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Indications for pediatric keratoplasty in India.

PURPOSE: To study the associations and indications for pediatric keratoplasty. METHODS: We undertook a retrospective analysis of 415 grafts performed in 393 children, aged 12 years or younger between January 1993 and December 1997. The parameters evaluated were demographic data, socioeconomic status, preoperative diagnosis, and any systemic or ocular associations. RESULTS: Of the 415 eyes operated on, 296 (71.32%) eyes underwent keratoplasty for acquired nontraumatic corneal opacities, 51 (12.28%) had congenital corneal conditions, 45 (10.85%) were regrafts, and 23 (5.54%) eyes had acquired traumatic corneal opacities. The cause of corneal opacification in the acquired nontraumatic group was infectious keratitis in 215 (72.63%) eyes and keratomalacia in 81 (27.36%) eyes. Systemic associations with the onset of the opacity in the acquired nontraumatic group were fever in 74 (25%) cases, diarrhea in 56 (18.9%) cases, and malnutrition in 98 (33.1%) cases. Seventeen (4.32%) children belonged to the lower middle class, 64 (16.28%) were in the upper lower class, and 312 (79.38%) were from the lower socioeconomic families based on the Kuppuswamy scale. CONCLUSIONS: Infectious keratitis is the major indication for pediatric keratoplasty in India. In the majority of the cases, the occurrence of corneal opacification is associated with poverty.

Age of Onset↗

[Central corneal diseases].

BACKGROUND: Central corneal pathologies can lead to an irreversible decrease of best corrected visual acuity if not diagnosed and treated appropriately. This article reviews the differential diagnosis of central corneal opacities in the newborn, of central infectious corneal ulcers, and the therapy of sterile, central keratolysis. MATERIAL AND METHODS: Authors' personal experience and review of the literature. RESULTS: Flow charts for diagnosis and treatment strategy have been elaborated. CONCLUSIONS: Corneal opacities in newborns create an emergency situation. In order to treat successfully and avoid or diminish amblyopia, it is imperative to rule out congenital glaucoma. The aetiology of central corneal ulcers should always be confirmed by positive cultures to be able to treat specifically. When the standard topic therapy fails, one has to consider rare bacteria, parasites, virus, or patients' compliance. The treatment of central sterile keratolysis in rheumatoid arthritis must be intensive and immunosuppression has to be performed early enough in the course of prevent the formation of a descemetocoele or spontaneous corneal perforation.

Arthritis, Rheumatoid↗

Two brothers with gelatinous drop-like dystrophy at different stages of the disease: role of mutational analysis.

PURPOSE: A report of two Japanese brothers with gelatinous drop-like corneal dystrophy, one with and one without the typical gelatinous drop-like region. DESIGN: Interventional case report and observational case report. METHODS: After penetrating keratoplasty, the corneal button, right eye, of the elder brother, 39 years of age, was stained and examined by microscopy. The M1S1 and BIGH3 genes were examined for mutations using the polymerase chain reaction and direct sequencing. Corneal abnormalities in the younger brother, 37 years of age, were observed. RESULTS: The elder brother had bilateral gelatinous prominences and band-shaped corneal opacities, whereas the younger brother had only bilateral band-shaped opacities. Histologically, corneal deposits beneath the epithelium stained with Congo red. Molecular genetic analysis revealed that M1S1 was homozygously mutated in both brothers (Q118X). CONCLUSION: The Q118X mutation of the M1S1 gene can produce either a gelatinous drop-like region or band-shaped opacities.

Adult↗

[Phototherapeutic keratectomy as the primary option before perforating keratoplasty].

BACKGROUND: Argon fluoride excimer laser (193-nm) can perform precise corneal ablation with submicron accuracy and minimal reaction to the adjacent tissue. A possible application is anterior lamellar keratectomy to remove superficial corneal opacities and to smooth corneal irregularities. PATIENTS AND METHODS: Between August 1993 and June 1994 we performed a phototherapeutic keratectomy (PTK) in 35 patients with different corneal diseases by excimer laser (Aesculap-Meditec). 3 cases (2 corneal dystrophies and 1 patient with a traumatic corneal opacity after chemical burn) are described more exactly with operative methods and postoperative results. RESULTS: By slitlamp microscopy we observed an improvement of the primary corneal disorder in all cases. Changing of refraction (hyperopic shift and newly formed astigmatism) arose in 2 out of 3 patients. CONCLUSIONS: Phototherapeutic keratectomy by excimer laser represents a secure, less invasive alternative to surgical methods promising quicker visual recovery. Often a penetrating keratoplasty can be avoided or delayed.

