Author
Information
Goel A*,
Walasangikar VB*, Pawde A**, Chauhan AR
(*Second Year Resident, ** Fourth Year Resident,
*** Additional Professor, Department of Obstetrics and
Gynecology, Seth GS Medical College & KEM Hospital ,
Mumbai , India
Abstract
Unicornuate uterus with a
rudimentary horn is the rare congenital anatomic anomaly of the female genital
system (incidence of 1/100,000), causing many obstetric and gynecological
complications.[1] We report a case of huge hematosalpinx secondary to unicornuate
non-communicating horn presenting as lump in abdomen in a teenage girl.
Introduction
The
reported incidence of congenital uterine anomalies is 0.5 – 1%.[2]
Unicornuate uterus is classified as Type II mullerian duct anomaly by the
American Fertility Society.[3] The rudimentary horn is connected to
unicornuate uterus with a fibrous or fibromuscular band in 10-15% of cases.[4]
The diagnosis of this anomaly is usually delayed, as it remains asymptomatic and
initial symptoms are non-specific. It may be diagnosed due to complications
such as hematometra, endometriosis, infertility and ectopic pregnancy.
Case
Report
A 14 year old girl
presented with dysmenorrhea for last 6 months and pain in abdomen for last 3
months, both of which had increased in intensity for last 2 months. The pain
started a day prior to menses, continued during menses and was associated with
vomiting. She also complained of a lump in the lower abdomen for last three
months. She had attained menarche at the age of 11 years and her menstrual
cycles were regular.
On examination, general
and systemic examinations were normal. On abdominal examination a firm,
non-tender, irregular and immobile mass arising from the pelvis in midline was palpable corresponding to 18-20 weeks’ uterine size. Speculum
and vaginal examination was not done as the patient was unmarried and hymen was
intact. The patient was not willing for rectal examination. Our clinical
impression was ovarian cyst or more unlikely, a large endometrioma.
Ultrasound was
suggestive of a uterine horn on the right side in pelvis with normal size,
shape and normal endometrium, but with a displaced axis. Another uterus- like
structure was seen on left side at a higher location suggestive of second horn with
contents within the endometrial cavity with fluid levels and echoes suggestive
of hematometra. This was seen to be draining into a large elongated tubular
structure (20 x 15 x 7 cm) with partial septae with echoes within it, most
likely representing hydro- or hematosalpinx. Bilateral ovaries were normal.
Mild hydronephrosis and hydroureter of left kidney were seen; right kidney was
normal. Her routine hematological and biochemical investigations were within
normal limits. CA 125 level was not elevated indicating non endometriotic/ benign
lesion. Magnetic resonance imaging (MRI) was suggestive of bicornuate uterus
with obstructed left horn not communicating with the cervix resulting in
hematometra and hematosalpinx. Left horn was displaced anterosuperiorly
abutting the anterior abdominal wall in left iliac fossa and normal appearing
right horn along with cervix was seen in situ.
Figure 1. T2 weighted coronal MRI image showing unicornuate uterus in
communication with hematosalpinx.
Figure 2. T1 weighted
axial MRI image showing unicornuate uterus and hematosalpinx,
Figure 3. T2 weighted
coronal MRI image showing unicornuate uterus and hematosalpinx.
On exploratory
laparotomy, a unicornuate uterus was seen in continuation with a normal
functional cervix on the right side. Right fallopian tube and ovary were
normal. Left rudimentary horn was seen connected to the uterus with a
fibromuscular band. Left fallopian tube was retort shaped, distended, tense, 16
x 10 x 8 cm in size, lying anterosuperior to the rudimentary horn with
agglutinated fimbrial end. It was class [FaL 16 cm - UtR 5 cm Fn NoObst - UtL1 3 cm Fn Obst (left hematosalpinx)] by the new EAC classification of congenital malformations of the female genital
Figure.4 Intraoperative
image showing unicornuate uterus, fibromuscular band, rudimentary horn in
communication with left hematosalpinx with agglutinated fimbrial end and left
ovary.
Excision of left
rudimentary non-communicating horn with salpingectomy was done: left round
ligament was clamped, cut and transfixed; fibromuscular band was clamped, cut
and transfixed using polyglactin No.1. A window was created in the broad
ligament and mesosalphinx was dissected by sharp dissection. Left ovary was
separated from the rudimentary horn by sharp dissection and cauterization. Left ovary with its vascular pedicle was conserved.
