THE ASSOCIATION OF LARGE OVARIAN CYSTS WITH CLOACAL EXSTROPHY

Background/Purpose: Cloacal exstrophy can now be managed with excellen t survival rates and reasonable long-term outcomes with many of these patients living into their late teens and early adulthood. In this rep ort, the authors describe for the first time the association of large ovarian cysts with cloacal exstrophy. Methods: From 1974 to 1996, 12 p atients with cloacal exstrophy have been treated at C.S. Mott Children 's Hospital. Massive ovarian cysts developed in four of these. These p atients represent the subjects of this study. Results: All four patien ts have been followed up beyond puberty and massive ovarian cysts have developed, which have caused significant morbidity. Three patients ha ve required surgical intervention. All the patients had reached menarc he before the development of the cysts. In all cases, the presentation was severe pelvic pain. Urinary tract obstruction from the large pelv ic cysts developed in three of the four. The cysts were bilateral in t hree of four patients and measured 8 to 10 cm in diameter on ultrasoun d scan or computed tomography (CT). Cyst aspiration was attempted in t wo cases and was unsuccessful. Three of the four patients have require d bilateral salpingo-oophorectomy. The indications for surgery were un controllable pelvic pain in one and urinary obstruction and uncontroll able pelvic pain in two. Surgical findings demonstrated massive thin-w alled cysts with essentially no normal ovarian tissue in association w ith duplicated mullerian structures. The pathology findings were corpu s luteal cyst in two and mucinous cystadenoma in one. The fourth patie nt with an 8- x 10-cm unilateral cyst is being followed up. Conclusion s: The authors have described, for the first time, the association of massive ovarian cysts with cloacal exstrophy. These cysts can lead to severe pelvic pain and urinary tract obstruction. Bilateral oophorecto my has been required in most of these patients. Copyright (C) 1998 by W.B. Saunders Company.