Author Information
Prakash S*, Parulekar SV**, Hira Priya***
(* Third Year Resident, **Professor and Head of the
department, Department of Obstetrics and Gynecology; *** Associate Professor, Department
of radiology, Seth GS Medical College & KEM Hospital, Mumbai , India
Abstract
Mullerian anomalies constitute an important
aspect of various conditions that affect a woman. A large number of syndromes
are associated with these anomalies. It is important to know about them because
that would permit one to look for other associated anomalies whose management
could be critical to the patient’s survival. We present a case with unicornuate uterus, hemivagina, imperforate hymen and
hematocolpos
This is the first case of this
occurrence in the world literature.
Introduction
Mullerian anomalies are
of different types, such as mullerian agenesis, mullerian hypoplasia, vertical
fusion defects, lateral fusion defects, and unusual configurations of
vertical-lateral fusion defects.[1] An obstruction to the outflow
tract produces conditions like hematocolpos, hematometra, and hematosalpinx. We present a case which may be a new syndrome characterized by
unicornuate uterus, hemivagina, imperforate hymen and hematocolpos This is the first case of this occurrence in
the world literature.
Case
report
A 16 year old single
girl was referred to us with primary amenorrhea, cyclical abdominal pain, and
an ovarian cyst on ultrasonography (USG). There was no contributory medical,
surgical and family history. She was averagely built and nourished. Her general
condition was fair, vital parameters were normal, and systemic examination
revealed no abnormality. There was a mildly tender, cystic lump arising from
the pelvis, extending to 2 cm above the pubis. The hymen was imperforate. It
was bluish in color and bulging mildly. A rectal examination showed a 12 cm
diameter cystic, mildly tender mass in the pelvis. A diagnosis of hematocolpos
secondary to an imperforate hymen was made. However in view of an USG report
showing an ovarian cyst, the test was repeated at our center. The report was a
left ovarian cyst measuring 10 cm in diameter, and a uterus with a small
hematometra. In view of two different sonologists reporting the presence of an
ovarian cyst, we obtained a computerized tomography (CT) scan (figures 1 to 5).
It showed an imperforate hymen, hematocolpos in the midline in the lower
one-third, on the left side of the midline in the upper two-thirds, and left unicornuate
uterus. The left ovary was close to the lateral end of the left horn. The right
ovary was lower down on the right side of the hematocolpos. There was no
uterine horn, even rudimentary one, on the right side. Both kidneys were normal
in size, shape and location.
Figure 1. CT scan of the
abdomen and pelvis. Hematocolpos in midline (arrow).
Figure 2. CT scan of the
abdomen and pelvis. Hematocolpos (yellow arrow) is seen deviated to the left
side. The left unicornuate uterus (blue arrow) is seen attached to the
hematocolpos.
Figure 3. CT scan of the
abdomen and pelvis. Hematocolpos (yellow arrow) deviated to the left side, the
left unicornuate uterus (blue arrow), and the left ovary (pink arrow) are seen.
Figure 4. CT scan of the
abdomen and pelvis. At a higher level than in figure 3, hematocolpos (yellow
arrow) deviated to the left side, the left unicornuate uterus (blue arrow), and
the left ovary (pink arrow) are seen.
Figure 5. CT
scan of the abdomen and pelvis. At a lower level than in figure 3, hematocolpos
(yellow arrow) deviated to the left side and the right ovary (pink arrow) are
seen.
The hymen was excised
under general anesthesia and the hematocolpos was drained. Examination after
the drainage confirmed the diagnosis of left unicornuate uterus. The lower
one-third of the vagina was symmetrical to the midline, while the upper
two-third of the vagina was deviated to the left. The patient made an
uneventful recovery. She was well at the first follow up examination after 15
days, and had normal menstruation after a month.
