Giovanni Spinucci

Uveitis in childhood: an Italian clinical and epidemiological study.

Purpose: To investigate demographics, causes, features, and visual outcomes of pediatric uveitis. Methods: Retrospective cohort study including 257 patients younger than 16 years. Results: Mean age at onset of uveitis was 8.54 ± 3.98 years; 54.5% of the patients were girls. Anterior uveitis occurred in 47.8%, intermediate in 19.4%, posterior in 24.9%, panuveitis in 7.8%. Ocular involvement was bilateral in 67.8%. Infectious uveitis represented 31% of all cases. Causes of severe visual loss were cataract, macular scars, macular edema/maculopathy, and secondary glaucoma. At follow-up 79.3% of eyes maintained a visual acuity between 20/32 and 20/20. Conclusions: Uveitis is rarer in children than in adults. Patients with anterior uveitis comprised the largest group. Posterior uveitis in the pediatric population has a lower incidence than some decades ago. Visual prognosis of pediatric uveitis is improving, owing to an earlier diagnosis and a correct treatment.

Spectral-Domain Optical Coherence Tomography in Uveitic Macular Edema: Morphological Features and Prognostic Factors

Aim: To assess the morphological characteristics of uveitic macular edema studied with Spectralis optical coherence tomography (OCT) and to investigate the correlation between the tomographic features and visual acuity. Methods: 71 eyes of 55 patients underwent examination with Spectralis OCT (Heidelberg Engineering, Germany). Data was correlated with logMAR best-corrected visual acuity (BCVA). Results: Two morphological patterns were observed: cystoid macular edema (CME) in 69% and diffuse macular edema in 31% of eyes. BCVA was 0.2 in CME, 0.1 in diffuse edema (p = 0.008). Foveal thickness was 413.4 ± 212 µm in CME, 311.27 ± 53 µm in diffuse edema (p = 0.03). BVCA was 0.3 in eyes with serous retinal detachment (SRD), 0.2 in eyes without SRD (p = 0.02). BCVA was 0.4 in eyes with inner segment/outer segment (IS/OS) disruption, 0.1 in eyes with integrity of the IS/OS junction (p = 0.01). Conclusions: BCVA is negatively correlated with cystoid pattern, foveal thickening and SRD. Disruption of the IS/OS junction is associated with poor vision in uveitic macular edema.

Intermediate Uveitis in a Pediatric Italian Population

Purpose: To investigate clinical data, outcome, and treatment of intermediate uveitis in children. Methods: Retrospective cohort study, including 116 children affected by intermediate uveitis. Results: Sixty-two percent of patients were males. Ocular involvement was bilateral in 84%. Mean age at uveitis onset was 10.3 ± 3.6 years and 12.2 ± 7 years at first visit. Ninety-seven percent were classified as having idiopathic uveitis. The incidence of ocular complications during follow-up was 0.131/eye-year. Macular edema or a marked vitreous haze at presentation may be risks factors for incident visual loss. The most frequent causes of visual loss were macular edema (64%) and cataract (21%). Conclusions: Most cases are idiopathic (pars planitis); only few children presented associated systemic disease. Macular edema and severe vitritis at presentation are associated with increased risk of visual impairment. Cystoid macular edema is the main cause of visual impairment.

Retinal complications of juvenile idiopathic arthritis-related uveitis: a microperimetry and optical coherence tomography study.

Purpose: To evaluate ocular complications of juvenile idiopathic arthritis (JIA)-related uveitis; to study macular morphology and sensitivity. Methods: Retrospective chart review of 67 patients. Fourteen consecutive patients (24 eyes) observed from January to December 2008 were submitted to optical coherence tomography study (OCT) and microperimetry MP-1 examination. Results: Most frequent complications were posterior synechiae (63.0%), band keratopathy (53.9%), and cataract (31.8%). Posterior complications were documented in 37.0% of eyes: 13.8% macular edema, 12.0% papillitis, 6.8% epiretinal membranes, 2.5% retinal vasculitis, 1.7% retinal detachment. At OCT examination 25.0% of eyes presented macular edema; macular sensitivity by MP-1 was ≤16.5 dB in 25.0% of eyes. Conclusions: Visual prognosis is good despite ocular complications. Incidence of macular edema seems to be higher when using OCT. Microperimetry may represent a useful tool in detecting alteration in retinal sensitivity that may supplement visual acuity in the follow-up of macular edema.

