PRIMARY IMMOTILE ESOPHAGUS IN YOUNG-PATIENTS

Since 1984 we have identified 14 patients under the age of 45 years wi th severe, unexplained loss of oesophageal motility. The ages at prese ntation ranged from 20 to 42 years and nine were female. Barium swallo w was normal in eight patients and showed minor abnormalities in six. Twenty-four hour pH studies were normal in all patients and the only e ndoscopic abnormality was minimal oesophagitis in three. Upper oesopha geal sphincter resting pressures and cervical motility were normal exc ept in one patient. Motility in the body of the oesophagus was severel y reduced in all patients with a mean peristaltic amplitude of 10 mm H g. Lower oesophageal sphincter resting pressures were reduced in 11 pa tients and normal in three, with incomplete sphincter relaxation in th re:e. All patients have been followed up for a minimum of 3 years with out progression of their symptoms or the development of a secondary ca use. Six patients have undergone repeat manometry and pH studies with no changes. We conclude that there is a group of young patients who ap pear to undergo severe primary loss of oesophageal motility for reason s that have not vet been identified. It is important to identify these patients so that inappropriate surgery can be avoided.