Uterine Didelphys with Transverse Vaginal Septum – A Complex rare Müllerian anomaly

During the development of the female genital tract, any insult to the normal development process results in a set of intriguing abnormalities known as Müllerian duct abnormalities. The uterine didelphys is the second least common type of anomaly among these, which may commonly be associated with a longitudinal vaginal septum (lateral fusion defect). However uterine didelphys along with a transverse vaginal septum (lateral fusion plus resorption defect) is a very rare finding and to the best of our knowledge, thecase that we hereby report is the second one in literature.A 16-year-old unmarried girl presented with primary amenorrhoea and cyclical pain for 18months.On clinical examination and imaging, a case of uterine didelphys and transverse vaginal septum was found. Her urinary tract was normalon USG and MRI evaluation. Excision of the septum was done by abdomino-vaginal approach. The patient was discharged well.We conclude that a patient presenting with primary amenorrhea especially with cyclical dysmenorrhea with a transverse vaginal septum on examination should be thoroughly investigated for associated upper genital tract abnormalities as the treatment strategy and prognosis is largely dependent on the correct classification of the anomaly.

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Management of Transverse Vaginal Septum by Vaginoscopic Resection: Hymen Conservative Technique

Revista Brasileira de Ginecologia e Obstetrícia / RBGO Gynecology and Obstetrics ◽

10.1055/s-0038-1670714 ◽

2018 ◽

Vol 40 (10) ◽

pp. 642-646

Author(s):

Gennaro Scutiero ◽

Pantaleo Greco ◽

Piergiorgio Iannone ◽

Giulia Bernardi ◽

Francesca Greco ◽

...

Keyword(s):

Surgical Treatment ◽

Genital Tract ◽

Cultural Beliefs ◽

Foley Catheter ◽

Female Genital Tract ◽

Vaginal Septum ◽

Menstrual Flow ◽

Transverse Vaginal Septum ◽

Female Genital

AbstractTransverse vaginal septum is a rare female genital tract anomaly, and little is described about its surgical treatment. We report the case of a patient who wished to preserve hymenal integrity due to social and cultural beliefs. We performed a vaginoscopic resection of the septum under laparoscopic view, followed by the introduction of a Foley catheter in the vagina, thus preserving the hymen. After 12 months of follow-up, no septal closure was present, and the menstrual flow was effective. Vaginoscopic hysteroscopy is an effective method of vaginal septum resection, even in cases in which hymenal integrity must be preserved due to social and cultural beliefs.

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Traumatic recto-vaginal fistula in two patients with congenital vaginal septum: a case report

African Journal of Midwifery and Women s Health ◽

10.12968/ajmw.2018.0029 ◽

2020 ◽

Vol 14 (3) ◽

pp. 1-5

Author(s):

Weston Khisa ◽

Winnie Mwebia ◽

Alice Muli ◽

Linda Nasengo

Keyword(s):

Case Report ◽

Sexual Assault ◽

Genital Tract ◽

Pelvic Examination ◽

Vaginal Fistula ◽

Rare Form ◽

Vaginal Septum ◽

Primary Amenorrhoea ◽

Transverse Vaginal Septum

Congenital vaginal septum is a rare form of genital tract anomaly. It presents with dyspareunia, dysmenorrhoea or primary amenorrhoea associated with hematometra and hematocolpos. Others may be asymptomatic, with the anomaly being found during routine pelvic examination. This case study presents two patients with congenital vaginal septae, who developed traumatic recto-vaginal fistulae. The first patient had two incomplete longitudinal vaginal septae, incidentally discovered during repair of a recto-vaginal fistula that had developed following sexual assault. The second patient developed a recto-vaginal fistula following repair of a transverse vaginal septum.

