Dilated Superior Ophthalmic Vein: Clinical and Radiographic Features of 113 Cases.

PURPOSE: Dilated superior ophthalmic vein (SOV) is an uncommon radiographic finding. The authors review the presentation, etiology, radiography, and visual implications of 113 patients with dilated SOV. METHODS: An observational case series and multicenter retrospective chart review were conducted. There were 113 patients with a dilated SOV. Outcome measures included patient demographics, clinical features, radiographic findings, diagnosis, and treatment, and treatment outcomes were assessed. RESULTS: Cases included 75 women (66%) and 38 men (34%) with a mean age of 49 ± 24 years (range, 0.4-90 years). Diagnoses fell under 6 categories: vascular malformation (n = 92, 81%), venous thrombosis (n = 11, 10%), inflammatory (n = 6, 5%), traumatic hemorrhage (n = 2, 2%), lymphoproliferative (n = 1, 1%), and infectious (n = 1, 1%). Imaging modalities utilized included MRI (n = 98, 87%), digital subtraction angiography (n = 77, 68%), CT (n = 29, 26%), and ultrasonography (n = 4, 4%). Disease status at last follow up included no evidence of disease (n = 57, 50%), alive with persistent disease (n = 53, 47%), and expired from disease (n = 3, 3%). Treatment and management was tailored to the underlying disease process with a mean follow up of 18 months (range, 1 day to 180 months). Visual impairment observed at presentation and last follow up across all cases was 26% and 22%, respectively. CONCLUSION: Dilated SOV is a rare radiographic finding resulting from a wide spectrum of etiologies with clinical implications ranging from benign to sight- and life-threatening. Dilated SOV is most often found with dural-cavernous fistula or carotid-cavernous fistula, orbital or facial arteriovenous malformation, and venous thrombosis. Recognition of this finding and management of the underlying condition is critical.

Orbital fat decompression for thyroid eye disease: retrospective case review and criteria for optimal case selection.

PURPOSE: The purpose of this study is to identify the subgroups of thyroid eye disease (TED) patients most likely to benefit from orbital fat decompression. METHODS: This retrospective study reviews 217 orbits of 109 patients who underwent orbital fat decompression for proptosis secondary to thyroid eye disease. Charts were reviewed for demographic, radiographic, clinical, and surgical data. Three groups of patients were defined for the purposes of statistical analysis: those with proptosis secondary to expansion of the fat compartment (group I), those with proptosis secondary to enlargement of the extraocular muscles (group II), and those with proptosis secondary to enlargement of both fat and muscle (group III). RESULTS: Groups I and II, and those patients with greater preoperative proptosis and those with a history of radiation therapy were most likely to benefit from orbital fat decompression. However, even those in group III or with lesser proptosis appreciated significant benefit. CONCLUSIONS: While orbital fat decompression can and, at times, should be combined with bone decompression to treat proptosis resulting from thyroid eye disease, orbital fat decompression alone is associated with lower rates of surgical morbidity, and is especially effective for group I and II patients, those with greater preoperative proptosis, and those with a history of radiation.

Autofluorescence imaging and spectral-domain optical coherence tomography in incomplete congenital stationary night blindness and comparison with retinitis pigmentosa.

PURPOSE: To test the hypothesis that the evaluation of retinal structure can have diagnostic value in differentiating between incomplete congenital stationary night blindness (CSNB2) and retinitis pigmentosa (RP). To compare retinal thickness differences between patients with CSNB2 and myopic controls. DESIGN: Prospective cross-sectional study. METHODS: Ten eyes of 5 patients diagnosed with CSNB2 (4 X-linked recessive, 1 autosomal recessive) and 6 eyes of 3 patients with RP (2 autosomal dominant, 1 autosomal recessive) were evaluated with spectral-domain optical coherence tomography (SD OCT) and fundus autofluorescence (FAF). Diagnoses of CSNB2 and RP were confirmed by full-field electroretinography (ERG). Manual segmentation of retinal layers, aided by a computer program, was performed by 2 professional segmenters on SD OCT images of all CSNB2 patients and 4 age-similar, normal myopic controls. Seven patients were screened for mutations with congenital stationary night blindness and RP genotyping arrays. RESULTS: Patients with CSNB2 had specific findings on SD OCT and FAF that were distinct from those found in RP. CSNB2 patients showed qualitatively normal SD OCT results with preserved photoreceptor inner segment/outer segment junction, whereas this junction was lost in RP patients. In addition, CSNB2 patients had normal FAF images, whereas patients with RP demonstrated a ring of increased autofluorescence around the macula. On SD OCT segmentation, the inner and outer retinal layers of both X-linked recessive and autosomal recessive CSNB2 patients were thinner compared with those of normal myopic controls, with means generally outside of normal 95% confidence intervals. The only layers that demonstrated similar thickness between CSNB2 patients and the controls were the retinal nerve fiber layer and, temporal to the fovea, the combined outer segment layer and retinal pigment epithelium. A proband and his 2 affected brothers from a family segregating X-linked recessive CSNB2 had a mutation, p.R614X, in the gene encoding calcium channel, α 1F subunit. CONCLUSIONS: CSNB2 patients (X-linked recessive and autosomal recessive) had significantly thinner retinas than myopic controls. However, they demonstrated qualitatively normal SD OCT and FAF images, and therefore can be differentiated from RP patients with these techniques. Although ERG testing remains the gold standard for the diagnosis of these conditions, FAF and SD OCT systems are more widely available to community ophthalmologists, offer shorter acquisition times, and, unlike ERG, can be performed on the same day as the initial clinic visit. Therefore, as a supplement to ERG and genetic testing, we advocate the use of FAF and SD OCT in the examination of patients with CSNB2 and RP.

Incidence of visually significant pseudophakic macular edema after uneventful phacoemulsification in patients treated with nepafenac.

PURPOSE: To compare the incidence of visually significant pseudophakic macular edema after uneventful phacoemulsification in patients treated postoperatively with topical prednisolone and those treated with topical prednisolone and nepafenac 0.1% suspension (Nevanac). SETTING: Edward S. Harkness Eye Institute of Columbia University, New York, New York, USA. METHODS: This retrospective chart review was of consecutive patients who had phacoemulsification at a single institute and were given topical prednisolone alone or topical prednisolone and nepafenac to prevent cystoid macular edema. Data collection included preexisting ocular and systemic diseases, concurrent use of ocular and systemic medications, surgical technique, intraoperative and postoperative complications, follow-up visual and ocular assessments, and postoperative optical coherence tomography (OCT) assessment for macular edema. RESULTS: Postoperatively, 240 patients were treated with prednisolone and 210 patients, with prednisolone-nepafenac. Preoperatively, the 2 groups were demographically and clinically comparable in sex distribution (P = .8400), history of diabetes (P = .7267), hypertension or cardiac disease (P = .8690), and concurrent use of oral nonsteroidal anti-inflammatory drugs (P = .7303). Iris manipulation was done in 16 patients in the prednisolone-alone group and 10 patients in the prednisolone-nepafenac group (P = .3876). Capsule staining was done in 5 patients and 4 patients, respectively. All patients were followed for at least 1 month postoperatively. Visually significant pseudophakic macular edema was documented by OCT in 5 patients treated with prednisolone alone and in no patients treated with prednisolone and nepafenac (P = .0354). No significant intraoperative or postoperative complications were reported. CONCLUSION: Patients treated with topical prednisolone alone had a significantly higher incidence of visually significant pseudophakic macular edema after uneventful cataract surgery than those treated with topical prednisolone and nepafenac.