[Intramural hematoma of the thoracic aorta: Medical treatment or surgery?]. / Hématome intramural de l'aorte thoracique : traitement médical ou chirurgie ?

Intramural hematoma (IMH) of the aorta is an uncommon entity. This disease shares many characteristics with acute aortic dissection. Treatment of IMH remains controversial. We report the case of a 58 years old man with hypertension disease who was admitted in emergency department with suspicion of acute aortic dissection. Transoesophageal echocardiography showed IMH involving the descending aorta which spread afterwards to the ascending aorta. Patient was treated medically and echocardiographic follow-up showed that aortic hematoma remains stable. Two years later, patient is alive and the last TEE reveals disappearance of hematoma in ascending aorta and decrease of it in descending aorta.

Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease.

The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.

[Epithelioid hemangioendothelioma mimicking hypersensitivity pneumonitis]. / Hémangioendothéliome épithélioïde pulmonaire mimant une pneumopathie d'hypersensibilité

Epithelioid haemangioendothelioma is a rare vascular tumour of slow growth and unfavourable outcome. The diagnosis of the pulmonary localisation is difficult and can mimic by clinical and radiological features other diagnosis as hypersensitivity pneumonitis. We report the case of a 28-year-old man, farmer handling with palm tree pollens, admitted to the hospital for dry cough. Clinical and thoracic computed tomography findings revealed diffuse infiltrating pneumopathy; bronchoalveolar lavage results and professional exposure were suggestive for hypersensivity pneumonitis. Surgical lung biopsy with immunochemistry study concluded to pulmonary epithelioid haemangioendothelioma. Extra pulmonary localisation research was negative. No treatment was indicated. At three years, the patient is steel asymptomatic. Epithelioid haemangioendothelioma is a tumour of intermediate malignancy, of which pulmonary localisation has a nonspecific clinical presentation mimicking diffuse infiltrating pneumonitis. Diagnosis is essentially made by surgical lung biopsy with pathological and immunohistochemical study.

[Acute chest syndrome as the inaugural sign of sickle cell anemia. A case report and review of the literature]. / Syndrome thoracique aigu révélateur d'une drépanocytose. A propos d'un cas avec revue de la littérature.

Acute chest syndrome is a frequent complication of sickle cell disease. This syndrome is characterized by recent infiltrate on chest X-ray with chest pain or fever or dyspnea. We report the case of a 26-year-old man in whom an acute chest syndrome with fat embolism was the inaugural sign of sickle cell disease. This report illustrates the frequency of potentially serious fat embolism in the acute chest syndrome and the importance of bronchoscopy and bronchoalveolar lavage (fatty macrophages) for determining the etiology of acute chest syndrome.