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Motor Disorders

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Pathology of Pediatric Gastrointestinal and Liver Disease

Abstract

The phrase intestinal dysmotility refers to a broad range of clinical conditions that are characterized by impaired peristalsis. In children, common symptoms of intestinal dysmotility include constipation, encoporesis, diarrhea, vomiting, and abdominal pain. Less frequently, severe chronic constipation, failure-to-thrive, abdominal distension, enterocolitis, or other obstructive symptoms are present. The majority of patients with a history of dysmotility suffer from functional disorders which have no established organic etiology and are diagnosed using symptom-based criteria.1,2 However, for pediatric patients, particularly those with severe symptoms, anatomic pathology is often sought and occasionally found to explain their clinical problems. In some cases, the cause will be an acute or intermittent obstructive condition (e.g., atresia, volvulus, intussusception, and so on) associated with obvious gross pathology, subjects that are covered in Chapter 1. Conditions dominated by diarrhea, which are principally inflammatory or infectious disorders, are also discussed elsewhere in this text (see Chapter 5). The pathological correlates of the remaining infants and children who exhibit severe chronic constipation or pseudo-obstruction are the focus of this chapter.

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References

  1. Youssef NN, Di Lorenzo C. Childhood constipation: evaluation a nd treatment. J Clin Gastroenterol. 2001;33: 199–205.

    Article  PubMed  CAS  Google Scholar 

  2. Stordal K, Nygaard EA, Bentsen B. Organic abnormalities in recurrent abdominal pain in children. Acta Pediatr. 2001;90:638–642.

    Article  CAS  Google Scholar 

  3. Rudolph CD, Hyman PE, Altschuler SM, et al. Diagnosis and t reatment of chronic intestinal pseudo-obstruction in children: Report of a consensus workshop. J Pediatr Gastroenterol Nutr. 1997;24: 102–112.

    Article  PubMed  CAS  Google Scholar 

  4. Kapur RP. Hirschsprung disease and other enteric dysganglionoses. Crit Rev Clin Lab Sci. 1999;36:225–273.

    Article  PubMed  CAS  Google Scholar 

  5. Newgreen DF, Young HM. Enteric nervous system: development and developmental disturbances-part 1. Pediatr Dev Pathol. 2002;5:224–247.

    PubMed  CAS  Google Scholar 

  6. Young HM, Hearn CJ, Ciampoli D, et al. A single rostrocaudal colonization of the rodent intestine by enteric neuron precursors is revealed by the expression of Phox2b, Ret, and p75 and by expiants grown under the kidney capsule or in organ culture. Dev Biol. 1998;202: 67–84.

    Article  PubMed  CAS  Google Scholar 

  7. Okamoto E, Ueda T. Embryogenesis of intraneural ganglia of the gut and its relation to Hirschsprung’s disease. J Pediatr Surg. 1967;2:437–443.

    Article  Google Scholar 

  8. Bughaighis AG, Emery JL. Functional obstruction of the intestine due to neurological immaturity. Prog Pediatr Surg. 1971;3: 37–52.

    PubMed  CAS  Google Scholar 

  9. Kapur RP. Colonization of the hindgut by sacral crest-derived enteric neural precursors: support for an evolutionarily conserved model. Dev Biol. 2000;227: 146–155.

    Article  PubMed  CAS  Google Scholar 

  10. Newgreen DF, Southwell B, Hartley L, et al. Migration of enteric neural crest cells in relation to growth of the gut in avian embryos. Acta Anat. 1996;157: 105–115.

    Article  PubMed  CAS  Google Scholar 

  11. Pham TD, Gershon MD, Rothman TP. Time of origin of neurons in the murine enteric nervous system: sequence in relation to phenotype. J Comp Med. 1991;314:789–798.

    CAS  Google Scholar 

  12. Smith B. The neuropathology of pseudo-obstruction of the intestine. Scand J Gastroenterol Suppl. 1982;71:103–109.

    PubMed  CAS  Google Scholar 

  13. Gianino S, Grider JR, Cresswell J, Enomoto H, Heuckeroth RO. GDNF availability determines enteric neuron number by controlling precursor proliferation. Development. 2003; 130:2187–2198.

    Article  PubMed  CAS  Google Scholar 

  14. Micci M-A, Learish RD, Li H, et al. Neural stem cells express RET, produce nitric oxide, and survive transplantation in the gastrointestinal tract. Gastroenterology. 2001;121:757–766.

    Article  PubMed  CAS  Google Scholar 

  15. Smith W. Intestinal neuronal density in childhood: a baseline for the objective assessment of hypo-and hyperganglionosis. Pediatr Pathol. 1993;13:225–237.

    Article  PubMed  CAS  Google Scholar 

  16. Heinicke EA, Kiernan JA, Wijsman J. Specific, selective, and complete staining of neurons of the myenteric plexus using Cuprolinic blue. J Neurosci Meth. 1987;21:45–54.

    Article  CAS  Google Scholar 

  17. Krammer HJ, Karahan ST, Sigge W, et al. Immunohistochemistry of markers of the enteric nervous system in whole-mount preparations of the human colon. Eur J Pediatr Surg. 1994;4: 274–278.

    Article  PubMed  CAS  Google Scholar 

  18. Karaosmanoglu T, Aygun B, Wade PR, et al. Regional differences in the number of neurons in the myenteric plexus of guinea pig small intestine and colon: an evaluation of markers used to count neurons. Anat Rec. 1996;244: 470–480.

    Article  PubMed  CAS  Google Scholar 

  19. Koberle F. Chagas’ disease and Chagas’ syndromes: the pathology of American trypanosomiasis. Adv Parasitol. 1968;6: 63–73.

    Article  PubMed  CAS  Google Scholar 

  20. de Souza RR, Moratelli HB, Borges N, et al. Age-induced nerve cell loss in the myenteric plexus of the small intestine in man. Gerontology. 1993;39:183–193.

    Article  PubMed  Google Scholar 

  21. Schuffler MD, Jonak Z. Chronic idiopathic intestinal pseudoobstruction caused by a degenerative disorder of the myenteric plexus: the use of Smith’s method to define the neuropathology. Gastroenterology. 1982;82:476–486.

    PubMed  CAS  Google Scholar 

  22. Richardson KC. Studies on the structure of autonomic nerves in the small intestine, correlating the silver-impregnated image in light microscopy with the permanganate-fixed ultrastructure in electronmicroscopy. J Anat. 1960;94: 457–472.

    PubMed  CAS  Google Scholar 

  23. Tanner MS, Smith B, Lloyd JK. Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Arch Dis Child. 1976;51: 837–841.

    Article  PubMed  CAS  Google Scholar 

  24. Krishnamurthy S, Schuffler MD, Rohrmann CA, et al. Severe idiopathic constipation is associated with a distinctive abnormality of the colonic myenteric plexus. Gastroenterology. 1985;88: 26–34.

    PubMed  CAS  Google Scholar 

  25. Navarro J, Sonsino E, Boige N, et al. Visceral neuropathies responsible for chronic intestinal pseudo-obstruction syndrome in pediatric practice: analysis of 26 cases. J Pediatr Gastroenterol Nutr. 1990;11: 221–228.

    Article  Google Scholar 

  26. Qualman SJ, Jaffe R, Bove KE, et al. Diagnosis of Hirschsprung disease using the rectal biopsy: multi-institutional survey. Pediatr Dev Pathol. 1999;2:588–596.

