A UNIQUE CASE REPORT OF MRKH SYNDROME

Abstract

Mullerian duct malformation occurs in various forms and each anomaly is distinctive. Cervical atresia or agenesis(AFS class 2B) is a rare anomaly and is associated with absence of a portion or whole of vagina. These patients present with primary amenorrhea and well developed secondary sexual characters and cyclical abdominal pain. A comprehensive pre operative evaluation is essential for patients with functioning uterus with congenital absence of cervix and vagina. Hysterectomy is a definitive initial procedure.

A 16 year old girl came to Saims opd on 1/9/2020 with complain of cyclical abdominal pain since 6 months and leakage of urine pervaginal since 2 months. With similar complaints she went to private hospital 4 months back where USG was done suggestive of hematometra of 8X4.7X3.6cm.Vaginal dilatation with drainage of hematometra by abdominovaginal route was attempted but was not successful. Post operatively she had leakage of urine and was referred to higher center.

O/E Patient was stable with well developed secondary sexual characters. P/A 5x8cm. firm mass palpable in right iliac fossa. L/E a blind vaginal pouch of 1x1.5cm was seen with catheter in situ. On cystoscopy large VVF was seen at bladder neck ,2cm.proximal to vaginal orifice and on MRI 14x5.6x5cm distended uterus was seen with atretic cervix and vagina along with absent right kidney and dysplastic wedge shaped vertebra in lumbo sacral area consistent with type 2 MRKH syndrome. Patient taken in OT on 18/9/2020 for hysterectomy and VVF repair .Abdomen was opened 14x5x5.6cm size uterus seen .Hysterectomy done.1.5cm. vaginal length left.  2x1 cm . VVF seen at bladder neck and repaired. Postoperative SPC along with urethral catheter and perivesical drain placed. Patient discharged on day 5 with SPC and urethral catheter in situ.

Cervical agenesis hinders outflow tract for menstruation affects reproduction and represents therapeutic dilemma .Hysterectomy is eventual treatment  which eliminates suffering from cryptomennrohea , sepsis, endometriosis and multiple operations. Recent advances for restoration or reconstruction of cervical canal is yet to be explored.

Keywords: type 2 MRKH syndrome