Adult↗

[The expression of extracellular matrix in epithelial ingrowth after LASIK].

PURPOSE: To observe the expression of extracellular matrix in cornea and its relationship with corneal opacity after epithelial ingrowth in LASIK. METHODS: Corneal flap was made on rabbit eyes, and epithelial cells mechanically scraped from surroundings the flap were implanted underneath. The corneas were harvested 1, 3, 7 and 30 days following surgery, histological and immunohistochemical examination was performed. RESULTS: On slit-lamp examination, corneal opacity was observed in the area where epithelial cells were implanted. Histological study revealed clusters of epithelial cells at the flap and stroma bed interface. One week and one month post-operation intense immunoreactivity for collagen type IV was detected at the perimeters of the intrastromal epithelial island, but not in the ordinary interface area, strong staining for collage type III was also observed around the epithelial islands. CONCLUSION: The marked deposition of collagen type IV surrounding the ingrowth epithelial cells indicated that it might be the essential component of interface haze in epithelial ingrowth after LASIK.

Animals↗

[Postoperative complications of penetrating subtotal keratoplasty in children].

Accordingly to experience of corneal transplantations during 1981-2001 in the children's clinic of the Helmholz Moscow Research Institute of Ocular Diseases the authors present the clinical picture, rate, prognostic value and modes of correction of postoperative complications after keratoplasty. A retrospective study analyzed the incidence of complications after 94 penetrating keratoplasties performed in 82 children aged from 10 months to 14 years in 89 eyes for congenital corneal opacity (n = 22) or corneal opacity caused by trauma (n = 40) or inflammation (n = 20). 60 (73%) within 1 year after operation developed different reactions and complications due to transplant failure in 41 (44%) cases. Among them there were graft rejection episodes (34%), anterior synechiae and retrocorneal membrane formation (17%), donor corneal neovascularization (11%), elevated intraocular pressure (11%). There were a few cases with shallow anterior chamber, hyphema/hemophthalmos, retinal detachment, primary graft failure, corneal ulcer, suture infiltrates and abscesses. Subatrophy of the eye was an outcome in 3 cases. The development of complications after penetrating keratoplasty is determined mainly by the physiology of children, age, severity of underlying and concomitant pathology of the eye. Nevertheless, the odds of donor tissue for transparent graft survival increase if such complications are detected and removed.

Child↗

Comparative trial of different anti-bacterial combinations with propolis and ciprofloxacin on Pseudomonas keratitis in rabbits.

The aim of the current study was to evaluate the effects of five different treatment combinations to find out whether propolis could be an alternative or an adjunctive treatment, in experimental Pseudomonas aeruginosa keratitis. Intrastromal P. aeruginosa strains were given to both eyes of 20 young New Zealand white rabbits. The rabbits were randomly divided equally into five treatment groups; ciprofloxacin and dexamethasone drops (C+D), ciprofloxacin drop (C), ciprofloxacin and propolis drops (C+P), propolis drop (P), 3% ethanol drop (control), respectively. Directly before the first treatment and 108 h after inoculation, the eyes were examined by slit lamp to assess the corneal opacity and rabbits were sacrificed for bacterial count. The mean corneal opacity scores and the mean bacterial counts log cfu/ml were significantly different in the treatment groups (P=0.001; ANOVA). According to post hoc tests for both the mean bacterial counts and corneal opacity scores, C+D, C, C+P groups were found to be statistically the same (P>0.05), and although the P group had significantly better scores than the control group it did not reach the scores of the rest of the treatment groups (P<0.01). We conclude that propolis may be a useful adjunctive agent but should not be regarded as a replacement for traditional antibiotic therapy for P. aeruginosa keratitis in rabbits.

Animals↗

Central corneal thickness is lower in osteogenesis imperfecta and negatively correlates with the presence of blue sclera.

