Prenatal Diagnosis and Optimal Management of Occipital Encephalocele: A Report of Four Cases and Literature Review

Occipital encephalocele, a rare congenital malformation resulting from defective neural tube closure, accounts for 20.4% of cerebral encephaloceles, with surgical repair being the only effective treatment. This retrospective study presents four cases diagnosed at Mohammed VI University Hospital in Marrakech, highlighting prognostic variability based on lesion characteristics. The four patients, who had not received periconceptional folic acid supplementation and had inadequate prenatal follow-up, were incidentally diagnosed between 34 and 39 weeks of gestation. The first case involved an isolated encephalocele containing only cerebellar herniation, surgically treated on day 5 with a favorable outcome despite mild neurodevelopmental delay. The second case presented a massive encephalocele associated with spina bifida and clubfoot, requiring combined neurosurgical and orthopedic management, resulting in moderate sensorimotor deficits. The third case exhibited a giant encephalocele with severe cerebral herniation and secondary microcephaly, while the fourth case showed associated craniofacial anomalies; both of these latter cases resulted in early neonatal demise. This study underscores several key aspects: the importance of ultrasound as an initial screening tool, supplemented by fetal MRI for precise prognostic evaluation; the critical need for early multidisciplinary management involving obstetricians, radiologists, neurosurgeons, and pediatricians; and the essential role of periconceptional folic acid supplementation, particularly in populations at risk of nutritional deficiency or consanguinity.