Descemet's membrane folds in ochronosis: a case report.

BACKGROUND: We present this report of a new ophthalmic finding in a patient with ochronosis. CASE PRESENTATION: An 85-year-old Caucasian male patient with bilateral dark temporal and nasal pigmentation of conjunctiva and sclera was referred to our hospital owing to low visual acuity. On biomicroscopic examination, bilateral horizontal Descemet's membrane folds were observed. Corneal tomography revealed irregular and asymmetric "against-the-rule" astigmatism in both eyes. Anterior segment optical coherence tomography demonstrated numerous central Descemet's without edema or other corneal structure alterations. CONCLUSION: This is the first report of Descemet's membrane folds in ochronosis. These corneal findings suggest that the accumulation of homogentisic acid in the sclera leads to thickening and stiffness of this region. These alterations could remarkably decrease visual acuity owing to topographic corneal curvature alterations, especially in elderly patients.

Standardized pachymetry-assisted manual lamellar dissection for Descemet stripping endothelial keratoplasty.

PURPOSE: To present an alternative technique (Pachy-DSEK) for the manual preparation of thin endothelial lamellae in Descemet stripping endothelial keratoplasty (DSEK), as well as to evaluate its visual and anatomic outcomes. METHODS: A retrospective chart review was conducted in 15 cases who underwent DSEK at a private eye clinic in Brazil (INOB, Brasília) from June 2017 to December 2019. All patients had ocular comorbidities and relative contraindications to Descemet membrane endothelial keratoplasty (DMEK). All endothelial lamellae were manually prepared by using a standardized technique. Best corrected visual acuity (BCVA), tomographic parameters and graft's thickness were evaluated preoperatively and at 6 months. Endothelial cell counts were evaluated preoperatively and at 12 to 24 months. RESULTS: During preparation there was one case (6%) of peripheral tear and no tissue was lost. At 6 months, the median BCVA improved from 1.60 to 0.40 logMAR (p = 0.0009). There was no significant change in anterior (p = 0.507) and posterior astigmatism (p = 0.483), anterior (p = 0.683) and posterior mean keratometry (p = 0.767), and total corneal power (p = 0.952). The median central graft thickness at 6 months was 80 µm. Ultrathin grafts (<130 µm) were achieved in 80% of cases. At 12 to 24 months endothelial cell count decreased significantly. Graft's detachment occurred in two cases (13%) and endothelial rejection in one case (6%). CONCLUSION: By using ultrasonic pachymetry intra-operatively and standardizing graft preparation, most manually dissected endothelial lamellae were ultrathin. Pachy-DSEK was safe and effective for treating endothelial disfunction in eyes with ocular comorbidities. It may be a cost-effective alternative to automated dissection methods.

In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease.

PURPOSE: To evaluate microstructural differences between corneas with and without Kayser-Fleischer rings in age-matched subjects with Wilson's disease with neurological symptoms, using confocal laser scanning microscopy. METHODS: The study included 12 subjects with Wilson's disease with neurological symptoms. Twelve corneas presented clinically with classic Kayser-Fleischer rings, visible on slit lamp examination; the other 12 served as controls. The subjects underwent a comprehensive clinical examination. Microstructural analysis using confocal laser scanning microscopy evaluated increased corneal thickness, decreased number of cells, increased debris or specific deposits, and unusual microstructures. RESULTS: Clinically, the subjects with Kayser-Fleischer rings had similar corneal findings and normal intraocular pressure; two had typical sunflower cataracts and decreased visual acuity. The control eyes all presented normal visual acuity, intraocular pressure, and corneal appearance. The microstructural analysis demonstrated similar findings in all the affected corneas. Compared with the control corneas, there were fewer keratocytes in the anterior stroma (17.380 vs. 22.380/mm3). Round, "hollow" dark areas were observed between the keratocytes; these were universal and similar in appearance in all affected corneas and all cornea layers. In the peripheral posterior stroma, there were dust-like, bright, granular deposits that tended to increase in number and density toward Descemet's membrane, masking the peripheral endothelium. The control corneas presented a normal microstructure apart from dust-like granular deposits in the periphery. CONCLUSIONS: In vivo confocal microscopy is a useful tool for evaluating the corneal microstructure when a Kayser-Fleischer ring is clinically present. The ring consists of granular, bright particles that increase in density toward Descemet's membrane, and is associated with a decreased number of keratocytes and peculiar dark, round areas in all stromal layers, probably a sign of corneal damage. When the ring is not visible in subjects with Wilson's disease, changes to the corneal microstructure are insignificant.

