Múltiplas camadas na membrana de descemet / Multiple layers on the Descemet membrane

RESUMO Relato de caso não descrito na literatura oftalmológica nacional de múltiplas camadas da membrana de Descemet. Mulher de 59 anos, pseudofácica, com diagnóstico de ceratopatia bolhosa, foi submetida à ceratoplastia penetrante em olho direito, sendo encontrado achado incomum de multiplicidade de camadas da membrana de Descemet, variáveis em forma e espessura, além de corpos ovoides com coloração de metamina de prata de Gocott-Gomori (GMS) negativa. Como a membrana de Descemet tem no seu desenvolvimento um período fetal e outro pós-natal que frequentemente é bem identificável nos cortes histológicos em adultos. No caso apresentado, em que outras camadas estão presentes, há evidência de corpos esféricos que poderiam significar que a potencialidade de produzir outras camadas pode permanecer na fase adulta mais tardia.

ABSTRACT To relate a case not described previously in the national multilateral ophthalmological literature of Descemet's membrane. A 59-year-old pseudophakic woman diagnosed with bullous keratopathy underwent penetrating keratoplasty with an unusual finding of multiple layers of Descemet's membrane, which were variable in shape and thickness. In addition, ovoid bodies with negative Gocott-Gomori (GMS) color were present. Discussion and Conclusion: As Descemet's membrane has a fetal and a postnatal period in its development, it is frequently and well identifiable in histological sections in adults. In the case presented, in which other layers are present, there is evidence of spherical bodies that could mean that the potential to produce other layers may remain into later adulthood.

Anterior Segment Optical Coherence Tomography Imaging of Haab Striae.

The authors present a case series demonstrating the anterior segment optical coherence tomography (AS-OCT) findings of Haab striae in three patients with congenital glaucoma. The use of AS-OCT in the assessment of Haab striae in pediatric glaucoma is novel, previously undescribed, and possibly allows differentiation between acute and chronic corneal changes.

Distrofia policromática posterior da córnea / Posterior polychromatic corneal dystrophy

Os autores descrevem dois casos de distrofia policromática posterior da córnea, uma distrofia pré-Descemet, pouco descrita nas literaturas nacional e mundial, em que se observam pontos policromáticos difusos no estroma posterior da córnea, sem aparente comprometimento da visão.

The authors describe two cases of posterior polychromatic corneal dystrophy, a pre-Descemet dystrophy, poorly described in national and world literatures, characterized by diffuse polychromatic points on the posterior corneal stroma, without visual impairment.

Hyperglycemia-induced abnormalities in rat and human corneas: the potential of second harmonic generation microscopy.

BACKGROUND: Second Harmonic Generation (SHG) microscopy recently appeared as an efficient optical imaging technique to probe unstained collagen-rich tissues like cornea. Moreover, corneal remodeling occurs in many diseases and precise characterization requires overcoming the limitations of conventional techniques. In this work, we focus on diabetes, which affects hundreds of million people worldwide and most often leads to diabetic retinopathy, with no early diagnostic tool. This study then aims to establish the potential of SHG microscopy for in situ detection and characterization of hyperglycemia-induced abnormalities in the Descemet's membrane, in the posterior cornea. METHODOLOGY/PRINCIPAL FINDINGS: We studied corneas from age-matched control and Goto-Kakizaki rats, a spontaneous model of type 2 diabetes, and corneas from human donors with type 2 diabetes and without any diabetes. SHG imaging was compared to confocal microscopy, to histology characterization using conventional staining and transmitted light microscopy and to transmission electron microscopy. SHG imaging revealed collagen deposits in the Descemet's membrane of unstained corneas in a unique way compared to these gold standard techniques in ophthalmology. It provided background-free images of the three-dimensional interwoven distribution of the collagen deposits, with improved contrast compared to confocal microscopy. It also provided structural capability in intact corneas because of its high specificity to fibrillar collagen, with substantially larger field of view than transmission electron microscopy. Moreover, in vivo SHG imaging was demonstrated in Goto-Kakizaki rats. CONCLUSIONS/SIGNIFICANCE: Our study shows unambiguously the high potential of SHG microscopy for three-dimensional characterization of structural abnormalities in unstained corneas. Furthermore, our demonstration of in vivo SHG imaging opens the way to long-term dynamical studies. This method should be easily generalized to other structural remodeling of the cornea and SHG microscopy should prove to be invaluable for in vivo corneal pathological studies.

