Author
Information
Amin
K*, Khadkikar R**, Saxena N***, Chauhan AR****.
(*Third
Year Resident, ** Assistant Professor, *** Ex-Third Year Resident,
**** Additional Professor. Department of Obstetrics and Gynaecology,
Seth G S Medical College and KEM Hospital. Mumbai, India.)
Introduction
Cervical
agenesis or aplasia is a rare Mullerian anomaly with incidence of 1
in 80,000 to 1,00,000 live births.[1]
It is classified as Class IB Mullerian anomaly under American
Fertility Society Classification.[2]
The cervix is absent, and the lower uterine segment narrows to
terminate in the peritoneal sleeve at a point above the normal
communication with the vaginal apex. It is often associated with
absence of portion or all of the vagina. We report a case of
recurrent hematometra and hematocolpos due to cervical agenesis.
Case
Report
A
19 year old unmarried girl was admitted to our tertiary center two
years ago in view of primary amenorrhea and cyclical abdominal pain.
General examination findings were normal and secondary sexual
characteristics were well developed. On abdominal examination, a
cystic mobile mass of 16-18 weeks' size was palpable. On local
examination, pin point vaginal orifice was noted with no features
suggestive of imperforate hymen. Ultrasound was suggestive of
hematometra with right ovarian chocolate cyst of 6 × 5 cm. Magnetic
Resolution Imaging (MRI) was suggestive of hematometra probably due
to cervical stenosis with 5.2 cm right ovarian chocolate cyst.
Tentative diagnosis was made as transverse vaginal septum. Resection
of transverse vaginal septum through a very narrow introitus, with
drainage of uterine collection (hematometra) was done. Cervix was not
felt on examination. Foley's catheter was inserted into the cavity of
hematometra and was distended to keep the cavity patent. One week
later, examination under anesthesia was done with removal of Foley's
catheter. On per vaginal examination, vaginal opening of one finger
size was felt through vaginal septum. Uterus was felt but cervix was
not palpable. Patient and her parents were counseled about poor
prognosis in view of cervical stenosis and were asked to follow up as
the definitive plan in this case was hysterectomy.
However
patient did not follow up and presented two years later with
progressively increasing dysmenorrhea, and lower abdominal pain in
between the cycles. She gave history of menstrual cycles occurring
every 15 - 20 days, lasting for 5 to 7 days. General examination was
unremarkable. On abdominal examination, mild tenderness was present
but no mass was felt. On per rectal examination, uterus was 6 weeks'
size, anteverted, with restricted mobility. No forniceal mass was
felt. Her baseline routine investigations were normal.
MRI
revealed hematometra with right sided distal hematosalpinx measuring
3.2 cm in diameter. Bilateral kidneys were normal.
Figure
1. MRI showing hematometra and hematosalpinx.
Exploratory
laparotomy with subtotal hysterectomy was done. Intraoperatively,
uterus was 10 weeks' size, bilateral fallopian tubes were normal,
left ovary was normal. A right ovarian endometriotic cyst of 4 × 4
cm was seen with omental adhesions. This was enucleated, fulgurated
and the ovary was reconstructed. Upper vagina was distended with
blood (hematocolpos) ending blindly, indicative of partial vaginal
agenesis. During hysterectomy, after applying clamps at the junction
of cervix and vagina, uterine cavity was opened and 20 ml of
chocolate coloured fluid was drained from uterine cavity and upper
vagina. Cavity was examined. Findings were suggestive of absence of
cervix and presence of blind and ballooned out vaginal pouch.
Figure
2. Surgical findings.
Histopathological
report was suggestive of endometrium in proliferative phase,
unremarkable myometrium with absence of ectocervix and focal thinning
of endometrium at endocervix. Post
operative course was uneventful. GnRH agonist injection Luprolide
acetate 3.75 mg intramuscularly was given at monthly interval for
three months. Patient has been counseled and advised a vaginoplasty
prior to marriage.
Discussion
Cervical
agenesis is
the absence of cervix.
