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.)
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.
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. 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.
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.
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). 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. 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. 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. 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.
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.
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