Congenital malformation of the gastrointestinal tract in Aseer region, Saudi Arabia.

OBJECTIVE The aim of this prospective study was to evaluate the prevalence and pattern of congenital malformations of the gastrointestinal tract among the Saudi newborn population in Aseer region, Kingdom of Saudi Arabia. METHODS Every consecutive newborn admitted to the neonatal intensive care unit of Aseer Central Hospital, Kingdom of Saudi Arabia with features of gastrointestinal tract anomaly during the period January 1995 to December 2000 had relevant data obtained and entered into a program form. RESULTS During the 6 year period, a total of 1386 Saudi infants were admitted into the neonatal intensive care unit of Aseer Central Hospital. Of these, 12.4% were confirmed to have congenital malformation of the gastrointestinal tract; male/female ratio of 1.7:1. The total number of live births by Saudi mothers in Aseer region during the period was 128,093, giving an incidence rate of 1.3 per 1000 live births. The 172 newborns presented with 174 anomalies of the gastrointestinal tract. The leading malformations were imperforate anus (78 cases or 44.8%), tracheosophageal fistula/atresia (42 cases or 24.1%) and intestinal atresia (37 cases or 21.3%). Other lesions included Hirschsprung's disease (14 cases or 8%) and stenosis (2 pyloric and one duodenal) (1.7%). Some patients had more than one defect within the tract (1%) and multisystemic defects (23%). The overall fatality rate was (12%), due largely to post-operative infection (75% of cases) and multiple anomalies (25% of cases). CONCLUSION The prevalence of congenital defect of the gastrointestinal tract in Aseer region appears to be high. The incidence of associated multisystemic anomalies is also high. Fatality incidence is influenced by post-operative sepsis and associated multiple defects. A high incidence of consanguineous marriage in the region may be the underlying etiological factor hence genetic counseling may be helpful.