1/37. persistent hyperplastic primary vitreous with retinal tumor in tuberous sclerosis: report of a case including tumoral immunohistochemistry and cytogenetic analyses.OBJECTIVE: The authors describe an ocular lesion combining the characteristics of persistent hyperplastic primary vitreous (PHPV) and a retinal tumor in an infant with tuberous sclerosis complex (TSC). STUDY DESIGN: Case report. methods: immunohistochemistry and cytogenetic studies were performed on TSC cells from an intraocular tumor in a 6-week-old infant. RESULTS: Histopathologic examination showed a thick fibrovascular membrane between the aspect of the lens and the astrocytic component of the mass. glial fibrillary acidic protein (GFAP) showed a variable intracytoplasmic reaction in the astrocytic proliferation, involving approximately 50% of the cells. Tissue culture studies showed a fairly rapid proliferation of fusiform cells, consistent with bipolar astrocytic cells. Cytogenetic studies showed one abnormal clone consisting of three hyperdiploid cells with a loss of chromosome 9 and a gain of chromosomes 6 and 12. CONCLUSION: The atypical localization of the retinal tumor could be explained by the fact that it was trapped during its proliferation by the retinal detachment associated with the PHPV.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/37. Ocular findings in ichthyosis follicularis, atrichia, and photophobia syndrome.ichthyosis follicularis, atrichia, and photophobia (IFAP) are typical features of a rare neuroichthyosis termed IFAP syndrome. We demonstrate the ultrastructural findings of the eyes from a 33-year-old patient with IFAP syndrome. Clinically, eyebrows and eyelashes were absent from birth, and photophobia was noted at the age of 1 year. The globes measured 28 and 29 mm, respectively, and both eyes showed a posterior staphyloma. Histopathologically, bilateral centrally located subepithelial avascular corneal scarring with secondary corneal amyloid deposition was found. In addition to already described ocular abnormalities in IFAP syndrome we demonstrate ultrastructural anomalies of desmosomes and tonofilaments in corneal epithelium; defects of basement membrane, Bowman layer, and anchoring fibrils; secondary corneal amyloid deposition; and keratocyte degeneration. A defective tear film, recurrent atopic keratoconjunctival inflammations, or a primary anomaly of corneal epithelial adhesion are potential causes for the corneal defects. photophobia is most likely due to corneal abnormalities.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
3/37. Successful treatment of tractional corectopia using 2 mJ of energy with an Nd:YAG laser.Although the Nd:YAG laser is most commonly used to perform posterior capsulotomies after cataract surgery, it has also been used to treat a variety of other anterior segment abnormalities including tractional corectopia, iridocorneal adhesions, persistent pupillary membranes, and posterior synechiae. Numerous reports on the use of the Nd:YAG laser to treat structures in the anterior segment have emphasized the need to use higher pulse energy for pupillary membranes, compared with the lower settings required for posterior capsulotomy. Steinert and Puliafito noted that single pulses of 4 to 12 mJ may be required to treat pupillary membranes "in a manner similar to that of a stonemason chipping at marble" in their description treating a membrane considerably thicker than what we describe. We report the successful treatment of tractional corectopia due to an anterior membrane strand in a child with only 2 mJ of total energy.- - - - - - - - - - ranking = 5keywords = membrane (Clic here for more details about this article) |
4/37. 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.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
5/37. persistent hyperplastic primary vitreous with myopia: a case study.BACKGROUND: persistent hyperplastic primary vitreous (PHPV) is a congenital disorder that manifests a range of ocular anomalies, including leukocoria, microphthalmia, cataract, and a retrolental fibrovascular membrane. In general, the prognosis for visual acuity with PHPV has been poor. A recent report on six patients who have myopia associated with PHPV showed that these patients were not microphthalmic, did not manifest leukocoria, and showed a mean visual acuity at final followup of 20/160. CASE REPORT: The case of a 3-year-old boy with myopic PHPV of the left eye is presented. Full-time wear of the cycloplegic refraction, combined with patching of the non-amblyopic eye, resulted in an improvement in visual acuity. RESULTS: PHPV with myopia may constitute a distinct subtype of PHPV. patients with myopic PHPV tend to seek treatment later than patients with anterior or posterior PHPV as a result of the lack of microphthalmia and leukocoria. These individuals do not show the long-term complications, such as secondary glaucoma and intraocular hemorrhages, associated with PHPV. CONCLUSION: The acuity improvement in this case of myopic PHPV suggests that a more conservative approach to its surgical management may be appropriate while pursuing refractive correction and amblyopia therapy to attain the best acuity possible.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
