Skip to main content

Hirschsprung’s Disease: Its Aetiology, Pathogenesis and Differential Diagnosis

  • Conference paper
Current Topics in Pathology

Part of the book series: Current Topics in Pathology ((CT PATHOLOGY,volume 59))

Abstract

Congenital aganglionosis of the distal colon was first described as a clinical entity in its own right in 1887 by the Danish physician Harald Hirschsprung. He did not, however, recognise the true cause of the disease, but merely drew attention to the dilated and hypertrophied colon proximal to the aganglionosis. Tittel in 1901 still assumed that Hirschsprung’s disease was due to congenital ectasia of the colon. Dalla-Valle was the first to point out, in papers published in 1920 and 1924, that Hirschsprung’s disease resulted from absence from the distal colon of the myenteric plexus. At the same time Alvarez (1922) showed that the spastic segment of the supra-anal colon was the cause of Hirschsprung’s disease.

Dedicated to Prof. Dr. med. W. Schopper on the occasion of his 75th birthday.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Aaronson, J., Nixon, H. H.: A clinical evaluation of anorectal pressure studies in the diagnosis of Hirschsprung’s disease. Gut 13, 138–146 (1972)

    Article  PubMed  CAS  Google Scholar 

  • Adamson, W. A. D., Aird, J.: Megacolon: Evidence in favour of a neurogenic origin. Brit. J. Surg. 20, 220–233 (1932)

    Article  Google Scholar 

  • Ahmed, S., Cohen, S. J., Jacobs, S. I.: Total intestinal aganglionosis presenting as duodenal obstruction. Arch. Dis. Childh. 46, 868–869 (1971)

    Article  PubMed  CAS  Google Scholar 

  • Albertini, A. Von: Histologische Geschwulstdiagnostik. Stuttgart: Georg Thieme 1955

    Google Scholar 

  • Althoff, W.: Zur Genetik der Hirschsprungschen Krankheit. Z. menschl. Vererb.- u. Konstit.-Lehre 36, 314–340 (1962)

    Google Scholar 

  • Alvarez, W. C.: The mechanics of the digestive tract. New York: P. B. Hoeber Inc., Div. of Harper & Row 1922

    Google Scholar 

  • Auguste, C., Paris, J., Herand, M., Demaille, A.: Rectal localization of Recklinghausen’s disease. Arch. Mai. Appar. dig. 48, 1001–1003 (1959)

    CAS  Google Scholar 

  • Baumgarten, H. G.: Über die Verteilung von Catecholaminen im Darm des Menschen. Z. Zellforsch. 83, 133–146 (1967)

    Article  PubMed  CAS  Google Scholar 

  • Bennett, A., Garrett, J. R., Howard, E. E.: Adrenergic myenteric nerves in Hirschsprung’s disease. Brit. med. J. 1968I, 487–489

    Google Scholar 

  • Bentley, J. F. R.: Some new observations on megacolon in infancy and childhood with special reference to the management of megasigmoid and megarectum. Dis. Colon Rect. 7, 462–470 (1964)

    Article  CAS  Google Scholar 

  • Bentley, J. F. R.: Constipation in infants and children. Gut 12, 85–90 (1971)

    Article  PubMed  CAS  Google Scholar 

  • Berdon, W. E., Koontz, P., Baker, D. H.: Diagnosis of colonic and terminal ileal aganglionosis. Amer. J. Roentgenol. 91, 680–689 (1964)

    PubMed  CAS  Google Scholar 

  • Berger, H.: Das große atonische Colon bei gewissen Obstipationsformen der Kinder und seine Behandlung mit Sympathicolytica. Ann. paediat. 178, 187–206 (1952)

    Google Scholar 

  • Bodian, M.: Chronic constipation in children. Practitioner 169, 517–529 (1952)

    PubMed  CAS  Google Scholar 

  • Bodian, M., Carter, C. O.: A family study of Hirschsprung’s disease. Ann. hum. Genet. 26, 261–277 (1963)

    Article  Google Scholar 

  • Bodian, M., Carter, C. O., Ward, B. C.: Hirschsprung’s disease. Lancet 1951I, 302–309

    Google Scholar 

  • Bodian, M., Stephens, F. D., Ward, B. C. H.: Hirschsprung’s disease and idiopathic megacolon. Lancet 1949I, 6–11

    Google Scholar 

  • Boggs, J. D., Kidd, J. M.: Congenital abnormalities of intestinal innervation; absence of innervation of jejunum, ileum and colon in siblings. Pediatrics 21, 261–265 (1958)

    PubMed  CAS  Google Scholar 

  • Bülbring, E.: Smooth muscle of the alimentary tract. In: Modern trends in gastroenterology, edit, by F. Avery Jones. New York: Hoeber 1958

    Google Scholar 

  • Campbell, P. E., Noblett, H. R.: Experience with rectal suction biopsy in the diagnosis of Hirschsprung’s disease. J. Pediat. Surg. 4, 410–415 (1969)

    Article  PubMed  CAS  Google Scholar 

  • Cannon, W. B.: A Law of denervation. Amer. J. med. Sci. 198, 737–750 (1939)

    Article  Google Scholar 

  • Chalkley, T., Bruce, J. W.: Neurofibromatosis of colon, small intestine and mesentery in child. Amer. J. Dis. Childh. 64, 888–894 (1942)

