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

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.     

An unusual cause of dysphagia: thoracic aorta aneurysm

The vascular structure related compression of esophagus is rather rare. Aberrant right subclavicular artery accounts for the majority of the rare entity, while the thoracic aorta aneurysm is a more dangerous type, called as dysphagia aortica. Delay in diagnosis and treatment of the dysphagia aortica predisposes to rupture and death. Herein, we reported a female patient with thoracic aorta aneurysm. A quick diagnosis by using chest contrast computed tomography (CT) scan and angiography of heart was made, and followed by emergent surgery. In the process, there was no delay on the diagnosis and treatment. The patient is going on well in the follow up.KEYWORDS : Dysphagia, thoracic aorta aneurysm, dysphagia aortica, saccular aneurysm, 128-slice computed tomography (CT) angiography     

Benign esophageal lesions: Endoscopic and pathologic features

Benign esophageal lesions have a wide spectrum of clinical and pathologic features. Understanding the endoscopic and pathologic features of esophageal lesions is essential for their detection, differential diagnosis, and management. The purpose of this review is to provide updated features that may help physicians to appropriately manage these esophageal lesions. The endoscopic features of 2997 patients are reviewed. In epithelial lesions, the frequency of occurrence was in the following order: glycogenic acanthosis, heterotopic gastric mucosa, squamous papilloma, hyperplastic polyp, ectopic sebaceous gland and xanthoma. In subepithelial lesions, the order was as follows: hemangioma, leiomyoma, dysphagia aortica and granular cell tumor. Most benign esophageal lesions can be diagnosed according to their endoscopic appearance and findings on routine biopsy, and submucosal lesions, by endoscopic resection. Management is generally based upon the confidence of diagnosis and whether the lesion causes symptoms. We suggest endoscopic resection of all granular cell tumors and squamous papillomas because, while rare, these lesions have malignant potential. Dysphagia aortica should be considered in the differential diagnosis of dysphagia in the elderly.     

Thoracic Endovascular Aortic Repair With Subclavian Revascularization for Symptomatic Nonaneurysmal Aberrant Right Subclavian Artery

Aberrant right subclavian artery is a common aortic arch anomaly that can cause dysphagia as a result of compression by the aberrant artery. For patients with an aneurysm associated with an aberrant right subclavian artery, surgical or endovascular intervention is a well-described treatment. However, for patients with a nonaneurysmal aberrant right subclavian artery, treatment with thoracic endovascular aortic repair has been limited. We describe the use of thoracic endovascular aortic repair and subclavian revascularization to treat esophageal stricture in a patient with a symptomatic nonaneurysmal aberrant right subclavian artery. The patient''s dysphagia was successfully relieved after the operation.     

Dysphagia aortica

Three patients with dysphagia caused by compression of the distal esophagus by a tortuous nonaneurysmal atherosclerotic aorta are described. All three patients were elderly women; systemic hypertension and cardiomegaly were present in two patients. Barium studies of the esophagus showed displacement and compression of the distal esophagus by the thoracic aorta. Debilitating dysphagia was treated surgically in one patient. The other two patients had milder symptoms and were managed conservatively. Esophageal manometry in these three patients showed superimposed pulsations and elevated intraluminal pressure just proximal to the lower esophageal sphincter. To evaluate the significance of these manometric findings and their correlation with clinical symptoms, we reviewed manometric tracings in 47 normal subjects. Ten of these subjects had an elevation of baseline intraluminal esophageal pressure as a result of superimposed vascular pulsations. We conclude that (1) compression of the distal esophagus by a tortuous atherosclerotic aorta in the appropriate setting can lead to clinically significant dysphagia and (2) a manometric finding of vascular compression of the esophagus does not necessarily correlate symptomatic dysphagia.     

Combined surgical and catheter‐based treatment of extensive thoracic aortic aneurysm and aortic valve stenosis

An extensive thoracic aortic aneurysm (TAA) is a potentially life‐threatening condition and remains a technical challenge to surgeons. Over the past decade, repair of aortic arch aneurysms has been accomplished using both hybrid (open and endovascular) and totally endovascular techniques. Thoracic endovascular aneurysm repair (TEVAR) has changed and extended management options in thoracic aorta disease, including in those patients deemed unfit or unsuitable for open surgery. Accordingly, transcatheter aortic valve replacement (TAVR) is increasingly used to treat patients with symptomatic severe aortic valve stenosis (AS) who are considered at high risk for surgical aortic valve replacement. In this report, we describe the combined surgical and catheter‐based treatment of an extensive TAA and AS. To our knowledge, this is the first report of hybrid TAA repair combined with TAVR. © 2014 Wiley Periodicals, Inc.     

