarding limb function after complete segmental forearm resection-replantation are not available. Distraction lengthening has been used to correct deformity and shortening in congenital and traumatic skeletal deficiencies of the forearm in children.6e8 The maximum extra length obtained by forearm lengthening appears to be limited to approximately 13 cm, probably owing to a decreased regeneration potential of the tapered bone and soft tissue and an increased chance for complications when a prolonged period of treatment is required.6e8 In our case, despite gaining 11 cm of length, a discrepancy in forearm length remained. The patient’s manual dexterity was impaired, but recovery of sensory function and pinching and grasping functions was sufficient for performing activities of daily living. Although the final appearance and function of the upper extremity was by no means normal, she was satisfied with the outcome. We believe that resectionreplantation with subsequent lengthening of the forearm causes less body image disturbance than does amputation. Complications that might be encountered during distraction lengthening of the forearm include pin track infection, joint contractures, neurovascular injury, delayed union, malunion, fracture, and recurrence of the deformity.6e8 On rare occasions, the distraction by an external fixator causes the interosseous membrane to drag the radius distally.6 The radius migration leads to a

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cubitus valgus deformity, similar to our observation, because elbow joint stability is diminished. Fixation between the ulna and the radius is advised to prevent radius migration.6 Intramedullary pinning is advocated to manage angular deformity during forearm lengthening.7,8 We offered further lengthening and correction of the posterior bowing of the ulna by this method, but the patient and her parents did not want further corrective surgery because they were satisfied with the function and appearance of the upper extremity. REFERENCES 1. Muramatsu K, Ihara K, Yoshida K, Tominaga Y, Hashimoto T, Toguchi T. Musculoskeletal sarcomas in the forearm and hand: standard treatment and microsurgical reconstruction for limb salvage. Anticancer Res. 2013;33(10):4175e4182. 2. Hatano H, Morita T, Kobayashi H, Otsuka H. A ceramic prosthesis for the treatment of tumours of the distal radius. J Bone Joint Surg Br. 2006;88(12):1656e1658. 3. Windhager R, Millesi H, Kotz R. Resection-replantation for primary malignant tumours of the arm. An alternative to fore-quarter amputation. J Bone Joint Surg Br. 1995;77(2):176e184. 4. Hahn SB, Choi YR, Kang HJ, Shin KH. Segmental resection and replantation have a role for selected advanced sarcomas in the upper limb. Clin Orthop Relat Res. 2009;467(11):2918e2924. 5. El-Gammal TA, El-Sayed A, Kotb MM. Resection replantation of the upper limb for aggressive malignant tumors. Arch Orthop Trauma Surg. 2002;122(3):173e176. 6. Masada K, Tsuyuguchi Y, Kawai H, Kawabata H, Noguchi K, Ono K. Operations for forearm deformity caused by multiple osteochondromas. J Bone Joint Surg Br. 1989;71(1):24e29. 7. Abe M, Shirai H, Okamoto M, Onomura T. Lengthening of the forearm by callus distraction. J Hand Surg Br. 1996;21(2):151e163. 8. Seitz WH Jr, Shimko P, Patterson RW. Long-term results of callus distraction-lengthening in the hand and upper extremity for traumatic and congenital skeletal deficiencies. J Bone Joint Surg Am. 2010; 92(Suppl 2):47e58. 9. Imaeda T, Toh S, Nakao Y, et al. Validation of the Japanese Society for Surgery of the Hand version of the Disability of the Arm, Shoulder, and Hand questionnaire. J Orthop Sci. 2005;10(4):353e359. 10. Enneking WF, Dunham W, Gebhardt MC, Malawar M, Pritchard DJ. A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop Relat Res. 1993;286:241e246.

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Osteosarcoma of the distal radius treated with segmental forearm resection, hand replantation, and subsequent limb lengthening: case report.

A 9-year-old girl with osteosarcoma of the radius was treated with segmental forearm resection and replantation followed by forearm lengthening of 11 ...
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