A Case of Mayer-Rokitansky-K üster-Hauser Syndrome Diagnosed in Infancy After Evaluation of Palpable Gonads

Mayer-Rokitansky-K üster-Hauser (MRKH) syndrome is defined as incomplete development of Müllerian structures (uterus, fallopian tubes, proximal vagina) in an otherwise phenotypic female. MRKH syndrome typically presents in adolescence with primary amenorrhea but has been diagnosed in younger patients who present wit h other associated abnormalities, most commonly renal and skeletal.
Source: Journal of Pediatric and Adolescent Gynecology - Category: OBGYN Authors: Source Type: research