Author
Information
Pawde A*, Khadkikar R**, Mayadeo NM***, Chauhan AR****.
(* Senior Registrar, **
Assistant Professor, *** Professor, ****Additional Professor. Department of Obstetrics and
Gynecology, Seth GS Medical college and KEM
Hospital , Mumbai , India
Abstract
Mullerian
anomalies are rare and usually asymptomatic. Patients with unicornuate uterus
commonly present with repeated second trimester spontaneous
abortions, preterm deliveries or malpresentations; rarely as ectopic pregnancy. Though uncommon, they can also
present with gynecological conditions such as endometriosis or
infertility; adenomysosis is extremely rare. We report a case of unicornuate
uterus with endometriosis and adenomyosis in rudimentary non-communicating
horn, who presented with chronic pain in abdomen and secondary infertility.
Introduction
The
overall incidence of mullerian anomalies in general population is about 0.4%.[1]
Mullerian anomalies are developmental anomalies arising due to defects in
fusion or resorption of the paired mullerian ducts, responsible for the
formation of uterus, cervix and upper two-thirds of the vagina. When either of
the mullerian duct is not formed, it results in an isolated unicornuate uterus.
When mullerian duct is formed but either not fused or abnormally fused, it
results in formation of other variants of unicornuate uterus.[2] Unicornuate
uterus constitutes 20% of all anomalies and is usually associated with
ipsilateral renal abnormalities.
Case
Report
A
29 year old para 1, living 0, abortion 2, case of secondary infertility,
presented with a history of pain in lower abdomen since 3 years, aggravated
since last one year. Pain was continuous and occasionally associated with
vomiting. Her menstrual cycles were
regular, but she had severe congestive as well as spasmodic dysmenorrhea. There
were no urinary complaints. She had two spontaneous first trimester abortions
followed by a preterm birth which resulted in neonatal death.
On
examination, her vital parameters were normal. On abdominal examination, a firm
mass corresponding to 16 to 18 weeks’ size uterus was palpable, arising from
pelvis in the midline. It was nontender, with restricted mobility. On speculum
examination, cervix was pulled high up. Findings were confirmed on vaginal
examination; no adnexal mass was palpable. Clinical impression was fibroid uterus
which was consistent with a magnetic resonance imaging (MRI) report done
elsewhere one year ago; this showed a bulky uterus with posterior fundal intramural
8.1 x 7.5 cm fibroid and another fundal fibroid of 4.3 x 3.8 cm, with splaying
of endometrium at fundus, and absence of the right kidney.
Figure
1: MRI suggestive of broad fundus
Ultrasonography
(USG) was suggestive of an enlarged bicornuate or septate uterus with multiple
fibroids, largest being 7.3 x 4.3 cm on posterior wall, with a 6.5 x 3.3 cm
endometriotic cyst in right ovary. Hysterosalpingogram showed a unicornuate
uterus with patent left fallopian tube. Other routine investigations and husband’s
semen analysis were normal.
On
exploratory laparotomy, a large broad uterus of 14 weeks’ size with restricted
mobility was seen. Left fallopian tube was normal; left ovary was polycystic.
Right ovary had a large chocolate cyst (approximately 6 x 5 cm), and was
adherent to both the posterior surface of uterus and right lateral pelvic wall.
Right sided round ligament was not identified due to adhesions. Right tube was
under-developed, distorted and adherent to ovary. Urinary bladder was advanced
and small bowel was adherent to right ovary and posterior surface of uterus.
After adhesiolysis and dissection of the bladder peritoneum, the uterus and
right chocolate cyst were eventrated, at which time the cyst ruptured;
cystectomy was difficult due to adhesions with right pelvic wall, hence cyst
wall was partially removed and the base was fulgurated. Cervix was felt on the
left side at the base of the uterus.
Figure
2: In situ findings.
