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1.
J Cataract Refract Surg ; 50(10): 1094-1098, 2024 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-39313861

RESUMEN

In May 2023, a 36-year-old carpenter complained of a sudden decrease in visual acuity in both eyes after his fourth COVID-19 vaccination. He underwent extensive evaluation by ophthalmological, neurological, and internal medicine specialists elsewhere, which was unremarkable, except for a computed tomography scan of his brain showing minor occipital calcifications. In 2021, he had been diagnosed with anterior basement membrane dystrophy and treated with phototherapeutic keratectomy (PTK) of the left eye, leading to significant postoperative haze. On referral in July 2023, slitlamp examination showed significant anterior basement membrane dystrophy in the right eye, whereas the left eye had an extensive central scar in the anterior stroma, measuring up to 6 mm in width and 140 µm in depth (Figures 1-3JOURNAL/jcrs/04.03/02158034-202410000-00017/figure1/v/2024-09-18T130724Z/r/image-tiffJOURNAL/jcrs/04.03/02158034-202410000-00017/figure2/v/2024-09-18T130724Z/r/image-tiffJOURNAL/jcrs/04.03/02158034-202410000-00017/figure3/v/2024-09-18T130724Z/r/image-tiff). His corrected distance visual acuity was 20/80 in his right eye and 20/200 in his left eye, with a manifest refraction of -0.50 -3.75 × 170 and +0.00 -4.75 × 180, respectively. Corneal Scheimpflug topography showed regular corneal astigmatism of 3.3 diopters (D) and 5.5 D in the right and left eyes, respectively, with a corneal thickness of 550 and 566 µm (Figure 4JOURNAL/jcrs/04.03/02158034-202410000-00017/figure4/v/2024-09-18T130724Z/r/image-tiff). The Schirmer tear test was 20-20 mm. Fundoscopy, electrophysiological testing, and retinal optical coherence tomography (OCT) showed no abnormalities. The patient mentioned he had Crohn disease, managed with ustekinumab (a monoclonal antibody inhibitor of cytokines interleukin [IL]-12 and IL-23), but he had discontinued it because of the apparent remission of the disease. Attempts to improve visual acuity involved a soft bandage contact lens in the right eye, which was discontinued after 2 days because of the occurrence of a small corneal infiltrate that healed with a short course of topical antibiotics. Unfortunately, because of his vision, he cannot perform his tasks as a carpenter anymore. Which is your recommended treatment for both eyes?


Asunto(s)
Queratectomía Fotorrefractiva , Agudeza Visual , Humanos , Masculino , Adulto , Queratectomía Fotorrefractiva/métodos , Agudeza Visual/fisiología , Cicatriz/fisiopatología , Cicatriz/etiología , COVID-19/complicaciones , Complicaciones Posoperatorias , SARS-CoV-2 , Láseres de Excímeros/uso terapéutico , Tomografía de Coherencia Óptica , Topografía de la Córnea , Distrofias Hereditarias de la Córnea/fisiopatología , Distrofias Hereditarias de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/diagnóstico
2.
Medicine (Baltimore) ; 103(31): e39211, 2024 Aug 02.
Artículo en Inglés | MEDLINE | ID: mdl-39093770

RESUMEN

RATIONALE: There are reports of safe cataract surgery in eyes with posterior polymorphous corneal dystrophy (PPCD); however, to our knowledge, there are no reports of minimally invasive glaucoma surgery (MIGS) in eyes with PPCD. Herein, we report a case of poor intraoperative visibility with gonioscopy, postoperative corneal edema, and corneal astigmatism in eyes with PPCD treated with trabecular micro-bypass stent combined with cataract surgery. PATIENT CONCERNS/DIAGNOSIS: A 78-year-old man was referred to our hospital for MIGS. He presented with bilateral corneal endothelial vesicular changes and band lesions. Endothelial cell density was 2983/2871 cells/mm2 (right/left eye), central corneal thickness was 581 µm/572 µm, best-corrected visual acuity values (Snellen equivalent) were 20/32 (right) and 20/100 (left), and corneal astigmatism was -2.7D in the right eye and -2.5D in the left eye. INTERVENSIONS/ OUTCOMS: After phacoemulsification and aspiration with intraocular lens implantation with a 2.4-mm corneal incision in both eyes, trabecular micro-bypass stents were inserted successfully despite the poor intraoperative visibility with gonioscopy. One week after surgery, the central corneal thickness was 614 µm/609 µm, and Descemet's membrane folds and mild corneal edema were observed. Best-corrected decimal visual acuity was 20/40 for the right eye and 20/50 for the left eye. In the left eye, total corneal astigmatism increased from -2.5D to -5.5D. Corneal astigmatism and edema showed gradual improvement. LESSONS: Although reports have shown that cataract surgery can be safely performed in eyes with PPCD, MIGS in eyes with PPCD may require caution regarding intraoperative visibility with gonioscopy and visual function in the early postoperative period.


