Persistence of granular corneal dystrophy type-1 deposits in the predescemetic layer after big-bubble deep anterior lamellar keratoplasty / Persistência de depósitos da distrofia corneal granular tipo-1 na camada pre-Descemet após transplante lamelar anterior profundo

ABSTRACT We report intraoperative finding of Granular Corneal Dystrophy Type-1 (GCD1) deposits after stromal pneumodissection in deep anterior lamellar keratoplasty (DALK) in a 61-year-old female. Pneumodissection was performed from the center to the periphery of the cornea, characterizing a big bubble type 1 technique which dissects the deep stroma from the predescemetic layer. After stromal removal, persistence of whitish deposits inside the predescemetic layer was noted. During post-operative evaluation, anterior biomicroscopy and anterior segment optical coherence tomography showed granular opacities between the patient's Descemet's membrane and the donor cornea, suggesting possible involvement of the predescemetic layer in GCD1. This may require the surgeon's attention to choose between DALK keratoplasty or penetrating keratoplasty.

RESUMO Relatamos o achado intraoperatório de persistência dos depósitos de Distrofia Granular Tipo 1 (GCD1) após pneumodissecção estromal no transplante de córnea lamelar anterior profundo (DALK) em uma mulher de 61 anos. A pneumodissecção começou a partir do centro para a periferia da córnea, caracterizando uma big bubble tipo 1, que disseca o estroma profundo da camada pré-Descemet. Após a remoção do estroma, notamos a persistência de depósitos esbranquiçados no interior da camada pré-Descemet. Na avaliação pós-operatória, a biomicroscopia anterior e a tomografia de coerência óptica do segmento anterior evidenciaram opacidades granulares entre a membrana de Descemet e a córnea doadora, sugerindo o possível envolvimento da camada pré-Descemet na GCD1, o que pode chamar atenção do cirurgião para decidir entre manter o DALK ou converter para transplante penetrante.

Changes in Spherical Aberration after Various Corneal Surface Ablation Techniques

PURPOSE: The corneal change induced by refractive procedures influence both the postoperative refractive status and the ocular spherical aberration (SA). We evaluated changes in corneal SA after three types of surface ablation: phototherapeutic keratectomy (PTK), myopic photorefractive keratectomy (PRK), and myopic wavefront-guided laser epithelial keratomileusis (LASEK). METHODS: Twenty-six eyes (25 patients) were subjected to PTK 26 eyes (14 patients) to PRK, and 34 eyes (17 patients) to wavefront-guided LASEK. Corneal SA was measured with the iTrace in all patients both preoperatively and 6 months postoperatively. RESULTS: Six months after surgery, mean corneal SA was -0.173 +/- 0.171 micrometer in the PTK group, 0.672 +/- 0.200 micrometer in the PRK group, and 0.143 +/- 0.136 micrometer in the wavefront-guided LASEK group. The mean difference between the preoperative and postoperative corneal SA (DeltaSA) was -0.475 micrometer in the PTK group, 0.402 micrometer in the PRK group, and -0.143 micrometer in the wavefront-guided LASEK group. CONCLUSIONS: Surgically induced changes in corneal SA vary with procedure. The prediction of the pattern of SA change induced by various surface ablation procedures may be helpful for developing future surgical procedures.

Changes in Spherical Aberration after Various Corneal Surface Ablation Techniques

PURPOSE: The corneal change induced by refractive procedures influence both the postoperative refractive status and the ocular spherical aberration (SA). We evaluated changes in corneal SA after three types of surface ablation: phototherapeutic keratectomy (PTK), myopic photorefractive keratectomy (PRK), and myopic wavefront-guided laser epithelial keratomileusis (LASEK). METHODS: Twenty-six eyes (25 patients) were subjected to PTK 26 eyes (14 patients) to PRK, and 34 eyes (17 patients) to wavefront-guided LASEK. Corneal SA was measured with the iTrace in all patients both preoperatively and 6 months postoperatively. RESULTS: Six months after surgery, mean corneal SA was -0.173 +/- 0.171 micrometer in the PTK group, 0.672 +/- 0.200 micrometer in the PRK group, and 0.143 +/- 0.136 micrometer in the wavefront-guided LASEK group. The mean difference between the preoperative and postoperative corneal SA (DeltaSA) was -0.475 micrometer in the PTK group, 0.402 micrometer in the PRK group, and -0.143 micrometer in the wavefront-guided LASEK group. CONCLUSIONS: Surgically induced changes in corneal SA vary with procedure. The prediction of the pattern of SA change induced by various surface ablation procedures may be helpful for developing future surgical procedures.

Late occurrence of granular dystrophy in bilateral keratoconus: Penetrating keratoplasty and long-term follow-up.

