Multiple exostoses in child - A report of ten cases

被引：0

作者：

Y. Teklali

Z. F. El Alami

T. El Madhi

H. Gourinda

A. Miri

机构：

[1] Pediatric Orthopedics Department, Childrens Hospital, Madinat Al Irfane, 10101 Rabat

来源：

European Journal of Orthopaedic Surgery & Traumatology | 2003年 / 13卷 / 4期

关键词：

Child; Multiple hereditary exostosis;

D O I：

10.1007/s00590-003-0097-y

中图分类号：

学科分类号：

摘要：

Hereditary multiple exostoses, or Bessel-Hagen disease, is an autosomal-dominant disorder causing serious progressive skeletal deformities in children, with no negligible risk of sarcomatous transformation. The authors repot a study of ten cases, managed over 10 years, in the pediatric orthopedics department in Rabat, Morocco. Patients were six boys and four girls aged 3-16 years, presenting with aesthetic concerns and pain at diagnosis; eight had bony distortions. Bone localizations were multiple, evident in several skeletal sites. In two patients, the extent of the disorder was generalized. Surgical treatment was necessary in eight cases (80%); no case of sarcomatous degeneration was noted. The aims of this study were to analyze different clinical, therapeutic, and developmental aspects of this pathologyin which the prognosis is dependent on localization.

引用

页码：245 / 247

页数：2

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