The
fibroid described on USG was not clearly defined; however on palpation, a
fibroid of size 6 x 4 cm was felt over the fundus on the right side. Primary
anterior vertical incision was taken over this area and deepened but no
identifiable plane within the myometrium was found; rather the tissues were
adenomyotic. A decision for wedge resection was taken: on deepening the
incision, a small non-communicating endometrial cavity was seen, which helped
us to correctly identify the anomaly and define the plane between the right
non-communicating horn and the normally functioning left unicornuate uterus.
This adenomyoma ended abruptly at the level of the isthmus of the left horn.
The adenomyotic horn was approximately one and a half times the size of the
left horn.
Figure
3: Adenomyotic right sided horn being excised.
The
adenomyotic horn was removed completely after dividing the right cornual
structures and ligating the right uterine artery; care was taken to preserve
the left horn. Haemostatic sutures were taken with number 1 polyglactin, dead
space was obliterated, and redundant serosa was excised. Ultimate serosal
suture line was situated along the right lateral margin of the normal left
horn, and was closed with number 2-0 polyglactin.
Post
operatively the patient recovered uneventfully, had one normal cycle and relief
from her dysmenorrhea and chronic pain. She received three doses of depot GnRh
analogue (injection Leuprolide acetate 3.75 mg intramuscularly monthly for 3
doses). Histopathology report confirmed myometrial tissue with features of adenomyosis
and endometrium within.
Discussion
American
Fertility Society has classified unicornuate uterus as Class II, which is
further subdivided into 4 types:
A.
Communicating- where
one duct is well developed, other is partially developed and is in
communication with main duct.
B.
Non-communicating,
where partially developed horn is not in communication with main uterine cavity
(with cavity/without cavity), occurs in about 22% of unicornuate uterus. No
horn (isolated unicornuate uterus) is the commonest type of unicornuate uterus (33%)
C.
No cavity.
D.
No horn.
As per
ESHRE/ ESGE consensus in 2013 unicornuate uterus is categorized as U4 and
subdivided in two types- type a with rudimentary cavity (communication or not
horn) and type b. without rudimentary cavity (horn without cavity or no horn).[3]
Patients with type II B in AFS classification have functional
endometrium, hence are likely to have endometriosis due to retrograde
menstruation. The non-communicating horn may be fused with the main horn with
flimsy adhesions or with thick bands.[4] In our patient it was a
thick band which was further fused with the main horn due to endometriosis,
giving appearance of broad fundus; reported as bicornuate or septate uterus on
USG. However the exact incidence of adenomyosis in this anomaly is difficult to
estimate as most of the literature available is in the form of case reports of
only endometriosis.[5,6] Early diagnosis and excision of the horn
along with ipsilateral salpingectomy is recommended to prevent further
progression of disease as well as serious complications such as ectopic
pregnancy.
Conclusion
Though endometriosis has been reported, adenomyosis in rudimentary
horn is extremely rare. Early diagnosis and treatment with excision of
non-communicating horn with ipsilateral salpingectomy is required.
References
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presentation, imaging findings, and associated complications. J Ultrasound Med
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distal tubal occlusion, secondary to tubal ligation, tubal pregnancies,
mullerian anomalies and intrauterine adhesions. Fertil Steril 1988;49:944-955.
3. Grimbizis GF, Gordts S, Di Spiezio SA, Brucker S, De Angelis C, Gergolet
M, et al. The
ESHRE/ESGE consensus on the classification of female genital tract congenital
anomalies. Hum Reprod 2013;28(8):2032-2044.
4. Falcone T, Gidwani G,
Paraiso M, Beverly C, Goldberg J. Anatomical variation in the rudimentary horns
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5. Borah T, Das A, Panda S,
Singh S. A case of
unilateral dysmenorrhea. J Hum Reprod Sci. 2010;3(3):158-159.
6. Frontino G, Bianchi S,
Ciappina N, Restelli E, Borruto F, Fedele L. The unicornuate uterus with an
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Citation
Pawde A, Khadkikar R,
Mayadeo NM, Chauhan AR. Adenomyosis In Non-Communicating Uterine Horn. JPGO 2014. Vol 1 No. 12. Available from: http://www.jpgo.org/2014/12/adenomyosis-in-non-communicating.html