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A 63-year-old man with a 3-month history of progressively worsening dysphagia to solids and liquids and a weight loss of 20 kg was referred following high-resolution manometry (HRM). He was only able to tolerate moderately thick liquids. There was no history of abdominal pain, chest pain, heartburn or night sweats. He had no medical history of GI reflux or chronic alcohol abuse however he was an ex-smoker with a 40-pack year history. He had a brother with a diagnosis of pancreatic cancer at the age of 60. On examination, his vital signs were stable, and his abdomen was soft and flat without tenderness.
Laboratory tests revealed a normal haemoglobin, white cell count and ferritin. The patient had a gastroscopy demonstrating a tight lower oesophageal sphincter (figure 1) and a barium swallow showing suspicion of achalasia (figure 2). The patient eventually underwent HRM (figure 3).
What is the diagnosis?
This patient was initially thought to have achalasia. Figure 1 shows severe intrinsic stenosis and the barium swallow demonstrated focal narrowing of the distal oesophagus just above the gastro-oesophageal junction. There was no significant upstream oesophageal dilatation. HRM demonstrated spastic peristalsis, premature contractions fitting Chicago 4 classification for type III achalasia. Rapid drink challenge showed inducible obstruction at the lower oesophageal sphincter. However, given the rapid weight loss and acute onset of symptoms, a repeat gastroscopy was performed. This time the lower oesophageal sphincter could be traversed and a fungating mass extending to the gastro-oesophageal junction was found. Biopsies revealed an undifferentiated carcinoma with focal neuroendocrine differentiation. Positron emission tomography scan demonstrated gastro-oesophageal junction uptake but no lymphadenopathy or metastatic disease.
There is overlap in the symptoms for achalasia and oesophageal stricture, in particular dysphagia and weight loss. This case highlights the importance of careful endoscopic evaluation of the gastro-oesophageal junction for mucosal assessment and biopsy in order to exclude malignancy, particularly in patients with rapid weight loss and a smoking history before considering idiopathic achalasia. A CT scan of the chest is another complementary investigation to rule out structural lesions. In this male, a repeat gastroscopy was performed rather than a CT scan in order to obtain tissue to help guide further treatment.
It is difficult to distinguish between mechanical oesophago-gastric obstruction secondary to a tight distal oesophageal stricture from type III achalasia on HRM. It is important to distinguish between these two pathologies in order for patients to have definitive treatment. Treatment modalities for achalasia include pneumatic dilation, laparoscopic Heller myotomy and peroral endoscopic myotomy, whereas malignant oesophageal strictures include endoscopic stenting, surgery, radiation and chemotherapy.
The patient went on to have an oesophageal stent inserted as a bridge for chemoradiotherapy. His swallow improved and he was able to tolerate soups, yoghurt and pudding.
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Correction notice This article has been corrected since it published Online First. The order of authors has been corrected and figure legends amended.
Contributors YSK collected data, history and investigation findings to formulate a manuscript. MW critically reviewed and edited case report, collected investigation results, provided and cared for the patient of the case report. RH critically reviewed and edited case report.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.