Successive clamps were applied in the meso-salpinx to remove the left
rudimentary horn with hematosalphinx. Extra-peritonization of broad ligament
stump and left round ligament was done.
Figure 5. intraoperative
image showing unicornuate uterus and conserved left ovary.
Post-operatively the
patient recovered uneventfully and resumed normal cycles and without
dysmenorrhea. Histopathology report confirmed a rudimentary horn showing
pre-pubertal changes with left sided hematosalpinx.
Discussion
The differential diagnosis
of acute abdominal pain in adolescent girls should include mullerian anomalies.
Although the incidence is low, evaluation and treatment should be performed expeditiously
as the conditions can pose risk to life or future fertility. Although hematosalpinx
is not common in teenage years, its prompt treatment is needed.
Physical examination may
not diagnose these anomalies accurately; imaging modalities such as ultrasound, Hysterosalpingography HSG and MRI are important tools for diagnosis. Typically, HSG
films show a deviated banana-shaped cavity with a single fallopian tube, Hematosalpinx is also diagnosed accurately with HSG. Rudimentary uterine
horn in association with a unicornuate uterus is best diagnosed by sonography.[6]
MRI is useful for early diagnosis.
As unicornuate uterus
with functional uterine horn is associated with complications such as
endometriosis, infertility, ectopic pregnancy; excision of a cavitary rudimentary
horn is indicated.[7] Whereas, excision of nonfunctioning horn (solid,
with no functional endometrium) is not routinely recommended, as no adverse
effects are reported. Salpingectomy or salpingo-oophorectomy on the side with
the rudimentary horn, however, has been suggested to prevent ectopic pregnancy
in women with a unicornuate uterus.
Hematosalpinx in
unicornuate uterus secondary to backflow from an obstructed horn leading to
endometriosis as well as adenomyosis is known. However, occurrence of a large hematosalpinx
like in the case presented here is rare due to limited capacity of the
fallopian tube to distend as well as its communication with the peritoneal
cavity. Such huge hematosalpinges tend to undergo torsion, though this was not
seen in our case. Exploratory laparotomy with de-torsion of hematosalpinx may help to conserve the tube. However in our case, large
size as well as agglutinated fimbriae warranted salpingectomy along with
excision of non-communicating rudimentary horn.
References
- Atmaca R, Germen AT, Burak F, Kafkasli A. Acute abdomen in a case with non-communicating rudimentary horn and unicornuate uterus. JSLS 2005; 9(2): 235–237.
- Khanna V, D'Souza J, Tiwari SS, Sharma RK, Shrivastava AK. Unicornuate uterus with an obstructed rudimentary horn: A report of two cases with an imaging perspective Medical Journal Armed Forces India. 2013; 69(1): 78–82.
- The American Fertility Society. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Müllerian anomalies and intrauterine adhesions. Fert Ster. 1988; 49:944–955.
- Goel P, Aggarwal A, Devi K, Takkar N, Saha PK, Huria A, Unicornuate uterus with non-communicating rudimentary horn – different clinical presentations, Journal of Obstetric Gynaecology India 2005;55: 155-158.
- Parulekar SV. Classification Of Congenital Malformations Of The Female Genital Tract. JPGO 2015. Volume 2 No. 4, Available from: http://www.jpgo.org/2015/04/eac-classification-of-congenital.html
- Szkodziak P, Woźniak S, Czuczwar P, Paszkowski T, Milart P, Wozniakowska E, Szlichtyng W. Usefulness of three dimensional transvaginal ultrasonography and hysterosalpingography in diagnosing uterine anomalies. Ginekol Pol. 2014 May; 85(5):354-9.
- Fujimoto VY, Klein NA, Miller PB, Late-onset hematometra and hematosalpinx in a woman with a non-communicating uterine horn A case report. J Reprod Med 1998;43(5):465-7.
Citation
Goel A, Walasangikar VB, Pawde A, Chauhan AR. Large Hematosalpinx Secondary To Unicornuate
Noncommunicating Uterine Horn. JPGO 2015. Volume 2 No. 5. Available from: http://www.jpgo.org/2015/05/large-hematosalpinx-secondary-to.html