    Article  PubMed  CAS  Google Scholar 

  27. Aldridge RT, Cambell PE. Ganglion cell distribution in the normal rectum and anal canal: a basis for the diagnosis of Hirschsprung’s disease by anorectal biopsy. J Pediatr Surg. 1968; 3: 475–489.

    Article  PubMed  CAS  Google Scholar 

  28. Weinberg AG. The anorectal myenteric plexus: Its relation to hypoganglionosis of the colon. Am J Clin Pathol. 1970;54:637–642.

    PubMed  CAS  Google Scholar 

  29. Venugopal S, Maneer K, Shandling B. The validity of rectal biopsy in relation to morphology and distribution of ganglion cells. J Pediatr Surg. 1981;16:433–437.

    Article  PubMed  CAS  Google Scholar 

  30. Monforte-Munoz H, Gonzales-Gomez I, Rowland JM, et al. Increased submucosal nerve trunk caliber in aganglionosis. A “positive” and objective finding in suction biopsies and segmental resections in Hirschsprung’s disease. Arch Pathol Lab Med. 1998;122:721–725.

    PubMed  CAS  Google Scholar 

  31. Mebis J, Penninckx F, Geboes K, et al. Neuropathology of Hirschsprung’s disease: en face study of microdissected intestine. Hepato-gastroenterol. 1990;37:596–600.

    CAS  Google Scholar 

  32. Meier-Ruge W, Hunziger O, Tobler H-J, et al. The pathophysiology of aganglionosis of the entire colon (Zuelzer-Wilson syndrome): morphometric investigations of the extent of sacral parasympathetic innervation of the circular muscles of the aganglionic colon. Beitr Path Bd. 1972;147:228–236.

    Article  CAS  Google Scholar 

  33. Howard ER, Garrett JR. Electron microscopy of myenteric nerves in Hirschsprung disease and in normal bowel. Gut. 1970; 11:1007–1014.

    Article  PubMed  CAS  Google Scholar 

  34. Wedel T, Holschneider AM, Krammer H-J. Ultrastructural features of nerve fascicles and basal lamina abnormalities in Hirschsprung disease. Eur J Pediatr Surg. 1999;9:75–82.

    Article  PubMed  CAS  Google Scholar 

  35. Tam PKH, Boyd GP. Origin, course, and endings of abnormal enteric nerve fibers in Hirschsprung’s disease defined by whole-mount immunohistochemistry. J Pediatr Surg. 1990;25:457–461.

    Article  PubMed  CAS  Google Scholar 

  36. Meier-Ruge W, Lutterbeck PM, Herzog B, et al. Acetylcholinesterase activity in suction biopsies of the rectum in the diagnosis of Hirschsprung disease. J Pediatr Surg. 1972;7:11–16.

    Article  PubMed  CAS  Google Scholar 

  37. Chow CW, Chan WC, Ue PCK. Histochemical criteria for the diagnosis of Hirschsprung’s disease in rectal suction biopsies by acetylcholinesterase activity. J Pediatr Surg. 1977;12:675–680.

    Article  PubMed  CAS  Google Scholar 

  38. Lowichik A, Weinberg AG. Eosinophilic infiltration of the enteric neural plexuses in Hirschsprung’s disease. Ped Pathol Lab Med. 1997;17:885–891.

    Article  CAS  Google Scholar 

  39. Weinberg AG. Hirschsprung’s disease-a pathologist’s view. Perspect Ped Pathol. 1975;2:207–239.

    CAS  Google Scholar 

  40. White MD, Langer JC. Circumferential distribution of ganglion cells in the transitional zone in Hirschsprung disease. Mod Pathol 1998;11:6P (abstract).

    Google Scholar 

  41. Meier-Ruge W. Epidemiology of congenital innervation defects of the distal colon. Virchows Arch [A] 1992;420:171–177.

    Article  CAS  Google Scholar 

  42. Moore SW, Laing D, Kaschula ROC, et al. A histological grading system for the evaluation of co-existing NID with Hirschsprung’s disease. Eur J Pediatr Surg. 1994,4:293–297.

    Article  PubMed  CAS  Google Scholar 

  43. Puri P, Lake BD, Nixon HH, et al. Neuronal colonic dysplasia: an unsual association of Hirschsprung’s disease. J Pediatr Surg 1977;12:681–685.

    Article  PubMed  CAS  Google Scholar 

  44. Meyrat BJ, Lesbros Y, Laurini RN. Assessment of the colon innervation with serial biopsies above the aganglionic zone before the pull-through procedure. Pediatr Surg Int. 2001;17:129–135.

    Article  PubMed  CAS  Google Scholar 

  45. Schmittenbecher PP, Sacher P, Cholewa D, et al. Hirschsprung’s disease and intestinal neuronal dysplasia-a frequent association with implications for the postoperative course. Pediatr Surg Int. 1999;15:553–558.

    Article  PubMed  CAS  Google Scholar 

  46. Hanimann B, Inderbitzin D, Briner J, et al. Clinical relevance of Hirschsprung-associated neuronal intestinal dysplasia (HANID). J Pediatr Surg. 1992;27:344–348.

    Article  Google Scholar 

  47. Banani SA, Forootan HR, Kumar PV. Intestinal neuronal dysplasia as a cause of surgical failure in Hirschsprung’s disease: a new modality for surgical management. J Pediatr Surg. 1996; 31:572–574.

    Article  PubMed  CAS  Google Scholar 

  48. Kapur RP. Hypothesis: Pathogenesis of skip areas in long-segment Hirschsprung disease. Pediatr Pathol. 1900;15:23–37.

    Article  Google Scholar 

  49. Yunis E, Sieber WK, Akers DR. Does zonal aganglionosis really exist? Report of a rare variety of Hirschsprung’s disease and review of the literature. Ped Pathol. 1983;1:33–49.

    Article  CAS  Google Scholar 

  50. Smith W, Gregson N, Foggensteiner L, et al. Acquired intestinal aganglionosis and circulating autoantibodies without neoplasia or other neural involvement. Gastroenterology. 1997; 112:1366–1371.

    Article  PubMed  CAS  Google Scholar 

  51. Vaos GC. Quantitative assessment of the stage of neuronal maturation in the developing human fetal gut-a new dimension in the pathogenesis of developmental anomalies of the myenteric plexus. J Pediatr Surg.1989;24:920–925.

    Article  PubMed  CAS  Google Scholar 

  52. Burns AJ, Le Douarin NM. The sacral neural crest contributes neurons and glia to the post-umbilical gut: spatiotemporal analysis of the development of the enteric nervous system. Development. 1998; 125:4335–4347.

    PubMed  CAS  Google Scholar 

  53. Martin LW, Buchino JJ, LeCoultre C, et al. Hirschsprung’s disease with skip area (segmental aganglionosis). J Pediatr Surg. 1979; 14:686–687.

    Article  PubMed  CAS  Google Scholar 

  54. Neilson IR, Yazbeck S. Ultrashort Hirschsprung’s disease: myth or reality. J Pediatr Surg. 1990;25:1135–1138.

    Article  PubMed  CAS  Google Scholar 

  55. Ballard ET. Ultrashort segment Hirschsprung’s disease: a case report. Pediatr Pathol Lab Med 1996;16:319–325.

    Article  PubMed  CAS  Google Scholar 

  56. Davidson M, Bauer CH. Studies of distal colonic motility in children. IV: Achalasia of the distal rectal segment despite presence of ganglia in the myenteric plexuses of this area. Pediatrics. 1958;21:746–761.

