Uterus didelphys with an obstructed unilateral vagina by a transverse vaginal septum associated with ipsilateral renal agenesis, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation: a case report

Coşkun, Ayhan; Okur, Nazan; Özdemir, Özgür; Grover, Kiran; Arýkan, Deniz Cemgil

doi:10.1016/j.fertnstert.2008.05.030

Cited by 38 publications

(27 citation statements)

References 15 publications

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“…Embryologically [4], the sinovaginal bulbs invaginate from the urogenital sinus and meet the mullerian tubercle on the caudal end of the mullerian ducts to form the vaginal plate that is then canalized to form the lower part of the vagina (24th week of gestation). Failure of the vaginal canalization results in the transverse vaginal septum.…”

Section: Discussionmentioning

confidence: 99%

Management of hematocolpos in adolescents with transverse vaginal septum

Deligeoroglou

Iavazzo

Sofoudis

et al. 2011

Arch Gynecol Obstet

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Section: Discussionmentioning

confidence: 99%

Management of hematocolpos in adolescents with transverse vaginal septum

Deligeoroglou

Iavazzo

Sofoudis

et al. 2011

Arch Gynecol Obstet

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“…The exact etiology of this malformation is not entirely known, but the simultaneous occurrence of malformations in the Mullerian and Wolffian ducts has been considered. The association between uterovaginal and renal malformations is frequent as the Wolffian ducts play a role in the formation of the kidneys and the internal genitalia (Gholoum et al, 2006;Zurawin et al, 2004;Vercellini et al, 2007;Coskun et al, 2008;Acien et al, 2009;Prada Arias et al, 2005) superior portion of the vagina and the uterus. Uterus didelphys occurs due to a failure in the fusion between both Mullerian ducts resulting in the duplication of the uterine body, the canal, and also possibly of the vagina.…”

Section: Discussionmentioning

confidence: 99%

Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome)

Piazza

Carvalho

Peixoto

et al. 2015

JBRA Assisted Reproduction

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This paper aimed to report a series of 19 cases of uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis. This retrospective descriptive observational study included the medical records of 19 patients seen at the Endocrinology Gynecology Sector of the Obstetrics and Gynecology Department of the Federal University of Paraná, and focused on clinical data, complementary exams, patient management, and follow-up. From the 229 patients with genital anomalies seen between 1984 and 2009, 19 (8.3%) were diagnosed with uterus didelphys with vaginal septum and renal agenesis. The patients had a median age of ±16.3 years. Eight subjects (42.6 %) reported abdominal pain; two of them (0.1%) had pelvic masses and seven (36.8%) had dysmenorrhea. Blood retention was confirmed by ultrasound and/ or magnetic resonance imaging of the pelvis, which revealed the existence of a duplicated uterus of the didelphys type with a cystic mass containing old blood obliterating the hemivagina. Urinary tract examination revealed the existence of ipsilateral renal agenesis. Unusual manifestations such as associated infection and rupture of the vaginal septum during sexual intercourse were also reported. The prevalence of uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis was 8.3% in a series of 229 genital anomalies. The observance of clinical features is essential for the early identification of the syndrome and the adequate management of the patients.

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“…Duplication of the IVC with HWWS has been reported in only one case in the English‐language literature . Coskun et al .…”

Section: Discussionmentioning

confidence: 99%

“…This syndrome may have concomitant anomalies that have important clinical implications. Recently, a report on a patient with HWWS with intestinal malrotation and abdominal vascular anomalies was published . We report on another patient with HWWS who had intestinal malrotation and vascular anomalies.…”

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confidence: 84%

Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation

Morino

Hoshino

Musha

2013

Pediatrics International

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The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn-Werner-Wunderlich syndrome (HWWS) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non-rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high-riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS. The patient underwent a preventative Ladd's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations.

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Uterus didelphys with an obstructed unilateral vagina by a transverse vaginal septum associated with ipsilateral renal agenesis, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation: a case report

Cited by 38 publications

References 15 publications

Management of hematocolpos in adolescents with transverse vaginal septum

Management of hematocolpos in adolescents with transverse vaginal septum

Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome)

Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation

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