Clin Colon Rectal Surg 2018; 31(02): 099-107
DOI: 10.1055/s-0037-1609024
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Chronic Intestinal Pseudo-obstruction

Khalil El-Chammas
1   Division of Pediatric Gastroenterology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
,
Manu R. Sood
2   Division of Pediatric Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, Wisconsin
3   Division of Pediatric Gastroenterology, Children's hospital of Wisconsin, Milwaukee, Wisconsin
› Author Affiliations
Further Information

Publication History

Publication Date:
25 February 2018 (online)

Abstract

Chronic intestinal pseudo-obstruction (CIP) is defined by either continuous or intermittent symptoms of bowel obstruction in the absence of fixed lumen excluding lesion. CIP includes a heterogeneous group of disorders which result either from diseases affecting the enteric neurons and smooth muscle lining or those involving the autonomic innervation of the bowel. Symptoms associated with CIP are nonspecific, which can sometimes contribute to the delay in recognizing the condition and making the correct diagnosis. The diagnostic workup should include imaging and manometry studies and, occasionally, full-thickness bowel biopsies for histopathological examination may be required. Multidisciplinary team approach for the management of these patients is recommended, and the team members should include a gastroenterologist, surgeon, chronic pain specialist, clinical nutritionist, and a psychologist. The treatment goals should include optimizing the nutritional status and preventing or delaying the development of intestinal failure. The majority of the patients require enteral or parenteral nutrition support, and chronic pain is a common and distressing symptom. Small bowel transplantation may be required if patients develop liver complications due to parenteral nutrition, have difficult central line access, or have poor quality of life and worsening pain despite aggressive medical management.