Asunto(s)
Astigmatismo , Complicaciones Posoperatorias , Stents , Humanos , Masculino , Anciano , Astigmatismo/etiología , Astigmatismo/cirugía , Stents/efectos adversos , Complicaciones Posoperatorias/etiología , Distrofias Hereditarias de la Córnea/cirugía , Facoemulsificación/efectos adversos , Facoemulsificación/métodos , Implantación de Lentes Intraoculares/efectos adversos , Implantación de Lentes Intraoculares/métodos , Gonioscopía , Agudeza Visual
3.
Am J Ophthalmol ; 266: 37-45, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-38735449

RESUMEN

PURPOSE: To evaluate the recurrence characteristics on optical coherence tomography and clinical outcomes after phototherapeutic keratectomy (PTK) or penetrating keratoplasty (PKP) in patients with Reis-Bücklers corneal dystrophy (RBCD). DESIGN: Retrospective interventional case series. METHODS: Seventeen patients with RBCD (31 eyes, including 6 surgery-naïve eyes and 25 surgical eyes) received 44 surgical interventions from 1996 through 2022. PTK or PKP was performed as the initial surgical procedure. Significant recurrence was determined when best spectacle-corrected visual acuity decreased at least 2 lines with increased opacity in the superficial cornea. Repeated PTK or PTK on the corneal graft (CG-PTK) was considered if patients could not endure poor vision due to significant recurrence. Recurrence depth and annual increase in thickness of the central cornea and subepithelial deposits were assessed by anterior segment optical coherence tomography. RESULTS: The mean follow-up time was 12.8 ± 8.5 years (range, 2.0-25.5 years). The mean logMAR best spectacle-corrected visual acuity improved from 1.24 ± 0.48 preoperatively to 0.27 ± 0.09 postoperatively in the initial PTK group (13 eyes, P < .001), from 1.84 ± 0.69 to 0.40 ± 0.13 in the PKP group (12 eyes, P < .001), from 1.04 ± 0.46 to 0.30 ± 0.07 in the repeated PTK group (12 times in 7 eyes, P < .001), and from 1.29 ± 0.43 to 0.39 ± 0.11 in the CG-PTK group (7 times in 5 eyes, P = .001). The median significant recurrence time was 27 months (95% confidence interval 23.9-30.1), 96 months (84.1-107.9), 31 months (28.8-33.1), and 24 months (19.8-28.2), respectively (P < .001). The depth of superficial deposits located between the epithelium and the anterior stroma was approximately 115 µm (85-159 µm). The annual thickening of subepithelial deposits was 14 ± 2 µm after initial PTK, 7 ± 3 µm after PKP, 14 ± 3 µm after repeated PTK, and 30 ± 11 µm after CG-PTK, compared to 4 ± 2 µm in surgery-naïve eyes (P = .002, .515, .002, <.001). The thickness of the central cornea increased by 15 ± 2 µm, 7 ± 2 µm, 15 ± 3 µm, and 31 ± 10 µm per year in the 4 surgery groups, respectively, compared to 5 ± 2 µm in surgery-naïve eyes (P = .001, .469, .001, <.001). CONCLUSIONS: Better visual acuity can be achieved after PTK than PKP for treatment of RBCD. The annual thickening of subepithelial deposits may approximate an increase in central corneal thickness. The superficial distribution of subepithelial deposits makes it feasible to perform repeated PTK, even on the corneal allograft, for recurrent RBCD.


Asunto(s)
Distrofias Hereditarias de la Córnea , Queratoplastia Penetrante , Queratectomía Fotorrefractiva , Recurrencia , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Tomografía de Coherencia Óptica/métodos , Queratectomía Fotorrefractiva/métodos , Masculino , Femenino , Estudios Retrospectivos , Agudeza Visual/fisiología , Adulto , Distrofias Hereditarias de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/fisiopatología , Distrofias Hereditarias de la Córnea/diagnóstico , Persona de Mediana Edad , Estudios de Seguimiento , Adulto Joven , Adolescente , Láseres de Excímeros/uso terapéutico , Córnea/cirugía , Córnea/patología , Córnea/diagnóstico por imagen , Anciano
4.
BMJ Case Rep ; 17(5)2024 May 08.
Artículo en Inglés | MEDLINE | ID: mdl-38719268

RESUMEN

A young a presented with painless, progressive diminution of vision in both eyes (BE). Slit lamp examination revealed the presence of a single central corneal opacity in the right eye and multiple corneal opacities of varying sizes in the left eye (LE), limited to the anterior-mid corneal stroma. Microcornea with reduced central corneal thickness and complete inferonasal iris coloboma along with inferior fundal coloboma, sparing both the disc and macula, were noted in BE. A diagnosis of BE macular corneal dystrophy (MCD) and iridofundal coloboma (IFC) was made. The patient underwent LE sutureless anterior lamellar therapeutic keratoplasty. On histopathological examination, the excised corneal tissue revealed stromal lamellar disarray with positive colloidal iron staining, strongly suggestive of MCD. Whole-exome sequencing revealed the presence of a likely pathogenic carbohydrate sulfotransferase 6 (CHST6) mutation, confirming the diagnosis of MCD. This concurrent presence of IFC with a corneal stromal dystrophy is previously unreported in the literature, to the best of our knowledge.


Asunto(s)
Coloboma , Distrofias Hereditarias de la Córnea , Humanos , Coloboma/genética , Coloboma/diagnóstico , Coloboma/complicaciones , Distrofias Hereditarias de la Córnea/genética , Distrofias Hereditarias de la Córnea/diagnóstico , Distrofias Hereditarias de la Córnea/complicaciones , Distrofias Hereditarias de la Córnea/cirugía , Masculino , Iris/anomalías , Iris/patología , Carbohidrato Sulfotransferasas , Sulfotransferasas/genética , Trasplante de Córnea/métodos , Opacidad de la Córnea/genética , Opacidad de la Córnea/diagnóstico , Opacidad de la Córnea/complicaciones , Córnea/anomalías , Córnea/patología
5.
Graefes Arch Clin Exp Ophthalmol ; 262(10): 3253-3260, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-38619603

