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Keywords
EUS, Dysphagia, Dysphagia Lusoria, vascular rings.
Introduction
A 42-year-old woman presented with new onset dysphagia, weight loss, and an abnormal upper GI barium study. EUS was used as the definitive test to uncover an unusual extraesophageal finding which was responsible for her condition.
Methods for Image Cature/Processing
SVHS tapes were digitally captured using
Dazzle software and processed using Adobe Premiere.
Disclaimer
No experimental therapies were used. All
procedures were performed only after obtaining informed consent.
Case/Body
A 42-year-old woman presented with one month
of solid food dysphagia. She noted solid foods sticking in
her cervical esophagus, but had no problem with liquids. She
had no prior history of dysphagia and denied heartburn and
regurgitation. She had lost ten pounds in a month owing to
poor intake.
During the physical exam, the patient appeared healthy, with no jaundice. Her neck was supple. No masseses were felt. Her heart was regular, and no vascular bruits were noted in the chest or neck.
An upper GI was performed which showed extrinsic compression of the esophagus just below the aortic arch and a filling defect posteriorly.
An EGD was performed (Video Clip 1) which revealed posterior extrinsic compression of the esophagus.
An EUS was done (Video Clip 2) to assess the extrinsic compression. An anomalous artery was seen arising off of the aortic arch and encircling the esophagus from behind.
Video Clip 3 shows the Visible Human anatomy
as one goes through the esophagus starting below the aortic
arch and moving proximally. As shown, there are normally no
vascular structures between the esophagus and spine at the
level of the aortic arch.
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Video Clip 1:
An EGD in the proximal esophagus, in the region seen to be abnormal on the barium esophogram. |
Video Clip 2: EUS starting proximally above the aortic arch and proceeding distally through the aortic arch and then proximally again. The spine is inferior. |
Video Clip 3:
Visible Human anatomy as one goes through the esophagus starting below the aortic arch at the level of the carina and moving proximally. |
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Figures 2A-2E show images extracted from the EUS video as well as VH images to help in orientation of the videos.
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Figure 2A |
Figure 2B |
Figure 2C |
Figure 2D |
Figure 2E |
Figures 3A and 3B shows Visible Human models of the normal situation and the anatomy found in the patient.
MRI of the great vessels did not show any other vascular anomalies.
The patient underwent surgical correction. Briefly, the sternum was opened in the midline, and the incision
was extended a short distance into the right neck. The thymic fat on the right side was removed. The vena cava and innominate vein were freed up, as was the left carotid artery. The arch, trachea, and esophagus were dissected free and retracted anteriorly and to the left. The aberrant right subclavian artery was exposed down to its origin on the posterior, distal aortic arch. The aberrant artery was divided at its origin and the opening on the aorta oversewn. The artery was then withdrawn from behind the esophagus and attached to the right carotid artery.
Figures 4A and 4B show schematic drawings of the preoperative and postoperative anatomy.
The patient tolerated the procedure well. Her symptoms have since resolved.
DISCUSSION
Dysphagia Lusoria is generally caused by a vascular ring consisting
of an aberrant right subclavian artery which passes between
the esophagus and spine (1-5). It can occur with or without
other associated vascular anomalies (6,7).
Although it is a congenital anomaly, new onset symptoms in
adults have been reported (3,4), though the symptoms are generally
not as pronounced and precipitous as in our adult patient.
With the more widespread use of EUS, anomalous right subclavian
arteries are being found incidentally (8,9). A retrospective
review of 3,334 mediastinal EUS cases uncovered 12 instances
in which there was an anomalous right subclavian artery, but
none of these patients were symptomatic (8). However, as EUS
is used as an evaluation for dysphagia and extrinsic masses,
more cases should be reported.
As illustrated in this report, cases can present in unusual
ways. The presentation of this case is unusual due to the
rapid onset of significant symptoms in adulthood, and the
diagnosis established by EUS.
Surgical repair consisting of transection and reimplantation
of the anomalous artery has been reported (10). Surgical repair
was curative in our patient.
SUMMARY
This report underscores the utility of EUS
in the evaluation of extrinsic esophageal disease, and the
care one should take in performing EUS of the mediastinum
to ensure that these types of findings are not missed. A good
sense of normal anatomy is imperative when doing EUS.
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