# Preoperative Considerations for Uterine Fibroid Removal in Patients With Mayer-Rokitansky-Küster-Hauser Syndrome and Klippel-Feil Syndrome: A Case Report

## TL;DR

This case report highlights the unique challenges and preoperative considerations for removing uterine fibroids in patients with Mayer-Rokitansky-Küster-Hauser and Klippel-Feil syndromes.

## Contribution

The paper emphasizes the need for tailored surgical and anesthetic planning in patients with rare congenital syndromes and uterine fibroids.

## Key findings

- Magnetic resonance angiography helped locate the mass and rudimentary uterus in a patient with MRKHS.
- Preoperative screening for concomitant malformations like KFS is essential for safe surgical planning.
- Laparoscopic surgery was successfully performed after careful multidisciplinary planning.

## Abstract

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a congenital disorder characterized by dysplasia of the uterus and upper vagina due to Müllerian duct dysplasia. Fibroids arise from the rudimentary uterus. The implementation of surgical intervention for uterine fibroids in patients with MRKHS presents several challenges and considerations. The rudimentary uterus is tiny, making it difficult to determine the location of the pelvic mass and diagnose uterine fibroids. Preoperative planning is often inadequate, necessitating intraoperative decision-making. In addition, patients with MRKHS may present with Klippel-Feil syndrome (KFS), characterized by cervical spine fusion, further complicating intraoperative management. In contrast to the typical practice, careful surgical planning along with anesthesia administration and orthopedic surgery is required. In this case, magnetic resonance angiography was utilized to determine the location of the mass and the rudimentary uterus. Screening was conducted to detect any concomitant malformation, such as KFS. Subsequently, a safe laparoscopic surgery was carried out. In this case report, we present the importance and unique considerations of preoperative planning for the surgical intervention of uterine fibroids in patients with MRKHS.