RESUMEN

BACKGROUND: To evaluate the efficacy and safety of trans-epithelial phototherapeutic keratectomy (TE-PTK) as a treatment for recurrent corneal erosion syndrome (RCES) in patients with symptoms refractory to conventional treatments. METHODS: All patients who received TE-PTK treatment for RCES had failed 3 or more conventional treatments and were reviewed, and if met criteria, approved by healthcare workers of the British Columbia public health authority (Medical Services Plan (MSP). A retrospective chart review and telephone survey were conducted at the Pacific Laser Eye Centre (PLEC). Exclusion criteria were ocular co-morbidities potentially affecting treatment efficacy. RESULTS: This study included 593 eyes of 555 patients (46.2% male; 50.9 ± 14.2 years old) who underwent TE-PTK. The leading identified causes of RCES were trauma (45.7%) and anterior basement membrane dystrophy (44.2%). The most common pre-PTK interventions were ocular lubricants (90.9%), hypertonic solutions (77.9%), and bandage contact lenses (50.9%). Thirty-six eyes had undergone surgical interventions such as stromal puncture, epithelial debridement, or diamond burr polishing. Post-PTK, 78% of patients did not require any subsequent therapies and 20% required ongoing drops. Six patients (1.1%) reported no symptom improvement and required repeat TE-PTK for ongoing RCES symptoms after initial TE-PTK. All 6 eyes were successfully retreated with TE-PTK (average time to retreatment was 11.3 ± 14.9 months). There was no significant difference in best corrected visual acuity pre- vs. post-operatively. The mean post-operative follow-up was 60.5 months (range: 5-127 months). CONCLUSION: TE-PTK has a good efficacy and safety profile for treatment-resistant RCES. The third-party public health-reviewed nature of this study, the low recurrence rate of RCES, and the low PTK retreatment rate suggest that TE-PTK might be considered for wider use in the management of RCES.


Asunto(s)
Epitelio Corneal , Láseres de Excímeros , Queratectomía Fotorrefractiva , Agudeza Visual , Humanos , Masculino , Femenino , Estudios Retrospectivos , Queratectomía Fotorrefractiva/métodos , Persona de Mediana Edad , Agudeza Visual/fisiología , Láseres de Excímeros/uso terapéutico , Epitelio Corneal/cirugía , Epitelio Corneal/patología , Resultado del Tratamiento , Estudios de Seguimiento , Recurrencia , Adulto , Distrofias Hereditarias de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/fisiopatología , Distrofias Hereditarias de la Córnea/diagnóstico , Anciano , Enfermedades de la Córnea/cirugía , Enfermedades de la Córnea/diagnóstico , Enfermedades de la Córnea/fisiopatología , Adulto Joven
6.
Eur J Ophthalmol ; 34(4): 1281-1285, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38676318

RESUMEN

PURPOSE: To describe the outcomes of five Salzmann's Nodular Degeneration (SND) cases treated with a combination of alcohol delamination, superficial keratectomy and amniotic membrane patch with fibrin glue. METHODS: Five patients affected by SND with significant discomfort and decreased visual acuity were evaluated with refraction, best corrected visual acuity, Break-up time test, corneal topography, Anterior Segment of Optical Coherence Tomography and images of anterior segment with a full slit lamp examination. All the patients underwent alcohol delamination of the corneal epithelium followed by superficial keratectomy to remove the subepithelial nodules. A patch of amniotic membrane was applied to all of them with a ring shape and a banana shape in two patients due to the location of the disease. A bandage contact lens was inserted. The patch was kept in place for 14 days. Another bandage contact lens was inserted for 21 days until the corneal epithelium had completely healed. RESULTS: The surgical procedure was successful in removing the nodules in all patients. Vision was restored in all cases with a significant regularization of the corneal surface and reduction of ocular discomfort. After a 12-month follow-up, none of the patients showed recurrence of the disease. CONCLUSIONS: Combining alcohol delamination with superficial keratectomy and amniotic membrane patch with fibrin glue is an effective procedure for the management of SND to regularize the corneal surface and avoid the recurrence of subsequent nodules. This report is the first description of the outcomes of SND cases treated with a combination of the aforementioned techniques.


Asunto(s)
Amnios , Topografía de la Córnea , Etanol , Adhesivo de Tejido de Fibrina , Queratectomía , Adhesivos Tisulares , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Amnios/trasplante , Femenino , Adhesivo de Tejido de Fibrina/uso terapéutico , Masculino , Adulto , Persona de Mediana Edad , Etanol/administración & dosificación , Adhesivos Tisulares/uso terapéutico , Distrofias Hereditarias de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/fisiopatología , Estudios de Seguimiento , Refracción Ocular/fisiología , Resultado del Tratamiento , Epitelio Corneal
7.
Arq Bras Oftalmol ; 87(3): e20230109, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38537050

