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The description of this case is to contribute with all uncommon cases of Mullerian anomalies reports and clinical treatment protocols, which is not yet established. The anatomical disorders of the female reproductive system occur frequently ranging from congenital absence of the vagina or uterus, until fusion defects of the Mullerian ducts. Generally, these changes are associated with genetic mutations, developmental disabilities, or environmental causes that have an effect on embryonic development stages [ 1 ].

The descriptions of Mullerian anomalies have changed over the years and they have been presently classified under those provided by the American Fertility Society. However, septate uterus with cervical duplication and longitudinal vaginal septum is not described by this classification [ 2 ].

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Since McBean and Brumsted [ 3 ] described a similar case, the reports have suggested a change in the classical theory of unidirectional caudal to cephalic fusion of the Mullerian ducts.

In this context, this paper additionally brings a rare case report of a patient with complete septate uterus with double cervix and longitudinal vaginal septum, submitted to the vaginal septoplasty. A year-old primigravida presented to our service for missed abortion 8 weeks age.

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She reported menarche at 11 years old with normal pubertal development. Nowadays she complains dyspareunia associated with penetration difficulties.

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She did not report knowledge of urinary systems malformations and had no history of maternal exposure to diethylstilbestrol. Pelvic examination revealed normal external genitalia, with the presence of longitudinal vaginal septum and two uterine cervices Figure 1.

The magnetic resonance imaging MRIwith intravenous contrast, showed the presence of uterus cavity with complete septate uterus and without indentations in uterine serous. Ultrasonography of the kidneys and urinary tract showed no changes. The patient underwent uterine evacuation.

Return to the service, the resection of vaginal septum was made with the patient in the dorsal lithotomy position and adequate vaginal retraction to allow exposure of the septum. Initially, the septum is grd with Allis clamps, and a horizontal incision is made through the septum to resect.

An extremely rare brachydactyly syndrome with characteristics of short broad hands and feet with brachydactyly associated with congenital flexion contractures of the proximal and/or distal interphalangeal joints of the fingers, as well as syndactyly of feet. Polydactyly, septate vagina and urinary incontinence were also occasionally reported The septate uterus, the most common form of structural uterine anomaly, has the highest reproductive failure rate. A rare developmental variant is a septate uterus with cervical duplication and a complete longitudinal vaginal septum. The existence of this anomaly challenges classical Cited by: 27 I had a septate (double) vagina. Had it removed when I was 19 or Also have a septate uterus dividing it into two. I left the septum in my uterus. The dr left part of the vaginal septum on my cervix so my cervix would not get damaged. At 29 became pregnant carried to full term four days past my due date

After 45 days of vaginal septoplasty, the patient reports improvement in dyspareunia. Multiple anomalies involve the fusion of the Mullerian ducts, such as septate uterus, double cervix, and longitudinal vaginal septum. Traditionally the embryological events describe the fusion of the ducts occurring in the cranial-caudal direction, unidirectional [ 4 ].

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However, the theory has been questioned by the reports of rare anomalies, such as presented in this paper [ 356 ]. In the case of septate uterus with cervical duplication and longitudinal vaginal septum, the anomaly suggests a failure in the fusion of the distal Mullerian ducts. In the presence of uterus and vaginal septum, the cervical duplication cannot be explained by the unidirectional theory.

A new embryological theory, described initially by McBean and Brumsted [ 3 ], proposes that the fusion and resorption of ducts proceed in both directions, cranial and caudal. The embryological events are not clear yet and require further investigation.

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Brown and Badawy [ 7 ], in their case report, suggest that the system of classification of uterine anomalies, proposed by the American Society for Reproductive Medicine ASRMneeds to be revised, because it does not include these cases. The increasing number of described cases in the literature seems to be related to the availability of diagnosis.

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Several techniques have been used to investigate and confirm these cases, including hysteroscopy and laparoscopy, magnetic resonance imaging, and others, such as 3D ultrasound [ 5 ].

A precise diagnosis is necessary since the treatment and the reproductive outcome are dependent of anomaly.

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Patton et al. In symptomatic patients, as described in our case report, resection of the vaginal septum is important for well-being of dyspareunia penetration.

Ribeiro et al. Badalotti et al. The description of the case suggests that surgical interventional treatments were observed only in women with a history of fetal loss, premature labor, and other obstetric complications which could be attributed to this anomaly. The prophylactic metroplasty of uterine septum in patients with no history of previous miscarriages or pregnancy complications is still under discussion and its impact on fertility was not elucidated yet.

Our expectative with the description of this case is to contribute with all uncommon cases of Mullerian anomalies reports. The septate uterus, double cervix, and longitudinal vaginal septum were not contemplated in the classification of the American Society for Reproductive Medicine and clinical treatment protocols have not been defined yet.

