Cystic periventricular leukomalacia and type of cerebral palsy in preterm infants

NEURODEVELOPMENTAL DISABILITIES Sponsored by the Society for Developmental Pediatrics

Cystic periventricular leukomalacia and type of cerebral palsy in preterm infants B. Rogers, MD, M. Msall, MD, T. O w e n s , BS, K. Guernsey, BS, A. Brody, MD, G. Buck, PhD, a n d M. Hudak, MD From the Departments of Pediatrics, Radiology, and Social and Preventive Medicine, State University of New York at Buffalo, School of Medicine and Biomedical Sciences

A case series design was used to identify cases of cystic periventricular leukomalacia (N = 31) identified by neurosonography at one regional tertiary intensive care nursery. Patients were preterm infants born at ~ 3 2 weeks of gestation who had cysts involving predominantly the middle-posterior or posterior periventricular regions. Neurodevelopmental evaluations were m a d e for 26 (96%) of 27 survivors. All infants assessed had cerebral palsy (i.e., 54% quadriplegia, 42% diplegia, and 4% hemiplegia). Most cognitive delays and all sensory impairments occurred in children with quadriplegia. Periventricular cysts were most extensive on parasagittal, anteroposterior views. The parasagittal, anteroposterior extent of periventricular cysts was most accurate in predicting the type and severity of motor and cognitive disabilities. Quadriplegia was associated with larger and more extensive cysts. (J PEDIATR1994;125:$1"8)

Periventricular leukomalacia is defined as necrosis of the white matter, dorsal and lateral to the external angles of the lateral ventricles; PVL characteristically is located at the level of the optic radiations adjacent to the trigone and at the level of the frontal cerebral white matter near the foramen of Monro.l3 The corticospinal tracts are frequently involved, and PVL has classically been associated with the development of spastic diplegia in preterm infants. Characteristics of preterm infants that appear to be important in the pathogenesis of PVL include periventricular arterial "distal fields," pressure-passive cerebral circulation, and enhanced vulnerability of actively differentiating and/or myelinating periventricular glial cells.l4 Periventricular leukomalacia has been defined by the presence of hyperechoic or hypoechoic lesions (cysts) on cranial ultrasound studies. 5, 6 Transient periventricular hySupported in part by the Children's Guild of Buffalo, N.Y., and the New York State Office of Mental Retardation and Developmental Disabilities (RF 150-6619 D). Reprint Brian Rogers, MD, Robert Warner Rehabilitation Center of the Children's Hospital of Buffalo, 936 Delaware Ave., Buffalo, NY 14209. Copyright ® 1994 by Mosby-Year Book, Inc. 0022-3476/94/$3.00 + 0 9/73/55237

perechoic lesions have not been associated with developmental disabilities. On the other hand, periventricular cysts are the most sensitive and specific neurosonographic predictors of cerebral palsy in preterm infants. 7"11 Surviving infants with cystic PVL have variable motor outcomes. The prevalence of all types of cerebral palsy has ranged from 38% to 93%. T M Spastic cerebral palsy has uniformly been reported, including 21% to 69% diplegia, 27% to 71% quadriplegia, and 0% to 33% hemiplegia. T M Functional motor skills of survivors have rarely been reported. 11, 12 Additionally, efforts have been made to examine the relation between the location and extent of cysCAT-CLAMS [VH PVL

Clinical Adaptive Test-Clinical Linguistic Auditory Milestone Scale Intraventricular hemorrhage Periventricular leukomalacia

tic PVL and motor outcome. The methods used in these studies, however, have been difficult to interpret and apply in the follow-up of high-risk preterm infants. T M The purpose of this study was to evaluate the location and size of cystic PVL in relation to type and severity of cerebral palsy in preterm infants. Such research will provide a

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The Journal of Pediatrics July 1994

Anterior-Posterior

T a b l e I. Description of study sample by select sociodemographic and neonatal characteristics (n = 31) Sample Characteristic Cranial-Caudal

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Medial-lateral M e a s u r e m e n t

1 Fig. t. A, Neurosonographicmeasurements on parasagittal view. B, Neurosonographic measurements on coronal view.

clearer understanding of motor outcome, type of cerebral palsy, and functional skills of surviving infants with cystic PVL, and thereby will facilitate counseling of parents and early intervention efforts. METHODS A case series design was used for study purposes. The study population consisted of preterm infants born at __2 cm on at least one side was uniformly associated with quadriplegia. Greater anteroposterior measure-

ments of cystic PVL in children with quadriplegia was also reflected by the number of periventricular zones involved. All children with diplegia (n = 11) had cystic PVL involving only one zone (middle or posterior). In contrast, only 3 (21%) of the 14 children with quadriplegia had cystic PVL that was confined to a single zone. The sole child with hemiplegia had a left-sided cystic PVL