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Pathology of Gut Motility Disorders: Chronic Intestinal Pseudoobstruction and Entities Other than Hirschsprung’s Disease

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Surgical Pathology of the Gastrointestinal System

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Abstract

Chronic gastrointestinal pseudo-obstruction (CIPO) is a disorder characterized by poor digestive tract motility due to inadequate neuromuscular function [1]. Patients with this disorder manifest with recurrent episodes of failure to pass stools, like mechanical obstruction but with no proven evidence of anatomical intestinal obstruction or stricture on further investigations [2]. However, at times it is difficult clinically to distinguish these two entities. Symptoms of CIPO vary from patient to patient depending on the location of the non-function and the degree of pathological involvement; however, the small intestine is nearly always involved. Common presenting features include dysphagia, gastroesophageal reflux, abdominal pain, nausea, vomiting, bloating, abdominal distension, constipation or diarrhea, and involuntary weight loss [3]. Unfortunately, these symptoms are nonspecific, which can contribute to misdiagnosis or a delay in diagnosis and treatment. Since many of the symptoms and signs suggest a mechanical bowel obstruction, diagnostic tests to uncover a mechanical obstruction are needed. The severity of the clinical picture, generally characterized by disabling digestive symptoms even between sub-occlusive episodes, contributes to the deterioration of the quality of life of affected patients. The common causes of mechanical intestinal obstruction are inflammatory or malignant strictures as, intestinal tuberculosis, Crohn’s disease, or the presence of an occluding tumor mass. After ruling out all possible causes of mechanical obstruction, the diagnosis of CIPO is considered. CIPO can be caused by disturbances in the enteric nervous system, extrinsic nervous system, intestinal smooth muscle layers, or absence of the pulse-generating interstitial cell of Cajal (ICC). The disorders can be inherited or acquired secondary to systemic disease involvement, as amyloidosis, hypothyroidism, etc. (Please refer to 7 Chap. 14 for a detailed description.) Hence, the primary CIPO can be classified as congenital (present at birth), familial (most likely genetic), or sporadic (seemingly occurring at random) types. While in adults CIPO is primarily sporadic, in children, these are mostly inherited [4]. Not infrequently the cause of CIPO cannot be determined. Detailed clinical and histopathological investigation is needed including clinical examination, imaging, manometric studies, and diligent histological examination of the affected segment [3]. It is relatively difficult to treat patients affected with CIPO, and supplementation of the nutritional requirement often becomes the primary challenge.

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References

  1. Stanghellini V, Cogliandro RF, De Giorgio R, Barbara G, Salvioli B, Corinaldesi R. Chronic intestinal pseudo-obstruction: manifestations, natural history and management. Neurogastroenterol Motil. 2007;19:440–52.

    CAS  PubMed  Google Scholar 

  2. Antonucci A, Fronzoni L, Cogliandro L, Cogliandro RF, Caputo C, De Giorgio R, et al. Chronic intestinal pseudo-obstruction. World J Gastroenterol. 2008;14:2953–61.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. De Giorgio R, Cogliandro RF, Barbara G, Corinaldesi R, Stanghellini V. Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy. Gastroenterol Clin N Am. 2011;40(4):787–807.

    Google Scholar 

  4. Lacy BE, Lowe BJ. Diagnosis, treatment and nutritional management of chronic intestinal pseudo-obstruction. Pract Gastroenterol. 2009;33(8):9–24.

    Google Scholar 

  5. Chial HJ, Camilleri M, Burton D, Thomforde G, Olden KW, Stephens D. Selective effects of serotonergic psychoactive agents on gastrointestinal functions in health. Am J Physiol Gastrointest Liver Physiol. 2003;284(1):G130–7.

    CAS  PubMed  Google Scholar 

  6. Nezami BG, Srinivasan S. Enteric nervous system in the small intestine: pathophysiology and clinical implications. Curr Gastroenterol Rep. 2010;12(5):358–65. https://doi.org/10.1007/s11894-010-0129-9.

    Article  PubMed  PubMed Central  Google Scholar 

  7. 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(2):156–64.

