Advances in Clinical and Experimental Medicine

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Advances in Clinical and Experimental Medicine

2007, vol. 16, nr 2, March-April, p. 309–315

Publication type: review article

Language: Polish

Creative Commons BY-NC-ND 3.0 Open Access

Neuronalna dysplazja jelitowa

Intestinal Neuronal Dysplasia

Anna Piaseczna−Piotrowska1,

1 Department of Pediatric Surgery and Urology, Chair of Pediatric Procedures Medical University in Łódź, Poland Polish Mother’s Memorial Institute and Research Centre, Łódź, Poland

Streszczenie

Neuronalna dysplazja jelitowa (intestinal neuronal dysplasia) jest zaburzeniem dotyczącym śródściennego jelitowego układu nerwowego, którego kliniczne objawy przypominają chorobę Hirschsprunga. Od wielu lat istnieje wiele kontrowersji dotyczących kwestii, czy neuronalna dysplazja jelitowa jest oddzielną jednostką chorobową czy tylko objawem wtórnym powstałym w wyniku przewlekłych zaparć lub procesów zapalnych toczących się w dalszym odcinku jelita grubego. Praca jest przeglądem aktualnej wiedzy i próbą wyjaśnienia niektórych z tych kontrowersji.

Abstract

Intestinal neuronal dysplasia is a malformation of the enteric nervous system, which clinically resemble Hirschsprung’s disease. Many questions have been raised about the existence of intestinal neuronal dysplasia as a distinct clinicopathological entity. This paper is a survey of the present knowledge about intestinal neuronal dysplasia and also an attempt at explaining some of the controversies concerning this entity.

Słowa kluczowe

neuronalna dysplazja jelitowa, dysganglionoza, śródścienny jelitowy układ nerwowy, zaparcia u dzieci

Key words

intestinal neuronal dysplasia, dysganglionosis, enteric nervous system, constipation in childchood

References (50)