We report a rare case of keratoconus with granular dystrophy with a follow-up of two decades, documenting the sequential presentation of two diseases confirmed by histology and genetic studies. A 13-year-old boy was diagnosed in 1988 with keratoconus in both eyes (BE) based on slit-lamp biomicroscopy findings of corneal ectasia in BE accompanied by Fleischer's ring, Vogt's striae, a small, old, healed hydrops. The left eye (LE) had central corneal thinning and scar in the central area involving the mid and posterior stroma secondary to healed hydrops. Penetrating keratoplasty (PKP) was advised. The boy was lost to follow-up till 1991 and presented with white, dot-like opacities in the central cornea in the RE only, suggestive of granular corneal dystrophy. Similar findings of white, dot-like opacities were noted in the LE in 1995 and the patient subsequently underwent PKP in BE. Histopathology of corneal buttons confirmed the presence of patchy, crystal-like orange deposits, which stained bright red with Masson's trichrome. Mutational analysis of the TGFBI gene in patient's DNA revealed a heterozygous mutation corresponding to a change in Arg555Trp in the keratoepithelin protein. Granular dystrophy recurred after 8 years in the RE.

Alcohol epitheliectomy with mechanical debridement in a case of granular corneal dystrophy with r555w homozygous mutation of TGF B1 gene.

An eight-year-old girl, an offspring of a consanguineous marriage presented with multiple anterior stromal geographic corneal opacities in both eyes. She was diagnosed to have superficial variant of granular dystrophy based on the family history, clinical features and mutation of TGF B1 gene. She was treated by alcohol-assisted removal of epithelium followed by mechanical debridement of abnormal deposits. Postoperatively, the cornea in both eyes was clear with no trace of opacity and the patient had an unaided visual acuity of 20/20 partial.

Amniotic membrane transplant with superficial keratectomy in superficial corneal degenerations: efficacy in a rural population of north India.

AIM: To evaluate the applicability and efficacy of superficial keratectomy with transplantation of preserved amniotic membrane in superficial corneal degenerations in a rural population of Northern India in terms of visual improvement and surface regularization. SETTINGS: Peripheral referral center in rural north India. MATERIALS AND METHODS: This was a prospective non-comparative interventional case series where 24 eyes of 20 farmers from peripheral rural areas (M:F = 19:1) with visually significant superficial degenerative disorders (15 eyes with climatic droplet keratopathy one of which was associated with Salzmann nodular degeneration and nine eyes with band-shaped keratopathy) were subjected to amniotic membrane transplantation (single or multiple layer) combined with superficial keratectomy. Subjective and objective outcomes after surgery were evaluated and analyzed and statistical significance of the outcomes in various disorders was evaluated. RESULTS: Eighty-eight per cent (21 eyes) had symptomatic relief from distressing preoperative symptoms while postoperative visual improvement by two or more lines was achieved in 23 eyes (96%) over a mean follow-up period of 26.8 +/- 10.2 months. The surface irregularity present preoperatively was relieved in 23 cases while postoperative decline of vision with visually significant scarring was seen in one case (4%), which was labeled as failure. CONCLUSIONS: Amniotic membrane transplant with superficial keratectomy helped achieve subjective comfort, visual rehabilitation and clinical regularization of the corneal surface in superficial corneal degenerations during the mean followup of 26.8 +/- 10.2 months in rural setups.

Diamond burr superficial keratectomy in the treatment of visually-significant anterior corneal lesions / Ceratectomia superficial com broca de diamante no tratamento de lesões anteriores da córnea

PURPOSE: To evaluate the efficacy and safety of diamond burr superficial keratectomy in the treatment of visually-significant anterior corneal lesions. METHODS: A retrospective review of 23 eyes (23 patients). Pre- and postoperative visual acuities and refractions, slit-lamp biomicroscopic findings, and the incidence of recurrence of disease after keratectomy were studied. RESULTS: Nineteen eyes had map-dot-fingerprint basement membrane dystrophy and 4 had Salzmann's nodular degeneration. All patients presented with decreased vision, as well as varying degrees of glare, halos, and monocular diplopia. Postoperative follow-up ranged from 3 to 39 months (mean 10.6 months), and no recurrence of the original disease occurred within this period. This procedure improved the best-corrected visual acuity from 20/36 (LogMar 0.250) to 20/24 (LogMar 0.076) by LogMar statistical evaluation (p<0.001) and caused a statistically non-significant (p=0.232) myopic change in the mean refractive spherical equivalent (-0.36 diopter ± SD 2.28 preoperatively to -0.71 ± 2.26 postoperatively). Glare and monocular diplopia were subjectively reduced or eliminated in all patients. One patient had mild anterior stromal haze which decreased the bestcorrected visual acuity from 20/25 to 20/30. CONCLUSION: Diamond burr superficial keratectomy appears to be an effective and safe method of removing visually-significant anterior corneal opacities.

