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Biomedical subjects

W V Good

Publications and source records attributed to W V Good.

At least 19 recordsLinked to original sources
PubMed1999

Chronic cortical visual impairment in children: aetiology, prognosis, and associated neurological deficits.

BACKGROUND/AIMS: To evaluate prevalence, aetiology, prognosis, and associated neurological and ophthalmological problems in children with cortical visual impairment (CVI). METHODS: The records of 7200 outpatients seen in the paediatric ophthalmology practice over the past 15 years were reviewed in order to compile data concerning CVI. In addition, the authors devised and applied a system for grading visual recovery in order to assess prognosis. RESULTS: CVI occurred in 2.4% of all patients examined. The four most common causes of CVI were perinatal hypoxia (22%), cerebral vascular accident (14%), meningitis (12%), and acquired hypoxia (10%). Most children with CVI had associated neurological abnormalities. The most common were seizures (53%), cerebral palsy (26%) hemiparesis (12%), and hypotonia (5%). Associated ophthalmological problems were esotropia (19%), exotropia (18%), optic nerve atrophy (16%), ocular motor apraxia (15%), nystagmus (11%), and retinal disease (3%). On average, CVI patients improved by two levels as measured by the authors' scale. CONCLUSION: The majority of children with CVI showed at least some recovery. In this group of children, CVI is often accompanied by additional ophthalmological problems and is nearly always associated with other, serious neurological abnormalities.

Blindness, Cortical↗
PubMed1998

Macular folds and poor vision associated with zone III retinopathy of prematurity.

PURPOSE: To describe two cases of zone III retinopathy of prematurity associated with macular folds and poor vision. METHOD: Case reports. RESULTS: Two premature infants with zone III retinopathy of prematurity developed clinically notable elevation of the neovascular ridge with macular folds and poor vision. CONCLUSIONS: Retinopathy of prematurity in zone III may lead to compromised anatomic and functional outcomes. Notable elevation of the neovascular ridge may be an important risk factor for an adverse outcome.

Female↗
PubMed1998

Healing of corneal epithelial defects in plasminogen- and fibrinogen-deficient mice.

PURPOSE: The local deposition of fibrinogen and other plasma products from tears within corneal wounds and the expression of plasminogen activator by corneal epithelial cells suggest that the coagulation and fibrinolytic systems play an important role in corneal wound healing. The authors used mouse lines deficient in plasminogen (Plg), fibrinogen (Fib), or both to elucidate the roles of these key fibrinolytic and coagulation factors in the healing of corneal epithelial defects. METHODS: Mice were anesthetized, and corneal epithelial defects (3 mm) were created with a blade. The authors conducted histologic examination and immunohistochemical analysis on the healing of injured corneas. RESULTS: The corneal epithelial defects of wild-type mice with transparent corneas healed quickly in 7 days, whereas the healing of plasminogen-deficient mice was impaired and complicated by severe and persistent inflammatory responses, the formation of retrocorneal fibrin deposits, corneal cloudiness caused by scar-tissue formation, and often stromal neovascularization. To determine whether these defects in corneal wound repair were specifically related to an impediment in fibrinolysis, corneal wound healing was compared in mice with a combined deficiency in plasminogen and fibrinogen. The loss of fibrinogen in mice lacking plasminogen resulted in the restoration of normal healing with transparent corneas in 7 days, similar to that of wild-type mice. CONCLUSIONS: These results provide direct evidence that hemostatic factors play a crucial role in corneal wound repair despite the lack of local hemorrhage. Furthermore, they demonstrate that the essential role of plasmin in corneal would healing is fibrinolysis. It prevents the adverse inflammatory responses caused by prolonged fibrin and fibrinogen deposition in injured corneas.

Afibrinogenemia↗
PubMed1997

Norrie disease in a family with a manifesting female carrier.

OBJECTIVES: To show that Norrie disease can occur in a girl and to describe her ophthalmologic and genetic features. METHODS: Amplification of DNA polymerase chain reaction and sequencing of asymmetric polymerase chain reaction for exon 3 were performed on the blood specimen obtained from a girl born with bilateral retinal detachments. PATIENT: A female child with bilateral retinal detachment who had 2 uncles in whom Norrie disease had already been diagnosed. RESULTS: The child had a mutation in the third exon (T776-->A; Ile 123-->Asn) identical to the mutation found in her uncles. CONCLUSIONS: Norrie disease can occur in girls. The most likely explanation is nonrandom or unfavorable X inactivation, although timing of development of the peripheral retina and its blood supply could render it vulnerable to effects of the mutant allele at a critical developmental phase.

