Obstructed Hemivagina and Renal Anomalies in Patients with and without Anorectal Malformations

STUDY OBJECTIVE:To compare the anatomic variation between patients with a diagnosis of obstructed hemivagina with an anorectal malformation (ARM) and those without ARM. METHODS:This was a retrospective chart review conducted at a single tertiary Children's Hospital. Patients with an obstructed hemivagina seen from 2004 to 2019 were included. RESULTS:We identified a total of 9 patients diagnosed with obstructed hemivagina: 4 patients with history of ARM and 5 patients without ARM. Patients presented with obstructive symptoms between the ages of 11 and 20. Two-thirds of patients had a left sided obstruction. All patients without ARM had ipsilateral congenital anomalies of the kidney and urinary tract. Half the patients with a history of ARM had an ipsilateral renal anomaly and the other half had a contralateral renal anomaly. CONCLUSIONS:Obstructed hemivagina occurs in patients with a history of anorectal malformation. However, unlike patients with isolated obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), patients with ARM and an obstructed hemivagina can present with associated renal anomalies on either the ipsilateral or contralateral side. In our small case series, patients with history of ARM had high septa and required more complex surgical management due to the inability to access the septum vaginally. Knowledge of renal anatomy and ureteral path is important since hysterectomy may be needed to relieve the obstruction in patients with ARM. A larger case series is needed to better characterize the spectrum of complex anomalies in patients with anorectal malformations.