Findings in the anterior segment on ultrasound biomicroscopy in Maroteaux-Lamy syndrome.

PURPOSE: Maroteaux-Lamy syndrome is one of the mucopolysaccharidoses caused by enzyme deficiency (arylsulfatase B) that leads to incomplete degradation and storage of dermatan sulfate. We report a case of mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome) with corneal involvement and introduce ultrasound biomicroscopy (UBM) as an examination with which to follow disease progression in relation to deposition in cornea, angle, and iris. METHODS: We describe a 11-year-old boy with a clinical and laboratorial diagnosis of MPS VI who developed increasing bilateral corneal opacification and decreased visual acuity. He underwent two seriate UBM (50-MHz transducer) evaluations. RESULTS: UBM examination showed diffuse and homogeneous stromal hyper-reflective deposit in both eyes and an increase in peripheral corneal thickness throughout time. CONCLUSION: High-frequency ultrasound documentation of corneal deposit and anterior segment involvement in a patient with Maroteaux-Lamy syndrome is unique, and follow-up revealed thickening of the corneal periphery, which may be related to the progression of the disease (continuous mucopolysaccharide deposits in corneal stroma). UBM was used to locate and document the deposit, as well as to accompany the deposit's evolution, characterizing corneal changes and angle structure involvement.

Myopic angle-supported intraocular lenses: two-year follow-up.

OBJECTIVE: To evaluate clinical and refractive results of myopic angle-supported intraocular lenses (IOLs). DESIGN: Prospective, noncomparative case series. PARTICIPANTS: Twenty-one eyes of 12 patients (7 female) with a mean age of 29.5 years and a follow-up of 24 months. The prospective study included highly myopic eyes (more than -11.00 diopters [D]) with spectacle-corrected visual acuity better than 20/200. METHODS: Twenty-one eyes underwent implantation of a single-piece IOL with Z-shaped haptics for angle support (each haptic with two footplates) and an optical zone of 4.5 mm (NuVita, Bausch & Lomb Surgical, Irvine, CA). The dioptric power of the IOL was calculated considering refraction, keratometry, and anterior chamber depth (specific nomogram), and its diameter was determined by adding 0.5 mm to the corneal diameter. MAIN OUTCOME MEASURES: We evaluated the following clinical and refractive data: visual acuity without and with correction (VAsc and VAcc), spherical equivalent (SE) obtained under cycloplegia, slit-lamp biomicroscopy, keratometry, applanation tonometry, endothelial cell count, ultrasound pachymetry, gonioscopy, indirect ophthalmoscopy, subjective complaints, and descriptions of complications. RESULTS: Mean postoperative VAsc was 20/74. Mean preoperative VAcc (20/50) increased to 20/30; 65% gained at least two lines of VAcc, and no eye had a decrease in VAcc. Preoperative SE (-18.95 D) evolved to -2. 06 D, stabilizing after one month. Iris retraction (pupil ovalization) more than 0.5 mm was noted in eight eyes (40%). Mean keratometry, corneal astigmatism, and ultrasound pachymetry were stable during the study (P > 0.01). Significant endothelial cell loss was demonstrated in the second year. Gonioscopy showed 70 (87. 5%) footplates ideally positioned with no iris depression. Reports of glare and haloes in dark environment were considered light in 80% and not referred in 20%, and spectacles were used for residual refraction in 75%. Intraocular lens exchange was needed in one eye because of undersizing, and the IOL was removed in one eye because of chronic inflammatory reaction associated with ocular hypertension (this patient was excluded from the statistical analysis). CONCLUSIONS: This report demonstrates good efficacy for correction of high myopia by the phakic IOL used during the two years of follow-up. Long-term complications (safety) such as iris retraction and endothelial cell loss remain a concern.

Inflammation-related scarring after photorefractive keratectomy.

PURPOSE: To study two cases of corneal scarring due to trauma (one case) and infection (one case) unrelated to a previous photorefractive keratectomy (PRK) for the correction of myopia. METHODS: The patients are part of an Institutional Review Board-approved study of the safety and efficacy of PRK. Both patients underwent bilateral, nonsimultaneous corneal ablation based on preoperative spherical equivalent. RESULTS: Seven months after PRK, the first patient sustained an alkali burn in one eye that caused severe corneal scarring and myopic regression. The other patient developed bilateral keratoconjunctivitis, more intense in the right eye (1 month after the operation) than in the left eye (4 months after the operation). This right eye subsequently developed diffuse scarring and loss of visual acuity. CONCLUSION: Patients who have undergone PRK may develop severe corneal scarring after relatively mild ocular traumas or infections.