Discussion
The paramesonephric
(Müllerian) ducts arise from the mesoderm lateral to the mesonephric ducts in the seventh week of fetal development.[2]
They grow caudally passing lateral to the urogenital ridges, lying medial to
the mesonephric ducts in the eighth week. The upper two thirds develop into the
fallopian tubes, while the lower one thirds fuse with each other between the
seventh and ninth weeks to form the uterus, cervix and upper two thirds of the
vagina. The lowest part of the fused paramesonephric ducts fuses with the
ascending endoderm of the sinovaginal node in the eighth week. Canalization of
the sinovaginal node results in formation of the lower third of the vagina. The
sinovaginal node gets inserted into the urogenital sinus at Müller’s tubercle.
The membrane separating the vagina from the urogenital sinus (hymen) gets
perforated before birth. The cloacal membrane, formed in the third fetal week,
gets delimited laterally by the cloacal
folds by the fifth week. The cloacal folds fuse anteriorly to give rise to the
genital tubercle. The urorectal septum
fuses with the inner surface of the cloacal membrane and divides it to
form the urogenital membrane anteriorly and the anal membrane posteriorly in
the seventh week. The definitive
urogenital sinus develops from the primary urogenital sinus. It has a pelvic
portion and a caudal phallic portion. The vestibule (vaginal introitus) develop
from the urethral groove and phallic portion. The pelvic portion develops into
the urethra and lower third of the
vagina. A failure of the hymen to perforate results in hematocolpos when the
girl starts menstruation. If the septum between the lowest part of the fused
paramesonephric ducts and the sinovaginal node does not canalize, a transverse
vaginal septum develops. The mullerian dusts develop from the cranial end
caudally. A total absence of development of one mullerian duct would cause
unicornuate uterus, single cervix, and half of the upper two-thirds of the vagina.
It is possible that the upper part of the duct develops forming the fallopian
tube and the lower part does not develop so that there is unicornuate uterus
and lateral half of upper two-thirds of the vagina. Since the lower one-third
of the vagina develops from the sinovaginal node, it is present in such cases.
These two communicate with each other when the septum between the two
canalizes.[2]
In
the case we have presented, the patient had left unicornuate uterus, left
hemivagina (upper two-thirds), normal lower vagina (lower one-third),
imperforate hymen and as a result of that hematocolpos involving the entire
lower one-third and lateral half of the upper-two third of vagina. This
combination of mullerian anomalies has not yet been described. However it is
likely that the cases of unicornuate uterus and imperforate hymen causing
hematocolpos were of this type, but were missed. Careful evaluation of the CT
scan images of the hematocolpos in those cases may show many more cases and
perhaps it would then be a syndrome.
Conclusion
Unicornuate
uterus, hemivagina (upper two-thirds), normal lower vagina (lower one-third),
imperforate hymen and as a result of that hematocolpos involving the entire
lower one-third and lateral half of the upper-two third of vagina is a new
combination of mullerian anomalies. The actual incidence of this may be high
and will be evident if the CT or MRI images of the hematocolpos in these cases
are reviewed critically.
References
1. American Fertility Society classification of mullerian
anomalies. Fertil Steril 1988;49:952.
2. Sadler TW. Langman’s medical embryology. 12th ed. Philadelphia
3. Rock JA, Baramki TA, Parmley TH, Jones HW Jr. A unilateral functioning uterine anlage with Müllerian duct agenesis.
Int J Gynaecol Obstet 1980;18(2):99-101.
4. Rock JA, Breech LL. Surgery for Anomalies of the Mullerian
Ducts. In Rock JA, Jones HW III, editors. Te Linde’s Operative Gynecology. 10th
ed. New Delhi
Citation
Prakash S, Parulekar SV, Hira P. Unciornuate Uterus, Hemivagina, Imperforate
Hymen, Hematocolpos Syndrome. JPGO 2014 Volume 1 Number 11. Available
from: http://www.jpgo.org/2014/11/unicornuate-uterus-hemivagina.html