Abstract

ObjectivesHereditary multiple exostosis (HME) often involves forearm deformities. The aim of this study was to present the clinical results of 37 children who underwent ulnar lengthening with two different types of unilateral external fixators and to investigate the risk factors of complications.MethodsWe evaluated 37 children with forearm deformities caused by HME treated in our hospital from January 2008 to July 2019. The surgical procedures included resection of exostosis, osteotomy of the ulna, and gradual lengthening of the ulna with a unilateral external fixator. According to the type of fixator they received, the children were divided into two groups: group A received monorail fixators and group B received multi-joint fixators. Radiographic and functional parameters were assessed. Complications were recorded.ResultsAll patients were followed-up for an average of 4.6 years (3.0 to 6.5). In both group A and group B, the ulna shortening (US), radial articular angle (RAA), carpal slip (CS), elbow flexion, forearm pronation, supination, and Mayo Elbow Performance Score (MEPS) values improved significantly from preoperatively to postoperatively (p < 0.05). However, the ulnar deviation was observed in 4 cases in group B and no cases in group A. According to logistic regression, the difference was only related to age (p < 0.05) and the type of external fixator (p < 0.05).ConclusionsUlnar lengthening with unilateral external fixation is a safe and effective procedure for the treatment of HME. Regarding complications, deviation of the ulna axis was more likely to occur in older children with multi-joint external fixators.

Highlights

  • Hereditary multiple exostosis (HME) is a type of autosomal dominant inheritance-induced skeletal dysplasia, with an incidence of approximately 1/50,000 [1]

  • The distal ulna is affected by exostoses, which lead to dysplasia and shortening

  • According to the type of single-arm external fixators applied in surgery, the 37 patients were divided into two groups: group A received monorail fixators and group B received multi-joint fixators

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Summary

Introduction

Hereditary multiple exostosis (HME) is a type of autosomal dominant inheritance-induced skeletal dysplasia, with an incidence of approximately 1/50,000 [1]. 30–60% of cases involve the forearm, affecting the longitudinal growth of the metaphysis and resulting in slow or stagnant growth of the ulna or radius. Deformities such as ulnar shortening, radius curvature, wrist ulnar deviation, and radial head dislocation gradually appear with age [2]. The distal ulna is affected by exostoses, which lead to dysplasia and shortening It tethers the ulnar side of the distal radius, hinders the growth of the distal radius, increases the RAA and CS, and weakens the support provided by the ulna to the wrist joint. Treatments of forearm deformity caused by HME should primarily involve the early correction of ulnar shortening

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