Dysphagia aortica: A neglected symptom of aortoesophageal fistula

Aortoesophageal fistula, secondary to thoracic aortic aneurysm, is an uncommon cause of gastrointestinal bleeding that is uniformly fatal without surgical intervention. Typical symptoms are midthoracic pain and/or dysphagia followed by a usually short, albeit unpredictable, symptom-free interval and by a 'herald' haemorrhage, which is observed in 80% of patients before fatal exsanguinations. Dysphagia is present in 45% of patients, sometimes for several weeks, before the first bleeding occurs. However, dysphagia aortica is rarely considered in the differential diagnosis of dysphagia and lack of awareness, as well as symptom's underevaluation, both contribute to a significant diagnostic and therapeutic delay. We present a case of a 77-year-old woman who died for a bleeding AEF consequent to a thoracic aortic aneurysm and whose main symptom during the past 2 months was dysphagia, which was not taken seriously into consideration by her general practitioner. This case report emphasises that primary care physicians should be alerted to evaluate carefully the alarming symptoms like dysphagia -- especially in elderly patients -- before life threatening complications occur, as they are the ones who could suspect early the diagnosis and make a proper referral.     

Pseudoachalasia: A peculiar case report and review of the literature

Pseudoachalasia is a rare secondary achalasia, which accounts for only a small subgroup of patients. We describe a 77-year-old woman with recent onset of dysphagia and typical esophageal manometric findings of achalasia. Moreover, esophageal manometric findings of vascular compression at 36 cm from the nose were associated with dysphagia. An upper endoscopy showed the absence of lesions both in the esophagus and gastro-esophageal junction, whilst a 15-mm ulcer on the gastric angulus was detected. The gastric ulcer resulted in being a diffuse signet ring cell carcinoma at histology, suggesting pseudoachalasia. An abdominal computed tomography scan showed an irregular concentric thickening of the gastro-esophageal junction wall extending for 7 cm and a dilated ascending thoracic aorta with no presence of the inferior vena cava, with an enlarged azygos as the source of vascular compression of esophagus. Moreover, cardia involvement from diffuse signet ring cell carcinoma of the gastric angulus was also recognized as the cause of dysphagia. The cancer was not suitable for a surgical approach in an old patient with cardiovascular comorbidities and support therapy was started. In our ambulatory series, pseudoachalasia was eventually diagnosed in 4.7% of 234 consecutive patients with esophageal manometric finding suggestive of achalasia. We also reviewed cases in the literature and aimed to evaluate the reported causes of pseudoachalasia.     

TEVAR for non‐aneurysmal thoracic aortic pathology

Objective : Management of penetrating atherosclerotic ulcers (PAU), intramural hematomas (IMH), and acute aortic dissections (AD) of the thoracic aorta remain controversial in the endovascular era. Methods : Between 2001 and 2007, patients with PAU (13 patients), and/or IMH (7 patients) were treated with thoracic endografts (TEVAR) in the endovascular suite under general anesthesia. Indications for intervention were intractable chest pain, expanding hematoma or contained rupture, or distal malperfusion. End‐points were early morbidity and mortality, incidence of endoleak, device‐related complications, and secondary interventions. Results : Of the 20 patients with a median age of 67 (25–83), 13 (65%) were men, 2 (10%) had contained aortic rupture, and 10 were symptomatic. One patient had carotid‐subclavian bypass debranching before endograft implantation. Ten patients had cerebrospinal fluid drainage. Mean length of aorta treated was 122.1 mm (range 36–300). All endografts were technically successful. Average blood loss was 50 mL. Thirty‐day mortality was 0%. Symptoms resolved in all patients; there were no neurologic complications. Average length of stay was 5 days. Mean follow‐up was 2.0 years (range 0.1–5.8). All patients remained asymptomatic. Three had early (<180 days) endoleaks: Two type II and 1 type I treated successfully with an additional cuff, which was the only patient requiring reintervention. Two patients had late (>180 days) endoleaks (type 2) observed with no aortic expansion. Two deaths at 5.4 and 5.8 years were due to severe aortic valve stenosis and metastatic lung cancer. Conclusion : TEVAR is a feasible option for repair of non‐aneurysmal thoracic aortic pathology with resolution of symptoms, no mortality, and no neurologic complications. © 2009 Wiley‐Liss, Inc.     

