Periventricular leukomalacia

  • Definition
    • Periventricular leukomalacia (PVL) is a type of brain injury that affects infants. The condition involves the death of small areas of brain tissue around fluid-filled areas called ventricles. The damage creates "holes" in the brain. "Leuko" refers to the brain's white matter. "Periventricular" refers to the area around the ventricles.

  • Alternative Names
    • PVL; Brain injury - infants

  • Causes
    • PVL is much more common in premature infants than in full-term infants.

      A major cause is thought to be changes in blood flow to the area around the ventricles of the brain. This area is fragile and prone to injury, especially before 32 weeks of gestation.

      Infection around the time of delivery may also play a role in causing PVL. The risk for PVL is higher for babies who are more premature and sicker at birth.

      Premature babies who have intraventricular hemorrhage (IVH) are also at increased risk for developing this condition.

  • Exams and Tests
    • Tests used to diagnose PVL include ultrasound and MRI of the head.

  • Treatment
    • There is no treatment for PVL. Premature babies' heart, lung, intestine, and kidney functions are watched closely and treated in the newborn intensive care unit (NICU). This helps reduce the risk of developing PVL.

  • Outlook (Prognosis)
    • PVL often leads to nervous system and developmental problems in growing babies. These problems most often occur during the first to second year of life. It may cause cerebral palsy (CP), especially tightness or increased muscle tone (spasticity) in the legs.

      Babies with PVL are at risk for major nervous system problems. These are likely to include movements such as sitting, crawling, walking, and moving the arms. These babies may need physical therapy. Extremely premature babies may have more problems with learning than with movement.

      A baby who is diagnosed with PVL should be monitored by a developmental pediatrician or a pediatric neurologist. The child should see the regular pediatrician for scheduled exams.

  • References
    • Greenberg JM, Narendran V, Schibler MD, Warner B, et al. Neonatal morbidities of prenatal and perinatal origin. In: Creasy RK, Resnick R, Iams JD, et al, eds. Creasy and Resnik's Maternal-Fetal Medicine: Principles and Practice. 7th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 72.

      Johnston MV. Encephalopathies. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 598.

      Marlow N, Wolke D, Bracewell MA, Samara M; EPICure Study Group. Neurologic and developmental disability at six years of age after extremely preterm birth. N Engl J Med. 2005;352(1):9-19. PMID: 15635108

      Volpe JJ. Hypoxic-ischemic encephalopathy: neuropathology and pathogenesis. In: Volpe JJ, ed. Neurology of the Newborn. 5th ed. Philadelphia, PA: Elsevier Saunders; 2008:chap 8.