    PubMed  Google Scholar 

  8. Boeckxstaens GE. Understanding and controlling the enteric nervous system. Best Pract Res Clin Gastroenterol. 2002;16(6):1013–23.

    CAS  PubMed  Google Scholar 

  9. De Giorgio R, Sarnelli G, Corinaldesi R, Stanghellini V. Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction. Gut. 2004;53:1549–52.

    PubMed  PubMed Central  Google Scholar 

  10. Deglincerti A, De Giorgio R, Cefle K, Devoto M, Pippucci T, Castegnaro G, Panza E, Barbara G, Cogliandro RF, Mungan Z, et al. A novel locus for syndromic chronic idiopathic intestinal pseudo-obstruction maps to chromosome 8q23-q24. Eur J Hum Genet. 2007;15:889–97.

    CAS  PubMed  Google Scholar 

  11. Gargiulo A, Auricchio R, Barone MV, Cotugno G, Reardon W, Milla PJ, Ballabio A, Ciccodicola A, Auricchio A. Filamin A is mutated in X-linked chronic idiopathic intestinal pseudo-obstruction with central nervous system involvement. Am J Hum Genet. 2007;80:751–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  12. Hirano M, Silvestri G, Blake DM, Lombes A, Minetti C, Bonilla E, Hays AP, Lovelace RE, Butler I, Bertorini TE. Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): clinical, biochemical, and genetic features of an autosomal recessive mitochondrial disorder. Neurology. 1994;44:721–7.

    CAS  PubMed  Google Scholar 

  13. Valentino ML, Marti R, Tadesse S, Lopez LC, Manes JL, Lyzak J, Hahn A, Carelli V, Hirano M. Thymidine and deoxyuridine accumulate in tissues of patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). FEBS Lett. 2007;581:3410–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Rodrigues CA, Shepherd NA, Lennard-Jones JE, Hawley PR, Thompson HH. Familial visceral myopathy: a family with at least six involved members. Gut. 1989;30(9):1285–92.

    CAS  PubMed  PubMed Central  Google Scholar 

  15. Sigurdsson L, Guze C, Hyman PE, Flores A, Payne V, Di Lorenzo C. Children growing up with hollow visceral myopathy: 63. J Pediatr Gastroenterol Nutr. 1997;25(4):458.

    Google Scholar 

  16. Sen R, Gupta V, Hasija S, Ralli M, Garg S. Visceral myopathy causing intestinal obstruction: a case report. J Gastroenterol Hepatol Res. 2014;3(8):1213–5.

    Google Scholar 

  17. Kharbuja P, Thakur R, Suo J. Visceral myopathy presenting as acute appendicitis and Ogilvie syndrome. Case Rep Surg. 2013;2013:906457.

    PubMed  PubMed Central  Google Scholar 

  18. Vallejo V, Wiener I, Stroh BC. Idiopathic muscular hypertrophy of the duodenum. J Surg Case Rep. 2012;2012(1):9.

    PubMed  PubMed Central  Google Scholar 

  19. Koh S, Bradley RF, French SW, Farmer DG, Cortina G. Congenital visceral myopathy with a predominantly hypertrophic pattern treated by multivisceral transplantation. Hum Pathol. 2008;39(6):970–4.

    PubMed  Google Scholar 

  20. Dranove JE, Applegate K, Croffie JM. Type I familial visceral myopathy presenting with CT findings of superior mesenteric artery syndrome in an adolescent. J Pediatr Gastroenterol Nutr. 2009;49(4):379.

    PubMed  Google Scholar 

  21. Fogel SP, DeTar MW, Shimada H, Chandrasoma PT. Sporadic visceral myopathy with inclusion bodies. A light-microscopic and ultrastructural study. Am J Surg Pathol. 1993;17(5):473–81.

    CAS  PubMed  Google Scholar 

  22. Reddy SB, Hamersky CM, Gavankar SS, Singson ZJ, Deshmukh-Rane S, Carmichael JC, Wu ML, Lawry GV, Lee RH, Albers GC, Parekh NK. Sporadic visceral myopathy: full thickness rectal biopsy to clinch the diagnosis. J GHR. 2012;1(8):161–4.