    Google Scholar 

  • Connell, A.M.: Colonic motility in Megacolon. Proc. roy. Soc. Med. 54, 1040–1043 (1961)

    Google Scholar 

  • Connell, A. M.: Problems of methodology and interpretation and analysis of motility records. Amer. J. dig. Dis. 13, 397–409 (1968)

    Article  PubMed  CAS  Google Scholar 

  • Connell, A.M.: Methodology of investigations of alimentary motility. In: Gastrointestinal motility. Edit.: L. Demling and R. Oettenjann, p. 1–6. Stuttgart und New York-London: Academic Press 1971

    Google Scholar 

  • Costa, M., Gabella, G.: Adrenergic innervation of the alimentary canal. Z. Zellforsch. 122, 357–377 (1971)

    Article  PubMed  CAS  Google Scholar 

  • Dahl, E. V., Waugh, J. M., Dahlin, D. C.: Gastrointestinal ganglioneuromas. Amer. J. Path. 33, 953–965 (1957)

    PubMed  CAS  Google Scholar 

  • Dalla-Valle, A.: Ricerche istologiche su di un caso di megacolon congenito. Pediatria (Barcelona) 28, 740–752 (1920)

    Google Scholar 

  • Dalla-Valle, A.: Contributo alia conoscenza della forma famigliare del megacolon congenito. Pediatria (Barcelona) 32, 569–599 (1924)

    Google Scholar 

  • Davenport, H. W.: Physiology of the digestive tract. Chicago: Year Book Publ. 1961 ]

    Google Scholar 

  • Davidson, M., Sleisenger, M. H., Steinberg, H., Almy, TH. P.: Studies of distal colon motility in children. III. The pathologic physiology of congenital megacolon (Hirschsprung’s disease). Gastroenterology 29, 803–823 (1955)

    PubMed  CAS  Google Scholar 

  • Dobbins, W.O., Bill, A. H.: Diagnosis of Hirschsprung’s disease excluded by rectal suction biopsy. New Engl. J. Med. 272, 990–993 (1965)

    Google Scholar 

  • Ehrenpreis, T.: Megacolon in the newborn. A clinical and roentgenological study with special regard to the pathogenesis. Acta chir. scand. 94, Suppl. 112, 1–114 (1946)

    Google Scholar 

  • Ehrenpreis, T.: Hirschsprung’s disease in the neonatal period. Arch. Dis. Childh. 30, 8–12 (1955)

    Article  PubMed  CAS  Google Scholar 

  • Ehrenpreis, T.: Pseudo-Hirschsprung’s disease. Arch. Dis. Childh. 40, 177–179 (1965a)

    Article  PubMed  CAS  Google Scholar 

  • Ehrenpreis, T.: Acquired megacolon as a complication of rectosigmoid ectomy for Hirschsprung’s disease. Arch. Dis. Childh. 40, 180–182 (1965b)

    Article  PubMed  CAS  Google Scholar 

  • Ehrenpreis, T.: Some newer aspects on Hirschsprung’s disease and allied disorders. J. Pediatr. Surg. 1, 329–337 (1966)

    Article  PubMed  CAS  Google Scholar 

  • Ehrenpreis, T.: Megacolon and megarectum in older children and young adults. Classification and Terminology. Proc. roy. Soc. Med. 60, 799–801 (1967)

    PubMed  CAS  Google Scholar 

  • Ehrenpreis, T.: Hirschsprung’s disease. Chicago: Year Book Medical Publ. Inc. 1970

    Google Scholar 

  • Ehrenpreis, T., Norberg, K.-A., Wirsen, C.: Sympathetic innervation of the colon in Hirschsprung’s disease: A histochemical study. J. Pediat. Surg. 3, 43–49 (1968)

    Article  Google Scholar 

  • Emanuel, B., Padorr, M. P., Swenson, D.: Familial absence of myenteric plexus (Congenital megacolon). J. Pediat. 67, 381–386 (1965)

    Article  PubMed  CAS  Google Scholar 

  • Emery, J. L., Finch, E., Lister, J.: Use of circumferential tangential cryostat sections of the intestine in the diagnosis of Hirschsprung’s disease. J. clin. Path. 20, 263–266 (1967)

    Article  PubMed  CAS  Google Scholar 

  • Enemar, A., Falck, B., Hakanson, R.: Observation on the appearance of norepinephrine in the sympathetic nervous system of chick embryo. Develop. Biol. 11, 268–283 (1965)

    Article  PubMed  CAS  Google Scholar 

  • Ferreira-Santos, R.: Megacolon and megarectum in Chagas’ disease. Proc. roy. Soc. Med. 54, 1047–1053 (1961)

    PubMed  CAS  Google Scholar 

  • Frech, R. S.: Aganglionosis involving the entire colon and a variable length of small bowel. Radiology 90, 249–257 (1968)

    Google Scholar 

  • Freeman, N. V.: Long-segment Hirschsprung’s disease. Proc. roy. Soc. Med. 64, 378–380 (1971)

    CAS  Google Scholar 

  • Gabella, G., Costa, M.: Adrenergic innervation of the intestinal musculature. Experientia (Basel) 25, 395–396 (1969)