In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease / Análise microestrutural confocal in vivo de córneas apresentando anel de Kayser-Fleischer em pacientes com doença de Wilson

ABSTRACT Purpose: To evaluate microstructural differences between corneas with and without Kayser-Fleischer rings in age-matched subjects with Wilson's disease with neurological symptoms, using confocal laser scanning microscopy. Methods: The study included 12 subjects with Wilson's disease with neurological symptoms. Twelve corneas presented clinically with classic Kayser-Fleischer rings, visible on slit lamp examination; the other 12 served as controls. The subjects underwent a comprehensive clinical examination. Microstructural analysis using confocal laser scanning microscopy evaluated increased corneal thickness, decreased number of cells, increased debris or specific deposits, and unusual microstructures. Results: Clinically, the subjects with Kayser-Fleischer rings had similar corneal findings and normal intraocular pressure; two had typical sunflower cataracts and decreased visual acuity. The control eyes all presented normal visual acuity, intraocular pressure, and corneal appearance. The microstructural analysis demonstrated similar findings in all the affected corneas. Compared with the control corneas, there were fewer keratocytes in the anterior stroma (17.380 vs. 22.380/mm3). Round, "hollow" dark areas were observed between the keratocytes; these were universal and similar in appearance in all affected corneas and all cornea layers. In the peripheral posterior stroma, there were dust-like, bright, granular deposits that tended to increase in number and density toward Descemet's membrane, masking the peripheral endothelium. The control corneas presented a normal microstructure apart from dust-like granular deposits in the periphery. Conclusions: In vivo confocal microscopy is a useful tool for evaluating the corneal microstructure when a Kayser-Fleischer ring is clinically present. The ring consists of granular, bright particles that increase in density toward Descemet's membrane, and is associated with a decreased number of keratocytes and peculiar dark, round areas in all stromal layers, probably a sign of corneal damage. When the ring is not visible in subjects with Wilson's disease, changes to the corneal microstructure are insignificant.

RESUMO Objetivo: Avaliar, ao nível microestrutural, através de microscopia confocal in vivo a lazer, 12 córneas com anel de Kayser-Fleischer visível ao exame da lâmpada de fenda e compará-las com 12 córneas clinicamente normais de indivíduos com idades correspondentes aos pacientes com doença de Wilson e sintomas neurológicos. Métodos: O estudo incluiu 12 indivíduos com doença de Wilson e sintomas neurológicos (24 córneas). Doze córneas apresentavam clinicamente o anel clássico de Kayser-Fleischer e as outras 12 serviram como controle. Todos os pacientes foram submetidos a um exame clínico abrangente e a uma análise microestrutural subsequente utilizando microscopia confocal in vivo de varredura a laser. Os principais resultados observados foram: aumento da espessura da córnea, diminuição do número de células, aumento de resíduos/depósitos específicos e microestrutura atípica. Resultados: Clinicamente, todos os indivíduos com anel de Kayser-Fleischer (12 olhos) apresentaram achados similares da córnea e pressão intraocular normal. Dois indivíduos também apresentaram uma catarata de girassol típica e diminuição da acuidade visual. Todos os olhos do grupo controle apresentaram acuidade visual, pressão intraocular e aparência corneana normais. A microscopia confocal in vivo com varredura a laser revelou achados semelhantes em todas as córneas afetadas. O número de ceratócitos no estroma anterior era menor, 17.380/mm3 (22.380/mm3 no grupo controle), e entre eles foram identificadas áreas escuras arredondadas "vazias". Essas zonas escuras eram generalizadas e similares em todas as córneas examinadas e em todas as camadas da córnea. No estroma posterior periférico, havia presença de depósitos granulares brilhantes e com aparência de pó que tendiam a aumentar em número e densidade no sentido da membrana de Descemet, mascarando o endotélio periférico. As córneas controle apresentaram estrutura normal, com exceção de depósitos granulares com aparência de pó na periferia. Conclusões: A microscopia confocal in vivo é uma ferramenta útil para a avaliação da microestrutura da córnea quando o anel de Kayser-Fleischer está clinicamente presente. O anel é constituído de partículas granulares brilhantes com densidade aumentada no sentido da membrana de Descemet. Sua presença está associada com uma diminuição do número de ceratócitos e com áreas circulares escuras "peculiares" em todas as camadas estromais, que representam, provavelmente, um sinal de dano da córnea. Quando o anel não está clinicamente visível, a estrutura da córnea in vivo encontra-se insignificantemente alterada.

Persistence of granular corneal dystrophy type-1 deposits in the predescemetic layer after big-bubble deep anterior lamellar keratoplasty.

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.

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.

Management of Descemet Membrane Detachment After Forceps Birth Injury.

PURPOSE: To describe the clinical signs of Descemet membrane (DM) detachment due to forceps-related birth injury and its subsequent management using optical coherence tomography. METHODS: Case report. RESULTS: A 3-day-old term infant presented with left eye corneal clouding and a definitive history of traumatic forceps-assisted delivery. Despite topical therapy, corneal clouding persisted, necessitating an examination under anesthesia using ultrasound and handheld optical coherence tomography. This revealed not only a tear in DM but also a large detachment. Injection of air alone failed to achieve apposition of DM to the posterior stroma. Apposition was achieved only after penetration of the overlying cornea with the needle of a 10-0 nylon suture and release of clear viscous fluid. The cornea cleared within the first week and continued in the months to follow. CONCLUSIONS: Prolonged corneal edema should alert the physician to probable DM detachment after forceps-related birth injury. Injecting air alone may not be sufficient to reattach the detached DM.

