Choi M L, Wey P D, Borah G L
Department of Surgery, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903, USA.
Ann Plast Surg. 1998 May;40(5):528-32. doi: 10.1097/00000637-199805000-00016.
Proteus syndrome is a rare congenital disorder comprised of subcutaneous and internal hamartomas, pigmented skin nevi, skull exostoses, hemihypertrophy, and macrodactyly of the hands and feet. A 5-year-old girl diagnosed with Proteus syndrome presented with distal median compression neuropathy with the primary complaint of severe pain involving the left hand. Surgical exploration of the hand revealed a lipofibromatous hamartoma of the median nerve. The transverse carpal ligament was released and epineurectomy of the median nerve was performed. The patient remains symptom free at the 9-month follow-up. This report is the first description of a hamartoma directly involving a peripheral nerve in Proteus syndrome. Decompression of the nerve with the removal of the fibrofatty neural sheath resulted in the resolution of the symptoms in this patient. The surgeon should consider this approach as a potential first line of treatment before a more radical resection of the nerve is contemplated.
变形综合征是一种罕见的先天性疾病，由皮下和内部错构瘤、色素性皮肤痣、颅骨外生骨疣、半身肥大以及手足巨指（趾）症组成。一名被诊断为变形综合征的5岁女孩出现了正中神经远端压迫性神经病变，主要症状是左手剧痛。对手部进行手术探查发现正中神经有一个脂肪纤维瘤性错构瘤。松解了腕横韧带并对正中神经进行了神经外膜切除术。在9个月的随访中，患者症状消失。本报告首次描述了变形综合征中直接累及周围神经的错构瘤。通过切除纤维脂肪性神经鞘对神经进行减压，使该患者的症状得到缓解。在考虑对神经进行更彻底的切除之前，外科医生应将这种方法视为潜在的一线治疗方法。