    Article  CAS  Google Scholar 

  • Gannon, B. J., Burnstock, G., Noblett, H. R., Campbell, P. E.: Histochemical diagnosis of Hirschsprung’s disease. Lancet 1969Ia, 894–895

    Google Scholar 

  • Gannon, B. J., Noblett, H. R., Burnstock, G.: Adrenergic innervation of bowel in Hirschsprung’s disease. Brit. med. J. 1969IIb, 338–340

    Google Scholar 

  • Garrett, J. R., Howard, E. R.: Histochemistry and the pathology of Hirschsprung’s disease. Proc. roy. micr. Soc. 4, 76–78 (1969)

    Google Scholar 

  • Garrett, J. R., Howard, E. R., Nixon, H. H.: Autonomic nerves in rectum and colon in Hirschsprung’s disease. Arch. Dis. Childh. 44, 406–417 (1969a)

    Article  PubMed  CAS  Google Scholar 

  • Garrett, J. R., Howard, E. R., Nixon, H. H.: Histochemical diagnosis of Hirschsprung’s disease. Lancet 1969IIB, 436

    Google Scholar 

  • Garrett, J. R., Howard, E. R., Nixon, H. H.: Bowel innervation in Hirschsprung’s disease. Brit. med. J. 1969IIIc, 718–719

    Google Scholar 

  • Gerald, B.: Aganglionosis of the colon and terminal ileum. Longterm survival. Amer. J. Roentgenol. 95, 230–234 (1965)

    PubMed  CAS  Google Scholar 

  • Gertz, T. C., Hansen, L. K: Mb. Hirschsprung hos voksne. Nord. Med. 75, 325–328 (1966)

    Google Scholar 

  • Gherardi, G. J.: Pathology of the ganglionic-aganglionic junction in congenital megacolon. Arch. Path. (Chic.) 69, 520–523 (I960)

    Google Scholar 

  • Gillis, D. A., Grantmyre, E. B.: The meconium-plug syndrome and Hirschsprung’s disease. Canad. med. Ass. J. 92, 225–227 (1965)

    CAS  Google Scholar 

  • Goerttler, K.: Der konstruktive Bau der menschlichen Darmwand. Gegenbaurs morph. Jb. 69, 329–379 (1932)

    Google Scholar 

  • Goerttler, K.: Der Bau der,,Muscularis mucosae” des menschlichen Darmes und ein Befund über den Bau seiner,,Muscularis propria”. Gegenbaurs morph. Jb. 90, 33–58 (1951)

    Google Scholar 

  • Gordon, LI., Torrington, M., Louw, J. H., Cywes, S.: A genetic study of Hirschsprung’s disease: congenital intestinal aganglionosis. S. Afr. med. J. 40, 720–721 (1966)

    PubMed  CAS  Google Scholar 

  • Graivier, L., Sieber, W. K.: Hirschsprung’s disease and mongolism. Surgery 60, 458–461 (1966)

    PubMed  CAS  Google Scholar 

  • Grodsky, L.: Neurofibroma of rectum in patient with von Recklinghausen’s disease. Amer. J. Surg. 95, 474–476 (1958)

    Article  PubMed  CAS  Google Scholar 

  • Hamberger, B., Norberg, K.-A.: Studies on some systems of adrenergic synaptic terminals in the abdominal ganglia of the cat. Acta physiol. scand. 65, 235–242 (1965)

    Article  PubMed  CAS  Google Scholar 

  • Henley, F. A.: Pelvic colectomy for obstinate constipitation. Proc. roy. Soc. Med. 60, 806–807 (1967)

    PubMed  CAS  Google Scholar 

  • Hermann, R. E., Izant, R. J., Jr., and Bolande, R. P.: Aganglionosis of the intestine in siblings. Surgery 53, 664–669 (1963)

    Google Scholar 

  • Herzog, B.: Zur Hirschsprungschen Krankheit mit total aganglionärem Colon (Megacolon congenitum ohne Megacolon). Helv. chir. Acta 34, 446–449 (1967)

    PubMed  CAS  Google Scholar 

  • Hess, R., Scarpelli, D. G., Pearse, A. G. E.: The cytochemical localization of oxidative enzymes. II. Pyridine nucleotide-linked dehydrogenases. J. biophys. biochem. Cytol. 4, 753–760 (1958)

    Article  PubMed  CAS  Google Scholar 

  • Hiatt, R. B.: The pathologic physiology of congenital megacolon. Ann. Surg. 133, 313–320 (1951)

    Article  PubMed  CAS  Google Scholar 

  • Hiatt, R. B., Hinderstein, C. S., Moore, A.: Development of neural control of alimentary motor function in the vertebrates. J. Surg. Res. 8, 286–292 (1968)

    Article  PubMed  CAS  Google Scholar 

  • Hill, C. J.: A contribution to our knowledge of the enteric plexuses. Phil. Trans. B 215, 355–387 (1927)

    Article  Google Scholar 

  • Hirschsprung, H.: Stuhlträgheit Neugeborener in Folge von Dilatation und Hypertrophie des Colon. Jb. Kinderheilk. 27, 1–7 (1888)

    Google Scholar 

  • Hofmann, S., Rehbein, F.: Hirschsprungsche Krankheit im Neugeborenenalter. Z. Kinderchir. 3, 182–194 (1966)

