3例结节性硬化症合并肾血管平滑肌脂肪瘤的诊断与治疗

目的：探讨结节性硬化症伴巨大肾血管平滑肌瘤（AML）的临床诊治特点和自发性肾破裂出血的治疗措施。方法：报告3例结节性硬化症伴多发性双肾AML的临床诊治资料。3例均为女性,年龄16～26岁,均有典型的智力低下、面部皮脂腺瘤体症,2例因腰部剧烈胀痛伴休克入院,1例因腹胀并癫痫发作入院。3例均经CT检查证实大脑钙化结节灶。结果：1例行DSA肾肿瘤栓塞治疗,1例行肾切除术治疗,1例经抗癫痫药物治疗稳定后行肾切除治疗。3例均成功治愈出院。结论：结节性硬化症合并肾血管平滑肌脂肪瘤肿瘤巨大、症状严重、存在潜在破裂出血及可疑癌变者,可行根治切除或部分切除,破裂出血者宜首选选择性肾动脉栓塞术。

Diagnosis and treatment of tuberous sclerosis accompanying renal angiomyolipoma:report of 3 cases

Objective:To study the clinical features of tuberous sclerosis accompanying large renal angiomyolipoma and treatment of hemorrhage due to spontaneous renal rupture.Methods:The clinical data about 3 female cases of tuberous sclerosis accompanying bilateral and multiple renal angiomyolipomas were reported.Their age ranged 16-26 years.All the cases had typical facial angiofibroma and mental retardation.Of them,2 were admitted to hospital for severe abdominal distending pain and shock,1 was admitted to hospital...