Fetal extraperitoneal rectal perforation: a rare neonatal emergency.

Purpose: Intraperitoneal bowel perforation may occur in utero as a result of a variety of abnormalities and typically results in sterile meconium ascites, pseudocysts, and/or calcification in the fetus. On the other hand, extraperitoneal bowel perforation in intrauterine life is extremely rare. The object of this report is to present our experience of prenatal extraperitoneal rectal perforation, defining the clinical presentation, management, and progress.
Methods and Materials: Nine babies who were identified from 2 centers in the Republic of South Africa with fetal extraperitoneal rectal perforation are presented. The details of these babies were obtained retrospectively from the case notes.
Results: All patients presented at or shortly after birth with air and meconium tracking below the pelvic floor manifesting as either an expanding, meconium-stained aerocele or with perirectal spreading sepsis. Where abdominal signs were present, laparotomy confirmed the extension of the meconium perforation into the peritoneal cavity. Management was by diverting colostomy, drainage of the perineal collection, and supportive therapy. A posterior approach to the rectum and excision of a fibrotic section of the lower rectal wall was performed in one case. One case developed rectal stenosis that was treated by dilatation before colostomy closure. In all the other cases, digital examination performed before colostomy closure ruled out significant narrowing. There was no mortality, and the site of the rectal perforation healed in all cases to leave good anorectal function after treatment.
Conclusions: Fetal extraperitoneal perforation is extremely rare, but the clinical features are easily recognizable, and when appropriate therapy is instituted, the outcome is likely to be good with normal anorectal function to be expected in the long-term. The exact cause of the condition is unknown.