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Biomedical subjects

M J Greenwald

Publications and source records attributed to M J Greenwald.

At least 37 records · Page 2Linked to original sources

Optic gliomas in children with neurofibromatosis type 1.

To determine the frequency and natural history of tumors of the optic nerves and chiasm in patients with neurofibromatosis type 1, we obtained computed tomographic scans of 65 children who had no known visual or ocular abnormalities before their initial evaluation. Optic gliomas were detected in 10 children (15%). The median age of children with gliomas was 4.3 years (mean 5.8 years, range 9 months to 21 years). Three children (30%) had isolated, unilateral tumors, three (30%) had bilateral tumors, and four (40%) had involvement of the optic chiasm and of one or both nerves. Definite abnormalities of vision were found in only two children (20%). Five additional children were referred to the clinic after evaluation of ophthalmologic complaints led to the diagnosis of neurofibromatosis type 1: three had unilateral exophthalmos and two had plexiform neurofibromas of the eyelid with associated glaucoma. Ipsilateral optic gliomas were found in all five children; one child also had a contralateral tumor. Optic gliomas are commonly identified in young children with neurofibromatosis type 1 who have no ocular or visual abnormalities. Optic nerve gliomas may be associated with plexiform neurofibromas of the eyelid and glaucoma.

Adolescent↗

Management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children.

A retrospective analysis of 21 consecutive patients hospitalized with either Stevens-Johnson syndrome or toxic epidermal necrolysis was carried out to assess morbidity and mortality rates and to establish the value of a specific management practice. Fourteen children with Stevens-Johnson syndrome and seven with toxic epidermal necrolysis were cared for at the Children's Memorial Hospital, Chicago, between 1978 and 1988. All were managed in a well-staffed medical ward or, when necessary, in the pediatric intensive care unit. Supportive measures included reverse barrier isolation, intravenous fluids and nutritional support, meticulous skin care, early detection and treatment of infection, and daily ophthalmologic examination. No patient was treated with systemic steroids. The mortality rate was zero. Eye complications, consisting of dry eyes or mild chronic symblepharon, were the most significant long-term sequelae.

Adolescent↗

Orbital roof fractures in childhood.

The authors documented 36 orbital roof fractures in 32 children over a 5-year period. With the aid of computed tomography (CT), 16 fractures were classified as isolated and 20 were found to be associated with more extensive damage to the skull. Isolated fractures typically occurred in younger children (mean age, 2.8 years) after relatively minor trauma, most often a fall from a height of less than 10 feet. Birth trauma was the cause in one case. Three fourths of the isolated fractures were linear; with extensive trauma, the orbital roof was usually comminuted. Upper eyelid hematoma characteristically developed hours after the traumatic event. There were no significant chronic disturbances of vision, motility, or lid function. Acute neurologic concerns were common, and two patients died, but full recovery was otherwise the rule. In two cases, late development of ocular pulsation led to CT demonstration of encephaloceles. Both of these children had comminuted roof fractures with displacement of bone fragments into the orbit. The authors conclude that fracture of the orbital roof is a common but frequently overlooked occurrence in childhood. Anatomic factors may account for differences in the effect of frontal impact at different ages.

Accidental Falls↗

Orbital roof fractures in the pediatric population.

Twenty-three patients aged 3.3 +/- 1.6 years (mean +/- SD) presented between January of 1984 and September of 1987 with fronto-orbital trauma resulting in fractures of one (N = 20) or both (N = 3) orbital roofs. All patients had computed tomography (CT) with axial and coronal sections that revealed three fracture patterns of the orbital roof (nondisplaced, superiorly displaced, and inferiorly displaced fractures). Orbital dystopia was exhibited in 35 percent (N = 8) of the patients. Exophthalmos was noted in 61 percent (N = 14) of the patients. Only 30 percent of the patients (N = 7) sustained associated maxillofacial fractures. Eight percent of fractures exhibited orbital encephaloceles. All patients lacked frontal sinus pneumatization. The majority of children with orbital roof fractures do not exhibit concomitant facial fractures. CT utilizing both axial and coronal sections is valuable in defining the extent and pattern of the fracture as well as in identifying associated neurologic injuries. Large, displaced orbital roof fractures, which occurred in 3 of 13 patients with displaced fractures in our series, should undergo early reduction to avoid late development of encephalocele.

Child, Preschool↗

Clinical suppression and amblyopia.

In individuals with abnormal binocular vision, such as strabismics and anisometropes, it is common for all or part of one eye's view to be suppressed so binocular confusion and diplopia are eliminated. We examined the relation between the depth of suppression (the amount by which the monocular contrast increment threshold for an eye was elevated by stimulation in the contralateral eye) and the degree of amblyopia (difference in monocular contrast thresholds for the two eyes). There was a significant negative correlation between suppression and amblyopia, so that clinical suppressors with no amblyopia exhibited deep suppression (ie, large threshold elevation) while observers with amblyopia exhibited weaker or no suppression. This negative correlation was found when the two eyes viewed orthogonally oriented contours as well as identically oriented contours. These results suggest that when an eye is amblyopic there is no longer a need for strong suppression of that eye by the contralateral eye.

