Related Experiment Videos
[Iterative intestinal intussusception and appendiceal mucocele in an infant with mucoviscidosis]
P Ekert1, J F Mougenot, P de Lagausie
1Service d'Hépatogastroentérologie et d'Endoscopie Digestive, Centre Hospitalier de Fountainebleau, France.
Insights
Cystic fibrosis (CF) can cause rare appendiceal issues like mucoceles and intussusceptions. Prompt diagnosis and surgical intervention, potentially including cecal resection, are crucial for managing these complications in CF patients.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Genetics
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, notably the digestive system.
- Appendiceal complications are infrequently reported in CF patients, presenting diagnostic challenges.
Observation:
- A 4.5-year-old boy with CF presented with an appendiceal mucocele and recurrent intussusceptions.
- Initial appendectomy did not resolve the intussusception, necessitating a subsequent ileocolic resection.
Findings:
- Appendiceal diseases in CF encompass a spectrum, including mucoceles and perforation, often with delayed diagnosis.
- Intussusception, though rare in CF, can be precipitated by appendiceal mucoceles.
Implications:
- Appendiceal mucoceles are a potential cause of intussusception in cystic fibrosis.
- Consideration of cecal resection during appendectomy may prevent recurrence of appendiceal mucoceles.
Unlabelled:
Cystic fibrosis is a common and potentially life-threatening hereditary disease which can affect numerous organs, particularly the digestive tract.
Case Report:
A 4.5-year-old boy exhibited two little known clinical manifestations: an appendiceal mucocele and repeated intussusceptions. In spite of an appendectomy, intussusception relapsed and an ileocolic resection was necessary 2 years later.
Discussion:
Appendiceal diseases in cystic fibrosis represent a large spectrum, ie, distention on the appendiceal lumen, engorged with sticky mucous matter, which becomes an appendiceal mucocele, peritonitis with an appendiceal perforation due to delayed diagnosis since acute appendicitis is difficult to diagnose in these patients. Intussusception is rarely observed in cystic fibrosis.
Conclusion:
Appendiceal mucocele could be a cause of intussusception. If an appendectomy is performed, resection of a part of the cecum, around the appendix, could be useful in preventing again mucocele formation.