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Chronic intestinal pseudo-obstruction

Author
Michael Camilleri, MD
Section Editor
Lawrence S Friedman, MD
Deputy Editor
Shilpa Grover, MD, MPH

INTRODUCTION

Pseudo-obstruction is a syndrome characterized by signs and symptoms of a mechanical obstruction of the small or large bowel in the absence of an anatomic lesion that obstructs the flow of intestinal contents. Pseudo-obstruction may be acute or chronic and is characterized by the presence of dilation of the bowel on imaging. When there is evidence of chronic small intestinal motility disorder in the absence of bowel dilatation, the preferred term is chronic intestinal dysmotility.

This topic review will discuss the etiology, clinical manifestations, diagnosis, and treatment of chronic intestinal pseudo-obstruction. Acute pseudo-obstruction, chronic intestinal dysmotility, and slow transit constipation/colon inertia are discussed separately. (See "Acute colonic pseudo-obstruction (Ogilvie's syndrome)" and "Etiology and evaluation of chronic constipation in adults".)

ETIOLOGY

Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder that may be due to an underlying neuropathic disorder (involving the enteric nervous system or extrinsic nervous system), a myopathic disorder (involving the smooth muscle), or abnormality in the interstitial cell of Cajal (ICC) [1]. Therefore, there are parallels in the pathobiological mechanisms of CIPO and gastroparesis.

Neuropathic, myopathic, or ICC abnormalities may be idiopathic or secondary to another disease. Approximately half of the cases of CIPO are secondary to neurologic, paraneoplastic, autoimmune, metabolic/endocrine, and infectious diseases.

More than one of the elements of the neuromuscular apparatus of the gut may be affected in certain diseases. For example, there is an intrinsic neuropathic phase of scleroderma, before the smooth muscle involvement results in myopathy. Similarly, mitochondrial cytopathy results in neuropathy and eventually myopathy. Moreover, diabetes affects extrinsic nerves through autonomic neuropathy, and the ICCs and amyloidosis causes an extrinsic neuropathy followed by myopathic CIPO.

                                         

