Ectopic Kidney as a Clinical Manifestation of Type II MRKH Syndrome Patient: A Case Report

Introduction: Mayer-Rokitansky-Kuster-Hauser Syndrome is a reproductive system disorder that occurs due to failure in the development of the Müllerian ducts. This disorder is characterized by the absence of the uterus and upper part of the vagina. MRKH syndrome is divided into type I and type II where type II is accompanied by non-gynecological disorders. The most common non-gynecological disorder is renal abnormality. Objectives: This study aims to describe one of the rare clinical manifestations of type II MRKH syndrome. Case Presentation: We reported a case of a sexually inactive woman in her early 20s presenting with primary amenorrhea. The patient had normal secondary female sexual characteristics. In the examination, we found the patient had vaginal agenesis, uterine dysgenesis, normal ovaries, normal right kidney, and left ectopic kidney located in the lower abdomen. Laparoscopic Davydov procedure was done to create a neovagina. Discussion: In our case, the patient was diagnosed with MRKH Syndrome, a syndrome of abnormal uterine and vaginal formation with normal ovarian function. The presence of ectopic kidney in the patient showed the MRKH Syndrome type II, which required comprehensive management. The patient was treated with Davydov procedure, which is a simple, fast and safest surgical procedure in managing MRKH syndrome. Conclusion: A thorough examination is needed in MRKH patients to find other possible non- gynecological disorders, such as renal, vertebral, and hearing disorders, in order to carry out comprehensive management.