A rare case report on complete cervical agenesis with vaginal atresia and suspended didelphys uterus with hematometra and left haematosalpinx

Congenital uterine malformations are deviations from normal anatomy resulting due to defective fusion of Mullerian ducts or the paramesonephric ducts in the developing embryo. These anomalies may be isolated or in combination with urological abnormalities. The mean prevalence of female congenital malformations in general population is up to ⁓ 7%. Patients with these anomalies usually present during pubertal age due to absence of onset of menses, cyclical abdominal pain, or in reproductive age group as infertility or recurrent pregnancy loss depending upon the degree of malformation. Cervical agenesis is a rare Mullerian anomaly with an incidence of 1 in 80,000 females. It represents 3% of all uterine anomalies. It is rarely associated with a functioning uterus (4.8%). Cervical agenesis is often associated with vaginal atresia (less than 50%). It is important to classify these anomalies for easy diagnosis and plan appropriate preoperative treatment.

Cervical and vaginal agenesis report.

Cervical agenesis is a very rare condition often associated with atresia of vagina. Clinical diagnosis is usually difficult before surgery. Transverse vaginal septum or vaginal agenesis is also a rare condition that results from incomplete fusion between vaginal components of the mullerian ducts and urogenital sinus. Here we presented case of 16 years old girl who presented with primary amenorrhea and cyclical lower abdominal pain for 6 months. Abdomino-pelvic scan showed hematometra with bilateral hematosalpinx with mild free fluid in pouch of Douglas with collapsed vagina and cervix. MRI pelvis confirmed USG findings giving differential diagnosis of high vaginal septum or cervical agenesis. She was operated for cervical recanalization and vaginosplasty.

A Rare Case Of Mullerian Anomaly- (HEMATOMETRA With Cervical Agenesis)

Cervical Agenesis is a relatively infrequent mullerian anomaly. Sixteen years old, unmarried girl presented to ELMC&H Lucknow, with complaints of primary amenorrhoea and cyclical abdominal pain for 1 year. On Per-rectal examination, tense cystic mass was felt on right side, approximately 5x5 cm in size, tender and freely mobile. Cervix could not be palpated. Small knob like structure was felt on left side, 1.5x1.5 cm in size, firm in consistency and freely mobile. USG showed uterus of size 5x4x3 cm with collection in endrometrial cavity with? hypoplastic cervix suggestive of- Hematometra with cervical agenesis. On laparotomy, tense unicornuate uterus on right side and solid rudimentary horn on left side was found, which was attached to the uterus by peritoneal fold. Cervix was absent. Both ovaries and tubes were normal. Right cornua of uterus was completely excised leaving both the tubes and ovaries.

Unilateral Functional Uterine Horn with Non Functioning Rudimentary Horn and Cervico-Vaginal Agenesis: Case Report.

Developmental anomalies involving Mullerian ducts are one of the most fascinating disorders in Gynaecology. The incidence rates vary widely and have been described between 0.1-3.5% in the general population. We report a case of a fifteen year old girl who presented with primary amenorrhea and lower abdomen pain, with history of instrumentation about two months back. She was found to have abdominal lump of sixteen weeks size uterus. On examination vagina was found to be represented as a small blind pouch measuring 2-3cms in length. A rectovaginal fistula (2x2 cms) was also observed. Ultrasonography of abdomen revealed bulky uterus (size 11.2x6 cm) with 150 millilitre of collection. Adiagnosis of hematometra with iatrogenic fistula was made. Vaginal drainage of hematometra was done which was followed by laparotomy. Peroperatively she was found to have a left side unicornuate uterus with right side small rudimentary horn. Left fallopian tube and ovary showed dense adhesions and multiple endometriotic implants. Both cervix and vagina were absent. Total abdominal hysterectomy was done and rectovaginal fistula repaired. The present case is reported due to its rarity as it involved both mullerian agenesis with cervical and vaginal agenesis along with disorder of lateral fusion. This is an asymmetric type of mullerian duct development in which arrest has occurred in different stages of development on two sides.