    PubMed  CAS  Google Scholar 

  57. Kapur RP. Hirschsprung disease: pathology and molecular pathogenesis. Adv Pathol Lab Med. 1995;8:201–221.

    Google Scholar 

  58. Stebbing JF. Nitric oxide synthase neurones and neuromuscular behavior of the anorectum. Ann R Coll Surg Eng. 1998;80:137–145.

    CAS  Google Scholar 

  59. Buntzen S, Nordgren S, Hult’en L, et al. The role of nitric oxide in the acetylcholine-induced relaxation of the feline internal anal sphincter, in vitro. Scand J Gastroenterol. 1996;31:1189–1194.

    Article  PubMed  CAS  Google Scholar 

  60. Moore BG, Singaram C, Eckhoff DE, et al. Immunohistochemical evaluations of ultrashort-segment Hirschsprung’s disease. Report of three cases. Dis Colon Rectum. 1996;39:817–822.

    Article  PubMed  CAS  Google Scholar 

  61. Minkes RK, Langer JC. A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung’s disease. J Pediatr Surg. 2000;35:1733–1736.

    Article  PubMed  CAS  Google Scholar 

  62. Krebs C, Acuna R. Transanal internal sphincter myomectomy: indications, operative procedure and results. Eur J Pediatr Surg. 1994;4:151–157.

    Article  PubMed  CAS  Google Scholar 

  63. Kubota M, Ito Y, Taguchi T, et al. Regional differences in the pattern of neurogenic responses in the aganglionic colon from congenitally aganglionic rats. J Pediatr Surg. 1989;24:911–919.

    Article  PubMed  CAS  Google Scholar 

  64. Scharli AF, Sossai R. Hypoganglionosis. Semin Pediatr Surg. 1998;7:187–191.

    PubMed  CAS  Google Scholar 

  65. Shimotake T, Iwai N, Yanagihara J, et al. Biliary tract complications in patients with hypoganglionosis and chronic idiopathic intestinal pseudoobstruction syndrome. J Pediatr Surg. 1993;28: 189–192.

    Article  PubMed  CAS  Google Scholar 

  66. Ure BM, Holschneider AM, Schulten D, et al. Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients. Pediatr Surg Int. 1997;12:377–382.

    Article  PubMed  CAS  Google Scholar 

  67. Krishnamurthy S, Heng Y, Schuffler MD. Chronic intestinal pseudo-obstruction in infants and children caused by diverse abnormalities of the myenteric plexus. Gastroenterology. 1993; 104:1398–1408.

    PubMed  CAS  Google Scholar 

  68. Schuffler MD, Bird TE, Sumi SM, et al. A familial neuronal disease presenting as intestinal pseudoobstruction. Gastroenterology. 1978;75:889–898.

    PubMed  CAS  Google Scholar 

  69. Madsen CM. Hirschsprung’s Disease. Copenhagen: Charles C. Thomas; 1964:245.

    Google Scholar 

  70. Yamataka A, Fujiwara T, Nishiye H, et al. Localization of intestinal pacemaker cells and synapses in the muscle layers of a patient with colonic hypoganglionosis. J Pediatr Surg. 1996;31: 584–587.

    Article  PubMed  CAS  Google Scholar 

  71. Ehrenpreis T. Hirschsprung’s Disease. Chicago: Year Book Medical Publisher; 1970.

    Google Scholar 

  72. Meier-Ruge WA, Brunner LA, Engert J, et al. A correlative morphometric and clinical investigation of hypoganglionosis of the colon in children. Eur J Pediatr Surg. 1999;9:67–74.

    Article  PubMed  CAS  Google Scholar 

  73. Mishalany H, Olson A, Khan F, et al. Deficient neurogenic innervation of the myenteric plexus with normal submucous plexus involving the entire small and large intestine. J Pediatr Surg. 1989;24:83–87.

    Article  PubMed  CAS  Google Scholar 

  74. Parisi M, Kapur RP. Genetics of Hirschsprung disease. Curr Opin Pediatr. 2000;12:610–617.

    Article  PubMed  CAS  Google Scholar 

  75. Stannard VA, Fowler C, Robinson L, et al. Familial Hirschsprung’s disease: report of autosomal dominant and probable recessive X-linked kindreds. J Pediatr Surg. 1991;26:591–594.

    Article  PubMed  CAS  Google Scholar 

  76. Inoue K, Shimotake T, Tomiyama H, et al. Mutational analysis of the RET and GDNF gene in children with hypoganglionosis. Eur J Pediatr Surg. 2001;11:120–123.

    Article  PubMed  CAS  Google Scholar 

  77. Dyer NH, Dawson AM, Smith B, et al. Obstruction of bowel due to lesion in the myenteric plexus. Brit Med J. 1969; 1:688–689.

    Article  Google Scholar 

  78. Costa M, Brookes SJH, Steele PA, et al. Neurochemical classification of myenteric neurons in the guinea-pig ileum. Neuroscience. 1996,75:949–967.

    Article  PubMed  CAS  Google Scholar 

  79. Wheatley JM, Hutson JM, Chow CW, et al. Slow-transit constipation in childhood. J Pediatr Surg. 1999;34:829–833.

    Article  PubMed  CAS  Google Scholar 

  80. Imaji R, Kubota Y, Hengel P, et al. Rectal mucosal biopsy compared with laparoscopic seromuscular biopsy in the diagnosis of intestinal neuronal dysplasia in children with slow-transit constipation. J Pediatr Surg. 2000;35:1724–1727.

    Article  PubMed  CAS  Google Scholar 

  81. FitzPatrick DR, Stain L, Thomas AE, et al. Neurogenic chronic idiopathic intestinal pseudo-obstruction, patent ductus arteriosus, and thrombocytopenia segregating as an X-linked recessive disorder, J Med Genet. 1997;34:666–669.

    Article  PubMed  CAS  Google Scholar 

  82. 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;58:743–748.

    PubMed  CAS  Google Scholar 

  83. Fox JW, Lamperti ED, Eksioglu YZ, et al. Mutations in filamin 1 prevent migration of cerebral cortical neurons in human periventricualr heterotopia. Neuron. 1998;21:1315–1325.

    Article  PubMed  CAS  Google Scholar 

  84. Smith B. Effect of irritant purgatives in the my enteric plexus in man and the mouse. Gut. 1968;9:139–143.

    Article  PubMed  CAS  Google Scholar 

  85. Riemann JF, Schmidt H. Ultrastructural changes in the gut autonomic nervous system following laxative abuse and in other conditions. Scand J Gastroenterol. 1982;17(suppl 71):111–124.

    Google Scholar 

  86. Oster JR, Materson BJ, Rogers AI. Laxative abuse syndrome. Am J Gastroenterol. 1980;74:451–458.

    PubMed  CAS  Google Scholar 

  87. Smith B. Pathologic changes in the colon produced by anthraquinone purgatives. Dis Colon Rectum. 1973; 16:455–458.

    Article  PubMed  CAS  Google Scholar 

  88. Dufour P, Gendre P. Ultrastructure of mouse intestinal mucosa and changes observed after long term anthraquinone administration. Gut. 1984;25:1358–1363.

    Article  PubMed  CAS  Google Scholar 

  89. Dufour P, Gendre P. Long-term mucosal alterations by sennosides and related compounds. Pharmacology. 1988;36 Suppl 1: 194–202.