    Google Scholar 

  23. Smith VV, Milla PJ. Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood. Histopathology. 1997;31(2):112–22.

    CAS  PubMed  Google Scholar 

  24. Lee EY, Stenson WF, DeSchryver-Kecskemeti K. Thickening of muscularis mucosae in Crohn’s disease. Mod Pathol. 1991;4(1):87–90.

    CAS  PubMed  Google Scholar 

  25. Pawel BR, de Chadarévian JP, Franco ME. The pathology of fibrosing colonopathy of cystic fibrosis: a study of 12 cases and review of the literature. Hum Pathol. 1997;28(4):395–9.

    CAS  PubMed  Google Scholar 

  26. Slavotinek AM. Fryns syndrome: a review of the phenotype and diagnostic guidelines. Am J Med Genet A. 2004;124(4):427–33.

    Google Scholar 

  27. Anand S, Dhua AK, Sankar J, Das P, Goel P, Bajpai M, Sharma A. Segmental ileal dilatation presenting with neonatal intestinal obstruction. J Indian Assoc Pediatr Surg. 2020;25(5):306.

    PubMed  PubMed Central  Google Scholar 

  28. Eşrefoğlu M, Çetin A. Development of small and large intestine. Bezmialem Sci. 2017;4:36–40.

    Google Scholar 

  29. Benedeczky I, Fekete E, Resch B. Ultrastructure of the developing muscle and enteric nervous system in the small intestine of human fetus. Acta Physiol Hung. 1993;81:193–206.

    CAS  PubMed  Google Scholar 

  30. Jain P, Rajeshwari SM, Singh J, Kumar T, Agarwal SP, Das P. Myofibrillar myopathy presenting as neonatal intestinal pseudo-obstruction: an extremely rare entity. Fetal Pediatr Pathol. 2016;35(2):124–8.

    PubMed  Google Scholar 

  31. Ariza A, Coll J, Fernandez-Figueras MT, Lopez MD, Mate J, García O, Fernandez-Vasalo A, Navas-Palacios J. Desmin myopathy: a multisystem disorder involving skeletal, cardiac, and smooth muscle. Hum Pathol. 1995;26(9):1032–7.

    CAS  PubMed  Google Scholar 

  32. Abraham SC, Denofrio D, Loh E, Minda JM, Tomaszewski JE, Pietra GG, Reynolds C. Desmin myopathy involving cardiac, skeletal, and vascular smooth muscle: report of a case with immunoelectron microscopy. Hum Pathol. 1998;29(8):876–82.

    CAS  PubMed  Google Scholar 

  33. Modolell I, Mearin F, Baudet JS, Gámez J, Cervera C, Malagelada JR. Pharyngo-esophageal motility disturbances in patients with myotonic dystrophy. Scand J Gastroenterol. 1999;34(9):878–82.

    CAS  PubMed  Google Scholar 

  34. Eckardt VF, Nix W, Kraus W, Bohl J. Esophageal motor function in patients with muscular dystrophy. Gastroenterology. 1986;90(3):628–35.

    CAS  PubMed  Google Scholar 

  35. Kuiper DH. Gastric bezoar in a patient with myotonic dystrophy. A review of the gastrointestinal complications of myotonic dystrophy. Am J Dig Dis. 1971;16(6):529–34.

    CAS  PubMed  Google Scholar 

  36. Rönnblom A, Danielsson A, El-Salhy M. Intestinal endocrine cells in myotonic dystrophy: an immunocytochemical and computed image analytical study. J Intern Med. 1999;245(1):91–7.

    PubMed  Google Scholar 

  37. Goldberg HI, Sheft DJ. Esophageal and colon changes in myotonia dystrophica. Gastroenterology. 1972;63(1):134–9.

    CAS  PubMed  Google Scholar 

  38. Yoshida MM, Krishnamurthy S, Wattchow DA, Furness JB, Schuffler MD. Megacolon in myotonic dystrophy caused by a degenerative neuropathy of the myenteric plexus. Gastroenterology. 1988;95(3):820–7.

