Cystic fibrosis is not rare autosomal recessive transmitted disease of Caucasian population and it belongs still under untreatable disease. It was running deadly already in childhood in last century, but life expectance lies these days by 50th year of age. Distal intestinal obstruction syndrome is a rare complication of patient suffering from cystic fibrosis. Usually ileum and right colon are obstructed by putty like material. Cause is multifactorial. The absence of pancreatic enzymes alone has been disproved. But in combination with malfunction of bowel mucous excretion and broken gut motility can cause real gut obstruction. Incidence of mucoviscidosis decreases in Europe from 1 to 2500 live giving birth in the last century to 1 to 3000 - 6000 today. 1,8 % of these patients will develop intestinal obstruction. Therapy aim is as soon as possible to start with treatment. On beginning is indicated conservative therapy and surgical intervention to undertake only if unavoidable. Because of growing of affected population is acute therapy not only for centers intended. Each surgeon should be aware of this condition and prime diagnose is important for right choice of therapy.
Three adult cases are reported, two from specialized centre and one from general surgical unit. One case was treated by surgical intervention and two cases were healed through high osmotic pressure of laxatives.
1. Bell S.C., Mall M.A., Gutierrez H., ; M., Madge M., Davies J.C., Burgel P.R., Tullis E., Castanos C., Castellani C., et al. The Future of Cystic Fibrosis Care: A Global Perspective. Lancet Respir. Med. 2020;8:65–124. doi: 10.1016/S2213-2600(19)30337-6. [ PMC free article ] [ PubMed ] [ CrossRef ] [ Google Scholar ]
2. Bishop, W. Distal intestinal obstruction syndrome. Pediatric pulmonology. 2002. 24, p. 112 – 113.
3. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry. 2021 Annual Data Report. Patient Registry | Cystic Fibrosis Foundation (cff.org)
4. Chaun, H. Colonic disorders in adult cystic fibrosis. Canadian Journal of Gastroenterology. 2001. 15, p. 586 – 90.
5. Černý, J. a kol.: Chirurgia tráviacej rúry. Bratislava. Osveta 1988. 499, s 256 – 257.
6. De Boeck K. Cystic fibrosis in the year 2020: A disease with a new face. Acta Paediatr. 2020 doi: 10.1111/apa.15155. in press. [ PubMed ] [ CrossRef ] [ Google Scholar ]
7. European (ECFS) Cystic Fibrosis Registry Annual Data Report 2021. [(accessed on 20 April 2020)]; Available online: https://www.ecfs.eu/sites/default/files/general-content-images/working-groups/ecfs-patient-registry/ECFSPR_Report2021_v1.3.pdf [ Ref list ].
8. Farrell P.M., Rock M.J., Baker M.W. The Impact of the CFTR Gene Discovery on Cystic Fibrosis Diagnosis, Counseling, and Preventive Therapy. Genes. 2020;11:401. doi: 10.3390/genes11040401. [ PMC free article ] [ PubMed ] [ CrossRef ] [ Google Scholar ] [ Ref list ]
9. Green J, Carroll W, Gilchrist FJ. Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis. Cochrane Database Syst Rev. 2018 Aug 3;8(8):CD012798. doi: 10.1002/14651858.CD012798.pub2. Update in: Cochrane Database Syst Rev. 2021 Dec 22;12:CD012798. PMID: 30075058; PMCID: PMC6513219.
10. Harutyunyan M, Huang Y, Mun KS, Yang F, Arora K, Naren AP. Personalized medicine in CF: from modulator development to therapy for cystic fibrosis patients with rare CFTR mutations. Am J Physiol Lung Cell Mol Physiol. 2018 Apr 1;314(4): L529-L543. doi: 10.1152/ajplung.00465.2017. Epub 2017 Dec 14. PMID: 29351449; PMCID: PMC5966781.
11. Holmes, M., Murphy, V. et al.: Intusception in cistic fibrosis. Archives of Diseases in Childhood. 1991. 66, p. 726 – 7.
12. Jeffrey, I., Durrans, D. et al.: The pathology of meconium ileus equivalent. Journal of Clinical Pathology. 1983. 36, p. 356 – 60.
13. Koletzko, S., Corey, M. et al.: Effects of cisapride in patients with cystic fibrosis and distal intestinal obstruction syndrome. Journal of pediatrics. 1990. 117, p. 812 – 22.
14. Lillibridge, C., Docter, J. et al.: Oral administration of n-acetyl cystein in the prophylaxis of meconium ileus equivalent. Journal of Pediatrics. 1967. 71, p. 887 – 9. 15. Minkes, K., Langer, J. et al.: Intestinal obstruction after lung transplantation in children with CF. Journal of Pediatric Surgery. 1999. 34, p. 1489 – 90.
16. Pashankar, D., Bishop, W. et al.: Efficacy and optimal dose of daily polyethylene glycol 3350 for treatment of constipation and encopresis in children. Journal of Pediatrics. 2001. 139, p. 428 – 432.
17. Scotet V., Gutierrez H., Farrell P.M. Newborn screening for CF across the globe —Where is it worthwhile? Int. J. Neonatal Screen. 2020;6:18. doi: 10.3390/ijns6010018. [ CrossRef ] [ Google Scholar ]
18. Scotet V, L'Hostis C, Férec C. The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery. Genes (Basel). 2020 May 26;11(6):589. doi: 10.3390/genes11060589. PMID: 32466381; PMCID: PMC7348877.
19. Vávrová, V. a kol.: Cystická fibróza v praxi. Praha. Kreace 1999. 150.
20. Vilanova-Sanchez A, Gasior AC, Toocheck N, Weaver L, Wood RJ, Reck CA, Wagner A, Hoover E, Gagnon R, Jaggers J, Maloof T, Nash O, Williams C, Levitt MA. Are Senna based laxatives safe when used as long term treatment for constipation in children? J Pediatr Surg. 2018 Apr;53(4):722-727. doi: 10.1016/j.jpedsurg.2018.01.002. Epub 2018 Jan 31. PMID: 29429768.