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Hypergammaglobulinemia and corneal opacities in patients with human T-cell lymphotrophic virus type-1.

PURPOSE: To investigate the relationship between serum immunoglobulin levels and corneal opacities in a cohort of patients with human T-cell lymphotrophic virus type-1 (HTLV-1). DESIGN: Retrospective case series. METHODS: Complete ophthalmologic examination was performed on 44 patients with HTLV-1 infection (25 patients with adult T-cell leukemia/lymphoma [ATL], 18 patients with HTLV-1 that was associated myelopathy/tropical spastic paraparesis [HAM/TSP], and one patient who was asymptomatic). Corneal opacities were described by shape, size, color, and location. Serum immunoglobulin (Ig) levels (IgG, IgM, and IgA) were measured by nephelometry. RESULTS: Corneal opacities were identified in 15 of 25 patients (60%) with ATL and five of 18 patients (28%) with HAM/TSP. The prevalence of corneal opacities was associated statistically with elevated IgG level (P = .023) in patients with ATL, but not in patients with HAM/TSP (P > .99). CONCLUSION: Although the mechanism remains unclear, hypergammaglobulinemia is associated with the development of the corneal opacities in patients of African descent with ATL.

Corneal Opacity↗

[Clinical use of the excimer laser in treatment of surface corneal opacities--therapeutic strategy and case reports].

BACKGROUND: The authors report on the Phototherapeutic 193nm-Excimer Laser Keratectomy (PTK) for the treatment of superficial corneal opacities. MATERIAL AND METHOD: In two cases with corneal opacities after contact lens wearing and after therapy resistant keratoconjunctivitis epidemica respectively the treatment strategy is presented. The epithelium and the corneal opacities are removed mechanically as far as possible. Then the Excimer laser smoothing with masking fluids of different viscosity is performed. RESULTS: In both patients the visual acuity improved from 1/50 and 0.16 respectively to 0.7. Both eyes had an hyperopic shift. The patients were without pain within a follow-up period of 8 months after treatment. CONCLUSION: The phototherapeutical use of the 193nm Excimer laser to treat superficial corneal opacities can be regarded as a real alternative to conventional surgical techniques.

Adult↗

Marked hyper-HDL2-cholesterolemia associated with premature corneal opacity. A case report.

We report here a peculiar case with premature corneal opacity and extremely high levels of HDL cholesterol in serum. The patient is a 54-year-old man who was first noticed to have marked corneal opacities at age 19. His serum HDL cholesterol level was elevated to the level of 135-160 mg/dl, while total serum cholesterol and triglyceride concentrations were 254 mg/dl and 56 mg/dl, respectively. Serum apoprotein A-I and E levels analyzed by single radial immunodiffusion method were elevated in the case. Serum lipoprotein fractions isolated by preparative ultracentrifugation revealed that increased levels of HDL cholesterol were accounted for solely by the HDL2 fraction. HDL2 of the patient contained relatively higher amounts of apoprotein E than normal control HDL2. Elution profiles of lipoproteins in high performance liquid chromatography revealed that HDL2 particles from the patient were larger in size than those from normal controls. These characteristics of HDL are in part similar to those of HDLC which appears in experimental animals after cholesterol feeding. Such abnormalities in HDL2 fractions associated with premature corneal opacity have not been reported so far and appear to constitute a new disease entity.

Cholesterol↗

Corneal transplants for the treatment of congenital corneal opacities.