Central serous chorioretinopathy as a presenting symptom of endogenous Cushing syndrome: a case report

Purpose To report a case of Cushing syndrome due to adrenocortical adenoma revealed by central serous chorioretinopathy. Methods A 45-year-old man presented with blurred vision and metamorphopsia in the left eye. He reported few episodes of high blood pressure in the last 3 months. Results Visual acuity was 20/40 in the left eye. Fundus oculi examination revealed central serous chorioretinopathy in the left eye. Grade 1 hypertension was found. Increased serum and urinary levels of Cortisol and reduced serum levels of ACTH were observed. Diagnosis of Cushing syndrome was made. Computed tomography scan revealed a right adrenal mass that was surgically removed; histologic examination showed an adrenocortical adenoma. Three months after surgical treatment, visual acuity improved to 20/20 and central serous chorioretinopathy completely resolved. Conclusions Central serous chorioretinopathy may be the presenting symptom of Cushing syndrome in a patient with adrenocortical adenoma.

A branch retinal artery occlusion in a patient with Gaucher disease

Gaucher disease (GD) is a rare familial autosomal recessive disorder of lipid metabolism, resulting in an accumulation of abnormal glucocerebrosides in the reticulo-endothelial system. Patients with GD may present with hepatosplenomegaly, anemia, thrombocytopenia, and destructive bone disease. An enzyme replacement therapy with intravenous infusions of glycosylceramidase has been successfully proposed for treating the visceral manifestations. Gaucher disease can be divided into three subtypes: non-neuronopathic (type 1) which is the most common, acute neuronopathic (type 2), and subacute neuronopathic (type 3) [1]. Several studies have reported ocular manifestations such as strabismus, conjunctival pterygia, corneal opacities, vitreous opacities and retinal involvement [2–7]. To our knowledge, this is the first reported case of Gaucher disease complicated by branch retinal artery occlusion.

Reproducibility of retinal thickness measurements in eyes with uveitic macular oedema using Spectralis optical coherence tomography

and invariably fatal. In most cases, the first symptoms of CJD consist of rapidly progressive dementia, leading to memory loss, personality changes and hallucinations accompanied by physical changes such as speech impairment, myoclonus, balance and coordination dysfunction (ataxia), changes in gait, rigid posture, and seizures. The duration of the disease varies greatly, but sCJD can be fatal within months or even weeks (Ladogana et al. 2005; WHONovember 2002). However, for a small subset of patients with CJD, the first symptoms may be purely visual impairment. A disease course of CJD with the leading signs of a visual disorder has been referred to as the (Heidenhein 1992). The Heidenhain variant can manifest in different ways: disturbed perception of structures ⁄ colours, problems in reading, optical hallucinations, cortical blindness and optical agnosia (Anton Syndrome) (Kropp et al. 1999). Visual disturbances misleadingly ascribed to coexisting cataracts might be the leading symptom in patients with CJD.

Severe Macular Edema in Patients with Juvenile Idiopathic Arthritis-Related Uveitis

Purpose. To report the onset of severe macular edema in adolescent female patients affected by juvenile idiopathic arthritis (JIA). Methods. Four female patients affected by JIA-related chronic anterior uveitis (CAU), complicated by severe macular edema, were retrospectively analyzed. Macular area was evaluated by fluorescein angiography and optical coherence tomography (OCT). Results. CAU was bilateral in three patients. Mean age of uveitis and arthritis onset was, respectively, 4.5 ± 1.7 years and 6.0 ± 3.9 years. All patients underwent cataract extraction surgery. Despite ocular inflammation being controlled by topical/systemic therapy, during adolescence (mean age of appearance/diagnosis: 12.7 ± 3.9 years) patients developed severe unilateral macular edema. OCT revealed massive macular thickening (range from 550 μm to 1214 μm). Conclusions. Macular edema appeared in female adolescent patients in eyes with long-dating CAU submitted to cataract surgery. In such patients, in presence of age-related microvascular changes due to the enhancer effect of sex hormones, cataract extraction should be a factor triggering the retinal complication.

Optical coherence tomography of retinal astrocytic hamartomas in a 4-year-old boy affected by tuberous sclerosis.

Tuberous sclerosis is an hereditary disease characterized by disseminated hamartomas involving the central nervous system, skin, kidney and eye. Astrocytic hamartomas are typical retinal lesions. We report a case of a child affected by tuberous sclerosis in which spectralis optical coherence tomography (OCT) provided highresolution images of astrocytic hamartomas. Astrocytic hamartomas are typical retinal lesions in tuberous sclerosis [1] and may be multifocal and bilateral. They are benign tumours of the nerve fiber layer of the retina and specifically involve the enlargement and proliferation of astrocytes [2]. They can arise from any location in the retina, from the optic disc to the extreme periphery and typically remain stable throughout life.