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Lethal short-bowel syndrome
1Department of Pediatric General Surgery, University of Manitoba, Winnipeg, Canada.
Insights
Infants with severe short bowel syndrome often have poor outcomes despite medical and surgical interventions. Survival is unlikely for infants with less than 6 cm of small bowel, suggesting limited benefits of further treatment.
Area of Science:
- Pediatric surgery
- Gastroenterology
- Neonatal intensive care
Background:
- Short bowel syndrome (SBS) in infants presents significant management challenges.
- Current supportive measures including parenteral nutrition and surgical interventions offer limited success.
- Severe SBS often leads to poor prognoses and high mortality rates.
Purpose of the Study:
- To review outcomes of infants diagnosed with severe short bowel syndrome.
- To evaluate the efficacy of current management strategies for SBS.
- To determine prognostic indicators for survival in infants with SBS.
Main Methods:
- Retrospective review of seven infants with severe SBS.
- Analysis of causes including volvulus, atresias, and intestinal aganglionosis.
- Documentation of surgical procedures, total parenteral nutrition (TPN) duration, and survival times.
Main Results:
- Survival ranged from 15 days to 8 months, with death averaging 9 weeks post-diagnosis.
- Infants with less than 6 cm of small bowel beyond the Ligament of Treitz had an inevitably fatal outcome.
- TPN-related complications, including liver dysfunction and failure, were observed in multiple infants.
Conclusions:
- Severe SBS in infants, particularly with limited bowel length (<6 cm), is associated with a grim prognosis.
- While interventions may prolong survival, they do not alter the ultimate outcome and increase morbidity.
- Withholding further therapy upon diagnosis may be a reasonable consideration given the limited therapeutic options and poor prognosis.
Abstract:
Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included volvulus (3), multiple atresias (2), and total intestinal aganglionosis (2). Survival time ranged from 15 days to 8 months. During the hospital course, each infant underwent one to three operative procedures to diagnose and manage the short bowel and all received total parenteral nutrition (TPN) ranging from 10 days to 6 months. One infant died of liver failure and two others developed significant liver dysfunction secondary to TPN. Most infants remained hospitalized until their death. Death occurred at an average of 9 weeks following the diagnosis of short-bowel syndrome. This review suggests that infants with less than 6 cm of small bowel beyond the Ligament of Treitz will inevitably die of their disease or treatment complications. Until bowel transplant becomes a viable alternative, operative intervention and nutritional support may prolong survival but will not change the outcome of these infants and will only contribute to additional morbidity. A decision to withhold further therapy would be reasonable at the time the diagnosis is established.
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