Abstract
We evaluated 85 children with congenital chronic intestinal pseudoobstruction (CIP) over the past 10 years. Twelve (14%) were born prematurely. One had a family history of CIP. Six had systemic diseases. Thirty-five (41%) had urinary bladder involvement. Manometric features were consistent with myopathy in 32, neuropathy in 48, and mixed disease in 5. Of 48 patients with neuropathy, 6 had urinary bladder involvement (12.5%) (P < 0.0001 vs myopathy), and 10 had malrotation (21%) (P = NS vs myopathy). Upon referral, 53 (62%) were dependent on partial or total parenteral nutrition (PN). At the time of chart review (median 25 months after evaluation), 22 patients had died, 14 of whom were on total PN, 13 of them died because of PN-related complications and 1 died of sepsis. Three others died of sepsis while on partial PN (P = 0.007 vs mortality in patients fed enterally) and five died after small bowel transplantation. In conclusion, in children with congenital CIP, the risk for prematurity is increased twofold, the majority of cases are sporadic, abnormal bladder function is more common in myopathic CIP, and complications related to parenteral nutrition are the main cause of death in children with CIP.
Similar content being viewed by others
REFERENCES
Rudolph CD, Hyman PE, Altschuler SM, Christensen J, Colletti RB, Cucchiara S, Di Lorenzo C, Flores AF, Hillemeier AC, McCallum RW, Vanderhoof JA: Diagnosis and treatment of chronic intestinal pseudo-obstruction in children: report of consensus workshop. J Pediatr Gastroenterol Nutr 24:102–112, 1997
Di Lorenzo C: Pseudo-obstruction: Current approaches. Gastroenterology 116:980–987, 1999
Byrne WJ, Cipel L, Euler AR, Halpin TC, Ament ME: Chronic idiopathic intestinal pseudo-obstruction syndrome in children–clinical characteristics and prognosis. J Pediatr 90:585–589, 1977
Vargas JH, Sachs P, Ament ME: Chronic intestinal pseudoobstruction syndrome in pediatrics: results of a national survey by members of the North American Society of Gastroenterology and Nutrition. J Pediatr Gastroenterol Nutr 7:323–332, 1989
Guze CD, Hyman PE, Payne VJ: Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome. Am J Med Genet 82:114–122, 1999
Goulet O, Jobert-Giraud A, Michel JL, Jaubert JF, Lortat-Jacob S, Colomb V, Guenod-Jabri B, Jan D, Brousse N, Caillard D, Nihoul-Fekete C, Ricour C: Chronic intestinal pseudoobstruction syndrome in pediatric patients. Eur J Pediatr Surg 9:83–89, 1999
Huang-YC, Lee HC, Huang FY, Kao HA, Yeh ML, Chang PY, Shen JC, Shih SL, Chen BF: Neonatal onset chronic intestinal pseudo-obstruction syndrome. Clin Pediatr 34:241–247, 1995
Cucchiara S. Annese V, Minella R, Franco MT, Iervolino C, Emiliano M, Auricchio S: Antroduodenojejunal manometry in the diagnosis of chronic idiopathic intestinal pseudoobstruction in children. J Pediatr Gastroenterol Nutr 18:294–305, 1994
Tomomasa T, Di Lorenzo C, Morikawa A, Uc A, Hyman PE: Analysis of fasting antroduodenal manometry in children. Dig Dis Sci 41:2195–2203, 1996
Malagelada J-R, Camilleri M, Stanghellini V: Manometric Diagnosis of Gastrointestinal Motility Disorders. New York, Theime Medical, Publishers, 1986
Dooley CP, Di Lorenzo MC, Valenzuela JE: Variability of migrating motor complex in humans. Dig Dis Sci 73:723–728, 1992
Hyman PE, Mc Diramid SV, Napolitano JA, Abrams CE, Tomomasa T: Antroduodenal motility in children with chronic intestinal pseudo-obstruction. J Pediatr 112:899–905, 1988
Di Lorenzo C, Flores AF, Reddy SN, Snape WJ Jr, Bazzocchi G, Hyman PE: Colonic manometry in children with chronic intestinal pseudo-obstruction. Gut 34:803–807, 1993
Hyman PE: Chronic intestinal pseudo-obstruction. In PE Hyman, (ed). Pediatric Gastrointestinal Motility Disorders. New York, Academy Professional Information Services, 1994, pp 115–128.
Hyman PE, Bursch B, Beck D, Di Lorenzo C, Zeltzer LK: Discriminating Munchausen's syndrome by proxy from chronic intestinal pseudo-obstruction. J Child Abuse Neglect (in press)
Sigurdsson L, Reyes J, Kocoshis S, Mazariegos G, Abu-Elmagd KM, Bueno J, Di Lorenzo C: Intestinal transplantation in children with chronic intestinal pseudo-obstruction. Gut 45:570–574, 1999
Mousa H, Bueno J, Griffiths J, Kocoshis S, Todo S, Reyes J, Di Lorenzo C: Intestinal motility after small bowel transplantation. Transplant Proc 30:2535–2536, 1998
Cucchiara S, Borrelli O, Salvia G, Iula VD, Fecarotta S, Gaudiello G, Boccia G, Annese V: A normal gastrointestinal motility excludes chronic intestinal pseudoobstruction. Dig Dis Sci 45:258–264, 2000
Fell JM, Smith W, Milla PJ: Infantile chronic intestinal pseudo-obstruction: The role of small intestinal manometry as a diagnostic tool and prognostic indicator. Gut 39:306–311, 1996
Di Lorenzo C, Flores AF, Buie T, Hyman PE: Intestinal motility and jejunal feedings in children with chronic intestinal pseudo-obstruction. Gastroenterology 108:1379–1385, 1995
Hyman PE, Di Lorenzo C, McAdams L, Flores AF, Tomomasa T, Garvey TQ III: Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction. Am J Gastroenterol 88:832–836, 1993
Isozaki K, Hirota S, Miyagawa J, Taniguchi M, Shinomura Y, Matsuzawa Y: Deficiency of c-kit + cells in patients with a myopathic form of chronic idiopathic intestinal pseudoobstruction. Am J Gastroenterol 92:332–334, 1997
Greydanus MP, Camilleri M, Colemont LJ, Phillips SF, Brown ML, Thomforde GM: Ileocolonic transfer of solid chyme in small intestinal neuropathies and myopathies. Gastroenterology 99:158–164, 1990
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mousa, H., Hyman, P.E., Cocjin, J. et al. Long-Term Outcome of Congenital Intestinal Pseudoobstruction. Dig Dis Sci 47, 2298–2305 (2002). https://doi.org/10.1023/A:1020199614102
Issue Date:
DOI: https://doi.org/10.1023/A:1020199614102