Objective:

 

To report a spontaneous pregnancy after surgical management of Mayer Rokitansky Kauser Hauser syndrome.

 

Introduction:

 

Vaginal agenesis is a rare congenital anomaly associated with primary amenorrhoea and cyclical pelvic pain. The incidence is 1 in 4500 female births. The most common cause leading to this condition is Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome.It is a disorder that affects the female reproductive system.

In this condition the external genitalia development is normal and often the uterus is absent. In 9% of the cases, vaginal agenesis can occur in the presence of the uterus (3)

The failure in the development of the terminal portion of the paramesonephric ducts causes vaginal agenesis (4)

 

Case report:

 

A 28 year old female married for 7 months  presented in the outpatient clinic with history of primary amenorrhoea and severe cyclical dysmenorrhoea.

On examination , she had well developed secondary sexual characteristics but  there was blind ending vagina.

Ultrasound scan and MRI showed cervical atresia and non- developed upper 1/3rd of the vagina. Uterus and ovaries appeared normal.

 

A vaginocervicoplasty was performed.

Intraoperatively on vaginal examination a blind ended vagina and a small fibrotic cervical nodule was felt.

 

Pfennanstein incision  was given and Uterus mobilised.Bilateral ovaries appeared normal.

A passage was created from the cervix to the vagina with the help of a probe. Uterus was pushed down and cervix stitched with the vaginal wall by interrupted stitches to ensure continuity of the tract.

A nelton tube was passed from the cervix to vagina and secured in the vagina by means of a stitch. The tube was to be retained for 4-6weeks.

Pelvic cavity was washed thoroughly and routine closure performed.

Patient  was  commenced on Norethisterone tablets for 4 weeks.

At 6 weeks follow up appointment the  Nelton tube was removed in theatre.

The cervix was open and a good vaginal length was felt on examination.

 

Patient presented with spontaneous conception at 20 months post surgery.

 

Conclusion:

 

In this particular case timely surgical intervention lead to a favourable outcome.