[Retained Descemet's membrane after penetrating keratoplasty for macular corneal dystrophy]. / Retention der Descemet-Membran nach perforierender Keratoplastik bei makulärer Hornhautdystrophie.

A 35-year-old woman underwent excimer laser penetrating keratoplasty for macular corneal dystrophy in the right eye. Two months after surgery, a thin transparent membrane behind the clear corneal graft was noted. Anterior segment optical coherence tomography revealed duplication of the anterior chamber by this membrane, originating from the host cornea without direct contact to the donor tissue. Trypan-blue-assisted descemetorhexis was performed, and histopathology confirmed the diagnosis of retained Descemet's membrane. Four months later, the graft was clear with good visual acuity and no sign of retrocorneal membrane.

Cornea with Peters' anomaly: perturbed differentiation of corneal cells and abnormal extracellular matrix in the corneal stroma.

PURPOSE: We examined histopathologically the anterior ocular segment including the cornea and lens of an eye which had been enucleated in a patient with Peters' anomaly because of untreatable corneal perforation. Special effort was made to differentiate the corneal stromal and endothelial cells, and the stromal extracellular matrix. METHODS: Light microscopy, with hematoxylin and eosin staining, and transmission electron microscopy were employed. RESULTS: Corneal endothelial cells and Descemet's membrane were not detected in the central cornea, where there were immature cells with a fibroblastic configuration. The inner surface of the peripheral cornea was covered with cells containing pigment granules in the cytoplasm. Cell density in the central corneal stroma was relatively high. The diameter of the stromal collagen fibrils was not uniform. A mature collagen fibril-free area was also seen in the central corneal stroma. CONCLUSIONS: Differentiation of neural crest-derived cells in corneal stroma and endothelium might have been perturbed in the cornea of this patient with Peters' anomaly, inducing the defect in the corneal endothelium and the qualitative and quantitative abnormalities of the extracellular matrix.

Clinicopathological report of cerebroocular myopathy syndrome.

PURPOSE: To report a case of cerebroocular myopathy syndrome with a focal absence of the Descemet membrane. DESIGN: Clinicopathological case report. METHODS: A clinical and histopathologic examination of eye, brain, and viscera of a white male newborn of normal term, who died at 11 days of age with the diagnosis of cerebroocular myopathy syndrome. RESULTS: The autopsy revealed hazy left cornea, hypoplasia of the cerebellum, and corpus collusum with mild microcephaly. Microscopy of the left eye showed the additional findings of a central focal defect of the Descemet membrane with an absence of the posterior third of central corneal stroma and delicate fibrous strands connected to the iris. CONCLUSION: The absence of the Descemet membrane and the missing posterior corneal keratocytes as well as the fibrous strands connecting the iris are findings to be added to the previously reported signs of cerebro-ocular myopathy.

Possible new syndrome of microcephaly with cortical migration defects, Peters anomaly and multiple intestinal atresias: a multiple vascular disruption syndrome.

A possible new multiple vascular disruption symdrome is described. The male patient had microcephaly with cortical migration defects, Peters anomaly and multiple inestinal atresias.

Ocular and systemic features of Peters' anomaly.

BACKGROUND: To clarify the relationship between associated systemic anomalies and ocular manifestations in patients with Peters' anomaly, a retrospective study was conducted. METHODS: We classified 37 patients with Peters' anomaly into two groups, one with (+) and one without (-) systemic anomalies. RESULTS: The systemic anomaly (+) group consisted of 13 patients, eight males and five females, with mean age of 2.3 months. Peters' anomaly was bilateral in six cases and unilateral in seven. Corneolenticular adhesion was observed in 11 cases. Associated ocular anomalies were seen in 12 cases, and developmental glaucoma was present in eight cases. The systemic anomaly (-) group comprised 24 patients, 13 males and 11 females, with mean age of 28.3 months. Peters' anomaly was bilateral in 11 cases and unilateral in 13. Corneolenticular adhesion was observed in five cases. The associated ocular anomalies were observed in 10 cases, and developmental glaucoma was accompanied in six cases. The incidences of cases with corneolenticular adhesion, those with other ocular anomalies, and those with glaucoma were significantly higher in the systemic anomaly (+) group than in the systemic anomaly (-) group. CONCLUSIONS: Peters' anomaly accompanying corneolenticular adhesion and/or other ocular anomalies should be evaluated for the presence of systemic anomalies.

