From: Myasthenia gravis with achalasia secondary to thymoma: a case report and literature review
 | Kaminski (1999) [16] | Kornizky et al. (2000) [13] | Rison (2009) [14] | Desuter et al. (2015) [11] | Seabi and Mokgoko (2020) [17] | Vinod (2020) [15] | The current study |
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Gender and age | F/ 75 | F/ 69 | M/ 83 | M/ 79 | M/ 35 | M/ 11 | M/ 43 |
Ethnicity | Unknown | Unknown | USA | Unknown | Unknown | Indian | Egyptian |
GIT symptoms | Food sticking in the upper chest and regurgitation of undigested food | Dysphagia and occasional non-specific retrosternal pain | Painless secretions in the back of his throat inhibit swallowing | Aphagia, followed by nasogastric feeding, then percutaneous endoscopic gastrostomy | Dysphagia | Persistent vomiting, difficulty swallowing, and regurgitation | Dysphagia (food sticking in the upper chest and regurgitation of undigested food) |
Other associated conditions | No | Polymyositis | DM2, arthritis, cervical spinal stenosis. Past peptic ulcers with a previous gastrointestinal bleed secondary to past steroid use. Left nephrectomy secondary to prior staghorn calculi, previous lumbar spine surgery, previous cholecystectomy, and previous bariatric surgery | No | No | No | No |
Acetylcholine antibody titer | 4.4 nmol/ L (negative if < 0.4 nmol/l) | Positive | 7.94 nmol/ L (negative if < 0.4 nmol/l) | N/A | N/A | 0.69 nmol/l (serum reference value < 0.25 nmol/l) | 7.9 nmol/l (negative if < 0.4 nmol/l) |
CT chest | Unremarkable | Unremarkable | Unremarkable | Unremarkable | N/A | Dilated esophagus | Anterior mediastinal soft tissue mass iso-dense in the non-contrast study with minute foci of calcification with homogenous enhancement in the post-contrast study |
Treatment of MG | Anticholinesterase medication Six session PE | Pyridostigmine | Pyridostigmine (60 mg PO q 4 h) and azathioprine (50 mg PO bid, for the last 8–9 years). PE in case of MG crisis | Anticholinesterase medication | Anticholinesterase inhibitor and immunosuppression with prednisone and azathioprine | Steroids, Acetylcholinesterase inhibitor (pyridostigmine), and nifedipine | Acetylcholinesterase inhibitor (pyridostigmine). Immunosuppression with prednisolone and azathioprine. PE in case of MG crisis |
Thymoma/type | Yes/ resection of mixed epithelial and lymphocytic thymoma one year before the symptoms Recurrent thymoma and carcinoid tumor in the lung | No thymoma | No thymoma | No thymoma | Yes/ resection of thymoma in 2017 | No thymoma | Yes/ resection of thymoma in 2022. Thymoma type B2, p T1 |
Esophageal motility study | Absence of lower esophageal sphincter relaxation with multiple peristalses and simultaneous contraction of the body | Absent peristaltic waves in the esophageal body, with a normal pressure in the LES (17 mmHg, normal 15–30) | Not done | Not done | Not done | Failure of the lower esophageal sphincter to relax, elevated basal pressure, and peristalsis of the esophageal body suggestive of achalasia cardia | HRM showed achalasia type II. Failure of LES relaxation, high resting LES pressure, and absent esophageal peristalsis (100%) with panesophageal pressurization to greater than 30 mmHg |
Barium swallow/esophageal transit study | Moderate esophageal dilatation | Marked dilated esophageal body with a beak-like narrowing of the terminal portion | Prominent cricopharyngeal sphincter with incomplete relaxation and no evidence of any cricopharyngeal bar | N/A | N/A | Dilated esophagus | Dilated esophagus with bird beak appearance |
Endoscopy findings | Not done | Not done | Not done | Primary aspiration did not reveal regurgitation or UES blockage, but it did identify a significant lack of hypopharyngeal contraction | N/A | Dilated esophagus | Dilated esophagus with retained fluid and spastic cardia |
Pathology/ histopathology | Loss of myenteric ganglion cells was confirmed/ no antibody binds to the Auerbach plexus | Not done | Not done | Not done | Not done | Â | Not done |
Management of dysphagia/ response to the treatment | Botulinum toxin injection Laparoscopic Heller myotomy. Improvement in swallowing and weight loss after the procedure | Prednisone and methotrexate. Some improvement in the dysphagia | Direct laryngoscopy followed by esophagoscopy with mechanical dilation of the cricopharyngeal sphincter. The patient’s swallowing markedly improved | Medical treatment of MG (pyridostigmine). The 4-year follow-up showed no deterioration in his oral feeding ability | N/A | Medical treatment for MG improves the dysphagia | Elective pneumatic balloon dilatation was successful and remarkably improved the patient’s symptoms |