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A 15-year-old patient with a history of left-sided type 3 radial longitudinal deficiency
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A 15-year-old, right-hand dominant female patient presented to our office with a history of left-sided type 3 radial longitudinal deficiency. She was initially treated in infancy at an outside hospital with centralization and pollicization procedures. Subsequent to her initial surgeries, she developed worsening of her limb-length discrepancy and bowing of her forearm secondary to damage to her distal ulnar physis during centralization. Physical examination of her left upper extremity on presentation was notable for elbow range of motion from 0° to 110° flexion. She exhibited complete absence of forearm pronation and supination, 10° arc of motion in wrist flexion and extension, and 30° radial deviation without any ulnar deviation. Her past medical history was otherwise unremarkable, and she lacked any syndromic associations with her radial longitudinal deficiency (RLD).
Given continued deformity with dissatisfaction in both functional and aesthetic results after her prior procedures, as well as lack of remaining growth, the patient was referred to the Limb Lengthening & Complex Reconstruction Service for continued care. Radiographs of the patient’s left forearm performed on the day of the clinic visit demonstrated complete absence of the distal radius with associated hypoplasia of the proximal radius and post-pollicization changes in the hand with a hypoplastic carpus (Figure 1).
Source: S. Robert Rozbruch, MD
What treatment would improve the patient’s functional and aesthetic outcome?
See answer on next page.
Distraction osteogenesis
Preoperative templating was performed to identify center of rotation of angulation and locations for osteotomy. A proximal and distal location was identified for optimal correction of angular deformity. This allowed for distraction osteogenesis at two sites with the potential to increase the rate of distraction, given the patient’s young age.
Correction of deformity proceeded with application of a three-ring Ilizarov frame (Figure 2). A half ring was placed in the proximal ulna stabilized with a wire and two dorsal half pins. This was perpendicular to the proximal ulnar segment in both planes. Two hinges at the apex of the proximal deformity were placed along the convexity of the deformity to allow for maximal correction of angular deformity. The middle segment was stabilized with a full ring held by one tension wire and two dorsal half pins. A second set of hinges were placed from the middle to the distal ring along the convexity at maximum deformity. The distal ring was stabilized with a tension olive wire, a tension smooth wire and a half pin. A metacarpal wire was placed with an olive on the far side of the distal ring.
Due to severe angular deformity, a compression rod was placed along the convexity at the proximal deformity and a distraction rod was placed along the concavity at the distal deformity to mirror the frame’s hinge constructs. Multiple drill hole transverse osteotomy was performed in the proximal and distal ulna to provide two sites of length and angular correction. A 3-day latency period occurred before starting distraction.
The duration of lengthening was 63 days. Distraction length was 35 mm (Figure 3). External fixation index was 5.6 weeks/cm. The frame was left in place for an additional 2 months to allow for consolidation prior to removal. These rates of lengthening were similar to those reported in the literature. Matsuno and colleagues reported 6.8 weeks mean radius lengthenings in types 2 and 3 RLD. Peterson and colleagues reported an average index of 8.6 weeks for ulnar lengthening.
RLD represents a wide spectrum of upper extremity anomalies. The goals of RLD treatment include correction of radial forearm bowing, radial and volar subluxation of the carpus and limb-length inequality to improve function and cosmesis.
Treatment algorithms for RLD include splinting, centralization, radialization, bilobed flap and vascularized bone transfer. For severe limb-length discrepancy, distraction osteogenesis has been proposed to gradually lengthen the affected limb, rebalance soft tissues and correct the axis of the forearm. This case report describes the surgical technique used to attain such results.
It is important to consider the complete clinical picture. Distraction osteogenesis is arduous and frequently complicated by pin site infection. Reports have noted complications of delayed union or nonunion when lengthening proceeded at too rapid a pace or was extreme. As such, it is important to adjust for appropriate lengthening indices with adequate consolidation times for bony healing. Extensive patient and family preoperative education aids compliance and the success of distraction osteogenesis. Postoperative treatment includes frequent follow-up visits, frame adjustments and pin site care. Given the care of such devices, this technique is reserved for patients who are mature at the time of lengthening and patients with family support to assist with care of the device.
- References:
- Bednar MS, et al. J Hand Surg Am. 2009;doi:10.1016/j.jhsa.2009.09.002.
- Colen DL, et al. J Hand Surg Am. 2017;42(7):546-563. doi:10.1016/j.jhsa.2017.04.012.
- Farr S, et al. J Bone Joint Surg Am. 2016; doi:10.2106/JBJS.16.00007.
- Farr S, et al. J Hand Surg Am. 2012;doi:10.1016/j.jhsa.2012.08.029.
- Matsuno T, et al. J Hand Surg Am. 2006;doi:10.1016/j.jhsa.2006.01.017.
- Oberg KC, et al. J Hand Surg Am. 2009;doi:10.1016/j.jhsa.2009.04.042.
- Peterson et al. J Hand Surg Am. 2007;doi:10.1016/j.jhsa.2007.07.024.
- Vuillermin C, et al. J Hand Surg Am. 2015;doi:10.1016/j.jhsa.2015.01.004.
- For more information:
- Pooja Prabhakar, BA, can be reached at UT Southwestern Medical Center, School of Medicine, 5323 Harry Hines Blvd., Dallas, TX 75390; email: pooja.prabhakar@utsouthwestern.edu.
- S. Robert Rozbruch, MD; Samir K. Trehan, MD; and Lauren E. Wessel, MD, can be reached at Hospital for Special Surgery, 535 E. 70th St., New York, NY 10021; Rozbruch’s email: rozbruchsr@hss.edu. Trehan’s email: trehans@hss.edu. Wessel’s email: wessell@hss.edu.
- Edited by Michael C. Ciccotti, MD, and Michael C. Fu, MD, MHS. Ciccotti is a chief resident in the department of orthopedic surgery at Thomas Jefferson University and Rothman Orthopaedic Institute and will be a sports medicine fellow at the Steadman Phillipon Research Institute in Vail, Colorado following residency. Fu is a chief resident at Hospital for Special Surgery and will be a sports medicine and shoulder surgery fellow at Rush University Medical Center following residency. For information on submitting Orthopedics Today Grand Rounds cases, please email: orthopedics@healio.com.
Disclosures: Rozbruch reports he is a consultant for Ellipse Technologies and Smith & Nephew, and is a consultant for and receives royalties from Stryker. Prabhakar, Trehan and Wessel report no relevant financial disclosures.