Terrien's marginal degeneration associated with posterior polymorphous dystrophy.

PURPOSE: To document an association between Terrien's marginal degeneration and posterior polymorphous dystrophy. METHODS: A 23-year-old Saudi man presented with decreased vision, peripheral corneal thinning with vascularization and scarring, and abnormalities of the posterior stroma and Descemet's membrane. RESULTS: Clinical examination, corneal topography, and specular microscopy were consistent with a diagnosis of Terrien's marginal degeneration and posterior polymorphous dystrophy. CONCLUSION: We report the first case, to our knowledge, of the simultaneous occurrence of Terrien's marginal degeneration with posterior polymorphous dystrophy.

Anterior segment dysgenesis in mosaic Turner syndrome.

AIMS/BACKGROUND: Females with Turner syndrome commonly exhibit ophthalmological abnormalities, although there is little information in the literature documenting findings specific to Turner syndrome mosaics. Ophthalmic findings are described in four patients with mosaic Turner syndrome. All had anterior chamber abnormalities and all four had karyotypic abnormalities with a 45, X cell line. The possible relation between the karyotypic and the phenotypic findings in these patients is discussed. METHODS: Four girls with mosaic Turner syndrome underwent a full ophthalmological assessment, including examination under anaesthesia where indicated. RESULTS: Three of the four patients presented with congenital glaucoma. Two had the karyotype 45, X/46, X, idic(Y) and one a 45, X/47, XXX karyotype. The remaining child had a Rieger malformation of the iris and the karyotype 45, X/46, X, r(X). CONCLUSIONS: These findings suggest that Turner syndrome mosaicism (where there are two abnormal cell lines) is associated with anterior segment dysgenesis. The findings in these four patients are compared with those seen in other mosaic phenotypes and it is postulated that the presence of two or more genetically different cell lines may have an adverse effect on anterior segment development.

[Ocular manifestations in Peters' syndrome]. / Manifestari oculare în sindromul Peters.

The analysis of twenty-one observations with Peter Syndrome, shows that the disease was established by a disorder in the development of the mesoderm who gives birth at the posterior, stratum of cornea, iris stroma and angle of the posterior chamber. The disease was associated with cataract (nine cases), strabismus (seven cases), congenital glaucoma (five cases), microophthalmia (three cases), microcornea (four cases), remainders of the pupillary membrane (one case), vitreous opacities (one case), facial malformations (one case), congenital dacryocystitis (one case), nystagmus (four cases). The presence at the same patient of the posterior keratoconus or staphiloma of cornea at one eye and Peter syndromme at the other eye, is possible to say that these diseases are evolutionary stages by one and the same mesodermal disgenesia.

Radiating folds of descemet's membrane associated with deep stromal infiltrates

Dobras radiais da membrana de Descemet já foram descritas em associaçäo com olhos atróficos e também como sinal de perfuraçäo iminente. Os autores descrevem três casos onde essa alteraçäo foi encontrada em associaçäo com infiltrados profundos do estroma corneano, sendo dois deles devidos a infecçäo por Herpes Zoster e um por Haemophilus influenza. A pressäo intra-ocular era normal quando do surgimento das dobras e näo havia perfuraçäo ocular. Os autores acreditam que as dobras säo devidas a alteraçöes cicatriciais ao nível da membrana de Descemet

Peters' anomaly: an unusual case.

The authors report on a case of congenital bilateral protruding corneal opacities. Clinical and histological examination was consistent with a diagnosis of Peters' anomaly with a defect in the endothelium and Descemet's membrane but without abnormalities of iris and lens. Corneal grafting was performed on both eyes. During a follow-up period of one year the corneas have remained clear. However, surgical intervention for glaucoma has been necessary.