Abstract
Ulnar lengthening has gained popularity in treating forearm deformity due to hereditary multiple exostoses (HME). Whether a simultaneous radius angular correction is necessary for bowing deformity remains debatable. We aimed to evaluate effectiveness and safety of ulnar lengthening alone in HME children. HME patients with forearm deformity who underwent ulnar lengthening between 2011 and 2016 were included. Patients were divided into two groups: eight juniors (age ≤ 10 years) and six seniors (>10 years). The mean age of two groups was 8.1 ± 2.5 and 16.7 ± 4.4 years, respectively. The juniors underwent ulnar lengthening alone, and the seniors received an additional radial corrective osteotomy. Pre-operative and post-operative parameters of supination, pronation, ulnar variance (UV), radial articular angles (RAA), and carpal slip (CS) were assessed. The juniors rather the seniors had an improvement in supination (p < 0.05 and p = 0.109, respectively). The juniors and seniors improved in pronation (p < 0.05). UV, RAA, and CS were corrected in the seniors (p < 0.05). In the juniors, parameters improved in UV, RAA, and CS (p < 0.05). For HME children, ulnar lengthening alone can restore radiologic anatomy and functions, providing comparable surgical outcomes in cosmetic results and clinical parameters.
Highlights
Hereditary multiple exostoses (HME), an autosomal dominant disease, is associated with loss-of-function mutations of the EXT1 and EXT2 genes [1]
There is no consensus regarding the surgical modalities nor timing of surgical intervention. In this single institutional study, we reviewed the outcomes of gradual ulnar lengthening for ulnar shortening deformities in HME patients who underwent consistent procedures
Our patients were further divided into two groups: the senior group comprised six patients who underwent a combination of ulnar lengthening and radial corrective osteotomy
Summary
Hereditary multiple exostoses (HME), an autosomal dominant disease, is associated with loss-of-function mutations of the EXT1 and EXT2 genes [1]. It is characterized by the growth of benign, cartilage-capped osseous tumors called osteochondromas (or exostoses) over the long bones and axial skeleton. These osteochondromas, usually forming adjacent to the physis of the bones, can cause growth plate disturbances and growth deformity. About 30–60% of HME patients have forearm deformities [5,6], and relative shortening of the ulna is the most typical deformity. Ulnar shortening is usually accompanied by bowing of the radius to compensate radio-ulnar length discrepancy, resulting in ulnar translocation of the carpus, angular deviation of the distal radial epiphysis and possible radial head dislocation
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