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Long-term outcome of congenital intestinal pseudoobstruction
Hayat Mousa1, Paul E Hyman, Jose Cocjin
1Division of Pediatrics Gastroenterology, Ohio-State University, Columbus Children's Hospital, 43205, USA.
Insights
Congenital chronic intestinal pseudoobstruction (CIP) in children often presents with prematurity and bladder issues. Parenteral nutrition complications are the primary cause of mortality in these patients.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
- Neonatology
Background:
- Congenital chronic intestinal pseudoobstruction (CIP) is a rare, severe gastrointestinal motility disorder.
- Understanding the clinical spectrum and outcomes of pediatric CIP is crucial for management.
- Previous studies have not fully characterized the associated conditions and mortality factors in a large pediatric cohort.
Purpose of the Study:
- To evaluate the clinical characteristics, associated conditions, and mortality of children diagnosed with congenital chronic intestinal pseudoobstruction (CIP).
- To identify risk factors for mortality in pediatric CIP patients.
- To analyze the relationship between manometric findings, bladder involvement, and outcomes.
Main Methods:
- Retrospective chart review of 85 children diagnosed with CIP over a 10-year period.
- Analysis of patient demographics, clinical history, manometric findings, systemic diseases, urinary bladder involvement, nutritional support, and mortality data.
- Statistical comparison of outcomes based on disease subtype (myopathy vs. neuropathy) and nutritional support method.
Main Results:
- 14% of patients were born prematurely; most cases were sporadic.
- Urinary bladder involvement was observed in 41% of patients, significantly more common in myopathic CIP (P < 0.0001).
- 62% required partial or total parenteral nutrition (PN) at referral. Mortality was higher in patients on PN (22 deaths), with PN-related complications causing 13 deaths.
Conclusions:
- Children with congenital CIP have an increased risk of prematurity.
- Abnormal bladder function is more prevalent in myopathic CIP.
- Complications associated with parenteral nutrition represent the leading cause of death in pediatric patients with congenital CIP.
Abstract:
We evaluated 85 children with congenital chronic intestinal pseudoobstruction (CIP) over the past 10 years. Twelve (14%) were born prematurely. One had a family history of CIP. Six had systemic diseases. Thirty-five (41%) had urinary bladder involvement. Manometric features were consistent with myopathy in 32, neuropathy in 48, and mixed disease in 5. Of 48 patients with neuropathy, 6 had urinary bladder involvement (12.5%) (P < 0.0001 vs myopathy), and 10 had malrotation (21%) (P = NS vs myopathy). Upon referral, 53 (62%) were dependent on partial or total parenteral nutrition (PN). At the time of chart review (median 25 months after evaluation), 22 patients had died, 14 of whom were on total PN, 13 of them died because of PN-related complications and 1 died of sepsis. Three others died of sepsis while on partial PN (P = 0.007 vs mortality in patients fed enterally) and five died after small bowel transplantation. In conclusion, in children with congenital CIP, the risk for prematurity is increased twofold, the majority of cases are sporadic, abnormal bladder function is more common in myopathic CIP, and complications related to parenteral nutrition are the main cause of death in children with CIP.