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Moorfields Eye Hospital, London EC1V 2PD, UK
Keywords: encephalocele; orbital; varices; wall defects
Orbital varices are a vascular hamartoma typified by a plexus of low pressure, low flow, thin walled and distensible vessels that intermingle with the normal orbital vessels.1–4 If freely communicating with the orbital circulation, engorgement of varices can occur by increasing venous pressure through the Valsalva manoeuvre,5 bending posture,6 coughing or straining and these, in turn, lead to the clinical characteristics of variable proptosis, intermittent pain, and orbital haemorrhage.7,8
Observation is usually warranted for small lesions, but surgical intervention may be necessary in advanced cases: indications for surgical intervention include non-resolving episodes of thrombosis, severe disfiguring proptosis or displacement of the globe, and optic nerve compression.1–3 Surgery can be extremely difficult, as varices are very friable and intimately intermixed with normal orbital structures; there is also a significant risk of visual loss as a result of haemorrhage or optic nerve damage, the latter being generally caused by vascular compromise.9,10 The association of orbital venous anomalies with orbital wall defects provides a further source of surgical difficulty because of the close proximity of intracranial structures and the continuity with extraorbital or intracranial venous anomalies.
Case series
The orbital database, at Moorfields Eye Hospital, was used to identify patients with a clinical diagnosis of low pressure orbital varices and their orbital imaging (computed tomography and/or magnetic resonance image) was reviewed. Images were examined for evidence of orbital expansion, osseous defects of the orbit, nose or sinuses, and anomalies of the frontal lobes. Patients who had either orbital or intracranial surgery before the date of imaging were excluded from the investigation.
The clinical diagnosis of orbital varices was identified in 310 patients, and imaging was available for 223 patients (72%). Six patients with previous orbital or intracranial surgery were excluded and nine cases (4%) had associated anomalies of the neighbouring orbital walls (table 1).
Table 1 Characteristics of nine patients with orbital wall defects in association with orbital varices
Four cases (patients 1–4) were associated with "pitting" of the orbital wall secondary to orbital varices (fig 1A). Another three cases (patients 6–8) were associated with enlarged superior orbital fissure and two cases (patients 5 and 9) with multiple orbital roof "defects" (fig 1B). Orbital varices were present up to the dural space in two cases (patients 4 and 5), and involved the frontal lobe parenchyma in one case (patient 6; fig 1C, D).
Figure 1 (A) (Patient 1) Extensive left orbital varices (white arrows) causing orbital expansion, globe displacement, and "pitting" of the orbital roof and lamina papyracea (black arrows). (B) (Patient 5) Bilateral orbital varices associated with multiple defects, rather than pitting, of the orbital walls. (C) (Patient 6) Right orbital varices, with phleboliths, extending through the orbital apex into the middle cranial fossa (white arrows) and (D) associated with intracranial bone "pitting" and "defects" (black arrows). (E) Coronal and (F) axial CT scans of patient 2 with superonasal varices (white arrows) of right orbit in 1981. (G–J) Repeat coronal and axial CT scans in 2001 show significant enlargement of the bone defect with complete loss of mineralisation, and expansion of the frontal lobe meninges into the orbital wall defect (black arrow).
One patient (case 2) had thinning of the superonasal quadrant of the orbital wall, nasal orbital wall pitting, and a low ipsilateral cribriform plate, when first seen at age 21 in 1981 (fig 1E, F). On repeat imaging 20 years later (2001), this patient was noted to have developed proptosis, a defect in the superonasal wall of the orbit, and a new mid-line nasal encephalocele (fig 1I, J).
Comment
Fine cut (3 mm) orbital CT scans easily delineate varices and diagnostic phleboliths, which occur from thrombus formation,7 and provide an excellent natural contrast between brain, bone, and varix. The typical findings for varices include an ill defined multiloculated mass, with some patchy contrast enhancement, in communication with the neighbouring orbital circulation4–7; diffuse expansion of the orbital walls is well recognised in some cases, especially in childhood lesions.
Several factors may have biased the study population: many are symptomatic patients, having been referred from other ophthalmic units in consideration for surgical intervention. The apparent incidence of orbital wall defects (4%) in our series may, therefore, be a slight overestimate. In a minority of patients, orbital varices may be associated with orbital wall defects, and such defects may, eventually, lead to an encephalocele formation. Clinicians should be aware of these, apparently unreported, associations before embarking on surgical intervention for orbital varices.
References
Rootman J . Diseases of the orbit. A multidisciplinary approach. 2nd ed. Philadelphia: Lippincott Williams and Wilkins, 2002:517–22.
Beyer R , Levine MR, Sternberg I. Orbital varices: a surgical approach. Ophthal Plast Reconstr Surg 1985;1:205–10.
Wright JE, Sullivan TJ, Garner A, et al. Orbital venous anomalies. Ophthalmology 1997;104:905–13.
Rubin PAD, Remulla HD. Orbital venous anomalies demonstrated by spiral computed tomography. Ophthalmology 1997;104:1463–70.
Shields JA, Dolinskas C, Augsburger JJ, et al. Demonstration of orbital varix with computed tomography and Valsalva manoeuvre. Am J Ophthalmol 1984;97:108–10.
Cohen JA, Char DH, Norman D. Bilateral orbital varices associated with habitual bending. Arch Ophthalmol 1995;113:1360–2.
Bullock JD, Goldberg SH, Connelly PJ. Orbital varix thrombosis. Ophthalmology 1990;97:251–6.
Foroozan R , Shields CL, Shields JA, et al. Congenital orbital varices causing extreme neonatal proptosis. Am J Ophthalmol 2000;129:693–4.
Harris GJ, Sakol PJ, Bonavolonta G, et al. An analysis of thirty cases of orbital lymphangioma. Pathophysiologic considerations and management recommendations. Ophthalmology 1990;97:1583–92.
Weill A , Cognard C, Castaings L, et al. Embolization of an orbital varix after surgical exposure. Am J Neuroradiol 1998;19:921–3.