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Assessment and outcome of pediatric intestinal pseudo-obstruction: A tertiary-care-center experience from Turkey
Yeliz Çağan Appak1, Maşallah Baran2, Mustafa Onur Öztan3
1Department of Pediatric Gastroenterology, Hepatology and Nutrition, SBU Tepecik Training and Research Hospital, İzmir, Turkey.
Insights
Pediatric intestinal pseudo-obstruction (PIPO) management varies. While some patients achieve oral nutrition or require small bowel transplantation (SBT), individualized supportive care is crucial for this rare motility disorder.
Area of Science:
- Pediatric Gastroenterology
- Motility Disorders
- Surgical Gastroenterology
Background:
- Pediatric intestinal pseudo-obstruction (PIPO) is a severe, rare gut motility disorder.
- Management is complex, sometimes necessitating intestinal transplantation.
- This study evaluates PIPO patient management and outcomes at a specialized center.
Purpose of the Study:
- To assess the management strategies and follow-up results for pediatric patients diagnosed with intestinal pseudo-obstruction.
- To identify factors influencing treatment response and long-term outcomes in PIPO patients.
Main Methods:
- Retrospective review of 13 pediatric intestinal pseudo-obstruction cases.
- Analysis of demographic data, clinical features, etiologies, and treatments (pharmacological, surgical, nutritional).
- Evaluation of outcomes including nutritional support, anthropometric changes, complications, small bowel transplantation (SBT), and survival rates.
Main Results:
- Etiology remained unidentified in 5 patients; 38.4% showed pharmacological treatment response.
- Total oral nutrition was achieved in 38.4% of previously TPN-dependent patients, with improved anthropometrics.
- Complications included liver cirrhosis (1 patient) and venous thrombosis (4 patients); 3 patients underwent SBT.
Conclusions:
- Pediatric intestinal pseudo-obstruction presents with diverse clinical symptoms and requires individualized management.
- While supportive care can be sufficient for some, others may need interventions like small bowel transplantation.
- Tailored treatment approaches are essential for optimizing outcomes in PIPO patients.
Background/Aims:
Pediatric intestinal pseudo-obstruction (PIPO) is a severe disorder of gut motility. In this rare and difficult-to-manage disease, complex treatment method, such as intestinal transplantation, is sometimes needed. This study evaluated the management and follow-up results of patients with PIPO who received treatment at our center.
Materials And Methods:
The cases of 13 patients with PIPO were reviewed retrospectively. Demographic data, clinical features, etiologies, pharmacological and surgical treatments, nutritional support, anthropometric findings, small bowel transplantation (SBT), and survival rates were assessed.
Results:
Two of the patients were diagnosed at 1 and 5 years of age, while other patients were diagnosed during neonatal period. The etiological cause could not be identified for 5 patients. Pharmacological treatment response was observed in 38.4% of patients. Post-pyloric feeding was applied in 4 patients, but no response was observed. Gastrostomy decreased the clinical symptoms in 3 patients during the abdominal distension period. Total oral nutrition was achieved in 38.4% of the total-parenteral-nutrition (TPN)-dependent patients. It was observed that anthropometric findings improved in patients with total oral nutrition. Liver cirrhosis developed in 1 patient. Venous thrombosis developed in 4 patients. The SBT was performed on 3 patients. One of these patients has been followed up for the last 4 years.
Conclusion:
Pediatric intestinal pseudo-obstruction is a rare disease that can present with a wide range of clinical symptoms. While some patients require intestinal transplantation, supportive care may be sufficient in others. For this reason, patients with PIPO should be managed individually.
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