Case report
Dysphagia aortica in a young patient with Behcet’s Disease: Case report

https://doi.org/10.1016/j.ajg.2019.07.001Get rights and content

Abstract

Dysphagia aortica is a rare aetiology of dysphagia resulting from an abnormality in thoracic aorta that causes extrinsic compression on the oesophagus. Dysphagia aortica includes aortic aneurysm, aortic dissection or even tortuous aorta and is seldom considered in the differential diagnosis of dysphagia.

Herein, we report a 30-year-old man with Behcet’s disease who presented with rapid progressive dysphagia and diagnosed as dysphagia aortica caused by saccular aortic aneurysm complicated by large para-aortic haematoma compressing the oesophagus. The case reveals the importance of early and proper identification of the rare causes of dysphagia in young adults with complaint of dysphagia and history of recurrent oral and genital ulcers in absence of obvious cardiovascular diseases.

Introduction

A 30-year-old man presented with dysphagia of four weeks duration. The patient was referred to our Department of Internal Medicine in Sohag University Hospital for clinical evaluation and to perform oesophagogastroduodenoscopy (EGD). The patient was distressed and history was obtained from his accompanying relative. The patient developed dysphagia to solid food of four weeks duration with acute onset and progressive course. Three weeks later, he developed dysphagia to fluids. Dysphagia was associated with chest tightness and there was no other cardiopulmonary symptoms. On admission, the patient was alert, distressed, average body built, haemo-dynamically stable, and was in a knee chest position and on oxygen therapy. There were no dilated superficial veins on neck or chest wall. No other significant physical abnormality was detected. Initial laboratory investigations were unremarkable. Additionally, ECG was within normal.

Oesophagogastroduodenoscopy was performed a few hours after admission. The procedure was performed in left lateral position under conscious sedation and nasal oxygen using EG-2985k scope with 9.8 mm insertion tube diameter (Pentax, Tokyo, Japan). It revealed dilatation of the proximal part of the oesophagus, and showed minimal retained food particles. At the middle third of the oesophagus, 30 cm from the upper incisors, there was a significant luminal narrowing. The endoscopic picture was highly consistent with extrinsic compression of the oesophagus (Fig. 1, Fig. 2). The compressed part of the oesophagus showed congested bluish mucosa and had a kinked course with visible pulsations. We passed the scope through the narrow part of the oesophagus very slowly with great caution. The distal oesophageal mucosa and the oesophagogastric junction showed no significant abnormality. Examination of the stomach and duodenum also showed no significant abnormality. On withdrawal of the scope, there was visible blanching and refilling the compressed sub-mucosal vessels (Fig. 3). We did not take oesophageal biopsies. Computed tomography (CT) scan of the chest and echocardiography were recommended.

Trans-thoracic echocardiography revealed severe compression of the left atrium by large haematoma measuring 5.7 × 7.8 cm with no other significant cardiac abnormality (Fig. 4). Chest CT scan with oral and IV contrast showed oesophageal obstruction by large para-aortic haematoma measuring 5 × 6 × 10 cm and saccular dilatation of the descending thoracic aorta measuring 3 × 3 cm (Fig. 5, Fig. 6).

Based on the clinical presentation and the imaging findings, we hypothesized that our patient fits the diagnosis of dysphagia aortica which got aggravated by development of large para-aortic haematoma. Cardiothoracic consultation was requested and they decided referral of the patient to a higher cardiothoracic center at Cairo University for further evaluation and endovascular management.

We communicated by telephone with the relative of the patient who informed us that the patient had past history of recurrent oral and genital ulcers in addition to ocular inflammation. The last attack of oral and genital ulcers had been 8 months ago. There was no past history of DVT or similar condition. The family history was unremarkable. Based on the clinical and radiological evaluation and history of the patient, diagnosis of Behcet’s disease was hypothesized. In the referred cardiothoracic center in Cairo University, the patient received an aortic stent and three days later he gradually resumed oral feeding.

Section snippets

Discussion

Dysphagia is a common complaint among the general population, especially the elderly group. Extrinsic oesophageal compression caused by vascular structure is an uncommon presentation of dysphagia. Dysphagia aortica is a rare aetiology of dysphagia resulting from extrinsic compression of the oesophagus by tortuous thoracic aorta, thoracic aortic aneurysm or aortic dissection as a result of age related degeneration. It was first described by Pape in 1932 [1]. Dysphagia aortica is rare and seldom

Reference (12)

  • International Study Group for Behcet’s disease

    Criteria for diagnosis of Behcet’s disease

    Lancet

    (1990)
  • R. Pape

    Uber einen abnormen verlauf (‘tiefe Rechtslage’) der mesa aotitischen aorta descendens

    Fortschr Roetgenstr

    (1932)
  • H. Lau

    Dysphagia aortica: harbinger of aortoesophageal fistula?

    Surgery

    (2000)
  • P.G. Keates et al.

    Dysphagia associated with sclerosis of the aorta

    Br J Radiol

    (1955)
  • R.L. Baron et al.

    Clinical and radiographic manifestations of aortoesophageal fistulas

    Radiology

    (1981)
  • S. Amin et al.

    Aortoesophageal fistula: case report and review of the literature

    Dig Dis Sci

    (1998)
There are more references available in the full text version of this article.

Cited by (0)

View full text