Intestinal obstruction secondary to malformation in a child with Mayer-Rokitansky-Küster-Hauser Syndrome.

Abstract Background : Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is a congenital disorder characterized by underdevelopment of the uterus and vagina. Case : A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Mullerian remnants attached to abnormal fibrous tissue. The fibrous tissue extended from the descending colon to the rectum and connected to a urinary bladder duplication cyst. The fibrous tissue formed into a constricted band around the rectum. The cause of death was Streptococcus pyogenes sepsis with intestinal obstruction secondary to the rectal fibrotic band and urinary bladder duplication cyst. Summary and conclusion : MRKH syndrome is a rare congenital disorder and the malformations rarely cause death. We described the features of abnormal fibrous tissue and urinary bladder duplication cyst in a patient with MRKH syndrome.