ABSTRACT
To determine the pattern of major congenital malformations in neonates admitted in a tertiary care NICU and evaluate their early outcome. Descriptive Study. Department of Pediatrics, Liaquat National Hospital and Medical College Karachi, from January to December 2009. A total of 431 neonates were admitted in NICU including those referred from outside. Children with major congenital malformations were identified by clinical examination and confirmed by appropriate radio-diagnostic methods. These neonates were immediately referred to the surgical team for intervention. A total of 57 neonates were admitted during the study period. Thirty one were males and 26 females. Fetal anomalies were diagnosed correctly in 17 cases out of a total of 19 inborn deliveries on maternal ultrasound while it was missed in one fetus and incorrectly diagnosed in one case. A total of 48 patients had surgery out of which 4 [8.3%] died in the neonatal period. Five cases were booked for elective surgery beyond the neonatal period. Out of 4 neonates with congenital heart disease one case was referred outside, one neonate died preoperatively while 2 infants were managed conservatively. Due to detection of fetal anomalies, early surgical intervention, and intensive neonatal care, most infants can be rescued after a successful primary operation