Elsevier

Ophthalmology

Volume 111, Issue 6, June 2004, Pages 1244-1247
Ophthalmology

Case report
Orbital varices, cranial defects, and encephaloceles: An unrecognized association☆

https://doi.org/10.1016/j.ophtha.2003.10.022Get rights and content

Abstract

Purpose

To highlight an apparently unreported association between orbital varices and malformations of the cranial skeleton with or without abnormalities of the underlying cerebral parenchyma.

Design

Retrospective, noncomparative review of case notes and radiologic imaging.

Participants

Three hundred ten patients attending the Orbital Clinic at Moorfields Eye Hospital, London, with a diagnosis of low-pressure, low-flow orbital venous anomalies.

Methods

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.

Main outcome measures

The presence and type of orbital roof or medial wall defects and associated nasal or other cranial anomalies.

Results

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).

Conclusions

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.

Section snippets

Patients and methods

Ethical committee approval was not required for this retrospective study. Patients with a clinical diagnosis of low-pressure orbital varices were identified from the orbital database at Moorfields Eye Hospital and a review of the orbital imaging (computed tomography [CT], magnetic resonance imaging, or both) performed. Films were reviewed for bony defects of the cranio-orbital interface or for anomalies of the frontal and temporal lobes of the brain. Those patients in whom there had been either

Results

Three hundred ten patients were identified as having a diagnosis of orbital varices, from among whom adequate imaging was available for 222 (72%); 6 patients with previous orbital or intracranial surgery were excluded. Associated anomalies of the neighboring extraorbital tissues were found in 10 of 222 images (4.5%; Table 1). Although the association seems more common in men (7/10 cases), this proportion in patients with cranial anomalies did not differ significantly from that of patients

Patient 1: type I anomaly

A 22-year-old woman was referred to the Orbital Clinic with a 2-year history of headaches and a slowly enlarging right medial canthal mass. She had no nasal symptoms, but adduction of her right eye had been known to be weak since childhood.

Visual functions and ocular motility were normal, but there was 4-mm pulsatile right relative proptosis and a 1-cm diameter varix causing prolapse and secondary keratinization of the right plica semilunaris (Fig 2A). Examination of her right nasal space

Discussion

Thin-slice CT scans with intravenous contrast provide excellent detail of orbital varices and allow visualization of diagnostic phleboliths. Typical findings include a serpiginous mass with slow and patchy enhancement, with variable communication with the normal orbital vessels.4, 5, 6, 7 The juxtaposition of the cranial anomalies and the abnormal orbital vessels suggests the cause to be a local failure of craniofacial development, with asymptomatic craniofacial defects otherwise being

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☆

Manuscript no. 230298.

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