P150. Hybrid Surgical Treatment of Dysphagia Lusoria in a patient with Loeys-Dietz Syndrome
McGovern Medical School at UTHealth
Houston, TX
United States - Contact Me
Avanced Aortic Surgery Fellow at Memorial Hermann Hospital, Houston.
I currently live in Houston, where I am completing a two- year Fellowship in advanced aortic surgery. I am greatly engaged in open surgery for aortic aneurysms and dissections, as well as in modern vascular surgery, with large interest in vascular innovations, latest technology in vascular and aortic endovascular surgery, and greatly interested in medical and academic education, as well as surgical simulation.
I previously joined La Fe University Hospital in 2014 as a junior physician in angiology and vascular surgery, within the department of vascular surgery.
Prior to La Fe, I was the first Spanish junior vascular surgeon to complete a senior clinical Fellowship in aortic surgery at St. Mary’s Hospital, Imperial College of London, during an 18- month period,
and one of the first Spanish surgeons to become an official fellow of the European board of vascular and endovascular surgery (FEBVS).
Prior to this experience, I completed my residency in vascular surgery at Gregorio Marañón University hospital in Madrid, during a five- year period.
During my tenure in Madrid, I helped the hospital with the medical and residents training program, and participated in several national and international simulation courses, workshops, and meetings.
I am a graduate of the University of Alcala in Madrid, where I majored my bachelor in medicine.
Description
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.
Authors
Lucas Ribe (1), Yuki Ikeno (1), Lucas Ruiter (2), Thanila Macedo (3), Rana Afifi (4), Akiko Tanaka (5), Gustavo Oderich (1)
Institutions
(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