BACKGROUND: Congenital or acquired neonatal short bowel syndrome (SBS) carries significant morbidity and mortality rates. No accurate population estimates of incidence and mortality exist because of differences in definition, follow-up, and regional referral patterns.METHODS: A retrospective cohort study was performed involving 175 surgical neonates admitted to our institution from January 1, 1997 to December 31, 1999 and followed up until July 1, 2001. Institution and population-based estimates of incidence and mortality were performed using postcensal population figures (1997) from Statistics Canada.RESULTS: The overall incidence of SBS was 22.1 per 1,000 neonatal intensive care unit (NICU) admissions (95% CI = 15.3, 28.9) and 24.5 per 100,000 live births (95% CI = 12.1, 36.9). The incidence was much greater in premature infants (less than 37 weeks). The SBS case fatality rate was 37.5% (95% CI = 22.5, 52.5) and the cause-specific and proportional mortality rates (for children less than 4 years old) were 2.0 of 100,000 population per year (0.4 to 3.6/100,000/year) and 1.4% (0.3% to 2.6%), respectively.CONCLUSIONS: Patients with neonatal SBS pose a complex management challenge and are responsible for a significant cost to the health care system. To our knowledge, this study represents the first population-based estimates for neonatal SBS incidence and mortality rates. Accurate estimates will assist clinicians in counseling parents, allocating resources, and planning clinical trials.
Cohort Studies Female