RESUMEN

PURPOSES: This study aims to assess and compare the postoperative visual and topographic outcomes, complications, and graft survival rates following deep anterior lamellar keratoplasty and penetrating keratoplasty in patients with macular corneal dystrophy. METHODS: In this study we enrolled 59 patients (23 male; and 36 female) with macular corneal dystrophy comprising 81 eyes. Out of these, 64 eyes underwent penetrating keratoplasty, while 17 eyes underwent deep anterior lamellar keratoplasty. The two groups were analyzed and compared based on best-corrected visual acuity, corneal tomography parameters, pachymetry, complication rates, and graft survival rates. RESULTS: After 12 months, 70.6% of the patients who underwent deep anterior lamellar keratoplasty (DALK) and 75% of those who had penetrating keratoplasty (PK) achieved a best-corrected visual acuity of 20/40 or better (p=0.712). Following surgery, DALK group showed lower front Kmean (p=0.037), and Q values (p<0.01) compared to the PK group. Postoperative interface opacity was observed in seven eyes (41.2%) in the DALK group. Other topography values and other complications (graft rejection, graft failure, cataract, glaucoma, microbial keratitis, optic atrophy) did not show significant differences between the two groups. The need for regrafting was 9.4% and 11.8% in the PK and DALK groups, respectively (p=0.769). Graft survival rates were 87.5% and 88.2% for PK and DALK; respectively (p=0.88 by Log-rank test). CONCLUSION: Both PK and DALK are equally effective in treating macular corneal dystrophy, showing similar visual, topographic, and survival outcomes. Although interface opacity occurs more frequently after DALK the visual results were comparable in both groups. Therefore, DALK emerges as a viable surgical choice for patients with macular corneal dystrophy without Descemet membrane involvement is absent.


Asunto(s)
Distrofias Hereditarias de la Córnea , Trasplante de Córnea , Queratocono , Humanos , Masculino , Femenino , Queratoplastia Penetrante/efectos adversos , Queratoplastia Penetrante/métodos , Trasplante de Córnea/efectos adversos , Trasplante de Córnea/métodos , Agudeza Visual , Resultado del Tratamiento , Distrofias Hereditarias de la Córnea/cirugía , Estudios Retrospectivos , Queratocono/cirugía , Estudios de Seguimiento
8.
J Med Case Rep ; 18(1): 98, 2024 Mar 10.
Artículo en Inglés | MEDLINE | ID: mdl-38461261

RESUMEN

BACKGROUND: Familial calcific band-shaped keratopathy (BSK) is a very rare disease, with no underlying cause. There is no underlying disease in this form of the disease. This article introduces a family with seven children, three of whom were diagnosed with familial primary calcific BSK. One of them developed a systemic disease 38 years after ocular manifestation. CASE PRESENTATION: In this case report, three Iranian siblings from a family with familial calcific band-shaped keratopathy (BSK) are introduced. Systemic and ocular examinations performed on these patients indicated the occurrence of chronic kidney disease in the older child, a 41-year-old woman, 38 years after ocular manifestation. The examinations conducted on the other two siblings revealed no pathological findings. The 41-year-old sister and 37-year-old brother underwent unilateral deep anterior lamellar keratoplasty (DALK), while the 33-year-old sister underwent bilateral superficial keratectomy (SK). CONCLUSION: Considering the late onset of systemic disease in one of the siblings diagnosed with familial calcific band-shaped keratopathy (BSK), it is crucial to emphasize the necessity of long-term follow-up for these patients and their families.


Asunto(s)
Calcinosis , Distrofias Hereditarias de la Córnea , Masculino , Niño , Femenino , Humanos , Adolescente , Adulto , Irán , Distrofias Hereditarias de la Córnea/cirugía , Ojo/patología , Calcinosis/complicaciones , Calcinosis/diagnóstico por imagen , Calcinosis/genética , Estudios Retrospectivos
9.
Cornea ; 43(6): 784-789, 2024 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-38437155

RESUMEN

PURPOSE: The purpose of this study was to highlight characteristic clinical and microscopic findings and report the long-term follow-up of pediatric excimer laser-assisted penetrating keratoplasty (excimer-PKP) for congenital stromal corneal dystrophy (CSCD). METHODS: A 2-year-old Greek child presented with CSCD at our department. Clinical examination showed bilateral flake-like whitish corneal opacities affecting the entire corneal stroma up to the limbus. Genetic testing identified a mutation of the decorin gene (c.962delA). The variant was not present in the parents and represented a de novo mutation. The uncorrected visual acuity was 20/100 in both eyes. Excimer-PKP (8.0/8.1 mm) was performed on the right eye at the age of 2.5 years and on the left eye at the age of 3 years. Postoperatively, alternating occlusion treatment was performed. RESULTS: The light microscopic examination demonstrated a disorganized extracellular matrix of the corneal stroma characterized by a prominent irregular arrangement of stromal collagen lamellae with large interlamellar clefts containing ground substance, highlighted by periodic acid-Schiff- and Alcian blue-positive reaction detecting acid mucopolysaccharides. Electron microscopy showed disorganization and caliber variation of collagen lamellae and thin filaments within an electron-lucent ground substance. The postoperative course was unremarkable. Both grafts remained completely clear 14 years postoperatively. Corneal tomography showed moderate regular astigmatism with normal corneal thickness. The corrected distance visual acuity was 20/25 in both eyes. CONCLUSIONS: Excimer-PKP for CSCD might be associated with excellent long-term results and a good prognosis, particularly when the primary surgery is performed at a very young age. However, this requires close postoperative follow-up examinations by an experienced pediatric ophthalmologist to avoid severe amblyopia.