Blindness↗
PubMed1996

Cortical visual impairment caused by twin pregnancy.

PURPOSE: To report a possible relationship between twin pregnancy and cortical visual impairment. METHODS: Three children who had been the products of twin pregnancies were identified as having cortical visual impairment. One child (Patient 2), a dizygotic twin, developed cortical visual impairment after a preterm birth. Two children (Patients 1 and 3), the products of monochorionic pregnancies, developed cortical visual impairment. All children were examined ophthalmologically and neurologically. RESULTS: An evaluation of the gestations of these children indicates that twin pregnancy per se was probably etiologic in the development of cortical visual impairment. In Patient 2, twin pregnancy probably caused preterm birth and resulting cortical visual impairment. In Patients 1 and 3, twin-to-twin transfusion syndrome was the cause of cortical visual impairment. In Patient 1, fetal demise precipitated an acute twin-to-twin transfusion syndrome. CONCLUSIONS: Children who show cortical visual impairment at or shortly after birth should be evaluated for the possibility of twin pregnancy. Twin pregnancy increases the risk of neurologic damage, including damage to the visual cortex, to optic radiations, or both.

Child, Preschool↗
PubMed1996

Hypertropia and the posterior blowout fracture: Mechanism and management.

OBJECTIVE: To better understand the pathophysiology and proper management of a subgroup of patients with orbital blowout fracture which manifests by a vertical diplopia and hypertropia on the affected side. PATIENTS AND METHODS: This report is based on a series of ten consecutive patients with orbital floor blowout fractures who had diplopia and hypertropia on the affected side. All patients were followed through at least 13 days of conservative care. Computed tomography demonstrated a characteristic depressed fracture of the posterior orbital floor extending to the posterior wall of the maxillary sinus in all patients. In many patients, the inferior rectus looped inferiorly and then rose to contact the globe at a steep angle. Diplopia did not spontaneously resolve in any patient. At surgery, the orbital contents were elevated to the posterior extent of the fracture, and the floor defects were bridge. Patients were followed for resolution of diplopia. RESULTS: Eight patients had resolution of the hypertropia and diplopia within 2 months of surgery, and two patients had residual diplopia in extreme downgaze but were significantly improved. CONCLUSIONS: When hypertropia and vertical diplopia are noted after orbital trauma, a posterior blowout fracture should be suspected. In these patients, infraduction may be diminished due to changes in the effective origin and insertion of the inferior rectus muscle. The diagnosis of a posterior blowout fracture should be supported by characteristic findings on computed tomography. If the motility abnormality persists for 10 to 14 days, posterior orbital exploration and fracture repair should be undertaken.

Adolescent↗
PubMed1996

Cytomegalovirus retinitis and optic neuritis in a child with severe combined immunodeficiency syndrome.

BACKGROUND: Although cytomegalovirus (CMV) infection of the retina and brain is common in patients with acquired immunodeficiency syndrome (AIDS), it is exceedingly rare in patients with immunodeficiencies due to other causes. This is the first report on ocular and cerebral histopathology of disseminated CMV in a child with severe combined immunodeficiency syndrome (SCID). METHODS: The authors examined by routine histopathologic methods the eyes of a 2-year-old white boy with SCID and bilateral CMV retinitis who died after failure of a third attempt at allogeneic bone marrow transplantation (BMT). RESULTS: Cytomegalovirus inclusions were found in the necrotic retinal remnants, in the hyperplastic and scarred retinal pigment epithelium, and bilaterally in the optic nerves. There were infiltrates of macrophages in response to the infection or the infused silicon, but no lymphoid infiltrates. Cytomegalovirus inclusions also were found in brain tissue. CONCLUSION: The histologic features resembled those of CMV retinitis and optic neuritis in AIDS.

Bone Marrow Transplantation↗
PubMed1996

Visually impaired children with sensory defect nystagmus, normal appearing fundi and normal ERGS.

The differentiation of congenital motor nystagmus (CMN) from sensory defect nystagmus (SDN) in early life is a common diagnostic problem, since their wave forms are indistinguishable. A diagnosis of SDN is made when fundal or ERG abnormalities are present. CMN is generally diagnosed by exclusion. This approach can lead to late or incorrect diagnosis, and to the overlooking of important genetic implications. In addition, the classification of congenital nystagmus into pure motor and sensory types is inadequate. The authors present case reports illustrating this problem and suggest strict criteria for a diagnosis of CMN. They also point out that a miscellaneous group of rare disorders of the macula tend to be overlooked and misdiagnosed.

