European Journal of Cardio-Thoracic Surgery, Vol 7, 337-341, Copyright © 1993 by European Association for Cardio-thoracic Surgery
Primary immotile oesophagus in young patients
SM Allen, DE Van Raemdonck, IP Adams and HR Matthews
Regional Department of Thoracic Surgery, East Birmingham Hospital, UK.
Since 1984 we have identified 14 patients under the age of 45 years with
severe, unexplained loss of oesophageal motility. The ages at presentation
ranged from 20 to 42 years and nine were female. Barium swallow was normal
in eight patients and showed minor abnormalities in six. Twenty-four hour
pH studies were normal in all patients and the only endoscopic abnormality
was minimal oesophagitis in three. Upper oesophageal sphincter resting
pressures and cervical motility were normal except in one patient. Motility
in the body of the oesophagus was severely reduced in all patients with a
mean peristaltic amplitude of 10 mmHg. Lower oesophageal sphincter resting
pressures were reduced in 11 patients and normal in three, with incomplete
sphincter relaxation in three. All patients have been followed up for a
minimum of 3 years without progression of their symptoms or the development
of a secondary cause. Six patients have undergone repeat manometry and pH
studies with no changes. We conclude that there is a group of young
patients who appear to undergo severe primary loss of oesophageal motility
for reasons that have not yet been identified. It is important to identify
these patients so that inappropriate surgery can be avoided.
