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Hirschsprung's disease
Also known as:
Hirschsprung's syndrome
Hirschsprung-Galant infantilism (Nikolai Fedorovich Galant)
Mya’s disease
Ruysch’s disease
Associated persons:
Nikolai Fedorovich Galant
Harald Hirschsprung
Giuseppe Mya
Frederik Ruysch
Description:
Megacolon due to lack of ganglion cells with failure of development of the myenteric plexus of the rectosigmoid area of the large intestine. The colon above the inactive area of the sigmoid dilates and there is chronic constipation, abdominal distension, and faecal impaction in infancy, with growth retardation. The symptoms become less severe later in life, but constipation and recurrent faecal impaction may persist. Prevalent in males. Occurrence is 1 in 5000 newborn. No definite hereditary pattern established (polygenetic), but some cases are familial, inherited as an autosomal recessive trait.
The first recorded cases of congenital idiopathic dilation of the colon (Hirschprung’s disease) was published in a posthumous work by the English physician Caleb Hillier Parry (1755-1822).
In Italy this condition is termed Mya’s disease.
Bibliography:
- C. H. Parry:
Collections From the Unpublished Writings.
London, Underwood, 1825.
- H. Hirschsprung:
Stuhlträgheit Neugeborener in Folge von Dilatation und Hypertrophie des Colons.
Jahrbuch für Kinderheilkunde und physische Erziehung, Berlin, 1888, 27: 1-7.
Erweiterung und Hypertrophie des Dickdarms.
Berliner klinische Wochenschrift, 1899, 36: 977.
- G. Mya, in: Sperimentale, 1894, 48: 215.
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