Chronic intestinal pseudo-obstruction (CIPO) is a rare clinical syndrome characterized by impairment of gastrointestinal (GI) motility which resembles mechanical obstruction, in the absence of any obstructive process. Incidence and prevalence of CIPO is still unknown and the few data available come from small case series. CIPO can affect any segment of the GI tract (the small bowel and the colon are more frequently involved) and represents the most severe form of GI dysmotility. CIPO may have different etiologies: primary (familial or sporadic), secondary and idiopathic. Primary CIPO are usually diagnosed in childhood and may be sporadic or familiar. This condition is characterized by abnormalities within the development, degeneration, or inflammation of the enteric nervous system (ENS) and/or of the enteric muscles. Secondary CIPO results from a well-defined disease affecting the intestinal smooth muscle, enteric neurons and the interstitial cell of Cajal (ICC) network and represents up to 50% of the causes in adult population. The last form of CIPO, defined idiopathic, is established when neither a primary or secondary etiology is identified, and represents the majority ofpediatric CIPO cases. Based on abnormal histopathological findings along with the GI system, CIPO has been classified into different groups (neuropathic, myopathic or mesenchymopathic). Frequently, more than one histopathological alteration coexists in a single CIPO patient.
Author(s) Details:
Giulia Radocchia
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Bruna Neroni
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Massimiliano Marazzato
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Elena Capuzzo
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Simone Zuccari
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Fabrizio Pantanella
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Letizia Zenzeri
NESMOS Department, Paediatric Unit, Faculty of Medicine and Psychology, Sapienza University of Rome, Sant’Andrea University Hospital, Rome, Italy and Paediatric Emergency Department, Santobono-Pausilipon Children’s Hospital, Naples, Italy
Melania Evangelisti
NESMOS Department, Paediatric Unit, Faculty of Medicine and Psychology, Sapienza University of Rome, Sant’Andrea University Hospital, Rome, Italy.
Francesca Vassallo
NESMOS Department, Paediatric Unit, Faculty of Medicine and Psychology, Sapienza University of Rome, Sant’Andrea University Hospital, Rome, Italy.
Pasquale Parisi
NESMOS Department, Paediatric Unit, Faculty of Medicine and Psychology, Sapienza University of Rome, Sant’Andrea University Hospital, Rome, Italy.
Giovanni Di Nardo
NESMOS Department, Paediatric Unit, Faculty of Medicine and Psychology, Sapienza University of Rome, Sant’Andrea University Hospital, Rome, Italy.
Serena Schippa
Department of Public Health and Infection Disease, Microbiology Section, Sapienza University of Rome, Italy
Recent Global Research Developments in Clinical Features, Diagnosis, and Therapy of Chronic Intestinal Pseudo-Obstruction
Associations with Gut Microbiota and Intestinal Serotonergic Pathway:
A study published in BMC Microbiology [1] investigated the interplay between gut microbiota and the intestinal serotonergic pathway in pediatric CIPO patients.
PIPO (Pediatric Intestinal Pseudo-Obstruction) is a rare condition characterized by symptoms suggestive of intestinal obstruction without mechanical blockages.
The study found that PIPO patients exhibit a distinct mucosa-associated microbiota (MAM) composition, with dysbiosis (lower biodiversity and altered species connections) compared to healthy controls.
Additionally, alterations in the expression of serotonin-related genes (TPH1, SLC6A4, 5-HTR3, and 5-HTR4) were observed in PIPO patients.
The dysfunction of the serotonin pathway, possibly triggered by an altered microbiota, may contribute to dysmotility in PIPO patients.
Progress and Challenges in Pediatric Intestinal Pseudo-Obstruction:
A review published in Frontiers in Pediatrics [2] provides an overview of etiology, pathophysiology, clinical features, diagnostics, and available treatments for PIPO as of 2022.
Despite being underdiagnosed, research in this area continues to evolve.
Scoping Review on PIPO:
A scoping review published in Springer [3] highlights the challenges in managing PIPO due to limited evidence on medical and surgical therapies.
Advances in Diagnosis, Treatment, and Prognosis:
Research by Köttgen et al. (MDPI)4 contributes to our understanding of CIPO, although specific details were not provided in the search results.
References
- Radocchia, G., Marazzato, M., Harbi, K.B. et al. Chronic intestinal pseudo-obstruction: associations with gut microbiota and genes expression of intestinal serotonergic pathway. BMC Microbiol 24, 48 (2024). https://doi.org/10.1186/s12866-024-03200-z
- Turcotte M-C and Faure C (2022) Pediatric Intestinal Pseudo-Obstruction: Progress and Challenges. Front. Pediatr. 10:837462. doi: 10.3389/fped.2022.837462
- Nham, S., Nguyen, A. T., & Holland, A. J. (2022). Paediatric intestinal pseudo-obstruction: a scoping review. European Journal of Pediatrics, 181(7), 2619-2632.
- De Giorgio, R., Cogliandro, R. F., Barbara, G., Corinaldesi, R., & Stanghellini, V. (2011). Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy. Gastroenterology Clinics, 40(4), 787-807.
