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At least 199 records · Page 11Linked to original sources
PubMed1979

Simple glaucoma before the age of 30 years.

'Juvenile glaucoma' is a meaningless term which should not be used. Glaucoma in young subjects can be hydrophthalmus, secondary or simple glaucoma. In this paper, early simple glaucoma is described in 15 patients (8 males, 7 females) below the age of 30 years, starting in some subjects already at 8 years of age. Inheritance was dominant in 6 patients. Anticipation could be shown in 2 pedigrees through 4 and 5 generations, respectively. The course of the disease was severe in 8 patients, with intraocular pressures over 40 mm Hg and/or heavy functional loss. There were usually no subjective symptoms. The chamber angles were gonioscopically normal in all patients. Miotics were effective but did not normalize the intraocular pressure. Surgical treatment is discussed.

Adolescent↗
PubMedDate not supplied

Phthisis bulbi and buphthalmos as presenting signs of retinoblastoma: a report of two cases and literature review.

PURPOSE: To report two cases of bilateral retinoblastoma (RB) with unusual presentations. METHODS: The medical records of 321 patients from the Retinoblastoma Referral Center in Siena were reviewed. A total of 111 patients had bilateral RB, 2 of them presenting with phthisis bulbi and buphthalmos. Both patients underwent bilateral enucleation. Clinical features, imaging studies, and histopathology were reviewed. RESULTS: These 2 cases represent 0.62% (2/321) in our series. Histopathology did not reveal viable tumor cells in the phthisical eyes; in both buphthalmic eyes the tumor was active, infiltrating the choroid and optic nerve. CONCLUSIONS: Phthisis bulbi and buphthalmos are unusual presenting signs of RB. This very rare combination of these two signs in different eyes of the same patient is probably due to a delay in diagnosis.

Child, Preschool↗
PubMedDate not supplied

Congenital glaucoma associated with an arginase deficit: a case report.

PURPOSE: Argininemia is a rare congenital disease caused by deficiency. We present here a case in which argininemia and buphthalmos are associated. CASE REPORT: This female patient, who died at eighteen, had intraocular pressure correlated with her blood levels or arginine. Hyperammoniemia causes a change in pH that could explain an increase in aqueous humor (from the ciliary body). Ammonium ions could also damage the matrix of the trabecular meshwork. CONCLUSIONS: The association of argininemia and buphthalmos have never been described. This might be because of the different expressions of the disease, or because nobody has ever looked for ocular hypertension in these patients unless they had subjective symptoms.

Adolescent↗
PubMed1966

Lowe's syndrome.

Explore the source record for details and available documents.

Chronic Kidney Disease-Mineral and Bone Disorder↗
PubMedDate not supplied

Fibrinogen concentration in the aqueous humor of buphthalmic rabbits.

Fibrinogen concentration in the aqueous humor of buphthalmic rabbits, AXBU/J, and those of the normal parent strain AX/J are 0.36 +/- 0.14 and 0.08 +/- 0.01 mg/ml, respectively. There was no detectable fibrinogen in the aqueous humor of normal inbred strain III/J rabbits. Among the buphthalmic rabbits, elevated fibrinogen concentration in the aqueous humor was observed in two month old prodromal rabbits, indicating a small leakage of fibrinogen into the anterior chamber before the development of buphthalmia. Protein concentration in the aqueous humor was close to normal, in spite of an elevated level of fibrinogen in the young buphthalmic rabbits. Severe protein leakage was seen in only some of the old buphthalmic rabbits.

Age Factors↗
PubMed1989

Buphthalmos and progressive elephantiasis in neurofibromatosis. A report of three cases.

Two cases of buphthalmos and regional gigantism in peripheral neurofibromatosis (Von Recklinghausen disease) are described. The ocular hypertension was detected at birth and thereafter the facial hemihypertrophy, café-au-lait spots and neurofibromas. One case presented a progressive facial elephantiasis without buphthalmos. The evolution in neurofibromatous elephantiasis of these three cases is discussed.

Child↗
PubMed1984

Photoinduced buphthalmic avian eyes. 1. Continuous fluorescent light.

Numerous investigations of light induced avian buphthalmia using incandescent light have not defined an etiology. Chickens raised from 2 weeks of age in continuous fluorescent light developed shallow anterior chambers and buphthalmos within the first month of treatment. Chickens raised in continuous fluorescent light developed ocular enlargement similar to that reported in chickens raised in continuous incandescent light. Corneal diameters and axial globe lengths were an inconsistent means of documenting ocular enlargement. Iridocorneal angles were narrowed on scanning electron microscopy.

Animals↗
PubMed1985

Photoinduced buphthalmic avian eyes: II. Continuous darkness.

Chickens raised from 2 to 14 weeks of age in continuous darkness (OL:24D) developed shallow anterior chambers and buphthalmos (megaloglobus). Chickens raised in continuous darkness developed ocular changes similar to that reported in chickens raised in continuous fluorescent and incandescent light and in dim light. Corneal diameters and axial globe lengths were an inconsistent means of documenting ocular enlargement. Iridocorneal angles were narrowed on scanning electron microscopy (SEM). Intraocular pressure (IOP) was not different from control chickens. The chicken is proposed as a model to study ocular development, the effects of lid suture on infants, axial myopia, preglaucomatous changes, and possibly pharmacologic effects of antiglaucoma preparations.

Animals↗
PubMedDate not supplied

Bilateral retinal vascular hypoplasia associated with persistence of the primary vitreous: a new clinical entity?

Three infant boys with a bilateral congenital retinopathy characterized by retinal vascular hypoplasia and persistence of the primary vitreous are described. The infants were healthy and had no non-ocular physical abnormalities. None had histories of prematurity or low birth weight. Family histories were negative to similar retinopathy, and the fundi of all examined blood relatives were normal. The described congenital retinopathy is compared and contrasted with persistent hyperplastic primary vitreous, Norrie's disease, idiopathic retinal dysplasia, familial exudative vitreoretinopathy, retinopathy of prematurity, and incontinentia pigmenti.

Electroretinography↗
PubMedDate not supplied

Choroidal neurofibromatosis with congenital iris ectropion and buphthalmos: relationship and significance.

We report a case of unilateral buphthalmos in neurofibromatosis-1 without the other classical characteristics of the François syndrome (triad of unilateral buphthalmos, homolateral eyelid plexiform neuroma, and homolateral facial hemihypertrophy) and emphasize the difficulties in early diagnosis. The painful buphthalmic right eye was enucleated at the age of 13 months. Histopathology demonstrated diffuse choroidal neurofibromatosis in association with congenital iris ectropion syndrome. Cutaneous manifestations of neurofibromatosis subsequently developed in the patient and stigmata of the disease were later identified in other asymptomatic family members.

Choroid Neoplasms↗