OBJECTIVE To describe the progression of ocular changes in patients with mucopolysaccharidosis I receiving enzyme replacement therapy. METHODS Three male and five female patients with mucopolysaccharidosis I were followed up for 4 years while undergoing enzyme replacement therapy with alpha-l-iduronidase (Aldurazyme). Visual acuity, corneal clouding, intraocular pressure, ophthalmoscopy, and ...

MANY of the stationary corneal opacities causing diminution of vision are central, avascular, and superficial, and are surrounded by clear cornea or have a neighbouring peripheral clear corneal area of 4 x 4-5 mm. Donor homogenous corneal grafts are not always available, but a rectangular lamellar autogenous corneal. graft can be dissected and turned 1800 so that the clear lamellae are brought ...

Macular corneal dystrophy is an inherited corneal disease characterized by corneal opacities resulting from intra- and extracellular deposits within the corneal stroma. Several monoclonal antibodies developed against antigens of corneal fibroblasts were screened for their reactivity with these abnormal deposits in corneas with macular dystrophy using an indirect peroxidase-conjugated immunostai...

The purpose of this case report is to correlate clinical features of granular dystrophy using 820 nm optical coherence tomography (OCT) in order to determine the depth of intrastromal corneal deposits. We report a case of a 38-year-old man with granular dystrophy. After complete ophthalmologic examination the patient was submitted to OCT, using the Stratus OCT III, originally proposed for study...

REFERENCES 1. Groden LR, Arentsen JJ. Ipsilateral rotating autokeratoplasty. Ann Ophthalmology 1983;15:899e901. 2. Jonas J, Rank R, Budde W. Autologous ipsilateral rotating penetrating keratoplasty. Am J Ophthalmol 2001;131:427e30. 3. Lee J, Winokur J, Hallak J, et al. Femtosecond dovetail penetrating keratoplasty: surgical technique and case report. Br J Ophthalmol 2009;93:861e3. 4. Holzer MP,...

Optical coherence tomography (OCT) of the anterior segment, in particular corneal OCT, has become a reliable tool for the cornea specialist, as it provides the acquisition of digital images at high resolution with a noncontact technology. In penetrating or lamellar keratoplasties, OCT can be used to assess central corneal thickness and pachymetry maps, as well as precise measurements of deep st...

We describe two patients (a Filipino boy aged 2.7 years and a Kuwaiti girl aged 4.8 Years) with clinical and MRI findings consistent with the diagnosis of pontine tegmental cap dysplasia (PTCD) and compare them with 23 other cases reported in the literature. Both presented with feeding problems (VII nerve), sensori-neural deafness (VIII nerve) and hypotonia from birth and later developed cornea...

The results of a retrospective study of the effects of silicone oil removal from 85 eyes in 85 patients are reported. Pars plana vitrectomy and fluid/silicone oil exchange had been undertaken for retinal detachments associated with giant retinal tears or with proliferative vitreoretinopathy in all cases. Clinically significant cataract developed in 60% of lenses that were clear at the time of o...