Description, Causes and Risk Factors:
This dysphagia said to be due to compression by the right subclavian artery arising abnormally from the descending aorta and passing behind the esophagus.
Compression of the esophagus by a vascular structure is an uncommon cause of dysphagia. There have been reports of almost every major vasculature structure within the chest cavity causing somedegree of esophageal compression and symptomatic dysphagia. In 1794, David Bayford noted the association of dysphagia with esophageal compressionin in a 62-year-old woman caused by a right aberrant subclavian artery. He coined the term 'dysphagia lusoria,' which is Latin for `freak of nature.' An aberrant right subclavian artery is the most common congenital anomaly of the aortic arch,with an incidence ranging from 0.5% to 1.8%.
Dyspahagia lusoria is a congenital vascular anomly involving aberrant right subclavian artery. The most common embryologic abnormality of the aortic arch is an aberrant right subclavian artery, which occurs in 0.5% to 1.8% of the population.
As hypothesized by Edwards, this abnormal origin of the right subclavian artery can be explained by the involution of the 4th vascular arch with the right dorsal aorta. The 7th intersegmental artery remains attached to the descending aorta, and this persistent intersegmental artery becomes the right subclavian artery. This leads to the aberrant artery, which often follows a retro-esophageal course.
In our opinion, the supraclavicular approach provides good exposure, rapid recovery, and avoids the morbidity associated with the classically described median sternotomy or thoracotomy.
The presence of this anomaly is often asymptomatic,and may be discovered incidentally on imaging or onpostmortem analysis. As many as 60-80% of patientsremain symptom-free in their lifetime. Symptomaticadult patients usually present with dysphagia consistentwith a mechanical obstruction. Symptoms are primarilyfor solids and are associated with regurgitation of unchewed food, postprandial bloating, chest pain,and symptoms that frequently change with position.Other complaints include coughing, thoracic pain,or Horner's syndrome. In rare cases, patients presentwith rupture of an aneurysmal aberrant artery orKommerell's diverticulum.
In infants, respiratory symptoms are the pre-dominant mode of presentation. This is believeddue to the absence of tracheal rigidity, allowing fortis compression and leading to stridor, wheezing,cyanosis, or recurrent pneumonia
. Interestingly, patientsusually do not present in childhood, becomingsymptomatic in young adulthood, and even in themiddle or elder ages. The average age of patients inour review was 48 years. Theories explaining thisdelayed presentation include physiologic and anatomicchanges that may occur with the aging process suchas increased esophageal rigidity, rigidity of the vesselwall due to atherosclerosis, elongation of the aorta,and aortic aneurysm formation, especially in thepresence of a Kommerell's diverticulum.
The physical examination is usually normal. Asymmetrical radial pulses may be present, especially inthe setting of other vascular abnormalities.
The diagnosis can be overlooked at endoscopy, but barium contrast study of the esophagus will reveal the abnormality. Manometry cannot be used to diagnose this condition or to predict surgical outcome. Diagnosis is confirmed on CT with contrast study or MR angiography. When the symptoms are intractable, surgical correction should be considered even if coexisting esophageal abnormalities are present.
In 1936, Kommerell described the radiologic findings of this persistent route of the aortic arch as an aortic diverticulum (Kommerell's diverticulum).
The management of patients with dysphagia lusoria primarily depends on the severity of symptoms. Mild-to-moderate symptoms are often treated symptomatically, with changes in lifestyle and dietary modi?cation, such as avoiding exacerbating foods, eating slower, chewing well, taking smaller bites, sipping liquids, as well as reassurance. Additionally, acid suppression and promotility agents have been used. In a series of six patients, were able to treat three with a proton pump inhibitor alone, or in combination with the prokinetic drug cisapride. Whether or not this indicates that the dysphagia associated with this vascular anomaly is secondary to underlying gastroesophageal re?ux disease or a motility disorder, and the vascular anomaly is merely an incidental ?nding, is a matter of speculation. Other patients do not respond to conservative therapy, and in these situations, surgical intervention is justi?ed.
The surgical approach largely depends on the speci?c vascular anatomy as well as the surgeon's personal preference. The goal of surgery is to remove the aberrant vessel and to reconstruct the vessel in its appropriate position. The reconstruction can bed one via anastomosis of the native vessel, or by interposing a synthetic graft in its place. There does not appear to be a general consensus in the surgical literature as to the best surgical approach. The traditional approach consists of dividing the aberrant artery at its origin through a median sternotomy and translocating the distal subclavian artery to the aortic arch or right carotid artery. A cervical approach is preferred because there is a decreased rate of complications, as well as better visibility of the subclavian and carotid arteries. Through a cervical approach, the aberrant artery is ligated near its root and connected with the right carotid artery. There are even reports of severing the artery without reconstruction, without signi?cant reports of upper extremity ischemia. Potential damage to nearby structures and/or altering blood ?ow to the right upper extremity are possible complications. In the past, mortality rates have been reported to be as high as 16-25%, largely due to a two-step surgical repair.
In patients who are poor surgical candidates, endoscopic dilation of the esophageal narrowing may temporarily relieve symptoms of dysphagia. There are not enough cases in the literature to know the success rate of this procedure. However, its utility is primarily palliative in patients who cannot undergo more de?nitive therapy. Alternatively, there are various procedures that interventional radiology can perform in an effort to endovascular ligate the aberrant vessel while maintaining blood ?ow to the appropriate organs.
NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.