Physical examination showed no chemosis, vision loss or cranial nerve palsies.  Because of this the patient was brought back for repeat contrast enhanced CT of the orbits with Valsalva maneuver.  This study showed mild additional enlargement of the already prominent left superior ophthalmic vein and also of the right sided one (see below).  The combination of imaging and clinical findings was thought to be most compatible with orbital varices.  The patient opted for conservative management.

Orbital varices are hamartomas composed of slow flow, low pressure and thinned walled and distensible blood vessels.  As they communicate with the rest of the circulation, they enlarge with Valsava, bending or prone position, and coughing and straining.  They produce proptosis which may be painful and because they may bleed, their symptoms may become acutely exacerbated.  They may also erode adjacent bone.  Treatment is very difficult and is reserved for those with repeated hemorrhages, thrombosis, optic nerve compression and disfigurement.  Orbital vascular processes included in the differential diagnosis are carotid cavernous fistulas of both types and less likely, venous thrombosis.

In CC fistulas, the ipsilateral cavernous sinus may be enlarged particularly in the direct ones (see below).  Extra-ocular muscles may also be large and the retro-ocular fat may have a “dirty” appearance.  In most patients with direct CCFs, chemosis, decreased vision and cranial nerve palsies are present.  Acute thrombosis of the superior ophthalmic vein may present with symptoms that are similar to those of a direct CCF.  Indirect CCFs may have less acute symptoms and be clinically similar to varices.  The diagnosis is confirmed with catheter angiography as shown here.

Suggested readings:

YO Arat, ME Mawad, M Boniuk. Orbital Venous Malformations: Current Multidisciplinary Treatment Approach. Arch Ophthalmol 2004; 122: 1151 – 1158

N Islam, K Mireskandari, GE Rose. Orbital varices and orbital wall defects. Br J Ophthalmol 2004; 88: 1092 – 1093

A Weill, C Cognard, L Castaings, G Robert, J Moret. Embolization of an orbital varix after surgical exposure. AJNR Am. J. Neuroradiol. 1998; 19: 921 – 923

You may have heard that Propanolol is currently being used as treatment for some infantile hemangiomas of both types (RICH and NICH).  In this presentation prepared for our weekly case conference by Mr. Danilo Bernardo the results of such therapy are illustrated and discussed.

The authors state that “CT is still the method of choice for detecting intraocular calcium and investigating orbital pathologies”, but support this with articles from 1997 and 1999. At CHOP, we have virtually eliminated the use of CT in our patients presenting with suspected retinoblastoma, and for follow up of treated patients. Have we all just changed our approach and not documented it very well in the literature?

As fewer enucleations are performed, in favor of eye-sparing procedures, it is increasingly important that all of the orbital structures be accurately assessed for residual and recurrent disease. In the same setting as the orbit MRI, brain MRI can assess for intracranial lesions. The ophthalmologic examination can often be coordinated to precede or follow the MRI, with the same sedation or anesthesia, as most affected are young children.

Anyone out there still routinely using CT for retinoblastoma?

This patient had left central retinal artery occlusion and acute ischemic optic neuropathy. There is restricted diffusion in the anterior left optic nerve which is much more obvious than the mild perineural enhancement on coronal postcontrast images.