The whole spectrum of cystic periventricular leukomalacia of the preterm infant: results from a large consecutive case series

Purpose

The purpose of this study is to describe features of cystic periventricular leukomalacia (PVL) in a large consecutive cohort study including long-term neurodevelopmental follow-up.

Methods

We performed a retrospective single-centre cohort study including all preterm infants ≤35 weeks of gestational age with PVL diagnosed by ultrasound scans (US) from a tertiary care university hospital between 1988 and 2012.

Results

The majority of 160 consecutively diagnosed cases had a gestational age between 28 and 32 weeks (60.6 %), and male sex was predominant (60.6 %). The most common associated clinical findings included respiratory distress syndrome, preterm premature rupture of the membranes, and chorioamnionitis (57.5, 49.4, and 39.4 %, respectively). Infants presented with apnoeas in 66.3 and neonatal seizures in 23.1 %. Any kind of respiratory support was present in 75.0 %. Associated low-grade intraventricular haemorrhage was evident in 33.1, high-grade haemorrhage in 9.4 %. Cysts were located on both hemispheres in 75 % and PVL grades 3 and 4 were predominant (75.6 %). Neurodevelopmental follow-up of 146 cases at a median age of 72 months revealed normal development in 11.0, mental retardation in 50.0, and cerebral palsy in 83.6 %. Visual impairment was diagnosed in 21.9 % and hearing impairment in one case. A quarter of cases (27.4 %) developed seizure disorders. Outcome data were significantly better in unilateral compared to bilateral PVL.

Conclusions

Long-term neurodevelopmental outcome of bilateral PVL always was adverse and different from unilateral PVL. The latter might be negatively influenced by associated intra- and periventricular haemorrhages.