Rapid Communication| Volume 42, ISSUE 6, e11-e13, June 2007

Progress over 32 years of a survivor of giant exomphalos and outcome of pregnancy


      We report the progress of a female survivor of ruptured giant exomphalos over 32 years. Two-stage repair was performed at birth, resulting in a giant ventral hernia, which was repaired at 31 months. She developed gross benign ascites at 24 years, not previously described in association with congenital abdominal wall defects. This was associated with a misleading radiologic finding of a complex ovarian cyst and led to laparotomy. There were dense adhesions. Benign ascites and loculated pelvic fluid were drained, but the ovaries appeared healthy. A further laparotomy was performed for acute abdominal pain 2 years later. She subsequently had a successful pregnancy complicated by urinary retention. The abdomen expanded without difficulty, unlike the only other such case reported in the literature. A healthy baby girl was delivered by cesarean section at 37 weeks' gestation, which was necessary because of a footling breach presentation.

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