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Literature review current through: Nov 2016. | This topic last updated: Mon Jul 18 00:00:00 GMT+00:00 2016.
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References
Top
  1. De Giorgio R, Sarnelli G, Corinaldesi R, Stanghellini V. Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction. Gut 2004; 53:1549.
  2. Hall KE, Wiley JW. Neural injury, repair and adaptation in the GI tract. I. New insights into neuronal injury: a cautionary tale. Am J Physiol 1998; 274:G978.
  3. Sodhi N, Camilleri M, Camoriano JK, et al. Autonomic function and motility in intestinal pseudoobstruction caused by paraneoplastic syndrome. Dig Dis Sci 1989; 34:1937.
  4. Kidher ES, Briceno N, Taghi A, Chukwuemeka A. An interesting collection of paraneoplastic syndromes in a patient with a malignant thymoma. BMJ Case Rep 2012; 2012.
  5. Darnell RB, DeAngelis LM. Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies. Lancet 1993; 341:21.
  6. Lennon VA, Sas DF, Busk MF, et al. Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma. Gastroenterology 1991; 100:137.
  7. De Giorgio R, Stanghellini V, Barbara G, et al. Primary enteric neuropathies underlying gastrointestinal motor dysfunction. Scand J Gastroenterol 2000; 35:114.
  8. Ruuska TH, Karikoski R, Smith VV, Milla PJ. Acquired myopathic intestinal pseudo-obstruction may be due to autoimmune enteric leiomyositis. Gastroenterology 2002; 122:1133.
  9. Khairullah S, Jasmin R, Yahya F, et al. Chronic intestinal pseudo-obstruction: a rare first manifestation of systemic lupus erythematosus. Lupus 2013; 22:957.
  10. Jäkel J, Heise JW, Gassler N, Dietrich CG. Raising awareness about chronic intestinal pseudo-obstruction (CIPO): a case report showing CIPO as initial manifestation of atypical seronegative systemic sclerosis. Z Gastroenterol 2012; 50:1100.
  11. Ohlsson B, Veress B, Janciauskiene S, et al. Chronic intestinal pseudo-obstruction due to buserelin-induced formation of anti-GnRH antibodies. Gastroenterology 2007; 132:45.
  12. Törnblom H, Lang B, Clover L, et al. Autoantibodies in patients with gut motility disorders and enteric neuropathy. Scand J Gastroenterol 2007; 42:1289.
  13. 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; 58:25.
  14. Mariani A, Camilleri M, Petersen IA, et al. Audit of suspected chronic intestinal pseudo-obstruction in patients with gynecologic cancer. Eur J Gynaecol Oncol 2008; 29:578.
  15. Stanghellini V, Corinaldesi R, Barbara L. Pseudo-obstruction syndromes. Baillieres Clin Gastroenterol 1988; 2:225.
  16. Sekino Y, Inamori M, Yamada E, et al. Characteristics of intestinal pseudo-obstruction in patients with mitochondrial diseases. World J Gastroenterol 2012; 18:4557.
  17. Coulie B, Camilleri M. Intestinal pseudo-obstruction. Annu Rev Med 1999; 50:37.
  18. Pingault V, Bondurand N, Kuhlbrodt K, et al. SOX10 mutations in patients with Waardenburg-Hirschsprung disease. Nat Genet 1998; 18:171.
  19. Di Nardo G, Blandizzi C, Volta U, et al. Review article: molecular, pathological and therapeutic features of human enteric neuropathies. Aliment Pharmacol Ther 2008; 28:25.
  20. Deglincerti A, De Giorgio R, Cefle K, et al. A novel locus for syndromic chronic idiopathic intestinal pseudo-obstruction maps to chromosome 8q23-q24. Eur J Hum Genet 2007; 15:889.
  21. O'Donnell AM, Puri P. A role for Pten in paediatric intestinal dysmotility disorders. Pediatr Surg Int 2011; 27:491.
  22. Reinstein E, Frentz S, Morgan T, et al. Vascular and connective tissue anomalies associated with X-linked periventricular heterotopia due to mutations in Filamin A. Eur J Hum Genet 2013; 21:494.
  23. Wangler MF, Beaudet AL. ACTG2-Related Disorders. In: GeneReviews, Pagon RA, Adam MP, Ardinger HH, et al. (Eds), University of Washington, Seattle 2015.
  24. Bonora E, Bianco F, Cordeddu L, et al. Mutations in RAD21 disrupt regulation of APOB in patients with chronic intestinal pseudo-obstruction. Gastroenterology 2015; 148:771.
  25. Notarnicola C, Rouleau C, Le Guen L, et al. The RNA-binding protein RBPMS2 regulates development of gastrointestinal smooth muscle. Gastroenterology 2012; 143:687.