    Article  PubMed  CAS  Google Scholar 

  90. Heinicke EA, Kiernan JA. Resistance of myenteric neurons in the rat’s colon to depletion by 1,8-dihydroxyanthraquinone. J Pharm Pharmacol 1990;42:123–125.

    Article  PubMed  CAS  Google Scholar 

  91. Dimmick JE, Bove KE. Cytomegalovirus infection of the bowel in infancy: pathogenic and diagnostic significance. Ped Pathol. 1984;2:95–102.

    Article  CAS  Google Scholar 

  92. Hershlag A, Ariel I, Lernau OZ, et al. Cytomegalic inclusion virus and Hirschsprung’s disease. Z Kinderchir. 1984;39:253–254.

    PubMed  CAS  Google Scholar 

  93. Greenfield JG. Infectious diseases of the central nervous system. In: Blackwood W, McMenemey WH, Meyer A, Norman RM, Russell DS, eds. Greenfield’s Neuropathology. Baltimore: Williams and Wilkins Co.; 1966:130–234.

    Google Scholar 

  94. Gesser RM, Koo SC. Oral inoculation with herpes simplex virus type 1 infects enteric neuron and mucosal nerve fibers within the gastrointestinal tract in mice. J Virol. 1996,70:4097–4102.

    PubMed  CAS  Google Scholar 

  95. Todd IP, Porter NH, Morson BC, et al. Chagas disease of the colon and rectum. Gut. 1969;10:1009–1014.

    Article  PubMed  CAS  Google Scholar 

  96. Sterin-Borda L, Goin JC, Bilder CR, et al. Interaction of human chagasic IgG with human colon muscarinic acetylcholine receptor: molecular and functional evidence. Gut. 2001,49:699–705.

    Article  PubMed  CAS  Google Scholar 

  97. Scurry J. Ganglion cells in colonic mucosa. J Paediatr Child Health. 1990;26:280–282.

    Article  PubMed  CAS  Google Scholar 

  98. Berger S, Linke F, Tiling Sv, Hofmann-v. Kap-herr S. The clinical relevance of non-classifiable dysganglionoses and implications for a new grading system. Pediatr Surg Int. 2002; 18:361–367.

    Article  PubMed  CAS  Google Scholar 

  99. Shekitka KM, Sobin LH. Ganglioneuromas of the gastrointestinal tract. Relation to von Recklinghausen disease and other multiple tumor syndromes. Am J Surg Pathol. 1994; 18:250–257.

    Article  PubMed  CAS  Google Scholar 

  100. Kanter AS, Hyman NH, Li SC. Ganglioneuromatous polyposis: a premalignant condition. Dis Colon Rectum. 2001;44:591–593.

    Article  PubMed  CAS  Google Scholar 

  101. Pham BN, Villanueva RP. Ganglioneuromatous proliferation associated with juvenile polyposis coli. Arch Pathol Lab Med. 1989;113:91–94.

    PubMed  CAS  Google Scholar 

  102. Mendelsohn G, Diamond MP. Familial ganglioneuromatous polyposis of the large bowel. Report of a family with associated juvenile polyposis. Am J Surg Pathol. 1984;8:515–520.

    Article  PubMed  CAS  Google Scholar 

  103. Lashner BA, Riddell RH, Winans C. Ganglioneuromatosis of the colon and extensive acanthosis in Cowden’s disease. Dig Dis Sci. 1986;31:213–217.

    Article  PubMed  CAS  Google Scholar 

  104. Weary PE, Gorlin RG, Gentry WC, et al. Multiple hamartoma syndrome (Cowden’s disease). Arch Dermatol. 1972;106:682–690.

    Article  PubMed  CAS  Google Scholar 

  105. d’Amore EG, Manivel JC, Pettinato G, et al. Intestinal ganglioneuromatosis: mucosal and transmural types. Hum Pathol. 1991;22:276–286.

    Article  CAS  Google Scholar 

  106. Wick MJ. Clinical and molecular aspects of multiple endocrine neoplasia. Clin Lab Med. 1997; 17:39–57.

    PubMed  CAS  Google Scholar 

  107. O’Riordain DS, O’Brien T, Crotty TB, et al. Multiple endocrine neoplasia type 2B: more than an endocrine disorder. Surgery. 1995;118:936–942.

    Article  PubMed  Google Scholar 

  108. Kim HR, Kim YJ. Neurofibromatosis of the colon and rectum combined with other manifestations of Reckinghausen’s disease: report of a case. Dis Colon Rectum. 1998,41:1187–1192.

    Article  PubMed  CAS  Google Scholar 

  109. Bahau M, Laurendeau I, Pelet A, et al. Tandem duplication within the neurofibromatosis type 1 gene (NF1) and reciprocal t(15;16)(q26.3;ql2.1) translocation in familial association of NF1 with intestinal neuronal dysplasia type B (IND B). J Med Genet. 2000;37:146–150.

    Article  Google Scholar 

  110. Shulman DI, McClenathan DT, Harmel RP, et al. Ganglioneuromatosis involving the small intestine and pancreas of a child and causing hypersecretion of vasoactive intestinal polypeptide. J Pediatr Gastroenterol Nutr. 1996;22:212–218.

    Article  PubMed  CAS  Google Scholar 

  111. Ritter SA. Neuroblastoma of the intestine. Am J Pathol. 1925;1: 519–526.

    PubMed  CAS  Google Scholar 

  112. Barr H, Page R, Taylor W. Primary small bowel ganglioneuroblastoma and Friedreich’s ataxia. J R Soc Med. 1985,79:612–613.

    Google Scholar 

  113. Kapur RP, Shepard TH. Intestinal ganglioneuroblastoma in a 22-week fetus. Pediatr Pathol. 1992; 12:583–592.

    Article  PubMed  CAS  Google Scholar 

  114. Fadda B, Maier WA, Meier-Ruge W, et al. Neuronale intestinale Dysplasie: Eine kritische 10-Jahres-Analyse klinischer und bioptischer Diagnostik. Z Kinderchir. 1983;38:305–311.

    PubMed  CAS  Google Scholar 

  115. Schofield DE, Yunis EJ. What is intestinal neuronal dysplasia? Pathol Ann. 1992;27:249–262.

    Google Scholar 

  116. Csury L, Pena A. Intestinal neuronal dysplasia: myth or reality? Literature review. Pediatr Surg Int. 1995; 10:441–446.

    Article  Google Scholar 

  117. Lake BD. Intestinal neuronal dysplasia. Why does it only occur in parts of Europe? Virchows Arch. 1995;426:537–539.

    Article  PubMed  CAS  Google Scholar 

  118. Cord-Udy CL, Smith VV, Ahmed S, et al. An evaluation of the role of suction rectal biopsy in the diagnosis of intestinal neuronal dysplasia. J Pediatr Gastwenterol Nutr. 1997;24:1–6.

    Article  CAS  Google Scholar 

  119. Sacher P, Briner J, Hanimann B. Is neuronal intestinal dysplasia (NID) a primary disease or a secondary phenomenon. Eur J Pediatr Surg. 1993;3:228–230.

    Article  PubMed  CAS  Google Scholar 

  120. Berry CL. Intestinal neuronal dysplasia: does it exist or has it been invented? Virchows Arch [A]. 1993;422:183–184.

    Article  CAS  Google Scholar 

  121. Meier-Ruge W. Ueber ein Erkarakungsbild des Kolons mit Hirschsprung Symptomatik. Verh Dtsch Ges Pathol. 1971,55: 506–511.