OBJETIVO: Avaliar a eficácia e segurança da ceratectomia superficial com broca de diamante no tratamento das lesões anteriores da córnea. MÉTODOS: Foi realizado estudo retrospectivo de 23 olhos de 23 pacientes. Foram avaliados acuidade visual e refração pré e pós-operatório, biomicroscopia e incidência de recorrência da doença após ceratectomia. RESULTADOS: Dos 23 olhos avaliados, 19 olhos apresentavam distrofia da membrana basal (map-dot-fingerprint) e 4 degeneração nodular de Salzmann. Todos os pacientes apresentavam diminuição da acuidade visual, assim como graus variados de ofuscamento, halos e diplopia monocular. O seguimento pós-operatório variou entre 3 e 39 meses (média de 10,6 meses) e não houve recorrência da doença original nesse período. O procedimento melhorou a acuidade visual com melhor correção de 20/36 (LogMar 0,250) para 20/24 (LogMar 0,076) com p<0,001. Em relação as mudanças refracionais não houve significância (p=0,232) sendo o equivalente esférico pré-operatório de - 0,36 ± 2,28DE e pós-operatório de -0,71 ± 2,26DE. As queixas de ofuscamento e diplopia monocular diminuíram ou foram eliminadas em todos os pacientes. Apenas 1 paciente apresentou nubécula no estroma anterior com diminuição da acuidade visual com melhor correção de 20/25 para 20/30. CONCLUSÃO: Ceratectomia superficial com broca de diamante parece ser método efetivo e seguro para remover opacidades anteriores de córnea.

Queratectomía fototerapéutica en distrofia reticular corneal / Phototherapeutical keratetocomy in corneal lattice dystrophy

Se realizó un estudio retrospectivo en pacientes con distrofia corneal reticular en los que la recurrencia de la afección ocurrió después de diez años o más de realizada la queratoplastia perforante y en pacientes que presentaban la afección sin tratamiento quirúrgico previo, a los cuales se les realizó queratectomía fototerapéutica con excímer láser. Esta alternativa terapéutica se aplica en afecciones del estroma anterior de la córnea en la que se producen irregularidades en la superficie de esta y disminución de la visión. Fueron tratados 5 ojos correspondientes a 4 pacientes, 3 de ellos pertenecían a una misma familia. Los resultados terapéuticos y visuales mejoraron la calidad de vida de los pacientes, y se postergó la necesidad de retrasplante o trasplante. No tuvimos complicaciones. El excímer láser se convirtió en una alternativa, segura y eficaz en el tratamiento de estas afecciones.

A retrospective study was performed on patients with lattice corneal dystrophy, who suffered recurrence ten or more years after penetrating keratoplasty and on patients suffering the same problem but without any previous surgical treatment, who had undergone phototherapeutical keratectomy with Excimer laser. This therapeutic alternative is useful for anterior stromal keratopathy in where corneal irregularities decrease the vision. Five eyes have been treated from 4 patients, 3 of them were relatives. The visual and therapeutical results improved the quality of life of patients, thus postponing the need for transplant or retransplant. No complications were encountered. Laser Excímer became an effective and safe alternative in the treatment of such diseases.

Queratoplastía en distrofia corneal endotelial hereditaria temprana postnatal: evolución exitosa por más de 10 años: caso clínico / Keratoplasty in endothelial hereditary early postnatal corneal distrophy

Objetivo: Describir la evolución clínica exitosa por más de una década, de una queratoplastía en una Distrofia Corneal Endotelial Hereditaria Temprana de forma Postnatal. Método: Paciente hombre, consulta a los 10 años de edad por mala visión bilateral (cuenta dedos a 50 cm LBP), córneas con edema difuso avascular, grosor corneal aumentado 2 a 3 veces, escasas bulas epiteliales, diámetros corneales normales, tensión normal, nistagmus negativo, ortotropia; el 30 de julio de 1985 se practica queratoplastía penetrante OD, de 7,5 mm de diámetro dador y receptor. Resultados: La excelente transparencia del transplante y de los medios permitió obtener visión de 0,3 la cual se mantiene después de 10 años. Conclusión: La queratoplastía tiene vida limitada en estos casos, por profunda alteración endotelial del receptor; el sobrepasar los 10 años es de significativa importancia clínica. La ambliopía no contraindica la queratoplastía en ningún momento de la evolución de la distrofia, porque ésta es parcial

Results of penetrating keratoplasty with cataract extraction and intraocular lens implantation in Fuchs' dystrophy.

Combined procedure was performed in 25 eyes and triple procedure in 32 eyes of Fuchs' dystrophy. Pre-operative visual acuity was 20/100 or less in 63.2% of eyes. The graft was clear in 76% of combined procedure and in 97% of triple procedure eyes. Post-operative visual acuity of 20/40 or better was obtained in 57.9% of eyes. The common cause of graft failure was allograft reaction. The other causes attributable to visual failure were opacification of posterior capsule, secondary glaucoma, macular degeneration and pre-existing glaucomatous cupping. Intraocular lens insertion at the time of surgery helps in rapid visual rehabilitation.