PURPOSE: To describe the results of corneal transplants in infants and young children with congenital corneal disorders. METHODS: The charts of 38 children who underwent corneal transplantation for congenital corneal opacities from 1988 to 1999 at the Hospital for Sick Children, Toronto, Ontario, were reviewed. Parameters reviewed included indication, age at diagnosis and surgery, graft size, graft clarity, repeat surgery, glaucoma pre- and postsurgery, visual acuity, and refraction. RESULTS: Thirty-eight children (63 eyes) underwent 86 corneal transplants. The four most common indications for surgery included posterior polymorphous dystrophy, Peters anomaly, congenital glaucoma, and sclerocornea. Mean follow-up was 40.4 +/- 30.2 months (range, 2-111 months). Seventy-eight percent of the grafts were clear at the final follow-up visit. There were 23 repeat grafts in the entire group. CONCLUSIONS: The overall success rate of graft clarity was 78% for children undergoing corneal transplantation for congenitally opaque corneas. Best results were achieved in patients with posterior polymorphous dystrophy, followed by patients with Peters anomaly. Sclerocornea and congenital glaucoma carried a 50% chance of success, with repeated transplants needed for many of the eyes.

Child, Preschool↗

Electrolysis for corneal opacities in a young patient with superficial variant of granular corneal dystrophy (Reis-Bücklers corneal dystrophy).

PURPOSE: To report the efficacy of electrolysis as a treatment of corneal opacities in a young patient with the superficial variant of granular corneal dystrophy. DESIGN: Interventional case report. METHODS: An 11-year-old boy presented with subepithelial opacities in both eyes. His visual acuity was 0.2 in the left eye; he received corneal electrolysis under topical anesthesia. RESULTS: The electrolysis, which required only 5 minutes, resulted in the disappearance of the subepithelial opacities. His visual acuity improved to 0.4 on the next day and was 1.0 eight months later. The corneal curvature and thickness were not altered by the electrolysis. CONCLUSIONS: Corneal electrolysis proved to be an effective treatment for subepithelial opacities, and we recommend electrolysis as an effective and simple treatment for young patients with SGCD.

Child↗

Sclerotic scattering illumination during phototherapeutic keratectomy for better visualization of corneal opacities.

PURPOSE: To report on a simple method to obtain a better view of corneal opacities during excimer laser phototherapeutic keratectomy (PTK). DESIGN: An interventional case report. METHODS: Phototherapeutic keratectomy was performed under sclerotic scattering illumination with a vitrectomy-endoilluminator at the limbus. RESULTS: During conventional PTK with diffuse illumination, the intraoperative observations of corneal opacities are not as good as that with a slit lamp. Conversely, subtle corneal opacities can be easily seen with sclerotic scattering illumination even under a surgical microscope. CONCLUSIONS: The use of sclerotic scattering illumination during PTK is valuable, because it provides a better view of opacified tissues and avoids excessive ablation.

Corneal Opacity↗

Optical sector iridectomy in corneal opacities.

PURPOSE: To evaluate the visual outcome after optical sector iridectomy in cases of corneal opacities. METHODS: Seventeen eyes of 17 patients with preoperative visual acuity of <6/60 in the affected eye underwent sector iridectomy in a prospective clinical study at a tertiary eye care center. Of 17 cases, 11 had corneal opacities after healed keratitis, four after trauma, and one each due to alkali burns or trachoma. The sector iridectomy was undertaken in cases in which one sector of the cornea and lens was fairly clear. Success was defined as attainment of ambulatory visual acuity of 6/60 or better. RESULTS: Mean age of the patients was 32+/-2.3 years. Most eyes had deep vascularized corneal scars (13 eyes). Optical sector iridectomy was performed most frequently in the lower nasal quadrant (11 eyes) followed by the lower temporal quadrant (four eyes). Of 17 eyes, 16 achieved a visual acuity of 6/60 or better. No intra-or postoperative complications were encountered. CONCLUSION: Optical sector iridectomy in cases of corneal opacities is a simple and safe procedure that can improve visual outcome and provide ambulatory vision to patients. It is a valuable alternative to penetrating keratoplasty if penetrating keratoplasty is not possible or not promising for various reasons.

Adolescent↗

Visual performance of rigid gas permeable contact lenses in patients with corneal opacity.

