[Neonatal lenticulostriate vasculopathy. Mid and longterm outcome (70 cases)].

Arch Pediatr

Service de médecine néonatale de Port-Royal, AP-HP, 123, boulevard de Port-Royal, 75679 Paris 14, France.

Published: January 2004

Objectives: If the pattern of neonatal lenticulostriate vasculopathies (NLSV) is well-known, the prognosis is unknown except in TORCH syndromes. This study was aimed to describe the short, mid and long-term outcome of NLSV of various origins.

Population And Methods: Of 9138 neonates (1981-2000) which were submitted to an early brain ultrasound study, 70 presented with a pattern of minor (35), moderate (27) or severe (8) NLSV, a single finding in 42 cases and in association with minor peri-intraventricular haemorrhage and/or leukomalacias in 28. The maternal and neonatal charts were reviewed, and the survivors were followed according to our usual policy.

Results: Of nine deaths, eight cases included severe congenital defects (metabolic or malformations or acquired: transfused monochorial twins). Of 61 survivors, eight were lost to follow-up within the first year, 53 were followed for 21 months to 9 years and 7 months (median 4 years 5 months). Of 53 children, 35 (66%) were strictly normal, eight had minor deviations, four had moderate and six had major neurodevelopmental deficits. Of 34 survivors with isolated NLSV and known follow-up, 27 were normal (79%) versus 8/19 (42%) in associated NLSV.

Conclusions: Minor or moderate isolated NLSV generally have a good long-term prognosis. Associated forms of any severity depend mainly upon the severity of periventricular leukomalacias. Major forms of NLSV must be a warning sign of a possible underlying congenital anomaly which will rule the vital and functional prognosis.

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http://dx.doi.org/10.1016/j.arcped.2003.10.003DOI Listing

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