Abstract
Background: Distal intestinal obstruction syndrome (DIOS) presents in both children and adults with cystic fibrosis (CF). When DIOS is characterized by bilious vomiting or when washout therapy with polyethylene glycol has failed, radio-opaque hypertonic enema (ROHE) is recommended as the next therapeutic option. Techniques for use of ROHE in infants with meconium ileus are described in the literature but have not been reported for children and adults with DIOS.
Methods: We described the technique, volume, and results of Gastrografin enema usage for DIOS in children and adults with CF at our institution between 2006 and 2011. We reviewed the incidence of DIOS reported to the CF Foundation Patient Registry (CFFPR) between 2001 and 2011
Results: The mean amount of dilute Gastrografin used was 1220ml or 31 ml/kg, (range 240 - 3500ml; 19 – 59 ml/kg). Three patients had incomplete resolution after an initial enema and underwent repeat enemas on consecutive days. The incidence of DIOS in the CFFPR increased from 223 to 498 cases in children <18 years and 380 to 813 cases in adults >18 years over the ten year period studied.
Conclusions: Large volume radio-opaque hypertonic enemas can resolve episodes of acute DIOS and can be repeated on subsequent days if needed, without complications. The incidence of DIOS reported to the United States CF Foundation Patient Registry has increased in both children and adults over the past decade.
References
Gelfond D, Borowitz D. Gastrointestinal complications of cystic fibrosis. Clin Gastroenterol Hepatol 2013; 11: 333-42. http://dx.doi.org/10.1016/j.cgh.2012.11.006
Colombo C, Ellemunter H, Houwen R, et al. on behalf of the ESPGHAN Cystic Fibrosis Working Group. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. JCF 2011; 10(Suppl 2): S24-S28
Houwen RH, van der Doef HP, Sermet I, et al. on behalf of the ESPGHAN Cystic Fibrosis Working Group. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J Pediatr Gastroenterol Nutr 2010; 50: 38-42. http://dx.doi.org/10.1097/MPG.0b013e3181a6e01d
Andersen HO, Hjelt K, Waever E, Overgaard K. The age-related incidence of meconium ileus equivalent in a cystic fibrosis population: the impact of a high energy intake. J Pediatr Gastroenterol Nutr 1990; 11: 355-60.
Shinohara T, Tsuda M, Koyama N. Management of meconium-related ileus in very low-birth weight infants. Pediatrics Int 2007; 49: 641-44. http://dx.doi.org/10.1111/j.1442-200X.2007.02457.x
O’Halloran SM, Gilbert J, McKendrick OM, et al. Gastrografin in acute meconium ileus equivalent. Arch Dis Child 1986; 61: 1128-11?0
Cappell MS, Colonic toxicity of administered drugs and chemicals. Am J Gastroenterol 2004; 99(6): 1175-90. http://dx.doi.org/10.1111/j.1572-0241.2004.30192.x
Maisonneuve P, Marshall BC, Knapp EA, Lowenfels AB. Cancer risk in cystic fibrosis: a 20-year nationwide study from the United States. J Natl Cancer Inst 2013; 105(2): 122-9. http://dx.doi.org/10.1093/jnci/djs481
Borthne AS, Abdelnoor M, Storaas T, Pierre-Jerome C, Kløw NE. Osmolarity: a decisive parameter of bowel agents in intestinal magnetic resonance imaging. Eur Radiol 2006; 16(6): 1331-6. http://dx.doi.org/10.1007/s00330-005-0063-7
Cottart CH, Bonvin E, Rey C, Wendum D, Bernaudin JF, Dukont S, et al. Impact of nutrition of phenotype in Cftr-deficient mice. Pediatr Res 2007; 65(5): 528-32. http://dx.doi.org/10.1203/PDR.0b013e318155a61d
Subhi R, Ooi R, Kotsimbos T, Wilson J, Lee WR, Wale R, Warrier S. Distal intestinal obstruction syndrome in cystic fibrosis: presentation, outcome and management in a tertiary hospital (2007-2012). ANZ J Surg 2013. [Epub ahead of print
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright (c) 2014 Mahmoud Zahra, Carla Frederick, Richard Thomas, Vaseem Iqbal , Drucy Borowitz