Pediatric Periventricular Leukomalacia Clinical Presentation

  • Author: Terence Zach, MD; Chief Editor: Ted Rosenkrantz, MD   more...
 
Updated: Mar 18, 2010
 

History

Periventricular leukomalacia (PVL) occurs most commonly in premature infants born at less than 32 weeks' gestation who have a birth weight of less than 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.

Next

Physical

Initially, most premature infants are asymptomatic. If symptoms occur, they are usually subtle. Symptoms may include the following:

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

Causes

  • Mechanically ventilated premature infants born at less than 32 weeks' gestation are at greatest risk for periventricular leukomalacia.
  • Hypotension, hypoxemia, and acidosis may result in ischemic brain injury and periventricular leukomalacia.
  • Marked hypocarbia in ventilated premature infants has been associated with increased risk of developing periventricular leukomalacia.
  • Other associated risk factors include the following:
    • Placental vascular anastomoses, twin gestation, antepartum hemorrhage
    • Chorioamnionitis and funisitis
    • Sepsis
    • Maternal cocaine abuse
Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

James C Brown, MD  Codirector 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.

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.

Specialty Editor Board

Scott S MacGilvray, MD  Clinical Associate 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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Arun K Pramanik, MD, MBBS  Professor of Pediatrics, Director of Neonatal Fellowship, Louisiana State University Health Sciences Center

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

Disclosure: Nothing to disclose.

Carol L Wagner, MD  Professor of Pediatrics, Medical University of South Carolina

Carol L Wagner, MD is a member of the following medical societies: American Academy of Pediatrics, American Chemical Society, American Medical Women's Association, American Public Health Association, American Society for Bone and Mineral Research, American Society for Clinical Nutrition, Massachusetts Medical Society, National Perinatal Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Ted Rosenkrantz, MD  Professor, Departments of Pediatrics and Obstetrics/Gynecology, Division of Neonatal-Perinatal Medicine, University of Connecticut School of Medicine

Ted Rosenkrantz, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Pediatric Society, Connecticut State Medical Society, Eastern Society for Pediatric Research, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Nelson KB, Ellenberg JH. Predictors of low and very low birth weight and the relation of these to cerebral palsy. JAMA. Sep 20 1985;254(11):1473-9. [Medline].

  2. Perlman JM, Risser R, Broyles RS. Bilateral cystic periventricular leukomalacia in the premature infant: associated risk factors. Pediatrics. June 1996;97:822-7. [Medline].

  3. Kadhim H, Tabarki B, Verellen G, et al. Inflammatory cytokines in the pathogenesis of periventricular leukomalacia. Neurology. May 22 2001;56(10):1278-84. [Medline].

  4. Ellison VJ, Mocatta TJ, Winterbourn CC, et al. The relationship of CSF and plasma cytokine levels to cerebral white matter injury in the premature newborn. Pediatr Res. Feb 2005;57(2):282-6. [Medline].

  5. Kaukola T, Herva R, Perhomaa M, et al. Chorioamnionitis and cord serum proinflammatory cytokines: lack of association with brain damage and neurologic outcomes in very preterm infants. Pediatr Res. 2005;[Medline].

  6. Khwaja O, Volpe JJ. Pathogenesis of cerebral white matter injury of prematurity. Arch Dis Child Fetal Neonatal Ed. Mar 2008;93(2):F153-61. [Medline].

  7. Okumura A, Hayakawa F, Kato T, et al. Hypocarbia in preterm infants with periventricular leukomalacia: the relation between hypocarbia and mechanical ventilation. Pediatrics. Mar 2001;107(3):469-75. [Medline]. [Full Text].

  8. Wiswell TE, Graziani LJ, Kornhauser MS, et al. Effects of hypocarbia on the development of cystic periventricular leukomalacia in premature infants treated with high-frequency jet ventilation. Pediatrics. Nov 1996;98(5):918-24. [Medline].

  9. Shankaran S, Langer JC, Kazzi SN, Laptook AR, Walsh M. Cumulative index of exposure to hypocarbia and hyperoxia as risk factors for periventricular leukomalacia in low birth weight infants. Pediatrics. Oct 2006;118(4):1654-9. [Medline].

  10. Tzarouchi LC, Astrakas LG, Zikou A, et al. Periventricular leukomalacia in preterm children: assessment of grey and white matter and cerebrospinal fluid changes by MRI. Pediatr Radiol. Dec 2009;39(12):1327-32. [Medline].

  11. Baud O, Foix-L'Helias L, Kaminski M, et al. Antenatal glucocorticoid treatment and cystic periventricular leukomalacia in very premature infants. N Engl J Med. Oct 14 1999;341(16):1190-6. [Medline].

  12. Canterino JC, Verma U, Visintainer PF, et al. Antenatal steroids and neonatal periventricular leukomalacia. Obstet Gynecol. Jan 2001;97(1):135-9. [Medline].

  13. Bass WT, Jones MA, White LE, et al. Ultrasonographic differential diagnosis and neurodevelopmental outcome of cerebral white matter lesions in premature infants. J Perinatol. Jul-Aug 1999;19(5):330-6. [Medline].

  14. Baud O, d'Allest AM, Lacaze-Masmonteil T, et al. The early diagnosis of periventricular leukomalacia in premature infants with positive rolandic sharp waves on serial electroencephalography. J Pediatr. May 1998;132(5):813-7. [Medline].

