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August 1957

Aganglionic Megacolon, Pheochromocytoma, Megaloureter, and Neurofibroma: Co-Occurrence of Several Neural Abnormalities

AMA Am J Dis Child. 1957;94(2):185-191. doi:10.1001/archpedi.1957.04030030079012

This is a report of a patient with multiple neural abnormalities. It is presented because (a) multiple anomalies of a single system are traditionally of interesf, (b) because aspects of the combination in this instance may reflect the underlying embryological error which gives rise to aganglionic megacolon,1 and (c) because unrecognized pheochromocytoma is frequently associated with operative catastrophe when surgery is undertaken for a non-adrenal lesion.

Report of a Case  History.—Constipated since birth, an 18-year-old youth * had been medically discharged from the U. S. Navy in early 1955 and advised to seek surgery. Even in the neonatal period rectal suppositories had be,en necessary for each infrequent bowel movement. A colon roentgenogram at the age of 2 years was followed by a diagnosis of megacolon. Periodic dosing with laxatives and frequent enemas had always been necessary; nevertheless, the patient had matured normally in every way. Very occasionally, 7 or