PURPOSE: To evaluate the visual performance of rigid gas permeable (RGP) contact lenses in patients with nebular and nebulo-macular corneal opacity and correlate the improvement in visual acuity with other visual parameters. METHODS: In a prospective study, visual performance of RGP contact lenses was evaluated in eyes with corneal opacity involving the pupillary area. The baseline visual acuity and other visual parameters with glasses were compared with those recorded with RGP contact lenses. The visual functions evaluated were visual acuity, contrast sensitivity, glare acuity, and mesopic vision. Binocular status with RGP contact lenses was assessed. All patients were followed up for 3 months. RESULTS: Twenty-eight eyes (26 patients) with nebular or nebulo-macular corneal opacity were included in this study. All eyes (100%) achieved significantly better visual performance with RGP contact lenses than with glasses (P< 0.0020). Visual acuity of 6/9 or better was achieved in 50% of patients with RGP contact lenses compared to none with glasses. RGP contact lenses improved the visual acuity by 0.27 decimals over the baseline value with glasses, butthe improvement for mesopic vision and glare acuity was significantly lower at 0.15 and 0.06 decimals respectively. Similarly, contrast sensitivity did not improve to the same extent as visual acuity. Stereopsis was present only in patients with best corrected visual acuity of 6/9 or better with RGP lenses. CONCLUSION: RGP contact lenses improved the visual acuity and other visual performance in patients with reduced vision due to nebular or nebulo-macular corneal opacity. However, contrast sensitivity, glare acuity, and mesopic vision did not improve to the same extent as visual acuity.

Adolescent↗

Congenital corneal opacities in a cornea referral practice.

PURPOSE: To address the primary causes of the congenital corneal opacities seen on the Cornea Service at Wills Eye Hospital (Philadelphia, PA), the associated systemic anomalies and their management. METHODS: Retrospective review of the medical records of all patients with the diagnosis of congenital corneal opacities seen at the Cornea Service from January 1, 1992 to June 30, 2003. Children 12 years old or younger at the first visit to our department were included in the study. We classified the location and extent of corneal pathology. We divided the management into medical and surgical. RESULTS: Seventy-two eyes of 47 patients were included in the study. The mean follow-up time from the first to the last visit was 33.1 months. The most common primary cause of congenital corneal abnormalities was Peters anomaly (40.3%), followed by sclerocornea (18.1%), dermoid (15.3%), congenital glaucoma (6.9%), microphthalmia (4.2%), birth trauma, and metabolic disease (2.8%). Seven eyes (9.7%) were classified as idiopathic. Ten patients had systemic abnormalities associated with their ocular condition. The management was medical in 38 eyes (52.7%). Twenty-four eyes (32.4%) underwent only 1 penetrating keratoplasty (PK). Only 1 eye received a regraft during the follow-up period. Eight grafts failed during the follow-up period. CONCLUSION: The importance of this study is to share our experience with this rare entity, congenital corneal opacities, describing their clinical presentation and their management.

Child↗

A new case of Balci's syndrome (corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spasticity associated with congenital heart disease).

The association of corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spasticity with hyperglycinemia was presented for the first time by Balci and colleagues in 1974. After this report, some similar cases in the literature were referred to as Balci's syndrome. In this paper we describe a new case of Balci's syndrome, a 2.5-month-old female patient with corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spacticity. All of these findings are acceptable as Balci's syndrome, and in addition she had congenital heart disease (ventricular septal defect) and renal anomalies. In this paper other syndromes associated with corneal opacity and mental retardation are discussed.

Abnormalities, Multiple↗

Oversized corneal grafts for corneal opacities with iridocorneal adhesions.

