Senin, 01 Agustus 2011

Achalasia


Features of Achalasia on Radiographic Studies
1.) Markedly Dilated Esophagus
2.) Air-fluid level in esophagus on upright view
3.) Tertiary non-stripping peristalsis or aperistalsis
4.) Narrowed “bird-beak” appearance at LES
5.) Absent gastric air bubble

Achalasia on Plain Film:

Widening of mediastinum due to dilated esophagus
Absence of gastric air bubble due to failure of LES relaxation.


Upright Barium Swallow:

Dilated Esophagus filled with air and a bit of barium.
No evidence of peristalsis at fluoroscopy.


Pooling at LES w/ food bolus evident.
Tight LES with “bird beak” appearance

Supine Barium Swallow:
Air-filled, dilated esophagus

Note dilated esophagus.
Note constricted LES.

LES represents a “bird’s beak” appearance.
Small tertiary waves present.

Achalasia with significant tertiary peristalsis is termed “vigorous achalasia”.


Treatment
1. Medical Therapy – Nitrates and Calcium channel blockers (e.g. nifedipine). No treatment reliably restores function of esophagus.
2. Pneumatic Balloon Dilation of LES – weakens LES by tearing muscle fibers; risk of perforation
• 50% require further treatment within 5 years of dilation
• Risk of perforation ~ 5%
3. Surgical Myotomy (Heller, Laparoscopic) – weakens LES by cutting muscle fibers; thoracotomy necessary if not laparascopic
• 70-85% success at 10 yrs for modified Heller
4. Botulinum Toxin – Relatively new. Endoscopic injection of toxin into LES.
• Short term success. Limited evidence of long term efficacy.
Achalasia patients have 16x risk for esophageal carcinoma – risk is not eliminated by therapy.


Bibliography
• Boeckxstaens, GE. “Achalasia”. Best Practice & Research Clinical Gastroenterology 2007. Vol. 21, No. 4, pp. 595-608.
• Farrokhi and Vaezi Orphanet Journal of Rare Diseases 2007 2:38
• Finley RJ et al. “Practical approaches to the surgical management of achalasia” The American Surgeon. February 2008.
• Kraichely RE, Farrugia G. “Achalasia: physiology and etiopathogenesis”. Diseases of the Esophagus 2006. Vol 19, 213-223.
• Levine MS. Radiology of the Esophagus. Saunders Company. 1989. Philadelphia.
• Mittal RK, Balaban DH. “The Esophagogastric Junction” NEJM. March 27, 1997. Vol 336 No 13. 924-932.
• Mueller CF et al. “Achalasia – Chest Case of the Day”. American Journal of Roentgenology. 2000; 175: 870-871.
• Novelline RA. Squire’s Fundamentals of Radiology 6th Ed. Harvard University Press. Cambridge, MA. 2004.
• Pohl D, Tutuian R. “Achalasia: an overview of diagnosis and treatment”. J Gastrointest Liver Dis. September 2007 Vol 16 No 3, 297-303.
• Richter JE. “Oesophageal motility disorders”. Lancet 2001; 358: 823-828.
• Spechler, SJ. “Approach to the patient with dysphagia”. UpToDate. www.uptodate.com. Accessed 5 5/21/08.
• Spechler, SJ. “Clinical manifestations and diagnosis of achalasia”. UpToDate. www.uptodate.com. Accessed 5/21/08.
• Spechler, SJ. “Overview of the treatment of achalasia”. UpToDate. www.uptodate.com. Accessed 5/21/08.
• Spechler, SJ. “Pathophysiology and etiology of achalasia”. UpToDate. www.uptodate.com. Accessed 5/21/08.
• Spiess AE, Kahrilas PJ. “Treating achalasia: from whalebone to laparascope” JAMA 1998; 280






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