Faculty Bibliography

Radioimmunotherapy with yttrium-90-ibritumomab tiuxetan may result in hemorrhagic tumor necrosis with acute orbital and intracranial inflammation

PURPOSE: To evaluate the relationship between levator excursion and both eyelid lag and lagophthalmos in thyroid eye disease. METHODS: We retrospectively reviewed 104 eyelids of 52 thyroid eye disease patients over a 9-month interval by measuring levator function (mm), eyelid lag (0-4+) and lagophthalmos (mm). RESULTS: Lower levator excursion is associated with higher eyelid lag scores (p < 0.001) and with greater degrees of lagophthalmos (p < 0.001). Both associations were upheld after adjustment for upper eyelid margin reflex distance and Hertel exophthalmometry (p < 0.0001). For every 1-mm decrease in levator function, eyelid lag score increases on average by 0.29 and lagophthalmos increases on average by 0.23 mm. CONCLUSIONS: Diminished levator excursion is associated with increasing levels of eyelid lag and lagophthalmos. Levator excursion is an important clinical measurement in thyroid eye disease patients and may replace eyelid lag grading and lagophthalmos as a more accurate indicator of eyelid retraction in thyroid eye disease

OBJECTIVE: To identify components of a provisional clinical response index for thyroid eye disease using a modified Delphi technique. METHODS: The International Thyroid Eye Disease Society conducted a structured, 3-round Delphi exercise establishing consensus for a core set of measures for clinical trials in thyroid eye disease. The steering committee discussed the results in a face-to-face meeting (nominal group technique) and evaluated each criterion with respect to its feasibility, reliability, redundancy, and validity. Redundant measures were consolidated or excluded. RESULTS: Criteria were parsed into 11 domains for the Delphi surveys. Eighty-four respondents participated in the Delphi 1 survey, providing 220 unique items. Ninety-two members (100% of the respondents from Delphi 1 plus 8 new participants) responded in Delphi 2 and rated the same 220 items. Sixty-four members (76% of participants) rated 153 criteria in Delphi 3 (67 criteria were excluded because of redundancy). Criteria with a mean greater than 6 (1 = least appropriate to 9 = most appropriate) were further evaluated by the nominal group technique and provisional core measures were chosen. CONCLUSIONS: Using a Delphi exercise, we developed provisional core measures for assessing disease activity and severity in clinical trials of therapies for thyroid eye disease. These measures will be iteratively refined for use in multicenter clinical trials

A 28-year-old woman presented with a 2-week history of right upper eyelid swelling and intermittent frontal headaches. CT demonstrated an ill-defined superior right orbital mass with adjacent right frontal bone erosion and undeveloped frontal sinuses. The orbital biopsy revealed tissue strongly positive for CD1a and S100, diagnostic of Langerhans cell histiocytosis. The systemic workup was negative for multifocal lesions and for diabetes insipidus. In addition to subtotal resection, the patient was treated with a 6-month course of oral prednisone and intravenous vinblastine

Optic nerve glioma has been a topic of great interest and controversy, particularly with respect to its tendency to invade the optic chiasm. This case report demonstrates histologically verified optic nerve glioma in an otherwise healthy 5-year-old girl. MRI revealed sequential prechiasmal, chiasmal, and postchiasmal disease during the untreated course of 18 months. The authors also discuss the results obtained with a specific immunohistochemical technique, the mindbomb homolog 1 (MIB-1) labeling index, and speculate that this information, if known early in the course of disease, might influence early surgical decision-making

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