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Journal of Child Neurology Feb 2022Periventricular leukomalacia (PVL) is a term reserved to describe white matter injury in the premature brain. In this review article, the authors highlight the common...
AIM
Periventricular leukomalacia (PVL) is a term reserved to describe white matter injury in the premature brain. In this review article, the authors highlight the common and rare pathologies mimicking the chronic stage of PVL and propose practical clinico-radiological criteria that would aid in diagnosis and management.
METHODS AND RESULTS
The authors first describe the typical brain MRI (magnetic resonance imaging) features of PVL. Based on their clinical presentation, pathologic entities and their neuroimaging findings were clustered into distinct categories. Three clinical subgroups were identified: healthy children, children with stable/nonprogressive neurological disorder, and those with progressive neurological disorder. The neuroradiological discriminators are described in each subgroup with relevant differential diagnoses. The mimics were broadly classified into normal variants, acquired, and inherited disorders.
CONCLUSIONS
The term "PVL" should be used appropriately as it reflects its pathomechanism. The phrase "white matter injury of prematurity" or "brain injury of prematurity" is more specific. Discrepancies in imaging and clinical presentation must be tread with caution and warrant further investigations to exclude other possibilities.
Topics: Brain; Cerebral Palsy; Female; Gestational Age; Humans; Infant, Newborn; Leukomalacia, Periventricular; Magnetic Resonance Imaging; Male; Pregnancy; Pregnancy Complications; Risk Factors
PubMed: 34937403
DOI: 10.1177/08830738211026052 -
Neonatal Network : NN Jun 1992Serial ultrasound examinations in the preterm infant have documented a strong predictive relationship between PVL and adverse neurologic sequelae, particularly cerebral... (Review)
Review
Serial ultrasound examinations in the preterm infant have documented a strong predictive relationship between PVL and adverse neurologic sequelae, particularly cerebral palsy. Good obstetric management with carefully timed intervention as well as prevention of hypoxic-ischemic episodes postnatally may prevent PVL. The nurse plays a significant role in preventing PVL in the preterm infant. She should understand the risk factors associated with PVL and establish protocols in nursing care to promote minimal handling. Prevention of hypoxemia and wide fluctuations in blood pressure are critical. Careful attention to adequate ventilation and the continuous monitoring of oxygen saturation will help avoid and treat hypoxemia episodes.
Topics: Brain; Diagnostic Imaging; Humans; Infant, Newborn; Leukomalacia, Periventricular; Maternal-Child Nursing; Prognosis
PubMed: 1608381
DOI: No ID Found -
Research in Developmental Disabilities Apr 2023Thanks to Magnetic Resonance Imaging (MRI) it is now possible to diagnose lesions of the central nervous system (CNS) such as periventricular leukomalacia (PVL) from the... (Review)
Review
BACKGROUND
Thanks to Magnetic Resonance Imaging (MRI) it is now possible to diagnose lesions of the central nervous system (CNS) such as periventricular leukomalacia (PVL) from the first days of life. However, there are still few studies aimed at describing the relationship between MRI and the outcome of visual function in patients with PVL.
AIM
To systematically review and investigate the relationship between MRI neuroimaging and visual impairment arising from PVL.
METHODS AND PROCEDURES
Three electronic databases (PubMed, SCOPUS, Web of Science) were consulted from 15 June 2021-30 September 2021. Of the 81 records identified, 10 were selected for the systematic review. The STROBE Checklist was used to assess the quality of the observational studies.
OUTCOME AND RESULTS
PVL on MRI was found to have a strong association with visual impairment in the various aspects of visual function (visual acuity, ocular motility, visual field); in 60% of these articles, the selected subjects also reported damage to optical radiations.
CONCLUSION AND IMPLICATIONS
there is a clear need for more extensive and detailed studies on the correlation between PVL and visual impairment, in order to set up a personalized early therapeutic-rehabilitation plan. WHAT THIS PAPER ADDS?: Over the past decades numerous studies have reported increasing evidence that one of the most frequent sequelae in subjects with PVL, in addition to motor impairment, is the impairment of visual function even if it is still not clear what different authors mean with the term visual impairment. This systematic review presents an overview of the relationship between structural correlates of MRI and visual impairment in children with periventricular leukomalacia. Interesting correlations emerge between MRI radiological finding and consequences on visual function especially between damage to the periventricular white matter and the impairment of various aspects of visual function and also between the impairment of optical radiation and visual acuity. Thanks to this literature revision, it is now clear that MRI plays an important role in the screening and diagnosis of significant intracranial brain changes in very young children in particular about the outcome of visual function. This is of great relevance since that visual function represents one of the main adaptive functions in the development of the child.
