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Published on: March 5, 2018
Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients
S Heneyke1, V V Smith, L Spitz
1Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.
Insights
Chronic idiopathic intestinal pseudo-obstruction (CIIPS) in children often has a poor outcome, especially with malrotation or urinary tract issues. Surgical decompression stomas are more effective than medications for managing this condition.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
- Surgical Management of Pediatric GI Conditions
Background:
- Chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) is a severe condition affecting children.
- Understanding the long-term course and prognostic factors is crucial for effective management.
Purpose of the Study:
- To document the long-term course of CIIPS in children with enteric neuromuscular disease.
- To evaluate the role and types of surgical interventions used in CIIPS management.
- To identify factors predicting the outcome of CIIPS in pediatric patients.
Main Methods:
- Prospective investigation and treatment of children diagnosed with CIIPS.
- Detailed analysis of clinical presentation, histology, surgical interventions, and outcomes.
- Identification of prognostic indicators associated with disease progression and mortality.
Main Results:
- A significant proportion of children (24/44) experienced poor outcomes, including death or dependence on parenteral nutrition.
- Malrotation, short small bowel, urinary tract involvement, and myopathic histology were associated with adverse outcomes.
- Surgical decompression stomas provided symptom relief, whereas prokinetic drugs showed limited efficacy.
Conclusions:
- CIIPS in children frequently leads to poor long-term outcomes, particularly in those with specific comorbidities.
- Surgical decompression stomas are a valuable intervention for CIIPS, unlike pharmacological treatments.
- Early identification of prognostic factors is essential for guiding management strategies in pediatric CIIPS.
Aims:
To document the long term course of chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) in children with defined enteric neuromuscular disease, and the place and type of surgery used in their management; in addition, to identify prognostic factors.
Methods:
Children with CIIPS were investigated and treated prospectively.
Results:
Twenty four children presented congenitally, eight during the 1st year of life, and 10 later. Twenty two had myopathy and 16 neuropathy (11 familial). Malrotation was present in 16 patients, 10 had short small intestine, six had non-hypertrophic pyloric stenosis, and 16 had urinary tract involvement. Thirty two patients needed long term parenteral nutrition (TPN): for less than six months in 19 and for more than six months in 13, 10 of whom are TPN dependent; 14 are now enteral feeding. Prokinetic treatment improved six of 22. Intestinal decompression stomas were used in 36, colostomy relieved symptoms in five of 11, and ileostomy in 16 of 31. A poor outcome (death (14) or TPN dependence (10)) was seen with malrotation (13 of 16), short small bowel (eight of nine), urinary tract involvement (12 of 16), and myopathic histology (15 of 22).
Conclusions:
In CIIPS drugs are not helpful but decompression stomas are. Outcome was poor in 24 of 44 children (15 muscle disorder, 10 nerve disease).
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