Dataset Information

Modified osteotomy for treatment of forearm deformities (Masada IIb) in hereditary multiple osteochondromas: a retrospective review

ABSTRACT:

Purpose

Approximately 30% of patients with hereditary multiple osteochondromas (HMO) have forearm deformity and dysfunction. The aim of this retrospective study was to review our experience with the surgical treatment of children with HMO and Masada IIb forearm deformities.

Methods

Data of eight children treated for HMO Masada IIb forearm deformity at our hospital between 2015 and 2019 were collected from the hospital records and retrospectively reviewed. All patients underwent ulnar lengthening by distraction osteogenesis using either the Orthofix or Ilizarov external fixator. Range of movements at the elbow and wrist joints, and forearm supination/pronation, before and after the operation were recorded. Radiographs were evaluated by the Fogel method, and wrist joint function by the Krimmer method.

Results

Follow-up radiographs showed significant improvement in relative ulnar shortening after treatment (pre-operative 9.23 ± 5.21 mm; post-operative 0.33 ± 4.13 mm). Changes in radial articular angle (pre-operative 33.55° ± 3.88° to 32.78° ± 6.57°) and carpal slip (pre-operative 45.00% ± 19.09%; post-operative 43.13% ± 16.68%) were not significant. Elbow flexion and extension, wrist flexion and extension, ulnar and radial deviation at wrist, and forearm rotation were significantly improved after surgery. Wrist function was graded as excellent in seven patients and as good in one patient. One patient treated with the Ilizarov external fixator had poor radial head reduction.

Conclusion

Ulnar lengthening with distraction osteogenesis is an effective treatment for HMO Masada IIb deformities. The optimum site for ulnar osteotomy appears to be at the proximal one-third to one-fourth of the ulna.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12891-021-04829-x.

SUBMITTER: Yan G

PROVIDER: S-EPMC8582216 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Multiple osteochondromas (MO) is characterised by development of two or more cartilage capped bony outgrowths (osteochondromas) of the long bones. The prevalence is estimated at 1:50,000, and it seems to be higher in males (male-to-female ratio 1.5:1). Osteochondromas develop and increase in size in the first decade of life, ceasing to grow when the growth plates close at puberty. They are pedunculated or sessile (broad base) and can vary widely in size. The number of osteochondromas may vary significantly within and between families, the mean number of locations is 15-18. The majority are asymptomatic and located in bones that develop from cartilage, especially the long bones of the extremities, predominantly around the knee. The facial bones are not affected. Osteochondromas may cause pain, functional problems and deformities, especially of the forearm, that may be reason for surgical removal. The most important complication is malignant transformation of osteochondroma towards secondary peripheral chondrosarcoma, which is estimated to occur in 0.5-5%. MO is an autosomal dominant disorder and is genetically heterogeneous. In almost 90% of MO patients germline mutations in the tumour suppressor genes EXT1 or EXT2 are found. The EXT genes encode glycosyltransferases, catalyzing heparan sulphate polymerization. The diagnosis is based on radiological and clinical documentation, supplemented with, if available, histological evaluation of osteochondromas. If the exact mutation is known antenatal diagnosis is technically possible. MO should be distinguished from metachondromatosis, dysplasia epiphysealis hemimelica and Ollier disease. Osteochondromas are benign lesions and do not affect life expectancy. Management includes removal of osteochondromas when they give complaints. Removed osteochondromas should be examined for malignant transformation towards secondary peripheral chondrosarcoma. Patients should be well instructed and regular follow-up for early detection of malignancy seems justified. For secondary peripheral chondrosarcoma, en-bloc resection of the lesion and its pseudocapsule with tumour-free margins, preferably in a bone tumour referral centre, should be performed.