Partial Mullerian Agenesis Presenting as Hematometra and Hematosalpinx

Author Information

Hemraj R Narkhede*, Amol Pawar**, Jyoti P Mhatre***, Pravin N Mhatre****.

(* Assistant professor, ** Associate Professor, **** Professor. Department of Obstetrics and Gynecology, Seth G S Medical College & Nowrosjee Wadia Maternity Hospital, Parel, Mumbai, India. *** Senior Consultant, Kedar Hospital, Mazgaon, Mumbai, India.)

Abstract

A rare case of Mullerian agenesis presented with acute abdomen. hematometra, hematosalpix and bowel obstruction. We performed extirpative operation to save the life of patient. Vaginoplasty was performed in the same sitting to drain peritoneal cavity and for coital function. Type and time of presentation of Mullerian agenesis make these cases challenging and individualization of management on case to case basis is needed.

Introduction

Absent vagina is a rare Mullerian anomaly. The incidence of Mullerian anomaly reported is between 0.1-3.5%.^[1,,2,3,4] Vaginal agenesis has a reported incidence of 1 in 5000 women.^[5] The clinical presentation varies in each case depending on the type of anomaly, age at presentation and in relation to menarche. The surgical management of each case is challenging and should be individualised on case to case basis. Common presentation of vaginal agenesis is primary amenorrhea. Mullerian agenesis is second most common cause of primary amenorrhea in adolesence.^[6] Here we report a case of partial Mullerian agenesis involving absence of vagina and cervix, due to its rare occurrence and challenging surgical management.

Case report

A 17 year unmarried girl presented with acute abdomen, pelvic mass, fever with rapid thready pulse and mild hypotension The pain in abdomen which was initially cyclical had turned into continuous pain since one year. The patient was having vomiting and colicky abdominal pain with gradual distension of lower abdomen. She had a tense, tender mass corresponding to 30 weeks of pregnacy. Bowel sounds were absent. The secondary sexual characters were well developed with absence of menarche. On investigation she was found to be anemic with raised counts. The patient was kept nil by mouth with Ryle’s tube aspiration and started with injectable antibiotic. Ultrasonography showed a large mass measuring 14×13×12 cm with multiple septa and fluid and dilated bowels. Magnetic resonance imaging (MRI) was done for detailed anatomic evaluation (figures 1, 2, 3). It showed septate uterus with two complete separate uterine cavities, larger on right side with hematometra. Both fallopian tube were thickened and diffusedly dilated from cornu till fimbrial end with large hematosalpinx. Vagina was not seen. The diagnosis of hematometra, hematosalpinx with subacute intestinal obstruction was made.

Figure 1. MRI of abdomen and pelvis, longitudinal section showing extent of hematometra and right fallopian tube.

Figure 2. MRI of abdomen and pelvis, transverse section showing hematometra.

Figure 3. MRI of abdomen and pelvis, transverse sections showing showing both ovaries (arrows).

Exploratory laparotomy was done through Pfannenstiel incision. Hemoperitoneum along with foul smelling pus was drained. (figure 4). There was large hematometra with hematosalpinx, bilateral chocolate cysts and multiple dense adhesions with bowels in pouch of Douglas. The uterus was bicornuate with right horn enlarged as compared to the left horn. Uterus and both fallopian tubes were gangrenous in appearance. The bowel adhesions were separated with utmost care. A decision was taken to perform an extirpative surgery, to remove both the horns of the uterus along with the tubes and right ovary. Left ovary was preserved. Vaginoplasty was done in same sitting with a combined procedure of anterior peritoneum pull through and posteriorly implanting of freeze dried amnion graft.

Figure 4. Intraoperative findings and post-operative specimen.

Prophylactic nasogastric drainage was done. Postoperative period was uneventful with the patient being discharged on day 5 of surgery. The patient came for regular follow-up during which period she was trained for regular self vaginal dilatation using a glass dilator. She married post one year of surgery leading a satisfactory conjugal relationship.

Discussion

A vaginal atresia results when caudal portion mesonephric duct fails to canalise and fuse with urogenital sinus. This caudal portion of vagina is replaced by fibrous tissue. In 1998 American society for reproductive medicine classified the Mullerian anomalies.^[7] Vaginal atresia is type 1 anomaly. In considering management of Mullerian agenesis, the age of patient and psychological implication for the patients’s condition are of paramount importance. Accurate delineation of anatomy is a must before proceeding for surgical management. In this case presence of hematometra, hematosalpinx with acute abdomen and sub-acute intestinal obstruction presented with challenge for diagnosis and management of Mullerian agenesis. The reason for delayed presentation may be due to neglecting of symptoms of persistent pelvic pain. The absence of menstrual cycle by 17 years of age should have encouraged the patient to seek a medical opinion. Pelvic MRI is the preferred modality when there is a suspicion of any Mullerian anomaly.^[8] Secondary bacterial infection leads to the development of pyosalpinx and pyometra. Chocolate cyst and pelvic adhesion may be due implantation endometriosis leading to intestinal obstruction. We thought of uterine conservative procedure before start of surgery. However intraoperative gangrenous appearance of hemtometra and hematosalpinx prevented any such approach. Hence we peformed hysterectomy and bilateral salpingectomy and right oophorectomy. Left ovary was preserved. An early diagnosis and management of obstructive anomalies could have prevented such radical surgery. Vaginoplasty was performed in the same sitting so as to provide drainage and to give her coital function. We dissected the vaginal space from below and mobilized peritoneal flap from above. Due to adhesions in pouch of Douglas, partial mobilization of the peritoneum was possible anteriorly. Posteriorly freeze dried amnion graft was placed. In follow-ups we encouraged for vaginal dilatation. She is married and has normal sexual life after an year of surgery.

Conclusion

The vagaries in Mullerian agenesis, both in type and time of presentation make these cases challenging. The treatment protocool cannot be generalised but is based on individual clinical scenario. This case report highlights and confirms the same.

References

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Citation

Narkhede HR, Pawar A, Mhatre JP, Mhatre PN. Partial Mullerian agenesis presenting as Hematometra and Hematosalpinx. JPGO 2014 Volume 1 Number 9. Available from: http://www.jpgo.org/2014/09/partial-mullerian-agenesis-presenting.html