Perren F, Frey P
Department of Pediatric Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
J Urol. 1998 May;159(5):1681-3. doi: 10.1097/00005392-199805000-00091.
The exstrophy-epispadias complex in combination with a duplicated lower urinary tract is an extremely rare congenital malformation. We describe 2 cases of bladder exstrophy in association with a duplicated lower urinary tract.
We analyzed the case histories of 2 patients. The first case had anteroposterior duplication of the bladder communicating through an isthmus and a duplicate urethra. The anterior system was exstrophic and epispadiac. The second case had a duplicate bladder without epispadias. There was a communication between the anterior exstrophic and posterior normal bladders. No other malformations were present.
Both patients underwent excision of the duplicate exstrophic bladder in conjunction with primary closure of the abdominal wall. Postoperative continence was normal. Histological examination confirmed the diagnosed bladder duplication.
Although duplication of the lower urinary tract in combination with the exstrophy-epispadias complex is extremely rare, all children presenting with an exstrophic bladder and a normal urethra alone or in association with an epispadiac anterior urethra should be evaluated for a bladder duplication with possible isthmic communication.
膀胱外翻-尿道上裂综合征合并下尿路重复畸形是一种极为罕见的先天性畸形。我们描述了2例膀胱外翻合并下尿路重复畸形的病例。
我们分析了2例患者的病历。第一例患者膀胱呈前后重复，通过峡部相通且有重复尿道。前部系统为外翻性和尿道上裂性。第二例患者有重复膀胱但无尿道上裂。前部外翻膀胱与后部正常膀胱之间存在相通。无其他畸形。
两名患者均接受了重复外翻膀胱切除术并同期缝合腹壁。术后控尿正常。组织学检查证实了诊断的膀胱重复畸形。
尽管下尿路重复畸形合并膀胱外翻-尿道上裂综合征极为罕见，但所有单纯表现为膀胱外翻且尿道正常或合并尿道上裂性前尿道的儿童，均应评估是否存在可能有峡部相通的膀胱重复畸形。