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The authors declare that there is no conflict of interests regarding the publication of this paper. Copyright Samuel Barbanti et al. This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

The uterine septum was complete in all cases, extending from the fundal uterus to the external ostium of the cervix. Ten (%) of 67 women had a segmental uterine septum. This means that the uterine septum was very thin and par-tially open in the isthmic or cervical part of the uterus. Ultrasound scanning showed deviation of the uterine septum Unformatted text preview: CASE REPORT Double Cervix and Vagina with Septate Uterus: An Uncommon Mullerian Malformation Andrew F. Hundley, M.D., Julia R. Fielding, M.D.*, Lennox Hoyte, M.D. Departments of Obstetrics and Gynecology and Radiology*, Whittier St. Health Center, Brigham and Women's Hospital, Harvard Medical School Boston, MA Please address all correspondence to: Pronunciation of the word(s) "Septate Vagina".To stay ated with the latest additions, please subscribe to my dublintutorialcentre.com voice overs of medical, scientifi

Article of the Year Award: Outstanding research contributions ofas selected by our Chief Editors. Read the winning articles. Journal overview.

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Article Sections On this page Abstract Introduction Case Presentation Discussion References Copyright. Samuel Barbanti1 Nara Chiamulera, 1 and Beatriz Botelho 1 1 Gynecology and Obstetrics Service of Metropolitano Hospital, Medical School of Inga Faculty UningaAvenida D. Academic Editor: Loic Sentilhes.

Complete septate uterus with longitudinal vaginal septum is not associated with primary infertility, and pregnancy may progress successfully without surgical treatment. The results do not support elective hysteroscopic incision of the septum in asymptomatic patients or before first dublintutorialcentre.com by: A vaginal septum is a condition that happens when the female reproductive system doesn't fully develop. It leaves a dividing wall of tissue in the vagina that's not visible dublintutorialcentre.comted Reading Time: 5 mins

Received 24 Apr Accepted 09 Jul Short-rib thoracic dysplasia 20 with polydactyly. Smith-Lemli-Opitz syndrome.

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Recent clinical studies. Etiology Clinical Manifestations and Outcomes in Surgically Managed Gartner Duct Cysts.

Infertility Update: Septate Uterus Surgery, Hormones, Cysts, PCOS \u0026 Ovulation

Cope AG, Laughlin-Tommaso SK, Famuyide AO, Gebhart JB, Hopkins MR, Breitkopf DM J Minim Invasive Gynecol Mar - Apr;24 3 Epub Jan 12 doi: PMID: The anomalous clinical anatomy of congenital pouch colon in girls.

Chadha R, Choudhury SR, Pant N, Jain V, Puri A, Acharya H, Zargar NU, Kundal AK J Pediatr Surg Aug;46 8 doi: Hydrosalpinx in a patient with complex genitourinary malformation. Zhapa E, Rigamonti W, Castagnetti M J Pediatr Surg Nov;45 11 Chadha R, Gupta S, Mahajan JK, Bagga D, Kumar A Pediatr Surg Int Jul;15 A complex, high cloacal malformation: case report.

Wagner G, Holschneider AM, Gharib M Eur J Pediatr Surg Jun;8 3 See all 9.

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Fetal MRI of hydrometrocolpos with septate vagina and uterus didelphys as well as massive urinary ascites due to cloacal malformation. Liu YP, Chen CP Pediatr Radiol Aug;39 8 Epub Mar 10 doi: Communicating bicornuate uterus with double cervix and septate vagina: an uncommon malformation diagnosed with MR imaging.

La Fianza A, Campani R, Villa A, Dore R, Di Maggio EM, Preda L, Bertolotti GC Eur Radiol ;7 2 Ultrasound-guided fluid aspiration and prenatal diagnosis of duplicated hydrometrocolpos with uterus didelphys and septate vagina. Chen CP, Liu FF, Jan SW, Chang PY, Lin YN, Lan CC Prenat Diagn Jun;16 6 Hydrometrocolpos, uterus didelphys, and septate vagina: an antenatal sonographic diagnosis. Russ PD, Zavitz WR, Pretorius DH, Manco-Johnson ML, Rumack CM, Pfister RR, Greenholz SK J Ultrasound Med Apr;5 4 Uterus didelphys and septate vagina presenting as haematocolpos.

Konig HL S Afr Med J Oct 27;47 42 See all Cloacal reconstruction after a complex treatment of perineal haemangioma in a variant of PELVIS syndrome. Zalimas A, Posiunas G, Strupas S, Raugalas R, Raistenskis J, Verkauskas G BMC Pediatr Oct 8; PMID: Free PMC Article.

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See all 1. Extracranial midline defects in a patient with craniofrontonasal syndrome with a novel EFNB1 mutation. Acosta-Fernandez E, Zenteno JC, Chacon-Camacho OF, Pena-Padilla C, Bobadilla-Morales L, Corona-Rivera A, Romo-Huerta CO, Zepeda-Romero LC, Lopez-Marure E, Acosta-Leon J, Garcia-Cruz D, Maciel-Cruz EJ, Corona-Rivera JR Am J Med Genet A May; 5 Epub Feb 5 doi: Delivery of double singleton pregnancies in a woman with a double uterus, double cervix, and complete septate vagina.

Yang MJ, Tseng JY, Chen CY, Li HY J Chin Med Assoc Dec;78 12 Epub Oct 14 doi: Partial urorectal septum malformation sequence: a report of 25 cases. Wheeler PG, Weaver DD Am J Med Genet Oct 1; 2 See all 7.

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Clinical Manifestations and Outcomes in Surgically Managed Gartner Duct Cysts. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis complicated by pyocolpos and presenting as acute abdomen 11 years after menarche: presentation of a rare case with review of the literature.

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