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Complex mullarian duct abnormality in a young female: a theraputic dilemma

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20173510 ◽

2017 ◽

Vol 6 (8) ◽

pp. 3673

Author(s):

Mukta Agarwal ◽

Bhawana Tiwary ◽

Prajnanika Gurung

Keyword(s):

Lower Abdominal Pain ◽

Rare Condition ◽

Young Female ◽

Urogenital Sinus ◽

Incomplete Fusion ◽

Primary Amenorrhea ◽

Vaginal Septum ◽

Primary Amenorrhoea ◽

Transverse Vaginal Septum ◽

Mullerian Ducts

Genital outflow tract obstruction is a rare cause of primary amenorrhoea. Cervical agenesis is a very rare condition often associated with atresia of vagina. Clinical diagnosis is usually difficult before surgery. Transverse vaginal septum or vaginal agenesis is also a rare condition that results from incomplete fusion between vaginal components of the mullerian ducts and urogenital sinus. If the septum is complete, the menstrual flow will be obstructed causing primary amenorrhoea. The septum is basically a membrane of fibrous connective tissue with both muscular and vascular components formed anywhere along the vagina during embryological development. Here we present a case of 18 year old female who presented with primary amenorrhea, cyclical lower abdominal pain and menouria since 5 years. There was no history of attainment of menarche. The clinical examination revealed a small, blind ending lower vagina with a tough transverse membrane separating the lower portion from the upper genital tract. The ultrasound examination revealed a normal size uterus with hematometra. The magnetic resonance imaging of pelvis confirmed the presence of hematometra and transverse vaginal septum. Transverse vaginal septum resection followed by abdomino-perineal cervicoplasty was done in this patient.

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Acute abdomen in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome

Vojnosanitetski pregled ◽

10.2298/vsp0809706p ◽

2008 ◽

Vol 65 (9) ◽

pp. 706-709 ◽

Cited By ~ 1

Author(s):

Aleksandra Petric ◽

Milan Stefanovic ◽

Predrag Vukomanovic ◽

Radomir Zivadinovic ◽

Aleksandra Tubic ◽

...

Keyword(s):

Acute Abdomen ◽

Genital Tract ◽

Female Genital Tract ◽

Careful Examination ◽

Normal Female ◽

Vascular Leiomyoma ◽

Primary Amenorrhoea ◽

Healthy Women ◽

Dead End ◽

Female Genital

Background. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a malformation of female genital tract (incidence 1 in 4000 female newborn children). It appears as a result of a disorder in the development of Millerian cannals. Etiology is unknown. Syndrome MRKH is the most frequent cause of primary amenorrhoea (90%). Patients with MRKH have a normal female phenotype, with normal pubic hairness and thelarche, and female karyotype (46XX) followed by primary amenorrhoea. Hormonal status corresponds to healthy women, where the appearance of ovarian tumors and tumors on rudiment parts of uterus is possible. Case report. We presented a case of acute abdomen in a patient with previously not diagnosed MRKH. The diagnosis was done during the operation. Small pelvis and an abdominal part were filled with torquated tumor lump, where ovaries, oviducts, uterus or something resembling rudiment of uterus were not recognized through careful examination. Furthemore, the patient had a short, dead-end vagina. Tumorectomy was done and hystopathological finding showed the presence of vascular leiomyoma. Conclusion. The diagnosis of complex syndromes, such as MRKH, can, despite modern diagnostics, be absent for non-medical and psycho-social reasons. We can expect ovarian and uterine pathology on hypoplastic structures in these patients, as well as in healthy women. Vascular leiomyoma in the patients with MRKH was not found in the available literature.

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Transverse vaginal septum presenting as secondary amenorrhoea: a rare clinical presentation

BMJ Case Reports ◽

10.1136/bcr-2020-235374 ◽

2020 ◽

Vol 13 (8) ◽

pp. e235374 ◽

Cited By ~ 1

Author(s):

Garima Yadav ◽

Neha Agrawal ◽

Sureka Binit ◽

Pratibha Singh

Keyword(s):

Differential Diagnosis ◽

Abdominal Pain ◽

Menstrual Cycle ◽

Clinical Presentation ◽

Primary Amenorrhea ◽

Early Age ◽

Cell Debris ◽

Vaginal Septum ◽

Secondary Amenorrhea ◽

Transverse Vaginal Septum

Transverse vaginal septum is one of the variants of Mullerian duct anomaly, caused as a result of defective fusion or recanalisation of vaginal and Mullerian organs. At an early age, it commonly presents as primary amenorrhea along with cyclical abdominal pain while later on usually it presents as dyspareunia and infertility. Our 22-year-old patient presented with secondary amenorrhea. It is very unusual for a transverse vaginal septum to cause secondary amenorrhea. MRI and clinical examination raised the suspicion of transverse vaginal septum causing secondary amenorrhea. She attained regular menstrual cycle after septum excision. The proposed theory behind it is obliteration of microperforated transverse vaginal septum because of menstrual blood and cell debris. Thus, a rare possibility of transverse vaginal septum should also be considered as a differential diagnosis of secondary amenorrhea.

