Faculty Bibliography

Soft-tissue glomus tumors (or glomangiomas) are unrelated to neuroendocrine paragangliomas (glomus tympanicum, jugulare, and vagale). The authors present the first reported case of an orbital soft-tissue glomus tumor in a child. An 8-year-old girl developed rapidly progressive right-eye blindness, proptosis, and a sixth cranial nerve palsy. Magnetic resonance imaging demonstrated a homogeneously enhancing lesion extending from the right orbit through the superior orbital fissure to the cavernous sinus and middle cranial fossa. A biopsy specimen demonstrated the lesion to be a soft-tissue glomus tumor. Following angiography and embolization, a gross-total resection of the tumor was achieved. The patient was treated with adjuvant proton-beam radiotherapy. At 24 months follow-up her proptosis and sixth cranial nerve palsy had resolved and there was no evidence of tumor recurrence

PURPOSE: To present 3 cases of orbital compression syndrome caused by infarction of the greater wing of the sphenoid in patients with sickle cell disease. METHODS: Case report and review of the literature. RESULTS: Three patients with sickle cell disease (2 males aged 22 and 16 years, and a 10-year-old girl) who presented with proptosis, limited ocular motility, and chemosis were found to have an infarction of the marrow space of the greater wing of the sphenoid that produced an orbital subperiosteal hemorrhage and exudate demonstrated on MRI. Two patients suffered compressive optic neuropathy; both patients recovered normal optic nerve function. Orbital edema resolved within 48 hours of receiving 1 g methylprednosolone daily. The third patient had normal optic nerve function and his orbital edema improved with methylprednisolone 250 mg/day and intravenous Kefzol over 3 days. In the literature, there are 27 similar cases; 5 were treated surgically and the remainder were managed medically. CONCLUSIONS: Patients with sickle cell disease are at risk for orbital compression syndrome secondary to orbital bone infarction, in the setting of vaso-occlusive crises. This diagnosis should be considered when a patient with sickle cell disease presents with headache, proptosis, decreased motility, and/or optic nerve compromise

OBJECTIVES: We studied the efficacy and safety of image-guided balanced orbital decompression for Graves' orbitopathy. METHODS: The data of 24 patients (45 orbits) were reviewed for demographics, ophthalmologic outcomes, and complications in regard to image-guided (18 orbits) versus non-image-guided surgery (27 orbits). RESULTS: Overall, all patients had a reduction in proptosis (mean reduction, 6.2 mm in proptosis) as measured by Hertel exophthalmometry. There was improvement in the visual acuity of all 12 orbits with preoperative acuity of 20/40 or worse and either complete resolution (38%) or improvement (62%) in the 16 orbits with optic neuropathy. These measures reached statistical significance. Despite subjective improvement in surgeon confidence, the use of image guidance did not result in a statistically significant difference in postoperative ophthalmologic outcomes. Medical and sinonasal complications were experienced by 11.1% and 18.5% of patients who underwent image-guided and non-image-guided orbital decompression, respectively. CONCLUSIONS: Image guidance may be a useful adjunct to balanced orbital decompression for Graves' orbitopathy, but it was not associated with a statistically significant improvement in outcomes in this study

Thyroid Eye Disease (TED, Graves ophthalmopathy, thyroid ophthalmopathy) is the most common cause of orbital inflammation and proptosis in adults. There is no agreement on its management although corticosteroids and external beam orbital radiation (XRT) have traditionally been believed to provide benefit in active inflammation. Our review of the published literature in English disclosed an overall corticosteroid-mediated treatment response of 66.9% in a total of 834 treated patients who had moderate or severe TED. Intravenous corticosteroids used in repeated weekly pulses were more effective (overall favorable response = 74.6%, n = 177) and had fewer side effects than daily oral corticosteroids (overall favorable response = 55.5%, n = 265). A combination of corticosteroid and radiation therapy seemed to be more effective than corticosteroids alone. Our conclusions are tempered by a notable lack of standardization within and between study designs, treatment protocols, and outcome measures. Accordingly, the North American Neuro-Ophthalmology Society (NANOS), American Society of Ophthalmic Plastic and Reconstructive Surgery (ASOPRS) and the Orbital Society, in conjunction with Neuro-Ophthalmology Research and Development Consortium (NORDIC), will investigate the design and funding of a multi-center controlled trial