Endografts (or stent-grafts) and diseases of the descending thoracic aorta

The endovascular treatment of aorta diseases with S-Graft is considered as an alternative to surgery, especially interesting in patients with severe comorbidities. Indeed, the mid-term morbidity and mortality are comparable to surgery in relatively large series, and S-Graft implantation appeared as a safe, less invasive and efficient treatment for different affections of the thoracic aorta. This article reviews technical aspects, indications and results of endovascular repairs of thoracic aorta lesions. We will also assess the advantages and limitations of S-Graft therapy.     

Endovascular Repair of a Traumatic Thoracic Aortic Injury Resulting in Late Aortic Dilatation

We present an unusual complication following endovascular repair of traumatic thoracic aortic injury using a homemade stent-graft. Although an initial technical success was apparently obtained, the aorta showed remarkable dilatation without shortening or migration of the stent skeleton in late follow-up period. The possible cause of the dilatation was considered to be the expansion and gathering of the crimped, loosely attached, graft fabric in addition to the incomplete exclusion due to the insufficient landing zone and the limited radial force. Thus, the specific feature of stent-graft, considered to provide an advantage to treatment, has adversely affected the native aorta.     

Esophageal schwannoma with tracheal compression

An esophageal schwannoma was found in a 73-year-old woman with cough, exertional dyspnea, and progressive dysphagia. Chest imaging showed an upper mediastinal mass (apex, right thoracic cavity) with direct lower tracheal compression. Esophagography and esophagoscopy revealed a tumor in the cervical and upper thoracic esophagus; it was resected from the upper thoracic esophagus followed by cervical esophageal repair. Histology showed oval-to-fusiform cells with palisading and lymphocytic stromal infiltration (immunohistochemistry, S-100 protein-positive). The patient was well one year afterward.     

Transluminal endovascular stent grafting of aortic dissections and aneurysms: a concise review of the major trials

Transluminal endovascular repair of the vascular system is rapidly emerging as a leading therapeutic modality for aortic dissections and aneurysms. The use of transluminal endovascular stent grafting has been used to intervene successfully on the aorta and its major branch vessels. There have been several studies examining transluminal endovascular stent grafting of thoracic aortic aneurysms, dissections, and abdominal aortic aneurysms. This paper reviews some of the major trials of transluminal endovascular stent grafting as well as its major limitation, endoleak.     

Two-stage combined endovascular and surgical approach for recurrent thoracoabdominal aortic aneurysm.

PURPOSE: To present a 2-stage combined endovascular and surgical approach for recurrent thoracoabdominal aortic aneurysm (TAAA). CASE REPORT: A 78-year-old man with previous surgical repairs of infrarenal abdominal and descending thoracic aortic aneurysms was referred for dysphagia due to an enlarging 9-cm aneurysm extending from the mid thoracic to the suprarenal aorta. Because no suitable endograft was available, an open repair was attempted, but the presence of a "frozen" chest made the redo procedure extremely difficult. A 2-stage treatment was thus decided upon. First, a retrograde bifurcated bypass graft was implanted from the abdominal aortic graft to the superior mesenteric and celiac arteries. Twenty days later, the TAAA was successfully excluded with a stent-graft, during which spinal fluid drainage was performed to prevent paraplegia. At 6 months, computed tomography showed patency of the endoprosthesis and visceral grafts. At 1 year, the patient remains asymptomatic. CONCLUSIONS: This case illustrates that a 2-stage combined endovascular and surgical approach may be a safe and effective alternative to reoperation for recurrent TAAA.     

Objective Measures of Swallowing Function Applied to the Dysphagia Population: A One Year Experience

Quantitative, reliable measures of swallowing physiology can be made from an modified barium swallowing study. These quantitative measures have not been previously employed to study large dysphagic patient populations. The present retrospective study of 139 consecutive patients with dysphagia seen in a university tertiary voice and swallowing clinic sought to use objective measures of swallowing physiology to (1) quantify the most prevalent deficits seen in the patient population, (2) identify commonly associated diagnoses and describe the most prevalent swallowing deficits, and (3) determine any correlation between objective deficits and Eating Assessment Tool (EAT-10) scores and body mass index. Poor pharyngeal constriction (34.5 %) and airway protection deficits (65.5 %) were the most common swallowing abnormalities. Reflux-related dysphagia (36 %), nonspecific pharyngeal dysphagia (24 %), Parkinson disease (16 %), esophageal abnormality (13 %), and brain insult (10 %) were the most common diagnoses. Poor pharyngeal constriction was significantly associated with an esophageal motility abnormality (p < 0.001) and central neurologic insult. In general, dysphagia symptoms as determined by the EAT-10 did not correlate with swallowing function abnormalities. This preliminary study indicates that reflux disease is common in patients with dysphagia and that associated esophageal abnormalities are common in dysphagic populations and may be associated with specific pharyngeal swallowing abnormalities. However, symptom scores from the EAT-10 did not correspond to swallowing pathophysiology.     

