Mullerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. Most Mullerian duct-renal-cervicothoracic-upp... patients are diagnosed with primary amenorrhea from late adolescence. However, a few cases with MURCS association are not well diagnosed during childhood, and long-term outcomes are not well reported.
Management of patients with mullerian agenesis includes psychosocial counseling as well as treatment of the anatomic anomalies. Options include vaginal elongation and the surgical creation of a neovagina.
1. Vaginal Elongation: Primary vaginal elongation by dilation is the appropriate first-line approach in most patients because it is safer, patient-controlled, and more cost-effective than surgery. When well-counseled and emotionally prepared, almost all patients (90-96%) will be able to achieve anatomic and functional success by primary vaginal dilation
2. Surgical Creation of a Neovagina: The surgical creation of a vagina requires ongoing postoperative dilation or vaginal intercourse to maintain adequate vaginal length and diameter; therefore, it is not a method to avoid vaginal dilator therapy. Because primary vaginal dilation is successful for more than 90-96% of patients, surgery should be reserved for the rare patient who is unsuccessful with a primary dilator
3. General Gynecologic Care: Health care providers should be aware that some routine gynecology questions, including the date of last menstrual period, are unnecessary and may make patients have less confidence in the health care team. The patient should be asked about any vaginal discharge, bleeding, pelvic pain, or dyspareunia. Pelvic examinations should be performed if there are concerns about complications, vaginal stricture, or stenosis.
Abnormalities in female reproductive organs, neck, mouth, skeletal limbs, and nervous systems
Underdeveloped genetic organs,Defects in uterus, fallopian tubes, cervix,Issues in growth of height, spine, kidneys, and face