A 76-year old male with a medical history of coronary artery disease, hypertension, and hyperlipidemia presented with worsening dysphagia and reflux over the past three months. He was diagnosed with type 2 achalasia on recent manometry. Patient had previously undergone 2 esophageal dilation treatments with minimal improvement. Most recent upper endoscopy demonstrated normal biopsies and mild narrowing of the gastroesophageal (GE) junction.
Patient also reported night sweats, low grade fevers (37.2-38.2) and 9 kg weight loss over the past 4 months. Vitals revealed tachycardia to the 130s. Physical exam was unremarkable. Labs notable for leukocytosis of 14.4 with a left shift, transaminitis (AST 139, ALT 191, alk phos 446), and hypercalcemia (12.3 corrected). On hospital day 2, patient’s WBC count elevated to 26, uric acid 10.1, LDH 639, and phosphate 6.6. Patient underwent CT head and neck that revealed diffuse cervical, mediastinal and axillary lymphadenopathy. Patient was started on treatment for laboratory tumor lysis syndrome with rasburicase and continued IVF. Core biopsy of axillary lymph node and bone marrow biopsy confirmed the diagnosis of stage IV diffuse B-cell lymphoma with bone marrow infiltration. Patient received R-CHOP and intrathecal methotrexate with notable improvement in dysphagia. On 4 month follow up, post 6 cycles of R-CHOP treatment, patient was noted to have clinical remission and resolution of lymphadenopathy.
Discussion:
Pseudoachalasia can be a unique presenting feature of malignancy. Etiologies include tumor invading the esophageal myenteric plexus, paraneoplastic syndrome, or external compression from a malignant mass near the GE junction. Symptoms of pseudoachalasia are nearly identical to primary achalasia with similar imaging and manometric findings. Esophageal manometry in both cases will show aperistalsis, elevated lower esophageal sphincter (LES) pressure, and incomplete LES relaxation. Sudden onset or rapid progression of symptoms should heighten suspicion for pseudoachalasia. Patients presenting with pseudoachalasia may also be older in age and present with more pronounced weight loss. CT imaging or biopsy from upper endoscopy can help determine the true etiology of symptoms. While the initial treatment for achalasia is esophageal dilation, dilations will not help in pseudoachalasia. It is important to differentiate pseudoachalasia from achalasia during the work up for dysphagia to prevent delay in appropriate treatment.
Conclusions:
The differential for dysphagia is broad. Pseudoachalasia should be considered in this differential when performing the work up for achalasia. Pseudoachalasia as a presenting feature of malignancy can be missed with negative biopsy findings and with manometry and symptoms presenting similarly to primary achalasia. In older patients with rapid symptom progression, CT imaging can be very helpful for prompt diagnosis and treatment.