Woman With Dysphagia, Regurgitation, and Throat Burning

Medscape

2 days ago

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Background and Initial Presentation
A 56-year-old woman presents to the clinic with a 6-month history of intermittent dysphagia, primarily with solid foods, describing a sensation of "something stuck in her throat," along with a burning sensation in the throat and esophagus. She denies difficulty swallowing liquids. Her symptoms have remained relatively stable without significant progression over time. She also experiences gagging, coughing, nocturnal regurgitation, and congestion. She has a history of acid reflux that was treated with a proton pump inhibitor, which she is no longer taking. She takes antacids intermittently with minimal relief.
She has a history of congenital hiatal hernia, which was repaired at age 11 years. She denies any fever, chills, chest pain, shortness of breath, abdominal pain, unintentional weight loss, hemoptysis, nausea, vomiting, or blood per rectum.
On physical exam, the patient's abdomen was soft, non-tender, and non-distended. Her head, eye, ear, nose, and throat exams were also unremarkable, without a palpable neck mass. The rest of the physical exam was unremarkable. Basic labs were ordered, and the results, including thyroid function panel, were within normal limits.
Workup Results
The patient was prescribed a proton pump inhibitor and referred to a gastroenterologist. Given her persistent reflux symptoms without improvement despite anti-reflux medication, she was sent for esophagogastroduodenoscopy (EGD) to help stage her severity of reflux, to assess for laryngopharyngeal reflux, and to look for underlying structural abnormalities, such as recurrence of hiatal hernia. EGD also helps to evaluate for other potential, although less likely conditions, such as reflux esophagitis, Barrett's esophagus, or esophageal cancer.
While ECG may be helpful to evaluate for potential atypical presentation of cardiac causes for her symptoms, it is unlikely given her lack of risk factors such as diabetes or history of cardiac disease. Chest x-ray assesses lung disease and bony structures but is unlikely to provide the anatomical detail required to provide any useful information. Esophageal manometry is useful for assessing for motility disorders and is generally used for esophageal symptoms not diagnosed on EGD or for patients undergoing anti-reflux surgery.
EGD revealed mild gastritis without recurrence of hiatal hernia. Biopsies were negative. There was also no sign of laryngopharyngeal reflux, with no presence of pooling or residual material in the hypopharynx. Manometry was offered to the patient, but she opted for additional imaging.
To better assess other anatomical factors extrinsic to the gastrointestinal tract that may be causing the patient's symptoms, she underwent neck ultrasonography, which revealed a small right thyroid nodule (0.7 cm X 0.5 cm X 0.8 cm) without any concerning features.
She then underwent neck CT, which demonstrated an aberrant right subclavian artery originating from the aortic arch distal to the origin of the left subclavian artery, passing posterior to the esophagus while coursing toward the right. In addition, there is a fusiform aneurysmal dilatation of this aberrant right subclavian artery proximally, measuring approximately 1.6 cm in diameter, compared with a diameter of 0.6 cm more distally along the right subclavian artery (Figure 1).
Figure 1. Neck CT showing aberrant right subclavian artery.
The CT also demonstrated the sub-centimeter enhancing soft-tissue nodule within the right thyroid gland. No other pathologically enlarged or abnormally enhancing cervical lymph nodes were evident.
Discussion
Dysphagia lusoria is a rare condition caused by compression of the esophagus by an aberrant retroesophageal right subclavian artery. The right subclavian artery normally takes off as a branch of the brachiocephalic trunk, the most proximal great vessel of the aortic arch. Aberrant right subclavian artery (ARSA), however, takes off distal to the left subclavian artery, which is the most distal branch in a normal aortic anatomy. ARSA is the most common aortic arch anomaly, with a reported prevalence of 0.7%-2.0%.[1] It is caused by the involution of the right fourth vascular arch and proximal right dorsal aorta and the persistence of the seventh intersegmental artery originating from the proximal descending thoracic aorta.[2]
Esophageal cancer is unlikely, as no masses were found on EGD. Dysphagia megalatriensis is also unlikely because she has no history or clinical signs of cardiac disease (eg, mitral valve pathology or atrial fibrillation). Zenker diverticulum, or a weakening of mucosa and submucosa in the upper esophagus, can present with globus sensation or dysphagia but also generally presents with halitosis and is usually a disease of the 7th or 8th decade of life. Neither ultrasound nor CT demonstrated this anatomical pathology. Thyroid goiter causing compressive symptoms is usually of significant size and can be appreciated on exam.
The clinical presentation of dysphagia lusoria has significant heterogeneity. The most common symptoms are dysphagia, dyspnea, and chest pain.[3,4] It is more common in women than in men, with the mean age of symptom onset at 49.9 ± 19.4 years.[2] It is frequently associated with a Kommerell diverticulum, a dilation at the origin of the aberrant artery. The diverticulum results from failed regression of the previously mentioned fourth dorsal arch. The natural history of Kommerell diverticulum is unknown owing to the rarity of the condition, but both dissection and ruptures have been reported. In summary, dysphagia lusoria results from compression of esophagus by the retroesophageal aberrant right subclavian artery, which is an anatomical variant.
