Radical surgical resection for giant primary mediastinal endodermal sinus tumour with pulmonary metastasis after chemotherapy: can be curative.

Primary non-seminomatous germ cell tumours of anterior mediastinum are uncommon. Endodermal sinus tumour of the anterior mediastinum (yolk sac) is a rare but lethal neoplasm. We present a case of an 18-year-old man who presented with chest pain, cough and haemosputum with markedly raised serum α-fetoprotein (AFP) levels above 112,000 ng/mL. Chest roentgenogram and CT showed a giant anterior mediastinal mass. CT guided biopsy revealed a diagnosis of endodermal sinus tumour. After the completion of chemotherapy, extensive surgical resection was carried out along with the right lung metastastectomy. Five years postresection follow-up the patient is disease free with normal serum tumour markers. This is the longest survival ever reported of such tumours with highest AFP level (>112,000 ng/mL) and lung metastasis.

Tracheal diverticulum: a rare cause of hoarseness of the voice.

Tracheal diverticulum is a rare benign disease also known as paratracheal air cyst. Mostly these are asymptomatic and are discovered incidentally on radiologic examination of the chest. Common symptoms are cough, recurrent respiratory tract infections, and sometimes dysphagia. Herein we report a rare case of hoarseness of voice caused by tracheal diverticulum. Computed tomography scan of neck revealed a large tracheal diverticulum, probably compressing the recurrent laryngeal nerve as bronchoscopy showed impaired right vocal cord movement. Two weeks after diverticulectomy, the patient's voice returned to normal.

Lithobezoar, a rare cause of acute oesophageal obstruction: surgery after failure of endoscopic removal.

Oesophageal lithobezoar is a very rare clinical entity, especially presenting as a case of acute dysphagia. A 54-year-old man, with long-standing psychiatric illness, presented with a recent episode of vomiting and chest pain followed by dysphagia. Chest x-ray revealed a radio opaque shadow in the posterior mediastinum. Endoscopy showed a calcified foreign body in the lower oesophagus. Endoscopic attempts to remove or fragment the foreign body were unsuccessful. Foreign body was removed successfully through oesophagotomy using left posterolateral thoracotomy. Pathology revealed oesophageal lithobezoar.

The use of selective bronchography in predicting reversal of neoplastic obstructive atelectasis.

OBJECTIVES: To assess the ability of selective bronchography to predict which patients with neoplastic postobstructive atelectasis will respond to interventional therapies directed at the reexpansion of the affected lung. Furthermore, to compare the utility of selective bronchography with the current predictive standard that reversal of postobstructive atelectasis is unlikely when it is > or = 4 weeks in duration (ie, the 4-week rule). DESIGN: A prospective observational study. SETTING: A tertiary care referral center/medical school. PATIENTS: Twenty-seven consecutive patients with advanced lung cancer or other malignancy, with documented neoplastic postobstructive atelectasis involving a total of 44 lobes. INTERVENTIONS: Lobar collapse was documented radiographically. The duration of atelectasis was investigated and quantified as accurately as possible. Prior to the use of interventional therapies, selective bronchography was performed on each collapsed lobe, and the results were documented. Bronchography results did not influence the decision to proceed with interventional therapies. Patients had each of their collapsed lobes manipulated by interventional techniques that were directed at reexpansion of the lung. One week after the patient underwent the intervention, the degree of reexpansion was assessed radiographically. RESULTS: Interventional therapies leading to significant reversal of airway narrowing were completed in all 44 lobes. These were successful in reexpanding 28 of 44 collapsed lobes (64%). Selective bronchography demonstrated the following two distinct patterns: an intact bronchial tree (ie, tree pattern); or the absence of a distinguishable, distal bronchial tree (ie, blush pattern). The sensitivity of selective bronchography to predict reexpansion is 1.00 (95% confidence interval [CI], 0.90 to 1.00), and its specificity is 0.56 (95% CI, 0.30 to 0.80). There were no complications attributable to selective bronchography. The sensitivity of the 4-week rule to predict reexpansion is 0.61 (95% CI, 0.41 to 0.78), and its specificity is 0.75 (95% CI, 0.48 to 0.93). The results of selective bronchography and use of the 4-week rule were significantly different in predicting which lobes would reexpand and which would not (p = 0.0026). Using selective bronchography to predict the reversal of lobar atelectasis, the positive predictive value of the tree pattern was 0.80 and the negative predictive value of the blush pattern was 1.00. The values for the 4-week rule are 0.81 and 0.52, respectively. CONCLUSIONS: Selective bronchography is a useful tool for predicting whether patients with neoplastic postobstructive atelectasis would benefit from interventional techniques that are directed at lobar reexpansion. Selective bronchography appears to be superior to the 4-week rule in this regard.

Sickle cell disease, extreme hyperbilirubinemia, and pericardial tamponade: case report and review of the literature.

OBJECTIVE: Sickle cell disease is a relatively common disease seen predominantly in the African-American population with numerous important sequelae that require critical care management. We report a patient who presented with intrahepatic cholestasis, a rare and potentially lethal complication of sickle cell disease. DESIGN: Individual case report and review of the literature. SETTING: Medical intensive care unit of a tertiary care hospital. PATIENT: A 37-yr-old African-American male, with known sickle cell disease, who developed fulminant hepatic failure with subsequent extreme hyperbilirubinemia, coagulopathy, and pericardial tamponade. Additional organ dysfunction included renal insufficiency, respiratory failure, and cardiac dysrhythmias. INTERVENTIONS: The patient underwent serial measurements of complete blood count, hepatic profile, coagulation profiles, and hemoglobin electrophoresis. The patient received exchange transfusion, and his hemoglobin S concentration gradually decreased. Coagulopathy and anemia were corrected with transfusion of fresh frozen plasma and packed red blood cells. Serum bilirubin and other hepatic variables gradually improved. Pericardial tamponade was suggested by right heart catheterization measurements and diagnosed by echocardiographic findings. The tamponade resolved after pericardiocentesis and was managed by drainage through the pericardial catheter. Pulmonary, renal, and cardiac sequelae resolved with intensive supportive care that included intubation, mechanical ventilation, pulmonary artery catheterization, continuous renal replacement therapy, and permanent cardiac pacemaker. The patient was discharged home on hospital day 23 and has not required further hospitalization. CONCLUSION: Sickle cell intrahepatic cholestasis, a potentially fatal complication of sickle cell disease, can present with abdominal pain, acute hepatomegaly, coagulopathy, hyperbilirubinemia, and fulminant hepatic failure. Prompt recognition and early intervention with exchange transfusion and intensive supportive care of multiple organ dysfunction can result in a favorable outcome.