Amblyopia↗

Retinoblastoma and simulating lesions: role of CT and MR imaging.

Diagnosis of retinoblastoma is one of the most challenging problems of pediatric ophthalmology and radiology. It must be differentiated from numerous simulating lesions. Accurate diagnosis by means of ultrasonography, CT, and MR is essential for prompt treatment and prevention of metastasis. The CT and MR findings in 45 patients are presented in this article in order to evaluate and compare the usefulness of these two imaging techniques in the diagnosis of retinoblastoma.

Calcinosis↗

Selective losses in binocular vision in anisometropic amblyopes.

Human anisometropic amblyopes typically exhibit reduced contrast sensitivity in the amblyopic eye, especially at higher spatial frequencies. We determined whether this spatial frequency selective loss in contrast sensitivity is accompanied by selective losses in binocular function. Binocular summation (the improvement in one eye's detection performance produced by a subthreshold pattern presented to the fellow eye) was measured at several spatial frequencies. Normal observers exhibited equivalent binouclar summation at all spatial frequencies, whereas all anisometropic amblyopes exhibited normal summation at low spatial frequencies but none at high spatial frequencies. Stereoacuity (minimum resolvable disparity) was also measured as a function of spatial frequency. For normal observers, stereoacuity was best at the highest spatial frequency; for anisometropes stereoacuity was normal at low spatial frequencies, subnormal at intermediate spatial frequencies, and unmeasurable at higher spatial frequencies. Anisometropia may represent a form of selective binocular deprivation that affects neural mechanisms underlying binocular summation and stereopsis.

Adult↗

Traumatic retinoschisis in battered babies.

Five infants who were victims of physical abuse had extensive bilateral retinal hemorrhages on initial evaluation and subsequently developed signs of permanent retinal damage. None showed external evidence of trauma to the eyes. Vitreous hemorrhage developed after a delay of several days or more in three cases that were followed closely from the time of the traumatic incident. In several eyes, apparent intraretinal blood-filled cavities were seen acutely in the macular region and elsewhere. Late scarring of the macula typically had a cystic or crater-like configuration. Electroretinography showed loss or reduction of the positive B-wave with preservation of the negative A-wave in every case. We propose that splitting of the retina resulting from the direct mechanical effects of violent shaking was responsible for all of these findings.

Child Abuse↗

Sickle cell hyphema with secondary glaucoma in a non-black patient.

A 5-year-old Mexican-American boy developed marked elevation of intraocular pressure in association with a small traumatic hyphema. Laboratory studies prompted by this clinical presentation revealed that the child had sickle trait. Pressure returned to normal after surgical evacuation of blood from the anterior chamber. Sickle cell hemoglobinopathy should be considered in the differential diagnosis of secondary glaucoma following blunt ocular trauma even in non-black patients.

Anemia, Sickle Cell↗

SBS and SIDs.

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Child Abuse↗

Cicatricial pemphigoid in a 6-year-old child: report of a case and review of the literature.

Cicatricial pemphigoid has been documented previously in only four patients under the age of 20 years. We report a 6-year-old male who had erosions of the oral and genital mucosa, conjunctivitis, hoarseness, dysphagia, recurrent vomiting, and weight loss. Upper airway obstruction due to a chronically inflamed and scarred epiglottis necessitated tracheostomy. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct and indirect immunofluorescence confirmed a diagnosis of cicatricial pemphigoid. Therapy was begun consisting of prednisone, 2 mg per kg per day, in combination with dapsone, 2 mg per kg per day. He did not improve until the prednisone dosage was increased to 4 mg per kg per day.

Adolescent↗

Ocular manifestations of the neurocutaneous syndromes.

Ocular involvement occurs frequently in the disorders known as neurocutaneous syndromes or phakomatoses. Recognition of characteristic eye lesions in the context of related skin and systemic abnormalities may be crucial to making the diagnosis of a neurocutaneous syndrome. Ocular disease in these conditions may be the cause of serious morbidity, leading to blindness or disfigurement, but successful treatment is often possible. All clinicians involved in the care of patients with neurocutaneous syndromes should have some familiarity with their ophthalmologic aspects. This review deals with ocular manifestations of neurofibromatosis, tuberous sclerosis, Sturge-Weber syndrome, von Hippel-Lindau disease, ataxia telangiectasia, and Bloch-Sulzberger syndrome. Clinical signs and symptoms, differential diagnosis, and natural history are considered in detail, with brief discussion of pathophysiology and management. No specialized knowledge of eye disease in general is presumed.

Ataxia Telangiectasia↗