    PubMed  CAS  Google Scholar 

  122. Borchard F, Meier-Ruge W, Wiebecke B, et al. Innervationsstorungen des Dickdarms-Klassifikation und Diagnostik. Pathologe 1991,12:171–174.

    PubMed  CAS  Google Scholar 

  123. Meier-Ruge WA, Bronnimann PB, Gambassi F, et al. Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B). Virchows Arch. 1995,426:549–556.

    Article  PubMed  CAS  Google Scholar 

  124. Holschneider AM, Meier-Ruge W, Ure BM. Hirschsprung’s disease and allied disorders-a review. Eur J Pediatr Surg. 1994;4: 260–266.

    Article  PubMed  CAS  Google Scholar 

  125. Munakata K, Tomita R, Kurosu Y. Preliminary immunohistochemical new finding in the myenteric plexus of patients with intestinal neuronal dysplasia type B. Eur J Pediatr Surg. 1997;7: 21–29.

    Article  PubMed  CAS  Google Scholar 

  126. Oguzkurt P, Senocak ME, Akcoren Z, et al. Diagnostic difficulties in neuronal intestinal dysplasia and segmental colitis. J Pediatr Surg. 2000;35:519–521.

    Article  PubMed  CAS  Google Scholar 

  127. Wu TK, Wu AS, Tran TA, et al. Neuronal intestinal dysplasia presenting as an abdominal mass. Dis Colon Rectum. 1997;40: 862–865.

    Article  PubMed  CAS  Google Scholar 

  128. Kobayashi H, Hirakawa H, Puri P. What are the diagnostic criteria for intestinal neuronal dysplasia? Pediatr Surg Int. 1995; 10: 459–464.

    Article  Google Scholar 

  129. Lumb PD, Moore L. Are giant ganglia a reliable marker of intestinal neuronal dysplasia type B (IND B)? Virchows Arch. 1998; 432:103–106.

    Article  PubMed  CAS  Google Scholar 

  130. Lumb PD, Moore L. Back to the drawing board. Intestinal neuronal dysplasia type B: not a histological entity yet. Virchows Arch. 1998;432:99–102.

    Article  PubMed  CAS  Google Scholar 

  131. Scharli AF. Neuronal intestinal dysplasia. Pediatr Surg Int. 1992; 7:2–7.

    Google Scholar 

  132. Scharli AF, Meier-Ruge W. Localized and disseminated forms of neuronal intestinal dysplasia mimicking Hirschsprung’s disease. J PS. 1981;16:164–170.

    CAS  Google Scholar 

  133. Ure BM, Holschneider AM, Meier-Ruge W. Neuronal intestinal malformations: a retro-and prospective study on 203 patients. Eur J Pediatr Surg. 1994,4:279–286.

    Article  PubMed  CAS  Google Scholar 

  134. Schmittenbecher PP, Gluck M, Wiebecke B, et al. Clinical longterm follow-up results in intestinal neuronal dysplasia (IND). Eur J Pediatr Surg. 2000; 10:17–22.

    Article  PubMed  CAS  Google Scholar 

  135. Munakata K, Morita K, Okabe I, et al. Clinical and histological studies of neuronal intestinal dysplasia. J Pediatr Surg. 1985;20: 231–235.

    Article  PubMed  CAS  Google Scholar 

  136. Koletzko S, Ballauff A, Hadziselimovic F, et al. Is histological diagnosis of neuronal intestinal dysplasia related to clinical and manometric findings in constipated children? Results of a pilot study. J Pediatr Gastroenterol Nutr. 1993; 17:59–65.

    Article  PubMed  CAS  Google Scholar 

  137. Gillick J, Tazawa H, Puri P. Intestinal neuronal dysplasia: results of treatment in 33 patients. J Pediatr Surg. 2001;36:777–779.

    Article  PubMed  CAS  Google Scholar 

  138. Erdohazi M. Retarded development of the enteric nerve cells. Develop Med Child Neurol. 1974; 16:365–368.

    Article  PubMed  CAS  Google Scholar 

  139. Goebel HH. Extracerebral biopsies in neurodegenerative disease of childhood. Brain Dev. 1999;21:435–443.

    Article  PubMed  CAS  Google Scholar 

  140. Goutieres F, Mikol J, Aicardi J. Neuronal intranuclear inclusion disease in a child: diagnosis by rectal biopsy. Ann Neurol. 1990;27:103–106.

    Article  PubMed  CAS  Google Scholar 

  141. Malandrini A, Fabrizi GM, Cavallaro T, et al. Neuronal intranuclear inclusion disease: polymerase chain reaction and ultrastructural study of rectal biopsy specimen in a new case. Acta Neuropathol (Berl). 1996;91:215–218.

    Article  CAS  Google Scholar 

  142. Barnett JL, McDonnell WM, Appelman HD, et al. Familial visceral neuropathy with neuronal intranuclear inclusions: diagnosis by rectal biopsy. Gastroenterology. 1992; 102:684–691.

    PubMed  CAS  Google Scholar 

  143. Goedert M, Grazia Spillanti M, Davies SW. Filamentous nerve cell inclusions in neurodegenerative diseases. Curr Opin Neurobiol. 1998;8:619–632.

    Article  PubMed  CAS  Google Scholar 

  144. Koyano S, Uchihara T, Fujigasaki H, et al. Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labelling immunofluorescent study. Neurosci Lett. 1999;273:117–120.

    Article  PubMed  CAS  Google Scholar 

  145. Cervera R, Bruix J, Bayes A, et al. Chronic intestinal pseudoobstruction and ophthalmoplegia in a patient with mitochondrial myopathy. Gut. 1988;29:544–547.

    Article  PubMed  CAS  Google Scholar 

  146. Li V, Hostein J, Romero NB, et al. Chronic intestinal pseudoobstruction with myopathy and ophthalmoplegia. A muscular biochemical study of a mitochondrial disorder. Dig Dis Sci. 1992; 37:456–463.

    Article  PubMed  CAS  Google Scholar 

  147. Haftel LT, Lev D, Barash V, et al. Familial mitochondrial intestinal pseudo-obstruction and neurogenic bladder. J Child Neurol. 2000; 15:386–389.

    Article  PubMed  CAS  Google Scholar 

  148. Nishino I, Spinazzola A, Papadimitriou A, et al. Mitochondrial neurogastrointestinal encephalomyopathy: an autosomal recessive disorder due to thymidine phosphorylase mutations. Ann Neurol 2000,47:792–800.

    Article  PubMed  CAS  Google Scholar 

  149. Simon LT, Horoupian DS, Dorfman LJ, et al. Polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestina pseudoobstruction: POLIP syndrome. Ann Neurol. 1990;28:349–360.

    Article  PubMed  CAS  Google Scholar 

  150. Ionasescu V, Thompson SH, Ionasescu R, et al. Inherited ophthalmoplegia with intestinal pseudo-obstruction. J Neurol Sci. 1983;59:215–228.

    Article  PubMed  CAS  Google Scholar 

  151. Matulis SR, Mcjunkin B, Chang H. Familial visceral neuropathy as part of a diffuse neuronal syndrome: four fatal cases in one sibship. Am J Gastroenterol. 1994;89:792–796.

    PubMed  CAS  Google Scholar 

  152. Perez-Atayde AR, Fox V, Teitelbaum JE, et al. Mitochondrial neurogastrointestinal encephalomyopathy. Am J Surg Pathol. 1998;22:1141–1147.

