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I Kremer. 1999. Inclusive references.. https://doi.org/10.1016/s0886-3350(99)80110-2

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Growth hormone deficiency (GHD): a new association in Peters' Plus Syndrome (PPS).

We report a case of a girl with clinical features of Peters' Plus Syndrome (PPS) (association of anterior eye chamber defects; peculiar facies; cleft lip/palate; brachymelia; developmental delay; growth retardation) and documented growth hormone deficiency (height -3.5 SDS at chronological age 5 years 8 months; low growth factors; bone age delay; growth velocity 4.4 cm/year (<3rd centile); and peak growth hormone levels of 1.7 and 4.7 ng/ml by clonidine and insulin provocative testing, respectively). Treatment with recombinant human growth hormone (0.3 mg/kg/week) resulted in a dramatic increase in growth velocity, increasing the height from -3.5 to -1.5 SDS over 2.3 years of therapy, indicative of an excellent response. Growth retardation is a known association in PPS: a condition that includes other midline facial defects. This case supports a role for GHD in the pathogenesis of the short stature observed in these children; demonstrates the efficacy of GH treatment; and further reinforces the relationship of pituitary anomalies with common congenital defects.

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Intracorneal hematoma with descemet membrane detachment after viscocanalostomy.

PURPOSE: To describe a case of intracorneal hematoma with Descemet membrane detachment after viscocanalostomy. DESIGN: Interventional case report. METHODS: A 63-year-old with uncontrolled primary open-angle glaucoma underwent viscocanalostomy. During the introduction of hyaluronate 2.3% under the superficial flap, a limited lysis of Descemet membrane was observed. RESULT: Slit-lamp biomicroscopy showed an intracorneal hematoma with Descemet membrane detachment 1 day after viscocanalostomy. The creation of an intentional break of Descemet membrane and descemetopexy using sulfur hexafluoride gas were performed. The cornea regained transparency after surgery. CONCLUSIONS: Intracorneal hematoma with Descemet membrane detachment is a possible complication of viscocanalostomy.

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Retinoblastoma: Abnormal gadolinium enhancement of anterior segment of eyes at MR imaging with clinical and histopathologic correlation.

PURPOSE: To evaluate abnormal gadolinium enhancement of the anterior segment of eyes harboring retinoblastoma at magnetic resonance (MR) imaging and correlate this finding with clinical and histopathologic information. MATERIALS AND METHODS: Three neuroradiologists examined 46 eyes with 34 retinoblastomas in 25 children on gadolinium-enhanced T1-weighted orbital MR images obtained shortly after contrast material injection for evidence of abnormally high signal intensity in the anterior segment. Twenty-two of the 34 affected eyes were enucleated, and six of these 22 eyes were treated with preenucleation adjuvant therapy. Thus, correlation of the clinical, MR imaging, and histopathologic findings in 16 eyes was performed. Statistical analysis was performed with nonparametric methods (Fisher exact test). P <.05 indicated a statistically significant difference. RESULTS: Fourteen of the 34 affected eyes showed abnormal gadolinium enhancement of the anterior segment. Regarding the 16 eyes evaluated for statistical analysis, a significant correlation (P =.011) between abnormal gadolinium enhancement of the anterior segment and histopathologically documented optic nerve infiltration was noted. A trend toward an association between abnormal enhancement and elevated intraocular pressure (P =.215), tumor growth beyond the equator at MR imaging (P =.125), and histopathologically proved iris neoangiogenesis (P =.182) also was noted. Histopathologic evidence of optic nerve and/or choroid infiltration correlated significantly (P =.001; sensitivity, 100% [nine of nine eyes]; specificity, 86% [six of seven eyes]) with abnormal enhancement. CONCLUSION: Abnormal gadolinium enhancement of the anterior segments of eyes harboring retinoblastoma seems to indicate more aggressive tumor behavior.

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