Topics: Infant, Newborn; Humans; Child; Child, Preschool; Leukomalacia, Periventricular; Brain; Magnetic Resonance Imaging; Vision, Low
PubMed: 36796269
DOI: 10.1016/j.ridd.2023.104439 -
Monatsschrift Kinderheilkunde : Organ... Jul 1993The present knowledge on periventricular leucomalacia is reviewed. Its incidence is about 10% in preterms infants < 32 gestational weeks. By careful ultrasonographic... (Review)
Review
The present knowledge on periventricular leucomalacia is reviewed. Its incidence is about 10% in preterms infants < 32 gestational weeks. By careful ultrasonographic scanning four stages can be distinguished: Prolonged flares, resolution, cystic leucomalacia, ventriculomegaly. Periventricular leucomalacia (PVL) is much more correlated with later neurologic impairment than intraventricular haemorrhage (apart from grade IV). PVL is caused by ischemia of the periventricular white matter mainly due to arterial hypotension and disturbed regulation of cerebral blood flow. The interactions between blood pressure, pCO2, pO2 and cerebral blood flow are complex and poorly understood. Regarding the clinical management it seems important to avoid hypotension, fluctuating pCO2 and hypoxemia/anemia in order to prevent white matter ischemia. Research should be focussed on the development of monitoring methods (doppler sonography, near infrared spectroscopy, time compressed electroencephalography), which allow to detect ischemic events to prevent neurologic impairment in about 1000 preterm infants per year in Germany.
Topics: Blood Flow Velocity; Brain; Brain Damage, Chronic; Cerebral Ventricles; Echoencephalography; Follow-Up Studies; Humans; Infant, Newborn; Leukomalacia, Periventricular; Necrosis; Neurologic Examination
PubMed: 8413333
DOI: No ID Found -
Archives of Neurology Oct 2008Periventricular leukomalacia (PVL) is the predominant form of brain injury and the leading known cause of cerebral palsy and cognitive deficits in premature infants. The... (Review)
Review
Periventricular leukomalacia (PVL) is the predominant form of brain injury and the leading known cause of cerebral palsy and cognitive deficits in premature infants. The number of low-birth-weight infants who survive to demonstrate these neurologic deficts is increasing. Magnetic resonance imaging-based neuroimaging techniques provide greater diagnostic sensitivity for PVL than does head ultrasonography and often document the involvement of telencephalic gray matter and long tracts in addition to periventricular white matter. The neuropathologic hallmarks of PVL are microglial activation and focal and diffuse periventricular depletion of premyelinating oligodendroglia. Premyelinating oligodendroglia are highly vulnerable to death caused by glutamate, free radicals, and proinflammatory cytokines. Studies in animal models of PVL suggest that pharmacologic interventions that target these toxic molecules will be useful in diminishing the severity of PVL.
Topics: Animals; Brain; Encephalitis; Female; Gliosis; Humans; Infant, Newborn; Leukomalacia, Periventricular; Magnetic Resonance Imaging; Microglia; Nerve Fibers, Myelinated; Neural Pathways; Oligodendroglia; Oxidative Stress; Pregnancy
PubMed: 18852342
DOI: 10.1001/archneur.65.10.1291 -
Pediatric Research Apr 2001Periventricular leukomalacia is a white matter disorder, the neonatal cranial ultrasound images of which predict long-term developmental limitations among preterm... (Review)
Review
Periventricular leukomalacia is a white matter disorder, the neonatal cranial ultrasound images of which predict long-term developmental limitations among preterm infants. The vulnerability of oligodendrocytes has led to the hypothesis that oligodendrocytes suffer the primary damage, with axonal damage occurring as a consequence. In this article, we discuss the differential role of oligodendrocytes and axons in this disorder's etiology, offering analogies from the multiple sclerosis and hydrocephalus literature. We conclude that it is too early to view periventricular leukomalacia exclusively as a consequence of oligodendrocyte damage and/or maldevelopment.