  15. Dammann O, Hagberg H, Leviton A. Is periventricular leukomalacia an axonopathy as well as an oligopathy?. Pediatr Res. Apr 2001;49(4):453-7. [Medline]. [Full Text].

  16. Dammann O, Leviton A. Brain damage in preterm newborns: might enhancement of developmentally regulated endogenous protection open a door for prevention?. Pediatrics. Sep 1999;104(3 Pt 1):541-50. [Medline]. [Full Text].

  17. de Vries LS, Regev R, Dubowitz LM, et al. Perinatal risk factors for the development of extensive cystic leukomalacia. Am J Dis Child. Jul 1988;142(7):732-5. [Medline].

  18. De Vries LS, Van Haastert IL, Rademaker KJ, et al. Ultrasound abnormalities preceding cerebral palsy in high-risk preterm infants. J Pediatr. Jun 2004;144(6):815-20. [Medline].

  19. Dyet LE, Kennea N, Counsell SJ, et al. Natural history of brain lesions in extremely preterm infants studied with serial magnetic resonance imaging from birth and neurodevelopmental assessment. Pediatrics. Aug 2006;118(2):536-48. [Medline].

  20. Enzmann DR. Imaging of neonatal hypoxic-ischemic cerebral damage. In: Stevenson DK, Sunshine P, eds. Fetal and Neonatal Brain Injury: Mechanisms, Management, and the Risk of Practice. 2nd ed. Oxford, England: Oxford University Press; 1997:302-55.

  21. Hahn JS, Novotony EJ Jr. Hypoxic-ischemic encephalopathy. In: Stevenson DK, Sunshine P, eds. Fetal and Neonatal Brain Injury: Mechanisms, Management, and the Risk of Practice. 2nd ed. Oxford, England:. Oxford University Press;1997:277-286.

  22. Hayakawa F, Okumura A, Kato T, et al. Determination of timing of brain injury in preterm infants with periventricular leukomalacia with serial neonatal electroencephalography. Pediatrics. Nov 1999;104(5 Pt 1):1077-81. [Medline]. [Full Text].

  23. Haynes RL, Baud O, Li J, et al. Oxidative and nitrative injury in periventricular leukomalacia: a review. Brain Pathol. 2005;15:225-233. [Medline].

  24. Kuban K, Sanocka U, Leviton A, et al. White matter disorders of prematurity: association with intraventricular hemorrhage and ventriculomegaly. The Developmental Epidemiology Network. J Pediatr. May 1999;134(5):539-46. [Medline].

  25. Leviton A, Paneth N, Reuss ML, et al. Maternal infection, fetal inflammatory response, and brain damage in very low birth weight infants. Developmental Epidemiology Network Investigators. Pediatr Res. Nov 1999;46(5):566-75. [Medline].

  26. Liao SL, Lai SH, Chou YH, Kuo CY. Effect of hypocapnia in the first three days of life on the subsequent development of periventricular leukomalacia in premature infants. Acta Paediatr Taiwan. Mar-Apr 2001;42(2):90-3. [Medline].

  27. [Guideline] Ment LR, Bada HS, Barnes P, et al. Practice parameter: neuroimaging of the neonate: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. Jun 25 2002;58(12):1726-38. [Medline].

  28. Murata Y, Itakura A, Matsuzawa K, et al. Possible antenatal and perinatal related factors in development of cystic periventricular leukomalacia. Brain Dev. 2005;27:17-21. [Medline].

  29. Paul DA, Pearlman SA, Finkelstein MS, Stefano JL. Cranial sonography in very-low-birth-weight infants: do all infants need to be screened?. Clin Pediatr (Phila). Sep 1999;38(9):503-9. [Medline].

  30. Shankaran S. Hemorrhagic lesions of the central nervous system. In: Stevenson DK, Sunshine P, eds. Fetal and Neonatal Brain Injury: Mechanisms, Management, and the Risk of Practice. 2nd ed. Oxford, England: Oxford University Press; 1997:151-64.

  31. Volpe JJ. Brain injury in the premature infant: overview of clinical aspects, neuropathology, and pathogenesis. Semin Pediatr Neurol. Sep 1998;5(3):135-51. [Medline].

 
Previous
Next
 
Cranial ultrasound, coronal view, in 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.
Cranial ultrasound, coronal view, in 1-week-old premature infant without periventricular leukomalacia (PVL). The periventricular echotexture is normal. Compare with the previous image. Courtesy of Matthew Omojola, MD.
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.
Cranial ultrasound, sagittal view, in 3-week-old premature infant. Multiple periventricular cysts are typical of this stage of periventricular leukomalacia (PVL). Courtesy of Matthew Omojola, MD.
Cranial 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.
Cranial CT scan, axial image, in 14-month-old with periventricular leukomalacia (PVL). Ventriculomegaly is limited to the lateral ventricles secondary to diffuse loss of periventricular white matter. Courtesy of Matthew Omojola, MD.
Cranial 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.
Cranial 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.
Cranial MRI, sagittal T1-weighted image in the midline, in an 18-month-old with periventricular leukomalacia (PVL). Hypoplasia of the corpus callosum is present and is most evident, involving the body. Courtesy of Matthew Omojola, MD.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.