 
  • References

  • 1 Rudolph CD, Hyman PE, Altschuler SM. , et al. Diagnosis and treatment of chronic intestinal pseudo-obstruction in children: report of consensus workshop. J Pediatr Gastroenterol Nutr 1997; 24 (01) 102-112
  • 2 Muto M, Matsufuji H, Tomomasa T. , et al. Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: a report of a nationwide survey in Japan. J Pediatr Surg 2014; 49 (12) 1799-1803
  • 3 Mayer EA, Schuffler MD, Rotter JI, Hanna P, Mogard M. Familial visceral neuropathy with autosomal dominant transmission. Gastroenterology 1986; 91 (06) 1528-1535
  • 4 Roy AD, Bharucha H, Nevin NC, Odling-Smee GW. Idiopathic intestinal pseudo-obstruction: a familial visceral neuropathy. Clin Genet 1980; 18 (04) 291-297
  • 5 Faulk DL, Anuras S, Gardner GD, Mitros FA, Summers RW, Christensen J. A familial visceral myopathy. Ann Intern Med 1978; 89 (5, Pt 1): 600-606
  • 6 Haltia M, Somer H, Palo J, Johnson WG. Neuronal intranuclear inclusion disease in identical twins. Ann Neurol 1984; 15 (04) 316-321
  • 7 Patel H, Norman MG, Perry TL, Berry KE. Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a case and review of the literature. J Neurol Sci 1985; 67 (01) 57-65
  • 8 Schuffler MD, Bird TD, Sumi SM, Cook A. A familial neuronal disease presenting as intestinal pseudoobstruction. Gastroenterology 1978; 75 (05) 889-898
  • 9 Anuras S, Mitros FA, Nowak TV. , et al. A familial visceral myopathy with external ophthalmoplegia and autosomal recessive transmission. Gastroenterology 1983; 84 (02) 346-353
  • 10 Ionasescu V, Thompson SH, Ionasescu R. , et al. Inherited ophthalmoplegia with intestinal pseudo-obstruction. J Neurol Sci 1983; 59 (02) 215-228
  • 11 Schuffler MD, Lowe MC, Bill AH. Studies of idiopathic intestinal pseudoobstruction. I. Hereditary hollow visceral myopathy: clinical and pathological studies. Gastroenterology 1977; 73 (02) 327-338
  • 12 Schuffler MD, Pope II CE. Studies of idiopathic intestinal pseudoobstruction. II. Hereditary hollow visceral myopathy: family studies. Gastroenterology 1977; 73 (02) 339-344
  • 13 Auricchio A, Brancolini V, Casari G. , et al. The locus for a novel syndromic form of neuronal intestinal pseudoobstruction maps to Xq28. Am J Hum Genet 1996; 58 (04) 743-748
  • 14 Thorson W, Diaz-Horta O, Foster II J. , et al. De novo ACTG2 mutations cause congenital distended bladder, microcolon, and intestinal hypoperistalsis. Hum Genet 2014; 133 (06) 737-742
  • 15 Wangler MF, Gonzaga-Jauregui C, Gambin T. , et al; Baylor-Hopkins Center for Mendelian Genomics. Heterozygous de novo and inherited mutations in the smooth muscle actin (ACTG2) gene underlie megacystis-microcolon-intestinal hypoperistalsis syndrome. PLoS Genet 2014; 10 (03) e1004258
  • 16 Chetaille P, Preuss C, Burkhard S. , et al; FORGE Canada Consortium. Mutations in SGOL1 cause a novel cohesinopathy affecting heart and gut rhythm. Nat Genet 2014; 46 (11) 1245-1249
  • 17 Van Goethem G, Schwartz M, Löfgren A, Dermaut B, Van Broeckhoven C, Vissing J. Novel POLG mutations in progressive external ophthalmoplegia mimicking mitochondrial neurogastrointestinal encephalomyopathy. Eur J Hum Genet 2003; 11 (07) 547-549
  • 18 Sonsino E, Mouy R, Foucaud P. , et al. Intestinal pseudoobstruction related to cytomegalovirus infection of myenteric plexus. N Engl J Med 1984; 311 (03) 196-197
  • 19 Besnard M, Faure C, Fromont-Hankard G. , et al. Intestinal pseudo-obstruction and acute pandysautonomia associated with Epstein-Barr virus infection. Am J Gastroenterol 2000; 95 (01) 280-284
  • 20 Selgrad M, De Giorgio R, Fini L. , et al. JC virus infects the enteric glia of patients with chronic idiopathic intestinal pseudo-obstruction. Gut 2009; 58 (01) 25-32
  • 21 Connor FL, Di Lorenzo C. Chronic intestinal pseudo-obstruction: assessment and management. Gastroenterology 2006; 130 (02) (Suppl. 01) S29-S36
  • 22 Knowles CH, De Giorgio R, Kapur RP. , et al. The London Classification of gastrointestinal neuromuscular pathology: report on behalf of the Gastro 2009 International Working Group. Gut 2010; 59 (07) 882-887
  • 23 Kapur RP, Correa H. Architectural malformation of the muscularis propria as a cause for intestinal pseudo-obstruction: two cases and a review of the literature. Pediatr Dev Pathol 2009; 12 (02) 156-164
  • 24 Smith VV, Milla PJ. Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood. Histopathology 1997; 31 (02) 112-122
  • 25 Smith JA, Hauser SC, Madara JL. Hollow visceral myopathy: a light- and electron-microscopic study. Am J Surg Pathol 1982; 6 (03) 269-275
  • 26 Kapur RP. Pathology of intestinal motor disorders in children. Surg Pathol Clin 2010; 3 (03) 711-741
  • 27 Oton E, Moreira V, Redondo C. , et al. Chronic intestinal pseudo-obstruction due to lymphocytic leiomyositis: is there a place for immunomodulatory therapy?. Gut 2005; 54 (09) 1343-1344
  • 28 O'Donnell AM, Puri P. Skip segment Hirschsprung's disease: a systematic review. Pediatr Surg Int 2010; 26 (11) 1065-1069