Asunto(s)
Distrofias Hereditarias de la Córnea , Queratoplastia Penetrante , Láseres de Excímeros , Agudeza Visual , Preescolar , Humanos , Distrofias Hereditarias de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/fisiopatología , Sustancia Propia/cirugía , Sustancia Propia/patología , Decorina/genética , Estudios de Seguimiento , Queratoplastia Penetrante/métodos , Láseres de Excímeros/uso terapéutico , Agudeza Visual/fisiología
10.
J Cataract Refract Surg ; 50(6): 558-564, 2024 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-38270250

RESUMEN

PURPOSE: To determine the clinical outcomes of keratorefractive lenticule extraction (KLEx) in eyes with epithelial basement membrane dystrophy (EBMD) that developed after surgery. SETTING: Onnuri Smile Eye Clinic, Seoul, South Korea. DESIGN: Retrospective single-center study. METHODS: This study reviewed the medical records of 26 eyes of 16 patients who exhibited signs of EBMD after KLEx. Postoperatively, corneal findings and visual outcomes were evaluated. Statistical analyses were also performed on eyes without corneal complications after KLEx. RESULTS: Signs of EBMD were first observed within 1 week postoperatively in 22 eyes (84.6%): 6 eyes (23.1%) showed epithelial defects, 10 eyes (38.5%) showed subepithelial corneal opacity, 5 eyes (19.2%) showed diffuse lamellar keratitis, and 1 eye (3.9%) showed epithelial ingrowth. Symptoms such as pain, while corneal lesions were present, occurred in 21 eyes (80.8%). At 3 months postoperatively, 21 eyes (80.8%) showed no specific findings on slitlamp microscopy after medical treatment, 2 eyes (7.7%) showed subepithelial corneal opacity, 2 eyes (7.7%) showed corneal scar, and 1 eye (3.9%) showed epithelial ingrowth. Uncorrected distance visual acuity was 20/25 or better in 24 eyes (92.3%), and spherical equivalent was within ±1.0 D in 20 eyes (76.9%). The efficacy index of the EBMD group did not significantly differ from the control group, while the safety index was significantly lower. CONCLUSIONS: Manifestations of EBMD can occur after KLEx and can affect clinical outcomes. Most cases showed favorable results with appropriate nonsurgical treatment; however, some cases had long-term complications such as corneal scarring.


Asunto(s)
Distrofias Hereditarias de la Córnea , Cirugía Laser de Córnea , Agudeza Visual , Humanos , Estudios Retrospectivos , Agudeza Visual/fisiología , Masculino , Femenino , Adulto , Distrofias Hereditarias de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/fisiopatología , Cirugía Laser de Córnea/métodos , Cirugía Laser de Córnea/efectos adversos , Adulto Joven , Membrana Basal/cirugía , Epitelio Corneal/patología , Complicaciones Posoperatorias , Persona de Mediana Edad , Sustancia Propia/cirugía , Sustancia Propia/patología , Láseres de Excímeros/uso terapéutico , Miopía/cirugía , Miopía/fisiopatología , Topografía de la Córnea , Adolescente , Síndrome de Cogan
11.
Cornea ; 43(4): 404-408, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-37506370

RESUMEN

PURPOSE: This study aimed to report cases of bilateral corneal Bowman layer deposits in 4 patients with a history of keratorefractive surgery. To our knowledge, this condition has not previously been reported and should be distinguished from granular corneal dystrophy type 2 and other corneal dystrophies. METHODS: We reviewed all available medical records that were collected between January 2010 and December 2021 at a tertiary referral center and performed whole-exome sequencing to provide diagnostic information. RESULTS: Four patients exhibited similar bilateral corneal deposits that were observed more than 10 years after keratorefractive surgery. The patients' ages ranged from 36 to 53 years; 3 of the 4 patients were female. Three patients received laser in situ keratomileusis surgery, and 1 received radial keratotomy. All 4 patients denied having a family history of ocular diseases and reported an uneventful postoperative course. On examination, the best-corrected visual acuity ranged from 6/10 to 6/6 in all 4 patients. Slit-lamp examination revealed bilateral superficial corneal deposits involving the central cornea, and anterior segment optical coherence tomography revealed hyperreflective deposits located in the Bowman layer. Such unique manifestations suggested corneal dystrophy; thus, whole-exome sequencing was performed on all 4 patients. Only 1 patient exhibited a missense mutation in TGFBI . We further analyzed common de novo mutations to explore possible candidate genes associated with this presentation. CONCLUSIONS: We report a rare entity of presumed corneal dystrophy with deposits located in the Bowman layer in 4 patients who had received keratorefractive surgery. Clarifying the underlying pathophysiology and genetic predisposition of this disease may aid in diagnosing and preventing potential complications after keratorefractive surgery.


Asunto(s)
Distrofias Hereditarias de la Córnea , Opacidad de la Córnea , Queratomileusis por Láser In Situ , Humanos , Femenino , Adulto , Persona de Mediana Edad , Masculino , Córnea/cirugía , Distrofias Hereditarias de la Córnea/diagnóstico , Distrofias Hereditarias de la Córnea/genética , Distrofias Hereditarias de la Córnea/cirugía , Queratoplastia Penetrante , Opacidad de la Córnea/cirugía
12.
Acta Ophthalmol ; 102(3): 296-305, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-37289141

RESUMEN

PURPOSE: To describe four Finnish families with epithelial recurrent erosion dystrophy (ERED) caused by the pathogenic variant c.3156C>T in collagen type XVII alpha 1 chain gene (COL17A1). METHODS: Eleven affected and two unaffected individuals underwent clinical ophthalmological examination, anterior segment photography, and corneal topography. Two of them underwent phototherapeutic keratectomy (PTK). Genetic analysis included both next-generation and Sanger sequencing. Specimens from the manual keratectomy of one patient were available for ophthalmic pathologic examination, including immunohistochemistry. RESULTS: The common splice-site altering synonymous variant c.3156C > T, p.(Gly1052=) in COL17A1 was confirmed in 15 individuals with ERED from the four families. Subepithelial corneal scarring grades varied and increased with age, leading to decreased best-corrected visual acuity. PTK improved vision in 58- and 67-year-old individuals without reactivating the disease. The keratectomy specimens showed an uneven epithelium and a spectrum of basement membrane abnormalities, including breaks, fragmentation, multiplication and entrapment within the subepithelial scar, reflecting recurrent erosions. The stromal cells consisted of varying proportions of bland and activated fibroblasts and myofibroblasts, reflecting different ages of scars. The family with the largest number of known affected generations originated from Southern Sweden. CONCLUSION: The phenotype in the Finnish ERED families is consistent with earlier reports of the c.3156C > T variant, although the severity has varied between reports. The phenotype may be modulated by other genes. This study suggests a likely founder effect of the variant in both Finnish and Swedish populations due to their shared population histories. If vision is compromised, PTK can be considered especially in older patients.


