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1/69. Vitreoretinal findings similar to retinopathy of prematurity in infants with compound heterozygous protein s deficiency.

    OBJECTIVE: To present previously undescribed vitreoretinal findings similar to severe retinopathy of prematurity (ROP) in two siblings (daughter and son) with a thrombophilic disorder, compound heterozygous protein S (PS) deficiency. DESIGN: family genotype study and literature review. PARTICIPANTS: Two unrelated heterozygous PS-deficient parents and their two children with compound heterozygous PS deficiency were studied. The gestational age and birth weight of the daughter were 40 weeks and 3200 g, respectively, and those of the son were 34 weeks and 2150 g, respectively. Three other neonates with homozygous or compound heterozygous PS deficiency and ophthalmologic findings were identified in the literature. INTERVENTION: The daughter underwent lensectomy-vitrectomy at 48 weeks adjusted age bilaterally. The son underwent therapy developed for severe ROP: laser therapy of the peripheral avascular retina at 39 weeks adjusted age, and bilateral lensectomy-vitrectomy with membrane peel of intravitreous proliferation from the optic disc at 42 weeks adjusted age. MAIN OUTCOME MEASURES: The main clinical outcome measures were retinal appearance and functional vision. Genotypes of the family members were determined. RESULTS: One of the four eyes retained functional vision. A normal-appearing posterior retina, normal scotopic and photopic flash electroretinograms, and a normal flash visual-evoked response were documented from the left eye of the son at 62 weeks adjusted age. The other three eyes had inoperable retinal detachments and no functional vision. The mother had type I PS deficiency and the father had type II PS deficiency. Compound heterozygous PS deficiency was confirmed in both children. CONCLUSION: In both children, normal vasculogenesis was interrupted. At 39 weeks adjusted age, the retinal examination of the son revealed extraretinal fibrovascular proliferation at the optic disc (reactivation of the hyaloid system) and in the peripheral retina (interruption of inner retinal vascularization). patients with homozygous or compound heterozygous PS deficiency may present as infants with severe ROP. The authors' experience suggests that appropriately timed surgical procedures, which are efficacious for ROP, can preserve vision in infants with thrombophilic disorders.
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ranking = 1
keywords = retinopathy
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2/69. Angle-closure glaucoma after laser treatment for retinopathy of prematurity.

    Laser photocoagulation has become the standard for treatment of retinopathy of prematurity. In general, it has been found to be a safe and effective means of retinal ablation. We report a case of angle-closure glaucoma in an infant after diode laser treatment for retinopathy of prematurity, which required bilateral surgical peripheral iridectomies.
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ranking = 1.2
keywords = retinopathy
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3/69. Intralenticular candida species abscess in a premature infant.

    PURPOSE: To report the clinical and histopathologic findings of a premature infant with severe retinopathy of prematurity complicated by the development of an intralenticular fungal abscess. methods: Case report and literature review. RESULTS: A markedly premature infant developed candida septicemia at 29 weeks postconception. Over the ensuing 10 weeks, cataract and intraocular inflammation developed sequentially in each eye, as did progressive retinopathy of prematurity with tractional retinal detachment. Pars plana vitrectomy and lensectomy revealed intralenticular candida species abscess. CONCLUSION: Progressive cataract and intraocular inflammation in a low birth weight infant may be caused by endogenous intraocular infection secondary to systemic candidiasis. cataract secondary to retinopathy of prematurity is rare.
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ranking = 0.6
keywords = retinopathy
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4/69. Scleral perforation after scleral buckling surgery for retinopathy of prematurity.

    Scleral perforation occurred as a result of using a silicone band during scleral buckling surgery for subtotal retinal detachment in retinopathy of prematurity (ROP). The patient was initially treated by cryotherapy and scleral buckling surgery for ROP, and was later referred due to a dark bluish mass in the superotemporal quadrant of the eyeball. After removing the overlying whitish membrane, uveal tissue prolapsed through the melted scleral wound (5 mm x 5 mm). A silicone encircling band had passed through the wound and was exposed subconjunctivally around the temporal and the inferior limbus. The band was removed and a scleral allograft was performed. After three years, follow up revealed the eyeball was slightly microphthalmic. Though scleral bucking surgery is helpful for the treatment of advanced ROP, a scleral perforation may develop as a disastrous complication.
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ranking = 1
keywords = retinopathy
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5/69. Histopathology and vascular endothelial growth factor in untreated and diode laser-treated retinopathy of prematurity.

    OBJECTIVES: We had the unique opportunity to compare the eyes of a premature infant with stage 3 retinopathy of prematurity (ROP) in both eyes after the condition was treated by diode laser photocoagulation in one eye only. After the infant's death, we investigated the extent of structural damage incurred with the diode laser and examined the effect of treatment on vascular endothelial growth factor (VEGF) expression. methods: The eyes were fixed and embedded in paraffin. Adjacent 6 microns sections were either stained for histopathologic analysis or used for in situ hybridization. VEGF messenger rna (mRNA) was detected by using radiolabeled antisense riboprobes. RESULTS: In the treated eye, histopathologic results demonstrated the clinically evident dose-response effect, with sparing of inner retinal elements with mild laser burns and full-thickness retinal cell disruption with severe burns. Scleral and ciliary nerve effects were absent. VEGF mRNA was localized primarily in the ganglion cell layer but was also found in the inner nuclear layer. In the untreated eye, an increase in VEGF mRNA was detected at the peripheral edge of the vascularized retina anterior to the ridge. In the laser-treated eye, VEGF mRNA expression was dramatically upregulated in the ganglion cell layer in areas adjacent to laser burns. CONCLUSIONS: VEGF mRNA was found to be elevated in the peripheral, avascular retina of the untreated eye, consistent with the hypothesis that retinal hypoxia stimulates VEGF expression. In the treated eye with recurrent ROP, VEGF mRNA was not detected in the photocoagulated areas of retina but was increased between laser scars. This finding confirms the results of prior animal studies and validates the use of these models.
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ranking = 1
keywords = retinopathy
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6/69. Diode laser photocoagulation for retinopathy of prematurity: a histopathologic study.

