Author
Information
Anjali Munge*, Meena Satia**,
Shruti Panchbudhe***, Vibha More***, Kimaya Mali***
(*Third year resident, **
Professor, *** Assistant Professor. Department of Obstetrics and Gynecology, Seth G.S. Medical College
and K.E.M Hospital,
Mumbai, India.)
Abstract
We
report an interesting case of an unusual Mullerian anomaly of complete uterine
septum, with duplicated cervix and longitudinal vaginal septum in a patient who
presented with primary infertility. This is an unusual case, as findings of
speculum examination of two cervices
goes in favor of bicornuate or didelphic uterus but
correlation with MRI findings
confirmed that a complete uterine septum was present and may support an
alternative hypothesis for mullerian development.
Introduction
Mullerian
duct anomalies (MDA) result from improper development and fusion of the
embryologic Mullerian ducts with incidence being 0.1-3.5% in all women.[1]
MDA’s are associated with increased risk of
obstetric problems (mid trimester abortion, malpresentation, preterm
labor, IUGR, retained placenta, postpartum hemorrhage) and gynecological
problems (dysmenorrhea, dyspareunia and infertility). A case of complete
uterine septum with cervical duplication and longitudinal vaginal septum is
reported which does not fit in the common classification of mullerian anomalies. The main purpose of magnetic
resonance imaging (MRI), clinical, and surgical examination of patients is to
reach a accurate diagnosis as certain mullerian anomalies may benefit from
surgical correction.
Case
Report
A
22 years old woman, married for 4 years, was first seen in gynecology out
patient department for primary infertility. On general examination, she was
phenotypically female with well developed secondary sexual characteristics.
Speculum examination showed a longitudinal vaginal septum, with two cervices
seen on either side of the septum. On vaginal examination, two cervices were
felt on either side of the longitudinal vaginal septum, uterus was of normal
size, anteverted, and bilateral fornices were free and non tender. Routine
workup for infertility was advised and was normal. Ultrasonography was
suggestive of normal uterus and bilateral adnexa. In view of disparity between
clinical findings and ultrasonography, MRI pelvis with abdomen for further
evaluation was done. It showed a normal sized uterus with a muscular septum
extending into the cervix dividing the endometrial cavity and the cervix into
two. Fundal contour appeared almost flat with a mild indentation. Vagina was
also divided into two by a longitudinal vaginal septum. There were no urinary
tract anomalies. In view of above MRI findings patient was posted for
diagnostic hysteroscopy and laparoscopy with vaginal septum resection.
Figure
1. A. Complete longitudinal vaginal septum; B. Two cervices on either side of
complete longitudinal vaginal septum.
Figure
2. MRI showing muscular septum extending into the cervix dividing the
endometrial cavity and the cervix into two. Vagina was also divided into two by
a longitudinal vaginal septum.
Hysteroscopy
was done through both the cervices and both the respective ostia were seen with
a septum extending from the fundus to cervix through the entire uterine
activity. On laparoscopy uterus was normal in size with mild indentation at the
fundus with normal fallopian tubes and ovaries. On chromopertubation with
methylene blue dye from right cervix spill was present through the right
fallopian tube and similarly when the dye was injected through the left cervix
showed evidence of spill from left fallopian tube. In view of above operative
findings it was concluded that the uterus had a complete septum and there were
two cervices with a longitudinal vaginal septum. Resection of the vaginal
septum was done. The patient's postoperative course was uneventful and she was
discharged on the fifth day of surgery. On discharge patient was advised
follicular monitoring with planned relations.
Figure
3. Intraoperative laparoscopic finding of broad fundus &normal right ovary
&fallopian tubes.
Figure
4. Postoperative view after excision of longitudinal vaginal septum with two
cervices.
Discussion
Maldevelopment
of the mullerian duct occur in a variety of forms and each anomaly is
distinctive. This rare mullerian anomaly does not fit into the usual
classification of mullerian anomalies provided American Fertility Society (AFS)
Classification (1988).[2] This fits into the modified classification
of uterovaginal anomalies given by AFS. It belongs to class III B which includes
disorders of lateral fusion of the mullerian ducts, of the symmetric-
unobstructed variety of septate uterus with complete uterine septum, two
cervices, and complete longitudinal vaginal septum.
