WOFAPS 2025 8th World Congress of Pediatric Surgery

Ana Ramirez Calazans, María Rosa Ibarra Rodriguez, Veronica Vargas Cruz, Rosa María Paredes Esteban

Hospital Universitario Reina Sofía- Córdoba- Spain

INTRODUCTION: In recent years, the better categorization of OHVIRA syndrome (OS) has allowed early diagnosis in those patients with renal anomalies detected prenatally. Our objective is to describe the characteristics of these patients in our environment in order to avoid diagnostic and therapeutic delay.

MATERIAL AND METHODS: Retrospective review of medical records of patients under 18 years of age diagnosed with OS in the last 25 years. Analysis of the clinical and demographic characteristics, diagnosis, follow-up, treatment and complications of the patients.

RESULTS: 14 women, current mean age 15.85±6.35 years, with abnormalities of genitourinary development. All with a single functioning kidney (64.3% prenatal diagnosis): 50% multicystic renal dysplasia, 35.7% renal agenesis, 7.1% renal atrophy and 7.1% double excretory system. During ultrasound follow-up, diagnosis of uterine anomaly: 50% didelphys uterus, 42.9% bicornuate uterus and 7.1% unicornuate uterus. Mean age at diagnosis of uterine anomaly 10.95±6.13 years. 21.4% diagnosed in the first year of life, the rest at pubertal age. The diagnosis of OS was confirmed in 7 patients by MRI, the rest pending completion of the study due to prepubertal age or absence of symptoms. 3 patients with OS required urgent drainage due to gynecological complications (1 pyometrocolpos, 2 hydrometrocolpos). Surgical treatment of 6 patients by means of cystoscopy, vaginoscopy and vaginal septostomy.

CONCLUSIONS: Given the prenatal or early diagnosis of unilateral renal pathology in girls, it is necessary to rule out the presence of Müllerian anomalies in order to improve surgical planning and avoid complications that could cause obstetric problems in the future.