WOFAPS 2025 8th World Congress of Pediatric Surgery

Vanessa Medina ¹, Mayra Hernandez ², Isabel Brito ²

¹ Clínica Infantil Santa Maria del Lago, Bogotá, Colombia
² Clínica Universitaria Colombia

Introduction: Herlyn – Werner – Wunderlich syndrome, also called Ohvira syndrome, is a condition with low incidence and prevalence worldwide and is an example of an anomaly at the level of the Mullerian duct, its origin, a lateral fusion defect that includes the uterus didelphys, hemivagina obstructed and ipsilateral renal agenesis. Being one of the causes of abdominal pain in adolescents, its diagnosis can be difficult, with magnetic nuclear resonance (MRI) being the means of choice for diagnosis and for planning treatment, which must be done early, favoring the relief of symptoms and the prevention of complications.

Its estimated occurrence is 0.1%–3.8%. There are described various types of anomalies, we present the surgical management in two cases with cervicovaginal atresia.

Case presentation: The cases of two patients with a diagnosis of Ohvira syndrome in whom cervicovaginal atresia was identified and managed are presented. Both patients presented with abdominal pain associated with menstrual bleeding. The diagnosis was made by MRI and they were definitively treated with laparoscopic hemihysterectomy and hemivaginectomy due to the characteristics of their malformation. Additionally, one of the patients required laparoscopic excision of endometriosis foci, showing appropriate post-surgery evolution without complications.

Conclusion: Ohvira syndrome is a rare pathology that usually manifests after puberty. Diagnostic suspicion is important in order to make earlier diagnosis and treatment to avoid complications. Laparoscopic approach of patients specifically with cervicovaginal atresia is a feasible management.