Main

Sir,

Orbital varices are uncommon hamartomas1 and, in some cases, form part of a more widespread facial or cranial vascular anomaly. Other systemic pathology is very rare, although we have recently described 10 cases with associated cranial anomalies or encephalocoeles,2 a previously undescribed finding. We present a patient with both encephalocoele and orbital varices, in whom a severe intracerebral abscess and subdural empyema developed spontaneously, a life-threatening condition not previously known to present with enlargement of orbital varices and proptosis.

Case report

A 34-year-old white woman presented in March 2002 with a 3-day history of severe headache, marked right proptosis (Figure 1a) with restricted ductions, but normal vision with no evidence of optic neuropathy. Her optic discs and intraocular pressure were normal. She had first presented at 1 year of age with mild right proptosis, this remaining unchanged over about 20 years. At age 22, she attended the Orbital Clinic at Moorfields Eye Hospital with increased painful non-axial proptosis, gross caruncular prolapse, and conjunctival chemosis. Orbital CT confirmed medial orbital varices, but also demonstrated an unsuspected massive right intranasal encephalocoele, extending inferiorly almost to the level of the hard palate. The pre-equatorial and caruncular varices were excised through an uncomplicated transconjunctival orbitotomy.

Figure 1
figure 1

(Top) Marked right proptosis because of extensive medial orbital varices and an enlarging para-median intranasal encephalocoele. (Bottom) Partial resolution of proptosis following successful treatment with antibiotics.

Full size image

Orbital CT scan on this admission (March 2002) suggested an enlargement of both the varices and the encephalocoele, the latter now eroding the medial orbital walls (Figure 2a) and extending inferiorly to the level of the maxillary sinus (Figure 2b). The findings were considered to be compatible with either an acute haemorrhage, or thrombosis, within the pre-existing varices, and a nonsteroidal anti-inflammatory oral medication was prescribed.

Figure 2
figure 2

CT scan demonstrating orbital varices and nasal encephalocoele. (b) CT scan demonstrating encephalocoele causing obstruction to maxillary antral drainage. (c) CT scan showing brain abscess in the right frontal lobe. (d) Coronal MRI scan through the orbits to show the encephalocoele and extensive cerebritis/abscess formation. (e) Saggittal MRI of the spine showing an intraspinal subdural empyema (arrowheads).

Full size image

When the patient returned 2 weeks later, she had persistent headache and back pain, increased proptosis, and intermittent fevers; she had positive Kernig's and Brudzinski's signs despite being without neurological deficit (Glasgow coma scale 15). She had been started on oral amoxicillin 250 mg q.d.s. 3 days earlier by her general practitioner. There was a mild lymphopaenia and her erythrocyte sedimentation rate (Westergren) was 92 mm/h and C-reactive protein was 96 (range 0–10 mg/l). Repeat imaging showed a frontal lobe abscess (Figure 2c and d) and an intraspinal subdural empyema extending from T12 to S2 (Figure 2e). Pus aspirated from an L4-5 fenestration failed to culture any organisms and blood cultures were also negative. The patient was treated with intravenous cefotaxime (2 g q.d.s.) and metronidazole (500 mg q.d.s.), together with phenytoin prophylaxis.

With 3 weeks of antibiotic therapy, there was a rapid clinical improvement, resolution of the intracerebral abscess, and a significant reduction in her proptosis (Figure 1b) with maintenance of vision. The patient declined the possibility for surgical repair of her craniofacial anomaly.

Comment

Our patient demonstrated clear enlargement of her encephalocoele over the decade under our care, to such a size that it probably obstructed maxillary antral drainage, this causing an infective sinusitis with spread into the neighbouring (abnormal) central nervous system. Since intranasal encephaloceoles are a known predisposition to recurrent meningitis,3 it is very important to be aware of the association of varices with encephalocoeles.2

Surgical management of distensible orbital varices is very difficult and these hamartomas are best left alone unless optic neuropathy, severe cosmetic deformity, or bleeding become a problem. Although some undergo a slow, painless enlargement over years, the acute episode of painful proptosis (because of haemorrhage or thrombosis) would appear to be somewhat more common.

To our knowledge, the occurrence of widespread intracranial infection presenting with painful enlargement of orbital varices has not been previously described. Although rare, this is a potentially fatal condition that can be successfully treated with appropriate antibiotics.