[Digestive manifestations in a patient with type-IIB multiple endocrine neoplasms]

The IIB or third type of multiple endocrine neoplasia is a very uncommon hereditary disease. It includes a digestive ganglioneuromatosis, a typical dysmorphia, a medullary thyroid cancer (MTC) sometimes a pheochromocytoma and seldom a hyperparathyroidism. We report a Men IIB case in a 19 years old patient who had been suffering from severe constipation and afflicted with a megacolon, known since early infancy. Diagnosis was documented by discovering a colonic ganglioneuromatosis and a MTC. A complete thyroidectomy was performed but cervical node metastasis were already present. The ganglioneuromatosis usually affects the whole alimentary tract, but megacolon although not regularly found occurs most frequently. Awareness of the diagnosis is easier when patients have big lips, eyelids, tongue (due to mucosal neuromas) and marfanoid habitus. The prognosis depends upon the MTC. It occurs early in the life, is often bilateral and has a trend to become uncontrollable in the young adult. The pheochromocytoma is often latent and affects only half of patients. The etiopathogeny of this disease still remains unknown. Family inquiry may help to an earlier diagnosis and therefore to a better prognosis.