Topics: Axons; Brain; Humans; Infant, Newborn; Leukomalacia, Periventricular; Oligodendroglia
PubMed: 11264425
DOI: 10.1203/00006450-200104000-00003 -
Pediatric and Developmental Pathology :... 2006Periventricular leukomalacia (PVL), the main substrate for cerebral palsy, is characterized by diffuse injury of deep cerebral white matter, accompanied in its most... (Review)
Review
Periventricular leukomalacia (PVL), the main substrate for cerebral palsy, is characterized by diffuse injury of deep cerebral white matter, accompanied in its most severe form by focal necrosis. The classic neuropathology of PVL has given rise to several hypotheses about the pathogenesis, largely relating to hypoxia-ischemia and reperfusion in the sick premature infant. These include free radical injury, cytokine toxicity (especially given the epidemiologic association of PVL with maternofetal infection), and excitotoxicity. Among the recent findings directly in human postmortem tissue is that immunocytochemical markers of lipid peroxidation (hydroxy-nonenal and malondialdehyde) and protein nitration (nitrotyrosine) are significantly increased in PVL. Premyelinating oligodendrocytes, which predominate in periventricular regions during the window of vulnerability to PVL (24 to 34 postconceptional weeks), are the targets of this free radical injury, and suffer cell death. Susceptibility can be attributed, at least in part, to a relative deficiency of superoxide dismutases in the preterm white matter, including premyelinating oligodendrocytes. Several cytokines, including interferon-gamma (known to be directly toxic to immature oligodendroglia in vitro), as well as tumor necrosis factor-alpha and interleukins 2 and 6, have been demonstrated in PVL. Microglia, which express toll-like receptors to bacterial products such as lipopolysaccharide, are increased in PVL white matter and may contribute to the injury. Preliminary work suggests a role for glutamate receptors and glutamate transporters in PVL, as has been seen in experimental animals. These findings pave the way for eventual therapeutic or preventive strategies for PVL.
Topics: Amino Acid Transport System X-AG; Animals; Cerebral Palsy; Disease Models, Animal; Humans; Infant, Newborn; Infant, Premature; Leukomalacia, Periventricular; Receptors, Glutamate
PubMed: 16808630
DOI: 10.2350/06-01-0024.1 -
Survey of Ophthalmology 2000The immature visual system in infants born preterm is vulnerable to adverse events during the perinatal period. Periventricular leukomalacia affecting the optic... (Review)
Review
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.
Topics: Brain; Diagnosis, Differential; Eye Movements; Humans; Infant, Newborn; Leukomalacia, Periventricular; Magnetic Resonance Imaging; Ocular Motility Disorders; Optic Disk; Prognosis; Vision Disorders; Visual Acuity; Visual Fields
PubMed: 10946078
DOI: 10.1016/s0039-6257(00)00134-x -
The Journal of Neuroscience Nursing :... Dec 2002Periventricular leukomalacia (PVL) is a result of injury and necrosis of myelinated fibers around the lateral ventricles. PVL is now considered the principal form of... (Review)
Review
Periventricular leukomalacia (PVL) is a result of injury and necrosis of myelinated fibers around the lateral ventricles. PVL is now considered the principal form of brain injury in preterm infants. This injury can have long-term effects on physical, motor, sensory, cognitive, and social development. Some proposed pharmacological treatments being considered to aid in prevention of this injury are raising concerns because they have failed to show evidence of efficacy or have potential for deleterious long-term effects. Current treatment is aimed at injury prevention; therefore, nurses play a critical role. Awareness of the pathophysiologic concerns about preterm neonates can help nurses focus their assessments to identify patients at risk.
Topics: Brain; Brain Injuries; Hemodynamics; Humans; Infant, Newborn; Infant, Premature; Leukomalacia, Periventricular
PubMed: 12506812
DOI: 10.1097/01376517-200212000-00003 -
Seminars in Thoracic and Cardiovascular... 2004The dramatic reduction in surgical mortality following repair of congenital heart defects has been accompanied by increasing recognition of adverse neurodevelopmental... (Review)
Review
The dramatic reduction in surgical mortality following repair of congenital heart defects has been accompanied by increasing recognition of adverse neurodevelopmental sequelae in some children. Neurodevelopmental abnormalities following neonatal and infant cardiac surgery include mild cognitive impairment, expressive speech and language abnormalities, impaired attention and executive function, impaired visual and spatial motor skills, as well as learning disabilities. These defects result in a significant need for early intervention, as well as rehabilitative and special education services. Central nervous system injury following repair of congenital heart defects results from a complex interaction of patient specific and environmental factors. Recent studies suggest that cerebral white matter injury characterized by periventricular leukomalacia (PVL) is common in infants with congenital heart disease particularly following cardiac surgery. Studies at our institution show that this occurs in greater than 50% of neonates following cardiac surgery, but is rare in older infants. Prolonged exposure to cardiopulmonary bypass (with or without deep hypothermic circulatory arrest) is a risk factor for PVL, possibly secondary to the systemic inflammatory response to cardiopulmonary bypass. Hypotension and hypoxemia in the early postoperative period, especially diastolic hypotension, significantly increase the risk of PVL. Future studies are needed to determine significance of PVL as a marker for long-term developmental dysfunction.
Topics: Cardiac Surgical Procedures; Humans; Infant; Infant, Newborn; Leukomalacia, Periventricular; Magnetic Resonance Imaging; Risk Factors; Systemic Inflammatory Response Syndrome
PubMed: 15283363
DOI: 10.1053/j.pcsu.2004.02.007