BACKGROUND: Osteogenesis imperfecta (OI) is a rare, autosomal-inherited, connective tissue disorder characterised by bone fractures, deafness and blue sclera. Additional ocular findings are decreased ocular rigidity, myopia, glaucoma, keratoconus, corneal opacity, small corneal diameter and congenital Bowman's layer agenesis. PURPOSE: This cross-sectional, masked, case-control study aimed to assess whether central corneal thickness (CCT) is affected in patients with OI and to focus on the clinical significance of scleral blueness. MATERIALS AND METHODS: Twenty-three children with OI (13 boys, 10 girls) and 15 age-, sex- and refraction-matched healthy control subjects (eight boys, seven girls) were assessed for CCT by ultrasound pachymetry. The CCT was compared between two different patient subgroups (type-I OI with blue sclera, n = 12; type-IV OI without blue sclera, n = 11). Mann-Whitney U-test or analysis of variance was used as indicated and only right eyes of each subject were included in statistical analysis. Results were expressed as mean +/- S.D. and statistical significance was taken as p < 0.05. RESULTS: Mean age and sex distribution was similar between the groups (10.1+/-2.5 vs 9.8+/-1.8 years, p > 0.05). Patients with OI had significantly lower CCT (459.5+/-24.6 microm) than in control subjects (543.6+/-21.4 microm; p < 0.001). The CCT was below 500 microm in 22 of 23 children (95.6%) with OI, 15 of which (65.2%) were below 450 microm. In contrast, CCT was over 500 microm in all eyes in the control group. Type-I OI eyes with blue sclera had significantly (p = 0.005) lower CCT readings (446.5+/-16.3 microm) than type-IV OI eyes without blue sclera (473.6+/-25.0 microm). Mean keratometric values were similar between the groups (44.2+/-1.7 vs 43.8+/-1.6 dioptre, p > 0.05). Mean cycloplegic refraction was similar between the groups (-0.32+/-0.5 vs -0.18+/-0.4 dioptre; p > 0.05), although five of 23 OI patients had myopia, and mean intraocular pressure was lower in OI patients than controls (12.7+/-1.8 mmHg vs 15.6+/-1.9 mmHg; p < 0.001). CONCLUSIONS: The CCT is thinner and negatively correlated with the blueness of the sclera in patients with OI. The CCT readings may therefore be of utmost importance in the diagnosis of OI. An ophthalmologist should be aware of an artificially low intraocular pressure measurement in such patients. In addition, when considering a keratorefractive treatment, CCT must be evaluated carefully to avoid unexpected results or complications. Sturdy protective spectacles should be prescribed to those who are not bed bound. Possible correlation of low CCT with biochemical changes in scleral collagen or systemic parameters awaits further investigation.

Adolescent↗

Ocular complications in newly diagnosed borderline lepromatous and lepromatous leprosy patients: baseline profile of the Indian cohort.

AIM: To describe ocular manifestations in newly diagnosed borderline lepromatous (BL) and lepromatous leprosy (LL) patients in India. METHODS: Ocular complications, at enrolment, occurring in all new borderline lepromatous and lepromatous leprosy patients detected by active case finding within the geographically defined leprosy endemic area of the Gudiyattam Taluk in India from 1991 to 1997 who consented to ocular examinations every 6 months, during and 5 years after treatment with multidrug therapy (MDT), were studied. RESULTS: Orbicularis oculi weakness (4.62%), lagophthalmos (4.20%), ectropion (0.42%), trichiasis (0.84%), blocked nasolacrimal ducts (1.68%), pterygium (11.34%), impaired corneal sensation (53%), corneal opacity (10.5%), corneal nerve beading (1.68%), punctate keratitis (1.26%), keratic precipitates (4.62%), iris atrophy (1.68%), and cataract (12.6%) were ocular complications seen in the 301 lepromatous patients at enrolment. 4.6% had blind eyes. Increasing age was associated with ocular complications. 80% of patients were skin smear acid fast bacilli (AFB) positive. The LL/BL ratio was 1:6.4. 71% had some limb deformity. 44% had only leprosy related ocular complications (LROC), 28% had only general ocular complications (GOC) while 14% had both LROC and GOC. Ocular complications were significantly related to leg deformities. Corneal nerve beading was seen most in LL patients (100%) having high bacterial content. Lagophthalmos and muscle weakness were associated with reversal reactions. CONCLUSIONS: Corneal nerve beading occurs in LL patients with high bacillary count. Patients with reversal reaction are more likely to present with orbicularis oculi weakness and lagophthalmos. Leprosy related ocular complications and general ocular complications are significant problems in newly diagnosed lepromatous patients. Elderly, deformed, skin smear positive, lepromatous patients are associated with increased ocular morbidity and form a group that require acceptable and accessible eye care.

