Uterine Didelphys, Obstructed Hemivagina, and Ipsilateral Renal Agenesis: Presentation and Management

Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature

“…The original articles were selected based on features such as age, major symptom, obstructed side, ipsilateral/contralateral renal anomaly, Müllerian anomaly, types of diagnostic imaging, obstruction mechanism and treatment. Data extraction compiled 521 into a descriptive analysis [ [12] , [13] , [14] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] , [42] , [43] , [44] , [45] , [46] , [47] , [48] , [49] , [50] , [51] , [52] , [53] , [54] , [55] , [56] , [57] , [58] , [59] , [60] , [61] , [62] , [63] , [64] , [65] , [66] , [67] , [68] , [69] , [70] , [71] , [72] , [73] , [74] , [75] , [76] , [77] , [78] , [79] , …”

Unusual Cause of Sepsis in a 10-Year-Old Female

“…4 OHVIRA syndrome is rare, with an estimated incidence of 1:20 000. 1,2 In OHVIRA syndrome, the vaginal septum results in one vagina being unobstructed and one obstructed, which occurs in only 6% of cases with uterine duplication. 3 Renal agenesis is present in 92% to 100% of patients with obstructed hemivagina, where it is only present in 63% to 81% of patients with uterine duplication alone.…”

“…Typically, fusion of the Mullerian ducts occurs during week 9 of embryogenesis, but when this does not occur or occurs incompletely, female reproductive tract anomalies result. [1][2][3] Mullerian anomalies occur in approximately 7% of the female population, of which 40% also have an associated renal anomaly. 4 OHVIRA syndrome is rare, with an estimated incidence of 1:20 000.…”

“…3 Most cases of OHVIRA present after menarche with irregular menses, dysmenorrhea, and progressive pelvic pain associated with menstruation. [1][2][3] Over time, the menstrual blood collects within the obstructed hemivagina leading to hematocolops and causing distension and pain, particularly during menstruation. Additional presenting symptoms include dyspareunia and urinary retention, which typically occurs 1 to 2 years after menarche.…”

“…Additional presenting symptoms include dyspareunia and urinary retention, which typically occurs 1 to 2 years after menarche. [1][2][3][4][5] The distension may result in a vaginal bulge that is either felt by the patient or visualized on physical examination. 3,5 The size of hematocolops or hematosalpinx varies by patient and is dependent on individual vaginal expansibility.…”