No abstract

The clinical manifestations of carotid cavernous fistula, an abnormal arteriovenous connection between the cavernous sinus and the carotid artery, can closely mimic the cardinal signs of Graves' ophthalmopathy, an inflammatory disorder of the orbit usually associated with autoimmune thyroid disease. Therefore, carotid cavernous fistulas are generally considered in the differential diagnosis of Graves' ophthalmopathy, especially when the eye involvement is unilateral or asymmetric, and there is the need for exclusion of rarer etiologies of orbital disease. This is the first report of the simultaneous occurrence of Graves' ophthalmopathy and carotid cavernous fistula. The patient was a 67-yr-old woman who presented with a history of Graves' disease with mild bilateral ophthalmopathy treated with radioiodine following a 10-yr therapy with methimazole; after radioiodine treatment, ophthalmopathy deteriorated. At the time of our initial clinical evaluation the ocular involvement of the patient was symmetric, and no evidence of any associated condition was found. However, the response of eye disease to corticosteroid treatment was markedly unequal, resulting in evident asymmetry. This prompted a reconsideration of the diagnosis and a new evaluation of the patient with sensitive techniques, leading to the further diagnosis of carotid cavernous fistula.

To evaluate technetium Tc 99m (99m Tc) red blood cell scintigraphy as a diagnostic tool for orbital cavernous hemangioma and to differentiate between orbital masses on the basis of their vascularization. Methods: We performed 99m Tc red blood cell scintigraphy on 23 patients (8 female and 15 male; mean age, 47 years) affected by an orbital mass previously revealed with computed tomography (CT) and magnetic resonance imaging (MRI) and suggesting cavernous hemangioma. In our diagnosis, we considered the orbital increase delayed uptake with the typical scintigraphic pattern known as perfusion blood pool mismatch. The patients underwent biopsy or surgical treatment with transconjunctival cryosurgical extraction when possible. Results: Single-photon emission tomography (SPET) showed intense focal uptake in the orbit corresponding to radiologic findings in 11 patients who underwent surgical treatment and pathologic evaluation (9 cavernous hemangiomas, 1 hemangiopericytoma, and 1 lymphangioma). Clinical or histologic examination of the remaining 22 patients revealed the presence of 5 lymphoid pseudotumors, 2 lymphomas, 2 pleomorphic adenomas of the lacrimal gland, 1 astrocytoma, 1 ophthalmic vein thrombosis, and 1 orbital varix. Conclusions: The confirmation of the preoperative diagnosis by 99m Tc red blood cell scintigraphy shows that this technique is a reliable tool for differentiating cavernous hemangiomas from other orbital masses (sensitivity,100%; specificity,86%) when ultrasound, CT, and MRI are not diagnostic. Unfortunately, 99m Tc red blood cell scintigraphy results were positive in 1 patient with hemangiopericytoma and 1 patient with lymphangioma, which showed increased uptake in the lesion on SPET images because of the vascular nature of these tumors. Therefore, in these cases, the SPET images have to be integrated with data regarding clinical preoperative evaluation and CT scans or MRI studies. On the basis of our study, a complete diagnostic picture, CT scans or MRI studies, and scintigraphic patterns can establish the preoperative diagnosis of vascular orbital tumors such as cavernous hemangioma, adult-type lymphangioma, and hemangiopericytoma.

The authors describe an unusual case of orbital inflammatory myofibroblastic tumor (IMT) in a 17-year-old patient who presented with a painful exophthalmos of the left eye. After complete surgical excision, the mass was diagnosed as an IMT based on morphological and immunohistochemical features. No tumor recurrence was evident during 28-month follow-up. The authors discuss histopathological and immunohistochemical characteristics and review the literature of orbital IMT.

It is evident that by not excising the tumour in its entirety it is therefore possible to preserve the underlying rectus muscles and not interfere with ocular motility. Subsequent radiotherapy and chemotherapy helped to destroy any residual tumour. Alternatively, surgical excision of the mass alone could lead to recurrence of the tumour after some months or years.