|Year : 2013 | Volume
| Issue : 1 | Page : 105-108
Septate uterus with cervical and vaginal duplication: A rare Mullerian malformation
Meenal Patvekar, Vinayak Mahajan, Gazal Garg
Department of Obstetrics and Gynaecology, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, India
|Date of Web Publication||14-Mar-2013|
Department of Obstetrics and Gynaecology, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune
Source of Support: None, Conflict of Interest: None
A rare case of Mullerian anomaly in a 26-year-old woman who presented with recurrent pregnancy loss and was found to have a longitudinal vaginal septum with cervical duplication and two endometrial cavities separated by a complete septum. The diagnosis of this unusual anomaly was aided by magnetic resonance imaging and was confirmed by hysterolaparoscopy. It was concluded that hysteroscopy aided in the diagnosis and treatment of this Mullerian defect with the highest quality of precision. Hysteroscopy therefore is considered gold standard in the management of septate uterus, thereby helping to regain the anatomical normality and because of its minimally invasive nature, it improves the reproductive outcome.
Keywords: Hysteroscopy, mullerian anomalies, recurrent pregnancy loss, septate uterus
|How to cite this article:|
Patvekar M, Mahajan V, Garg G. Septate uterus with cervical and vaginal duplication: A rare Mullerian malformation. Med J DY Patil Univ 2013;6:105-8
|How to cite this URL:|
Patvekar M, Mahajan V, Garg G. Septate uterus with cervical and vaginal duplication: A rare Mullerian malformation. Med J DY Patil Univ [serial online] 2013 [cited 2019 Aug 17];6:105-8. Available from: http://www.mjdrdypu.org/text.asp?2013/6/1/105/108666
| Introduction|| |
Developmental anomalies of Mullerian duct system represent one of the most fascinating disorders that obstetricians and gynaecologist encounter. A wide variety of malformations can occur but their actual incidence in general population is unknown and are difficult to assess. Grimbizis et al. stated that the prevalence of Mullerian anomalies in general population is around 4.3%, about 3.5% in infertile women, and 13% in women with recurrent pregnancy loss.  Since the description by McBean and Brumsted of a women who had a septate uterus with cervical duplication and longitudinal vaginal septum, the number of similar cases has increased and challenged the classical theory of Mullerian development.  The unidirectional caudal to cephalic fusion of the Mullerian ducts is incompatible with the presence of two cervices and a single unified fundus and reinforces the alternative bidirectional theory.  Uterine dimorphisms, particularly the subseptate and septate uterus have acquired in the last few years, a special meaning in the pathogenesis of first and second trimester abortions. Mechanism depends on various factors but essentially linked to the lack of vascularization of uterine septum. Hysteroscopy has made the diagnosis of uterine septum easy and with advanced operative hysteroscopic techniques, these patients can be offered surgical treatment with less morbidity, maximum precision, and least damage to the endometrial cavity. Here, we report a rare case of recurrent pregnancy loss with in a septate uterus (complete septum) with a longitudinal vaginal septum.
| Case Report|| |
A 26-year-old woman presented with history of three early second trimester consecutive abortions, all the abortions were between 12 and 14 weeks of pregnancy. Physical examination revealed a longitudinal vaginal septum from the inferior two-third of the vagina extending up to the cervix. There was no difficulty in doing bimanual examination. Transvaginal ultrasound demonstrated two endometrial cavities [Figure 1] and [Figure 2]. Diagnosis was confirmed by magnetic resonance imaging (MRI) which showed a single uterus with two endometrial cavities and two cervices associated with a vaginal septum; there were no renal abnormalities noted. The patient was posted post-menstrually under general anesthesia. The longitudinal vaginal septum was visualized on speculum examination and was clamped and incised in the center [Figure 3]. Hysteroscopy, done with a 2.9-mm hysteroscope, revealed a complete uterine septum extending from the fundus up to the external os of the cervix [Figure 4]. The finding of septate uterus was confirmed by simultaneous laparoscopy which showed a minimal fundal depression. Tubes, ovaries, and pouch of Douglas were normal [Figure 5]. Hysteroscopic septum incision was done using monopolar resectoscope (Collins knife) [Figure 6] up to the internal os and part of the septum below the internal os was not incised to prevent cervical incompetence later. After resection of this dense septum, a single uterine cavity was achieved with two normal appearing cervices [Figure 7]. An intrauterine device was inserted for 7 days in order to prevent adhesions. Cyclical therapy with estradiol valerate, 2 mg was given once a day and medroxyprogesterone acetate was added from days 17 to 26 of cycle for two cycles.
|Figure 1: Pre-operative transabdominal sonography (TAS) showing two separate fundi|
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|Figure 7: Post-resection picture showing dilator and Collins knife in a single cavity|
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| Discussion|| |
Female genital ducts are derived from an autologous graft; the system of Mullerian ducts (paramesonephric ducts), which is evident by 6 th or 7 th week of gestation and develops progressively thus originating into salphinx, body, and neck of the uterus and 4/5 th of vagina.
Mullerian abnormalities have been classified on the basis of embryological, morphological, clinical, and functional criteria. Buttrams and Gibbons gave a clinical classification simpler than the previous ones.  American fertility society has distinguished the arcuate uterus in Class 6, previously included in Class 4, thus modifying the 6 original classification into 7. 
Rare finding of septate uterus with cervical duplication and longitudinal vaginal septum challenges the classic hypothesis of unidirectional (caudal to cranial) Mullerian development and supports an alternative embryological hypothesis which states that fusion and resorption begin at the isthmus and proceed simultaneously in both cranial and caudal directions. 