Adolescent↗
PubMed1996

An elevated level of copper zinc superoxide dismutase fails to prevent oxygen induced retinopathy in mice.

BACKGROUND: To determine whether a higher level of copper zinc superoxide dismutase (CuZnSOD) can reduce the severity of oxygen induced retinopathy (OIR) in a mouse model. METHODS: CuZnSOD transgenic mice with a threefold increase in CuZnSOD activity and control non-transgenic mice were exposed to 90% oxygen for 12 hours a day during the first 5 days of life. After oxygen treatment, all mice were reared in room air for 10 days. Another group of transgenic and non-transgenic mice were kept in room air for 15 days and served as control groups for the oxygen effect. At day 15, all mice were killed and perfused with India ink. The retinas were flat mounted on slides and examined with a light microscope. RESULTS: There was a statistically significant increase in the incidence of OIR in mice exposed to high levels of oxygen, whether or not they were transgenic. However, there was no statistically significant difference in the severity of OIR between oxygen treated transgenic and non-transgenic mice. CONCLUSION: A threefold higher CuZnSOD activity does not protect against OIR in mice. This is an unexpected finding, since oxygen radicals are considered a major factor causing OIR, and increased CuZnSOD activity has reduced oxygen radical induced damage in several neuronal and non-neuronal systems. The possibility of a damaging role for other radicals not affected by CuZnSOD cannot be excluded.

Animals↗
PubMed1996

Neurologic and ophthalmologic findings in children exposed to cocaine in utero.

We studied a cohort of 73 children exposed to cocaine in utero to characterize the spectrum of neurologic and ophthalmologic abnormalities; 36 children with no documented in utero drug exposure were selected as a control group. Most referrals of cocaine-exposed children to the child neurologist were made in the 1st year of life (81%). Reasons for referral were hypertonia (29%), seizures (22%), developmental delay (11%), tremulousness (11%), and hypotonia (4%). The most common abnormal finding in the cocaine-exposed children was hypertonia/hyperreflexia (63%), which was rarely seen in the control group. In addition, hypertonia/hyperreflexia was underdiagnosed by referring physicians. Similarly, hypotonia was seen on neurologic examination of cocaine-exposed children more frequently than documented by referral (16% versus 4%). Hypotonia was rarely seen in the control group. Twelve (43%) of 28 cocaine-exposed children seen by a pediatric ophthalmologist had structural ophthalmologic abnormalities. Neurologic and ophthalmologic findings suggesting structural lesions of the nervous system must be considered in cocaine-exposed children.

Child↗
PubMed1995

Vogt-Koyanagi-Harada syndrome in a 4-year old child.

PURPOSE: We studied a case of severe bilateral Vogt-Koyanagi-Harada syndrome in a 4-year-old boy. METHODS: We evaluated the patient's clinical course. RESULTS: The patient had severe bilateral, nongranulomatous uveitis and mild uveitic glaucoma. Initial examination and laboratory evaluation failed to provide a diagnosis. The patient subsequently developed areas of vitiligo, alopecia, and poliosis, suggesting the diagnosis of Vogt-Koyanagi-Harada syndrome. This diagnosis was confirmed by the eventual development of bilateral neurosensory retinal detachments. Vision was lost despite aggressive therapy with corticosteroids and chlorambucil. CONCLUSION: Although uncommon, Vogt-Koyanagi-Harada may affect young children, and may be severe.

Alopecia↗
PubMed1995

Familial paroxysmal kinesigenic choreo-athetosis in a child with visual hallucinations and obsessive-compulsive behaviour.

A teenage male is described, in whom Tourette Syndrome was suspected, which was later replaced by attacks of paroxysmal kinesigenic choreo-athetosis. He also exhibited bizarre, episodic perceptual distortions of his visual environment and manifestations of an obsessive-compulsive disorder. Carbamazepine treatment not only completely eliminated the recurring attacks of his choreo-athetosis but also, contrary to expectations, the visual disturbances and even the symptoms of his obsessive-compulsive disorder. Obsessive compulsive disorder should be searched for by direct questioning in all patients with basal ganglia disorders.

Adolescent↗