    Laser photocoagulation has largely supplanted cryotherapy as an effective treatment for retinopathy of prematurity. This case describes the ocular histopathologic findings of a pair of eyes in a severely premature male infant treated with diode laser photocoagulation for bilateral stage 3 retinopathy of prematurity (ROP) for 360 degrees in zone 1 with severe plus disease. The right eye responded to treatment; the left eye developed persistent vitreous hemorrhage and total retinal detachment. The histopathologic examination of laser burns in the right eye disclosed segmental areas of chorioretinal scarring with retinal atrophy and gliosis, loss of RPE and extensive atrophy of the choroid and its vasculature, which involved both the choriocapillaris and larger vessels. The left eye had iris neovascularization, a chronic organized vitreous hemorrhage and a totally detached retina. The histopathologic findings in an eye of a premature infant with threshold ROP treated with diode laser photocoagulation resembled those reported after transsceral cryotherapy. Diode laser photocoagulation may produce less severe chorioretinal damage.
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ranking = 1.2
keywords = retinopathy
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7/69. Acute gastric dilatation complicating the use of mydriatics in a preterm newborn.

    A 2-month-old girl who had been born at 27-weeks' gestation was admitted for her screening examination for retinopathy of prematurity and given two drops each of cyclopentolate 0.5% and phenylephrine 2.5%. Approximately 2 h after completion of the examination, the infant had episodes of apnoea and vomiting. She was noted to be distended, and an abdominal radiograph demonstrated acute gastric dilatation. Apnoea, vomiting and distension resolved after 18 h and a repeat abdominal radiograph demonstrated resolution of the gastric dilatation.
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ranking = 0.2
keywords = retinopathy
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8/69. Ultrasound biomicroscopy in the screening of retinopathy of prematurity.

    PURPOSE: To report the use of ultrasound biomicroscopy in the screening of two premature infants with retinopathy of prematurity. methods: Observational case reports. A premature infant born at 26 weeks' gestation had bilateral stage II, zone II retinopathy of prematurity at 36 weeks' gestation and was imaged with ultrasound biomicroscopy. A second premature infant born at 25 weeks' gestation developed subthreshold stage III, zone II retinopathy of prematurity in the right eye and was imaged with ultrasound biomicroscopy. RESULTS: In case 1, ultrasound biomicroscopy imaged a ridge present nasally in zone II of the right eye, consistent with stage II retinopathy of prematurity. In case 2, ultrasound biomicroscopy imaged a neovascular frond present nasally in zone II of the right eye, consistent with stage III retinopathy of prematurity. CONCLUSION: Ultrasound biomicroscopy may be useful for screening retinopathy of prematurity, when optical methods fail to visualize the peripheral retina.
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ranking = 2
keywords = retinopathy
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9/69. Screening for retinopathy of prematurity.

    AIM: A cross sectional (prevalence) study was performed to assess the usefulness and sensitivity of commonly employed criteria to identify infants for routine ophthalmoscopic screening for retinopathy of prematurity (ROP). methods: At a tertiary care centre between 1 January 1992 and 30 June 1998, experienced vitreoretinal specialists screened 438 premature infants for ROP. Retinal maturity and the presence of ROP were determined by indirect ophthalmoscopic examinations. RESULTS: Of the eligible infants surviving 28 days, 276 (91.7%) of 301 infants with birth weights
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ranking = 1
keywords = retinopathy
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10/69. Retinal neovascular markers in retinopathy of prematurity: aetiological implications.

    AIM: (1) To determine if expression of the blood-tissue barrier associated glucose transporter GLUT1 is preserved by the neovasculature of retinopathy of prematurity (ROP), in contrast with the reported loss of GLUT1 expression in preretinal vessels of proliferative diabetic retinopathy. (2) To compare the vascular immunophenotype of ROP to juvenile haemangioma, another perinatal neovascular disorder that has recently been shown to express placental type vascular antigens, including GLUT1 and Lewis Y antigen. methods: A retrospective case report was carried out. Immunoreactivities for GLUT1 and Lewis Y antigen were assessed in a human eye with stage 3 ROP and compared with those in a control (paediatric) eye. The presence or absence of endothelial GLUT1 and Lewis Y immunoreactivity was determined in preretinal and intraretinal vessels. RESULTS: Immunoreactivity was positive for GLUT1 and negative for Lewis Y in the intraretinal and preretinal neovasculature of the ROP affected eye and in the normal retinal vessels of the control eye. CONCLUSIONS: Retention of immunoreactivity for GLUT1 distinguishes ROP from proliferative diabetic retinopathy. Furthermore, absence of Lewis Y antigen co-expression distinguishes ROP from juvenile haemangioma, a perinatal form of GLUT1 positive neovascularisation that has recently been linked to placental vasculature.
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ranking = 1.4
keywords = retinopathy
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