OBJECTIVE: To evaluate the efficacy of 1-mm oversized corneal grafts in patients with acquired corneal opacities and extensive peripheral iridocorneal adhesions. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Twenty patients (20 eyes) aged 15 years or older with unilateral or bilateral corneal opacification and a shallow anterior chamber. INTERVENTION: Penetrating keratoplasty was performed with donor corneal buttons oversized by 1 mm. MAIN OUTCOME MEASURES: The various parameters evaluated were visual acuity, graft clarity, keratometry, anterior chamber depth, intraocular pressure, and spherical equivalent refraction 12 months after surgery. RESULTS: The keratoplasties were performed in 15 eyes with a corneo-iridic scar after infectious keratitis (75%) and 5 eyes with failed graft (25%). At the final follow-up, a clear graft was achieved in 17 eyes (85%), and 14 eyes (70%) achieved a best-corrected visual acuity of 6/12 or better. Three of the grafts failed because of rejection. The average keratometry was 44.1 +/- 1.0 diopters (D), and the mean spherical equivalent was -3.23 +/- 2.86 D. The oversized grafts provided a mean anterior chamber depth of 2.36 +/- 0.42 mm, and the mean intraocular pressure at the 12 month follow-up was 16.38 +/- 2.09 mmHg. CONCLUSIONS: Corneal grafts oversized by 1 mm provide adequate anterior chamber depth and reduce the risk of peripheral anterior synechiae and secondary glaucoma in patients with corneal opacities and extensive peripheral iridocorneal adhesions.

Adolescent↗

Non-trachomatous corneal opacities in the Gambia--aetiology and visual burden.

AIMS: National blindness surveys conducted in the Gambia in 1986 and 1996 showed an increase in blindness and visual impairment from non-trachomatous opacity. This study aimed to investigate the aetiology of these opacities and to assess the resulting visual burden. METHODS: A population-based, randomised blindness survey was conducted in the Gambia in 1996. Patients with visual impairment or blindness were examined by an ophthalmologist with a slit lamp. Causes of corneal opacity were determined as accurately as possible by clinical history and examination. RESULTS: A total of 154 patients with non trachomatous corneal opacity were examined of whom 39 had bilateral opacities and 115, unilateral. Causes included corneal infection, measles/vitamin A deficiency, harmful traditional practices and trauma (unilateral scarring). Overall, corneal pathology alone was responsible for bilateral visual impairment or blindness in 19 (12%) patients and unilateral visual impairment or blindness in 88 (57%) patients. Those patients with bilateral visual impairment or blindness (mean age 59, SD) were older (P= 0.003) than others (mean age 44, SD = 20). The use of harmful traditional eye practices was associated with bilateral corneal blindness or visual impairment (RR = 2.63, 95% CI 1.11-6.21, P = 0.04). Although none of the corneal scars reported here were attributed to trachoma, in patients over the age of 45, the prevalence of trachomatous conjunctival scarring in this group was 38.8% compared to 19.4% of the whole nationwide sample. DISCUSSION: Strategies for the prevention (including the quest for cheaper anti-microbial drugs and co-operation with traditional healers) and surgical treatment of these corneal opacities are discussed.

Adolescent↗

Corneal opacities associated with NTBC treatment.

PURPOSE: To describe a patient with hereditary tyrosinemia type I (HHT-I) treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) who developed corneal opacities. METHODS: A 14-month-old patient was diagnosed with HHT-I and began treatment with NTBC. Her serial ocular examinations were normal until age 4 years, when she developed ocular discomfort and was found to have bilateral, linear, branching subepithelial corneal opacities. RESULTS: Over the next 3 years, the extent of the opacities fluctuated, and increased opacities correlated with periods of poor compliance with a restricted protein diet. Serum tyrosine levels remained elevated at 238 to 602 umol/L (normal 26 to 83) throughout the duration of NTBC treatment. CONCLUSION: Corneal opacities are a potential consequence of NTBC treatment for HHT-I. The lesions probably result from elevated serum and ocular tyrosine levels due to inhibition of the tyrosine catabolic pathway and poor dietary compliance.

4-Hydroxyphenylpyruvate Dioxygenase↗

Mucopolysaccharidosis II (Hunter disease) with corneal opacities. Report on two patients at the extremes of a wide clinical spectrum.

