Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome – one clinical center case series and the systematic review of 734 cases

Kudela, Grzegorz; Wiernik, Agnieszka; Drosdzol–Cop, Agnieszka; Machnikowska-Sokołowska, Magdalena; Gawlik, Aneta; Hyla-Klekot, Lidia; Gruszczyńska, Katarzyna; Koszutski, Tomasz

doi:10.1016/j.jpurol.2021.06.023

Cited by 36 publications

(68 citation statements)

References 29 publications

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“…In patients with OHVIRA syndrome, a vaginal septum dissection is often sufficient, although hemi-hysterectomy has also been reported as a surgical solution, especially in cases of thick proximal vaginal septum, as in the case of our patient (case 1) [ 29 , 30 ].…”

Section: Discussionmentioning

confidence: 88%

Reassessment of Surgical Procedures for Complex Obstructive Genital Malformations: A Case Series on Different Surgical Approaches

Hoeller

Steinmacher

Schlammerl

et al. 2022

JCM

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The objective of this case series was to describe different uterus-preserving surgical approaches and outcomes in patients with complex obstructive Müllerian duct malformation caused by cervical and/or vaginal anomalies. A retrospective analysis was performed including patients undergoing uterovaginal anastomosis (n = 6) or presenting for follow-up (n = 2) at the Department for Gynecology at the University of Tuebingen between 2017 and 2022. Uterovaginal anastomosis was performed with a one-step combined vaginal and laparoscopic approach (method A), a two-step/primary open abdominal approach with primary vaginal reconstruction followed by abdominal uterovaginal anastomosis after vaginal epithelization (method B) or an attempted one-step approach followed by secondary open abdominal uterovaginal anastomosis due to reobstruction (method A/B). Patients presented at a mean age of 15 years. Two patients were treated by method A, four by method B and two by method A/B. Functional anastomosis was established in seven of eight patients, with normal vaginal length in all patients. Concerning uterovaginal anastomosis, the primary open abdominal approach with or without previous vaginal reconstruction seems to have a higher success rate with fewer procedures and should be implemented as standard surgical therapy for complex obstructive genital malformations including the cervix.

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Section: Discussionmentioning

confidence: 88%

Reassessment of Surgical Procedures for Complex Obstructive Genital Malformations: A Case Series on Different Surgical Approaches

Hoeller

Steinmacher

Schlammerl

et al. 2022

JCM

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“…In the uncommon case of OHVIRA, there can also be unusual periods, such as in the newborn, prepubertal period, or presenting with multiple variants [3,4,13]. A review of 734 cases showed dysplastic or multicystic kidney (7.8%), endometriosis (13.6%), a single uterus (0.4%) or in other case reports, an ectopic ureter or a duplicated ureter [14][15][16] . From this information, this condition is quite rare and can be caused by misdiagnoses [17].…”

Section: Discussionmentioning

confidence: 99%

“…Recently published data showed that the Holmium laser, an electrothermal bipolar vessel sealing device, may not be sufficient for removing a thick vaginal septum. There are still differences in the details of preoperative management and the technique of traditional septum excision, such as the role of preoperative hormonal treatments or whether to suture the wound after excision [14]. The previous data showed more conservative options for patients in the prepubertal age group.…”

Section: Discussionmentioning

confidence: 99%

Optional Method with Innovative Instrument for Ohvira Syndrome Treatment: Case Reports and Review of Literature

Satirapod¹

2023

J Gynecol Res Rev Rep

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OHVIRA syndrome is a rare condition usually present during early puberty, after menarche. The main symptoms are caused by the obstruction of hemivagina and accumulation of blood, which cause the symptoms such as dysmenorrhea, chronic pelvic pain or pelvic mass. Transvaginal septectomy is the best way to treat of patient’s obstructive hemivagina. However, in young patients, the small vagina canal is a significant limitation, and it is challenging to explore the surgical field. Due to the uncommon condition at young ages and variation in coexisting anomalies, approach techniques and instruments are still under development. In this case series, we present 2 cases of OHVIRA syndrome who received surgical treatment at Ramathibodi Hospital, Mahidol University. Both cases used the same innovative vaginal retractor, designed and modified for the young patient with OHVIRA syndrome. After the procedure, patients showed no complications or recurrence up to 2 years of follow-up.

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“…18 Uterine anomaly is not the cause for symptoms in patients with HWW syndrome, however, different uterine morphology may direct different surgical approaches and future treatment. For example, hemihysterectomy may occasionally be performed in complicated cases or when vaginal septoplasty is impossible, 49,50 however, it can only be performed in patients with didelphys uterus. For patients with the complete septate uterus, surgical correction with uterine septum hysteroscopic excision might be beneficial and should be considered.…”

Section: Treatmentmentioning

confidence: 99%

Preoperative MRI presentations of Herlyn–Werner–Wunderlich syndrome

Zhang

Zheng

Ning

et al. 2022

Congenital Anomalies

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Herlyn–Werner–Wunderlich (HWW) syndrome is a rare complex female urogenital anomaly, with diverse anatomical presentations. Due to obstruction, most patients with HWW syndrome need to be addressed surgically. The treatment strategy should be tailored to the different anatomical variants of each patient. Therefore, a detailed and comprehensive preoperative evaluation is needed. In this review, we describe the embryology and clinical manifestations of HWW syndrome and discuss and illustrate its diverse preoperative magnetic resonance imaging presentations to guide clinical treatment.

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Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome – one clinical center case series and the systematic review of 734 cases

Cited by 36 publications

References 29 publications

Reassessment of Surgical Procedures for Complex Obstructive Genital Malformations: A Case Series on Different Surgical Approaches

Reassessment of Surgical Procedures for Complex Obstructive Genital Malformations: A Case Series on Different Surgical Approaches

Optional Method with Innovative Instrument for Ohvira Syndrome Treatment: Case Reports and Review of Literature

Preoperative MRI presentations of Herlyn–Werner–Wunderlich syndrome

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