Abstract

Fetal occipital cephaloceles display significant morphologic heterogeneity resulting in variable cognitive and survival outcomes. The purpose of this study was to determine if specific imaging findings could provide predictive information on the clinical outcomes of patients with occipital cephalocele. We conducted a retrospective review of fetal occipital cephalocele patients. Fetal and post-natal imaging studies were evaluated for multiple parameters including: cephalocele size, ellipsoid volume, herniation of various neural tissues, and microcephaly. Based on the presence of certain findings, an imaging score (range: 0-11) and cephalocele grade (range: 0-4) were calculated. Higher fetal and post-natal imaging scores were positively correlated with higher cephalocele grade (p<0.0001). Higher cephalocele grade was positively correlated with cerebellum and occipital lobe involvement (p<0.05). A higher fetal cephalocele grade was associated with a significantly high risk of mortality (CI: 15.5-22.10; p<0.0001). Higher imaging scores and cephalocele grade were associated with a greater risk of mortality and verbal and motor delays. Imaging factors that appear to play a role in increasing cephalocele grade include involvement of the cerebellum, occipital lobes, and microcephaly. These findings may help counsel parents regarding the post-natal course of patients with occipital cephalocele.

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