    Article  PubMed  CAS  Google Scholar 

  153. Chow CW, Thorburn DR. Morphological correlates of mitochondrial dysfunction in children. Hum Reprod. 2000; 15Suppl 2:68–78.

    Article  PubMed  Google Scholar 

  154. Nishino I, Spinazzola A, Hirano M. Thymidine phosphorylase gene mutations in MNGIE, a human mitochondrial disorder. Science. 1999;283:689–692.

    Article  PubMed  CAS  Google Scholar 

  155. Baljet B, Drukker J. An acetylcholinesterase method for in toto staining of peripheral nerves. Stain Technol. 1975;50:31–36.

    PubMed  CAS  Google Scholar 

  156. Wells TR, Landing BH, Ariel I, et al. Normal anatomy of the myenteric plexus of infants and children. Perspect Pediatr Pathol. 1987; 11:152–174.

    PubMed  CAS  Google Scholar 

  157. Galvis DA, Nakazato Y, Wells TR, et al. Demonstration of myenteric plexus abnormalities in genetic diseases by a microdissection technique: preliminary studies. Am J Med Genet Suppl. 1987;3:329–342.

    Article  PubMed  CAS  Google Scholar 

  158. Meier-Ruge W. Desmosis of the colon: a working hypothesis of primary chronic constipation. Eur J Pediatr Surg. 1998;8:299–303.

    Article  PubMed  CAS  Google Scholar 

  159. Anderson NE, Hutchinson DO, Nicholson GI, et al. Intestinal pseudo-obstruction, myasthenia gravis, and thymoma. Neurology. 1996,47:985–987.

    Article  PubMed  CAS  Google Scholar 

  160. Chu G, Wilson PC, Cater CD, et al. Intestinal pseudo-obstruction, type 1 anti-neuronal nuclear antibodies, and small-cell carcinoma of the lung. J Gastroenterol Hepatol. 1993;8:604–606.

    Article  PubMed  CAS  Google Scholar 

  161. Condom E, Vidal A, Rota R, et al. Paraneoplastic intestinal pseudo-obstruction associated with high titres of Hu antibodies. Virch Arch A Pathol Anat Histopathol. 1993,423:507–511.

    Article  CAS  Google Scholar 

  162. Lennon VA, Sas DF, Busk MF, et al. Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma. Gastroenterology. 1991; 100:137–142.

    PubMed  CAS  Google Scholar 

  163. Gerl A, Storck M, Schalhorn A, et al. Paraneoplastic chronic intestinal pseudoobstruction as a rare complication of bronchial carcinoid. Gut. 1992;33:1000–1003.

    Article  PubMed  CAS  Google Scholar 

  164. Schobinger-Clement S, Gerber HA, Stallmach T. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction. Am J Surg Pathol. 1999;23:602–606.

    Article  PubMed  CAS  Google Scholar 

  165. De Giorgio R, Bovara M, Barbara G, Canossa M, Sarnelli G, De Ponti F, Stanghellini V, Tonini M, Cappello S, Pagnotta E, No-ile-Orazio E, Corinaldesi R. Anti-HuD-induced neuronal apoptosis underlying paraneoplastic gut dysmotility. Gastroenterol. 2003; 125:70–79.

    Article  Google Scholar 

  166. De Giorgio R, Barbara R, Stanghellini V, et al. Idiopathic myenteric ganglionitis underlying intractable vomiting in a young adult. Eur J Gastroenterol Hepatol. 2000;12:613–616.

    Article  PubMed  Google Scholar 

  167. Briellmann RS, Sturzenegger M, Gerber HA, et al. Autoantibodyassociated sensory neuronopathy and intestinal pseudo-obstruction without detectable neoplasia. Eur Neurol. 1996;36:369–373.

    Article  PubMed  CAS  Google Scholar 

  168. McDonald GB, Schuffler MD, Kadin ME, et al. Intestinal pseudoobstruction caused by diffuse lymphoid infiltration of the small intestine. Gastroenterology. 1985;89:882–889.

    PubMed  CAS  Google Scholar 

  169. Arista-Nasr J, Gonzalez-Romo M, Keirns C, et al. Diffuse lymphoplasmacytic infiltration of the small intestine with damage to the nerve plexus. A cause of intestinal pseudo-obstruction. Arch Pathol Lab Med. 1993,117:812–819.

    PubMed  CAS  Google Scholar 

  170. Ginies J-L, Francois H, Joseph M-G, et al. A curable cause of chronic idiopathic intestinal pseudo-obstruction in children: idiopathic myositis of the small intestine. J Pediatr Gastroenterol Nutr. 1996;23:436–439.

    Google Scholar 

  171. Nezelof C, Vivien F, Bigel P, et al. Idiopathic myositis of the small intestine. An unusual cause of chronic intestinal pseudoobstruction in children. Arch Fr Pediatr. 1985;42:823–828.

    PubMed  CAS  Google Scholar 

  172. Berdon WE, Baker DH, Blanc WA, et al. Megacystis-microcolonintestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. Am J Roentenol. 1976; 126:957–964.

    CAS  Google Scholar 

  173. Vintzileos AM, Eisenfeld LI, Herson VC, et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome: prenatal sonographic findings and review of the literature. Am J Perinatol. 1986;3:297–302.

    Article  PubMed  CAS  Google Scholar 

  174. Ozok G, Erdener A, Herek O, et al. Microcolon-intestinal hypoperistalsis without megacystis: uncommon form of neonatal intestinal pseudoobstruction. Eur J Pediatr Surg. 1995;5:124–127.

    Article  PubMed  CAS  Google Scholar 

  175. Winter RM, Knowles SAS. Megacystis-microcolon-intestinal hypoperistalsis syndrome: confirmation of autosomal recessive inheritance. J Med Genet. 1986;23:360–362.

    Article  PubMed  CAS  Google Scholar 

  176. Anneren G, Meurling S, Olsen L. Megacystis-microcolon-intestinal peristalsis syndrome (MMIHS), an autosomal recessive disorder: clinical reports and review of the literature. Am J Med Genet 1991;41:251–254.

    Article  PubMed  CAS  Google Scholar 

  177. Garber A, Shohat M, Sarti D. Megacystis-microcolon-hypoperistalsis syndrome in two male siblings. Prenat Diagn. 1990; 10: 377–387.

    Article  PubMed  CAS  Google Scholar 

  178. Richardson CE, Morgan JM, Jasani B, et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the alpha-3 nicotinic acetylcholine receptor subunit. Gastroenterol. 2001; 121:350–357.

    Article  CAS  Google Scholar 

  179. Puri P, Lake BD, Gorman F, et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome: a visceral myopathy. J Pediatr Surg. 1983;18:64–69.

    Article  PubMed  CAS  Google Scholar 

  180. Ciftci AO, Cook RCM, van Velzen D. Megacystis microcolon intestinal hypoperistalsis syndrome: evidence of a primary myocellular defect of contractile fiber synthesis. J Pediatr Surg 1996;13:1706–1711.

    Article  Google Scholar 

  181. Kubota M, Ikeda K, Ito Y. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: II. Electrophysiological study. J Pediatr Surg. 1989;24:1267–1270.

    Article  PubMed  CAS  Google Scholar 

  182. Tomomasa T, Itoh Z, Koizumi T, et al. Manometric study on the intestinal motility in a case of megacystis-microcolon-intestinal hypoperistalsis syndrome. J Pediatr Gastroenterol Nutr. 1985;4:307–310.