    Google Scholar 

  • Howard, E. R., Garrett, J. R.: Histochemistry and electron microscopy of rectum and colon in Hirschsprung’s disease. Proc. roy. Soc. Med. 63, 1264–1266 (1970)

    PubMed  CAS  Google Scholar 

  • Howard, E. R., Garrett, J. R.: Electron microscopy of myenteric nerves in Hirsch-sprung’s disease and in normal bowel. Gut 11, 1007–1014 (1970)

    Article  PubMed  CAS  Google Scholar 

  • Hüther, W.: Die Hirschsprungsche Krankheit als Folge einer Entwicklungsstörung der intramuralen Ganglien. Beitr. path. Anat. 114, 161–191 (1954)

    Google Scholar 

  • Hukuhara, T., Nakayama, S., Sumi, T.: The role of the intrinsic mucosal reflex in the fluid transport through the denervated intestinal loop. Jap. J. Physiol. 9, 406–418 (1959)

    Article  CAS  Google Scholar 

  • Hukuhara, T., Sumi, T., Kotani, S.: The role of the ganglion cells in the small intestine taken in the intestinal intrinsic reflex. Jap. J. Physiol. 11, 281–288 (1961)

    Article  CAS  Google Scholar 

  • Hunziker, O., Del Pozo, E., Wiesinger, D., Meier-Ruge, W.: Die Anwendung des Leitz-Classimat in der quantitativen Histomorphologie und in der experimentellen Pathologie. Leitz Mitt. Wiss. und Techn. 5, 51–55 (1972)

    Google Scholar 

  • Hupe, K., Schlosser, V.: Rektoskopische Probeexzision in der Diagnostik des Megacolon congenitum. Dtsch. med. Wschr. 87, 1155–1156 (1962)

    Article  PubMed  CAS  Google Scholar 

  • Irwin, D. A.: The anatomy of Auerbach’s plexus. Amer. J. Anat. 49, 141–166 (1931)

    Article  Google Scholar 

  • Ishikawa, N.: Experimentelle und klinische Untersuchungen über die Pathogenese und das Wesen des Megacolon. Mitt. med. Fak. kgl. Univ. Kyushu Fukuoka 7, 339–400 (1923)

    Google Scholar 

  • Jennings, P. J.: Megarectum and megacolon in adolescents and young adults: Results of treatment at St. Mark’s Hospital. Proc. roy. Soc. Med. 60, 805–806 (1967)

    PubMed  CAS  Google Scholar 

  • Jones, D. S.: The origin of the vagi and the parasympathetic ganglion cells of the viscera of the chick. Anat. Ree. 82, 185–194 (1942)

    Article  Google Scholar 

  • Kamijo, K., Hiatt, R. B., Koelle, F. B.: Congenital megacolon. A comparison of the spastic and hypertrophied segments with respect to Cholinesterase activities and sensitivities to acetylcholine, DFP and the barium ion. Gastroenterology 24, 173–185 (1953)

    PubMed  CAS  Google Scholar 

  • Karnovsky, M. J., Roots, L.: A “direct-coloring” thiocholine method for cholinesterases. J. Histochem. Cytochem. 12, 219–221 (1964)

    Article  PubMed  CAS  Google Scholar 

  • Katz, A.: Pseudo-Hirschsprung’s disease in Bantu children. In: Seminar on Pseudo-Hirschsprung’s disease and related disorders. Arch. Dis. Childh. 41, 152 (1966)

    Google Scholar 

  • Keefer, G. P., Mokrohisky, J. F.: Congenital megacolon (Hirschsprung’s disease). Radiology 63, 157–175 (1954)

    PubMed  CAS  Google Scholar 

  • Kleinschmidt, O.: Ätiologie des Megacolon. Langenbecks Arch. klin. Chir. 142, 91–94 (1926)

    Google Scholar 

  • Köberle, F.: Die Chagaskrankheit. Eine Erkrankung der neuro vegetativen Peripherie. Wien. klin. Wschr. 68, 333–339 (1956)

    PubMed  Google Scholar 

  • Köberle, F.: Die Chagaskrankheit, ihre Pathogenese und ihre Bedeutung als Volksseuche. Z. Tropenmed. Parasit. 10, 236–268 (1959)

    Google Scholar 

  • Koelle, G. B.: Autonomie and pharmacologic control of colonic activity. Ann. N.Y. Acad. Sci. 58, 307–315 (1954)

    Article  Google Scholar 

  • Koelle, G. B., Valk, A. De T.: Physiological implications of the histochemical localization of monoamine oxidase. J. Physiol. (Lond.) 126, 434–447 (1954)

    CAS  Google Scholar 

  • Kottmeier, P. K., Clatworthy, H.W.: Aganglionic and functional megacolon in children—a diagnostic dilemma. Pediatrics 36, 572–582 (1965)

    PubMed  CAS  Google Scholar 

  • Kuntz, A.: The development of the sympathetic nervous system in man. J. comp. Neurol. 32, 173–229 (1920)

    Article  Google Scholar 

  • Kuntz, A.: Visceral innervation and its relation to personality. Springfield: Ch. C. Thomas 1951

    Google Scholar 

  • Lassmann, G.: Histochemische Darstellung der spezifischen Cholinesterase (Acetylcholinesterase) in den nervösen Formationen bei einem Fall von neurogener Appendicopathie mit zentraler Neurombildung. Acta neuropath. 1, 308–310 (1961)