  26. Pelizzo G, Villanacci V, Salemme M, et al. Intestinal pseudo-obstruction due to small bowel α-actin deficiency in a child with Ehlers-Danlos syndrome. Tech Coloproctol 2013; 17:673.
  27. Holla OL, Bock G, Busk OL, Isfoss BL. Familial visceral myopathy diagnosed by exome sequencing of a patient with chronic intestinal pseudo-obstruction. Endoscopy 2014; 46:533.
  28. Mallick S, Prasenjit D, Prateek K, et al. Chronic intestinal pseudo-obstruction: systematic histopathological approach can clinch vital clues. Virchows Arch 2014; 464:529.
  29. Knowles CH, Silk DB, Darzi A, et al. Deranged smooth muscle alpha-actin as a biomarker of intestinal pseudo-obstruction: a controlled multinational case series. Gut 2004; 53:1583.
  30. Iida H, Ohkubo H, Inamori M, et al. Epidemiology and clinical experience of chronic intestinal pseudo-obstruction in Japan: a nationwide epidemiologic survey. J Epidemiol 2013; 23:288.
  31. Stanghellini V, Camilleri M, Malagelada JR. Chronic idiopathic intestinal pseudo-obstruction: clinical and intestinal manometric findings. Gut 1987; 28:5.
  32. Stanghellini V, Cogliandro RF, De Giorgio R, et al. Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study. Clin Gastroenterol Hepatol 2005; 3:449.
  33. Tatterton M, El-Khatib C. Caecal volvulus in a patient with chronic intestinal pseudo-obstruction. Ann R Coll Surg Engl 2011; 93:e131.
  34. de Betue CT, Boersma D, Oomen MW, et al. Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome. Eur J Pediatr 2011; 170:1591.
  35. Camilleri M. Disorders of gastrointestinal motility in neurologic diseases. Mayo Clin Proc 1990; 65:825.
  36. Hubball AW, Lang B, Souza MA, et al. Voltage-gated potassium channel (K(v) 1) autoantibodies in patients with chagasic gut dysmotility and distribution of K(v) 1 channels in human enteric neuromusculature (autoantibodies in GI dysmotility). Neurogastroenterol Motil 2012; 24:719.
  37. Ohkubo H, Kessoku T, Fuyuki A, et al. Assessment of small bowel motility in patients with chronic intestinal pseudo-obstruction using cine-MRI. Am J Gastroenterol 2013; 108:1130.
  38. Camilleri M, Zinsmeister AR, Greydanus MP, et al. Towards a less costly but accurate test of gastric emptying and small bowel transit. Dig Dis Sci 1991; 36:609.
  39. Maurer AH, Krevsky B. Whole-gut transit scintigraphy in the evaluation of small-bowel and colon transit disorders. Semin Nucl Med 1995; 25:326.
  40. Mayer EA, Elashoff J, Hawkins R, et al. Gastric emptying of mixed solid-liquid meal in patients with intestinal pseudoobstruction. Dig Dis Sci 1988; 33:10.
  41. Camilleri M, Brown ML, Malagelada JR. Relationship between impaired gastric emptying and abnormal gastrointestinal motility. Gastroenterology 1986; 91:94.
  42. von der Ohe MR, Camilleri M. Measurement of small bowel and colonic transit: indications and methods. Mayo Clin Proc 1992; 67:1169.
  43. Camilleri M, Zinsmeister AR. Towards a relatively inexpensive, noninvasive, accurate test for colonic motility disorders. Gastroenterology 1992; 103:36.
  44. Kuo B, Maneerattanaporn M, Lee AA, et al. Generalized transit delay on wireless motility capsule testing in patients with clinical suspicion of gastroparesis, small intestinal dysmotility, or slow transit constipation. Dig Dis Sci 2011; 56:2928.
  45. Malagelada C, De Iorio F, Azpiroz F, et al. New insight into intestinal motor function via noninvasive endoluminal image analysis. Gastroenterology 2008; 135:1155.
  46. de Giorgio R, Volta U, Stanghellini V, et al. Neurogenic chronic intestinal pseudo-obstruction: antineuronal antibody-mediated activation of autophagy via Fas. Gastroenterology 2008; 135:601.
  47. Camilleri M. Study of human gastroduodenojejunal motility. Applied physiology in clinical practice. Dig Dis Sci 1993; 38:785.
  48. Weston S, Thumshirn M, Wiste J, Camilleri M. Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol 1998; 93:1085.
  49. Camilleri M. Jejunal manometry in distal subacute mechanical obstruction: significance of prolonged simultaneous contractions. Gut 1989; 30:468.
  50. Amiot A, Joly F, Cazals-Hatem D, et al. Prognostic yield of esophageal manometry in chronic intestinal pseudo-obstruction: a retrospective cohort of 116 adult patients. Neurogastroenterol Motil 2012; 24:1008.