Asunto(s)
Distrofias Hereditarias de la Córnea , Epitelio Corneal , Queratectomía Fotorrefractiva , Anciano , Humanos , Persona de Mediana Edad , Distrofias Hereditarias de la Córnea/diagnóstico , Distrofias Hereditarias de la Córnea/genética , Distrofias Hereditarias de la Córnea/cirugía , Epitelio Corneal/patología , Finlandia/epidemiología , Suecia
13.
BMC Ophthalmol ; 23(1): 408, 2023 Oct 10.
Artículo en Inglés | MEDLINE | ID: mdl-37817122

RESUMEN

PURPOSE: To report indications and clinical outcomes of corneal grafts ≤ 5.5 mm in diameter ("mini-KP") in a German tertiary referral center. METHODS: Patients who had undergone mini-KP to treat corneal ulcers with or without perforation between 2011 and 2018 at the Department of Ophthalmology, University of Düsseldorf, Germany, were identified from the local keratoplasty registry. All patient records were reviewed for age, gender, laterality, systemic and ophthalmological diseases, etiology of the corneal ulcerative disease, pre- and postoperative visual acuity over a follow-up time of up to 12 months, graft size, postoperative complications and the need for and timing of further corneal interventions. RESULTS: 37 eyes of 37 patients (male: n = 20; female: n = 17) with a mean age (± standard deviation) at presentation of 70 ± 18.8 years (range: 22-92 years) were identified. Most common etiologies were neurotrophic keratopathy (n = 15), dysfunctional tear syndrome (n = 9) and atopic keratoconjunctivitis (9). Mean graft diameter was 4.51 ± 0.63 mm (range: 3-5.5 mm). 23/37 eyes (62%) required no further intervention in the acute phase. 14/37 patients (38%) required secondary corneal intervention, due to complications. One-year graft survival was 78.4%. One eye had to be eviscerated due to recurrent corneal ulceration and endophthalmitis. 36 of 37 eyes were preserved. We found a highly significant correlation between type 2 diabetes and the development of postoperative complications (r = .46; p = .005). Corrected distance visual acuity (CDVA) improved from 1.42 ± 0.75 logMAR to 0.9 ± 0.65 logMAR postoperatively (t (23) = 5.76; p < .001). CONCLUSION: Mini-KP can be used successfully in eyes with advanced corneal ulcers due to various infectious and noninfectious etiologies to restore tectonic stability in the long-term and with moderate visual gains.


Asunto(s)
Enfermedades de la Córnea , Distrofias Hereditarias de la Córnea , Trasplante de Córnea , Úlcera de la Córnea , Diabetes Mellitus Tipo 2 , Humanos , Masculino , Femenino , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Lactante , Queratoplastia Penetrante , Úlcera/cirugía , Enfermedades de la Córnea/cirugía , Úlcera de la Córnea/cirugía , Distrofias Hereditarias de la Córnea/cirugía , Complicaciones Posoperatorias/cirugía , Supervivencia de Injerto , Estudios Retrospectivos , Resultado del Tratamiento
14.
BMC Ophthalmol ; 23(1): 433, 2023 Oct 25.
Artículo en Inglés | MEDLINE | ID: mdl-37880638

RESUMEN

BACKGROUND: Endothelial Keratoplasty (EK) is now considered as the standard treatment for Congenital Hereditary Endothelial Dystrophy (CHED) by many surgeons. We present the 12-year clinical outcome of the youngest operated patient with CHED in which we successfully performed a bilateral EK procedure without removing the recipient endothelium-Descemet complex. CASE PRESENTATION: In November 2010 we performed EK without Descemet Stripping in a 3-month female newborn, thinking that the lower manipulation obtained by leaving the recipient endothelium-Descemet complex could be the key factor for the success of our surgery. Such a particular technique was new in newborns. The surgery was a success, but the long-term visual result was not predictable at that time. We followed the patient at 4 months, and then yearly. At the latest visit in October 2022 the visual, cognitive, and motorial developments were normal, with Best-corrected Distance Visual Acuity of 0.4 LogMAR with - 0.75 D sf + 2.75 D cyl @ 105° in the right eye (RE) and 0.4 LogMAR with + 1.50 D sf + 2.50 D cyl @ 60° in the left eye (LE). The endothelial microscope showed an unexpected healthy endothelium, with a cell count of 2383 cells/mm2 in the RE and of 2547 cells/mm2 in the LE from a starting donor count of 2900 cells/mm2. No secondary procedures were performed during the 12-year follow-up. CONCLUSION: EK without Descemet stripping has proved to be a successful procedure over time in our newborn. The unexpected healthy endothelium suggests a role of the Descemet membrane in CHED.