Surgical intervention is generally indicated for patients with compressive symptoms. In asymptomatic patients, there is no established indication for repair. Some have proposed repair if the diverticulum reaches a certain size, with surveillance in asymptomatic cases.
Some groups recommend surgical repair in all encountered cases of Kommerell diverticulum owing to a high rupture and dissection rate. In a single-center study of 45 patients, the average growth of Kommerell diverticulum was 1.45 ± 0.39 mm/year, with hypertension being the single statistically significant factor promoting growth.[5] The reported rupture and/or dissection rate at first encounter ranges broadly, from 0% to 50%.[1] A contained rupture of Kommerell diverticulum would lead to aortic hematoma.
There are no known associations between Kommerell diverticulum and esophageal cancer. Kommerell diverticulitis has not been reported. Diverticulitis, or inflammation/infection of the diverticulum, generally occurs in the colon. It is caused by obstruction, leading to venous congestion, inflammation, and infection. This pathophysiology is unlikely in a vascular structure with active flow such as the Kommerell diverticulum. Although dissection can occur with Kommerell diverticulum, leading to limb ischemia in flow-limiting cases, it would affect the right arm, not the left.
Surgical repair of an aberrant right subclavian artery should achieve two main goals: (1) exclusion or repair of any associated Kommerell diverticulum, which may pose a risk for aneurysmal degeneration or rupture, and (2) preservation or restoration of adequate perfusion to the right upper extremity, typically by revascularizing the right subclavian artery.[1,5] Traditionally, this had been done via an open approach, which requires thoracotomy or sternotomy. The diverticulum is then repaired with graft replacement of descending aorta, resection of aneurysm, or aneurysmorrhaphy. The right arm is then revascularized with either carotid to right subclavian bypass or transposition or re-anastomosis of right subclavian artery onto the aortic arch.[1]
With the advancement in endovascular techniques in the last two decades, a hybrid approach is becoming more common. It utilizes thoracic endovascular aortic repair (TEVAR) to exclude the Kommerell diverticulum, along with embolization or extra-anatomic bypass or transposition of the right subclavian artery, as mentioned above. This approach spares the patient from the more invasive thoracotomy or sternotomy, as the open portion can be performed via neck incisions only.
One potential complicating factor to endovascular repair is the small distance between the origins of the aberrant right subclavian artery and the left subclavian artery (Figure 2), preventing a good proximal seal zone of the stent graft without covering the left subclavian artery. In these cases, such as in this patient, a more advanced aortic stent graft can be used, such as a branched aortic endoprosthesis. This would allow the stent graft to extend into the left subclavian artery to maintain perfusion, achieve adequate seal, and exclude the Kommerell diverticulum (Figure 3). Alternatively, additional extra-anatomic bypass or transposition of the left subclavian artery can be performed.
Figure 2. Measurements of distance between aberrant right subclavian artery and left subclavian artery.
Figure 3. Kommerell diverticulum before and after repair by stent (a before repair; b and c after repair).
The other answers do not completely address the underlying problem, which include both the extrinsic compression of the esophagus by the retroesophageal aberrant right subclavian artery and the Kommerell diverticulum. An esophageal stent would exert additional force on the esophagus that may exacerbate the patient's symptoms, and it does not address the diverticulum. It will also put the patient at risk of stent erosion or migration. Percutaneous gastrostomy tube insertion would help with supplemental nutrition if the patient had severe dysphagia. However, this patient does not have severe dysphagia, and it would not relieve her symptoms or address their underlying cause. Right carotid to subclavian bypass without aortic repair will not exclude the diverticulum, which will continue to be pressurized by the aorta.
This patient underwent TEVAR with branched aortic endoprosthesis into the left subclavian artery, followed by right subclavian to right common carotid artery transposition. She had an uneventful postoperative recovery and was discharged after 3 days. At 1-month follow up, her symptoms had resolved, and CT angiography showed excluded and regressed Kommerell diverticulum with patent subclavian arteries bilaterally.
Given the rarity of the condition, no guidelines have been developed for follow-up surveillance after repair of aberrant right subclavian artery and Kommerell diverticulum utilizing a hybrid open and endovascular repair. However, because TEVAR was performed, the patient is at risk for the same postoperative complications as when it is performed for other aortic pathologies, so similar approaches can be taken in terms of postoperative surveillance. Current Society for Vascular Surgery guidelines recommend contrast-enhanced CT angiography at 1 month and 12 months after TEVAR for repair of descending thoracic aortic aneurysms, and then yearly for life, with consideration of more frequent imaging if an endoleak or other abnormality of concern is detected at 1-month follow-up.[6]