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Periventricular leukomalacia: an important cause of visual and ocular motility dysfunction in children
1Karolinska Institutet, St Eriks Eye Hospital, Stockholm, Sweden.
Insights
Periventricular leukomalacia in preterm infants causes significant visual impairment due to optic radiation damage. Early diagnosis and tailored educational strategies are crucial for affected children.
Area of Science:
- Ophthalmology
- Neonatology
- Neuroscience
Background:
- Infants born preterm have immature visual systems vulnerable to perinatal complications.
- Periventricular leukomalacia (PVL) affecting optic radiation is a leading cause of visual impairment in premature infants.
Purpose of the Study:
- To review the causes, characteristics, and management of visual dysfunction in preterm infants with periventricular leukomalacia.
Main Methods:
- Review of literature on periventricular leukomalacia and its impact on visual development.
- Discussion of diagnostic methods, particularly magnetic resonance imaging (MRI).
- Analysis of the clinical spectrum of visual and neurological deficits.
Main Results:
- PVL leads to visual dysfunction including delayed maturation, reduced acuity, visual field defects, and cognitive issues.
- MRI is the primary diagnostic tool for identifying PVL and associated brain lesions.
- Clinical presentation ranges from severe cerebral visual impairment with comorbidities to isolated esotropia.
Conclusions:
- Periventricular leukomalacia presents a spectrum of visual and neurological challenges in premature infants.
- Comprehensive ophthalmological assessment and tailored habilitation are essential.
- Developing optimal educational and support strategies is critical for this population.
Abstract:
The immature visual system in infants born preterm is vulnerable to adverse events during the perinatal period. Periventricular leukomalacia affecting the optic radiation has now become the principal cause of visual impairment and dysfunction in children born prematurely. Visual dysfunction is characterized by delayed visual maturation, subnormal visual acuity, crowding, visual field defects, and visual perceptual-cognitive problems. Magnetic resonance imaging is the method of choice for diagnosing this brain lesion, which is associated with optic disk abnormalities, strabismus, nystagmus, and deficient visually guided eye movements. Children with periventricular leukomalacia may present to the ophthalmologist within a clinical spectrum from severe cerebral visual impairment in combination with cerebral palsy and mental retardation to only early-onset esotropia, normal intellectual level, and no cerebral palsy. Optimal educational and habilitational strategies need to be developed to meet the needs of this group of visually impaired children.