Laparoscopic management of uterine myomatosis in a patient with Rokitansky syndrome. Case report and review of the literature
DOI:
https://doi.org/10.18597/rcog.94Keywords:
Mayer-Rokitansky-Küster-Hauser, leiomyoma, laparoscopyAbstract
Introduction: The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a spectrum of müllerian duct anomalies characterized by the congenital absence of the uterus and vagina in phenotypically normal women with a 46 XX karyotype and normal endocrine status. Many of these women have uterine remnants. The presence of fibroids in those remnants is a very rare finding, hence this case report.
Objective: To describe the laparoscopic surgical technique and review the clinical and differential diagnoses.
Materials and methods: Female patient with a prior diagnosis of MRKH syndrome who presented to the Gynecological Endoscopy Unit at Clinica del Prado, a private, level 3 institution in Medellin, which is a referral center for the laparoscopic management of patients with chronic pelvic pain in the Department of Antioquia, Colombia. A solid, nine-centimeter pelvic mass was found on ultrasound. During laparoscopy, a leiomyoma was found in one of the uterine remnants. Laparoscopic myomectomy was performed and the two remnants were resected. A systematic review of the literature was conducted through Medline in the Pubmed site using the following terms: “Mayer-Rokitansky-Hauser Syndrome”, “leiomyoma” and laparoscopy.
Results: In total, 25 articles of the past 20 years were found; of those, 17 were related directly to the topic, and 2 of these included laparoscopic management.
Conclusions: The presence of leiomyoma in a uterine remnant in a patient with MRKH syndrome is infrequent. The case presented here is the second reported in Colombia and the first managed laparoscopically. In patients with the Mayer-Rokitansky-Küster-Hauser syndrome and pelvic masses, laparoscopy is a diagnostic and therapeutic option.
Author Biographies
Guillermo Páez-López
José Fernando De los Ríos-Posada
Adriana María Arango-Martínez
Eduardo Serna-Agudelo
Ricardo Vásquez-Ruiz
Luis Alberto Almanza-Payares
Gustavo Adolfo Calle-Gómez
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