Palatopharyngeal incompetence in association with esophageal dysmotility, acquired glucocorticoid deficiency, and deficient tear production.

An 8 1/2-year-old male is described with the rare triad of acquired adrenal insufficiency, esophageal dysfunction, and alacrima. In addition, he had velopharyngeal insufficiency, which is a previously unreported feature of this syndrome. Although the pathophysiology of this disorder remains to be demonstrated, a defect may be present, linking hormone-receptor cyclic AMP-mediated processes with abnormalities in parasympathetic and voluntary neuronal innervation or transmission.