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During the period 1983-1987, 53 neonates (30 boys, 23 girls, mean birth weight 1,438 +/- 317 g, mean gestational age 30 +/- 2 weeks) were found to have cystic periventricular leukomalacias (CPVL) detected by brain ultrasonography. The neuro-developmental follow-up was 3-7 years. Seventeen CPVL were isolated and CPVL were associated with minor peri-intraventricular hemorrhage (0-1 to II-II) in 36 cases. CPVL included 16 minor forms (11 normal children; 2 minor, 3 moderate sequelae), 29 moderate forms (15 normal children; 4 minor, 7 moderate, 3 major sequelae) and 8 major forms (7 major sequelae, one moderate mental retardation without motor deficit). According to reverse analysis, a normal evolution (26 cases) followed CPVL which were sometimes widely, but thinly spread over the ventricles (thickness 1/5-1/3 of the cerebral mantle); minor sequelae (6 cases) were associated with a 1/4-1/3 thickness, moderate sequelae (11 cases) with a 1/3-1/2 thickness, major sequelae (10 cases) with a 1/2-2/3 thickness of CPVL. The thickness of the lesions appeared to be more predictive of sequelae than the sagittal extent, in particular that of the posterior CPVL which played a major role in the severity of sequelae, and mainly in the presence of motor deficits.

Thirty infant survivors had a wide spectrum of neonatal cystic periventricular leukomalacia (CPVL) diagnosed by ultrasound scans: 11 minor forms, 12 moderate forms, 7 major forms (categorized by both sagittal extent and coronal thickness). Eight cases were pure CPVL and 22 were associated with minor to moderate peri-intraventricular haemorrhage (PIVH), i.e. from (0-I) to (II-II). Mean gestational age: 31 +/- 2 weeks; mean birthweight: 1,532 +/- 356 g. The length of neurodevelopmental (ND) follow-up ranged from 13 months to 3 years and eight months. Of 11 children with minor CPVL, 9 were normal and two had moderate sequelae. Of 12 moderate CPVL, one was lost to follow-up, one had no motor disturbance but did have severe mental retardation (partial 7 p-), two were normal, one had a minor sequela, three had moderate and four had severe sequelae. Of the 7 major CPVL, one had moderate and 6 had severe sequelae. Cases including posterior lesions or presenting solely with such lesions had a worse outcome. In that range of PIVH, the outcome was related to the severity of CPVL and not to PIVH grade. All major late dilatations were associated with severe ND sequelae. Of 29 infants with early EEG tracings, the 7 children with early severe EEG abnormalities all had severe ND sequelae; 17 persistent EEG abnormalities, even mild, were associated with 9 severe and five moderate ND sequelae, and three normal outcomes. Combining US scans and EEG gives valuable prognostic information in minor and major CPVL, but less in the moderate forms.

In 30 surviving neonates, close prolonged ultrasonographic brain studies demonstrated cystic periventricular leucomalacias (CPVL) of varying degree (11 minor forms, 12 moderate forms, 7 severe forms). Clinical histories were reviewed for each case. There were 18 boys, 4 twins, 2 small-for-dates. Mean gestational age was 31 +/- 2 weeks, mean birthweight was 1532 +/- 356 g. No pregnancy was normal, but prenatal events were of a common occurrence in 26/30 cases (premature labor, toxaemia, twins...). Immediate perinatal events included cord difficulties in 5 cases (3 tight cords around the neck, 1 prolapse, 1 case where loose cord around the neck came down with the head), abruptio placentae (2), acute fetal distress (10, of which 8 were severe), Apgar scores 0-1 (9, of which 7 occurred after fetal distress and 2 were unexpected). In four cases, CPVL were of antenatal origin (already in the cystic stage on days 1-2). In 2 cases, CPVL occurred postnatally (infective shock on day 1 and day 46). Plausible mechanisms for anoxic-ischaemic lesions could only be found in 13 cases and remained unknown in the other 17. However, clinical histories suggested the following; cumulative minor events might become as damaging as single major events; "minor" fetal distresses should be scrutinized; pregnant women should be taught not to wait until late to arrive at hospital because this results in non-monitored delivery. No obvious relationship was found between the severity of known events and the degree of CPVL, but a number of pre- and perinatal periods were poorly monitored. The legal importance of early ultrasonographic studies was stressed.