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.
- Marcal L, Nothaft MA, Coelho F, Volpato R, Iyer R. Mullerian duct anomalies: MR imaging. Abdom Imaging. Dec 2011; 36(6):756-64. .
- Bermejo C, Martínez PT, Cantarero R, Diaz D, Pedregosa JP, Barrón E, et al. Three-dimensional ultrasound in the diagnosis of Müllerian duct anomalies and concordance with magnetic resonance imaging. Ultrasound Obstet Gynecol 2010; 35(5):593-601.
- Steinmetz GP. Formation of artificial vagina. West J Surg 1940; 48:169-3.
- Strassmann EO. Operations for double uterus and endometrial atresia. Clin Obstet Gynecol. 1961; 4:240.
- Rock JA. Surgery for anomalies of the mullerian ducts. In: Tompson JD, Rock JA, eds. TeLind's Operative Gynecology. 9th ed. Philadelphia, Pa: JB Lippincott Williams & Wilkins; 2003:705.
- Petrozza JC, Gray MR, Davis AJ, Reindollar RH. Congenital absence of the uterus and vagina is not commonly transmitted as a dominant genetic trait: outcomes of surrogate pregnancies. Fertil Steril 1997; 67(2):387-9.
- The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine adhesions. Fertil Steril. Jun 1988; 49(6):944-55.
- Marten K, Vosshenrich R, Funke M, Obenauer S, Baum F, Grabbe E: MRI in the evaluation of Mullerian duct anomalies. Clin Imaging 2003, 27:346–350.
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