04/06/2026
Today, I had the opportunity to be part of the management of a rare congenital condition — Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome with McINDOE VAGINOPLASTY.
🔹 Young Patient: Married for 6 months, presented with primary amenorrhea (never attained menstruation).
Clinical Findings:
• Normal breast development and secondary sexual characteristics
• Blind va**nal pouch on examination
• No history of menstruation
Investigations:
• MRI revealed absent uterus with features suggestive of MRKH Syndrome (Mayer-Rokitansky-Küster-Hauser Syndrome)
🔹 MRKH Syndrome (Mayer-Rokitansky-Küster-Hauser Syndrome)
What is MRKH?
• A rare congenital condition affecting approximately 1 in 4,500–5,000 female births.
• Females have normal XX chromosomes.
• Ovaries are normal and functional.
• Breast development and other female characteristics are normal.
• Uterus and upper va**na are absent or underdeveloped.
Common Signs:
• No menstruation despite normal puberty.
• Difficulty with sexual in*******se due to a short or absent va**na.
• Often diagnosed during adolescence or after marriage.
Management:
• Counseling and psychological support
• Creation of a neova**na (McIndoe vaginoplasty with amniotic membrane graft) to improve quality of life and sexual function
✨ A reminder that primary amenorrhea with normal secondary sexual characteristics should always be thoroughly evaluated to identify underlying congenital anomalies.
🙏 Deeply grateful to my mentor Gourisankar Kamilya sir for his guidance, support, and valuable teachings throughout this rare and educational case.
🙏 Special thanks to my HOD sirSurendra ChaudharyEntire OT team@Bishwanath Chaudhary anesthesia teamAditi Singhnursing staffTabassum Begam and all healthcare professionals involved in the successful management of this patient.
Every rare case is a lesson in medicine, teamwork, and compassionate care.
Every rare case reminds us that behind every diagnosis is a patient, a story, and an opportunity to make a difference.
Dr. Sweta Gupta
MBBS, MS (Obstetrics & Gynecology)
FMAS, DMAS, Fellowship in ART