Clinical Note

Hirschsprung's Disease

Hirschsprung's disease is a congenital disorder characterized morphologically by a narrowed segment of distal colon and a hypertrophic segment of intestine orad to the narrowing. Histologically, the narrowed segment is characterized by an absence of enteric nerves. Thus, it appears as if enteric nerves are primarily inhibitory, and their absence results in a failure of the gut smooth muscle to relax. The etiology of the disease appears to be a failure of neuroblasts from the neural crest to migrate properly during fetal development, perhaps because of a defect in the microenvironment of the developing bowel. Treatment usually involves surgical removal of the aganglionic segment.

sensory information with neural signals from the spinal cord to form the neural commands sent to effector organs via postganglionic fibers. Although it is now clear that these ganglia are not simply relays, little is understood about what kind of information is sent to these ganglia, what rules are used to integrate this information, and what roles postganglionic neural commands play in the regulation of organ function.

Suggested Readings

Furness JB, Bornstein JC, Kunze WAA, Clerc N. The enteric nervous system and its extrinsic connections, in Yamada T, Ed.,

Textbook of gastroenterology. Philadelphia: Lippincott/Williams & Wilkins, 1999

Hoffman BB, Taylor P. Neurotransmission: the autonomic and somatic motor nervous system, in Hardman JG, Limbird LE, Gilman AF, Eds., Goodman and Gilman's the pharmacological basis of therapeutics. New York: McGraw-Hill, 2001 Korczyn AD, Ed. Handbook of autonomic nervous system dysfunction.

New York: Marcel Dekker, 1995 McGrath MA, Vanhouette PM. Vasodilation caused by peripheral inhibition of adrenergic neurotransmission, in Vanhouette PM, Leusen S, Eds. Mechanisms of vasodilation. Basel: Karger, 1978, pp. 248-257

Vanhouette PM. Adrenergic neuroeffector interaction in the blood vessel wall. Fed. Proc. 1978, 37:181-186

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