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A Case Report of Type-II Mayer-Rokitansky-Küster-Hauser Syndrome with Ovarian Inguinal Hernia Presenting with an Adnexal Mass
Received: 26 May 2022 | Received in revised form: 01 Sep 2022
Accepted: 09 Sep 2022 | Available online: 14 Sep 2022
JCOG. 2023;33(1):46-9
DOI: 10.5336/jcog.2022-91522
Article Language: EN
Article Language: EN
Copyright Ⓒ 2024 by Türkiye Klinikleri. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)
ABSTRACT
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder of the genitourinary tract seen in pediatric females. It is of 2 types. Type I has mullerian anomalies while Type II has associated anomalies. It may be associated with an inguinal hernia. The contents of the hernial sac are mostly the omentum or small intestine. Rarely, unusual contents like ovary and fallopian tubes may also be found in the hernia. The association of MRKH syndrome with ovarian hernia has been reported in a few cases in the literature. Our case is a similar rare case of a pediatric female of MRKH syndrome Type II with an inguinal hernia having an ovary as its content who presented with an adnexal mass.
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder of the genitourinary tract seen in pediatric females. It is of 2 types. Type I has mullerian anomalies while Type II has associated anomalies. It may be associated with an inguinal hernia. The contents of the hernial sac are mostly the omentum or small intestine. Rarely, unusual contents like ovary and fallopian tubes may also be found in the hernia. The association of MRKH syndrome with ovarian hernia has been reported in a few cases in the literature. Our case is a similar rare case of a pediatric female of MRKH syndrome Type II with an inguinal hernia having an ovary as its content who presented with an adnexal mass.
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