Intestinal Obstruction Secondary to Malformation in a Child with Mayer-Rokitansky-Küster-Hauser Syndrome

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina. A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Müllerian remnants atta...

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Bibliographic Details
Published inJournal of pediatric & adolescent gynecology Vol. 35; no. 2; pp. 192 - 195
Main Authors Tan, Lii Jye, Tan, Geok Chin, Hussin, Huzlinda, Othman, Mohammad Shafie, Lai, Shau-Kong
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.04.2022
Subjects
Intestinal obstruction
Mayer-Rokitansky-Küster-Hauser syndrome
Müllerian aplasia
Urinary bladder duplication
Intestinal obstruction
Mayer-Rokitansky-Küster-Hauser syndrome
Urinary bladder duplication
MRKH
Müllerian aplasia
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Summary:Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina. A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Müllerian 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. MRKH syndrome is a rare congenital disorder, and the malformations rarely cause death. We describe the features of abnormal fibrous tissue and urinary bladder duplication cyst in a patient with MRKH syndrome.
ISSN:1083-3188
1873-4332
DOI:10.1016/j.jpag.2021.10.002