Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients
S Heneyke, V V Smith, L Spitz, P J Milla
Institute of Child
Health and Great Ormond Street Hospital for Children NHS Trust, London,
UK
Correspondence to: Dr P J Milla, Gastroenterology Unit, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. email: P.Milla{at}ich.ucl.ac.uk
Accepted 22 December
1998
AIMS
To document the
long term course of chronic idiopathic intestinal pseudo-obstruction
syndrome (CIIPS) in children with defined enteric neuromuscular
disease, and the place and type of surgery used in their management; in
addition, to identify prognostic factors.
METHODSChildren with
CIIPS were investigated and treated prospectively.
RESULTSTwenty four
children presented congenitally, eight during the 1st year of life, and
10 later. Twenty two had myopathy and 16 neuropathy (11 familial).
Malrotation was present in 16 patients, 10 had short small intestine,
six had non-hypertrophic pyloric stenosis, and 16 had urinary tract
involvement. Thirty two patients needed long term parenteral nutrition
(TPN): for less than six months in 19 and for more than six months in
13, 10 of whom are TPN dependent; 14 are now enteral feeding.
Prokinetic treatment improved six of 22. Intestinal decompression
stomas were used in 36, colostomy relieved symptoms in five of 11, and
ileostomy in 16 of 31. A poor outcome (death (14) or TPN dependence
(10)) was seen with malrotation (13 of 16), short small bowel (eight of
nine), urinary tract involvement (12 of 16), and myopathic histology
(15 of 22).
CONCLUSIONSIn CIIPS
drugs are not helpful but decompression stomas are. Outcome was poor in
24 of 44 children (15 muscle disorder, 10 nerve disease).
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Key messages
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Keywords: chronic intestinal pseudo-obstruction; decompression stomas; prognostic factors; total parenteral nutrition
© 1999 by Archives of Disease in Childhood
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