ABSTRACT
PURPOSE: To describe a case of retinitis pigmentosa and nanophthalmos in a patient with attenuated Hunter's syndrome. METHODS: Fundus photography, total field electroretinogram, ultrasound, computerized visual field examination, biochemical examination and genetic testing were obtained. RESULTS: The fundus exam showed diffuse arteriolar attenuation, optic disc with regular contours, and pigment agglomerates like "bone spicules" in the middle periphery. Ultrasound examination revealed scleral thickening and short axial diameter in both eyes. The total field electroretinogram exam showed a subnormal result with greater impairment of the scotopic phase of the exam. Computerized visual field examination demonstrated a diffuse reduction in retinal sensitivity in the periphery. Biochemical examination showed increased urine glycosaminoglycan excretion and iduronate-2-sulphatase activity (IDS) deficiency in leukocytes, confirming the type II mucopolysaccharidosis. Molecular analysis revealed a novel missense mutation (p.A77D) in the IDS gene. CONCLUSION: The case report is about a patient presented an attenuated form of the syndrome, with no cognitive impairment. Ophthalmologic follow-up is still an important part of multidisciplinary treatment for Hunter's syndrome.
Subject(s)
Microphthalmos , Mucopolysaccharidosis II , Retinitis Pigmentosa , Humans , Mucopolysaccharidosis II/complications , Mucopolysaccharidosis II/diagnosis , Mucopolysaccharidosis II/therapy , Microphthalmos/complications , Microphthalmos/diagnosis , Microphthalmos/genetics , Electroretinography , Retinitis Pigmentosa/complications , Retinitis Pigmentosa/diagnosis , Retinitis Pigmentosa/genetics , Mutation, MissenseSubject(s)
Diabetes Mellitus , Diabetic Retinopathy , Ophthalmologists , Diabetic Retinopathy/therapy , HumansABSTRACT
PURPOSE: To evaluate the influence of preoperative mitomycin C (MMC) on the proliferative behavior of fibroblasts and fibrovascular tissue derived from the primary pterygium using the immunohistochemical method (Ki67 and CD34). DESIGN: Randomized clinical trial. SUBJECTS, PARTICIPANTS AND/OR CONTROLS: Sixty-five patients with primary pterygium were randomly selected and divided into one of three groups. The control group had 29 patients that were only submitted to pterygium removal. The group that received the MMC injection a month before surgery had 16 patients, and the group that received the MMC 2 weeks before surgery had 20 patients. Each patient only had one eye operated on. METHODS: Sixty-five patients were selected to undergo pterygium excision surgery. We randomly placed the patients into three groups: one without MMC (n = 29), one with MMC application 1 month before surgery (n = 16) and another with MMC application 2 weeks before surgery (n = 20). Subconjunctival injection was applied with 0.1 ml of 0.02% MMC in the pterygium body, and patients were followed for 2 years. MAIN OUTCOME MEASURES: Proliferative behavior of fibroblasts and fibrovascular tissue using the immunohistochemical method (Ki67 and CD34) comparing the three groups. RESULTS: Of the total 29 patients (44.6%) in the control group (without MMC application), 11 cases had recurrence (37.9%), of which seven (63.6%) were within 3 months of follow-up and four (36.3%) within 6 months of follow-up. The mean proliferation index of the recurrent cases was 4.5%, and of the cases without recurrence, it was 6.1%. There were 16 patients (24.6%) in the MMC application group 1 month before surgery, in which one case (6.25%) recurred at 6 months. In the group with MMC application 2 weeks before surgery, of the total of 20 patients (30.7%), there was one case of recurrence (5%) at 6 months. The proliferation index of the group that had MMC administered and did not have a recurrence was 7.2%, and in the group with recurrence, it was 6.4%. The CD34-labeled cell count was 5.8% among cases with recurrence and 5.6% in cases without recurrence. No side effects of MMC application were reported during the study follow-up period. CONCLUSION: MMC was efficient to reduce the recurrence index despite the absence of a direct relation with its antimitotic and antiangiogenic effect in the samples that were analyzed.
Subject(s)
Conjunctiva/abnormalities , Mitomycin/administration & dosage , Ophthalmologic Surgical Procedures/methods , Preoperative Care/methods , Pterygium/drug therapy , Adult , Aged , Aged, 80 and over , Conjunctiva/surgery , Female , Follow-Up Studies , Humans , Male , Middle Aged , Nucleic Acid Synthesis Inhibitors/administration & dosage , Ophthalmic Solutions/administration & dosage , Prospective Studies , Pterygium/surgery , Recurrence , Young AdultABSTRACT
The occurrence of restrictive strabismus after conjunctival surgeries is widely described in the literature. Fibrosis causes adherence of the conjunctiva to the Tenon's capsule, intermuscular membrane, adipose orbital tissue, sclera, and extraocular muscle tissue. Fibrosis causes adherence of the conjunctiva to the Tenon's capsule, intermuscular membrane, adipose orbital tissue, sclera, and extraocular muscle tissue. We report a case of the use of a biodegradable collagen-glycosaminoglycan copolymer matrix implant (Ologen®) as an alternative adjuvant, used as a spacer to mechanically separate the subconjunctival and episcleral tissues to prevent fibrosis in a patient undergoing a surgical correction for a strabismus with a restrictive component due to previous surgeries of pterygium excision.