A 78-year-old man with a history of melanoma presented with a 2-week history of diplopia, pain, and intermittent blurriness in his right eye. Imaging showed a multicystic mass within the right lateral rectus muscle that was biopsy-proven metastatic melanoma. To our knowledge, this is the first case report of orbital metastasis from melanoma presenting as a multicystic mass intrinsic to the extraocular muscle with layering fluid-fluid levels

A 63-year-old woman presented subacutely with signs of orbital apex and cavernous sinus disease in the setting of widespread, untreated metastatic carcinoma of the uterus. MRI revealed a destructive lesion of the left sphenoid wing with compression of the orbital apex and with possible extension in the cavernous sinus. The underlying diagnosis was confirmed with repeat endometrial biopsy, revealing well-differentiated endometrial adenocarcinoma. Given the supporting clinical evidence for metastatic disease and the radiologic characteristics of the lesion, the decision was made to not perform a confirming orbital biopsy

Inflammatory myofibroblastic tumour (IMT) is a rare low-grade lesion in the spectrum of myofibroblastic tumours. Systemic IMT is a well-described entity, but orbital IMT is extremely uncommon. These tumours are detected incidentally or through site-specific complaints. Their presentation may clinically and radiologically mimic that of a malignant neoplasm, thus necessitating a biopsy. Even with reports of persistence, local recurrence and malignant transformation the overall prognosis remains favourable

PURPOSE: To describe prominent premalar and cheek swelling as a previously undescribed clinical feature of thyroid-associated orbitopathy (TAO). DESIGN: Retrospective interventional case series. METHODS: A retrospective case review of patients with prominent premalar and/or cheek swelling and TAO was undertaken. All patients who presented from March 2002 to February 2005 with complaints of premalar and/or cheek swelling in TAO were analyzed. RESULTS: Six female patients between the ages of 28 and 66 years (average, 44.2 years) who had complaints of prominent premalar and/or cheek swelling and TAO were included in the study, among 326 new patients with TAO (incidence: 1.84%). Five of the 6 patients had Graves hyperthyroidism. One initially had Hashimoto thyroiditis, which converted to Graves hyperthyroidism. In all cases, TAO preceded the thyroid disease or developed simultaneously (average time from TAO to thyroid disease was 3.0 months). No patient had received corticosteroids before premalar and/or cheek swelling. The premalar and/or cheek swelling was bilateral in all cases, but 2 of 6 were asymmetric. No diurnal fluctuation or tenderness in premalar and/or cheek swelling was noted. All improved incompletely over several months. Brow, eyelid swelling, and orbital-fat hypertrophy on radiologic examination was coincidentally noted in 5 of 6 cases. Pretibial myxedema was noted in one case. One case was associated with another autoimmune disease (rheumatoid arthritis). CONCLUSIONS: Prominent premalar and cheek swelling should be considered among the clinical features of TAO. The true incidence will become apparent as we question and examine patients more carefully regarding this entity and as we review premorbid photographs

PURPOSE: To descirbe a series of patients in which Thyroid Associated Orbitopathy (TAO) occurred after periocular surgery. METHODS: A retrospective case review of patients who developed TAO in close temporal association with periocular surgical interventions and presented at the orbital clinic from 1997 to 2004. History of previous thyroid abnormality and the lack of TAO signs and symptoms before surgery were reviewed and analyzed. RESULTS: Nine patients that developed TAO in association with periocular surgery were identified. All were women with an average age of 59.3years. (range: 45-75 years). The patients divided into two groups. Group 1 consisted of four patients who had previously been diagnosed with Graves' hyperthyroidism (GH). They ranged in age from 48 to 75 years (average: 58.8 years). The diagnosis of GH had been made an average of 50.5 months (range: 12-96 months) before presentation with TAO. Group 2 consisted of five patients who had no previous history of thyroid abnormality. They ranged in age from 45 to 74 years (average: 60.2 years). No patients had any signs or symptoms of TAO before their recent presentation. CONCLUSIONS: Periocular surgery may lead to local inflammatory events that may contribute to the instigation of TAO in predisposed individuals

A 33-year-old woman received a conjunctival laceration after accidental contact with the flexible outlet tip of a tank of compressed helium while filling balloons. The gas discharged during the contact, blowing compressed helium into her right orbit, with intracranial extension. The patient was asymptomatic, except for a transient headache. She was treated with prophylactic antibiotics and observed overnight, then discharged without complication. A literature search reveals that the usual outcome for this mechanism of injury is favorable