Skip to main content
SpringerLink
Log in

A vascular cause for aganglionic bowel

A new hypothesis

  • Published:
The American Journal of Digestive Diseases Aims and scope Submit manuscript

Abstract

It is suggested that three types of congenital gut lesions; atresia, stenosis and aganglionic bowel, may follow occlusion of the blood supply in utero, the type depending on the duration of ischemia. Interruption of the normal blood supply to the gut may lead to an infarct and the scar tissue narrows the lumen. In utero stenosis and atresia of the small bowel have been produced experimentally with this method. However, temporary ischemia for 4 hours can cause selective destruction of ganglion cells without permanently damaging other components of the gut wall. The same etiologic mechanism, differing only in its severity, could explain the anatomic distribution of atresias, stenoses and aganglionic bowel throughout the whole gut. It is proposed that the rotational strains, which the gut undergoes in its early development, could occlude the main arteries and produce this ischemia.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Barnett WO, Wall I: Megaduodenum resulting from the absence of the parasympathetic ganglion cells in the Auerbach's plexus. Ann Surg 141:525–535, 1965

    Google Scholar 

  2. Bodian M, Carter CO, Ward BCH: Hirschsprung's disease, with radiological observations. Lancet 1:302–309, 1951

    PubMed  Google Scholar 

  3. Bodian M, Carter CO: A family study of Hirschsprung's disease. Ann Hum Genet 26:261–277, 1963

    Google Scholar 

  4. Stone WD, Hendrix TR, Schuster MM: Aganglionosis of the entire colon in an adolescent. Gastroenterology 48:636–641, 1965

    PubMed  Google Scholar 

  5. Walker AW, Kempson RL, Ternberg JL: Aganglionosis of the small intestine. Surgery 60:449–457, 1966

    PubMed  Google Scholar 

  6. Earlam RJ, Zollman PE, Ellis FH Jr: Congenital esophageal achalasia in the dog. Thorax 22:466–472, 1967

    PubMed  Google Scholar 

  7. Derrick EH, St George-Grumbauer BM: Megacolon in mice. J Path Bacteriol 73:569–571, 1957

    Google Scholar 

  8. Earlam RJ, Ellis FH Jr, Nobrega FT: Achalasia of the esophagus in a small urban community. Mayo Clin Proc 44:478–483, 1969

    PubMed  Google Scholar 

  9. Althoff W: Zur Genetik der Hirschsprungen Krankheit. Z Menschl Vererb Konstitutionsl 36:324–340, 1960

    Google Scholar 

  10. Rickham PP, Boeckman CR: Achalasia of the esophagus in young children. Clin Pediat 2:676–680, 1963

    Google Scholar 

  11. Moersch HJ: Cardiospasm in infancy and childhood. Am J Dis Child 38:294–298, 1929

    Google Scholar 

  12. Cameron JAM: Suggested explanation of cardiospasm based on pathological changes. J Laryngol 43:218–221, 1928

    Google Scholar 

  13. Muller E, Perron G, Gerest F, et al: Un cas de mégaoesophage secondaire à une infection à virus neurotope. Bull Mém Soc Méd Paris 63:350–353, 1947

    Google Scholar 

  14. Etzel E: May the disease complex that includes megaesophagus (cardiospasm), megacolon and megaureter be caused by chronic Vitamin B1 deficiency? Am J Med Sci 203:87–100, 1942

    Google Scholar 

  15. Koeberle F: Chagas' disease and Chagas' syndromes: the pathology of American trypanosomiasis. Adv Parasitol 6:63–116, 1968

    PubMed  Google Scholar 

  16. Carveth SW, Schlegel, JF, Code CF, et al: Esophageal motility after vagotomy, phrenicectomy, myotomy and myomectomy in dogs. Surg Gynecol Obstet 114:31–42, 1962

    PubMed  Google Scholar 

  17. Higgs B, Ellis FH Jr: The effect of bilateral supranodosal vagotomy on canine esophageal function. Surgery 58:828–834, 1965

    PubMed  Google Scholar 

  18. Higgs B, Kerr RWL, Ellis FH Jr: The experimental production of esophageal achalasia by electrolytic lesions in the medulla. J Thorac Cardiov Surg 50:613–625, 1965

    PubMed  Google Scholar 

  19. Cassella RR, Brown AL, Sayre GP, et al: Achalasia of the esophagus: pathologic and etiologic considerations. Ann Surg 160:474–486, 1964

    PubMed  Google Scholar 

  20. Cassella RR, Ellis FH Jr, Brown AL: Fine-structure changes in achalasia of the esophagus. Am J Pathol 46:279–288, 1965

    PubMed  Google Scholar 

  21. Lawrentjew BI: Experimentell-morphologische Studien über den feineren Bau des autonomen Nervensystems. Z Mikroanat Forsch 18:233–262, 1929

    Google Scholar 

  22. Stohr P Jr: Ergebnisse über die mikroskopische Innervation des Magen-Darm-Kanals. Ergebn Anat Entwgesch 34:250–401, 1952

    Google Scholar 

  23. Schofield GC: Experimental studies on the myenteric plexus in mammals. J Comp Neurol 119:159–185, 1962

    PubMed  Google Scholar 

  24. Bannister R: Degeneration of the autonomic nervous system. Lancet 2:175–179, 1971

    PubMed  Google Scholar 

  25. Yntema CL, Hammond WS: Experiments on the origin and development of the sacral autonomic nerves in the chick embryo. J Exp Zool 129:375–404, 1955

