Azouz E M, Costa T, Fitch N
Department of Radiology, McGill University, Montreal, Quebec, Canada.
Pediatr Radiol. 1987;17(6):481-5. doi: 10.1007/BF02388283.
The radiological findings in two patients with the Proteus syndrome are described. Features in our two cases not previously mentioned or stressed include vertebral dysplasia and enlargement (megaspondylodysplasia), bilateral genu valgum, recurrent after surgery and intraabdominal and mesenteric lipomatosis. Emergency laparotomy was performed on the first patient who had a twisted necrotic portion of mesenteric fat. Macrodactyly, skeletal muscle atrophy and subcutaneous fat accumulation in the abdominal wall were present in both. In addition the second patient was mentally retarded and had frontal bony prominence of skull. Computed tomography was used for the specific diagnosis of the lipomatous tissues in both patients.
描述了两名患有变形综合征患者的放射学检查结果。我们这两个病例中以前未提及或强调的特征包括椎体发育异常和增大（巨脊椎发育不良）、双侧膝外翻、术后复发以及腹腔和肠系膜脂肪过多症。对第一名患有肠系膜脂肪扭转坏死部分的患者进行了急诊剖腹手术。两名患者均存在巨指（趾）症、骨骼肌萎缩和腹壁皮下脂肪堆积。此外，第二名患者智力发育迟缓且颅骨额部骨质突出。计算机断层扫描用于两名患者脂肪组织的特异性诊断。