Adolescent↗

Results of an ophthalmologic screening programme for identification of cases with Anderson-Fabry disease.

PURPOSE: Anderson-Fabry disease is an inherited lysosomal storage disease with a broad and unspecific range of symptoms, a painful course of disease and early death. The recent development of new enzyme therapy emphasises the need for early diagnosis and treatment of undiagnosed patients. One of the affected organs of Anderson-Fabry disease is the eye. Cornea verticillata--corneal opacities and corneal dystrophy--as well as tortuositas vasorum can occur in an early stage of the disease affecting almost all hemizygous men and more than 70% of heterozygous women. In order to identify unknown cases with Anderson-Fabry disease, we carried out a screening programme contacting Austrian ophthalmologists. METHODS: All 658 Austrian ophthalmologists were asked to record patients with cornea verticillata as well as tortuositas vasorum--twice at an interval of 3 months. RESULTS: 33% of the contacted ophthalmologists replied, identifying 5 patients suspected of having Anderson-Fabry disease. After additional examinations including tests for enzyme activities Anderson-Fabry disease was confirmed in 1 man. CONCLUSION: We have identified 1 case with Anderson-Fabry disease through our ophthalmology screening programme among a population of approximately of 8 million. Ophthalmologic screening programmes for ocular manifestations typical of Anderson-Fabry disease are limited because of the moderate visual affection in these patients. Nevertheless, considering the limited options to detect such cases otherwise, ophthalmologists have a major responsibility to identify patients with Anderson-Fabry disease on routine examinations.

Austria↗

Indocyanine green staining for the triple corneal procedure.

BACKGROUND AND OBJECTIVE: In the triple corneal procedure, successful completion of capsulorhexis is of the utmost importance. Another use of indocyanine green dye for better visualization of the anterior lens capsule of mature cataract during the triple corneal procedure is described. PATIENTS AND METHODS: Four consecutive patients (mean age, 69.5 years) with both mature cataracts and corneal opacity underwent the triple corneal procedure. After trephination of the recipient cornea, the anterior capsule of the lens was stained with indocyanine green. A continuous curvilinear capsulorhexis (CCC) was performed, after which conventional triple corneal procedures were followed. RESULTS: In all four cases, this technique markedly improved visualization of the lens capsule and resulted in successful and easy manipulation of the CCC and subsequent removal of residual lens cortex. CONCLUSIONS: Staining of the anterior capsule of mature cataract in the triple corneal procedure clearly defines the border of the capsule, thus allowing easy and complete execution of CCC.

Aged↗

Superficial reticular degeneration of Koby.

A 36-year-old white man with congenital ichthyosis and a history of a posterior uveitis beginning at age 8 presented with an unusual corneal opacity. At age 26 bilateral central corneal opacities were noted consisting of fine interlacing white lines forming a reticulum at the level of Bowman's membrane. A faint brown background tint of the basal epithelium was also present. Over the next 10 years the opacity progressed only slightly, with some mild superficial scarring of the underlying corneal stroma. The visual acuity remained good. This corneal pattern appeared virtually identical to the superficial reticular degeneration of Koby. As described by Koby in 1927, this rare corneal degeneration is characterised as a painless, slow, progressive development of a central fine, white reticulum at the level of Bowman's membrane. This rare corneal degeneration probably represents an atypical form of band keratopathy.

Adult↗

Corneal changes in spondyloepiphyseal dysplasia tarda.

BACKGROUND: A new type of corneal opacity with prominent corneal nerve fibers as an ocular complication of spondyloepiphyseal dysplasia tarda (SEDT). CASE: A 58-year-old woman, diagnosed with SEDT at 5 years of age, underwent a complete ophthalmological examination. OBSERVATIONS: The patient had no complaints and no history of eye disease. No relatives were reported to have suffered from SEDT. Slit-lamp examination disclosed a diffuse opacity in the central cornea in both eyes, which was localized in the middle to deep stroma. Dot opacities in the central and paracentral cornea were located in the middle of the stroma in both eyes. Optically clear regions were observed in the peripheral cornea of both eyes. More interestingly, corneal nerve fibers were visible passing from the limbus to the central cornea in both eyes. RESULTS: The etiology of the corneal opacities of this patient with SEDT is unknown. However, collagen and proteoglycan abnormalities in the skin of patients with SEDT have been reported. Therefore, such abnormalities may also be present in the cornea and these alterations may lead to corneal complications.