6/37. Incising the thick retrolental fibrovascular tissue with a hooked sclerotome in persistent hyperplastic primary vitreous.A technique for incising thick retrolental fibrovascular tissue and extensive cyclitic membrane is reported in a case of anterior persistent hyperplastic primary vitreous. A membranectomy was performed in a 1-month-old post-lensectomy baby via a limbal approach. A sclerotome tip was hooked to cut through an extremely thick fibrovascular tissue by rotating the sclerotome by its grip. Sutherland microscissors (Grieshaber, switzerland) and a vitrectomy cutter were used for further membranectomy. The baby was followed-up until age 18 months. A total of 3 membranectomy sessions were required because of rapid cyclitic membrane formation, severe centripetal retraction of the membrane on the ciliary processes, and posterior synechia. Thorough membranectomy and cutting the iris edge maintained a clear pupillary area during the 13-month postoperative period. Extremely thick retrolental fibrovascular tissue is a challenging condition that can be dealt with by delicate instrumentation.- - - - - - - - - - ranking = 7keywords = membrane (Clic here for more details about this article) |
7/37. Pseudopapilledema in neurofibromatosis type 2.PURPOSE: To report a case of neurofibromatosis type 2 with pseudopapilledema secondary to a prepapillary gliotic membrane. METHOD: Case report. Results of an ocular examination and fluorescein angiography of a patient are described. RESULTS: Fundus examination of a 14-year-old male with neurofibromatosis type 2 revealed an irregular elevation of the optic nerve and a perifoveal epiretinal membrane in the right eye. fluorescein angiography demonstrated no autofluorescence nor leakage in the area of the optic nerve. CONCLUSION: The patient has pseudopapilledema secondary to an epiretinal membrane overlying the optic disk of the right eye. The possibility of pseudopapilledema should be considered when evaluating patients with neurofibromatosis type 2 and abnormal optic nerves.- - - - - - - - - - ranking = 3keywords = membrane (Clic here for more details about this article) |
8/37. Basal lamina abnormality in the skeletal muscle of walker-warburg syndrome.The basal lamina of skeletal muscle fibers has been reported to be thinned and disrupted in patients with Fukuyama and laminin-alpha-2-deficient congenital muscular dystrophies. The basal lamina is normal in other, later-onset, muscular dystrophies, but the plasma membrane is disrupted. It is unknown whether the dystrophic process in walker-warburg syndrome (WWS) is characterized by a basal laminal abnormality, a sarcolemmal abnormality, or both. The present study examined the skeletal muscle of a 3-month-old patient with WWS by immunohistochemistry and electron microscopy and compared the findings with control muscle samples. In control samples the basal lamina of skeletal muscle fibers was a continuous, uniformly dense structure associated with sarcolemma. In WWS the basal lamina appeared deranged, with disruptions in nonnecrotic muscle fibers. Furthermore, in some fibers the basal lamina was thinner, and in others, it was duplicated. dystrophin, laminin-alpha-2, and adhalin stains revealed normal immunoreactivity. The disruptions in the basal lamina may play a primary role in the degeneration of muscle fibers in WWS. When compared with the dystrophies with a primary sarcolemmal defect, it appears that those with primary basal lamina abnormalities (WWS, laminin-alpha-2-deficient, and Fukuyama congenital muscular dystrophies) present early in life, and the phenotype is more severe.- - - - - - - - - - ranking = 5.9233145599587keywords = membrane, lamina (Clic here for more details about this article) |
9/37. Ocular findings in Fryns syndrome.PURPOSE: To demonstrate the ocular histopathologic findings in Fryns syndrome, a multiple congenital anomaly syndrome, with characteristic features including Dandy-Walker malformation, cleft palate, diaphragmatic hernia, lung hypoplasia, distal limb anomalies and polyhydramnios. The prevalence is about 0.7 per 10,000 births. Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. methods: Case report. The ocular histopathologic and ultrastructural findings in a male fetus with Fryns syndrome who died immediately after his birth at 26th week of gestation are shown. RESULTS: An abnormal Descemet's membrane was found in addition to "cloudy corneae". Electron microscopy demonstrated absence of the banded collagen fibrils in Descemet's membrane, indicating corneal endothelial dysfunction. Otherwise, the eye was morphologically normal for its age; none of the other reported ocular features of Fryns syndrome were found. CONCLUSION: Corneal endothelial dysfunction might cause abnormal composition of anterior Descemet's membrane and could contribute to the "cloudy cornea" known to occur in Fryns syndrome.- - - - - - - - - - ranking = 3keywords = membrane (Clic here for more details about this article) |
10/37. histology of microcornea complicated by bullous keratopathy.PURPOSE: To report a child with a microcornea complicated by bullous keratopathy and to present the results of a developmental and histological investigation of a microcornea. methods: Clinical and histopathological examination. RESULTS: Histopathological examination of the microcornea showed that the microcornea of our case lacked the non-banded zone of Descemet's layer and formed collagenous layer and secondary Descemet's membrane. CONCLUSION: This case demonstrates that histological finding of microcornea is not necessarily normal.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
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