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Morbidity and mortality of the short-bowel syndrome
J M Mayr1, P H Schober, U Weissensteiner
1Department of Pediatric Surgery, University of Graz, Austria, Europe.
Insights
Neonates with short-bowel syndrome fare better with an intact ileocecal valve and preserved colon. Intestinal length and motility significantly impact outcomes and complications in these pediatric patients.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatal Care
Background:
- Short-bowel syndrome (SBS) is a severe malabsorptive condition in neonates.
- Management of SBS is complex, with outcomes influenced by residual intestinal length and function.
Purpose of the Study:
- To evaluate the impact of the ileocecal valve and colon length on outcomes in neonates with short-bowel syndrome.
- To identify factors influencing complications and long-term prognosis in this patient population.
Main Methods:
- Retrospective review of 17 neonates treated for short-bowel syndrome between 1976 and 1998.
- Analysis of patient data including intestinal length, ileocecal valve status, colon preservation, motility, and clinical outcomes.
Main Results:
- Patients with an intact ileocecal valve and preserved colon had significantly better outcomes compared to those without.
- Intestinal length and motility were major determinants of complications and survival.
- Four patients (23.5%) died, all with severe risk factors (short intestinal length, dysmotility, absent ileocecal valve, incomplete colon).
- Hospitalization averaged 8.5 months for those weaned from parenteral nutrition, with ongoing need for supplementation.
- Long-term sequelae included d-lactic acidemia and psychomotor developmental delay.
Conclusions:
- Preservation of the ileocecal valve and colon is crucial for improved outcomes in neonates with short-bowel syndrome.
- Intestinal motility and length are critical prognostic indicators.
- Multifaceted long-term care, including nutritional support and developmental monitoring, is essential for survivors.
Abstract:
From 1976 to 1998 we have treated 17 neonates with short-bowel syndrome. Those 8 patients who had an intact ileocecal valve as well as the total colon preserved did significantly better than the 9 children without ileocecal valve and > 50% missing colon. In addition to the length of the intestinal remnants, motility had a major impact on the incidence of complications and final outcome. Four patients died (23.5%). All of them had an intestinal length of less than 30 cm, severe dysmotility, no ileocecal valve and an incomplete colon. The average duration of hospitalization of the children weaned from parenteral nutrition (n = 11) was 8.5 months. The majority of them still need supplementation of vitamins and/or trace elements. Two children suffer from recurrent d-lactic acidemia. Six children have a significant psychomotor developmental delay with three suffering from congenital cerebral abnormalities.