    CAS  PubMed  Google Scholar 

  39. Keschner M, Davison D. Dystrophia myotonica: a clinico-pathologic study. Arch Neurol Psychiatry. 1933;30(6):1259–75.

    Google Scholar 

  40. Abercrombie JF, Rogers J, Swash M. Faecal incontinence in myotonic dystrophy. J Neurol Neurosurg Psychiatry. 1998;64(1):128–30.

    CAS  PubMed  PubMed Central  Google Scholar 

  41. Henning Z, Le Grange SM, Du Toit RS. Degenerative leiomyopathy. SA J Radiol. 2006;10(2):16–7.

    Google Scholar 

  42. Brown R, Numanoglu A, von Delft D, Rode H. Degenerative hollow visceral myopathy mimicking Hirschsprung’s disease. In: Hirschsprung’s disease and allied disorders. Cham: Springer; 2019. p. 331–44.

    Google Scholar 

  43. Shaibani A, Shchelochkov OA, Zhang S, Katsonis P, Lichtarge O, Wong LJ, Shinawi M. Mitochondrial neurogastrointestinal encephalopathy due to mutations in RRM2B. Arch Neurol. 2009;66(8):1028–32.

    PubMed  PubMed Central  Google Scholar 

  44. Groisman GM, Cohen HI. Small intestinal amyloidosis: a rare cause of diverticular disease. Case Rep Pathol. 2014;2014:362835. 4 pages.

    PubMed  PubMed Central  Google Scholar 

  45. Tada S, Iida M, Yao T, Kitamoto T, Yao T, Fujishima M. Intestinal pseudo-obstruction in patients with amyloidosis: clinicopathologic differences between chemical types of amyloid protein. Gut. 1993;34:1412–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  46. Lyford G, Foxx-Orenstein A. Chronic intestinal pseudoobstruction. Curr Treat Options Gastroenterol. 2004;7:317–25.

    PubMed  Google Scholar 

  47. Muñoz-Yagüe MT, Solís-Muñoz P, Salces I, Ballestín C, Colina F, Ibarrola C, López-Alonso G, Carreira P, Cruz Vigo F, Solís Herruzo JA. Chronic intestinal pseudo-obstruction: a diagnosis to be considered. Rev Esp Enferm Dig. 2009 May;101(5):336–42.

    PubMed  Google Scholar 

  48. Schärli AF, Sossai R. Hypoganglionosis. In Seminars in pediatric surgery 1998 (7,3, pp. 187-191). WB Saunders.

    Google Scholar 

  49. Sreedher G, Garrison A, Novak R, Keisling M, Ganapathy SS. Congenital intestinal hypoganglionosis: a radiologic mimic of Hirschsprung’s disease. Radiol Case Rep. 2019;14(2):171–4.

    PubMed  Google Scholar 

  50. Dingemann J, Puri P. Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int. 2010;26(11):1111–5.

    PubMed  Google Scholar 

  51. Henna N, Nagi AH, Sheikh MA, Shaukat M. Morphological patterns in children with ganglion related enteric neuronal abnormalities. J Ayub Med Coll Abbottabad. 2011;23(3):14–7.

    PubMed  Google Scholar 

  52. Niramis R, Tongsin A, Lertsatit A, Tanvichien L, Chaiprapa H, Junyangdikul P. How to manage low gut obstruction in neonates with immature ganglion cells in the colonic wall. J Med Assoc Thai. 2014;97(suppl 6):S66–73.

    PubMed  Google Scholar 

  53. Accarino A, Colucci R, Barbara G, et al. Mast cell neuromuscular involvement in patients with severe gastrointestinal motility disorders. Gut. 2007;39:A18.

    Google Scholar 

  54. Meier-Ruge WA, Ammann K, Bruder E, Holschneider AM, Schärli AF, Schmittenbecher PP, Stoss F. Updated results on intestinal neuronal dysplasia (IND B). Eur J Pediatr Surg. 2004;14(06):384–91.

    CAS  PubMed  Google Scholar 

  55. Meier-Ruge WA, Bruder E, Kapur RP. Intestinal neuronal dysplasia: one giant ganglion is not enough. Pediatr Dev Pathol. 2006;9:444–52.