    Article  PubMed  CAS  Google Scholar 

  183. Taguchi T, Ikeda K, Shono T, et al. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: I. Immunohistochemical study. J Pediatr Surg. 1989;24:1264–1266.

    Article  PubMed  CAS  Google Scholar 

  184. Faulk DL, Anuras S, Gardner GD, et al. A familial visceral myopathy. Ann Int Med. 1978;89:600–606.

    PubMed  CAS  Google Scholar 

  185. Schuffler MD, Pope CE. Studies of idiopathic intestinal pseudoobstruction. II. Hereditary hollow visceral myopathy: family studies. Gastroenterol. 1977;73:339–344.

    CAS  Google Scholar 

  186. Schuffler MD, Lowe MC, Bill AH. Studies of idiopathic intestinal pseudoobstruction. I. Hereditary hollow visceral myopathy: clinical and pathological studies. Gastroenterol. 1977;73:327–338.

    CAS  Google Scholar 

  187. Mitros FA, Schuffler MD, Teja K, et al. Pathologic features of familial visceral myopathy. Hum Pathol. 1982; 13:825–833.

    Article  PubMed  CAS  Google Scholar 

  188. Jacobs E, Ardichvili D, Perissino A, et al. A case of familial visceral myopathy with atrophy and fibrosis of the longitudinal muscle layer of the entire small bowel. Gastroenterol. 1979;77: 745–750.

    CAS  Google Scholar 

  189. Fitzgibbons PL, Chandrasoma PT. Familial visceral myopathy: evidence of diffuse involvement of intestinal smooth muscle. Am J Surg Pathol. 1987;11:846–854.

    Article  PubMed  CAS  Google Scholar 

  190. Schuffler MD, Beegle RG. Progressive systemic sclerosis of the gastrointestinal tract and hereditary hollow visceral myopathy: two distinguishable disorders of intestinal smooth muscle. Gastroenterol. 1979;77:664–671.

    CAS  Google Scholar 

  191. Martin JE, Benson M, Swash M, et al. Myofibroblasts in hollow visceral myopathy: the origin of gastrointestinal fibrosis. Gut. 1993;34:999–1001.

    Article  PubMed  CAS  Google Scholar 

  192. Nowak TV, Ionasescu V, Anuras S. Gastrointestinal manifesta tions of the muscular dystrophies. Gastroenterol. 1982;82:800–810.

    CAS  Google Scholar 

  193. Boland BJ, Silbert PL, Groover RV, et al. Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy. Pediatr Neurol. 1996;14:7–12.

    Article  PubMed  CAS  Google Scholar 

  194. Fuger K, Barnert J, Hopfner W, et al. Intestinal pseudoobstruction as a feature of myotonic muscular dystrophy. Z Gastroenterol. 1995;33:534–538.

    PubMed  CAS  Google Scholar 

  195. Leon SH, Schuffler MD, Kettler M, et al. Chronic intestinal pseudoobstruction as a complication of Duchenne’s muscular dystrophy. Gastroenterol. 1986;90:455–459.

    CAS  Google Scholar 

  196. Laskin BL, Choyke P, Keenan GF, et al. Novel gastrointestinal tract manifestations in juvenile dermatomyositis. J Pediatr Gastroenterol Nutr. 1999; 135:371–374.

    CAS  Google Scholar 

  197. Mansell PI, Tattersall RB, Balsitis M, et al. Megaduodenum due to hollow visceral myopathy successfully managed by duodenoplasty and feeding jejunostomy.Gut. 1991,32:334–337.

    Article  PubMed  CAS  Google Scholar 

  198. Sjogren RW. Gastrointestinal motility disorders in scleroderma. Arthrit Rheumat. 1994;37:1265–1282.

    Article  CAS  Google Scholar 

  199. DeSchryver-Kecskemeti K, Clouse RE. Perineural and intraneural inflammatory infiltrates in the intestines of patients with systemic connective-tissue disease. Arch Pathol Lab Med. 1989; 113:394–398.

    PubMed  CAS  Google Scholar 

  200. Shriver JE. Intestinal obstruction: a neuroanatomic study. Mt Sinai JMed. 1981,48:330–338.

    CAS  Google Scholar 

  201. Gabella G. Size of neurons and glial cells in the intramural ganglia of the hypertrophic intestine of the guinea-pig. J Neurocytol. 1984;12:73–84.

    Article  Google Scholar 

  202. Brehmer A, Gobel D, Frieser M, et al. Experimental hypertrophy of myenteric neurones in the pig: a morphometric study. Neurogastroenterol Motil. 2000;12:155–162.

    Article  PubMed  CAS  Google Scholar 

  203. Jew JY, Williams TH, Gabella G, et al. The intestine as a model for neuronal plasticity. Arch Histol Cytol. 1989;52:167–180.

    Article  PubMed  Google Scholar 

  204. Okada A, Okamoto E. Myenteric plexus in hypertrophied intestine. J Neuro Vise Rel. 1971;32:75–89.

    Article  CAS  Google Scholar 

  205. Cloutier R. Intestinal smooth muscle response to chronic obstruction: possible applications in jejunoileal atresia. J Pediatr Surg. 1975;10:3–8.

    Article  PubMed  CAS  Google Scholar 

  206. Barbosa AJ, Tafuri WL. Ganglion cell number in hypertrophic colon above experimental stenosis. Braz J Med Biol Res. 1983;16:165–169.

    PubMed  CAS  Google Scholar 

  207. Moore CCM, Laing D, Melis J, et al. Secondary effects of prolonged intestinal obstruction on the enteric nervous system in the rat. J Pediatr Surg. 1993;28:1196–1199.

    Article  PubMed  CAS  Google Scholar 

  208. Earlam RJ. Ganglion cell changes in experimental stenosis of the gut. Gut. 1971;12:393–398.

    Article  PubMed  CAS  Google Scholar 

  209. Walker NI, Bennett RE, Axelsen RA. Melanosis coli: a consequence of anthraquinone-induced apoptosis of colonic epithelial cells. Am J Pathol. 1988; 131:465–476.

    PubMed  CAS  Google Scholar 

  210. Byers RJ, Marsh P, Parkinson D, et al. Melanosis coli is associated with an increase in colonic epithelial apoptosis and not with laxative use. Histopathology. 1997;30:160–164.

    Article  PubMed  CAS  Google Scholar 

  211. Walker NI, Smith MM, Smithers BM. Ultrastructure of human melanosis coli with reference to its pathogenesis. Pathology. 1993;25:120–123.

    Article  PubMed  CAS  Google Scholar 

  212. Steer HW, Colin-Jones DG. Melanosis coli: studies of the toxic effects of irritant purgatives. J Pathol. 1975;115:199–205.

    Article  PubMed  CAS  Google Scholar 

  213. Sanders KM. A case for interstitial cells of Cajal as pacemakers and mediators of neurotransmission in the gastrointestinal tract. Gastroenterol. 1996;111:492–515.

    Article  CAS  Google Scholar 

  214. Romert P, Mikkelsen HB. c-kit immunoreactive interstitial cells of Cajal in the human small and large intestine. Histochem Cell Biol. 1998; 109:195–202.

    Article  PubMed  CAS  Google Scholar 

  215. Hagger R, Gharaie S, Finlayson C, et al. Regional and transmural density of interstitial cells of Cajal in human colon and rectum. Am J Physiol. 1998;275:G1309–G1316.

