Dysphagia Lusoria: An Elusive Diagnosis-Juniper Publishers
Introduction
Dysphagia lusoria is a sporadic condition that causes
difficulty swallowing. The symptoms are attributed to an aberrant right
subclavian artery that has a retroesophageal course leading to vascular
compression of the esophagus.
Dysphagia lusoria is an uncommon anomaly and even
rarer cause of symptoms. However, when symptomatic and occurring in
conjunction with similar presenting conditions, it can create a
diagnostic and treatment challenge [1].
A 62-year-old female with a medical history
significant for coronary artery disease and gastritis presented to our
hospital on multiple occasions complaining of chronic nausea,
dyspnea, chest pain and dysphagia. Physical examination was often
unrevealing with the exception of hypertensive blood pressure.
Laboratory investigation often included completeblood cell count,
complete metabolic panel, serial troponins, D-Dimers, all usually within
normal limits.
Multiple subspecialists, most commonly being
gastroenterologists and cardiologists, were consulted to evaluate her.
Despite an extensive medical workup, including
esophagogastroduodenoscopy, computerized tomography (CT) scans of the
thorax, abdomen and pelvis, magnetic resonance angiogram (MRA) of the
abdomen and multiple echocardiograms, the cause of her symptoms remained
elusive.
Prior to her diagnosis, she had a total of twelve
emergency department visits and five hospitalizations before a coronary
CT was utilized. Her coronary CT revealed an anomalous origin of the
left coronary artery from the right coronary cusp with a retroaortic
course in addition to the left aortic arch exhibiting an aberrant right
subclavian artery (Figures 1 & 2).
Hence, a diagnosis of Dysphagia Lusoria was made and
the patient was referred to cardiothoracic surgery. Typically,
surgical treatment is the only curative option, however,
symptoms can be controlled by specific dietary modifications,
the use of proton pump inhibitors and antianginal medication.
Our patient has opted for conservative medical management
while exploring the possibility of surgical correction but
symptoms still persist.
Dysphagia lusoria is a term used to designate dysphagia
as a result of vascular compression of the esophagus. It was
first utilized by Bayford [2] in 1761 in describing a case of
longstanding dysphagia leading to emaciation and eventual
death of a 62-year old female patient. On autopsy the patient
was found to have an aberrant right subclavian artery running
anterior to and causing compression of her esophagus. This is
a common variant of embryonic aortic arch involution within
the general population. It is estimated at 0.4% to 0.7% in e
published literature [3]. 30%-40% of patients with vascular
anomalies have dysphagia, with the majority presenting with
solid bolus dysphagia.
Traditionally, catheter angiogram has been accepted as a
gold standard tool for diagnosis.
Nevertheless, today with the
availability of 3D spiral CT angiography and 3D MRA, we can
non-invasively demonstrate such vascular anomalies. Also,
some references mention dynamic barium swallow studies as
a diagnostic screening tool for dysphagia lusoria.
The management is reliant on the severity of symptoms and
impact on the ability of the patients to maintain their weight
and nutrition. It is imperative to first treat these patients
with prokinetic, anti-reflux drugs or dietary modification.Only if the symptoms are intractable should these patients be
subjected to surgical correction of the anomaly. In addition,
the decision to operate also depends on the presence of an
aneurysm near the root of the artery, and the age of the patient.
The most common approach to repair is a left postero-lateral
thoracotomy followed by division and ligation of the aberrant
artery with dissection or possible re-implant [4].
After thorough investigation of literature, we did not find
any association between anomalous origin of the left coronary
artery and aberrant right subclavian artery. Hence, this is the
first case report to raise the question of a common embryologic
etiology that may lead to both anomalies.
- The lusorian artery can create a diagnostic and treatment challenge.
- Contrast enhanced CT or MRI can easily confirm the diagnosis and demonstrate the exact relationship of the anomalous vessel with surrounding structures.
- Operative repair by open surgery or in combination with endovascular techniques is recommended to treat symptoms and reduce the risk of aneurysm rupture.
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