    PubMed  Google Scholar 

  56. Vougas V, Vardas K, Christou C, Papadimitriou G, Florou E, Magkou C, Karamanolis D, Manganas D, Drakopoulos S. Intestinal neuronal dysplasia type B in adults: a controversial entity. Case Rep Gastroenterol. 2014;8(1):7–12.

    PubMed  PubMed Central  Google Scholar 

  57. Vijayaraghavan R, Chandrashekar R, Jyotiprakash AM, Kumar R, Rashmi MV, Belagavi CS. Intestinal neuronal dysplasia (type B) causing fatal small bowel ischaemia in an adult: a case report. Eur J Gastroenterol Hepatol. 2006;18(7):773–6.

    PubMed  Google Scholar 

  58. de Arruda Lourenção PL, Ortolan EV, Rosa LL, Angelini MC, Terra SA, Rodrigues MA. Long-term follow-up of patients with intestinal neuronal dysplasia type B: protocol for an observational, ambispective, and comparative study. Medicine. 2017;96(28):e7485.

    Google Scholar 

  59. Meier Ruge WA, Bruder E. Immaturity of enteric nervous system. Pathobiology. 2005;72:34–6.

    Google Scholar 

  60. Kobayashi H, Mahomed A, Puri P. Intestinal neuronal dysplasia in twins. J Pediatr Gastroenterol Nutr. 1996;22:398–401.

    CAS  PubMed  Google Scholar 

  61. Al-Rikabi AC, Al-Sohaibani MO, Al-shaibi H, Al-sayyah A, Al Saigh A, Sayah H. Pseudo-obstruction of the gastric outlet caused by combined hyperganglionosis and ganglioneuromatosis in an adult: case report and literature review. Oman medical journal. 2011;26(5):e021.

    PubMed  PubMed Central  Google Scholar 

  62. Smith VV, Eng C, Milla PJ. Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2B: implications for treatment. Gut. 1999;45(1):143–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  63. Mitra S, Mukherjee S, Chakraborty H. Isolated ileal ganglioneuromatosis in an 11-year-old boy: case report and review of literature. Indian J Pathol Microbiol. 2016;59(3):365.

    PubMed  Google Scholar 

  64. Charagundla SR, Levine MS, Torigian DA, Campbell MS, Furth EE, Rombeau J. Diffuse intestinal ganglioneuromatosis mimicking Crohn’s disease. Am J Roentgenol. 2004;182(5):1166–8.

    Google Scholar 

  65. Donald G, Meier-Ruge WA, Aravinda C, Jacob C. Chronic constipation due to Hirschsprung’s disease and desmosis coli in a family. Pediatr Surg Int. 2002;18:110–4.

    Google Scholar 

  66. Mallick S, Prasenjit D, Prateek K, Shasanka PS, Virender S, Rajni Y, Gaurav J, Vijay MK, Arun KV, Mahajan JK, Sandeep A, Ranjan DN, Siddhartha DG. Chronic intestinal pseudo-obstruction: systematic histopathological approach can clinch vital clues. Virchows Arch. 2014;464(5):529–37.

    PubMed  Google Scholar 

  67. Meier-Ruge WA. Desmosis of the colon: a working hypothesis of primary chronic constipation. Eur J Pediatr Surg. 1998;8(5):299–303.

    CAS  PubMed  Google Scholar 

  68. Meier-Ruge WA, Bruder E. The morphological characteristics of aplastic and atrophic desmosis of the intestine. Pathologe. 2007;28(2):149–54.

    CAS  PubMed  Google Scholar 

  69. Desale J, Shah H, Kumbhar V, Sandlas G. Desmosis coli – a case report and review of the literature. Dev Period Med. 2017;21(4):390–2.

    PubMed  Google Scholar 

  70. Szymanska S, Markiewicz-Kijewska M, Pyzlak M, Wieckowska-Karkucinska A, Kalicinski P, Pronicki M. Familiar occurrence of desmosis of the colon–report of two cases and literature review. J Gen Pract (Los Angel). 2017;5(301):2.

