Uterine Hypoplasia in a Woman with Meier–Gorlin Syndrome: A Case Study, Rare Images, and Implications for Patient Care

Abstract

Background: Meier-Gorlin syndrome (MGS) is a rare genetic disorder associated with urogenital abnormalities, including hypoplastic labia and uterine hypoplasia. In the very few cases published in the literature, there have been instances of these reproductive abnormalities affecting some, but not all, women with the disorder. There have been no previously published pelvic laparoscopic images from a woman with MGS. Case: A 32-year-old nulliparous woman with MGS underwent an emergency laparoscopy for a hemoperitoneum caused by a ruptured cyst. Results: Unfortunately, due to undiagnosed uterine hypoplasia, a uterine perforation occurred from the uterine manipulator. Other features of her anatomy included short clubbed fallopian tubes and a bowl-shaped pelvis. Conclusions: The intraoperative images included in this article provide insight into a rare disorder and highlight the importance of considering uterine hypoplasia prior to uterine instrumentation. Laparoscopic or ultrasound guidance should be used for intrauterine procedures for women with unknown or suspected uterine hypoplasia. ( J GYNECOL SURG 36:161