A Rare Case of Esophageal Stenosis in Behcet's Disease

Background: Behcet's Disease (BD) is a multi-organ inflammatory disease characterized by aphthous oral ulcers, ocular manifestations and genital lesions. Various environmental and genetic factors contribute to the pathogenesis of BD, however the specific etiology remains unclear. Case:A 71-year-old female with a history of BD, pharyngeal ulcers, esophageal stenosis and tracheostomy presented to the emergency department with concerns of food being stuck in her throat for 4 days and inability to swallow. She has a history of dysphagia for many years, but was tolerant of thin liquids and soft foods. BD management consists of chronic steroids and previous dilation with balloon tracheoplasty. She is aphonic due to aphthous ulceration of the larynx. Fiberoptic nasal endoscopy and esophagogastroduodenoscopy were attempted with findings included a surgically absent larynx, unidentifiable upper esophageal sphincter and post-surgical scarring. These explorative studies were later aborted due to failure of scope advancement. A neck CT illustrated present trach and medially displaced carotid AA bilaterally without frank masses. Following recommendation of clinical swallow examination, contrast material passed into the pharynx and entered the upper portion of the airway; it appeared to remain in the glottis above the vocal folds. Patient attempted to swallow with minimal hyoid movement, however, a full swallow reflex was never achieved. Nutritional support with PEG tube was recommended and placed with no complications. Patient was subsequently discharged. Conclusion:This case illustrates the unique presentation of esophageal stenosis secondary to BD and warrants further investigation of the pharyngolaryngeal involvement in disease classification and progression.