Neonatal short bowel syndrome: population-based estimates of incidence and mortality rates

Presented at the 35th Annual Meeting of the Canadian Association of Paediatric Surgeons, Niagara-on-the-Lake, Ontario, Canada, September 18–21, 2003.
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Abstract

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.

Section snippets

Materials and methods

A retrospective cohort study was performed including all neonates admitted to the neonatal intensive care unit (NICU) at The Hospital for Sick Children (HSC) in Toronto between January 1, 1997 and December 31, 1999 with abdominal pathology requiring laparotomy. The conclusion of the study follow-up period was July 1, 2001. The study cohort was divided into patients with SBS (n = 40) and surgical neonates who did not have SBS (n = 135). The Canadian Association of Paediatric Surgeons (CAPS)

Patient population

During the 2-year enrolment period, 1,811 patients were admitted to the NICU. Two hundred nineteen patients had abdominal operations, but 44 were excluded for reasons outlined above. One hundred seventy-five patients made up the study cohort. The cohort represents 9.6% (175 of 1,811) of all NICU admissions. Follow-up continued to the date of death or study closure (July 1, 2001) in all but 2 patients. Both patients were out-of-province referrals and were transferred to an institution closer to

Study design

A retrospective cohort study allows determination of disease incidence directly, as it investigates the relationship between exposure and disease.8 The major limitation, however, of a retrospective cohort study is bias. Bias represents a systematic error that results in incorrect estimation of the association between exposure and disease.7 Every type of analytic study is susceptible to bias. A reader must decide if bias distorts the results sufficiently to disregard the conclusions.

Selection

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