Our Summary

This research paper is about a rare birth disorder known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), in which a female is born without a uterus and the upper part of the vagina. There are two types of this disorder, one that only involves the missing uterus and vagina, and the other that includes additional problems with the kidneys, heart, and other organs. Even though these women lack certain reproductive organs, they still develop normal physical traits associated with puberty, like breasts and pubic hair, and their ovaries function normally.

The paper describes a 26-year-old woman who came to the doctor because she had never had a period. Despite this, she had developed normally during puberty. A detailed examination, including an MRI scan, showed that she had MRKHS type II, meaning she had no uterus or upper vagina, and she was also missing a kidney. But her ovaries were normal.

A team of doctors performed a special kind of surgery to create a new vagina for her. This surgery was successful, and the newly created vagina was 7 cm long.

The paper points out that MRKHS is a difficult disorder to diagnose and treat because it’s rare and not fully understood. They believe it’s caused by a problem during the development of the fetus. It’s also important to distinguish it from other similar conditions. MRI scans are useful for diagnosis and for identifying any additional health problems. The goal of treatment is to enable the woman to have sexual intercourse. This case shows that it’s possible for a woman with MRKHS to have normal hormone levels and puberty development, and that surgery to create a new vagina can be an effective treatment.

FAQs

  1. What is Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS)?
  2. How is Mayer-Rokitansky-Küster-Hauser syndrome diagnosed?
  3. What is the purpose and effectiveness of vaginoplasty in the treatment of MRKHS?

Doctor’s Tip

A doctor might advise a patient undergoing vaginoplasty to follow post-operative care instructions carefully, including keeping the surgical site clean and dry, avoiding strenuous activities, and attending follow-up appointments to monitor healing and address any concerns. They may also recommend pelvic floor exercises to help with recovery and improve sexual function.

Suitable For

Patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) are typically recommended for vaginoplasty. MRKHS is a rare congenital disorder characterized by agenesis of the uterus and upper two-thirds of the vagina. Vaginoplasty is a surgical procedure that creates a neovagina, allowing individuals with MRKHS to restore sexual function. Other patients who may be recommended for vaginoplasty include those with vaginal agenesis or other conditions that result in the absence or underdevelopment of the vagina.

Timeline

  • Before vaginoplasty:
  1. Patient presents with primary amenorrhea.
  2. Physical examination shows normal secondary sexual characteristics.
  3. MRI confirms diagnosis of MRKHS type II, with absence of uterus and upper two-thirds of the vagina.
  4. Patient may undergo evaluation by a multidisciplinary team.
  • After vaginoplasty:
  1. McIndoe vaginoplasty is performed to create a neovagina.
  2. Neovagina measures 7 cm in length.
  3. Surgical intervention restores sexual function.
  4. Patient can experience improved quality of life and sexual satisfaction.

What to Ask Your Doctor

  1. What is vaginoplasty and how does it work?
  2. What are the risks and potential complications of vaginoplasty?
  3. How long is the recovery period after vaginoplasty?
  4. Will I need to follow any specific post-operative care instructions?
  5. How will vaginoplasty affect my sexual function and sensation?
  6. Will I need to undergo additional procedures in the future?
  7. What are the success rates of vaginoplasty in patients with MRKHS?
  8. Are there any alternative treatments or therapies available for MRKHS?
  9. What are the long-term outcomes and potential benefits of vaginoplasty for patients with MRKHS?
  10. How experienced are you in performing vaginoplasty procedures for patients with MRKHS?

Reference

Authors: Shah NA, Yadav M, Verma S, Yadav D, Yadav AK, Ghimire A, Rijal H, Koirala P. Journal: Clin Case Rep. 2024 Dec 15;12(12):e70009. doi: 10.1002/ccr3.70009. eCollection 2024 Dec. PMID: 39687662