Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation

Abstract

The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn-Werner-Wunderlich syndrome (HWWS) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non-rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high-riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS. The patient underwent a preventative Ladd's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations.

Herlyn-Werner-Wunderlich syndrome: a case report

“…Adenocarcinoma of the obstructed side of the uterine cervix and clear cell carcinoma of the obstructed portion of the vagina have also been described 17 . Delays in diagnosis are common due to a number of factors, especially in incomplete hemivaginal obstruction: as there is normal menstruation from the non-obstructed side, outflow obstruction is not suspected; the syndrome is also rare and thus it is not on the list of suspected diagnoses in most cases [16][17][18] . Another reason is that in patients presenting with cyclical dysmenorrhea, the patient's primary care provider often prescribes non-steroidal anti-inflammatory drugs and oral contraceptives, which suppresses or eliminates menses and further delays recognition of the condition.…”