eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Neonatology

Periventricular Leukomalacia: Differential Diagnoses & Workup

Author: Terence Zach, MD, Department Vice-Chair, Professor, Department of Pediatrics, Section of Newborn Medicine, Creighton University
Coauthor(s): James C Brown, MD, Codirector of Pediatric Radiology, Assistant Professor, Department of Radiology, Creighton University School of Medicine
Contributor Information and Disclosures

Updated: Feb 14, 2008

Differential Diagnoses

Periventricular Hemorrhage-Intraventricular Hemorrhage

Other Problems to Be Considered

Intraventricular hemorrhage
Periventricular hemorrhagic venous infarction

Workup

Imaging Studies

  • Cranial ultrasonography
    • Cranial ultrasonography is the modality of choice for the initial evaluation of hypoxic-ischemic damage of the CNS in premature infants. Ultrasonography may be performed in the NICU without the need to transport fragile infants.
    • The earliest ultrasonographic appearance of periventricular leukomalacia (PVL) is abnormal increased echotexture in the periventricular white matter. This is a nonspecific finding that must be differentiated from the normal periventricular halo and mild periventricular edema that may not result in permanent injury.
    • The abnormal periventricular echotexture of PVL usually disappears at 2-3 weeks. Approximately 15% of infants experiencing PVL demonstrate periventricular cysts first appearing at 2-3 weeks after the initial increased echodensities.
    • The severity of PVL is related to the size and distribution of these cysts. Initial cranial ultrasonographic findings may be normal in patients who go on to develop clinical and delayed imaging findings of PVL.
  • CT scanning: CT scanning is not a first-line modality in evaluating these fragile premature infants in the first weeks of life. CT scanning may be helpful to better evaluate the extent and severity of PVL. Findings include ventriculomegaly involving the lateral ventricles with irregular margins of the ventricles and loss of deep white matter.
  • MRI: Like CT scanning, MRI does not play a major role in the early evaluation of PVL. MRI is most helpful in monitoring infants with suspected PVL and evaluating infants who develop clinical signs suggestive of PVL. MRI demonstrates the loss of white matter, abnormal signal intensity of the deep white matter, and ventriculomegaly. MRI demonstrates thinning of the posterior body and splenium of the corpus callosum in severe cases of PVL. Volumetric MRI scanning is also helpful in determining the extent of injury to the descending corticospinal tracts. A relationship between the degree of injury to the descending corticospinal tracts as assessed by MRI and the severity of diplegia has been reported.

Other Tests

  • EEG

Histologic Findings

  • PVL lesions demonstrate widespread loss of oligodendrocytes and an increase in astrocytes.

More on Periventricular Leukomalacia

Overview: Periventricular Leukomalacia
Differential Diagnoses & Workup: Periventricular Leukomalacia
Treatment & Medication: Periventricular Leukomalacia
Follow-up: Periventricular Leukomalacia
Multimedia: Periventricular Leukomalacia
References

References

  1. 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].

  2. 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].

  3. 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].

  4. 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].

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

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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.

  13. 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.

  14. 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].

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

  16. 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].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

  21. 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.

  22. 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].

Further Reading

Keywords

periventricular leukomalacia, PVL, ischemic brain injury, cerebral palsy, CP, hypotension, ischemia, coagulation necrosis, intracranial hemorrhage, ICH, hypocarbia, vasculitis, chorioamnionitis, cytokines, white matter damage, spastic diplegia, quadriplegia, nystagmus, strabismus, blindness, retinopathy of prematurity, maternal chorioamnionitis, respiratory distress syndrome, pneumonia, patent ductus arteriosus, placental vascular anastomoses, twin gestation, antepartum hemorrhage, sepsis

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.

Medical Editor

Scott S MacGilvray, MD, Associate Professor, Department of Pediatrics, East Carolina University School of Medicine
Scott S MacGilvray, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

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, Head, Division of Neonatal-Perinatal Medicine, Professor, Departments of Pediatrics and Obstetrics/Gynecology, 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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.