    Google Scholar 

  71. Hübner U, Meier-Ruge W, Halsband H. Four cases of desmosis coli: severe chronic constipation, massive dilatation of the colon, and hypoperistalsis due to of changes in the colonic connective-tissue net. Pediatr Surg Int. 2002;18(2–3):198–203.

    PubMed  Google Scholar 

  72. Kenny SE, Vanderwinden JM, Rintala RJ, Connell MG, Lloyd DA, Vanderhaegen JJ, De Laet MH. Delayed maturation of the interstitial cells of Cajal: a new diagnosis for transient neonatal pseudoobstruction. Report of two cases. J Pediatr Surg. 1998;33(1):94–8.

    CAS  PubMed  Google Scholar 

  73. Tse Y, El-Zimaity H, Pallister C, Liu L. A novel case of overlapping neuropathy, mesenchymopathy, and myopathy resulting in recurrent volvulus in a patient with agenesis of corpus callosum, and a systemic review of the literature: 1316. Am J Gastroenterol. 2013;108:S392.

    Google Scholar 

  74. Sakaguchi T, Hamada Y, Nakamura Y, Hamada H, Kwon AH. Absence of the interstitial cells of Cajal in a neonate with segmental dilatation of ileum. J Pediatr Surg Case Rep. 2016;5:19–22.

    Google Scholar 

  75. Chang IY, Glasgow NJ, Takayama I, Horiguchi K, Sanders KM, Ward SM. Loss of interstitial cells of Cajal and development of electrical dysfunction in murine small bowel obstruction. J Physiol. 2001;536(2):555–68.

    CAS  PubMed  PubMed Central  Google Scholar 

  76. Marshall JB, Kretschmar JM, Gerhardt DC, Winship DH, Winn D, Treadwell EL, Sharp GC. Gastrointestinal manifestations of mixed connective tissue disease. Gastroenterology. 1990;98(5):1232–8.

    CAS  PubMed  Google Scholar 

  77. Hughes A, Ferguson I, Rankin E, Kane K. Polymyositis as a cause of total gut failure. Ann Rheum Dis. 2002;61(4):305.

    CAS  PubMed  PubMed Central  Google Scholar 

  78. Matsuda NM, Miller SM, Evora PR. The chronic gastrointestinal manifestations of Chagas disease. Clinics. 2009;64(12):1219–24.

    PubMed  PubMed Central  Google Scholar 

  79. Soriano-Arandes A, Angheben A, Serre-Delcor N, et al. Control and management of congenital Chagas disease in Europe and other non-endemic countries: current policies and practices. Tropical Med Int Health. 2016;21:590–6.

    Google Scholar 

  80. Kim MY, Noh JH. A case of Kawasaki disease with colonic edema. J Korean Med Sci. 2008;23(4):723–6.

    PubMed  PubMed Central  Google Scholar 

  81. Veress B, Nyberg B, Törnblom H, Lindberg G. Intestinal lymphocytic epithelioganglionitis: a unique combination of inflammation in bowel dysmotility:a histopathological and immunohistochemical analysis of 28 cases. Histopathology. 2009;54(5):539–49.

    PubMed  Google Scholar 

  82. Racalbuto A, Magro G, Lanteri R, Aliotta I, Santangelo M, Di Cataldo A. Idiopathic myenteric ganglionitis underlying acute ‘dramatic’ intestinal pseudoobstruction: report of an exceptional case. Case Rep Gastroenterol. 2008;2(3):461–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  83. De Giorgio R, Barbara G, Stanghellini V, et al. Idiopathic myenteric ganglionitis underlying intractable vomiting in a young adult. Eur J Gastroenterol Hepatol. 2000;12:613–6.

    PubMed  Google Scholar 

  84. Smith VV, Schäppi MG, Bisset WM, Kiparissi F, Jaffe A, Milla PJ, Lindley KJ. Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus. J Pediatr Gastroenterol Nutr. 2009;49(1):42–51.

    PubMed  Google Scholar 

  85. Akazawa Y, Hayashi T, Saito T, Niwa K, Kamiyama H, Sasahara N, Sakamoto K, Nagahara A, Yao T. Histological characteristics of eosinophilic myenteric ganglionitis: an under-recognised cause of chronic intestinal pseudo-obstruction. Virchows Arch. 2019;474(3):395–400.

