Hybrid Surgical Treatment of Dysphagia Lusoria in a patient with Loeys-Dietz Syndrome

P0150 

Abstract Submission 

Lucas Ribe (1), Yuki Ikeno (1), Lucas Ruiter (2), Thanila Macedo (3), Rana Afifi (4), Akiko Tanaka (5), Gustavo Oderich (1)

(1) McGovern Medical School at UTHealth, Houston, TX, (2) McGovern Medical School UTHealth, Houston, TX, (3) Memorial Hermann Hospital. UTHealth., Houston, TX, (4) Memorial Hermann, Houston, TX, (5) Memorial Hermann Heart and Vascular Institute, Houston, TX

Lucas Ribe    -  Contact Me
McGovern Medical School at UTHealth

Yuki Ikeno    -  Contact Me
McGovern Medical School at UTHealth

Lucas Ruiter Kanamori    -  Contact Me
McGovern Medical School UTHealth

Thanila Macedo    -  Contact Me
Memorial Hermann Hospital. UTHealth.

Rana Afifi    -  Contact Me
Memorial Hermann

Akiko Tanaka    -  Contact Me
Memorial Hermann Heart and Vascular Institute

Gustavo Oderich    -  Contact Me
McGovern Medical School at UTHealth

Lucas Ribe    -  Contact Me
McGovern Medical School at UTHealth

Objective

Dysphagia lusoria is a rare condition causing compression of the esophagus by an aberrant subclavian artery. When combined with connective tissue disorders, it poses unique surgical challenges.
The aim of this study is to present the case of a patient with Loeys-Dietz syndrome who underwent a two-stage procedure encompassing the ligation and resection of the aberrant right subclavian artery. We additionally report a bailout technique for an intraoperative unexpected complication, surgical bailout solutions, and postoperative outcome.

Methods

A 47-year-old Caucasian woman was transferred to our institution with a thirty-year history of dysphagia secondary to an ARSA (figure 1). Past medical history was significant for LDS, Hashimoto's disease, hypothyroidism, and hypertension. Pre-operative CTA demonstrated an ARSA with a retroesophageal course, and a 2 cm Kommerell diverticulum (figure 2).

Results

A two-stage elective procedure was planned considering the risks and technical challenges associated with LDS. The first stage was an ARSA transposition to the RCCA via a supra-clavicular approach Following this, the proximal and distal RCCA was clamped. Immediately after clamping the RCCA, we noticed discoloration of the arterial wall with a blueish colour, consistent with dissection. A RCCA-to-RSCA bypass was performed using an 8-mm interposition Dacron graft. To further reinforce the suture line and avoid future dilatation of the RCCA we placed a 12-mm Dacron graft over the previous Dacron graft across the proximal anastomosis. A separate 8-mm Dacron graft was connected to the distal ARSA to the carotid graft.
Completion angiography revealed residual dissection just distal to the carotid graft anastomosis extending into the RCCA, stopping just proximal to the origin of RICA. The RCCA was stented using a 14 x 60 mm self-expandable stent proximally and a 7 x 30 mm self-expandable stent distally (figure 3).
The procedure was well tolerated, and post-operative CTA (figure 4) confirmed the patency of the vasculature and resolution of the extrinsic esophageal compression.

Conclusions

The presented case highlights the importance of a multidisciplinary approach and meticulous surgical planning when treating dysphagia lusoria in patients with underlying connective tissue disorders such as Loeys-Dietz syndrome.

Descending/Thoracoabdominal Aorta