Incidence of Associated Brain and Ophthalmic Anomalies in Frontoethmoidal Encephalomeningocele Evaluated by Multidetector Computed Tomography Facial Bone Imaging.

INTRODUCTION: Frontoethmoidal encephalomeningocele (FEEM) is a congenital disorder characterized by herniation of brain and meninges through an anterior skull defect. The main pathological changes are found internally at the foramen cecum and externally at the frontonaso-orbital region. The aim of this study was to determine the incidence of developmental anomalies found in FEEM using multidetector computed tomography images of the facial bone.
METHODS: A total of 78 patients who underwent multidetector computed tomography scan of the facial bone during the January 1, 2003 to June 31, 2012 study period were retrospectively reviewed. Demographic data, size of internal defect, and intracranial anomalies were recorded.
RESULTS: Associated brain and ophthalmic anomalies were identified in 53 patients (67.9%), and all of those had brain anomalies. The most common brain anomalies were absent/undetermined septum pellucidum (75.5%), ventricular dilatation (71.7%), abnormal frontal horn (67.9%), and dysgenesis of the corpus callosum (58.5%). Eight patients (10.3%) had ophthalmic anomalies. Patients with brain and ophthalmic anomalies tended to have a higher incidence of large-size internal defects.
CONCLUSION: The authors found a high prevalence of developmental anomalies in FEEM patients. Absent/undetermined septum pellucidum was the most commonly found anomaly in this study. Brain and ophthalmic anomalies tended to have a higher incidence of large-size internal bone defects. Consistent with the authors' acknowledged limitation, further studies using dedicated brain MRI and magnetic resonance angiography to evaluate parenchymal abnormalities and vascular anomalies may be beneficial for surgical planning, prognosis, and the identification of clinical correlations.