Pediatric Periventricular Leukomalacia Workup
- Author: Terence Zach, MD; Chief Editor: Ted Rosenkrantz, MD more...
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 periventricular leukomalacia usually disappears at 2-3 weeks. Approximately 15% of infants experiencing periventricular leukomalacia demonstrate periventricular cysts first appearing at 2-3 weeks after the initial increased echodensities.
- The severity of periventricular leukomalacia 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 periventricular leukomalacia.
- Examples of cranial ultrasonography in periventricular leukomalacia are shown in the images below.
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.
- 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 periventricular leukomalacia.
- Findings include ventriculomegaly involving the lateral ventricles with irregular margins of the ventricles and loss of deep white matter. Examples of CT scanning in periventricular leukomalacia are shown in the images below.
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.
- MRI
- Like CT scanning, MRI does not play a major role in the early evaluation of periventricular leukomalacia. MRI is most helpful in monitoring infants with suspected periventricular leukomalacia and evaluating infants who develop clinical signs suggestive of periventricular leukomalacia.[10]
- 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 periventricular leukomalacia.
- 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.
- Examples of MRI in periventricular leukomalacia are shown in the images below.
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.
Other Tests
- EEG
Histologic Findings
- Periventricular leukomalacia lesions demonstrate widespread loss of oligodendrocytes and an increase in astrocytes.
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].
Perlman JM, Risser R, Broyles RS. Bilateral cystic periventricular leukomalacia in the premature infant: associated risk factors. Pediatrics. June 1996;97:822-7. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Canterino JC, Verma U, Visintainer PF, et al. Antenatal steroids and neonatal periventricular leukomalacia. Obstet Gynecol. Jan 2001;97(1):135-9. [Medline].
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].
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].
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].
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].
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].
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].
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].
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.
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.
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].
Haynes RL, Baud O, Li J, et al. Oxidative and nitrative injury in periventricular leukomalacia: a review. Brain Pathol. 2005;15:225-233. [Medline].
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].
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].
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].
[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].
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].
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].
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.
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].

