Orbital cellulitis in an infant secondary to Coats' disease.
An 8-week-old infant had advanced Coats' disease. Six months after the Coats' disease was noted, a severe, secondary orbital cellulitis developed.
Age Factors↗
Biomedical subjects
G F Judisch
Publications and source records attributed to G F Judisch.
Chloral hydrate sedation as a substitute for examination under anesthesia in pediatric ophthalmology.
We examined more than 300 children as outpatients with orally administered chloral hydrate sedation during the past three years; 175 of them have been examined in the last year. Without chloral hydrate sedation, examination under anesthesia would have been necessary or the results would have been less than satisfactory. Orally administered chloral hydrate has a wide margin of safety and is most appropriate for office or clinic use. It may also allow accurate measurement of the intraocular pressure in infantile glaucoma.
Administration, Oral↗
Oculodentodigital dysplasia. Four new reports and a literature review.
Four new patients with oculodentodigital dysplasia (ODD) have been examined. The salient and fairly constant features of ODD appear to be (1) unique facial appearance, (2) microcornea with other inconstant ocular findings, (3) syndactyly of the hands with additional characteristic phalangeal aberrations, (4) diffuse skeletal dysplasia, (5) enamel dysplasia, and (6) trichosis. Echographic studies indicate that ODD globes have microcornea with otherwise normal dimensions. An increased number of vessels crossing the optic discs was observed in three patients from one family. The distance between the inner canthi and the medial orbital walls in three patients we studied suggests that previous reports of hypertelorism may have been illusions resulting from microcornea, small palpebral fissures, and variably present epicanthus.
Adolescent↗
Persistent pupillary membrane: successful medical management.
Persistent pupillary membranes usually undergo considerable atrophy during the first year of life. A minimum pupillary diameter of 1.5 mm is necessary for normal retinal image formation. Mydriatics, fixation pattern determination, and occlusion therapy as indicated can obviate the need for coreoplasty in patients with an extensive persistence of the pupillary membrane. We successfully treated a patient who had an almost total persistence of the pupillary membrane.
Amblyopia↗
Rieger's syndrome. A case report with a 15-year follow-up.
A 15-year follow-up examination of a boy with Rieger's syndrome showed that the anterior segment changes in this disease may be slowly progressive in the absence of glaucoma or miotic treatment. The patient also had two recently recognized systemic features of the syndrome, umbilicus cutis and hypospadias.
Adolescent↗
Retinoblastoma Y79 cell line: a study of membrane structures.
The continuous retinoblastoma cell line Y79, grown in suspension culture, has been examined by transmission and scanning electron microscopy, including freeze-fracture replica preparations. Four classes of cells were distinguishable by their size and surface characteristics. The surface structures included blebs, filopodia, lamellipodia, microvilli, and microplicae. The possible origin of a most unusual type of cell, apparently forming natural clones, is discussed.
Cell Line↗
Clinical differentiation of recessive congenital hereditary endothelial dystrophy and dominant hereditary endothelial dystrophy.
Our review of previously published reports and familial cases revealed that corneal clouding in autosomal recessive congenital hereditary endothelial dystrophy was present at birth or within the neonatal period. Further, corneal changes with time were minimal, nystagmus was often present, and there were no other signs or symptoms. Patients with autosomal dominant endothelial dystrophy usually had clear corneas early in life; corneal opacification was slowly progressive, nystagmus was infrequent, and photophobia, as well as epiphora, may have been the first indications of the dystrophy. As there is usually little or no congenital evidence of the dominant type, "infantile" or "autosomal dominant" hereditary endothelial dystrophy would be more appropriate names for the dominant variant.
Chromosome Aberrations↗
Large orbital conjunctival cyst after enucleation.
A 33-year-old man, 28 years after enucleation for a perforating injury, had a four-year history of slowly progressive proptosis of his prosthesis. Examination revealed a spherical mass filling the space normally occupied by the globe. A cyst lined by conjunctival epithelium was encountered on surgical exploration.
Adult↗
Cardiac, skeletal and ophthalmologic abnormalities in relatives of patients with the Marfan syndrome.
Nine patients with the Marfan syndrome and 40 of their first degree relatives were evaluated for the presence of cardiac, skeletal and ophthalmologic abnormalities. Aortic root dilatation and mitral valve prolapse were sought by echocardiography, and the metacarpal index was calculated from hand X-rays. Abnormalities of all the tests performed were present in all nine index cases, except for one normal eye exam. Mitral prolapse was present in thirteen relatives (33%) and aortic root dilatation in seven (18%). At least one cardiac abnormality was present in nineteen (47%) relatives. Aortic root dilatation was more common in male relatives; the incidence of mitral prolapse was approximately equal in the two sexes. Abnormal metacarpal index (greater than 8.0) occurred in fifteen of twenty-six relatives examined (58%). Ophthalmologic abnormalities were found in only four relatives. Two relatives had abnormalities of all three organ systems evaluated, five others had abnormalities of two systems, and fourteen had abnormalities of one system. We conclude that cardiac and skeletal abnormalities are demonstrable in a high percentage of first degree relatives of patients with the Marfan syndrome.
