Comparison between prepubertal and postpubertal patients with obstructed hemivagina and ipsilateral renal anomaly syndrome

Abstract

Background: Obstructive hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome is a rare, complex congenital anomaly with an unknown prevalence. However, case reports and small studies on OHVIRA syndrome have increased rapidly in the last 20 years, which may be related to increased use of imaging, surgical techniques, and prenatal sonography.

Objective: This study aimed to analyze and compare patients with OHVIRA syndrome diagnosed in the prepubertal and postpubertal periods to understand the disease characteristics and improve clinical management.

Study design: A retrospective cohort study was conducted including 65 patients with OHVIRA syndrome who were diagnosed between January 2004 and September 2018 at a tertiary university hospital.

Results: Among the 65 patients, 44 patients were diagnosed with OHVIRA syndrome during the prepubertal period and 21 patients were diagnosed postpubertally. Compared with postpubertally diagnosed patients with OHVIRA syndrome, those diagnosed prepubertally were mostly asymptomatic at initial presentation (82% versus [vs.] 0%, P < 0.001), had a higher incidence of ectopic ureter (68% vs. 24%, P = 0.001), and presented with a higher incidence of multicystic dysplastic kidney (61% vs. 19%, P = 0.01). Approximately half of the patients with prepubertal OHVIRA syndrome (53%) showed spontaneous resolution of hemivaginal fluid within 5 years. Among the patients with postpubertally diagnosed OHVIRA syndrome, those in the pain-dominant group had a larger amount of hemivaginal fluid than those in the painless discharge-dominant group (54% vs. 10%, P = 0.036). Superimposed infection of hemivaginal fluid was markedly present in the discharge-dominant group (9% vs. 75%, P = 0.006).

Conclusions: Clinical characteristics of patients with OHVIRA syndrome are altered based on the time of initial diagnosis. Follow-up and timely intervention should be proceeded accordingly.

Keywords: Ectopic ureter; Herlyn-Werner-Wunderlich syndrome; Müllerian anomaly; OHVIRA syndrome; Obstructing vaginal septum; Renal anomaly.