  51. Vassallo M, Camilleri M, Caron BL, Low PA. Gastrointestinal motor dysfunction in acquired selective cholinergic dysautonomia associated with infectious mononucleosis. Gastroenterology 1991; 100:252.
  52. De Giorgio R, Camilleri M. Human enteric neuropathies: morphology and molecular pathology. Neurogastroenterol Motil 2004; 16:515.
  53. Park SH, Min H, Chi JG, et al. Immunohistochemical studies of pediatric intestinal pseudo-obstruction: bcl2, a valuable biomarker to detect immature enteric ganglion cells. Am J Surg Pathol 2005; 29:1017.
  54. Lindberg G, Törnblom H, Iwarzon M, et al. Full-thickness biopsy findings in chronic intestinal pseudo-obstruction and enteric dysmotility. Gut 2009; 58:1084.
  55. Ooms AH, Verheij J, Hulst JM, et al. Eosinophilic myenteric ganglionitis as a cause of chronic intestinal pseudo-obstruction. Virchows Arch 2012; 460:123.
  56. van den Berg MM, Di Lorenzo C, Mousa HM, et al. Morphological changes of the enteric nervous system, interstitial cells of cajal, and smooth muscle in children with colonic motility disorders. J Pediatr Gastroenterol Nutr 2009; 48:22.
  57. De Giorgio R, Guerrini S, Barbara G, et al. Inflammatory neuropathies of the enteric nervous system. Gastroenterology 2004; 126:1872.
  58. Uchida K, Otake K, Inoue M, et al. Chronic intestinal pseudo-obstruction due to lymphocytic intestinal leiomyositis: Case report and literature review. Intractable Rare Dis Res 2012; 1:35.
  59. Goulet O, Sauvat F, Jan D. Surgery for pediatric patients with chronic intestinal pseudo-obstruction syndrome. J Pediatr Gastroenterol Nutr 2005; 41 Suppl 1:S66.
  60. Abell TL, Camilleri M, DiMagno EP, et al. Long-term efficacy of oral cisapride in symptomatic upper gut dysmotility. Dig Dis Sci 1991; 36:616.
  61. Catnach SM, Fairclough PD. Erythromycin and the gut. Gut 1992; 33:397.
  62. Emmanuel AV, Shand AG, Kamm MA. Erythromycin for the treatment of chronic intestinal pseudo-obstruction: description of six cases with a positive response. Aliment Pharmacol Ther 2004; 19:687.
  63. Chami TN, Schuster MM, Crowell MD, et al. Effects of low dose erythromycin on gastrointestinal motility and symptoms in chronic intestinal pseudoobstruction (abstract). Gastroenterology 1991; 100:41.
  64. Fiorucci S, Distrutti E, Bassotti G, et al. Effect of erythromycin administration on upper gastrointestinal motility in scleroderma patients. Scand J Gastroenterol 1994; 29:807.
  65. Bouras EP, Camilleri M, Burton DD, et al. Prucalopride accelerates gastrointestinal and colonic transit in patients with constipation without a rectal evacuation disorder. Gastroenterology 2001; 120:354.
  66. Emmanuel AV, Kamm MA, Roy AJ, et al. Randomised clinical trial: the efficacy of prucalopride in patients with chronic intestinal pseudo-obstruction--a double-blind, placebo-controlled, cross-over, multiple n = 1 study. Aliment Pharmacol Ther 2012; 35:48.
  67. Camilleri M, Van Outryve MJ, Beyens G, et al. Clinical trial: the efficacy of open-label prucalopride treatment in patients with chronic constipation - follow-up of patients from the pivotal studies. Aliment Pharmacol Ther 2010; 32:1113.
  68. Müller-Lissner S, Rykx A, Kerstens R, Vandeplassche L. A double-blind, placebo-controlled study of prucalopride in elderly patients with chronic constipation. Neurogastroenterol Motil 2010; 22:991.
  69. Camilleri M, Brown ML, Malagelada JR. Impaired transit of chyme in chronic intestinal pseudoobstruction. Correction by cisapride. Gastroenterology 1986; 91:619.
  70. Camilleri M. Appraisal of medium- and long-term treatment of gastroparesis and chronic intestinal dysmotility. Am J Gastroenterol 1994; 89:1769.
  71. Camilleri M, Malagelada JR, Abell TL, et al. Effect of six weeks of treatment with cisapride in gastroparesis and intestinal pseudoobstruction. Gastroenterology 1989; 96:704.
  72. Camilleri M, Balm RK, Zinsmeister AR. Determinants of response to a prokinetic agent in neuropathic chronic intestinal motility disorder. Gastroenterology 1994; 106:916.
