Skip to main content
Top
Published in: Journal of Gastrointestinal Surgery 8/2009

01-08-2009 | Original Article

Distal Intestinal Obstruction Syndrome (DIOS) in Patients with Cystic Fibrosis After Lung Transplantation

Authors: Jonathan R. Morton, Nabila Ansari, Allan R. Glanville, Alan P. Meagher, Reginald V. N. Lord

Published in: Journal of Gastrointestinal Surgery | Issue 8/2009

Login to get access

Abstract

Background

Cystic fibrosis (CF) is the commonest inherited life-threatening disease in Caucasians. With increased longevity, more patients with CF are developing gastrointestinal complications including the distal intestinal obstruction syndrome (DIOS), in which ileocecal obstruction is caused by viscid mucofeculent material. The optimal management of DIOS is uncertain.

Methods

The medical records of all patients with CF who underwent lung transplantation at this institution during a 15-year period were reviewed. The definition of DIOS required the presence of both clinical and radiological features of ileocecal obstruction.

Results

One hundred twenty-one patients with CF underwent lung transplantation during the study period. During a minimum 2-year follow-up, there were 17 episodes of DIOS in 13 (10.7%) patients. The development of DIOS was significantly associated with a past history of meconium ileus (odds ratio 20.7, 95% C.I. 5.09–83.9) or previous laparotomy (odds ratio 4.93, 95% C.I. 1.47–16.6). All six patients who developed DIOS during the transplantation admission had meconium ileus during infancy, and five had undergone pretransplant laparotomy for CF complications. First-line treatment for all patients was a combination of medication (laxatives, stool softeners, and bowel preparation formulas). This was successful in 14 of the 17 DIOS but needed to be given for up to 14 days. The other three patients required laparotomy with enterotomy and fecal disimpaction. This provided definitive resolution of DIOS except in one patient who presented late and died despite ileal decompression and ileostomy.

Conclusions

DIOS occurred in approximately 10% of CF patients after lung transplantation. Patients with a history of meconium ileus or previous laparotomy are at high risk of developing DIOS. Patients with DIOS require early aggressive management with timely laparotomy with enterotomy and possible stoma formation when non-operative therapy is unsuccessful.
Literature
1.
go back to reference Foundation CF. Cystic Fibrosis Foundation: Patient Registry 2006 Annual Report Bethesda, Maryland. 2006. Foundation CF. Cystic Fibrosis Foundation: Patient Registry 2006 Annual Report Bethesda, Maryland. 2006.
5.
go back to reference Rosenstein BJ, Langbaum TS. Incidence of distal intestinal obstruction syndrome in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1983;2(2):299–301.PubMed Rosenstein BJ, Langbaum TS. Incidence of distal intestinal obstruction syndrome in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1983;2(2):299–301.PubMed
7.
go back to reference Koletzko S, Stringer DA, Cleghorn GJ, Durie PR. Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis. Pediatrics. 1989;83(5):727–33.PubMed Koletzko S, Stringer DA, Cleghorn GJ, Durie PR. Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis. Pediatrics. 1989;83(5):727–33.PubMed
11.
go back to reference Spratt P, Glanville AR, MacDonald P, et al. Heart/lung transplantation in Australia: early results of the St Vincent's program. Transplant Proc. 1990;22(5):2141–2.PubMed Spratt P, Glanville AR, MacDonald P, et al. Heart/lung transplantation in Australia: early results of the St Vincent's program. Transplant Proc. 1990;22(5):2141–2.PubMed
12.
go back to reference Davidson AC, Harrison K, Steinfort CL, Geddes DM. Distal intestinal obstruction syndrome in cystic fibrosis treated by oral intestinal lavage, and a case of recurrent obstruction despite normal pancreatic function. Thorax. 1987;42(7):538–41. doi:10.1136/thx.42.7.538.CrossRefPubMed Davidson AC, Harrison K, Steinfort CL, Geddes DM. Distal intestinal obstruction syndrome in cystic fibrosis treated by oral intestinal lavage, and a case of recurrent obstruction despite normal pancreatic function. Thorax. 1987;42(7):538–41. doi:10.​1136/​thx.​42.​7.​538.CrossRefPubMed
20.
go back to reference Kerem E, Corey M, Kerem BS, et al. The relation between genotype and phenotype in cystic fibrosis—analysis of the most common mutation (delta F508). N Engl J Med. 1990;323(22):1517–22.PubMedCrossRef Kerem E, Corey M, Kerem BS, et al. The relation between genotype and phenotype in cystic fibrosis—analysis of the most common mutation (delta F508). N Engl J Med. 1990;323(22):1517–22.PubMedCrossRef
22.
go back to reference Weller PH, Williams J. Clinical features, pathogenesis and management of meconium ileus equivalent. J R Soc Med. 1986;79(Suppl 12):36–7.PubMed Weller PH, Williams J. Clinical features, pathogenesis and management of meconium ileus equivalent. J R Soc Med. 1986;79(Suppl 12):36–7.PubMed
23.
go back to reference Khoshoo V, Udall JN Jr. Meconium ileus equivalent in children and adults. Am J Gastroenterol. 1994;89(2):153–7.PubMed Khoshoo V, Udall JN Jr. Meconium ileus equivalent in children and adults. Am J Gastroenterol. 1994;89(2):153–7.PubMed
25.
go back to reference Lord RVN, Sillin L. Motility disorders of the small bowel. In Bland KI BM, Csendes A, Garden OJ, Sarr MG, Wong J, ed. General Surgery, Principles and International Practice, Vol. Ch. 58: Springer 2009. pp. 2011. Lord RVN, Sillin L. Motility disorders of the small bowel. In Bland KI BM, Csendes A, Garden OJ, Sarr MG, Wong J, ed. General Surgery, Principles and International Practice, Vol. Ch. 58: Springer 2009. pp. 2011.
29.
go back to reference Littlewood JM. Cystic fibrosis: gastrointestinal complications. Br Med Bull. 1992;48(4):847–59.PubMed Littlewood JM. Cystic fibrosis: gastrointestinal complications. Br Med Bull. 1992;48(4):847–59.PubMed
30.
go back to reference Langer JC, Paes BM, Gray S. Hypernatremia associated with N-acetylcysteine therapy for meconium ileus in a premature infant. CMAJ. 1990;143(3):202–3.PubMed Langer JC, Paes BM, Gray S. Hypernatremia associated with N-acetylcysteine therapy for meconium ileus in a premature infant. CMAJ. 1990;143(3):202–3.PubMed
32.
go back to reference Hiatt RB, Wilson PE. Celiac syndrome: therapy of meconium ileus: report of eight cases with a review of the literature. Surg Gynecol Obstet. 1948;87:317.PubMed Hiatt RB, Wilson PE. Celiac syndrome: therapy of meconium ileus: report of eight cases with a review of the literature. Surg Gynecol Obstet. 1948;87:317.PubMed
Metadata
Title
Distal Intestinal Obstruction Syndrome (DIOS) in Patients with Cystic Fibrosis After Lung Transplantation
Authors
Jonathan R. Morton
Nabila Ansari
Allan R. Glanville
Alan P. Meagher
Reginald V. N. Lord
Publication date
01-08-2009
Publisher
Springer-Verlag
Published in
Journal of Gastrointestinal Surgery / Issue 8/2009
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI
https://doi.org/10.1007/s11605-009-0924-5

Other articles of this Issue 8/2009

Journal of Gastrointestinal Surgery 8/2009 Go to the issue