Adolescent↗
Ocular Ehlers-Danlos syndrome with normal lysyl hydroxylase activity.
We report two brothers affected with what has been called either fragilitas oculi or the Ehlers-Danlos syndrome type VI. Previously reported cases of the Ehlers-Danlos syndrome type VI showed a deficiency of lysyl hydroxylase in cultured fibroblasts. Assays of cultured skin fibroblasts from these two boys yielded normal activity of this enzyme, suggesting that there are two variants of this disease.
Adolescent↗
Four generations of arteriohepatic dysplasia.
Arteriohepatic dysplasia (AHD) is a multisystem disorder which is characterized by liver, heart, eye, and bony abnormalities. A characteristic facies is also present. The etiology is not clear, and some authors have favored an in utero toxin or infection as the cause. Other reports have shown parent-to-child transmission suggesting an autosomal dominant form of inheritance. This study documents the presence of AHD characteristics in four successive generations of a single kindred. Overall, 15 of 24 members had at least some characteristics of AHD. In addition to the usual findings, renal disease, a small flat face on lateral X-ray, and mild conductive hearing loss were frequently noted in this kindred. Physiological studies on one member revealed a normal bromosulfophthalein Tm and S but a late rise in plasma bromosulfophthalein and indocyanine green. This study strongly supports an autosomal dominant form of transmission for the AHD syndrome. The ease of determining the eye, bone, and heart abnormalities should simplify genetic studies of other patients' families. In addition, because these characteristics are recognizable at birth, documenting their presence in a jaundiced neonate should spare the infant unnecessary and potentially dangerous surgery to rule out extrahepatic obstruction, especially if one or more family members demonstrate the same abnormalities.
Abnormalities, Multiple↗
Unusual case of acute orbital cellulitis.
A three and one-half year-old female who presented with orbital cellulitis and leukocoria is described. Enucleation was performed as the eye was microophthalmic, blind, and painful. Histopathologic study revealed uveitis with total retinal detachment. The etiology of the retinal detachment was indeterminable. The differential diagnosis of orbital cellulitis in children is reviewed.
Cellulitis↗
Megalocornea: a clinical and echographic study of an autosomal dominant pedigree.
A family with autosomal dominant megalocornea was studied clinically and echographically. The anterior chamber angles, intraocular pressures and axial lengths were all normal. These findings strongly support the dualist proposition which contends that etiologically, megalocornea and congenital glaucoma are distinct.
Adult↗
Post-radiation retinal infiltrates simulating recurrent retinoblastoma: a clinical and pathologic case report.
An 8-month-old boy was found to have a sporadic unilateral stage IIa retinoblastoma, extending inferiorly and temporally from the right fovea. Within 5 weeks of diagnosis the tumor was treated with 4500 rads fractionated over 20 treatments. Examination under anesthesia (EUA) 1 month later revealed unequivocal clinical and echographic evidence of tumor regression. EUA 3 months after completion of radiation therapy revealed continued tumor regression with no evidence of new tumor formation. The third EUA, performed 6 months after radiation treatment, showed that multiple discreet gray-white retinal infiltrates had developed, predominantly in the area immediately below the regressed tumor. Because of concern that these lesions represented viable retinoblastoma, the eye was enucleated. Pathologic examination of these lesions revealed them to be collections of foamy cells containing melanin granules suggestive of pigment epithelial origin.
Diagnosis, Differential↗
X-linked congenital stationary night blindness with myopia and nystagmus without clinical complaints of nyctalopia.
Seven of eight patients presented initially or were followed for decreased acuity and nystagmus without complaints of night blindness. The diagnosis of congenital stationary night blindness was established with electroretinogram and dark adaptation testing. Careful electrodiagnostic testing is needed to provide accurate genetic counseling. Two patients showed pupillary constriction to darkness which is a sign of retinal disease in young patients.
Adolescent↗
Endophytic vs exophytic unilateral retinoblastoma: is there any real difference.
Explore the source record for details and available documents.
Choroid Neoplasms↗
Ophthalmic features of Alagille syndrome (arteriohepatic dysplasia).
Eight patients with Alagille syndrome (AGS) are reported. In addition to previously reported findings of posterior embryotoxon, pigmentary retinopathy, and choroidal folds, new findings include decreased axial eye lengths, small corneal diameters, and shallow anterior chambers. Optic disc swelling was noted ophthalmoscopically and abnormally increased orbital subarachnoidal fluid was detected through measurements of the arachnoidal diameters with standardized echography.
Adolescent↗