An unusual cause of dysphagia
Abstract
A 77-year old caucasian man presented with a four-month history of chest discomfort and dysphagia. A lateral chest radiograph demonstrated bony destruction (figure 1) of the sternum. The patient underwent an upper GI endoscopy which demonstrated a dilated and hypo-peristaltic oesophagus. The mucosa in the lower third was friable and irregular. A biopsy was taken of this area.
Figure 1: Lateral chest radiograph
Figure 2: Oesophageal biopsy
The oesophageal biopsy (figure 2) demonstrated amorphous eosinophilic material staining positive for Congo Red. A sternal mass biopsy confirmed the presence of amyloid and also plasma cell infiltration consistent with a plasmacytoma. A bone marrow specimen showed typical myeloma changes. A diagnosis of AL amyloidosis (kappa) with oesophageal infiltration secondary to myeloma was made. The patient deteriorated following a course of intravenous steroids, and died from chest sepsis.
Discussion
Presentation of amyloid infiltration of the GI tract includes malabsorption, intestinal infarction, perforation and obstruction, bleeding and protein- losing enteropathy. Motility disturbances may also occur and can be produced by muscle infiltration or by intestinal neural dysfunction. Oesophageal manometry can demonstrate an incompetent or hypertensive, poorly relaxing lower oesophageal sphincter (LOS). An pseudo-achalasia presentation is also recognised, for which pneumatic dilatation of the LOS can be performed.