Laparoscopic management of constellation of syndromes (Mullerian Aplasia, VACTERL and Klippel Feil) presenting with torsion of Hemorrhagic Ovarian Cyst

Autor: Hanji, Rishikesh Mallikarjun, Paliwal, Ashish, Misra, Richa, Joshi, Vikas, Tanger, Ramesh Chand, Mathur, Praveen
Zdroj: Journal of Pediatric Endoscopic Surgery; 20240101, Issue: Preprints p1-6, 6p
Abstrakt: Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is a rare congenital disorder due to agenesis of the Mullerian duct. The main characteristic of this syndrome is an underdeveloped or aplastic uterus, the absence of the upper part of the vagina with normal functional fallopian tubes and ovaries. Presence of VACTERL association and Klippel Feil syndrome with MRKH syndrome is even rarer. An 11-year-old girl presented to us with acute pain abdomen. Patient had a previous history of undergoing surgeries for anorectal malformation and tracheo-esophageal fistula. Radiological evaluation revealed torsion of hemorrhagic ovarian cyst and vertebral anomalies with Klippel Feil syndrome. Laparoscopic management of torsion of hemorrhagic ovarian cyst in complex syndromes (MRKH, VACTERL anomalies and Klippel Feil) is not reported.
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