Screening for retinopathy of prematurity--a problem solved?
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Biomedical subjects
Publications and source records attributed to W V Good.
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Eye-poking, -rubbing and -pressing are often incorrectly grouped together because of the assumption that they represent variations of the same self-stimulating behaviour. This prospective study of 21 children shows that eye-poking is a distinct, chronic, stereotyped, self-injurious act seen mainly among severely mentally disabled individuals, who may or may not be visually impaired. Eye-poking, which leads to intense, self-induced pain, is a harmful behaviour because it can result in permanent visual loss and even in total blindness. The cause is unclear, although it is probably multifactorial. The treatment is exceedingly difficult. Further studies are needed to develop specific management techniques to prevent eye-poking, which adds to the physical and emotional burden on the caregivers.
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We describe a 34-year-old man with acute, nontraumatic inferior branch oculomotor nerve palsy. Complete ophthalmologic, neurologic, and systemic examinations were otherwise normal. The oculomotor nerve palsy resolved, but the patient subsequently developed bilateral upper extremity numbness and painful dysesthesias in the distribution of the median nerves. These observations suggest that inferior branch oculomotor nerve palsy, although uncommon, may occur as part of a more generalized neurologic disorder, presumed in our patient to be either vasculitic or demyelinating in nature.
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Thirty two infants referred for in-patient genetics evaluation at the University of California at San Francisco, 1987-1992, were found to have a history of maternal cocaine use. Genetics reports and medical records were reviewed on all these infants to identify features distinctive for cocaine exposure. Among these 32 cases, 14 infants were exposed only to cocaine; 18 were exposed to alcohol and cocaine. The infants evaluated displayed a distinctive phenotype, consisting of neurologic irritability, large fontanels, prominent glabella, marked periorbital and eyelid edema, low nasal bridge with transverse crease, short nose, lateral soft tissue nasal buildup, and small toenails. Features consistent with the fetal alcohol syndrome appeared distinct and coexistent with the other described facial findings. Other severe abnormalities included cleft lip/palate, atypical facial cleft, abnormal BSER, intraventricular hemorrhages, arthrogryposes, and genitourinary abnormalities. Forty percent of the infants were born prematurely; 28% were small for gestational age; 43% showed head circumference values less than the 10th percentile. We conclude that these findings may be distinctive for a diagnosis of fetal cocaine syndrome; such findings should be further established by a future blinded prospective study of mothers and neonates.
The authors examined five young children with monocular vision loss who developed monocular nystagmus. The nystagmus was of fast frequency and small amplitude in all fields of gaze. In four children, successful treatment of the cause of the vision loss resulted in cessation of the nystagmus. No child showed signs of spasmus nutans, or of optic nerve or chiasmal tumor. The evaluation of the young child with unilateral nystagmus should begin with a careful eye examination.
The incidence of amblyopia was analysed in a group of 20 patients with early onset esotropia. These patients reached adulthood without any form of previous treatment. The incidence of amblyopia was compared in a group of 20 patients who received conventional treatment, including occlusion and early surgical alignment. Only three patients (15%) in the untreated group presented with amblyopia, compared with 16 (80%) in the treated group. After treatment 35% of the control group remained amblyopic. Spherical anisometropia of more than 2 dioptres was present in two of the patients with amblyopia in the untreated group, but was not associated with amblyopia in the control group. Early surgical alignment permits the development of peripheral fusion, allowing long term alignment stability, but amblyopia appears to be more common after surgical alignment.
In the past 15 years, we have examined 20 patients in whom myokymia of the superior oblique muscle was diagnosed. Medical treatment (carbamazepine) failed in three patients, and one patient did not accept drug therapy. These four patients (20%) were operated on for persistent oscillopsia and diplopia. One patient underwent only a superior oblique muscle myotomy, but required an inferior oblique muscle myectomy six months later because of iatrogenic superior oblique muscle palsy. The other three patients underwent simultaneous superior oblique muscle myotomy and inferior oblique muscle myectomy. The symptoms resolved postoperatively in all four patients. Symptomatic patients with superior oblique muscle myokymia in whom medical treatment fails or is intolerable can benefit from surgical treatment consisting of combined superior oblique muscle/inferior oblique muscle myectomy.
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The authors describe 13 cocaine-exposed infants with optic nerve abnormalities, delayed visual maturation, and prolonged eyelid edema. Prolonged and potentially vision-threatening eyelid edema is a new clinical entity. The pharmacology of cocaine, its easy access to fetal circulation, and its neurotropic characteristics can be used to explain optic nerve abnormalities and delayed visual maturation. In infants with any of these eye abnormalities, a careful investigation for cocaine abuse is advisable.
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We reviewed the records of 10 children with optic neuritis in whom recovery of vision was poor or incomplete. Our cases were otherwise similar to those described in previous studies in that they were always bilateral, often accompanied by a viral prodrome (seven of 10), and usually associated with disc oedema (seven of 10). Seven of twenty eyes had a final visual acuity of 6/60 or worse and only one patient regained 6/6 vision in either eye. In three patients the best vision in either eye was 6/60 or worse. Recovery of vision was often slow, taking up to six years. Five of 10 patients have developed multiple sclerosis (MS), and one child had acute disseminated encephalomyelitis (ADEM) with optic neuritis. Optic neuritis in children does not always carry a good prognosis for recovery of vision; however, the failure of vision recovery in a short period of time does not necessarily indicate a poor outcome. Some children with optic neuritis develop MS, which can develop even when optic neuritis follows a viral illness.
Four children are reported who always looked above objects of visual interest (overlooking). All had bilateral central scotomata (loss of central visual field). Three had optic nerve disease selectively affecting the papillomacular fibers; the fourth had ocular colobomata affecting the maculae. Overlooking is an important sign of bilateral central scotomata in children: it is an adaptation to loss of central vision.
We reviewed the records of 32 consecutive children (43 eyes) who underwent lensectomy/vitrectomy between January 1988 and August 1990 at the Medical Center of the University of California at San Francisco, to study the incidence and characteristics of clinically significant postoperative inflammation (CSPI). No eyes of patients 18 months old or younger (22 eyes) developed CSPI; nine of 21 eyes of older patients did develop CSPI. Other ocular abnormalities (microphthalmos, persistent hyperplastic primary vitreous, retinopathy of prematurity, and Axenfeld's syndrome) and systemic syndromes did not influence the incidence of CSPI. Prolonged inflammation delayed refractive and amblyopia therapy but did not affect final visual acuity. The absence of CSPI in younger patients may be related to the general immaturity of their immune system and a resultant weak inflammatory response, or to their specific immunologic tolerance to lens crystallins.