Milder
forms, in which the cervix is deformed and nonfunctional, are known
as cervical
atresia or cervical
dysgenesis.
These anomalies (complete agenesis and dysgenesis) are classified as
type IB in the American Fertility Society Classification of Mullerian
anomalies. According to EAC classification, it is classified as "Cx
no fn, obst". [3]
Cervical
atresia occurs due to defect in the elongation and canalization of
Mullerian duct. Diagnosis
is based on history, clinical examination, ultrasonography; confirmed
by magnetic resonance imaging. Patients affected by this anomaly
have
normal
secondary
sexual development, and
usually
present at the time
of
puberty with
primary amenorrhoea.
As these cases have a functional uterus, they present with cyclical
abdominal or pelvic pain due to hematometra. They may also have
history of recurrent surgeries, as illustrated in this case. Vaginal
atresia and renal tract anomalies such as renal agenesis, ectopic
kidneys and malrotation are commonly associated.[4]
Conservative
management in the form of laparoscopic uterovaginal anastomosis is
considered as the first line of management but it should be done in
highly specialized unit with the help of experts in laparoscopic
surgery and management
of complex Mullerian anomalies.[5]
This
was not possible in our case due to complete cervical agenesis. Also
the procedure to make a channel in the blind vagina was tried
previously in our case, but failed, leading again to hematocolpos,
hematometra and endometriotic cyst in the ovary; hence definitive
management that is hysterectomy was done instead of conservative
management like uterovaginal anastomosis. Preoperative urography
should be done to rule out associated anomalies in the kidney.
Complications
like endometritis, pelvic inflammatory disease,
persistent pelvic pain, bowel and bladder injury, repeated surgeries
and even death have been reported in the literature following
reconstructive surgeries. Creation
of the new vagina and cervix is a complex procedure associated with
high morbidity in the form of sepsis and resultant restenosis due to
fibrosis .
In nearly 50% of cases cervical agenesis is associated with vaginal
atresia and it is difficult to make a satisfactory fistulous tract,
as in our case. Our
patient developed hematometra and hematocolpos secondary to fibrosis
and closure of the original surgical opening created between uterus
and blind vaginal pouch.
Several
factors such as size of the created channel, duration of its
stenting, presence of rudimentary endocervical glands and native
vagina adjacent to the created channel and the number of menses
allowed to flow through the stented channel may influence the
surgical outcome. [6]
To
conclude, though a reconstructive procedure in the form of
cervicovaginal anastamosis is preferable, it can result in repeat
hematometra formation ultimately requiring hysterectomy. Hysterectomy
is warranted in extreme cases like this, as it saves repeated morbid
procedures as well as financial and psychological burden.
References
- Suganuma N, Furuhashi M, Moriwaki T, Tsukahara S, Ando T, Ishihara Y. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril 2002;77:1071–3.
- American Fertility Society (1988) The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion secondary to tubal ligation, tubal pregnancies, Müllerian anomalies and intrauterine adhesions. Fertil Steril 49: 944–955.
- Parulekar SV. Classification Of Congenital Malformations Of The Female Genital Tract. JPGO 2015. Volume 2 No. 4
- Lindenman E, Sherpard MK, Pescovitz OH. Müllerian agenesis, an update. Obstet Gynecol 1997; 90: 307-12.
- Creighton SM, Davies MC, Cutner A. Laparoscopic management of cervical agenesis. Fertil Steril 2006; 85: 1510.e13–5.
- Keepanasseril A, Saha, SC, Bagga, R, et al. Uterovaginal anastomosis for the management of congenital atresia of the uterine cervix. Gynecol Surg 2011; 8:161–164.
Citation
Amin
K, Khadkikar R, Saxena N, Chauhan AR. Recurrent
Hematometra And Hematocolpos Due To Cervical Agenesis. JPGO 2015.
Volume 2 Number 9. Available from: http://www.jpgo.org/2015/09/recurrent-hematometra-and-hematocolpos.html