A 24 Years old unmarried girl was referred to us from Dhaka, Bangladesh as a case of Primary Amenorrhea. Physical examination revealed well developed secondary sexual characteristics including normal breast development, axillary and pubic hair. Serum FSH, LH, Testosterone and DHEAS levels were all suggestive of female reproductive pattern. Ultrasound revealed absent uterus, normal ovaries, normal kidneys. Karyotype revealed normal female pattern.

Diagnostic Laparoscopy revealed:

Colposcopy & Hysteroscopy not done.

Discussion
Congenital anomalies of mullerian system are common defects. Incidence statistics differ from 1:4000 (at birth) to 1:20,000 (at hospital admission). Many are asymptomatic and therefore, unrecognized.
Mayer (1829) described congenital absence of vagina found in many stillbirths with multiple birth defects, Rokitansky (1838) and Kuster (1910) described the same entity with absent vagina, small bipartite uterus, normal ovarian and multi organ anomalies. Hauser emphasized the spectrum of associated anomalies.

Over the years, the disorder has come to be known as MAYER ROKITANSKY KUSTER HAUSER (MRKH) syndrome. It is characterised by congenital absence of vagina, primary amenorrhea, rudimentary cornua uteri, morphologically normal ovaries and fallopian tubes. Such patients have normal ovulation, normal breast development and hair distribution with a 46 XX chromosomal pattern. Polycystic ovaries & gonadal dysgenesis can occur, hence positive need for ovarian biopsy.

MRKH syndrome occurs due to failure of development and fusion of mullerian duct system which occurs around the fifth gestational week. This is possibly due to inappropriate production of mullerian regressive factor (MIF) in female embryonic gonad, regional absence or deficiency of estrogen receptors limited to lower mullerian duct, effect of teratogenic agents or sporadic gene mutation. It represents 15% cases of primary amenorrhea.

MRKH Syndrome is frequently associated with urinary tract (47%), skeletal (12%) & cardiac anomalies and inguinal hernia. Significant urinary anomalies include unilateral renal agenesis, unilateral or bilateral pelvic kidney, hydronephrosis, hydroureter and a variety of patterns of ureteral duplication. An evaluation of the urinary system is hence essential. Skeletal deformities mostly involve the spine, but can involve digits and ribs also.

Treatment
PRE OPERATIVE PREPARATION includes psychological support of the patient to ensure her full co-operation. IVP to assess the urinary system is mandatory. After laparoscopy for confirming the diagnosis and ovarian biopsy, the aim is to prepare a functional vagina. In cases associated with cyclical pain, removal of the partly functional rudimentary horns may be considered for pain relief.

There are several methods of surgical correction including grafting of vaginal canal. Abbe-Wharton-McIndoe operation techniques are the most common which involve split thickness skin grafting. Timing of surgery is when the lady is about to become sexually active. By doing the surgery at an appropriate time, the lady does not use vaginal moulds for very long time & risk of occlusion of neo vagina does not exist.

Laparoscopic creation of Neo vagina is achieved by applying pressure at the hymenal fossa with the help of an olive. This olive is attached on the anterior abdominal wall by traction sutures. So the traction applied from above progressively pulls the olive up, and creates a neo-vagina in the rectovesical space. The dilating olive and traction device are removed after the neo-vagina progresses to 6–7 cm depth. Thereafter, plastic moulds are required for passive dilatation for 2 weeks. Neo-vagina gets covered with stratified squamous epithelium in almost 80% after about 3 months of surgery. Laparoscopic creation

of neo-vagina appears to be a safe, simple and effective method. The simplicity comes from the fact that there is no dissection involved in rectovesical space and secondly there is no need for skin grafting. Efficacy has been proved by thorough follow up which shows that laparoscopic creation of neo vagina gives anatomically and functionally gratifying results. The patient will have a normal sexual functioning after surgical reconstruction, although conception cannot occur without the aid of a surrogate mother.

Bibliography

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