Congenital Generalized Hypoganglionosis: Clinical Aspect

  • Yoshio WatanabeEmail author


Congenital generalized hypoganglionosis (CGH) is a rare disorder with a poor prognosis. Both dedicated diagnosis and treatment improve the management of CGH patients. The first step in treating CGH is the creation of an appropriate stoma during the neonatal period. A jejunostomy less than 50 cm from the Treitz ligament is the preferred treatment option in neonatal period, even in advance of a definitive diagnosis. The second step is to preserve intestinal function. The habilitation procedure for the distal intestine avoids unnecessary massive bowel resection and intestinal bacterial overgrowth, which are the main management points for long-term survival. The following steps are to provide enteral caloric intake as soon as possible and to make the successful transition from parenteral to full enteral nutrition. This section focuses on the importance of CGH treatments during the neonatal and early infancy period.


Congenital generalized hypoganglionosis Hirschsprung’s allied disorders Upper jejunostomy Diagnosis and treatment 



This work was supported by a grant from the Ministry of Health, Labour, and Welfare of Japan (H24-Nanchi-Ippan-037, Health and Labour Sciences Research Grants for Research on intractable diseases).

Disclosures: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.


  1. 1.
    Taguchi T, Ieiri S, Miyoshi K, Kohashi K, Oda Y, Kubota A, et al. The incidence and outcome of allied disorders of Hirschsprung’s disease in Japan: results from a nationwide survey. Asian J Surg. 2017;40(1):29–34. Scholar
  2. 2.
    Watanabe Y, Kanamori Y, Uchida K, Taguchi T. Isolated hypoganglionosis: results of a nationwide survey in Japan. Pediatr Surg Int. 2013;29(11):1127–30. Scholar
  3. 3.
    Watanabe Y, Sumida W, Takasu H, Oshima K, Kanamori Y, Uchida K, et al. Early jejunostomy creation in cases of isolated hypoganglionosis: verification of our own experience based on a national survey. Surg Today. 2015;45(12):1509–12. Scholar
  4. 4.
    Kapur RP, Kennedy AJ. Histopathologic delineation of the transition zone in short-segment Hirschsprung disease. Pediatr Dev Pathol. 2013;16(4):252–66. Scholar
  5. 5.
    White FV, Langer JC. Circumferential distribution of ganglion cells in the transition zone of children with Hirschsprung disease. Pediatr Dev Pathol. 2000;3(3):216–22.CrossRefPubMedGoogle Scholar
  6. 6.
    Watanabe Y, Takasu H, Sumida W. A preliminary report on the significance of excessively long segment congenital hypoganglionosis management during early infancy. J Pediatr Surg. 2011;46(8):1572–7. Scholar
  7. 7.
    Watanabe Y, Ito F, Ando H, Seo T, Kaneko K, Harada T, et al. Morphological investigation of the enteric nervous system in Hirschsprung’s disease and hypoganglionosis using whole-mount colon preparation. J Pediatr Surg. 1999;34(3):445–9.CrossRefPubMedGoogle Scholar
  8. 8.
    Meier-Ruge W, Hunziker O, Tobler HJ, 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 Pathol. 1972;147:228–36.CrossRefPubMedGoogle Scholar
  9. 9.
    Watanabe Y, Ito T, Ando H, Seo T, Nimura Y. Manometric evaluation of gastrointestinal motility in children with chronic intestinal pseudo-obstruction syndrome. J Pediatr Surg. 1996;31(2):233–8.CrossRefPubMedGoogle Scholar
  10. 10.
    Torres C, Sudan D, Vanderhoof J, Grant W, Botha J, Raynor S, et al. Role of an intestinal rehabilitation program in the treatment of advanced intestinal failure. J Pediatr Gastroenterol Nutr. 2007;45(2):204–12. Scholar
  11. 11.
    Matarese LE. Nutrition and fluid optimization for patients with short bowel syndrome. J Parenter Enter Nutr. 2013;37(2):161–70. Scholar
  12. 12.
    Gensollen T, Iyer SS, Kasper DL, Blumberg RS. How colonization by microbiota in early life shapes the immune system. Science. 2016;352(6285):539–44. Scholar
  13. 13.
    Le HD, Fallon EM, de Meijer VE, Malkan AD, Puder M, Gura KM. Innovative parenteral and enteral nutrition therapy for intestinal failure. Semin Pediatr Surg. 2010;19(1):27–34. Scholar
  14. 14.
    Sumida W, Watanabe Y, Takasu H, Oshima K. Catheter-related bloodstream infection in patients with motility disorder of the alimentary tract. Pediatr Surg Int. 2014;30(9):915–8. Scholar

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© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.Obu-aozora clinicObuJapan

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