    Google Scholar 

  26. Leibowitz S, Bodian M: A study of the vesical ganglia in children and the relationship to the megaureter, megacystis syndrome and Hirschsprung's disease. J Clin Pathol 10:342–350, 1963

    Google Scholar 

  27. Cherney LS: Intestinal stenosis following strangulated hernia. Ann Surg 148:991–994, 1958

    PubMed  Google Scholar 

  28. Kessler E, Linden G: Die postischämische Kontraktur des Darmes. Frankfurt Z Pathol 73:363–381, 1964

    Google Scholar 

  29. Barnard C: The genesis of intestinal atresia. Surg Forum 7:393–396, 1956

    Google Scholar 

  30. Earlam RJ: Congenital stenosis of the gut: an experimental study in the chick embryo. (In press) Br J Surg, 1972

  31. Louw JH: Congenital intestinal atresia and stenosis in the newborn. Observations on its pathogenesis and treatment. Ann Roy Coll Surg Eng 25:209–234, 1959

    Google Scholar 

  32. Cannon WB, Burket IR: The endurance of anemia by nerve cells in the myenteric plexus. Am J Physiol 32:347–357, 1913

    Google Scholar 

  33. Okamoto E, Iwasaki T, Ueda T, et al: Pathogenesis of cardiospasm: clinical and experimental studies. Med J Osaka Univ 14:245–261, 1964

    PubMed  Google Scholar 

  34. Hukura T, Siina H, Fukuda H: Effects of destruction of intramural ganglion cells on the motility of the ileocecocolic region. Jap J Physiol 15:648–652, 1965

    Google Scholar 

  35. Earlam RJ, Schlegel JF, Ellis FH Jr: Effect of ischemia of lower esophagus and esophagogastric junction on canine esophageal motor function. J Thorac Cardiov Surg 54:822–831, 1967

    PubMed  Google Scholar 

  36. Szurszewski J, Steggerda FR: The effect of hypoxia on the mechanical activity of the canine small intestine. Am J Digest Dis 13:178–185, 1968

    PubMed  Google Scholar 

  37. Kiesewetter WB, Sukarochana K, Sieber WK: The frequency of aganglionosis with imperforate anus. Surgery 58:877–880, 1965

    PubMed  Google Scholar 

  38. Parkulainen KV, Hjelt L, Sulamaa M: Anal atresia combined with aganglionic megacolon. Acta Chir Scand 118:252–256, 1960

    PubMed  Google Scholar 

  39. Desjardins JG, Stephens CA, Moes CAF: Results of surgical treatment of congenital tracheo-esophageal fistula, with a note on cinefluorographic findings. Ann Surg 160:141–145, 1964

    PubMed  Google Scholar 

  40. Chrispin AR, Freidland GW: A radiological study of the neural control of oesophageal vestibular function. Thorax 21:422–427, 1966

    PubMed  Google Scholar 

  41. Kirkpatrick JA, Cresson SL, Pilling GP IV: The motor activity of the esophagus in association with esophageal atresia and tracheo-esophageal fistula. Am J Roentgenol 86:884–887, 1961

    Google Scholar 

  42. Alnor P: On the pathogenesis of cardiospasm. J Thorac Cardiov Surg 36Z:141–155, 1958

    Google Scholar 

  43. Lendrum FC: Anatomic features of the cardiac orifice of the stomach with special reference to cardiospasm. Arch Int Med 59:474–511, 1937

    Google Scholar 

  44. Harman JW, O'Hagarty MT, Byrnes CK: The ultrastructure of human smooth muscle: 1. Studies of cell surface and connections in normal and achalasia esophageal smooth muscle. Exp Molec Pathol 1:204–228, 1962

    Google Scholar 

  45. Goetz RH: The pathology of progressive systemic sclerosis (generalised scleroderma) with special reference to changes in the viscera. Clin Proc 4:337–392, 1945

    Google Scholar 

  46. Smith EI: The early development of the trachea and esophagus in relation to atresia of the esophagus and tracheo-esophageal fistula. Contributions to Embryology 36:41–57, Washington DC, Carnegie Institute, 1957

    Google Scholar 

  47. Haight C: Some observations on esophageal atresias and tracheo-esophageal fistulas of congenital origin. J Thorac Cardiov Surg 34:141–172, 1957

    Google Scholar 

  48. Lister J: Abnormal arteries in Hirschsprung's disease. Arch Dis Child 41:149, 1966

    Google Scholar 

  49. Ehrenpreis T: Acquired megacolon as a complication of rectosigmoidectomy for Hirschsprung's disease. Arch Dis Child 40:180–182, 1965

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. From the The London Hospital, Whitechapel, London, E. 1

    R. J. Earlam MA, MChir (Cantab) (FRCS)

Authors
  1. R. J. Earlam MA, MChir (Cantab)
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

The experimental work done by the author in support of this hypothesis was performed at the Mayo Clinic working with Dr. F. Henry Ellis, Jr. (Grant NIH AM-5457) and at The London Hospital (Professor Ritchie). This paper represents a shorter version of an essay entitled “Ischaemia—a hypothesis for the genesis of aganglionic bowel”, which won the Isabella Forshall surgery prize in 1968.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Earlam, R.J. A vascular cause for aganglionic bowel. Digest Dis Sci 17, 255–261 (1972). https://doi.org/10.1007/BF02232298

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02232298

Keywords

Search

Navigation