Abstract Background Dysphagia lusoria (DL) is characterised by swallowing difficulty due to external oesophageal compression from congenital aortic arch malformations. It is clinically rare and affects less than 2.5% of the population. The most prevalent form of DL is the aberrant right subclavian artery (ARSA) where approximately 10% of patients experience dysphagia. Despite AL being congenital, dysphagia often manifests later in life, likely due to age-related atherosclerotic changes in the vasculature and decreased oesophageal flexibility. Method Presented is a case report of a female in her seventies where, despite repeat hiatal hernia repair and maximal medical management, her index symptoms of dyspepsia and retrosternal discomfort persisted when reviewed in specialist upper gastrointestinal clinic. She underwent extensive investigation; however, her symptoms were unremitting, manifesting in numerous emergency department presentations to exclude acute coronary syndrome. This case report details the anatomical differences via diagrams and documents the comprehensive investigation and multi-disciplinary approach to the management of dysphagia in ARSA. Results Prior to referral to our team, the patient had a laparoscopic hiatus hernia repair and Nissen fundoplication undertaken. Oesophageal manometry revealed weak oesophageal motility and 24-hour pH monitoring did not demonstrate pathological acid-reflux. Recurrence of hiatus hernia was observed on oral contrast swallow and gastroscopy. Subsequently, the patient underwent laparoscopic revision of hiatus hernia and conversion of Nissen to a Dor. Post-operatively her symptoms required complete fundoplication reversal. Following reversal, assessment demonstrated improved distal oesophageal emptying. Her CT emphasised an ARSA causing significant compression and deviation of the proximal oesophagus. The patient was referred onwards to vascular surgery. Conclusion Initial diagnostic workup for patients presenting with dysphagia in the upper gastrointestinal clinic seeks to encompass several common aetiologies including oesophageal motility disorders, malignant compression and benign strictures. Clinicians must consider less common causes of extrinsic compression, such as ARSA, particularly in patients with unrelenting and persistent symptoms despite appropriate implementation of both medical and surgical interventions. Treatment approaches for ARSA reflect symptom severity, with both conservative and surgical options considered.
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