Imágenes Scheimpflug útiles en el diagnóstico de desprendimiento de la membrana de Descemet / Useful Scheimpflug images for the diagnosis of Descemet membrane detachment

El desprendimiento de la membrana de Descemet es una complicación infrecuente de la cirugía intraocular que puede resultar devastadora si no se diagnostica y se trata a tiempo. Habitualmente está asociado a la cirugía de la catarata, aunque tiene múltiples etiologías. Existen tratamientos muy variados, desde la simple observación hasta la queratoplastia penetrante. Se presenta una paciente femenina de 66 años de edad, con antecedentes de salud, quien fue operada de catarata del ojo derecho por la técnica de facoemulsificación con implante de lente intraocular de cámara posterior sin complicaciones aparentes durante la cirugía. En el posoperatorio presentó a las 24 horas edema corneal tres cruces con tensión ocular normal, el cual se mantuvo durante la primera semana a pesar del tratamiento intensivo con cloruro de sodio hipertónico y antinflamatorios esteroideos. No se recogieron alteraciones del endotelio corneal previas a la cirugía. En las imágenes de Scheimpflug del pentacam se observó desprendimiento de la membrana de Descemet en distintos puntos. Se realizó neumopexia y a las 24 horas la córnea se encontraba transparente(AU)

Descemet´s membrane detachment is an uncommon complication of the intraocular surgery that can be devastating if it is not diagnosed and treated on time. It is usually associated to cataract surgery, although has multiple etiologies. Treatments vary from the simple observation to the penetrating keratoplasty. This is the case of a 66 year-old woman, with a history of health problems, who was operated on of cataract in her right eye through the phacoemulsification technique with posterior chamber intraocular lens implantation without apparent complications during surgery. After 24 hours, she presented with corneal edema, three crosses and normal ocular pressure. She remained with the same condition during the first week despite treatment with hypertonic sodium chloride and steroid anti-inflammatory drugs. There were no alterations in the corneal endothelium before surgery. Scheimpflug images in Pentacam showed Descemet´s membrane detachment in several sites. It was decided to apply pneumopexia which rendered transparent cornea after other 24 hours(AU)

[Ultrasound biomicroscopy in deep sclerectomy: case report]. / Biomicroscopia ultra-sônica em esclerectomia profunda: relato de caso.

Case report of a woman with the diagnosis of primary open-angle glaucoma who was submitted to a successful surgical procedure of deep sclerectomy in the right eye. In the postoperative period, at month 3, the surgical procedure was evaluated with ultrasound biomicroscopy (UBM) utilizing two distinct equipments (UBM 840-Zeiss; UBM-VUMAX-Sonomed), with 50-MHz transducers. The diagnostic method of ultrasound biomicroscopy can be utilized as a non invasive method to evaluate the internal architecture of the eye at the topography of deep sclerectomy. It allows to identify the anterior segment structures, their anatomical relationship, and also the intact trabecular-Descemet membrane and the intrascleral space. Ultrasound biomicroscopy showed to be useful in evaluating postoperative status of the surgical procedure.

Concave trabeculo-Descemet's membrane as an early sign of viscocanalostomy failure.

PURPOSE: To correlate trabeculo-Descemet's membrane (TDM) changes with a late rise in intraocular pressure (IOP) in patients with successful viscocanalostomy. SETTING: Consultores Oftalmológicos, Hospital Oftalmológico Santa Lucía, and Centro de Estudios Médicos e Investigación, Buenos Aires, Argentina. METHODS: Patients who had a viscocanalostomy between July 1998 and September 2001 were retrospectively studied. Gonioscopy was performed in all patients. A subgroup of patients who had a late increase in IOP and were successfully treated with neodymium:YAG (Nd:YAG) laser goniopuncture were studied to correlate gonioscopic findings. Ultrasound biomicroscopy was performed in 10 cases. Intraocular pressure measurements were taken before viscocanalostomy and before and after Nd:YAG treatment. RESULTS: Sixty-eight eyes of 48 patients had a viscocanalostomy to control IOP. Twenty-six eyes had a late increase in IOP and were successfully treated with Nd:YAG laser goniopuncture. Before goniopuncture, 17 eyes (65.30%) had a concave TDM. The mean IOP in these patients increased from 14.27 mm Hg to 18.73 mm Hg (range 14 to 27 mm Hg). Goniopuncture was performed a mean of 10.7 months (range 1 to 36 months) after surgery. After Nd:YAG treatment, the TDM was flat in all patients who had a concave TDM before treatment. At the last examination, the mean IOP was 13.8 mm Hg, a mean decrease of 52.24% from the baseline preoperative IOP; 61.5% of eyes had a final IOP of 14.0 mm Hg or lower. CONCLUSIONS: A concave TDM was associated with a late IOP rise. The TDM was flat after laser goniopuncture and normalization of IOP.