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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cornelia de Lange Syndrome (CDLS) is characterized by mental retardation, growth retardation, skeletal anomalies, and a characteristic facies. We examined 22 children with CDLS to ascertain the relative frequencies of oculofacial and ophthalmologic abnormalities. We confirm prior reports of the frequent findings of brow hypertrichosis, synophrys, and long arcuate eyelashes. In addition we documented the association with ptosis, nystagmus, and high myopia. Contrary to previous reports, we did not find blue sclera or corneal, pupillary, or retinal disorders to be part of this syndrome. Hypertelorism and telecanthus were found, but they were dependent on the method of calculation. Two children had optic nerve pallor. We also report the new findings of poor macular reflex, chin lifts induced by ptosis, hypertropia, and nasolacrimal duct fistula.
J Pediatr Ophthalmol Strabismus
PMID:Ophthalmologic findings in the Cornelia de Lange syndrome. 234 18

Toxemic changes in the maternal fundus and their relationship to fetal prognosis have been studied. A study of the neonatal fundus in maternal toxemia revealed a 76.67% incidence of vascular changes similar to those seen in the maternal fundus. Arteriolar spasm, focal or generalized, was present in 23 of the 30 neonates seen. A pallor of the disc was present in six cases. Four infants, 13.33%, had either extensive dot and blot retinal hemorrhages with soft exudates, or a serous retinal detachment. The last four neonates died within the first postnatal week. This work highlights the fact that retinal vascular decompensation mirrors similar changes in the cardiopulmonary or cerebral circulation and should be considered as a determinant of the fetal prognosis in maternal toxemia.
J Pediatr Ophthalmol Strabismus
PMID:The neonatal fundus in maternal toxemia. 262 47

From 6 months of age on this girl experienced frequent episodes of hemiplegia involving both sides of the body and lasting up to 8 days. The attacks were often precipitated by tonic deviation of the head and/or the eyes to one side and nystagmus. At this stage the girl used to cry. Squinting, tonic stiffening of body and extremities, and dystonic posturing also occurred. Autonomic dysfunctions such as paleness of the skin, sweating, respiratory embarrassment, tachycardia, and mydriasis were associated features of the attacks. Motor and mental development of the girl is delayed. Improvement concerning severity, duration and frequency of the attacks has been achieved by permanent treatment with flunarizine in combination with acetazolamide and acetylsalicylic acid. If the child falls asleep immediately after rectal application of chloral hydrate at the onset of an attack there is no hemiplegia after awakening.
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PMID:[Alternating hemiplegia in childhood. Clinical report and single photon emission computed tomography study]. 326 84

An ophthalmic evaluation was undertaken in 250 consecutive cases of mental retardation where no specific etiological diagnosis could be established, ie, "idiopathic" mental retardation. Visual impairment was noted in 59 of these patients, and was mainly due to primary optic atrophy, besides cortical blindness, strabismus, nystagmoid eye movements, refractive errors and various associated congenital ocular anomalies. It was encouraging to find that 191 children had active vision, including 10 patients with definite disc pallor. The importance of useful vision in these retarded children cannot be overemphasized. This is probably the first report in the literature analyzing the ocular changes and their significance in a fairly large series of idiopathic mental retardation.
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PMID:The eye in idiopathic mental retardation. 358 8

New criteria for diagnosing congenital stationary night blindness include loss of the oscillatory potentials in the photopic and bright-flash dark-adapted electroretinogram, and atrophy or dysplastic changes, or both, in the optic nerve head. Ten patients (seven male and three female, ranging in age from 6 to 19 years) had typical findings of congenital stationary night blindness including congenital nonprogressive nyctalopia, no pigmentary retinopathy, and full visual fields consistent with myopia. Visual acuities ranged from 20/30 to 20/60, though one patient had a visual acuity of 20/200. Most patients had histories of strabismus. The photopic electroretinograms were subnormal. Of the male patients, five had tilted optic disks with temporal portions of the nerve missing, and two had misshapen nerve heads. The three female patients had pallor of the optic disk without evidence of tilt.
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PMID:Loss of electroretinographic oscillatory potentials, optic atrophy, and dysplasia in congenital stationary night blindness. 660 90