Septate uterus is associated with highest rate of obstetrical complications such as recurrent pregnancy loss, infertility, pre-term deliveries, and abnormal presentations , classically occurring between 8 and 16 weeks of gestation. The cause for repeated abortions, as stated by Candiani can be the damage caused by strongly reduced vascularization of septum which does not allow adequate modification of endometrium for implantation.  It also has been suggested that decreased estrogen progesterone receptors deficiency in malformed uterus may cause abnormal uterine contractions leading to fetal wastage. Dabirashrafi et al. found that there is less connective tissue and more muscular tissue in the uterine septum, leading to uncoordinated contractibility causing pregnancy loss. 
Among the various methods of diagnosis, tranvaginal sonography permits a better assessment with a sensitivity of 100% and specificity of 80% in the diagnosis of septate uterus.  In recent studies, 91.6% 3D ultrasound correlated with the external uterine configuration as observed by laparoscopy.  Hysterosalpingography can also detect a two-chambered uterus and allow assessment of size and extent of the septum but it cannot reliably differentiate between septate and bicornuate uterus. MRI has also been used to differentiate the structurally anomalous uterus. Letterie et al. found that MRI was not sufficiently accurate to qualify as a sole diagnostic test. 
Hysteroscopy however, still remains the gold standard in diagnosing and treating septate uterus.  It helps us to evaluate the exact extent and thickness of uterine septum. However, hysteroscopy does not allow a differential diagnosis between septate and bicornuate uterus; hence, a combined laparoscopic approach gives highest level of predictable diagnosis  and also gives the highest limit of hysteroscopic resection.
Hysteroscopic metroplasty is the procedure of choice since last few years. Considering the high number of complications such as reduction of intrauterine volume, risk of intrauterine and intraperitoneal adhesions, and caesarean section, the abdominal procedures are now obsolete.
| Conclusion|| |
Septate uterus is a major cause of pregnancy loss. Hysteroscopy being a simple, effective, and sure procedure with minimum intraoperative and post-operative morbidity remains the most ideal method of treating uterine septum hysteroscopic-guided septum resection not only eliminates an unsuitable site for implantation but also results in a better endometrial function, probably through revascularization of the connective tissue of the uterine fundus and significantly improves nidation, thereby improving the pregnancy outcome.
Pabuccu and Gomel studied 61 cases of recurrent pregnancy loss after hysteroscopic septum resection, of which 25 (41%) conceived within a period of 8-14 months. Eighteen (29.5%) patients had live birth, of which 13 carried their pregnancy to term and 5 delivered pre-term. However, seven patients had spontaneous abortions.  Hysteroscopic septum resection in women with septate uterus significantly improves the live birth rates without affecting future fertility.
| References|| |
|1.||Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey P. Clinical implications of uterine malformations and hysteroscopic treatment results. Hum Reprod Update 2001;7:161-74. |
|2.||Mc Bean JH, Brumsted JR. Septate uterus with cervical duplication: A rare malformation. Fertil Steril 1994;62:415-7. |
|3.||Musset R, Muller P, Netter A, Solal R, Vinourd JC, Gillet JY. Study of the upper urinary tract in patients with uterine malformations. Study of 133 cases. Presse Med 1967;75:1227-32. |
|4.||Buttram VC Jr, Gibbons WE. Müllerian anomalies: A proposed classification. (An analysis of 144 cases). Fertil Steril 1979;32:40-6. |
|5.||The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril 1988;49:944-55. |
|6.||Chang AS, Siegel CL, Moley KH, Ratts VS, Odem RR. Septate uterus with cervical duplication and longitudinal vaginal septum: A report of five new cases. Fertil Steril 2004;81:1133-6. |
|7.||Musich JR, Behrman SJ. Obstetric outcome before and after metroplasty in women with uterine anomalies. Obstet Gynecol 1978;52:63-6. |
|8.||Portuondo JA, Camara MM, Echanojauregui AD, Calonge J. Müllerian abnormalities in fertile women and recurrent aborters. J Reprod Med 1986;31:616-9. |
|9.||Candiani GB, Fedele L, Zamberletti D, De Virgiliis D, Carinelli S. Endometrial patterns in malformed uteri. Acta Eur Fertil 1983;14:311-8. |
|10.||Dabirashrafi H, Bahadori M, Mohammad K, Alavi M, Moghadami-Tabrizi N, Zandinejad K, et al. Septate uterus: New idea on the histologic features of the septum in this abnormal uterus. Am J Obstet Gynecol 1995;172:105-7. |
|11.||Pellerito JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH. Diagnosis of uterine anomalies: Relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 1992;183:795-800. |
|12.||Raga F, Bonilla-Musoles F, Blanes J, Osborne NG. Congenital Müllerian anomalies: Diagnostic accuracy of three-dimensional ultrasound. Fertil Steril 1996;65:523-8. |
|13.||Letterie GS, Haggerty M, Lindee G. A comparison of pelvic ultrasound and magnetic resonance imaging as diagnostic studies for müllerian tract abnormalities. Int J Fertil Menopausal Stud 1995;40:34-8. |
|14.||Prevedourakis C, Loutradis D, Kalianidis C, Makris N, Aravantinos D. Hysterosalpingography and hysteroscopy in female infertility. Hum Reprod 1994;9:2353-5. |
|15.||Marten K, Vosshenrich R, Funke M, Obenauer S, Baum F, Grabbe E. MRI in the evaluation of müllerian duct anomalies. Clin Imaging 2003;27:346-50. |
|16.||Pabuçcu R, Gomel V. Reproductive outcome after hysteroscopic metroplasty in women with septate uterus and otherwise unexplained infertility. Fertil Steril 2004;81:1675-8. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]