Familial amyloidotic polyneuropathy (FAP) is a hereditary disease which may present a wide range of ocular manifestations. Glaucoma is amongst the most serious complications of FAP. We report the results of ultrastructural study of the trabecular meshwork in a glaucomatous patient with the Portuguese form of FAP. This study showed that there was marked anatomical disruption of the uveoscleral, cornoscleral meshworks and juxtacanalicular tissue characterized by (a) accumulation of amyloid fibrils in the intertrabecular spaces; (b) degeneration of the endothelial cells; (c) homogeneous and/or multilayered plaques of basement membrane-like material loading the intertrabecular spaces or protruding to the lumen of the Schlemm's canal; and (d) degeneration of unmyelinated nerve fibres. These morphological changes and an analysis of the literature suggest that mechanical and neuropathic events can be co-existing factors which enhance the resistance to aqueous humour outflow, leading to increased intraocular pressure and glaucoma in the Portuguese form of FAP.

PurposeTo report a case of severe, acute ocular hypertension in a 6-year-old child, 7 days after initiating treatment with oral prednisolone, due to nephrotic syndrome.MethodsA 6-year-old female Caucasian child was diagnosed with nephrotic syndrome and treated with oral prednisolone (60 mg/day). Seven days later the child initiated complaints of headache, vomiting, ocular pain, and photophobia. Ophthalmologic examination revealed a severely increased intraocular pressure (IOP) of 52 mmHg in the right eye and 56 mmHg in the left eye. Anterior segment morphology was evaluated with ultrasound biomicroscopy. Optic disc status was evaluated by disc photography, kinetic perimetry, and optical coherence tomography.ResultsTreatment was initiated with latanoprost, brimonidine, and the fixed association of timolol and dorzolamide. At each follow-up examination, progressively better control of IOP was obtained. Simultaneous with corticosteroid dosage decrease we were able to reduce antiglaucomatous medication while maintaining IOP under control. Ultrasound biomicroscopy revealed an open angle with normal anterior segment echographic findings. Perimetric evaluation revealed normal visual fields in both eyes. Four months after presentation, steroid treatment had been completed and IOP was 10 mmHg in both eyes without any antiglaucomatous medication. Optical coherence tomography revealed normal retinal nerve fiber layer thickness in all peripapillary sectors.ConclusionsSystemic steroid treatment can cause a severe, acute increase in IOP in children. Children undergoing steroid treatment should have routine ophthalmologic examinations during treatment duration. Prompt antiglaucomatous treatment prevents retinal nerve fiber layer damage and visual acuity loss.

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ObjectiveTo compare a fixed combination of 0.03% bimatoprost and 0.5% timolol (BTFC) with latanoprost monotherapy (LM) in treatment-naïve patients with open-angle glaucoma (OAG) and risk factors for glaucomatous progression.MethodsPatients were enrolled at 15 sites in Spain and Portugal, and were randomized 1:1 to BTFC or LM. Patients instilled one drop of medication once per day at 8 pm for 12 weeks. The primary outcome was change in intraocular pressure (IOP) at 12 weeks.ResultsOf 81 patients enrolled, 43 were randomized to BTFC and 38 to LM. Mean (SD) change in IOP from baseline to 12 weeks was significantly greater for BTFC than for LM: −13.5 mmHg (4.48) versus −11.4 mmHg (3.19), respectively (P=0.003). Similarly, at 12 weeks, significantly more BTFC patients than LM patients had IOP reductions of ≥40% (74.4% versus 47.4%, P=0.015) or ≥50% (46.5% versus 15.8%, P=0.003). Adverse events were more frequent with BTFC than with LM (33 versus 13 events), but most were mild in severity. The only serious adverse event (colon cancer) was adjudged unrelated to the study medication.ConclusionBTFC was effective and well tolerated in treatment-naïve patients with OAG at high risk of progression.

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