    Article  Google Scholar 

  • Lassmann, G.: Hirschsprungsche Erkrankung. Neurohistologische Untersuchung. Pädiat. Pädol., Suppl 2, 65–70 (1972)

    Google Scholar 

  • Lee, C. M., Jr.: Megacolon, with particular reference to Hirschsprungss disease. Surgery 37, 762–777 (1955)

    PubMed  Google Scholar 

  • Leibowitz, S., Bodian, M.: A study of the versical ganglia in children and the relationship to the megaureter megacystis syndrom and Hirschsprung’s disease. J. clin. Path. 16, 342–350 (1963)

    Article  PubMed  CAS  Google Scholar 

  • Lenz, H.: Zur Physiologie der Peristaltik des Dünndarms. Dtsch. med. Wschr. 90, 1657–1663 (1965)

    Article  PubMed  CAS  Google Scholar 

  • Lichtenstein, B.W., Ragins, A.B.: Ganglioneuroma of the vermiform appendix. Arch. Path. 24, 786–790 (1937)

    Google Scholar 

  • Madsen, C. M.: Hirschsprung’s disease. Congenital intestinal aganglionosis. Copenhagen: Munksgaard 1964

    Google Scholar 

  • Mahnke, P. F.: Das Megacolon congenitum aus pathologisch-anatomischer Sicht unter Berücksichtigung des diagnostischen Wertes der Rektumbiopsie. Dtsch. Gesundh.- Wes. 22, 1280–1282 (1967)

    CAS  Google Scholar 

  • Mainguet, P., Dehon, Y.: Essai de traitement de mégacôlons et dolichocôlons fonctionnels par le méthanesulfonate de dihydroergotamine résultats obtenus dans 31 cas. Sem. Hôp. Paris 40, 415–419 (1964)

    CAS  Google Scholar 

  • Marshak, R. H., Lester, L. J., Friedman, A. J.: Megacolon, a complication of ulcerative colitis. Gastroenterology 16, 768–772 (1950)

    PubMed  CAS  Google Scholar 

  • McElhannon, F. M.: Experimental megacolon resembling Hirschsprung’s disease. Surg. Forum 10, 218–221 (1960)

    Google Scholar 

  • McInernney, G. T., Sauer, W. G., Baggenstoss, A. H., Hodgson, J. R.: Fulminating ulcerative colitis with marked colonic dilation: A clinicopathologic study. Gastroenterology 42, 244–257 (1962)

    Google Scholar 

  • McIver, A. G., Whitehead, R.: Zonal colonic aganglionosis: Variant of Hirschsprung’s Disease. Arch. Dis. Childh. 47, 233–237 (1972)

    Article  Google Scholar 

  • Meier-Ruge, W.: Das Megacolon, seine Diagnose und Pathophysiologie. Virchows Arch. Abt. A 344, 67–85 (1968a)

    Article  CAS  Google Scholar 

  • Meier-Ruge, W.: Experimentelle Untersuchungen zur Pathogenese des konnatalen Megaureter. Schweiz, med. Wschr. 98, 1122 (1968b)

    Google Scholar 

  • Meier-Ruge, W.: The pathology of megacolon. In “Progress in proctology”. Proc. of the 3rd Internat. Congr. of Hedrologicum Conlegium. Edit.: J. Hoferichter, p. 72–77. Berlin-Heidelberg-New York: Springer 1969a

    Google Scholar 

  • Meier-Ruge, W.: Beitrag zur Pathologie des hypoganglionären Megacolon. Verh. dtsch. Ges. Path. 53, 237–239 (1969b)

    CAS  Google Scholar 

  • Meier-Ruge, W.: Über ein Erkrankungsbild des Colon mit Hirschsprung-Symptomatik. Verh. dtsch. Ges. Path. 55, 506–510 (1971)

    CAS  Google Scholar 

  • Meier-Ruge, Wt.: Fortschritte in der Diagnostik des aganglionären Segments. Pädiat. Pädiol., Suppl. 2, 55–64 (1972)

    Google Scholar 

  • Meier-Ruge, W.: Fortschritte der morphologischen Diagnostik des Morbus Hirschsprung. Pädiat. Fortbildk. Praxis (Basel) 36, 80–97 (1973).

    Google Scholar 

  • Meier-Ruge, W., Bielser, W., Jr., Wiederhold, K. H., Meyenhofer, M.: Incubation media for routine laboratory work on enzyme histotopochemistry. Beitr. Path. 144, 409–431 (1971)

    Google Scholar 

  • Meier-Ruge, W., Hunziker, O.: Zur Histotopochemie der totalen Aganglionose des Colon (Zuelzer-Wilson-Syndrom). Schweiz, med. Wschr. 102, 817 (1972a)

    Google Scholar 

  • Meier-Ruge, W., Hunziker, O.: Pathophysiology of the extrinsic parasympathetic nerves of the descending colon and the rectosigmoid. Scand. J. Gastroent., Suppl. 17, 11 (1972b)

    Google Scholar 

  • Meier-Ruge, W., Hunziker, O., Tobler, H.-J., Walliser, CH.: 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. 147, 228–236 (1972a)

