[Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation]. / Malformation adénomatoïde kystique congénitale du poumon: à propos de trois cas de révélation tardive.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.

[Acute respiratory failure as the sol inaugural sign of Arnold-Chiari malformation. Two cases]. / Insuffisance respiratoire aiguë isolée révélant une malformation d'Arnold-Chiari.

Arnold-Chiari malformation is an occipitocervical malformation where the cerebellar amygdales descend below the occipital foramen. Acute respiratory failure is an exceptional inaugural sign. We report two cases disclosed by alveolar hypoventilation associated with type I Arnold-Chiari malformation. The two patients age 51 and 52 years had an uneventful past history and presented with hypercapnic encephalopathy with acute respiratory failure requiring ventilatory assistance. Respiratory function tests, helicoidal thoracic computed tomographic angiography, electromyogram, cardiac echography, and thyroid and immunological tests were normal. Blood gases and polysomnography were in favor of central hypoventilation without sleep apnea. Magnetic resonance imaging demonstrated type I Arnold-Chiari malformation. The course was complicated by recurrent respiratory failure in both patients. Surgical decompression performed for the first patient provided no improvement. This patient died two months after surgery subsequent to aspiration pneumonia. The second patient was treated with continuous positive pressure noninvasive ventilatory assistance and had a good outcome at 25 months. These two cases illustrate the absence of any neurological sign, acute respiratory failure being the only sign of Arnold-Chiari malformation.

[Hydatid pulmonary embolisms. Seven cases]. / L'embolie pulmonaire hydatique. A propos de 7 observations.

Hydatid pulmonary embolism is a rare condition. Diagnosis is difficult and there is no consensus on treatment. Prognosis is generally poor. We conducted a retrospective analysis of patients hospitalized between 1994 and 2002 who had a diagnosis of hydatid pulmonary embolism. Seven cases were identified. All patients had a cyst which spontaneously ruptured into the bloodstream. All patients were symptomatic. Chest x-ray revealed images of metastatic pulmonary echinococciasis in all patients. Thoracic CT and/or angioscan provided the diagnosis in five patients. In one, the diagnosis was established with pulmonary angiography. For one other, the diagnosis was established at the pathology examination of the surgical specimen. Searching for the primary localization of the hydatid disease revealed hepatic cysts in all patients, associated in one with a cyst in the right atrium. The cardiac cyst was not operated and all hepatic cysts were removed. Embolectomy was performed in one patient. Medical treatment with albendazole was given in five patients. The clinical course was marked by death in two patients, due to abundant hemoptysia. The five other patients are followed regularly and were doing well at six months to five years. The diagnosis of hydatid pulmonary embolisms is basically obtained with imaging. Treatment is surgical resection of the embologenic focus then embolectomy. The role of medical treatment is not clearly defined. The short- and mid-term prognosis is generally poor.

[A new case report of spinal cord compression secondary to beta-thalassemia]. / Un nouveau cas de compression médullaire secondaire à une bêta-thalassémie.

Extramedullary hematopoiesis is an unusual cause of spinal cord compression. We report the case of a 39-year-old man who presented symptoms of progressive dorsal spinal cord compression. Hemogramm showed erythrocytosis with microcytosis. Hemoglobin electrophoresis revealed heterozygous beta-thalassemia. Diagnosis of extramedullary hematopoiesis was based on magnetic resonance imaging findings. Radiological and therapeutic options in this rare condition are briefly discussed.

[Pleurodesis. Indications and review of symphyseal agents]. / La pleurodèse. Indications et revue des agents symphysants.

Recurrent or chronic pneumothorax and neoplasm of the pleura are the major indications for pleurodesis. However certain chronic or recurrent forms of pleural affections of mechanical origin or secondary chylothorax may not respond to aetiologic treatment and could benefit from therapeutic pleural symphysis. The objective may be reached with several physical and chemical agents including cyclins and talcum powder among the most effective products devoid of major risk. The classic surgical techniques have been abandoned in favour of interventional thoracoscopy to obtain pleural abrasion and pleurectomy. Promising results have been obtained.