盆腔脂肪增多症两例兄弟患者报道及文献分析

两例盆腔脂肪增多症兄弟患者,均因肾功能不全就诊.检查均发现双侧肾盂重度积水,肾皮质变薄,双侧输尿管全程扩张.输尿管上段扩张,下段狭窄;直肠腔受压.膀胱与直肠周围被大量脂肪组织包绕,膀胱呈梨形,位置抬高前移,均行"回肠膀胱术".结合文献复习讨论盆腔脂肪增多症诊治特点.通过Medline/Pubmed、中国生物医学文献数据库(CBMdisc)与清华同方医学期刊数据库文献检索系统,以"Pelvic lipomatosis"、"盆腔脂肪增多"或"骨盆脂肪增多"为主题词进行检索, 在1959年1月至2006年12月间共检索出96篇文献,198例病例报告,进行综合分析.198例中男∶女之比为27∶ 1.盆腔脂肪增多症是一种不明病因的良性疾病,表现为盆腔内膀胱和直肠周围间隙脂肪过度生长,常以泌尿系统或消化系统症状为主要表现,常与高血压、增生性膀胱炎及上尿路梗阻等合并存在,并存在发生血栓性疾病、尿路结石和膀胱腺癌的风险.最重要的鉴别疾病是脂肪肉瘤.盆腔脂肪增多症的治疗多以解除尿路梗阻的尿流改道手术为主.

Analysis of pelvic lipomatosis and a case report of two brothers

Two brothers presented with renal failure. CT and MRL showed bilateral hydronephrosis and hydroureters. A cystogram was performed and showed an elevated pear-shaped bladder secondary to thickening of the bladder wall. Bladder and sigmoid were surrounded by homogeneous fat tissue. The colon was symmetrically fusiform narrowed. Ileal bladder surgery was conducted. We reviewed a total of 96 studies reported between 1959 and 2005,a including 198 patients with pelvic lipomatosis. The ratio of males to females was 27:1. Pelvic lipomatosis was a rare disorder of benign mature adipose tissue proliferation around the bladder and rectum, with urological or digestive system symptoms as the chief presentation. Most cases were associated with hypertension, proliferative cystitis and upper tract obstruction. Also there were risks of thrombosis, calculi and bladder adenocarcinoma. The chief differential diagnosis was liposarcoma. Currently, urinary diversion is the main treatment to relieve urinary obstruction.