    Article  CAS  Google Scholar 

  • Meier-Ruge, W., Lutterbeck, P. M., Herzog, B., Morger, R., Moser, R., Schärli, A.: Acetylcholinesterase activity in rectum suction biopsies as diagnostic in Hirschsprung’s disease. J. Pediat. Surg. 7, 11–17 (1972b)

    Article  PubMed  CAS  Google Scholar 

  • Meier-Ruge, W., Morger, R.: Neue Gesichtspunkte zur Pathogenese und Klinik des Morbus Hirschsprung. Schweiz, med. Wschr. 98, 7, 209–214 (1968)

    CAS  Google Scholar 

  • Meier-Ruge, W., Morger, R., Rehbein, F.: Das hypoganglionäre Megacolon als Begleitkrankheit bei Morbus Hirschsprung. Z. Kinderchir. 8, 254–264 (1970)

    Google Scholar 

  • Morger, R.: Surgery of megacolon. In: Progress in proctology. Hrsg.: J. Hoferichter, p. 78–81. Berlin-Heidelberg-New York: Springer 1969

    Google Scholar 

  • Morger, R.: Probleme bei der Diagnostik des Megacolon congenitum und ihre Konsequenzen für die operative Therapie. Langenbecks Arch. klin. Chir. 327, 622 (1970)

    Article  CAS  Google Scholar 

  • Morger, R.: Zur Bedeutung der rectalen Saugbiopsie beim aganglionären Segment. Pädiat. Pädol., Suppl. 2, 48–54 (1972)

    Google Scholar 

  • Morger, R., Gloor, F.: Spontanperforation im Colon descendens bei einem Neugeborenen mit Megacolon congenitum (Hirschsprung). Praxis 52, 543–545 (1963)

    Google Scholar 

  • Morger, R., Meier-Ruge, W.: Morbus Hirschsprung. Neue Gesichtspunkte zur Pathogenese und Klinik. Sandorama, Nov. 4–7 (1968)

    Google Scholar 

  • Moser, R., Widmer, A., Meier-Ruge, W.: Zur enzymhistochemischen Diagnostik des Morbus Hirschsprung. Schweiz, med. Wschr. 101, 109–115 (1971)

    CAS  Google Scholar 

  • Myers, M. B., Bradburn, D., Vela, R., Paiyzant, A., Karlin, S.: Total aganglionic colon in an adult: first reported case. Ann. Surg. 163, 97–102 (1966)

    Article  PubMed  CAS  Google Scholar 

  • Nelson, M. M.: Teratogenic effects of pteroylglutamic acid deficiency in the rat. Ed.: G. E. W. Wolstenholme and C. M. O’Connor. Ciba Foundation Symposium “Congenital Malformations”. London: J. & A. Churchill 1960

    Google Scholar 

  • Niemi, M., Kouvalainen, K., Hjelt, L.: Cholinesterase and monoamine oxidase in congenital megacolon. J. Path. Bact. 82, 363–366 (1961)

    Article  PubMed  CAS  Google Scholar 

  • Nixon, H. H.: What is Pseudo-Hirschsprung’s disease. Arch. Dis. Childh. 41, 147–149 (1966)

    Google Scholar 

  • Nixon, H.H.: Progress in Hirschsprung’s disease. In: Irvine, W. T. (ed.), Modern trends in surgery. Vol. 2. London: Butterworth & Co. 1966

    Google Scholar 

  • Nixon, H. H.: Megarectum in the older child. Proc. roy. Soc. Med. 60, 801–803 (1967)

    PubMed  CAS  Google Scholar 

  • Nixon, H. H.: Hirschsprung’s disease. Brit. J. Hosp. Med. 5, 199–209 (1971)

    Google Scholar 

  • Nixon, H.H.: Problems in the diagnosis of Hirschsprung’s disease. Pädiat. Pädol., Suppl. 2, 21–31 (1972)

    Google Scholar 

  • Noblett, H. R.: A rectal suction tube for use in the diagnosis of Hirschsprung’s disease. J. Pediat. Surg. 4, 406–409 (1969)

    Article  PubMed  CAS  Google Scholar 

  • Norberg, K.-A.: Adrenergic innervation of the intestinal wall studied by fluorescence microscopy. Int. J. Neuropharmacol. 3, 379–382 (1964)

    Article  PubMed  CAS  Google Scholar 

  • Norberg, K.-A.: Transmitter histochemistry of the sympathetic adrenergic nervous system. Brain Res. 5, 125–170 (1967)

    Article  PubMed  CAS  Google Scholar 

  • Okamoto, E., Iwasaki, T., Kakutani, T., Ueda, T.: Selective destruction of the myenteric plexus: Its relation to Hirschsprung’s disease, achalasia of the esophagus and hypertrophic pyloric stenosis. J. Pediatr. Surg. 2, 444–454 (1967)

    Article  Google Scholar 

  • Okamoto, E., Ueda, T.: Embryogenesis of intramural ganglia of the gut and its relation to Hirschsprung’s disease. J. Pediat. Surg. 2, 437–443 (1967)

    Article  Google Scholar 

  • Pages, R., Duhamel, B.: Intrinsic non-propulsive colon. Arch. Dis. Childh. 41, 151 (1966)

    Google Scholar 

  • Passarge, E.: The genetics of Hirschsprung’s disease: evidence for heterogeneous etiology and a study of sixty-three families. New Engl. J. Med. 276, 138–143 (1967)

