Caught in a vice

Abstract

Esophageal dysmotility and dysphagia are well known in patients with scleroderma. Interstitial lung disease (ILD) in these patients is an indication for lung transplantation but is considered high risk in many centers. This report is an attempt to highlight how anatomical causes can contribute to dysphagia in such patients and complicate the post-operative course after lung transplantation. Such a finding is uncommon in this subset of patients and use of suitable imaging can help in arriving at the diagnosis. We present a patient following lung transplantation for scleroderma related ILD with an aberrant right subclavian artery compressing the esophagus in a vice like grip. Imaging is the key to prompt diagnosis and management.

Introduction

Vascular rings are rare congenital problems that present in the pediatric population. Although several variations of vascular rings exist, all have similar manifestations- mostly involving esophageal or tracheal compression such as dysphagia, dyspepsia, recurrent upper respiratory tract infections, or unremitting cough [1]. We describe a patient who presented with feeding difficulties after lung transplantation requiring readmission. Radiological studies uncovered an aberrant right subclavian artery (ARSA) as the putative factor [2].

Case report

A 69-year-old woman with a past medical history of Chagas Disease, Scleroderma associated interstitial lung disease (ILD), moderate pulmonary hypertension, type 2 diabetes mellitus and hypothyroidism, underwent a single right lung transplant. After transplantation she had a relatively uncomplicated course and was discharged on the 10th post-operative day. Two months later the patient presented with severe dyspepsia, dysphagia, and esophageal reflux. She was unable to tolerate oral intake.

A nuclear medicine study revealed moderately delayed gastric emptying of solids with 82% and 46% of radionuclide label remaining in the stomach at 2 and 4 h, respectively [3]. Upon review of a high-resolution contrast chest Computerized Tomography (CT), an ARSA was noted to arise from the aorta traversing posterior to the esophagus and compressing it. The current symptoms correlated with this anatomical finding. The patient failed to respond to pro-kinetic and antiemetic therapy. She was therefore treated by insertion of a percutaneous endoscopic gastrostomy-jejunal feeding tube (PEG-J). This allowed for nutrition to be provided while also aiding gastric decompression.

Discussion

Scleroderma is a disease process with several debilitating symptoms and affects approximately 240 per million population [4]. Lungs are one of the major systems; Interstitial Lung Disease and Pulmonary Hypertension being the most common subsets, responsible for over 60% of Scleroderma related deaths [5]. Scleroderma also presents with esophageal dysfunction due to scarring of the esophagus. Some reports put the incidence of this presentation as high as 26% in all scleroderma patients and most of them tend to co-exist with the pulmonary disease [6]. As a matter a fact, according to Crespo et al., many scleroderma patients are declined for lung transplantation due to their extra pulmonary manifestations, the most feared of which is esophageal dysmotility and gastro-esophageal reflux which puts recipients at risk for aspiration. The Crespo et al. study has shown a higher rate of post-operative esophageal dysmotility after lung transplantation [7].

Another pathology that is relevant to this case is Chagas disease. Achalasia of the esophagus presents in 7–10% of the people infected with the pathogen Trypanosoma cruzii [8]. Our patient underwent routine transplant evaluation prior to listing and did not have features of Achalasia. Prior to transplant, during evaluation, she underwent both a manometry and a pH study. The manometry reported an elevated mean residual Lower Esophageal Sphincter (LES) pressure. The patient was evaluated and cleared by the gastroenterology team prior to transplantation. The patient was found to have some reflux, which was managed medically.

A high-resolution contrast chest CT scan revealed an ARSA. The aberrant right subclavian artery comes off the aorta distal to the left subclavian artery and travels behind the esophagus, indenting it (Figs. 1a, b, 2a, b and 3a), to cause symptoms. The incidence of aberrant subclavian artery in the normal population is between 0.5–2% [9]. Radiological studies helped visualize this anomaly.

Fig. 1.

a Posterior view reconstruction of CT at level of aortic arch. Esophagus is shown in red with aberrant right subclavian artery shown wrapping around the esophagus and compressing it. Here we can see the branches of the aorta. The first is a bovine arch giving rise to the right common carotid and the left common carotid. The second branch is the left subclavian artery. b Left lateral view reconstruction of CT at level of aortic arch. Esophagus is shown in red. The bovine arch is shown in this image. The ARSA which is the third branch from the aortic arch takes a hairpin-like turn and dives medially thus traveling posterior to the esophagus and indenting it along its course

Fig. 2.

a Here, we see an axial cross section of the thorax providing a view of the aberrant subclavian artery (the radiolucent structure) traversing posterior to the esophagus.. Posterior to the trachea is the esophagus compressed by the aberrant subclavian artery. b This cross sectional view of the thorax demonstrates the ARSA in relation to the left subclavian artery- two separate branches but they are remarkably close in their origin from the aorta

Fig. 3.

a A coronal slice of the CT scan showing us the exact location of the ARSA- around the level of the tracheal bifurcation (T4). This view also demonstrating the esophagus being impinged by the ARSA. b Modified barium Swallow- The contrast stream is observed narrowing in the esophagus demonstrating that this is esophageal indentation. The oblique course of the ARSA is what gives the impression of the ‘wide’ indentation

A barium swallow study was performed (Fig. 3b) and confirmed the vascular vice. Our primary concern for the patient was for her to gain adequate nutrition. Hence, after the ARSA was identified, we arranged for the patient to have a PEG-J for feeding. Following insertion of the PEG-J tube the patient’s nutritional requirements were met and this also allowed symptomatic relief.

From a surgical standpoint, ARSA presenting as vascular rings around the esophagus can be solved in two ways: open repair or an endovascular hybrid approach [9, 10]. The endovascular approach is an attractive alternative in the current era. The ARSA’s vice-like grip on the esophagus might have contributed to the patient’s dysphagia in addition to her pre-existing medical conditions.

It is likely that she had other contributing factors, and this was an incidental finding; we however wanted to highlight the presence of an anatomical factor also which is known to play a role in feeding difficulties. The purpose was to highlight ARSA in this case since we tend to apportion blame to known medical conditions. We wish to state that it helps to keep an open mind and perhaps look for anatomical causes also in the evaluation phase. Physicians also tend to be caught in a vice when it comes to treating patients with known conditions. What the mind does not know the eyes cannot see!

Authors’ contributions

All authors contributed equally to the manuscript and read and approved the final version of the manuscript.

Funding

None.

Declarations

Conflict of interest

The Authors report no conflicts of interest for this manuscript.

IRB approval

The Temple IRB decided that this report was NHRS (Non-human research).

Human and animal rights statement

No animals or humans were used nor harmed for this research manuscript.

Informed consent

Informed consent was obtained from the patient prior to the procedure and their permission was also obtained for the publication of their material sans name or identification.