Clinically visible corneal opacities were observed in a patient with an extremely severe form of mucopolysaccharidosis II. In a second patient with an unusually mild form of mucopolysaccharidosis II, discrete corneal opacities were detected by slit-lamp examination. Thus clear corneae can no longer be regarded as a hallmark of mucopolysaccharidosis II.

Adult↗

The relationship between rabbit corneal opacity and immunohistochemical expression of heat shock protein 72/73 and c-fos after excimer laser photorefractive keratectomy.

To evaluate the etiology of corneal opacities, a study was done on the immunohistochemical staining for heat shock protein (HSP) 72/73 and c-fos after myopic excimer laser photorefractive keratectomy (PRK). New Zealand white rabbits (60 eyes) were divided into four groups: -6-, -9- and -15-dpt operation groups and a control group (only corneal epithelium removed). Using an excimer laser (VisX 20/20, Sunnyvale, Calif., USA), myopic PRK was performed on the rabbit's cornea by a single or multizone technique followed by an immunohistochemical stain to detect the expression of HSP 72/73 and c-fos at 6, 24, 48 h, 1 week and 8 weeks. Corneal epithelia were healed within 3 days in all groups, and 8 weeks after the operation, the mean density of corneal opacity was 0.83 (-6-dpt operation group), 1 (-9-dpt operation group) and 1.67 (-15-dpt operation group). All groups exhibited HSP 73 in corneal epithelium and stroma. However, the maximal expression of c-fos and HSP 72 appeared in corneal epithelium at 24 and 48 h, respectively, after the operation in the -15-dpt group. These results suggest that the expressions of HSP 72 and c-fos in rabbit corneal epithelium are significant factors in relation to corneal opacity after excimer laser PRK.

Animals↗

Histopathology and molecular cytogenetics of a corneal opacity associated with the trisomy 8 mosaic syndrome (46,XY/47,XY, +8).

The trisomy 8 mosaic syndrome (Tr8MS), karyotype 46,XY/47,XY, +8, is a rare multisystem disorder that may be associated with corneal opacity. We report the case of a dysmorphic infant with multiple congenital abnormalities referred to our unit with a congenital corneal opacity. Subsequent chromosomal analysis of peripheral leucocytes demonstrated constitutional Tr8MS. At 4 years of age, lamellar keratoplasty was performed. Histological examination confirmed the lesion to be consistent with a corneal choristoma. Cytogenetic studies using in situ hybridisation techniques showed the presence of trisomic cells in cell culture derived from the tissue in higher proportion (92%) than in the blood (44%). Amplification of the c-myc oncogene on chromosome-8 could not be detected in cells cultured from the corneal lesion. Although not proof, these findings lend support to the concept of the corneal lesion representing a focus of viable trisomic cells rather than an inflammatory response to a nidus of effete cells.

Abnormalities, Multiple↗

Trypan blue assisted phacoemulsification in corneal opacities.

AIM: To evaluate the efficacy of trypan blue for enhancing visualisation during phacoemulsification and foldable lens implantation in cases of cataract with corneal opacities. METHODS: 11 eyes of 11 patients with nebulomacular corneal opacities involving the visual axis with partially and visually debilitating cataract underwent trypan blue assisted phacoemulsification with foldable intraocular lens implantation. The patients were followed at the first day, first week, first month, and third month postoperatively. Completion of capsulorhexis, phacoemulsification with foldable lens implantation, and postoperative best corrected visual acuity were measured. RESULTS: The dye improved visualisation of the anterior capsule and a complete capsulorhexis could be performed successfully in all eyes. In all but one eye phacoemulsification was accomplished successfully. In one eye with Fuchs' dystrophy penetrating keratoplasty was performed later. Visual acuity was < or = 6/60 preoperatively in all eyes and improved to > or = 6/24 in eight eyes postoperatively. CONCLUSION: Trypan blue assisted phacoemulsification may be performed in selected cases of corneal haze/opacification with cataract. It provides acceptable visual outcome in cases awaiting penetrating keratoplasty or in cases where it is not feasible or promising.

Adult↗