    PubMed  CAS  Google Scholar 

  216. Huizinga JD. Neural injury, repair, and adaptation in the GI tract. IV. Pathophysiology of GI motility related to interstitial cells of Cajal. Am J Physiol. 1998;275:G381–G386.

    PubMed  CAS  Google Scholar 

  217. Ohshiro K, Yamataka A, Kobayashi H, et al. Idiopathic gastric perforation in neonates and abnormal distribution of intestinal pacemaker cells. J Pediati Surg. 2000;35:673–676.

    Article  CAS  Google Scholar 

  218. Isozaki K, Hirota S, Miyagawa MD, et al. Deficiency of c-kit+ cells in patients with a myopathic form of chronic idiopathic intestinal pseudo-obstruction. Am J Gastroenterol. 1997;92:332–334.

    PubMed  CAS  Google Scholar 

  219. Kenny SE, Connell MG, Rintala R, et al. Abnormal colonic interstitial cells of Cajal in children with anorectal malformations. J Pediati Surg. 1998;33:130–132.

    Article  CAS  Google Scholar 

  220. Yamataka A, Ohshiro K, Kobayashi H, et al. Abnormal distribution of intestinal pacemaker (C-KIT-positive) cells in an infant with chronic idiopathic intestinal pseudoobstruction. J Pediati Surg. 1998;33:859–862.

    Article  CAS  Google Scholar 

  221. He C-L, Burgart L, Wang L, et al. Decreased interstitial cell of Cajal volume in patients with slow transit constipation. Gastioenteiol. 2000;118:14–21.

    Article  CAS  Google Scholar 

  222. Faussone-Pelligrini MS, Fociani P, Buffa R, et al. Loss of interstitial cells and a fibromuscular layer on the luminal side of the colonic circular muscle presenting as megacolon in an adult patient. Gut. 1999;45:775–779.

    Article  Google Scholar 

  223. Vanderwinden J-M, Rumessen JJ, Hao L, et al. Interstitial cells of Cajal in human colon and in Hirschsprung disease. Gastioenteiol. 1996;111:901–910.

    Article  CAS  Google Scholar 

  224. Kenny SE, Vanderwinden J-M, Rintala R, et al. Delayed maturation of the interstitial cells of Cajal: a diagnosis for transient neonatal pseudoobstruction. Report of two cases. J Pediati Surg. 1998;33:94–98.

    Article  CAS  Google Scholar 

  225. Smith VV, Milla PJ. Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood. Histopathology. 1997;31:112–122.

    Article  PubMed  CAS  Google Scholar 

  226. Smith W, Lake BD, Kamm MA, et al. Intestinal pseudo-obstruction with deficient smooth muscle a-actin. Histopathology. 1992;21:535–542.

    Article  PubMed  CAS  Google Scholar 

  227. Meier-Ruge W, Morger R, Rehbein F. The hypoganglionated megacolon as accompanying disease of Hirschsprung disease. Z Kinderchir. 1970;8:254–264.

    Google Scholar 

  228. Ikeda K, Goto S, Nagasaki A, et al. Hypogenesis of intestinal ganglion cells: a rare cause of intestinal obstruction simulating aganglionosis. Z Kinderchir. 1988;43:54–55.

    Google Scholar 

  229. Meier-Ruge WA, Scharli AF, Stoss F. How to improve histopathological results in the biopsy diagnosis of gut dysganglionosis. A methodological review. Pediatr Surg Int. 1995; 10:454–458.

    Google Scholar 

  230. Sarnat HB, Seagram GF, Trevenen CL, et al. A fluorochromic stain for nuclei acids to demonstrate submucosal and myenteric neurons in Hirschsprung’s disease. Am J Clin Pathol. 1985;83: 722–725.

    PubMed  CAS  Google Scholar 

  231. Nemeth L, Puri P. Three-dimensional morphology of c-Kitpositive cellular network and nitrergic innervation in the human gut. Arch Pathol Lab Med. 2001;125:899–904.

    PubMed  CAS  Google Scholar 

  232. Porter AJ, Wattchow DA, Brookes SJH, et al. Cholinergic and nitrergic interneurones in the myenteric plexus of the human colon. Gut. 2002;51:70–75.

    Article  PubMed  CAS  Google Scholar 

  233. Kubota M, Yamauchi K, Yonekura T, et al. Clinicopathologic relationship of hypoganglionosis. J Pediatr Surg. 2001;36:898–900.

    Article  PubMed  CAS  Google Scholar 

  234. Meier-Ruge WA, Longo-Bauer CH. Morphometric determination of the methodological criteria for the diagnosis of intestinal neuronal dysplasia (IND B). Pathol Res Pract. 1997; 193:465–469.

    Article  PubMed  CAS  Google Scholar 

  235. Mya GH, Ng WF, Cheng W, et al. Colonic hyperganglionosis presenting as neonatal enterocolitis and multiple strictures. J Pediatr Surg. 1994;29:1628–1630.

    Article  PubMed  CAS  Google Scholar 

  236. Collins MH, Azzarelli B, West KW, et al. Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis. J Pediatr Surg. 1996;31:945–950.

    Article  PubMed  CAS  Google Scholar 

  237. Wildhaber J, Seelentag WKF, Spiegel R, et al. Cystic fibrosis associated with neuronal intestinal dysplasia type B: a case report. J Pediatr Surg. 1996;31:951–954.

    Article  PubMed  CAS  Google Scholar 

  238. Tozzi A, Ascione G, Carpentieri ML, et al. Intestinal neuronal dysplasia associated with cystic fibrosis. Arch Dis Child. 1997; 77:277.

    Article  PubMed  CAS  Google Scholar 

  239. Yunis EJ, Schofield DE. Intestinal neuronal dysplasia in a case of sigmoid stenosis. Pediatr Pathol. 1992; 12:275–280.

    Article  PubMed  CAS  Google Scholar 

  240. Huang C-C, Ko S-F, Chuang J-H, et al. Lipoblastomatosis combined with intestinal neuronal dysplasia. Arch Pathol Lab Med. 1998;122:191–193.

    PubMed  CAS  Google Scholar 

  241. Ekblad E, Sjuve R, Arner A, et al. Enteric neuronal plasticity and a reduced number of interstitial cells of Cajal in hypertrophic rat ileum. Gut. 1998,42:836–844.

    Article  PubMed  CAS  Google Scholar 

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  1. Raj P. Kapur
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Editors and Affiliations

  1. Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, 19104, USA

    Pierre Russo MD (Associate Professor, Director) & Eduardo D. Ruchelli MD (Assistant Professor) (Associate Professor, Director) &  (Assistant Professor)

  2. Division of Anatomic Pathology, The Children’s Hospital of Philadelphia, Philadelphia, PA, 19104, USA

    Pierre Russo MD (Associate Professor, Director) (Associate Professor, Director)

  3. Staff Pathologist, The Children’s Hospital of Philadelphia, Philadelphia, PA, 19104, USA

    Eduardo D. Ruchelli MD (Assistant Professor) (Assistant Professor)

  4. Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, USA

    David A. Piccoli MD (Professor, Division Chief) (Professor, Division Chief)

  5. Gastroenterology and Nutrition, The Children’s Hospital of Philadelphia, Philadelphia, PA, 19104, USA

    David A. Piccoli MD (Professor, Division Chief) (Professor, Division Chief)

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Kapur, R.P. (2004). Motor Disorders. In: Russo, P., Ruchelli, E.D., Piccoli, D.A. (eds) Pathology of Pediatric Gastrointestinal and Liver Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-9066-2_6

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