A six-year-old Bantu child presented with a mass in the right orbital region, proptosis, and moderate pallor of the optic disk. Orbital exploration revealed an unsuspected hydatid cyst. The characteristic histopathologic features of the organism Echinococcus granulosus are emphasized.
J Pediatr Ophthalmol Strabismus
PMID:Orbital hydatid cyst. 720 19

Adjustable suture surgery has become a popular method of strabismus correction in those cases in which results are less predictable, such as reoperations, thyroid ophthalmopathy, and blind eyes. Complications related to adjustable strabismus surgery are, in general, no different than those related to standard surgical techniques. We noticed a subgroup of patients who experienced significant vaso-vagal responses (V-VR) during postoperative adjustment and sought to discover a method of identifying these patients prior to adjustment. Sixty patients were studied prospectively from July 1991 to July 1992. All patients had surgery under general anesthesia and were adjusted 24 hours later. A positive oculocardiac reflex (OCR) occurred when a 10% or greater intraoperative change in heart rate was associated with traction on an extraocular muscle. A positive V-VR postoperatively consisted of one or more subjective findings (dizziness, light-headedness, nausea, or body temperature changes), and two or more objective findings (10% or greater change in heart rate, hypotension, pallor, diaphoresis, vomiting, disorientation, or loss of consciousness). Variables studied for predictive value included OCR, age, sex, strabismus type, previous surgery, muscle adjusted, and systemic disease. Twenty-five patients (41.6%) had a positive V-VR during adjustment. Twenty-seven patients (45%) had a positive OCR. Eighty-five percent of patients with a positive OCR and 9% of patients with a negative OCR had a positive V-VR. Younger patients were also more likely to have a positive V-VR. A positive intraoperative OCR under general anesthesia during strabismus surgery is highly predictive of a postoperative V-VR during planned adjustment.
J Pediatr Ophthalmol Strabismus
PMID:The intraoperative oculocardiac reflex as a predictor of postoperative vaso-vagal responses during adjustable suture surgery. 825 46

Intracranial aneurysms are rare in infancy. The commonest presentation is intracranial hemorrhage, but signs of mass effect are more frequent than in adults. We report 2 infants with cerebral aneurysms, one presenting with macrocephaly and another with strabismus. Both had visual loss and optic disc pallor; MRI revealed a suprasellar mass and anterior visual pathway compression. In both cases, the preoperative diagnosis was craniopharyngioma. It is essential to recognize that, although exceedingly uncommon, cerebral aneurysms do occur in infants and have features that differ from those in adults.
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PMID:Infantile cerebral aneurysms with visual pathway compression. 1070 33

A newborn male presented with complete external third nerve palsy of his right eye immediately at birth. Pediatric examination and MRI of the skull revealed no abnormalities. At the age of six weeks, strabismus surgery was performed to facilitate amblyopia treatment. The muscles appeared small and fibrotic. At the age of ten weeks, a brow suspension of the upper lid and a second strabismus surgery were performed. The amblyopia treatment and patching, applied for half of the waking hours over a period of six weeks, were unsuccessful. At the age of six months, a relative pallor of the right optic nerve head became evident. At the age of three years, at a new examination because of growth deficiency, a second MRI revealed defects involving the pituitary region. We concluded that extraocular muscle abnormality or oculomotor nerve palsy was present together with optic nerve dysplasia and pituitary gland malformation.
Strabismus 2001 Mar
PMID:Unilateral congenital oculomotor nerve palsy, optic nerve hypoplasia and pituitary malformation: a preliminary report. 1126 99

A 6-month-old infant was diagnosed with infantile malignant autosomal recessive osteopetrosis. An ophthalmologic examination revealed optic nerve pallor and computed tomography of the brain showed narrowing of the orbital fissure. Unrelated umbilical cord blood transplantation was performed. Four months later, the infant died of respiratory complications.
J Pediatr Ophthalmol Strabismus
PMID:Optic nerve compression in infantile malignant autosomal recessive osteopetrosis. 1530 37


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