Gastrointestinal: Successful Management of Refractory Achalasia Complicated by Delayed Diagnosis and Prolonged Sepsis

We report the case of a 51-year-old man who presented with a one-month history of lethargy, dyspnea, cough, and low-grade fever. An 18-month history of vague difficulty swallowing was elicited later. A chest x-ray revealed a dilated oesophageal shadow overlapping the right mediastinum containing debris and bilateral multifocal lung consolidation worse on the right. A chest CT scan (Figure 1) performed subsequently confirmed widespread airspace consolidation and a grossly dilated esophagus measuring 5.8 cm with marked tapering at the gastroesophageal junction (GOJ). The finding of dilated esophagus on imaging then prompted an endoscopy, which revealed a dilated tortuous esophagus containing particulate matter and a tight GOJ with ulcerations (Figure 2). A nasojejunal tube was left in situ for feeding, the patient nursed in the head-up position and was kept nil by mouth. Other measures were chest physiotherapy to assist in clearing respiratory secretions, adequate hydration, and oxygen therapy as needed. However, he developed multiresistant pseudomonas pneumonia (sensitive only to gentamicin) in the setting of Staphylococcus haemolyticus bacteremia and left pleural effusion requiring drainage. A tracheostomy was performed for hypoxic respiratory failure. An acid-fast bacillus was also isolated in the sputum. Bronchoalveolar washings and lung biopsy revealed chronic inflammation with identification of acid-fast bacilli on ZN staining. Culture on special media yielded growth of Mycobacterium thermoresistibile and an antimycobacterial regimen was started comprising amikacin, moxifloxacin, azithromycin, rifampicin, and ethambutol. Initial improvement in respiratory parameters were followed by deterioration, and repeated chest x-rays showed worsening consolidation, raising concern about recurrent aspiration. A repeat endoscopy showed persistently dilated esophagus containing large amounts of secretions. To address ongoing aspiration episodes, the patient underwent two sessions of pneumatic dilatation (PD) at the GOJ. The nasojejunal tube was removed and a percutaneous endoscopic gastrostomy (PEG) tube was inserted to facilitate feeding. However, all these measures failed to lead to any clinical improvement. Consequently, a peroral endoscopic myotomy (POEM) was performed. This resulted in immediate improvement in swallowing and the resolution of regurgitation. Within 3 weeks post-procedure, the patient was successfully weaned off the tracheostomy tube. The PEG tube was also removed, sepsis was resolved, and the patient was discharged following a tumultuous 10-month stay in the ICU. This case report highlights the difficulty of making a diagnosis and the complexity of managing a patient who presented with complications of achalasia. Dysphagia was not a prominent feature, and the diagnosis was further masked by prolonged sepsis and respiratory complications. Delayed diagnoses due to atypical presentation are not uncommon in achalasia and may lead to delayed treatment with sometimes catastrophic consequences [1]. The presence of clinical signs such as recurrent aspiration and regurgitation, along with radiological and endoscopic findings, prompted consideration of achalasia. As noted in many studies, a subset of individuals with achalasia may not respond effectively to PD [2]. Studies have shown that in patients with refractory achalasia following PD, fibrous stenosis hinders its success. These patients generally respond well to a myotomy [3]. There was immediate relief of dysphagia following POEM in our patient, and within a short while, his condition improved significantly, allowing him to head home. Our submission adheres to the journal‘s ethics and integrity policies. The authors confirm that they have secured all necessary patient consent forms. Patient has been informed that their name and initials will remain undisclosed in publications, with diligent efforts to safeguard their anonymity; however, complete anonymity cannot be guaranteed. The authors declare no conflicts of interest. Data supporting our findings will be made available upon request.