Asunto(s)
Distrofias Hereditarias de la Córnea , Queratoplastia Endotelial de la Lámina Limitante Posterior , Distrofia Endotelial de Fuchs , Recién Nacido , Humanos , Femenino , Endotelio Corneal , Estudios de Seguimiento , Queratoplastia Endotelial de la Lámina Limitante Posterior/métodos , Agudeza Visual , Distrofias Hereditarias de la Córnea/cirugía , Recuento de Células , Distrofia Endotelial de Fuchs/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
15.
Eye Contact Lens ; 49(11): 471-474, 2023 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-37616173

RESUMEN

PURPOSE: To describe a case of a patient treated for neurotrophic keratopathy (NK) with direct corneal neurotization (CN), where a modification to the CN technique allowed for semiscleral contact lens use postoperatively. OBSERVATION: Our patient had successful CN with improved corneal sensation. During the procedure, a 1.0 mm gutter was created between the limbus and nerve graft to allow for semiscleral contact lens fitting. CONCLUSIONS: With the use of preoperative planning and a limbal gutter during CN, a semiscleral contact lens can serve as a well-tolerated postoperative management option to improve visual acuity and protect the corneal surface in patients with NK.


Asunto(s)
Lentes de Contacto , Enfermedades de la Córnea , Distrofias Hereditarias de la Córnea , Queratitis , Transferencia de Nervios , Enfermedades del Nervio Trigémino , Humanos , Transferencia de Nervios/métodos , Enfermedades de la Córnea/cirugía , Córnea/cirugía , Córnea/inervación , Queratitis/cirugía , Distrofias Hereditarias de la Córnea/cirugía , Enfermedades del Nervio Trigémino/cirugía
16.
Ophthalmic Res ; 66(1): 1114-1127, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37490883

RESUMEN

BACKGROUND: Phototherapeutic keratectomy (PTK) has been increasingly used to treat severe recurrent corneal erosion syndrome (RCES) patients who do not respond to other treatments. However, the efficacy and complication of each study are currently uncertain due to varying rates. OBJECTIVES: The objective of this study was to investigate the safety and efficacy of the PTK for recurrent corneal erosions. METHODS: This article performed a systematic literature research in Cochrane, Embase, PubMed, Scopus, and the Web of Science for the literature on PTK treatment of RCES until December 20, 2022. The extracted data including recurrence rate and the adverse event rate were used for meta-analysis. RESULTS: The recurrence rate was 18% (95% CI, 13%-24%) (129/700 eyes). Subgroup analysis showed that the RCE recurrence was 17% (95% CI, 9%-24%) after trauma and 22% (95% CI, 11%-32%) in the corneal dystrophy group. Treatment-related adverse events included subepithelial haze, hyperopic shift, and decrease of the best spectacle-corrected visual acuity. In this study, the incidence of these events was 13% (95% CI, 6%-21%), 20% (95% CI, 11%-28%), and 11% (95% CI, 5%-16%), respectively. CONCLUSIONS: PTK represented a valuable treatment option for patients with recurrent corneal erosions, especially those with traumatic injuries, which had minimal side effects.


Asunto(s)
Enfermedades de la Córnea , Distrofias Hereditarias de la Córnea , Úlcera de la Córnea , Queratectomía Fotorrefractiva , Humanos , Láseres de Excímeros/uso terapéutico , Estudios de Seguimiento , Agudeza Visual , Distrofias Hereditarias de la Córnea/complicaciones , Distrofias Hereditarias de la Córnea/cirugía , Córnea/cirugía , Recurrencia , Resultado del Tratamiento , Enfermedades de la Córnea/cirugía
17.
Indian J Ophthalmol ; 71(6): 2462-2465, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37322661

RESUMEN

Purpose: To evaluate the effectiveness of repeat deep anterior lamellar keratoplasty (DALK) in patients of previous failed DALK. Methods: : A retrospective analysis of records of seven patients who had undergone repeat DALK following the failure of the primary DALK was done. The indications for repeat surgery, time elapsed since the first surgery, and pre- & postoperative best-corrected visual acuity (BCVA) were noted for all the patients. Results: The follow-up period ranged between one- to four-year post repeat DALK. The indication of primary DALK was keratoconus with vernal keratoconjunctivitis (VKC) (n = 3), corneal amyloidosis (n = 2), Salzman nodular keratopathy (n = 1), and healed keratitis (n = 1). The need for repeat surgery arose when the BSCVA dropped to less than 20/200. The time interval elapsed since the first surgery ranged from two months to four years. Postoperatively, the BSCVA improved from 20/120 to 20/30 at the end of one-year post repeat DALK in all except one patient. All regrafts were clear at the most recent examination, performed after a mean period of 18 months after the secondary graft. No complication was encountered during the resurgery. The dissection of the host bed was easier in the second surgery owing to weaker adhesions. Conclusion: The prognosis for repeat DALK for failed DALK is excellent, and the outcomes of secondary grafts were comparable to those of primary DALK grafts. Re DALK offers the advantage of an easier dissection and lower chances of graft rejection compared to penetrating keratoplasty.