  73. Lipton AB, Knauer CM. Pseudo-obstruction of the bowel. Therapeutic trial of metoclopramide. Am J Dig Dis 1977; 22:263.
  74. Soudah HC, Hasler WL, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med 1991; 325:1461.
  75. Verne GN, Eaker EY, Hardy E, Sninsky CA. Effect of octreotide and erythromycin on idiopathic and scleroderma-associated intestinal pseudoobstruction. Dig Dis Sci 1995; 40:1892.
  76. von der Ohe MR, Camilleri M, Thomforde GM, Klee GG. Differential regional effects of octreotide on human gastrointestinal motor function. Gut 1995; 36:743.
  77. Foxx-Orenstein A, Camilleri M, Stephens D, Burton D. Effect of a somatostatin analogue on gastric motor and sensory functions in healthy humans. Gut 2003; 52:1555.
  78. Hasler WL, Soudah HC, Owyang C. A somatostatin analogue inhibits afferent pathways mediating perception of rectal distention. Gastroenterology 1993; 104:1390.
  79. Borgaonkar MR, Lumb B. Acute on chronic intestinal pseudoobstruction responds to neostigmine. Dig Dis Sci 2000; 45:1644.
  80. Laustsen J, Harling H, Fallingborg J. Treatment of chronic idiopathic intestinal pseudoobstruction. Dig Dis Sci 1987; 32:222.
  81. Valdovinos MA, Camilleri M, Thomforde GM, Frie C. Reduced accuracy of 14C-D-xylose breath test for detecting bacterial overgrowth in gastrointestinal motility disorders. Scand J Gastroenterol 1993; 28:963.
  82. Ghirardo S, Sauter B, Levy G, et al. Primary intestinal autoimmune disease as a cause of chronic intestinal pseudo-obstruction. Gut 2005; 54:1206.
  83. 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; 54:1343.
  84. Haas S, Bindl L, Fischer HP. Autoimmune enteric leiomyositis: a rare cause of chronic intestinal pseudo-obstruction with specific morphological features. Hum Pathol 2005; 36:576.
  85. Badari A, Farolino D, Nasser E, et al. A novel approach to paraneoplastic intestinal pseudo-obstruction. Support Care Cancer 2012; 20:425.
  86. Pakarinen MP, Kurvinen A, Koivusalo AI, et al. Surgical treatment and outcomes of severe pediatric intestinal motility disorders requiring parenteral nutrition. J Pediatr Surg 2013; 48:333.
  87. Sabbagh C, Amiot A, Maggiori L, et al. Non-transplantation surgical approach for chronic intestinal pseudo-obstruction: analysis of 63 adult consecutive cases. Neurogastroenterol Motil 2013; 25:e680.
  88. Thompson AR, Pearson T, Ellul J, Simson JN. Percutaneous endoscopic colostomy in patients with chronic intestinal pseudo-obstruction. Gastrointest Endosc 2004; 59:113.
  89. Lee RG, Nakamura K, Tsamandas AC, et al. Pathology of human intestinal transplantation. Gastroenterology 1996; 110:1820.
  90. Quigley EM. Small intestinal transplantation: reflections on an evolving approach to intestinal failure. Gastroenterology 1996; 110:2009.
  91. Sigurdsson L, Reyes J, Kocoshis SA, et al. Intestinal transplantation in children with chronic intestinal pseudo-obstruction. Gut 1999; 45:570.
  92. Sauvat F, Fusaro F, Lacaille F, et al. Is intestinal transplantation the future of children with definitive intestinal insufficiency? Eur J Pediatr Surg 2008; 18:368.
  93. Ueno T, Wada M, Hoshino K, et al. A national survey of patients with intestinal motility disorders who are potential candidates for intestinal transplantation in Japan. Transplant Proc 2013; 45:2029.
  94. Stanghellini V, Cogliandro RF, de Giorgio R, et al. Chronic intestinal pseudo-obstruction: manifestations, natural history and management. Neurogastroenterol Motil 2007; 19:440.
  95. Stanghellini V, Cogliandro RF, De Giorgio R, et al. Natural history of intestinal failure induced by chronic idiopathic intestinal pseudo-obstruction. Transplant Proc 2010; 42:15.
  96. Mousa H, Hyman PE, Cocjin J, et al. Long-term outcome of congenital intestinal pseudoobstruction. Dig Dis Sci 2002; 47:2298.
  97. Faure C, Goulet O, Ategbo S, et al. Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology. Dig Dis Sci 1999; 44:953.
  98. Mann SD, Debinski HS, Kamm MA. Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults. Gut 1997; 41:675.
  99. Lauro A, Zanfi C, Pellegrini S, et al. Isolated intestinal transplant for chronic intestinal pseudo-obstruction in adults: long-term outcome. Transplant Proc 2013; 45:3351.