Blinking↗

Bilateral deep keratitis caused by systemic lupus erythematosus.

We report the optical and ultrasonic biomicroscopy and confocal microscopy findings in bilateral stromal keratitis (keratoendotheliitis), a rare ocular manifestation of systemic lupus erythematosus (SLE). Examination revealed deposits with polyrefringent crystals. Topical corticosteroid produced regression of the corneal edema, but there was an increase in corneal opacity. Ultrasound biomicroscopy images confirmed the deep location of the corneal opacities, and confocal microscopy showed a disruption of the corneal stroma and crystal-like bodies.

Adult↗

Corneal tattooing to mask subsequent opacification after amniotic membrane grafting for stromal corneal ulcer.

PURPOSE: We aimed to report on the cosmetic efficacy of corneal tattooing using India ink for resolving corneal opacities resulting from amniotic membrane grafting carried out to promote corneal wound healing in stromal corneal ulcers. METHODS: Amniotic membrane grafting of corneal ulcers was carried out in three patients. After corneal re-epithelialization, a 30-gauge needle filled with India ink was placed into the amniotic membrane space located between the regenerated corneal epithelium and the stromal bed. The ink was then carefully injected. RESULTS: The cornea was successfully stained with India ink in all cases. There were no significant complications during the mean follow-up period of 17 months. All the patients were satisfied with the corneal staining. CONCLUSION: When a corneal opacity occurs after an amniotic membrane graft for stromal corneal ulcer, corneal tattooing by intrastromal injection of India ink into the amniotic membrane space may be a very useful method of achieving a good cosmetic result.

Amnion↗

Penetrating keratoplasty in infants.

PURPOSE: To analyze graft survival and visual outcome after penetrating keratoplasty in infants with congenital corneal opacity. METHODS: We retrospectively reviewed the records of 11 patients with congenital corneal opacity who underwent penetrating keratoplasty as infants. Six patients had a diagnosis of Peter's anomaly, 1 of congenital hereditary endothelial dystrophy, 1 of posterior polymorphous dystrophy, and 2 of sclerocornea, and in the other patient, the cause of the opacity was unknown. RESULTS: In total, 26 penetrating keratoplasties were performed on 16 eyes of 11 infants. All patients initially underwent surgery before the age of 13 months. Five patients underwent bilateral penetrating keratoplasty, and 10 of the transplants were repeat operations on eyes that had already had at least one previously failed graft. The age of the infants at the time of first penetrating keratoplasty ranged from 2 to 56 weeks (median, 13 weeks). The graft survival time for all transplants ranged from 3 to 137 months (median, 16 months). Overall first graft survival at 12 months was 61% (95% CI, 33%-81%), with 10 of 16 eyes retaining a clear corneal graft. Peter's anomaly, lensectomy, and repeat penetrating keratoplasty were factors most highly associated with poor graft survival and a low final visual acuity. CONCLUSION: Early penetrating keratoplasty for congenital corneal opacity may prevent deprivation amblyopia. Although this procedure carries a high risk of failure, particularly in those patients with Peter's anomaly, careful case selection, optical correction, and management of postoperative amblyopia may result in a successful visual outcome.

Adolescent↗

Wound healing of grafts in congenitally opaque infant corneas.

Wound healing, determined by uneventful suture removal, was evaluated in 28 corneal transplants (20 eyes) with congenital corneal opacities. Seven transplants in seven eyes with the typical central corneal opacities of Peters' anomaly had suture removal between 17 and 90 days postoperatively. All seven transplants remained transparent. Corneal grafts for sclerocornea or regrafts in sclerocornea healed much more quickly than those in eyes with Peters' anomaly (as early as four days postoperatively in the 13 most recent cases). In these corneas suture microabscesses and superficial neovascularization of the wound were signs indicating wound healing and also a host inflammatory response that led to graft rejection if the sutures were not removed.

Child, Preschool↗