Author Information
Valvi D*, Parulekar SV**,
Kulkarni S***, Hira P****.
(* Assistant Professor, **
Professor and Head of Department, *** Third Year Resident, Department of Obstetrics and Gynecology ; **** Associate
Professor, Department of Radiology, Seth G S Medical college and K EM hospital,
Mumbai, India)
Abstract
Mullerian duct anomalies
are usually associated with wide range of gynaecological, urological, skeletal
abnormalites, rarely associated with cardiac and anorectal anomalies. We present a rare case of complex mullerian anomalies
with partial aplasia of pancreas.
Introduction
Mullerian duct anomalies
are common, reported in up to 3.2% of all women.[1] Some mullerian anomalies
are complex and have features of more than one class. They occur due to partial
development of the müllerian ducts. Vertical fusion defect or abnormal lateral
fusion of the ducts can form the arcuate uterus, bicornuate uterus and uterus
didelphys. Both atresia or agenesis of cervix and uterine didelphys can
coexist, It falls in category 4 according modified AFC classification of
uterovaginal anomalies by Rocks and Adam classification.[2] Failure of fusion may lead to failure to canalize,
with no endometrium developing within the uterine horns. Any portion or segment
of a duct may experience agenesis. Cervical agenesis or dysgenesis is
relatively infrequent mullerian anomaly.
Cervical agenesis or atresia results from failure of müllerian duct canalization
or increased local epithelial proliferation after canalization. Uterus didelphys is the
result of complete non-fusion of mullerian ducts forming complete uterine
duplication with no communication with each other. Uterus didelphys may be
associated with a longitudinal vaginal septum (70%), rarely with transverse
vaginal septum.
Case Report
A 15 year old girl
presented with complaints of primary amenorrhea and pain in abdomen for 2
months. Her elder sister and mother had menarche at the age of 12 and 14 years
repectively. She was averagely built and
thin. Her secondary sexual characters were in Tanner stage 3. There were no skeletal abnormalities. The
abdomen was soft and nontender with no evidence of any abdominal mass. The
external genitalia was normal. She had a blind vagina which was around 5 cm
deep. On per rectal examination, the cervix was not felt; a 3 cm soft,
nontender lump was felt on right of the midline. Abdominopelvic ultrasonography
showed a didelphys uterus, absence of cervix, absence of the body and tail of
pancreas; kidney, ureter, bladder were normal. Abdominopelvic computerized
tomography (CT) showed didelphys uterus with right uterine cavity of 6.4×3×4cm
containing fluid in the endometrial cavity (figure 111.1), left horn was 2×1×1cm
in size, lateral and away from right uterine cavity, without any fluid in it.
The cervix was absent. The vagina was
normal. There was a duplication cyst of cecum (figure 111.2), and absence of
the body and tail of the pancreas (figure 111.3).
Figure 111.1. CT showing
right hematometra (arrows).
Figure 111.2. CT showing
a right convoluted mass containing fluid, interpreted as cecal duplication cyst
(arrows).
Figure 111.3. CT showing
pancreatic head (arrow).\, but absence of body and tail.
We performed a diagnostic laparoscopy followed by
laparotomy on the patient. The finding were didelphys uterus with right hematosalphinx
and cervical agenesis, and bilateral
polycystic ovaries (figure 111.4). The enlarged, tortuous,
dilated right fallopian tube had been mistaken
as duplication cyst of the cecum on CT. The hematosalpinx was 22 cm long. Its end was buried under the cecum. There was no pelvic
endometriosis. The right uterine horn was removed along with the hematosalpinx. The left horn (figure 111.5) was left behind
because it was non communicating and non-functioning, and its removal could
have compromised the blood supply of the left ovary. The patient had uneventful
recovery. Histopathology showed
hematometra and hematosalpinx.
Figure 111.4. Right
uterine horn (U), polycystic right ovary (RO) and hematosalpinx (arrows)
Figure 111.5. Left
uterine horn (LH), polycystic left ovary (LO) and left fallopian tube (LO).
Figure 111.6. Surgical specimen showing the uterine horn (U) and hematosalpinx (arrows).
Discussion
The case presented had
uterus bicornis (with the left horn nonfunctional and right horn with
hematometra), cervical atresia, a very long hematosalpinx, and agenesis of body
and tail of the pancreas. Since there was no pelvic endometriosis, which would
have occurred prior to closure of the fimbrial end of the right fallopian tube,
and since the tube was 22 cm long, which is far greater than any hematosalpinx
of that diameter reported in the literature, we believe that the very long and
tortuous tube was so due to anomalous development of the right mullerian duct.
Its end being buried under posterior aspect of the cecum also supports
anomalous development. Mullerian anomalies are
usually associated with genitourinary abnormalities, skeletal deformities, and rarely
cardiac anomalies and anorectal anomalies. Mullerian anomalies associated with aplasia
of pancreas have not been reported in the literature. Partial agenesis of
pancreas was incidentally detected on imaging in our case. Mutation of HNF β1
gene is associated with genitourinary abnormalities, aplasia of pancreas.[3]
It results from an embryological failure of the dorsal pancreatic bud to
form the body and tail of the pancreas. Blood sugar levels were normal in our
patient in spite of absent body and tail of pancreas. However she is more
likely prone to early onset diabetes mellitus because most of the islet cells
are located in the pancreatic body and tail.[4] Most of the
mullerian abnormalities are associated with functioning ovaries and external
genitalia appropriate for age. Primary
amenorrhea is the commonest presentation in cases of absence of a functioning
uterus or outflow tract agenesis or obstruction. If functional uterus or uterine horn is
present, cyclic pain in the abdomen due to cryptomenorrhea adds to the
amenorrhea. Hysterectomy is usually recommended in such cases as associated
cervical agenesis may cause complications like endometriosis, sepsis and
multiple surgeries.[5]
The diagnosis of mullerian duct anomalies is based
upon the clinical examination, physical examination and imaging. Though USG is
sufficient in most of the cases, CT and
magnetic resonance imaging (MRI) have a leading role, especially in the
diagnosis of complex anomalies. MRI has
best accuracy in the evaluation of uterine anomalies and accuracies of up to
100% have been reported.[6] Perhaps the wrong diagnosis of
duplication cyst of cecum would have been corrected with the use of MRI in our
case.
Acknowledgments
Acknowledgments
We thank Dr Vibhav Manjrekar for taking photographs in the article.
References
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Radiology 1990;176:715–20.
Citation
Valvi D, Parulekar SV,
Kulkarni S, Hira P. A Rare Case Of Complex Mullerian Anomalies With
Partial Aplasia Of Pancreas. JPGO 2015. Volume 2 No. 1. Available from: http://www.jpgo.org/2015/01/a-rare-case-of-complex-mullerian.html