    PubMed  Google Scholar 

  86. Schäppi MG, Smith VV, Milla PJ, Lindley KJ. Eosinophilic myenteric ganglionitis is associated with functional intestinal obstruction. Gut. 2003;52(5):752–5.

    PubMed  PubMed Central  Google Scholar 

  87. Conti G, Scarpini E, Baron P, et al. Macrophage infiltration and death in the nerve during the early phases of experimental diabetic neuropathy: a process concomitant with endoneurial induction of IL-1beta and p75NTR. J Neurol Sci. 2002;195:35–40.

    CAS  PubMed  Google Scholar 

  88. Lennon VA, Sas DF, Busk MF, Scheithauer B, Malagaleda J-R, Camilleri M, Miller LJ. Enteric neuronal antibodies in pseudoobstruction with small-cell lung cancer. Gastroenterology. 1991;100:137–42.

    CAS  PubMed  Google Scholar 

  89. Wood JR, Camilleri M, Low PA, Malagelada J-R. Brainstem tumor presenting as an upper gut motility disorder. Gastroenterology. 1985;89:1411–4.

    CAS  PubMed  Google Scholar 

  90. Lhermitte F, Gray F, Lyon-Caen O, Pertuiset BF. Paralysis of the digestive tract with lesions of the myenteric plexus: a new paraneoplastic syndrome. Rev Neurol (Paris). 1980;136:825–36.

    CAS  Google Scholar 

  91. Shafer RB, Prentiss RF, Bond JH. Gastrointestinal transit in thyroid disease. Gastroenterology. 1984;86:852–5.

    CAS  PubMed  Google Scholar 

  92. Tobin MV, Fisken RA, Diggory RT, Morris AI. Orocecal transit time in health and in thyroid disease. Gut. 1989;30:26–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  93. Sonsino E, Mouy R, Foucaud P, Cezard JP, Aigrain Y, Bocquet L, Navarro J. Intestinal pseudoobstruction related to CMV infection of myenteric plexus. N Engl J Med. 1984;311:196–7.

    CAS  PubMed  Google Scholar 

  94. Hauer-Jensen M, Denham JW, Andreyev HJ. Radiation enteropathy—pathogenesis, treatment and prevention. Nat Rev Gastroenterol Hepatol. 2014;11(8):470.

    CAS  PubMed  PubMed Central  Google Scholar 

  95. Barrera-Herrera LE, Arias F, Rodríguez-Urrego PA, Palau-Lázaro MA. Small bowel obstruction due to intestinal Xanthomatosis. Case Rep Pathol. 2015;2015:231830.

    CAS  PubMed  PubMed Central  Google Scholar 

  96. Scheiman J, Elta G, Colturi T, Nostrant T. Colonic xanthomatosis. Dig Dis Sci. 1988;33(11):1491–4.

    CAS  PubMed  Google Scholar 

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The authors agree to the fact that all biopsy procedures and surgical resections were performed after taking informed consent from respective patients as per the individual Institutional policies, which also includes consent for publishing the unidentified clinical images for publication or research purposes. The authors also declare no conflict of interest.

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  1. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Prasenjit Das

  2. Department of Pathology, Armed Forces Medical College, Wanowrie, Pune, Maharashtra, India

    Prateek Kinra

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  1. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Prasenjit Das

  2. Department of Pathology, Al Sabah Hospital, Kuwait City, Kuwait

    Kaushik Majumdar

  3. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Siddhartha Datta Gupta

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Das, P., Kinra, P. (2022). Pathology of Gut Motility Disorders: Chronic Intestinal Pseudoobstruction and Entities Other than Hirschsprung’s Disease. In: Das, P., Majumdar, K., Datta Gupta, S. (eds) Surgical Pathology of the Gastrointestinal System. Springer, Singapore. https://doi.org/10.1007/978-981-16-6395-6_11

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  • DOI: https://doi.org/10.1007/978-981-16-6395-6_11

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-16-6394-9

  • Online ISBN: 978-981-16-6395-6

  • eBook Packages: MedicineMedicine (R0)

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