History

Periventricular leukomalacia (PVL) occurs most commonly in premature infants born at less than 32 weeks' gestation who have a birth weight below 1500 g. Many of these infants have a history of maternal chorioamnionitis. Most affected infants experience cardiorespiratory problems, such as respiratory distress syndrome or pneumonia, in association with hypotension or patent ductus arteriosus during their first days of life. Bacterial infection at birth also appears to be a risk factor.

Physical Examination

Initially, most premature infants with periventricular leukomalacia (PVL) are asymptomatic. If symptoms occur, they are usually subtle.

Symptoms of periventricular leukomalacia may include the following:

Decreased tone in the lower extremities
Increased tone in the neck extensor muscles
Apnea and bradycardia events
Irritability
Pseudobulbar palsy with poor feeding
Clinical seizures (may occur in 10-30% of infants)

Differential Diagnoses

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Media Gallery

Pediatric Periventricular Leukomalacia. Cranial ultrasound, coronal view, in a 1-week-old premature infant. The periventricular echotexture is abnormally increased (greater than or equal to that of the choroid plexus), which is consistent with the early changes of periventricular leukomalacia (PVL). Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial ultrasound, coronal view, in a 1-week-old premature infant without periventricular leukomalacia (PVL). The periventricular echotexture is normal. Compare this image with the previous image. Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial ultrasound, coronal view, in a 3-week-old premature infant. Multiple bilateral periventricular cysts are typical of this stage of periventricular leukomalacia (PVL). Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial ultrasound, sagittal view, in a 3-week-old premature infant. Multiple periventricular cysts are typical of this stage of periventricular leukomalacia (PVL). Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial computed tomography (CT) scan, axial image, in a 5-week-old premature infant with periventricular leukomalacia (PVL). The ventricular margins are irregular, which is consistent with incorporation of the periventricular cysts of PVL. Mild ventriculomegaly and loss of the periventricular white matter is observed. Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial computed tomography (CT) scan, axial image, in a 14-month-old child with periventricular leukomalacia (PVL). Ventriculomegaly is limited to the lateral ventricles secondary to diffuse loss of periventricular white matter. Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial magnetic resonance image (MRI), T1-weighted axial image, in an 18-month-old with periventricular leukomalacia (PVL). The lateral ventricles are enlarged without hydrocephalus. The periventricular white matter is diminished. Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial magnetic resonance image (MRI), T2-weighted axial image, in an 18-month-old with periventricular leukomalacia (PVL). Again, enlarged ventricles and loss of white matter are demonstrated. Also noted is the abnormal increased signal in the periventricular regions on this T2-weighted image. Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. Cranial magnetic resonance image (MRI), sagittal T1-weighted image in the midline, in an 18-month-old child with periventricular leukomalacia (PVL). Hypoplasia of the corpus callosum is present and is most evident, involving the body. Courtesy of Matthew Omojola, MD.
Pediatric Periventricular Leukomalacia. This image depicts cerebral white matter abnormalities in preterm infants found on neuropathological (autopsied) specimens correlating with neuroradiological findings on magnetic resonance images (MRIs) and outcomes. (Data extracted from references 2 Volpe, Pediatr Neurol, 2017], 9 [Volpe et al, Int J Dev Neurosci, 2011], 30 [Guo et al, Neurology, 2017], and 62 [Wagenaar et al, J Pediatr, 2017].)

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Author

Renjithkumar Kalikkot Thekkeveedu, MD Assistant Professor of Pediatrics, Neonatologist, Division of Newborn Medicine, University of Mississippi Medical Center

Renjithkumar Kalikkot Thekkeveedu, MD is a member of the following medical societies: American Academy of Pediatrics, Indian Academy of Pediatrics, National Academy of Medical Sciences (India)

Disclosure: Nothing to disclose.

Coauthor(s)

Nilesh Dankhara, MD, MBBS, FAAP Fellow in Neonatal-Perinatal Medicine, Department of Pediatrics, University of Mississippi Medical Center; Pediatrician, Winchester Pediatrics, Southern Tennessee Regional Health

Nilesh Dankhara, MD, MBBS, FAAP is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Jagdish Desai, MD, MPH Assistant Professor of Pediatrics, Neonatologist, University of Mississippi Medical Center

Jagdish Desai, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American Association of Physicians of Indian Origin, Brooklyn Pediatric Society, Midwest Society for Pediatric Research

Disclosure: Nothing to disclose.

Jaimin M Patel, MD, MBBS, MS Associate Professor, Department of Pediatrics, Assistant Chief Medical Information Officer, Department of Information Technology, University of Mississippi Medical Center

Jaimin M Patel, MD, MBBS, MS is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Medical Informatics Association, Healthcare Information and Management Systems Society, Mississippi State Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Arun K Pramanik, MD, DCH, FAAP, FIAP, FRCP Professor of Pediatrics, LSU Health, Louisiana State University School of Medicine in Shreveport

Arun K Pramanik, MD, DCH, FAAP, FIAP, FRCP is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, National Perinatal Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Santina A Zanelli, MD Associate Professor, Department of Pediatrics, Division of Neonatology, University of Virginia Health System

Santina A Zanelli, MD is a member of the following medical societies: American Academy of Pediatrics, American Epilepsy Society, Society for Neuroscience, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Terence Zach, MD Professor, Department Chair, Department of Pediatrics, Section of Newborn Medicine, Creighton University School of Medicine

Terence Zach, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Scott S MacGilvray, MD Clinical Professor, Department of Pediatrics, Division of Neonatology, The Brody School of Medicine at East Carolina University

Scott S MacGilvray, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Harold A Kaftan, MD Assistant Professor, Department of Pediatrics, Creighton University School of Medicine; Medical Director, Newborn Intensive Care Unit, Creighton University Medical Center; Staff Neonatologist, Joint Division of Newborn Medicine, Creighton University, University of Nebraska Medical Center

Harold A Kaftan, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Acknowledgements

James C Brown, MD Co-Director of Pediatric Radiology, Assistant Professor, Department of Radiology, Creighton University School of Medicine

James C Brown, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Radiology, American Medical Association, and Nebraska Medical Association

Disclosure: Nothing to disclose.