Asunto(s)
Distrofias Hereditarias de la Córnea , Trasplante de Córnea , Queratitis , Queratocono , Humanos , Estudios Retrospectivos , Queratoplastia Penetrante , Queratocono/diagnóstico , Queratocono/cirugía , Queratitis/cirugía , Distrofias Hereditarias de la Córnea/cirugía , Resultado del Tratamiento
18.
Jpn J Ophthalmol ; 67(4): 424-430, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37341848

RESUMEN

PURPOSE: To determine the effect of the formulation of topical medications on the healing of corneal epithelial cells after phototherapeutic keratectomy (PTK). STUDY DESIGN: Retrospective cohort study. METHODS: We studied 271 eyes of 189 consecutive patients (aged 67.6 ± 11.8 years) who had undergone PTK for granular corneal dystrophy (n = 140), band keratopathy (n = 47), or lattice corneal dystrophy (n = 2). Postoperatively, generic or brand-named levofloxacin, 0.1% betamethasone, or 0.1% bromfenac sodium hydrate was applied topically. Patients were examined on postoperative days 1, 2, and 5 and weekly thereafter. The time to re-epithelialization was assessed by use of Kaplan-Meier and Cox proportional hazards analyses. RESULTS: The time to re-epithelialization was significantly longer with generic 0.5% levofloxacin, at 8.2 ± 3.5 days, than with 0.5% Cravit (levofloxacin), at 6.7 ± 3.5 days (P = 0.018), or with 1.5% Cravit, at 6.3 ± 2.6 days (P = 0.000). In addition, the time to re-epithelialization was significantly longer with generic 0.1% betamethasone (Sanbetason), at 7.3 ± 3.4 days, than with brand-name 0.1% betamethasone (Rinderon), at 6.1 ± 2.5 days (P = 0.0002). The Cox proportional hazards model indicated that the use of generic formulations for levofloxacin eye drops and 0.1% betamethasone was a significant factor that delayed corneal re-epithelialization (hazard ratio [HR] = 0.72, P = 0.002 and HR = 0.77, P = 0.006, after adjustment for age). Re-epithelialization was significantly shorter in band keratopathy than in corneal dystrophy (HR = 1.56, P = 0.004). No other factors, including age, bandage contact lens, and diabetes mellitus, were significantly associated with time to re-epithelialization. CONCLUSION: Corneal epithelial healing can be significantly affected by different antibacterial or steroid eye drops. Clinicians need to be aware that a generic formulation may affect corneal epithelial healing.


Asunto(s)
Distrofias Hereditarias de la Córnea , Queratectomía Fotorrefractiva , Humanos , Estudios Retrospectivos , Composición de Medicamentos , Levofloxacino , Láseres de Excímeros/uso terapéutico , Distrofias Hereditarias de la Córnea/cirugía , Soluciones Oftálmicas
19.
BMJ Case Rep ; 16(6)2023 Jun 14.
Artículo en Inglés | MEDLINE | ID: mdl-37316286

RESUMEN

A male patient in his 30s, with a history of bilateral microkeratome-assisted myopic laser-assisted in situ keratomileusis (LASIK) 8 years ago at another centre, presented to us with gradually progressive diminution of vision and glare in both eyes for the last 4 years. On presentation, uncorrected distance visual acuity (UDVA) was 6/24 and 6/15 in the right eye and left eye, respectively, with normal intraocular pressures. Slit-lamp examination and anterior segment optical coherence tomography revealed well-defined white deposits, limited to an area within the LASIK flap. The deposits were confluent, at the level of the LASIK flap interface, and few discrete opacities were present in the posterior stroma. His father also had a similar clinical picture in both eyes. A diagnosis of both eyes post-LASIK exacerbation of granular corneal dystrophy with epithelial ingrowth was made. He underwent right eye femtosecond laser-assisted sutureless superficial anterior lamellar keratoplasty. At 6-month follow-up, UDVA improved to 6/12 with graft clarity of 4+ and coexistent grade 1 epithelial ingrowth.


Asunto(s)
Distrofias Hereditarias de la Córnea , Queratomileusis por Láser In Situ , Miopía , Masculino , Humanos , Queratomileusis por Láser In Situ/efectos adversos , Distrofias Hereditarias de la Córnea/cirugía , Ojo , Microscopía con Lámpara de Hendidura
20.
J Refract Surg ; 39(6): 422-429, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37306202

RESUMEN

PURPOSE: To evaluate the effectiveness of sequential custom phototherapeutic keratectomy (SCTK) for granular corneal dystrophy type 1 (GCD1). METHODS: Thirty-seven eyes of 21 patients with GCD1 were treated with SCTK to remove superficial opacifications, regularize the corneal surface, and decrease optical aberrations. SCTK is a sequence of custom therapeutic excimer laser keratectomies with step-by-step intraoperative corneal topography monitoring of results. Six eyes of 5 patients previously treated with penetrating keratoplasty received SCTK for disease recurrence. Pre-operative and postoperative corrected distance visual acuity (CDVA), refractive values, mean pupillary keratometry, and pachymetry were retrospectively analyzed. The mean follow-up period was 41.3 months. RESULTS: SCTK provided significant decimal CDVA improvement, from 0.33 ± 0.22 to 0.63 ± 0.24 (P < .0001) at the last available follow-up visit. One eye, initially treated with penetrating keratoplasty, showed visually significant disease 8 years after the first SCTK and was re-treated. Mean corneal pachymetry difference between preoperative and final follow-up values was 78.42 ± 62.26 µm. Mean corneal curvature and the spherical component did not show a statistically significant change or hyperopic shift. Astigmatism and higher order aberration reduction were statistically significant. CONCLUSIONS: SCTK is a powerful tool for the treatment of anterior corneal pathologies hindering vision and quality of life, such as GCD1. SCTK is less invasive and fosters more rapid visual recovery than penetrating keratoplasty or deep anterior lamellar keratoplasty. Providing significant visual improvement, SCTK can be the preferred initial treatment in eyes with GCD1. [J Refract Surg. 2023;39(6):422-429.].


Asunto(s)
Distrofias Hereditarias de la Córnea , Queratectomía Fotorrefractiva , Humanos , Calidad de Vida , Estudios Retrospectivos , Distrofias Hereditarias de la Córnea/cirugía , Queratectomía
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