With improved survival from RDS, congenital malformations might contribute more to mortality and morbidity of VLBW. We examined survival and 20 month developmental outcome among 136 VLBW with major malformations (3.5% of 3862 admissions; bwt 1140 gm, gest age 30 wks) admitted to our NICU between 1975 and 1995. Only 36 (26%) infants survived to 20 months. Survival to 20 months was similar prior to 1990 (26%), and between 1990-1995 (29%), and similar at<1 kgm bwt (22%) and between 1-1.49 kgm bwt (28%).

Of the 100 deaths, 33% occurred at <1 day, 46% at 1-27 days and 21% after 28 days of life. All infants with major cardiac or pulmonary malformations died. Table

Table 1 No caption available.
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Subnormal cognitive function (MDI<70) occurred in 58% of malformed children vs 14% of VLBW without malformations, and neurosensory abnormality in 53% vs 13% without malformation. Chromosomal anomalies had the worst, and musculoskeletal and gastrointestinal malformations the best 20 month neurodevelopmental outcome.

The combination of VLBW and major congenital malformation has a very poor prognosis.