Multifocal infantile hemangioma of the eyelid causing amblyopia requiring surgical resection.

Cutaneous vascular lesions in the pediatric population are rare and may represent a variety of localized and systemic diagnoses with diverse treatment protocols. We present a unique case of an infant with multiple cutaneous vascular lesions, initially diagnosed as congenital disseminated pyogenic granuloma based on histopathologic findings and later diagnosed as multifocal infantile hemangioma with extracutaneous hepatic involvement. The largest vascular lesion in our patient was on the left upper eyelid, which failed medical treatment and ultimately required surgical excision to prevent amblyopia progression.

In Vivo Laser Confocal Microscopy Using the HRT-Rostock Cornea Module: Diversity and Diagnostic Implications in Patients with Uveitis.

PURPOSE: Keratic precipitates (KP) are a common feature of uveitis. We prospectively examined KP with the Heidelberg Retinal Tomograph II confocal laser scanning microscope and Rostock Corneal Module (HRT-RCM) to explore their diagnostic implications. METHODS: Prospective, observational, multicenter study. HRT-RCM images were classified by two masked observers. RESULTS: 120 scans on 120 eyes from 110 subjects were included. The majority (N = 93) had non-infectious uveitis. Sixty eyes had active disease at scanning. Eight KP morphologies were defined. Agreement between the two masked graders was high (Kappa value across all categories = 0.81). Cluster and nodular KP were associated with active infectious uveitis (p < 0.01): patients with cluster KP (odds ratio [OR] = 3.03, 95% confidence interval [CI]: 1.43, 6.45) and nodular KP (OR = 3.89, 95% CI: 1.42, 10.65) were more likely to have infectious uveitis than those without. CONCLUSIONS: Laser confocal microscopy of KP may have a role in determining between infectious and non-infectious uveitis.

Antineutrophil cytoplasmic antibody-associated active scleritis.

OBJECTIVE: To determine whether antineutrophil cytoplasmic antibody (ANCA) testing provides prognostic information in evaluating scleritis. METHODS: Retrospective medical record review of patients evaluated at a tertiary care center from January 1, 1995, to June 30, 2006, was performed to compare clinical features, treatments, and associated systemic disease in patients who test positive for ANCA vs patients whose ANCA tests are negative. RESULTS: Among 78 patients identified, 14 tested positive for ANCA. Patients with positive ANCA test results were more likely to have an associated systemic disorder (10 of 14 or 71%) than were patients who tested negative for ANCA (26 of 64 or 41%; P = .04), and the disorder was more likely to have been diagnosed as a result of scleritis work-up (2 of 10 or 20% vs 19 of 26 or 73%; P = .007). Patients with positive ANCA test results had significantly more ocular complications (21 of 14 or 86% vs 20 of 64 or 31%; P < .001), including keratopathy (5 of 14 or 36% vs 6 of 64 or 9%; P = .02), visual acuity of 20/50 or worse (8 of 14 or 57% vs 11 of 64 or 17%; P = .001), and vascular pannus (3 of 14 or 21% vs 1 of 64 or 2%; P = .02). Aggressive therapy, such as chronic systemic corticosteroids (9 of 14 or 64% vs 9 of 64 or 14%; P < .001) and alkylator therapy (8 of 14 or 57% vs 7 of 64 or 11%; P < .001), was more likely to be recommended for patients who tested positive for ANCA. CONCLUSIONS: A substantial subset of patients with scleritis are also positive for ANCA. These patients are more likely to have severe ocular disease and undiagnosed primary vasculitic disease, thereby requiring more aggressive therapy. An ANCA test may be useful in the evaluation and treatment of patients with scleritis.

Primary sclerosing cholangitis and uveitis: a case series.

PURPOSE: To report the cases of six patients with primary sclerosing cholangitis and uveitis. DESIGN: Retrospective observational case series. METHODS: Two index cases were identified from the Uveitis Clinic of the Casey Eye Institute, Oregon Health and Science University and another from the Portland area. Additional cases were identified through a web-based survey of members of the American Uveitis Society. RESULTS: Three males and three females were identified. Five of six patients were diagnosed with uveitis prior to primary sclerosing cholangitis. Four had unilateral eye involvement. Five had insidious onset. All had persistent duration. Clinical course was chronic for three, recurrent for two, and chronic-recurrent in one. Vitreous inflammation was prominent in five. CONCLUSIONS: Although the number of patients who were studied is limited, there appears to be an association between primary sclerosing cholangitis and uveitis. Further studies are required to confirm such an association.

Intravital microscopy of leukocyte-endothelial dynamics using the Heidelberg confocal laser microscope in scleritis and allergic conjunctivitis.

PURPOSE: To examine leukocyte-endothelial cell rolling and arrest in human ocular vessels overlying sites of inflammation in various ocular inflammatory diseases in comparison to normal controls using the Heidelberg confocal laser microscope, which provides images with greater clarity and resolution than the tandem scanning microscope that uses white light. METHODS: Healthy controls (n=8) and patients with active anterior scleritis (n=7) or allergic eye disease (n=4) were scanned using the Heidelberg confocal laser microscope (HRT II) with the Rostock cornea module attachment for a minimum of 5 min at a depth of 45-120 microm from the conjunctival epithelial surface. RESULTS: There was a marked increase in the number of rolling leukocytes in scleritis patients (534+/-119 cells per mm2/min) versus controls (6+/-6 cells per mm2/min; p=0.0002) or allergic patients (59+/-44 cells per mm2/min; p=0.009). No statistically significant increase was seen in allergic patients compared to controls (p=0.059). A similar pattern was seen in the number of arrested leukocytes in patients with scleritis (56+/-23 cells per mm2) in comparison to either those with allergic eye disease or controls (each=0 cells per mm2; p=0.02). CONCLUSIONS: Patients with scleritis have a significantly increased number of rolling and arrested leukocytes in superficial ocular vessels in comparison to patients with mild allergic conjunctivitis and controls. The image quality with this microscope is superior to prior studies with a scanning microscope.