    Article  PubMed  CAS  Google Scholar 

  • Potts, W. J.: Discussion on papers by Dr. Riker and Drs. Sieber and Gird Any. Arch. Surg. 75, 395–396 (1957)

    Google Scholar 

  • Rehbein, F., Halsband, H., Hofmann, S.: Hirschsprungsche Krankheit mit langem, engem Segment. Dtsch. med. Wschr. 94, 708–716 (1969)

    Article  PubMed  CAS  Google Scholar 

  • Riker, W. L.: Diagnosis and treatment of aganglionosis of the myenteric plexus. Arch. Surg. 75, 362–375 (1957)

    CAS  Google Scholar 

  • Ritchie, J. A.: Colonic motor activity and bowel function: Parti. Normal movement of contents. Gut 9, 442–456 (1968)

    Article  PubMed  CAS  Google Scholar 

  • Robinson, J. W. L., Rausis, C., Basset, P., Mirkovitch, V.: Functional and morphological response of the dog colon to ischaemia. Gut 13, 775–783 (1972)

    Article  PubMed  CAS  Google Scholar 

  • Rosin, J. D., Bargen, J. A., Waugh, J. M.: Congenital megacolon of a man 54 years of age: report of a case. Mayo Clin. Proc. 25, 710–715 (1950)

    CAS  Google Scholar 

  • Schaerli, A. F.: Defecation and continence: Some new concepts. Dis. Colon Rectum 13, 81 (1970)

    Article  Google Scholar 

  • Schaerli, A. F.: Angeborene Mißbildungen des Rektums und Anus. Diagnose, Physiologie und Therapie.,,Aktuelle Probleme in der Chirurgie”, Bd. 15. Bern-Stuttgart- Wien: Hans Huber 1971 Schaerli, A. F.: Funktionelle Untersuchungen beim Morbus Hirschsprung. Pädiat.

    Google Scholar 

  • Pädol., Suppl. 2, 32–47 (1972)

    Google Scholar 

  • Schaerli, A. F., Kiesewetter, W. B.: Imperforate anus: Anorectosigmoid pressure studies as a quantitative evaluation of postoperative continence. J. Pediat. Surg. 4, 694 (1969)

    Article  Google Scholar 

  • Scherer, H. J.: Untersuchungen über den geweblichen Aufbau der Geschwülste des peripheren Nervensystems. Virchows Arch. path. Anat. 292, 479–553 (1934)

    Article  Google Scholar 

  • Shocket, E., Telok, H. A.: Aganglionic megacolon, pheochromocytoma, megaloureter, and neurofibroma: co-occurence of several neural abnormalities. Amer. J. Dis. Childh. 94, 185–191 (1957)

    CAS  Google Scholar 

  • Sieber, W. K., Gridany, B. R.: Management of symptomatic aganglionic megacolon in early infancy. Arch. Surg. 75, 388–395 (1957)

    CAS  Google Scholar 

  • Smith, A. N., Ridgeway, M.: Use of telemetering capsules in disorders of alimentary tract. II. Application to study of human gastro-intestinal motility. Gut 3, 371–376 (1962)

    Article  Google Scholar 

  • Smith, B.: The myenteric plexus in Hirschsprung’s disease. Proc. roy. Soc. Med. 60, 803 (1967a)

    PubMed  CAS  Google Scholar 

  • Smith, B.: Myenteric plexus in Hirschsprung’s disease. Gut 8, 308–312 (1967b)

    Article  PubMed  CAS  Google Scholar 

  • Soave, F.: Hirschsprung’s disease: Clinical evaluation, physiopathology and therapy. Clin. Med. 74, 19–26, 31 (1967)

    Google Scholar 

  • Staple, T. W., Mcalister, W. H., Anderson, M. S.: Plexiform neurofibromatosis of the colon simulating Hirschsprung’s disease. Amer. J. Roentgenol. 91, 840–845 (1964)

    PubMed  CAS  Google Scholar 

  • Stelzner, F.: Kontinenz, Superkontinenz und Inkontinenz im Anorektalbereich. Dtsch. med. Wschr. 90, 2275–2281 (1965)

    CAS  Google Scholar 

  • Stone, W. D., Hendrix, T. R., Schuster, M. M.: Aganglionosis of the entire Colon in an adolescent. Gastroenterology 48, 636–641 (1965)

    PubMed  CAS  Google Scholar 

  • Swenson, O.: Congenital defects in the pelvic parasympathetic system. Arch. Dis. Childh. 30, 1–7 (1955)

    Article  PubMed  CAS  Google Scholar 

  • Swenson, O.: Pediatric surgery. New York: Appleton 1958

    Google Scholar 

  • Swenson, O.: Hirschsprung’s disease (aganglionic megacolon). New Engl. J. Med. 260, 972–976 (1959)

    Article  PubMed  CAS  Google Scholar 

  • Swenson, O., Bill, A. H., Jr.: Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon. Surgery 24, 212–220 (1948)

    PubMed  CAS  Google Scholar 

  • Swenson, O., Fisher, J. H.: The relation of megacolon and megaureter. New Engl. J. Med. 253, 1147–1150 (1955)

    Google Scholar 

  • Swenson, O., Fisher, J. H., McMahon, H. E.: Rectal biopsy as aid in diagnosis of Hirschsprung’s disease. New Engl. J. Med. 253, 632–635 (1955)

