Orbital varices, cranial defects, and encephaloceles: An unrecognized association

To highlight an apparently unreported association between orbital varices and malformations of the cranial skeleton with or without abnormalities of the underlying cerebral parenchyma. Retrospective, noncomparative review of case notes and radiologic imaging. Three hundred ten patients attending the Orbital Clinic at Moorfields Eye Hospital, London, with a diagnosis of low-pressure, low-flow orbital venous anomalies. All available orbital imaging for patients with orbital venous anomalies was examined. For those with defects of the cranial base, the radiologic characteristics were noted and the clinical details were reviewed. The presence and type of orbital roof or medial wall defects and associated nasal or other cranial anomalies. Imaging was adequate for review in 222 of 310 patients (72%), and anomalies of the neighboring cranium or cerebral structure were found in 10 of 222 patients (4.5%). In the group with cranial anomalies, the proportion of men (7/10; 70%) did not significantly differ from that in the group with varices but without cranial anomalies (93/212; 44%; P = 0.19, Fisher exact test). Orbital varices were associated with 3 types of cranial anomaly: major midline encephaloceles (type I anomaly; 4 cases), large superomedial defects of the orbital wall (type II; 3 cases), or defects of the greater wing of the sphenoid (type III; 3 cases). Clinicians should be aware of the possibility of significant cranio-orbital or cranionasal anomalies in patients with orbital venous anomalies; these anomalies can vary from minor defects in the cranial base to large encephalomeningoceles. This rare association should not be overlooked when orbital varices become markedly inflamed, because intranasal encephaloceles are a known predisposition to recurrent meningitis.