Introduction: A wide spectrum of etiologies can obstruct foramen of Monro (FOM) and result in hydrocephalus. Congenital occlusion of FOM is a rare entity which may present either in childhood or in adulthood.
Methods: Between 2007 and 2016, we screened all pediatric patients with hydrocephalus of either one or both lateral ventricles. Congenital occlusion of FOM was confirmed in the absence of masses occupying the FOM, prenatal or postnatal central nervous system (CNS) infections, intraventricular hemorrhage, previous cerebral intervention, or associated CNS anomalies affecting the flow of cerebrospinal fluid (CSF). We have performed a comprehensive literature review of the previously reported cases and provided a tentative embryological pathogenesis of FOM occlusion.
Results: We introduce 10 new cases of congenital FOM obstruction. The mean age of the patients was 6.65 ± 10.51 months. Two patients underwent ventriculo-peritoneal (VP) shunting as the primary intervention, while endoscopic septostomy was performed in the others. The mean follow-up was 3.05 ± 2.16 years (1-8 years). Although the hydrocephalus was controlled, all patients remained hemiparetic with some degree of developmental and cognitive impairments. Previously, 38 similar cases were reported: 10 of them (26.3%) were adults. Overall, VP shunting was the treatment of choice in 44.7% of patients. While most adults fully recovered, 7.2% of pediatrics remained hemiparetic and 10.7% of them had cognitive and developmental delay.
Conclusion: Ten cases of congenital obstruction of the foramen of Monro have been managed through a period of 9-year study. Details of these patients in addition to 38 previously reported cases are presented in this study.
Keywords: Congenital anomaly; Foramen of Monro; Obstructive hydrocephalus; Septostomy.