  1. Meier−Ruge W: Uber ein Erkrankungsbild des Colon mit Hirschsprung−Symptomatic. Vehr Dtsch Ges Pathol 1971, 55, 506–510.
  2. Fadda B, Maier WA, Meier−Ruge W: Neuronale intestinale Dysplasie: Eine kritische 10−Jahers−Analyse klinischer und bioptischer Diagnostik. Z Kinderchir 1983, 38, 305–311.
  3. Puri P, Lake BD, Nixon HH: Neuronal colonic dysplasia an usual association of Hirschsprung disease. J Pediatr Surg 1977, 12, 681–685.
  4. Schärli AF: Neuronal Intestinal Dysplasia. Pediatr Surg Int 1992, 7, 2–7.
  5. Puri P: Variant Hirschsprung’s Disease. J Pediatr Surg 1997, 32, 149–157.
  6. Puri P, Wester T: Intestinal Neuronal Dysplasia. Semin Pediatr Surg 1998, 7, 181–186.
  7. Martucciello G, Caffarena PE, Lerone M, Mattioli G, Barabiano A, Bisio G, Jassoni V: Neuronal Intestinal Dysplasia: Clinical Experience in Italian Patients. Eur J Pediatr Surg 1994, 4, 287–292.
  8. Schärli AF, Meier−Ruge W: Localized and disseminated forms of neuronal intestinal dysplasia mimicking Hirschsprung’s disease. J Pediatr Surg 1981, 16, 164–170.
  9. Kobayashi H, Hirakawa H, Surana R, O’Briane S, Puri P: Intestinal neuronal dysplasia is possible cause of persistent bowel symptoms after pull through operation for Hirschsprung’s disease. J Pediatr Surg 1992, 30, 253–259.
  10. Meier−Ruge W, Brönninmann PB, Gambazzi F, Schmid PC, Schmidt CP, Stoss F: Histopathological citeria for intestinal neuronal dysplasia of the submucous plexus (Type B). Virchows Arch 1995, 426, 549–556.
  11. Pistor G, Hofmannvon Kap−herr S, Grüssner R, Munakata K, Müntefering H: Modern diagnosis and therapy – raport of 23 patients. Pediatr Surg Int 1987, 2, 352–358.
  12. Rintala R, Rapola J, Louhimo J: Neuronal Intestinal Dysplasia. Progr Pediatr Surg 1989, 24, 186–192.
  13. Moore SW, Kaschula ROC, Cywes S: Familial and genetic aspects of neuronal intestinal dysplasia and Hirschsprung’s disease. Pediatr Surg Int 1993, 8, 406–409.
  14. Feinstat T, Tesluk H, Schuffer MD, Krishnamurthy S, Verlenden L, Gilles W, Frey C, Trudeau W: Megacolon and neurofibromatosis: A neuronal intestinal dysplasia. Gastroenterology 1984, 86, 1573–1579.
  15. Mahaffey SM, Martin LW, McAdams AJ, Ryckman FC, Torres M: Multiple endocrine neoplasia type b with symptoms suggesting Hirschsprung’s disease: a case raport. J Pediatr Surg 1990, 25, 101–103.
  16. Cote GJ, Wohlk N, Evans D, Goepfert H, Gagel RF: RET protooncogene mutations in multiple endocrine neoplasia type 2 and medulary thyroid carcinoma. Baillieres Clin Endocrinol Metab 1995, 9, 609–630.
  17. Angrist M, Kauffman E, Slugenhaupt SA, Matise TC, Puffenberger EG, Washington SS, Lipson A: A gene for Hirschsprung’s disease (megacolon) in the pericentrometric region of human chromosome 10. Nat Genet 1993, 4, 351–356.
  18. Tou JF, Li MJ, Guan T, Li JC, Zhu XK, Feng ZG: Mutation of RET proto−oncogene in Hirschsprung’s disease and intestinal neuronal dysplasia. World J Gastroenterol 2006, 21, 1136–1139.
  19. Hatano M, Aoki T, Dezawa M, Yusa S, Iitsuka Y, Koseki H, Taniguchi M: A Novel Pathogenesis of Megacolon in Ncx/Hox11L.1 Deficient Mice. J Clin Invest 1997, 100, 795–801.
  20. Shirisawa S, Yunker AMR, Roth KA, Brown GA, Horning S, Korsmeyer SJ: ENX (HOX11L1) deficient mice develop myenteric neuronal hyperplasia and megacolon. Nat Med 1997, 3, 646–650.
  21. Van Boyen GB, Krammer HJ, Suss A: Abnormalities of the enteric nervous system in heterozygous endothelin B receptor deficient (spotting lethal) rats resembling intestinal neuronal dysplasia. Gut 2002, 51, 414–419.
  22. Meier−Ruge W: Zur Pathogenese anorektaler Anomalien und Dysganglionosen. In: Anorektale Fahlbilolugen. Eds.: Hofman–Von−kap−herr. Gustav Fisher, New York 1983, 19.
  23. Hirobe S, Doody DP, Ryan DP, Kim SH, Donahoe PK: Ectopic Class II Major Histocompatibility antigens in Hirschsprung’s disease and neuronal intestinal dysplasia. J Pediatr Surg 1992, 27, 357–363.
  24. Csury L, Peña A: Intestinal neuronal dysplasia. Myth or reality? Literature review. Pediatr Surg Int 1995, 10, 441–446.
  25. Meier−Ruge W: Das morphologische Erscheinungsbild der Neurolonalen Dysplasia des Plexus submucosus. Kindrarzt 1990, 21, 837–844.
  26. Borchard F, Meier−Ruge W, Wiebke B, Briner J: Innervationsstörungen des Dickdarms−Klassifikation and Diagnostik. Pathologe 1991, 12, 171–174.
