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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
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Publication History

Publication Date:
25 February 2018 (online)

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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.

Keywords

chronic intestinal pseudo-obstruction - bowel motility - antroduodenal manometry
 

  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 3 Mayer EA, Schuffler MD, Rotter JI, Hanna P, Mogard M. Familial visceral neuropathy with autosomal dominant transmission. Gastroenterology 1986; 91 (06) 1528-1535
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 6 Haltia M, Somer H, Palo J, Johnson WG. Neuronal intranuclear inclusion disease in identical twins. Ann Neurol 1984; 15 (04) 316-321
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 8 Schuffler MD, Bird TD, Sumi SM, Cook A. A familial neuronal disease presenting as intestinal pseudoobstruction. Gastroenterology 1978; 75 (05) 889-898
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 10 Ionasescu V, Thompson SH, Ionasescu R. , et al. Inherited ophthalmoplegia with intestinal pseudo-obstruction. J Neurol Sci 1983; 59 (02) 215-228
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 12 Schuffler MD, Pope II CE. Studies of idiopathic intestinal pseudoobstruction. II. Hereditary hollow visceral myopathy: family studies. Gastroenterology 1977; 73 (02) 339-344
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 21 Connor FL, Di Lorenzo C. Chronic intestinal pseudo-obstruction: assessment and management. Gastroenterology 2006; 130 (02) (Suppl. 01) S29-S36
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 24 Smith VV, Milla PJ. Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood. Histopathology 1997; 31 (02) 112-122
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 26 Kapur RP. Pathology of intestinal motor disorders in children. Surg Pathol Clin 2010; 3 (03) 711-741
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 28 O'Donnell AM, Puri P. Skip segment Hirschsprung's disease: a systematic review. Pediatr Surg Int 2010; 26 (11) 1065-1069
    CrossrefPubMedSearch in Google Scholar
  • 29 Dingemann J, Puri P. Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int 2010; 26 (11) 1111-1115
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    Thieme ConnectPubMedSearch in Google Scholar
  • 34 Gyger G, Baron M. Systemic sclerosis: gastrointestinal disease and its management. Rheum Dis Clin North Am 2015; 41 (03) 459-473
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    Thieme ConnectPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 42 Mazziotti MV, Langer JC. Laparoscopic full-thickness intestinal biopsies in children. J Pediatr Gastroenterol Nutr 2001; 33 (01) 54-57
    CrossrefPubMedSearch in Google Scholar
  • 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
    Thieme ConnectPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 49 Rosner H, Rubin L, Kestenbaum A. Gabapentin adjunctive therapy in neuropathic pain states. Clin J Pain 1996; 12 (01) 56-58
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 54 Bond GJ, Reyes JD. Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction. Semin Pediatr Surg 2004; 13 (04) 286-292
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar