Author
information
Valvi
Durga*, Parulekar SV**
(*Assistant Professor,
** Professor and Head. Department of Obstetrics
and Gynecology, Seth G.S. Medical College and K.E.M Hospital,
Mumbai, India.)
Abstract
A 36 years unmarried female
was presented with distension and chronic pain in abdomen which was more acute during menses. CT scan showed left sided
broad ligament fibroid with right fused ectopic kidney, absent left kidney and
both ureters on the same side. On exploration there was evidence of bicornuate
uterus with fibroid in the left horn.
Removal of left horn of bicornuate uterus was done.
Introduction
Abnormalities in the formation or fusion of the mullerian
duct results in a variety of anomalies of the uterus and vagina. Mullerian
anomalies are commonly associated with renal, spinal, cloacal anomalies.[1]
There is particular association of
unilateral renal agenesis or ectopia, uterine duplication or unicornis, and
vaginal agenesis.[2] The incidence of mullerian duct anomalies
ranges from 0.5 to 5%. Bicornuate uterus results from incomplete fusion of
uterovaginal horns at the level of the fundus and are thought to represent 25%
( range 10-39% ) of mullerian duct anomalies.[5,6] According to
American Fertility Society, bicornuate uterus is class 4 mullerian anomaly.[2]
The renal anomalies are always ipsilateral to rudimentary horn. Cytogenetic
abnormalities in the form of spontaneous chromosomal rearrangements are known
to occur in uterine leiomyomas. These chromosomal arrangements may be
responsible for the initiation and progressive growth of the leiomyomas.[3]
The possible reason for this uncommon occurrence could be a decreased
concentration or sensitivity of the estrogen receptors or a lesser genetic
predisposition for the clonal chromosomal abnormalities that are observed in
women with normal uterus with leiomyomas.[3] Hysterosalpingography,
hysteroscopy, laparoscopy, pelvic ultrasonography (USG) using the convex
abdominal and transvaginal probes, computerized tomography and magnetic
resonance imaging of the pelvic organs are increasingly being used as
diagnostic modalities. MRI is the standard method for imaging uterine
anomalies. It provides high-resolution images of the uterine body, fundus, and
internal structure. In addition, it can help evaluate the urinary tract for
concomitant anomalies.[4]
Case Report
A 36 years unmarried was
woman presented with distension and pain in abdomen for 7-8 months. It was
continuous, mild, in both lumbar region and iliac fossae, aggravated during
menses. Her menses were regular. She had a history of kyphoscoliosis and right
hip arthropathy. On examination she had a normal female body contour and normal
secondary sexual characteristics. General and systemic examination showed no abnormality.
On abdominal examination there was a nontender, and relatively fixed lump of
the size of approximately 24 weeks of gestation. External genitalia were
normal. Speculum vaginal examination showed no abnormality. Bimanual pelvic
examination showed the cervix to be continuous with the abdominal mass. Her
hemogram, blood sugar levels, renal and liver function tests were normal. CT scan report was suggestive of left sided
broad ligament leiomyoma, right ectopic fused kidney, and both ureter on the right
side. Both ureters were catheterized prior to an exploratory laparotomy, so as
to localize and safeguard them during surgery. The ureters were found to be on
the right side that of the malrotated kidney crossing over the normal one as
shown in figure 1.
Figure 1. Plain
radiograph of the abdomen and pelvis, anteroposterior view. Two ureters are
seen on the right side, the lower ureter belonging to the malrotated left
kidney crossing the right ureter from lateral to medial side.
A bicornuate uterus with
a leiomyoma in the left horn was found during laparotomy (figure 2). The
leiomyoma measured about 18 cm in diameter and was wedged in the pelvis.
However it could be lifted out of the pelvis. Left hemi-hysterectomy was done.
A myomectomy was not performed because the right horn was well developed, and
was more likely to carry a pregnancy to term or near term than a reconstructed
but scarred left horn. The ureteric
catheters were removed after the operation.
She made an uneventful recovery. Histopathological examination showed leiomyoma
with myxoid degeneration.
Figure 2. Leiomyoma in
the left horn of a bicornuate uterus.
Discussion
There are few cases of
leiomyoma in mullerian anomalies reported in the literature. Most of theses
presented with increasing dysmenorrhea, unilateral mass, and pain in abdomen,
as did our patient. Usually the diagnosis is not made clinically, because of
its low incidence. Cases of leiomyoma in bicornuate uterus has been reported
previously.[7,8] Occurrence
of a leiomyoma in mullerian anomalies is rare, the possible reason for this
uncommon occurrence could be a decreased concentration or sensitivity of the
estrogen receptors or a lesser genetic predisposition for the clonal
chromosomal abnormalities that are observed in women with normal uterus with
leiomyomas.[3] If the degree of separation of the two horns is
large, the leiomyoma can be relatively mobile. When mobile, the leiomyoma is
mistaken for a subserous pedunculated leiomyoma, and when fixed, a broad
ligament leiomyoma. If the horns are not widely separated, a leiomyoma in one
of the horns may be mistaken for one in the lateral wall of a normal uterus. If
it is lower in position in such a situation, it may be mistaken for a broad
ligament leiomyoma. If it is in one of two widely separated horns of a
bicornuate uterus, but is very large, it may be impacted in the pelvis and may
be mistaken for a broad ligament leiomyoma. In the case described, the leiomyoma
was of this last type. It may be missed on USG, but is usually diagnosed on CT
or MRI. The diagnosis was missed in our patient on USG, because the endometrial
cavity of the affected horn was not visualized. It was missed even on CT scan.
It is important to make the diagnosis of mullerian anomalies accurately in view
of the high risk of associated anomalies like spinal, renal and cloacal
anomalies.[1] However it is not practical to perform a CT or MRI in
every case of leiomyomas, because that would not be cost effective as a large
number of such scans would have to be performed in order to pick up one case.
Furthermore, a laparotomy would be required irrespective of the diagnosis, and
surgical approach to the removal of the leiomyoma could be decided based on the
findings without any deficiency in the results. Hence we do not recommend CT or
MRI for patients with uterine leiomyomas. Leiomyoma usually undergo hyaline
degeneration but our patient had myxoid degeneration.
References
1.
John A Rock,Howard W Johns,et al. Telinde of
operative gynaecology, Mullerian abnormalities,vol 12, 2010; 540-544.
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Jaya Singhe Y, Rane A, Stalewki H, et al. The Presentation
and Early Diagnosis of the Rudimentary Uterine Horn. Obstet Gynecol June 1,
2005; 105(6) : 1456-1467.
3.
Rein MS, Friedman
AJ, Barbieri RL et al. Cytogenetic abnormalities in uterine leiomyomata. Obstet Gynecol 199; 77:923-6.
4.
Ibrahim Syed, Hero K Hussain, et al. Imaging in
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8. O Ayoola, O Alao,
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And Fibroid Tumors: A Rare Combination Causing Infertility. The Internal
Journal of Radiology. 2006; Vol 6; Number 1.
Citation
Valvi D, Parulekar SV. Leiomyoma in a Bicornuate Uterus. JPGO Volume 1 Issue 3,
March 2014, available at: http://www.jpgo.org/2014/03/leiomyoma-in-bicornuate-uterus.html