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Periventricular leukomalacia in combination with intraventricular hemorrhage: sonographic features and sequelae.

Periventricular leukomalacia (PVL) is well recognized as a relatively uncommon yet particularly serious complication of prematurity. Although the sonographic features of PVL have been described, its association with intraventricular hemorrhage (IVH) has not been emphasized. Reviewing 26 consecutive cases of PVL in neonates of 34 weeks or less gestational age, significant associated hemorrhage was found in six (23%). Small quantities of blood were also noted in most of the other 20 infants. Of the six infants with both significant IVH and PVL, five required ventricular shunt and all had particularly poor clinical outcomes. In the neonates who required surgical intervention, rapid ventricular enlargement was accompanied by extensive periventricular cyst formation. Eventually, the septations within the cysts and frequently even the ependyma of superior/posterior lateral ventricles degenerated. Cysts merged imperceptibly with the ventricles giving an appearance that mimicked severe hydrocephalus. This was termed "pseudoventricle formation," as the large intracerebral cerebrospinal fluid spaces are primarily porencephaly and not enlarged ventricles. Response to shunting was minimal by sonography in all five cases and multiple shunt revisions were required in four. Clinical follow-up in children with significant IVH in combination with PVL has shown severe mental retardation and tetraplegia in all cases.