Dysphagia,reflux and related sequelae due to altered physiology in scleroderma

Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.     

Dysphagia of vascular origin

Three adult patients presented with dysphagia due to vascular compression of the esophagus. In one case, a dysphagia aortica was diagnosed. In the remaining two cases a congenital vascular anomaly--aberrant right subclavian artery and right aortic arc, respectively--was proved by arteriography. The final diagnosis was suspected after the barium meal and confirmed by computarized tomography in each case.     

Right pulmonary artery occlusion secondary to a dissecting aortic aneurysm

This report describes a case of right pulmonary artery obstruction caused by a dissecting aneurysm of the thoracic aorta. Initial ventilation-perfusion (V/Q) scan revealed no perfusion to the right lung. Lack of right lung perfusion plus other clinical abnormalities led to an initial diagnosis of massive embolism in the right lung. Bilateral pulmonary arteriography of the thoracic and abdominal aorta revealed extrinsic compression of the right pulmonary artery near its origin and the suggestion of an ascending aortic dissection. Subsequent ascending aortography and computed tomography (CT) confirmed a dissection of the ascending aorta. Aortography in the true lumen of the aorta revealed trivial aortic insufficiency. These diagnostic procedures confirmed the presence of an acute dissecting aneurysm of the ascending aorta that caused compression of the right pulmonary artery. The finding of a massive unilateral segmental defect with normal ventilation upon lung scanning does not always ensure a diagnosis of acute pulmonary embolus. Patient presentation with massive unilateral perfusion defect merits further evaluation with pulmonary arteriography.     

Complication of hybrid treatment in type B aortic dissection diagnosed by echocardiography

This case illustrates an unusual and fatal complication after endovascular treatment of type B aortic dissection and highlights the role of echocardiography in the early diagnosis of complications. In this case, a patient with previous diagnosis of chronic type B aortic dissection and moderate aortic regurgitation underwent endovascular repair of the proximal descending aorta and conservative surgical correction of the aortic valve. On early postoperative, a transesophageal echocardiogram and aortic angiotomography demonstrated proximal endoleak by contrast extravasation around the proximal graft attachment site, causing compression of the stent in its middle portion, resulting in narrowing with reduced cross‐sectional area.     

Abnormal solid bolus swallowing in the erect position

The routine use of solid boluses in the radiologic evaluation of the pharyngoesophagus has not been described in the literature. Because esophageal perforations have been reported as a result of delayed passage of caustic medications, this study was performed to determine the prevalence of solid bolus delay in a routine symptomatic radiologic population. Solid bolus erect swallowing was performed using either a 13 mm barium tablet or a 10 mm bagel bread sphere; occasionally, both were used. All individuals referred for an upper gastrointestinal (GI) examination or barium swallow who complained of dysphagia, heartburn, or chest pain were evaluated with a solid bolus. Any individual demonstrating gastroesophageal reflux, hiatal hernia, Schatzki's B ring, or esophageal motility disturbance was given a solid bolus as well. Individuals swallowing a sphere showed four times more frequent proximal pharyngoesophageal delay than tablet swallowers. The tablet arrested initially more frequently at both the aorta and lower esophageal sphincter than did the sphere. However, there was twice the total incidence of arrest of all swallowed spheres compared to tablets at the aorta. Approximately the same total number of spheres arrested at the lower esophageal sphincter as tablets. Any delay that allows a solid bolus to be overtaken in the erect position by the peristaltic contraction wave can be considered abnormal. The delays usually occur at anatomic narrowings. A sphere is more sensitive than a tablet in evaluating solid bolus pharyngoesophageal dysfunction in the erect position. Opinions and assertions contained herein are those of the authors and do not represent the official position of the U.S. Navy, Uniformed Services University of the Health Sciences or the Department of Defense.