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Cardiac arrest in two children with nonfamilial chronic intestinal pseudoobstruction on total parenteral nutrition
Insights
Two children with nonfamilial chronic intestinal pseudoobstruction, a rare condition, experienced whole-small intestine dilatation and required long-term parenteral nutrition. The cause of their fatal cardiac arrest remains unknown.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
- Clinical Case Reports
Background:
- Chronic intestinal pseudoobstruction (CIPO) is a rare motility disorder.
- Nonfamilial CIPO presents unique challenges compared to familial forms.
- Parenteral nutrition is often necessary for nutritional support in severe CIPO.
Observation:
- Two pediatric cases of nonfamilial CIPO with diffuse small intestinal dilatation were observed.
- Clinical manifestations were similar, but gastrointestinal histology differed between patients.
- Both children required long-term parenteral nutrition for nutritional support.
Findings:
- The observed whole-length small intestinal dilatation distinguished these cases from familial CIPO with segmental dilatation (megaduodenum).
- Both patients experienced fatal cardiac arrest during long-term parenteral hyperalimentation.
- Autopsy revealed normal heart findings in one child and a small cardiac infarction in the other.
Implications:
- The etiology of cardiac arrest in these nonfamilial CIPO cases is unclear, warranting further investigation.
- Understanding the distinct pathophysiology of nonfamilial CIPO is crucial for improved pediatric patient outcomes.
- This case series highlights potential risks associated with long-term parenteral nutrition in pediatric CIPO.
Abstract:
Two children with nonfamilial chronic intestinal pseudoobstruction are reported. Both had no family history. They had an exploratory laparotomy to rule out mechanical intestinal obstruction, and required long-term parenteral nutrition to obtain sufficient nutrients. These two children had dilatation of the whole length of the small intestine, which differs from short segmental dilatation (megaduodenum) in patients with familial chronic intestinal pseudoobstruction. There were also differences in the histology of the gastrointestinal tract between these two patients although they had similar clinical manifestations. Both patients died from cardiac arrest, one after 2 years and the other after 4 months on long-term parenteral hyperalimentation. At autopsy, heart examination was normal in one patient, and a small infarction (0.4 mm diameter) was found in the other. Although mild hypokalemia was found in one case, and mild hyperkalemia in the other, the cause of cardiac arrest in these two children is not known.