Congenital lacrimal fistula associated with Down's syndrome.
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Biomedical subjects
Publications and source records attributed to J D Morin.
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A 6-month-old female infant with biopsy-proved Hirschsprung's disease had associated sector heterochromia of the irides. The association between sector heterochromia and Hirschsprung's disease has been previously reported and both conditions have been ascribed to neural crest defects. Histologic characteristics of the ocular involvement have not previously been reported, to our knowledge. Histopathologic examination of the globes revealed decreased iris stroma, decreased pigmentation in the anterior stroma, and reduced numbers of pigment-producing cells in the affected areas. Both the ocular and gastrointestinal findings reflect abnormalities in tissues of neural crest origin.
BACKGROUND: Many authorities believe that the finding of retinal hemorrhages in a child younger than 3 years of age with a history of head trauma, in the absence of an obvious cause for the injury, is pathognomonic of child abuse. To date, no studies have examined the prospective retinal examination of children who have had head trauma. The authors undertook such a study because the presence of retinal hemorrhage from any head trauma in children may have medicolegal diagnostic significance in differentiating accidental from nonaccidental trauma. METHODS: Seventy-nine children younger than 3 years of age, each of whom experienced head injury, underwent an ophthalmologic assessment, which included a dilated funduscopic examination. RESULTS: Seventy-five children sustained accidental head injuries and had normal funduscopic examinations. Three children had nonaccidental head injuries and all were found to have varying degrees of retinal hemorrhages. One child, with a normal fundus examination, had injuries that were of indeterminate cause. CONCLUSION: The finding of retinal hemorrhages in a child with a head injury suggests a nonaccidental cause.
PURPOSE: Seventy premature infants 25 to 37 weeks' postconceptional age were examined during their first week of life to determine the correlation of corneal diameter, axial length, and intraocular pressure with gestational age and birth weight. METHODS: Corneal diameter measurement was determined with corneal templates, total axial length with standardized A-scan ultrasound, and intraocular pressure with a Tonopen II tonometer. RESULTS: Corneal diameter and total axial length showed parallel linear increases from 6.2 mm to 9.0 mm and 12.6 mm to 16.2 mm, respectively; however, no significant correlation was found between intraocular pressure and gestational age or birth weight. The mean intraocular pressure was 10.3 mmHg (standard deviation, 3.5). CONCLUSION: Normative values are established for corneal diameter and total axial length as they relate to birth weight and gestational age, and a mean and standard deviation for intraocular pressure in the premature newborn. These values will aid the ophthalmologist in assessing ocular dimensions in premature infants.
Congenital dacryocystocoele occurs when the nasolacrimal drainage apparatus in the newborn has concomitant blocks at the level of the junction of the common canaliculus with the lacrimal sac and at the distal end of the nasolacrimal duct. This results in a typical pink or blue swelling in the region of the medial canthus. Spontaneous resolution is common, although dacryocystitis may supervene. Treatment should be conservative unless dacryocystitis occurs, or intranasal extension coexists. A series of seven consecutive cases is presented and a management plan for the neonate with congenital dacryocystocoele is proposed.
Congenital lacrimal fistulae are developmental anomalies of the lacrimal apparatus that are usually symptomatic, frequently causing epiphora and occasionally causing fistulitis or dacryocystitis. They may be associated with other abnormalities of the lacrimal system or with systemic anomalies. Complete excision alone, or in combination with nasolacrimal intubation and/or dacryocystorhinostomy is recommended for treatment.
To evaluate the efficacy of screening ophthalmologic examinations in high-risk children, we reviewed the medical records for all patients hospitalized from 1985 through 1989 at The Hospital for Sick Children, Toronto, Ontario, who underwent ophthalmological consultation to rule out endogenous fungal endophthalmitis (n = 176). The patients were divided into groups: Group 1 (n = 47), those with deep-tissue fungal infection, and Group 2 (n = 129), those at risk for invasive fungal disease. Group 2 was subdivided further into two subgroups: Group 2a (n = 48), those with evidence of superficial fungal colonization (positive fungal culture) but no deep-tissue involvement, and Group 2b (n = 81), those with no evidence of fungal colonization (negative fungal culture). Of these 176 patients, 7 were diagnosed with endogenous fungal endophthalmitis: 6 from Group 1, 1 from Group 2a, and 0 from Group 2b. We found a significant association between the development of endogenous fungal endophthalmitis and the status of the fungal culture result (P less than .005). The odds ratio indicated the risk of endogenous fungal endophthalmitis in Group 1 patients with deep-tissue infection was at least 19 times that of Group 2 at-risk patients. The risk of endogenous fungal endophthalmitis in Group 1 patients was at least 7 times that of Group 2a colonized patients and 12 times that of Group 2b patients with no positive fungal culture. Our study confirms the necessity of careful dilated ophthalmoscopic examination in patients with invasive fungal disease and suggests screening for those at-risk patients with superficial fungal colonization.(ABSTRACT TRUNCATED AT 250 WORDS)
Between 1979 and 1988, 85 penetrating keratoplasty procedures were performed in 54 patients aged 1 month to 18.2 years at the Hospital for Sick Children, Toronto. The minimum length of follow-up was 3 months. A clear transplant was obtained in 27 eyes: 7 of 16 eyes with Peter's anomaly, 0 of 8 eyes with congenital glaucoma, 2 of 5 eyes with herpes simplex keratitis, 6 of 8 eyes with corneal dystrophy and 12 of 17 eyes with traumatic corneal scars. The most recent visual acuity was best in the trauma and dystrophy groups and worst in the congenital glaucoma group. Visual acuity results were better in older children and were fair in younger children and those with postoperative complications. Although penetrating keratoplasty is more difficult in children than in adults, it has a reasonable chance of success. However, the poor outcome in the congenital glaucoma group indicates that the procedure is not warranted in such patients.
