Perineal reconstruction of female conjoined twins.

Background/purpose: Conjoined twins are some of the most challenging patients faced by surgeons. Pygopagus and ischiopagus twins present particular gastrointestinal and genitourinary reconstructive challenges. This study reviews the authors' experience with the perineal reconstruction of these types of conjoined twins.

Methods: Retrospective analysis was performed for 3 sets of female conjoined twins undergoing separation between 1999 and 2001. Particular attention was given to the separation and reconstruction of the distal gastrointestinal and urogenital structures.

Results: Three sets of female conjoined twins underwent successful separation 2 pygopagus, one ischiopagus tripus) with 5 surviving infants. The sixth infant died of congenital anomalies incompatible with life. Four of the 5 surviving infants had diverting enterostomies. Two of these enterostomies have been closed. Perineal reconstruction consisted of anoplasty (5 of 5), vaginoplasty (4 of 5), and urethroplasty (4 of 5). Although fecal and urinary continence are not completely measurable at this age (<3 years), all 5 survivors void spontaneously. Three infants with intestinal continuity have apparently normal defecation without the need of a bowel regimen.

Conclusions: With careful preoperative planning and a multidisciplinary team of pediatric surgeons and urologists, satisfactory reconstruction and functional outcome of the female perineum can be achieved in conjoined twins.