    Article  PubMed  CAS  Google Scholar 

  • Swenson, O., Rheinlander, H. F., Diamond, I.: Hirschsprung’s disease: a new concept of the etiology. New Engl. J. Med. 241, 551–556 (1949)

    Article  PubMed  CAS  Google Scholar 

  • Ternberg, J. L., Winters, K.: Plexiform neurofibromatosis of the colon as a cause of congenital megacolon. Amer. J. Surg. 109, 663–665 (1965)

    Article  PubMed  CAS  Google Scholar 

  • Tiffin, M. E., Chandler, L. R., Faber, H. K.: Localized absence of the ganglion cells of the myenteric plexus in congenital megacolon. Amer. J. Dis. Childh. 59, 1071–1082 (1940)

    Google Scholar 

  • Tittel, K.: Über eine angeborene Mißbildung des Dickdarmes. Wien. klin. Wschr. 14, 903–907 (1901)

    Google Scholar 

  • Tobon, F., Reid, N. C. R. W., Talbert, J. L., Schuster, M. M.: Nonsurgical test for the diagnosis of Hirschsprung’s disease. New Engl. J. Med. 278, 188–194 (1968)

    Article  PubMed  CAS  Google Scholar 

  • Todd, I. P.: Discussion on megacolon and megarectum with the emphasis on conditions other than Hirschsprung’s disease. Proc. roy. Soc. Med. 54, 1035–1037 (1961)

    PubMed  CAS  Google Scholar 

  • Todd, I. P.: Some aspects of adult megacolon. Proc. roy. Soc. Med. 64, 561–565 (1971)

    PubMed  CAS  Google Scholar 

  • Todd, I. P., Porter, N. H., Morsen, B. C.: Chagas disease of the colon and rectum. Gut 10, 1009–1014 (1969)

    Google Scholar 

  • Toniolo, L. B., Macchitella, E.: Le megasindromi. IV. II megacolon. Chir. Pat. sper. 11, 143–184 (1963)

    Google Scholar 

  • Walker, A. W., Kempson, R. L., Ternberg, J. L.: Aganglionosis of the small intestine. Surgery 60, 449–457 (1966)

    PubMed  CAS  Google Scholar 

  • Whitehouse, F., Bargen, J. A., Dixon, C. F.: Congenital megacolon: Favorable end results of treatment by resection. Gastroenterology 1, 922–937 (1943)

    Google Scholar 

  • Whitehouse, F. R., Kernohan, J. W.: Mesenteric plexus in congenital megacolon. Study of eleven cases. Arch, intern. Med. 82, 75–111 (1948)

    CAS  Google Scholar 

  • Wilkinson, A. W.: Recent advances in pediatric surgery. Boston: Little Brown & Comp. 1963

    Google Scholar 

  • Willich, E.: Röntgendiagnostik der Hirschsprungschen Krankheit im Neugeborenenalter und bei atypischen Fällen. Pädiat. Pädol., Suppl. 2, 7–22 (1972)

    Google Scholar 

  • Wilson, J. S., Anderson, A. A.: Cutaneous and intestinal neurofibromatosis. Amer. J. Surg. 100, 761–763 (I960)

    Google Scholar 

  • Wurnig, P.: Morbus Hirschsprung. Neuere Probleme der Diagnose. Pädiat. Pädol. Suppl. 2 (1972)

    Google Scholar 

  • Yntema, C. L., Hammond, W. S.: Experiments on the sacral parasympathetic nerves and ganglia of the chick embryo. Anat. Ree. 115, 382 (1953)

    Google Scholar 

  • Yntema, C. L., Hammond, W. S.: Origin of intrinsic ganglia of trunk viscera from vagal neural crest in chick embryo. J. comp. Neurol. 101, 515–542 (1954)

    Article  PubMed  CAS  Google Scholar 

  • Zbinden, H.: Beitrag zur intestinalen Form der Neurofibromatosis Recklinghausen. Helv. chir. Acta 18, 524–531 (1951)

    PubMed  CAS  Google Scholar 

  • Zuelzer, W. W., Wilson, J. L.: Functional intestinal obstruction on a congenital neurogenic basis in infancy. Amer. J. Dis. Childh. 75, 40–64 (1948)

    CAS  Google Scholar 

  • Zypen, E. Van Der: Elektronenmikroskopisch faßbare Veränderungen am Plexus Myentericus (Auerbach) beim Magenulcus und bei Colitis ulcerosa. Prophylaxe (Wien) 5, 7–10 (1965a)

    Google Scholar 

  • Zypen, E. Van Der: Licht- und elektronenmikroskopische Befunde am vegetativen Nervensystem des Colons bei Colitis ulcerosa des Menschen. Dtsch. Z. Nervenheilk. 187, 787–836 (1965b)

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1974 Springer-Verlag Berlin · Heidelberg

About this paper

Cite this paper

Meier-Ruge, W. (1974). Hirschsprung’s Disease: Its Aetiology, Pathogenesis and Differential Diagnosis. In: Grundmann, E., Kirsten, W.H. (eds) Current Topics in Pathology. Current Topics in Pathology, vol 59. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-65857-0_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-65857-0_4

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-65859-4

  • Online ISBN: 978-3-642-65857-0

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics