Neuroendoscopic Management of Coexisting Congenital Agenesis of Bilateral Foramen of Monro with Aqueductal Stenosis and Chiari Malformation: Case Report and Review of the Literature

BACKGROUND: Bilateral occlusion of the foramen of Monro with aqueductal stenosis is a rare entity. Only 1 previous case has been reported in the literature. CASE DESCRIPTION: We present a 25-year-old female who presented with chronic headache of 7 years duration. Neurologic examination revealed only papilledema. Imaging showed symmetrical dilation of both lateral ventricles with mild dilation of the third ventricle and a normal fourth ventricle, suggesting occlusion of both foramina of Monro with membranous web formation in the upper part of the aqueduct. There was tonsillar herniation without syrinx. She underwent neuroendoscopic foraminoplasty, septostomy, and third ventriculostomy. The patient experienced total relief of headache and showed reduced volume of both lateral ventricles at follow-up. CONCLUSIONS: Bilateral occlusion of foramen of Monro with aqueductal stenosis and tonsillar herniation is rare cause of obstructive hydrocephalus, and can be managed effectively with neuroendoscopic procedure.