We report three cases of Hurler's disease in which glaucoma developed in early childhood. We draw attention to the fact that glaucoma may be a commonly unrecognised early complication of this condition.
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Using ultrasonography, the authors made biometric measurements in 92 eyes of 47 patients with primary angle-closure glaucoma (ACG) and calculated the lens thickness/axial length factor (LAF) for each patient. Graphical and statistical analysis suggest the presence of three distinct clinical groups of patients with primary ACG. ACG shows a dynamic clinical picture; it starts in an eye which is initially uncompromised (group A) and progresses to one of two identifiable advanced stages (groups B and C). Accurate diagnosis by following LAF values in the earliest stage of the disease could prevent its development into the later stages.
Biometric measurements were made by ultrasonography in 44 left eyes of 44 patients with primary angle-closure glaucoma. We defined the ratio between the lens thickness and the axial length (lens thickness/axial length factor) as a representative and unifying unit for biometric assessment of the eye. This factor defined the relationship between the lens, iris, and cornea and thus the status of the angle. Lens thickness/axial length factor values were found to be age dependent and were greater than normal for most age groups with angle-closure glaucoma. The mean normal value was 1.91 +/- 0.44; the mean values for patients in different age groups with angle-closure glaucoma ranged from 1.87 +/- 0.11 to 2.39 +/- 0.17.
Thirty-seven premature infants were examined without anesthesia to determine intraocular pressure (I.O.P.) and corneal diameter (C.D.) measurements. The anterior segment of the premature infant was examined and the presence of Bell's phenomenon was recorded. Measurements were done with a Perkin's applanation tonometer, calipers and clear corneal diameter templates. The systolic blood pressure, weight, length and postconceptional age were also recorded. Correlations of these factors were done to determine interdependency of these parameters. The mean values for I.O.P. were 18.04 mmHg for O.D. and 18.62 mmHg for O.S. The mean difference was statistically significant. Mean values for corneal diameters were 8.18 mm O.D. and 8.15 mm O.S. Statistically significant correlations occurred between I.O.P. with C.D. and between C.D. with weight, length and postconceptional age. Techniques for accurate measurement in this age group require particular attention to adequately-sized instrumentation to accommodate small palpebral fissures and corneas.
The corneal diameter was recorded and the ocular axial length measured by A-scan ultrasonography in 31 eyes of 17 children (ages 0.05 to 7.0 years) who had undergone or were about to undergo surgery for primary congenital glaucoma. These measurements were also done in 60 normal eyes of 33 children (ages 0.20 to 9.6 years) undergoing nonophthalmic surgery. Both measures were usually greater than normal in the glaucomatous eyes. However, the corneal diameter was more sensitive than the axial length in identifying congenital glaucoma. The axial length measurement did not provide additional useful information for any of the eyes. We conclude that the corneal diameter is a more reliable guide than the axial length in the assessment of congenital glaucoma. A transparent plastic gauge for rapid and accurate measurement of the corneal diameter is described.
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We studied the healing of trabeculectomies in rabbits with photographs, fluorescein filtration and histological sections. Initially all except 1 developed proven filtration. However, vigorous fibroblastic proliferation (and collagen production) from the subconjunctiva, uvea and sclera combined to close off the fistula by day 14. Groups of eyes treated with 8 mg of prednisolone injected subconjunctivally at operation showed slightly delayed wound healing but by day 30 there was little difference between treated and untreated groups. The steroid treated eyes had complications ranging from endophthalmitis in 1 eye to tissue necrosis in 3 eyes.
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