Wilcox D T, Quinn F M, Spitz L, Kiely E M, Ransley P G
Great Ormond Street Hospital, London, UK.
Br J Urol. 1998 Jun;81(6):905-10. doi: 10.1046/j.1464-410x.1998.00650.x.
To review the urological abnormalities in conjoined twins, a rare congenital abnormality arising from an incomplete separation of a pair of monozygotic twins, and who can be joined from the head to the pelvis with varying degrees of union.
The records and urological anomalies of conjoined twins presenting from 1985 to 1995 were reviewed retrospectively and the management and surgical reconstruction described.
Seven of 11 sets of conjoined twins underwent surgical exploration. Complex urological problems occurred in all twins with omphalo-ischiopagus. The urological anomalies were: Set 1; both twins had one lateral crossed fused renal ectopia with uncrossed ureters draining into a single bladder with one urethra. They had one set of genitalia. Each twin received one crossed fused ectopic kidney, half the bladder and genitalia, with one retaining the urethra and one a vesicostomy. Set 2; these twins had four kidneys (two lateral and two central that were fused), four ureters (two of which crossed) and these drained into two sagittal bladders that emptied by one urethra associated with a single set of external genitalia containing three corpora cavernosa. At separation, the crossed ureters were re-routed, each received a bladder and the genitalia were split. Set 3; there were four kidneys with two crossed ureters draining into two collateral bladders, there was a partial duplication of the urethra and a single hypospadiac penis. After separation, each had kidneys draining into a bladder, one received the urethra and external genitalia, and one a urostomy.
Complex urological problems occur in conjoined twins, although restricted to those with pelvic fusion (ischiopagus). Assessment and treatment needs to be individualised and overseen by a surgeon with experience in complex urology.
联体双胎是一种罕见的先天性畸形,由单卵双胎不完全分离所致,可从头部至骨盆以不同程度相连。本文旨在回顾联体双胎的泌尿系统异常情况。
回顾性分析1985年至1995年期间联体双胎的病历及泌尿系统异常情况,并描述其治疗及手术重建过程。
11对联体双胎中有7对接受了手术探查。所有胸腹联体双胎均出现复杂的泌尿系统问题。泌尿系统异常情况如下:病例1;两名双胎均有一侧交叉融合肾异位,未交叉的输尿管汇入单一膀胱并经单一尿道排尿。他们有一套生殖器官。每个双胎各获得一个交叉融合的异位肾、半个膀胱和生殖器官,其中一个保留尿道,另一个行膀胱造瘘术。病例2;这对双胎有四个肾脏(两个外侧和两个中央融合的肾脏)、四条输尿管(其中两条交叉),输尿管汇入两个矢状位膀胱,经单一尿道排尿,伴有一套包含三个海绵体的外生殖器。分离时,交叉的输尿管重新改道,每个双胎各获得一个膀胱,生殖器官分开。病例3;有四个肾脏,两条交叉的输尿管汇入两个并列的膀胱,尿道部分重复,阴茎有尿道下裂。分离后,每个双胎的肾脏均汇入一个膀胱排尿,其中一个获得尿道和外生殖器,另一个行尿路造口术。
联体双胎会出现复杂的泌尿系统问题,尽管仅限于骨盆融合(坐骨联体)的双胎。评估和治疗需要个体化,并由有复杂泌尿外科经验的外科医生进行监督。