Dysphagia

Case #: 003
Date: September 14th, 2009

History

This is a 17-year-old Caucasian male who presents to clinic complaining of more than 8 years history of feeling as if food that he swallows gets stuck in his throat. He says that this problem has been getting worse with time but especially the last several months. He reports that when he swallows foods (liquid or solid), that food gets caught in his throat, and he refers to the area of his thoracic inlet centered above his trachea. He has seen physicians on a couple occasions over the last several years who have offerered him anti-acid treatments without relief. Physical exam was unremarkable. Out of concern for dysmotility of the esophagus, an Esophagram was performed (see images below).

Images


serimg00025.jpg
Study Date: 11/2006. Smoothly dilated distal esophagus with "beak-like" narrowing at the gastroesophageal junction. Decision was made to treat conservatively with calium channel blockers and to follow-up.

img0012.jpg
Study Date: April 2009. Aperistaltic dilated esophagus with tight beaklike narrowing at the esophagogastric junction consistent with chronic achalasia. The degree of narrowing has increased since the outside study of 11/2006. Despite medical therapy, symptoms progressed. Decision was made by patient and doctors to undergo laporoscopic heller myotomy.


CT_achalasia.jpg
Courtesy of LearningRadiology.com (different patient): CT scan of the chest demonstrates a markedly dilated esophagus.



Diagnosis

Achalasia, as suggested by clinical history and radiographic findings above and subsequently confirmed by Esophagogastric manometry. The manometry demonstrated an elevated lower esophageal sphincter resting pressure at 47 mmHg upon liquid swallowing. The percent relaxation was only 37% which was below normal. The upper esophageal sphincter pressure was measured at 63 mmHg which was considered normal. The study was consistent with the presence of achalasia involving the distal half of the esophagus.

Explanation


Pathophysiology:
The gastroesophageal sphincter fails to relax because of wallerian degeneration of Auerbach's plexus. Microscopic examination reveals decreased number of ganglion cells in the myenteric esophageal plexus. The sphincter relaxes only when the hydrostatic pressure of the column of liquid or food exceeds that of the sphincter.

Types:
  1. Primary (Idiopathic)
  2. Secondary (destruction of myenteric plexus by tumor cells)
  • Metastases
  • Adenocarcinoma invasion from the gastric cardia
3. Infectious (i.e. Chagas)

Presentation:
- There is no gender predilection
- Primary type occurs in young adults (30-50 yrs), secondary occurs in older patient population
- Dysphagia to both solids and liquids (nearly 100%)
- Weight loss, (90%)
- Relatively pain-less as compared to esophageal spasm
- Recurrent aspiration pneumonia

Diagnosis:
- Need to exclude malignancy, especially in the elderly
- Neet to exclude spasm
- Esophagram is the initial study of choice although Manometry is the most sensitive method to diagnose elevated lower esophageal spincter (LES) pressure and incomplete relaxation.

Radiographic features:
A barium swallow (esophagram) is the primary screening test when achalaisa is suspected on clinical grounds. The diagnostic accuracy of barium swallow for achalasia is approximately 95 percent. The barium swallow typically shows a smooth, dilated esophagus that terminates in a beak-like narrowing caused by the persistently contracted lower esophageal sphincter (LES). Fluoroscopy reveals the absence of peristalsis in the smooth muscle portion of the esophagus. Other findings on chest radiography include diffuse mediatsinal widening with air-fluid levels and little or absent gastric air bubble. Computed Tomography findings include moderate to marked dilation of the distal esophagus with diameter greater than 4cm and decreased or normal esophageal wall thickness.


Treatment:
- Smooth muscle relaxants (Nitrates +/- Calcium channel blockers)
- Pneumatic balloon dilatation, performed fluoroscopically (high risk of perforation)
- Heller myotomy, more effective than pneumatic dilatation and with fewer complications
- Endoscopic injection of botulinum toxin








Resources

1. Weissleder et atl. Primer of Diagnositic Imaging, 4th Edition. Mosby Elsevier.
2. Franquet T et al: The retrotracheal space: normal anatomic and pathologic appearances. Radiographics. 22 Spec No:S231-46, 2002
3. Stark P et al: Manifestations of esophageal disease on plain chest radiographs. AJR Am J Roentgenol. 155(4):729-34,1990