  27. Meier−Ruge W, Gambazzi F, Käufeler RF, Schmid P, Schmidt ChP: The Neuropathological Diagnosis of Neuronal Intestinal Dysplasia (NID B). Eur J Pediatr Surg 1994, 4, 267–273.
  28. Kobayashi H, Hirakawa H, Puri P: Is intestinal neuronal dysplasia a disorder of the neuromuscular junctions? J Pediatr Surg 1996, 4, 575–579.
  29. Miyazaki E, Ohshiro K, Puri P: NADPH−diaphorase histochemical staining of suction rectal biopsies in the diagnosis of Hirschsprung’s disease and allied disorders. Pediatr Surg Int 1998, 13, 464–467.
  30. Bosman C, Devito R, Fisilli S, Boldrini R: A new hypotesis on the patogenesis of intestinal neuronal dysplasia (IND). Pathol Res Pract 2001, 197, 789–796.
  31. Rolle U, Piaseczna−Piotrowska A, Puri P: Abnormal vasculature in intestinal neuronal dysplasia. Pediatr Surg Int 2003, 19, 345–348.
  32. Kobayashi H, Hirakawa H, Puri P: What are the diagnostic criteria for intestinal neuronal dysplasia? Pediatr Surg Int 1995, 10, 459–464.
  33. Nogueira A, Campos M, Soares−Oliveira M, Estevao−Costa J, Silva P, Carneiro F, Carvalho JL: Histochemical and immunohistochemical study of the intrinsic innervation in colonic dysganglionosis. Pediatr Surg Int 2001, 17, 144–151.
  34. Imaji R, Kubota Y, Hengel P, Hutson JM, Chow CW: Rectal mucosal biopsy compared with laparoscopic seromuscular biopsy in the diagnosis of intestinal dysplasia in children with slow−transit constipation. J Pediatr Surg 2000, 35, 1724–1727.
  35. Hutson JM, Chow CW, Borg J: Intractable Constipation with a Decrease Substance P – Immunoreactive Fibres: Is It a Variant of Intestinal Neuronal Dysplasia? J Pediatr Surg 1996, 4, 580–583.
  36. Kobayashi H, Hirakawa H, Puri P: Abnormal internal anal sphincter innervation in patients with Hirschsprung’s disease and allied disorders. J Pediatr Surg 1996, 31, 794–799.
  37. Smith VV: Intestinal neuronal density in childchood: A baseline for the objective assessment of hypoand hyperganglionosis. Pediatr Pathol 1993, 13, 225–237.
  38. Schofield DE, Yunis EJ: What is intestinal neuronal dysplasia. Pathol Ann 1992, 27, 249–262.
  39. Meier−Ruge W, Schmidt PC, Stoss F: Intestinal neuronal Dysplasia and its morphometric evidences. Pediatr Surg Int 1995, 10, 447–453.
  40. Wester T, O’Briain DS, Puri P: The size of ganglia and ganglion cell density in submucosal wholemount preparation of normal human distal colon. J Pediatr Surg 1998, 33, 619–622.
  41. Coerdt W, Michel JS, Rippin G, Kletzki S, Gerein V, Muntefering H, Arnemann J: Quantitative morphometric analisis of the submucous plexus in age−related control group. Virchows Arch 2004, 444, 239–246.
  42. Meier−Ruge W, Ammann K, Bruder E, Holschneider AM, Scharli AF, Schmittenbecher PP, Stoss F: Updated results on intestinal neuronal dysplasia (IND B). Eur J Pediatr Surg 2004, 14, 384–391.
  43. Sacher P, Briner J, Hanimann B: Is neuronal dysplasia (NID) a primary disease or a secondary phenomenon? J Pediatr Surg 1993, 3, 228–230.
  44. Kobayashi H, Yamataka A, Lane GJ, Miyano T: Inflamatory changes secondary to postoperative compications of Hirschsprungs disease as a causeof histopathological changes typical of intestinal neuronal dysplasia. J Pediatr Surg 2004, 39, 152–156.
  45. Simpser E, Kahn E, Kenigsberg K, Duffy L, Markowitz J, Daum F: Neuronal intestinal dysplasia: quantitative diagnostic criteria and clinical management. J Pediatr Gastroenterol Nutr 1991, 12, 61–64.
  46. Moore SW, Laing D, Melis J, Cywes S: Secondary effects of prolonged intestinal obstruction on enteric nervous system in the rat. J Pediatr Surg 1993, 28, 1196–1199.
  47. Lumb PD, Moore L: Are giant ganglia a reliable marker of intestinal neuronal dysplasia B (IND B). Virchow Arch 1991, 432, 103–106.
  48. Martucciello G, Torre M, Pini Prato A, Lerone M, Campus R, Leggio S, Jasonni V: Associated anomalies in intestinal neuronal dysplasia. J Pediatr Surg 2002, 37, 219–223.
  49. Yunis EJ, Schofield DE: Intestinal neuronal dysplasia in a case of sigmoid stenosis. Pediatr Pathol 1992, 12, 275–280.
  50. Schimpl G, Uray E, Ratschek M, Hollwarth ME: Constipation and intestinal neuronal dysplasi type B: a clinical follow−up study. J Pediatr Gastoenterol Nutr 2004, 38, 308–311.