Lorenzoni Paulo José, Lange Marcos Cristiano, Kay Cláudia Sueli Kamoi, Silvado Carlos Eduardo, Scola Rosana Herminia, Werneck Lineu César
Serviço de Doenças Neuromusculares e Neurologia, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brasil.
Arq Neuropsiquiatr. 2005 Sep;63(3B):881-4. doi: 10.1590/s0004-282x2005000500033. Epub 2005 Oct 18.
Fibrolipomatous hamartoma is a rare benign neoplasm that in some cases is associated with macrodactylia. We describe a 31-year-old man who had a tissue enlargement in the wrist, second and third fingers of the left hand since infancy. At 23-years-old he began with continuous, progressive and high intensity pain that occurred more frequently at night, localized in the left hand. It was associated with paraesthesias and hypostesias predominantly at the fingers described above. Investigation with X-ray, ultrasonography, electrodiagnosis, magnetic resonance image of the left wrist and hand showed carpal tunnel syndrome with macrodactylia by fibrolipomatous hamartoma of the median nerve. The patient did not a have good response to clinical therapy, so he was submitted to a surgical decompression of the left carpal tunnel, and after three months of follow up is asymptomatic.
纤维脂肪瘤性错构瘤是一种罕见的良性肿瘤,在某些情况下与巨指症相关。我们描述了一名31岁男性,自婴儿期起左手腕、第二和第三指出现组织增大。23岁时,他开始出现持续、进行性且高强度的疼痛,夜间更为频繁,疼痛部位在左手。疼痛伴有上述手指为主的感觉异常和感觉减退。对左手腕和手部进行的X线、超声、电诊断、磁共振成像检查显示,正中神经纤维脂肪瘤性错构瘤导致腕管综合征伴巨指症。该患者对临床治疗反应不佳,因此接受了左手腕管减压手术,随访三个月后无症状。