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  • Title: [Iterative intestinal intussusception and appendiceal mucocele in an infant with mucoviscidosis].
    Author: Ekert P, Mougenot JF, de Lagausie P, Gerardin M, Le Bourgeois M, Munck A, Aigrain Y, Navarro J.
    Journal: Arch Pediatr; 1998 Apr; 5(4):400-3. PubMed ID: 9759160.
    Abstract:
    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.
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