  • 29 Dingemann J, Puri P. Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int 2010; 26 (11) 1111-1115
  • 30 Nezelof C, Guy-Grand D, Thomine E. Megacolon with hyperplasia of the myenteric plexua. An anatomo-clinical entity, apropos of 3 cases [in French]. Presse Med 1970; 78 (34) 1501-1506
  • 31 Meier-Ruge W. Casuistic of colon disorder with symptoms of Hirschsprung's disease [author's transl]. Verh Dtsch Ges Pathol 1971; 55: 506-510
  • 32 Negreanu LM, Assor P, Mateescu B, Cirstoiu C. Interstitial cells of Cajal in the gut--a gastroenterologist's point of view. World J Gastroenterol 2008; 14 (41) 6285-6288
  • 33 Bruhin-Feichter S, Meier-Ruge W, Martucciello G, Bruder E. Connective tissue in gut development: a key player in motility and in intestinal desmosis. Eur J Pediatr Surg 2012; 22 (06) 445-459
  • 34 Gyger G, Baron M. Systemic sclerosis: gastrointestinal disease and its management. Rheum Dis Clin North Am 2015; 41 (03) 459-473
  • 35 Heneyke S, Smith VV, Spitz L, Milla PJ. Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients. Arch Dis Child 1999; 81 (01) 21-27
  • 36 Mousa H, Hyman PE, Cocjin J, Flores AF, Di Lorenzo C. Long-term outcome of congenital intestinal pseudoobstruction. Dig Dis Sci 2002; 47 (10) 2298-2305
  • 37 Cucchiara S, Borrelli O. Nutritional challenge in pseudo-obstruction: the bridge between motility and nutrition. J Pediatr Gastroenterol Nutr 2009; 48 (Suppl. 02) S83-S85
  • 38 Fell JM, Smith VV, Milla PJ. Infantile chronic idiopathic intestinal pseudo-obstruction: the role of small intestinal manometry as a diagnostic tool and prognostic indicator. Gut 1996; 39 (02) 306-311
  • 39 Goulet O, Jobert-Giraud A, Michel JL. , et al. Chronic intestinal pseudo-obstruction syndrome in pediatric patients. Eur J Pediatr Surg 1999; 9 (02) 83-89
  • 40 Di Lorenzo C, Lucanto C, Flores AF, Idries S, Hyman PE. Effect of sequential erythromycin and octreotide on antroduodenal manometry. J Pediatr Gastroenterol Nutr 1999; 29 (03) 293-296
  • 41 Uc A, Hoon A, Di Lorenzo C, Hyman PE. Antroduodenal manometry in children with no upper gastrointestinal symptoms. Scand J Gastroenterol 1997; 32 (07) 681-685
  • 42 Mazziotti MV, Langer JC. Laparoscopic full-thickness intestinal biopsies in children. J Pediatr Gastroenterol Nutr 2001; 33 (01) 54-57
  • 43 Goulet O, Jobert-Giraud A, Michel JL. , et al. Chronic intestinal pseudo-obstruction syndrome in pediatric patients. Eur J Pediatr Surg 1999; 9 (02) 83-89
  • 44 Di Lorenzo C, Flores AF, Tomomasa T, Hyman PE. Effect of erythromycin on antroduodenal motility in children with chronic functional gastrointestinal symptoms. Dig Dis Sci 1994; 39 (07) 1399-1404
  • 45 Di Lorenzo C, Lucanto C, Flores AF, Idries S, Hyman PE. Effect of octreotide on gastrointestinal motility in children with functional gastrointestinal symptoms. J Pediatr Gastroenterol Nutr 1998; 27 (05) 508-512
  • 46 Gomez R, Fernandez S, Aspirot A. , et al. Effect of amoxicillin/clavulanate on gastrointestinal motility in children. J Pediatr Gastroenterol Nutr 2012; 54 (06) 780-784
  • 47 Di Lorenzo C, Reddy SN, Villanueva-Meyer J, Mena I, Martin S, Hyman PE. Cisapride in children with chronic intestinal pseudoobstruction. An acute, double-blind, crossover, placebo-controlled trial. Gastroenterology 1991; 101 (06) 1564-1570
  • 48 Raphael BP, Nurko S, Jiang H. , et al. Cisapride improves enteral tolerance in pediatric short-bowel syndrome with dysmotility. J Pediatr Gastroenterol Nutr 2011; 52 (05) 590-594
  • 49 Rosner H, Rubin L, Kestenbaum A. Gabapentin adjunctive therapy in neuropathic pain states. Clin J Pain 1996; 12 (01) 56-58
  • 50 Youssef NN, Barksdale Jr E, Griffiths JM, Flores AF, Di Lorenzo C. Management of intractable constipation with antegrade enemas in neurologically intact children. J Pediatr Gastroenterol Nutr 2002; 34 (04) 402-405
  • 51 Altaf MA, Werlin SL, Sato TT, Rudolph CD, Sood MR. Colonic volvulus in children with intestinal motility disorders. J Pediatr Gastroenterol Nutr 2009; 49 (01) 59-62
  • 52 Halter JP, Michael W, Schüpbach M. , et al. Allogeneic haematopoietic stem cell transplantation for mitochondrial neurogastrointestinal encephalomyopathy. Brain 2015; 138 (Pt 10): 2847-2858
  • 53 Cucchiara S, Borrelli O, Salvia G. , et al. A normal gastrointestinal motility excludes chronic intestinal pseudoobstruction in children. Dig Dis Sci 2000; 45 (02) 258-264
  • 54 Bond GJ, Reyes JD. Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction. Semin Pediatr Surg 2004; 13 (04) 286-292
  • 55 Lao OB, Healey PJ, Perkins JD, Horslen S, Reyes JD, Goldin AB. Outcomes in children after intestinal transplant. Pediatrics 2010; 125 (03) e550-e558
  • 56 Schwankovsky L, Mousa H, Rowhani A, DI Lorenzo C, Hyman PE. Quality of life outcomes in congenital chronic intestinal pseudo-obstruction. Dig Dis Sci 2002; 47 (09) 1965-1968