The
major part of the female genital tract develops from the Mullerian duct which
forms as an ingrowth of celomic epithelium in the lateral aspect of mesonephros
at about 5-6 weeks. This ingrowth forms a tube and grows caudally and divides
into 3 parts - cranial vertical, middle horizontal and caudal vertical. Uterus
is developed by the fusion of the intermediate horizontal and caudal vertical
by 12 weeks. Cervix is developed by the fusion of the lower vertical part of
the two Mullerian ducts. Septal resorption follows shortly beginning at any
point of fusion and can move in both directions. Apoptosis has been proposed as
a mechanism for septal resorption and is mediated by Bcl-2, a protein involved
in regulating apoptosis. It was found that absence of this critical protein
plays a crucial role in persistence of the septum and lateral fusion disorders.[3] Development of the vagina is composite -
upper part above the hymen is developed from the fused uterovaginal canal of
mullerian duct and lower part below the hymen from urogenital sinus.[4] Our case reported here cannot be explained by
this routine theory of development.
An
alternative hypothesis suggested by Musset et al in which development of female
genital tract is a three-stage process.[5] The medial aspects of the
mullerian ducts begin to fuse in the middle and proceed in both the cephalad
and caudal directions simultaneously, followed by rapid cellular proliferation
between the ducts, forming the uterine corpus and cervix, and then septal
resorption, all of which occur in both directions simultaneously. As per this
theory, the double vagina and cervix could be explained by failed fusion of the
mullerian ducts in the caudal direction beginning at the uterine isthmus. The
septate uterus and vagina could be explained by complete failure of septal
resorption after fusion.
In
a patient with mullerian anomalies it is a critical step to have an appropriate
preoperative evaluation of reproductive and pelvic anatomy before an operation
is preformed. For exact diagnosis of the mullerian anomalies internal as well
as external appearance of the uterus must be visualized. Several investigations
in combination like hysterography, hysteroscopy, ultrasonograghy, laparoscopy
and MRI may help to arrive at a diagnosis. Simultaneous evaluation of urinary
tract must also be done as its association with mullerian anomalies has been
proven. MRI is considered gold standard for imaging uterine anomalies. MRI
provides high resolution of uterine fundus, body and internal structures and
also helps in simultaneous evaluation of urinary tract for associated
anomalies. Maximal fundal indentation less than 10 mm and an angle between the
medial margins of the hemi cavities of less than 60 degrees are two diagnostic
criteria on MRI for differentiating between septate and bicornuate/didelphic uteri
as suggested by Fedele et al.[6,7] Mere presence of any malformation
per say it not an indication for any surgical intervention unless associated
with any obstetric or gynecological problem.
References
1.
Speroff L. The uterus. In: Mitchell C, ed.
Clinical Gynecologic Endocrinology and Infertility. 7thed. Philadelphia,
Pa: Lippincott Williams &
Wilkins; 2005. p 113.
2.
American Fertility Society .The American
Fertility Society classifications of adnexal adhesions ,distal tubal occlusion ,tubal occlusion secondary
to tubal ligation, tubal pregnancies ,mullerian anomalies and intrauterine
adhesions. Fertil Steril 1988;49: 944.
3.
Lee DM, Osathanondh r, Yeh J. Localization of
Bcl-2 in the human fetal mullerian tract. Fertil Steril 1998;70 :135.
4.
Crosby WM, Hill EC. Embryology of the mullerian
duct system. Obstet Gynecol 1962;20:507–15
5.
Musset R, Muller P, Netter A, Solal R, Vinourd
JC, Gillet JY. E´ tat du haut appareil urinaire chez
les porteuses demalformations uterines, Etude de 133 observations. LaPresse Medicale
1967;75:1331– 6.
6.
Pellerito JS. McCarthy SM, Doyle MB. Diagnosis
of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography,
and hysterosalphingraphy .Radiology 1992; 183:795.
7.
Fedele L, Dorta M,
Brioschi D, Massari C, Candiani GB .Magnetic resonance evaluation of double
uteri. Obstet
Gynecol 1989; 74: 844 –7.
Citation
Munge A, Satia M,
Panchbudhe S, More V, Mali
K. Atypical Mullerian Anomaly. JPGO Volume 1 Issue 3, March 2014, available at:http://www.jpgo.org/2014/03/atypical-mullerian-anomaly.html