Thymoma complicated with myasthenia gravis and Good syndrome - a therapeutic conundrum: a case report.

BACKGROUND: Thymomas are known to be associated with myasthenia gravis and Good syndrome. Good syndrome is the association of thymoma with combined B cell and T cell immunodeficiency. The combination of all three diseases has not been reported. We discuss the therapeutic dilemma of immunosuppression in such a case. CASE PRESENTATION: A 27-year-old Sinhalese man was evaluated for persistent cough which was associated with pleuritic chest pain and was found to have pleural-based lesions in his left hemithorax. Further evaluation confirmed these lesions to be implants from a thymoma. He subsequently developed myasthenia gravis and impending myasthenic crisis precipitated by pneumonia. He was found to have hypogammaglobulinemia with low B cell counts, confirming a diagnosis of Good syndrome. Treatment with intravenously administered broad-spectrum antibiotics, acetylcholinesterase inhibitors, orally administered glucocorticoids, plasma exchange, and intravenous immunoglobulin led to clinical improvement. He subsequently underwent thymectomy and debulking of the tumor and was maintained on regular intravenous immunoglobulins combined with low-dose prednisolone. CONCLUSIONS: Regular intravenous immunoglobulins combined with low-dose immunosuppression in addition to thymectomy appear to be safe when myasthenia gravis occurs in association with Good syndrome.

Ortner's syndrome presenting as thoracic aortic aneurysm mimicking thoracic malignancy: a case report.

INTRODUCTION: Ortner's syndrome is defined as left recurrent laryngeal nerve palsy caused by a cardiovascular pathology. Here we report the case of a 68-year-old man who presented to our hospital with hoarseness, whose initial chest imaging mimicked a thoracic neoplastic process with left pleural effusion. The final diagnosis was Ortner's syndrome due to the silent rupture of a thoracoabdominal aortic aneurysm. Diagnostic thoracentesis, before computed tomography, in resource-poor settings, may have resulted in an adverse outcome in our case. CASE PRESENTATION: A 68-year-old Sri Lankan man was referred to us by an otolaryngologist for further evaluation of a suspected thoracic malignancy. His presenting symptom was hoarseness of three months duration. He had essential hypertension for the last four years and had a history of 25 pack-years of cigarettes smoking. His chest X-ray showed a left-sided mediastinal mass with mild to moderate pleural effusion. An ultrasound appeared to show an encysted pleural fluid collection. However, we proceeded with computed tomography before diagnostic thoracentesis. The diagnosis of Ortner's syndrome was made after the computed tomography due to the silent rupture of his thoracoabdominal aortic aneurysm. CONCLUSIONS: Hoarseness due to left recurrent laryngeal nerve palsy can be the presenting symptom of cardiovascular pathologies, Ortner's syndrome. Silent rupture of thoracic aortic aneurysms can mimic that of thoracic malignancy, which is reported in literature. We illustrate the importance of a high degree of suspicion of cardiovascular pathology in order to avoid an adverse outcome following diagnostic thoracentesis.

Minithoracotomy oesophagectomy for oesophageal carcinoma with aberrant right subclavian artery: a rare case of dysphagia.

BACKGROUND: Aberrant R/subclavian artery is a rare congenital anomaly involving aortic arch. Oesophageal carcinoma with associated aberrant R/subclavian artery is very rare and only few cases has been reported in literature. If unrecognized and injured during oesophageal surgery, it can lead to disastrous complications. When associated with oesophageal carcinoma, it can cause diagnostic confusion as the symptoms are similar. CASE PRESENTATION: A 60 year old previously healthy female presented with intermittent dysphagia, odynophagia and loss of weight of 3 months duration. She was found to have a oesophageal carcinoma with incidentally co-existing aberrant R/subclavian artery. CONCLUSION: Although rare this entity should be considered as a differential diagnosis in a patient with dysphagia. In addition, pre-operative identification is important to prevent intra operative vascular complications. The diagnosis and treatment of this rare condition is discussed in this article.

Accuracy of two scoring systems for risk stratification in thoracic surgery.

We investigate the suitability of the two existing risk stratification systems available for predicting mortality in a cohort of patients undergoing lung resection under a single surgeon. Data from the 290 consecutive patients who underwent pulmonary resection between January 2008 and January 2011 were extracted from a prospective clinical data base. In-hospital mortality risk scores are calculated for every patient by using Thoracoscore and ESOS.01 and were compared with actual in-hospital mortality. The receiver operating characteristic (ROC) curve was used to establish how well the systems rank for predicting patient mortality. Actual in-hospital mortality was 3.1% (n = 9). Thoracoscore and ESOS values (mean ± SEM) were 4.93 ± 0.32 and 4.08 ± 0.41, respectively. The area under the ROC curve values for ESOS and Thoracoscore were 0.8 and 0.6, respectively. ESOS was reasonably accurate at predicting the overall mortality (sensitivity 88% and specificity 67%), whereas Thoracoscore was a weaker predictor of mortality (sensitivity 67% and specificity 53%). The ESOS score had better predictive values in our patient population and might be easier to calculate. Because of their low specificity, the use of these scores should be limited to the assessment of outcomes of surgical cohorts, but they are not designed to predict risks for individual patients.

Early outcomes of surgery for oesophageal cancer in a thoracic regional unit. Can we maintain training without compromising results?

OBJECTIVES: Meaningful exposure to oesophageal cancer surgery during general thoracic surgical training is restricted to few centres in the United Kingdom. Our Regional Tertiary Unit remains a rare 'large-volume' oesophagectomy centre. We aimed to determine the proportion of patients operated by trainees and their perioperative outcomes. METHODS: From January 2004 to September 2009, 323 patients (229 male and 94 female, median age of 69 (range 40-92) years) underwent oesophagectomy for carcinoma in our Thoracic Surgical Unit. Data were complete and obtained from a prospective departmental database. The preoperative characteristics, operative data and postoperative results were compared between the 120 patients (37%) operated by a trainee (group T) and the remainder 203 patients operated by a consultant (group C). RESULTS: The overall incidence of mortality, anastomotic leak and chylothorax were 6.5%, 5.3% and 2.2%, respectively. There were no differences in terms of age, gender, tumour location, tumour staging, preoperative spirometry or use of neoadjuvant chemotherapy between the two groups. There was no significant difference between the consultant group and the trainee group in the following key outcome measures: postoperative mortality (8% vs 4%), incidence of respiratory complications (30% vs 25%), hospital stay (14 days vs 13 days) and number of lymph nodes excised (median of 16 vs 14). CONCLUSIONS: Training in oesophageal cancer surgery can be provided in a large-volume thoracic surgical unit. It does not seem to compromise outcomes or use of resources.