The Internet Journal of Gastroenterology™ ISSN: 1528-8323

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An unusual cause of dysphagia

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TJS Cross
Institute of Liver Studies
King's College Hospital

D. Joshi
Institute of Liver Studies
King's College Hospital

Citation: T. Cross & D. Joshi : An unusual cause of dysphagia . The Internet Journal of Gastroenterology. 2008 Volume 7 Number 1

Keywords: Amyloid | Congo Red | Dysphagia

Discussion

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.

This article was last modified on Fri, 13 Feb 09 13:30:36 -0600

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The Internet Journal of Gastroenterology™ ISSN: 1528-8323

An unusual cause of dysphagia

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Abstract

Discussion


	The Internet Journal of Gastroenterology™ ISSN: 1528-8323 \| Home \| Editors \| Current Issue \| Archives \| Instructions for Authors \| Disclaimer \| Share with others \| An unusual cause of dysphagia Read printer friendly Subscribe in a reader Share with others Related Articles Plummer-Vinson syndrome in a black female Plummer Vinson Syndrome: A Case Report And Literature Review Localized Amyloidosis Of The Upper Respiratory System: Our Experience Of Three Cases And Review Of Article Primary nasal amyloidosis Primary Amyloidosis presenting as Cardiogenic Shock and Pseudoinfarction. A case presentation. TJS Cross Institute of Liver Studies King's College Hospital D. Joshi Institute of Liver Studies King's College Hospital Citation: T. Cross & D. Joshi : An unusual cause of dysphagia . The Internet Journal of Gastroenterology. 2008 Volume 7 Number 1 Keywords: Amyloid \| Congo Red \| Dysphagia Table of Contents Discussion 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. This article was last modified on Fri, 13 Feb 09 13:30:36 -0600 This page was generated on Tue, 09 Feb 10 11:11:07 -0600, and may be cached.
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