Clinical characteristics of 415 patients with obstructed hemivagina and ipsilateral renal anomaly syndrome: A nationwide multicenter retrospective study

BACKGROUND: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital genital tract anomaly that severely impacts quality of life and fertility in female adolescents. Most of the publications on OHVIRA have been case reports or small retrospective studies, and the clinical characteristics of this extremely rare anomaly have not been well documented. OBJECTIVE: To determine the clinical characteristics of OHVIRA, including its symptoms, anatomic features, concomitant malformations, surgeries, endometriosis and other complications according to subtype in a large nationwide cohort. STUDY DESIGN: We searched the Chinese Female Reproductive Tract Malformation registry platform and retrospectively identified 517 cases of OHVIRA that were diagnosed and treated at any of 30 centers in China between January 2015 and July 2024. After exclusion of 79 cases with incomplete or duplicated data and further exclusion of 23 cases that were not classifiable according to subtype, the clinical data for 415 patients were analyzed according to subtype of OHVIRA. RESULTS: Subtype I accounted for 42.2 % of patients (175/415), subtype II for 40.2 % (167/415), subtype III for 8.4 % (35/415), and subtype IV for 9.2 % (38/415). Subtypes I and II were more common. A rare hitherto unrecognized subtype was also identified. Skeletal malformation (66/438, 15.1 %) was the most common concomitant anomaly after urinary system malformation. Dysmenorrhea (337/438, 76.9 %) and irregular vaginal bleeding (218/438, 49.8 %) were prominent symptoms, with the latter being significantly more common in patients with subtype II or III (P < 0.05). Rates of pelvic inflammatory disease (PID) and endometriosis varied according to subtype (P < 0.05). Patients with incomplete obstruction (subtype II or III) were more susceptible to PID, and endometriosis was detected at a rate of 68.4 % (26/38) in those with subtype IV. Whether or not spontaneous pregnancy had been achieved preoperatively showed no correlation with subtype, uterine anomaly, symptoms, complication by PID or endometriosis, and concomitant malformation in patients who were engaged in regular sexual intercourse without contraception. CONCLUSIONS: The present system that classifies OHVIRA into 4 subtypes covers most of the anatomic variants of this disease and is easy to apply in a Chinese cohort. However, this study has identified a specific anatomic variant that has not been reported previously. Patients with incomplete obstruction (subtype II or III) are more likely to report irregular vaginal bleeding and are susceptible to PID. Dysmenorrhea is more prominent in patients with complete obstruction, particularly those with subtype IV. Rate of endometriosis in OHVIRA are higher than that in population, particularly those with complete obstruction. Whether spontaneous pregnancy occurs preoperatively has no correlation with the clinical features of OHVIRA.