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OC24.04: Diagnosis of septate uterus by 3D US using the ESHRE-ESGE consensus on classification of congenital anomalies in the female genital tract

Ultrasound in Obstetrics and Gynecology ◽

10.1002/uog.15103 ◽

2015 ◽

Vol 46 ◽

pp. 52-52

Author(s):

B. Graupera ◽

M. Pascual ◽

L. Hereter ◽

C. Pedrero ◽

J.L. Browne ◽

...

Keyword(s):

Congenital Anomalies ◽

Genital Tract ◽

Female Genital Tract ◽

Septate Uterus ◽

Female Genital

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Müllerian Anomalies: A Cause for Primary Amenorrhea

Journal of SAFOG with DVD ◽

10.5005/jp-journals-10006-1499 ◽

2017 ◽

Vol 9 (3) ◽

pp. 221-224

Author(s):

Anuja V Bhalerao ◽

Richa Garg

Keyword(s):

Correct Diagnosis ◽

Management Strategies ◽

Androgen Insensitivity Syndrome ◽

Primary Amenorrhea ◽

Vaginal Septum ◽

Imperforate Hymen ◽

Secondary Sexual Characters ◽

Transverse Vaginal Septum ◽

Preservation Of Fertility ◽

Secondary Sexual

ABSTRACT Introduction Primary amenorrhea is a challenging entity as it affects the reproductive outcome. Primary amenorrhea is an absence of secondary sexual characters until 14 years of age or absence of menstruation with secondary sexual characters until 16 years of age. Aim The aim of this article is to study the frequency, etiologic causes, presentation, diagnosis, and optimal mode of management of primary amenorrhea. Materials and methods All girls who had not attained menarche until 16 years of age in the absence of secondary sexual characters and until 14 years, if no secondary sexual characters, and were willing for follow-up were subjected to investigations and were treated as per the etiology. Results Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome was the most common cause of primary amenorrhea (42.85%) followed by imperforate hymen (28.57%) and transverse vaginal septum (21.42%), one each of low, mid, and high varieties. There was one case of androgen insensitivity syndrome. Conclusion Establishing correct diagnosis is essential for planning treatment and management strategies in primary amenorrhea as treatment goals are preservation of fertility and progression of normal development. How to cite this article Bhalerao AV, Garg R. Müllerian Anomalies: A Cause for Primary Amenorrhea. J South Asian Feder Obst Gynae 2017;9(3):221-224.

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A Rare Case of Primary Amenorrhea with Two Etiologies, Hypothalamic Amenorrhea, Transverse Vaginal Septum, and No Hematocolpos

Case Reports in Obstetrics and Gynecology ◽

10.1155/2015/989123 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3

Author(s):

Firouzeh Ghaffari ◽

Fatemeh Keikha ◽

Arezoo Arabipoor

Keyword(s):

Rare Case ◽

Intrauterine Insemination ◽

Uterine Cavity ◽

Primary Amenorrhea ◽

Hypothalamic Amenorrhea ◽

Vaginal Septum ◽

Transverse Vaginal Septum ◽

Systematic Strategy ◽

Atypical Presentations

We reported a rare case of hypothalamic amenorrhea and transverse vaginal septum. A 28-year-old woman presented with primary amenorrhea and no complaint of abdominal pain. Laparoscopy revealed a small rudimentary uterus with streak ovaries and a vaginal pouch. The patient with diagnosis of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome was subjected to a vaginoplasty in another fertility center. In our institute, after two courses of estrogen and progesterone, sonography revealed hematocolpos, while, under anesthesia, transverse vaginal septum was resected. Hysteroscopy revealed normal uterine cavity. She became pregnant 5 months postoperatively with controlled ovarian stimulation (COS) in conjunction with intrauterine insemination, and she has two healthy babies now. This case highlights the importance of careful evaluation of all primary amenorrheas. Clinicians should be aware of presence of more than one etiology which causes atypical presentations and accomplishes a systematic strategy for the evaluation of amenorrhea potential to avoid long-term side effects of a misdiagnosis.

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