Related Experiment Videos
Morbidity and mortality of short-bowel syndrome acquired in infancy: an update
Insights
Total parenteral nutrition (TPN) improves survival in infants with short-bowel syndrome (SBS). However, TPN-associated liver disease remains a concern, impacting long-term quality of life for these patients.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Nutritional Support
Background:
- Total parenteral nutrition (TPN) enables survival in infants with massive small intestinal loss.
- Long-term TPN use and short-bowel syndrome (SBS) complications limit quality of life.
Purpose of the Study:
- To evaluate improvements in morbidity and mortality for short-bowel syndrome (SBS) over the last decade.
- To analyze outcomes in infants with over 50% small intestinal loss.
Main Methods:
- Retrospective review of 16 patients with SBS diagnosed since 1973.
- Analysis of etiologies, survival rates, small intestinal length (SIL), and adaptation to enteral feeding.
Main Results:
- Overall survival rate was 81%.
- Small intestinal length (SIL) at diagnosis was not significantly different between survivors and non-survivors.
- Deaths were primarily due to TPN-associated cholestasis leading to end-stage liver disease, not directly to SIL.
Conclusions:
- While TPN improves survival in SBS, TPN-associated cholestasis is a critical factor affecting long-term outcomes.
- Further research is needed to mitigate TPN-related complications in pediatric SBS patients.
Abstract:
The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequelae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2 +/- 7.9 cm in survivors and 30.3 +/- 7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8 +/- 2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87 +/- 1.32 kg.(ABSTRACT TRUNCATED AT 250 WORDS)