[Marfan disease and cardiovascular pathology].
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Biomedical subjects
Publications and source records attributed to J O Defraigne.
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We report the case of an infected aneurysm of the abdominal aorta in a 18 month-old child, discovered by routine palpation of the abdomen during hospitalization for pneumonia. Ultrasonography and arteriography showed a 6 cm aneurysm of the abdominal aorta beginning distal to the renal arteries which occluded the right common iliac artery. The aneurysm was treated by interposing a 6 mm Gore-Tex graft between the infrarenal aorta and the aortic bifurcation. Pathologic examination of the aneurysmal wall demonstrated a leukocytic infiltrate and the presence of encapsulated Gram positive organisms. Arterial aneurysms are exceedingly rare in children. Their etiology is varied: infection, connective tissue disease, trauma, inflammatory arterial disease or other rare diseases such as tuberous sclerosis, neurofibromatosis, or Behçet's disease.
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Five cases of Marfan's syndrome with cardiovascular lesions are presented. Among these five patients, two with type I aortic dissection, one with true aneurysm of the ascending aorta and one with aortic regurgitation underwent composite grafting of the ascending aorta according to the Bentall technique. The fifth patient died preoperatively, due to extensive dissection of the transverse aorta. Marfan's syndrome is transmitted in an autosomal dominant manner. Fifty percent of the patients die at a mean age of 32 years, and 95 p. 100 of the deaths are caused by cardiovascular lesions. These lesions predominantly affect the left cardiac valves (aortic or mitral regurgitation) and the ascending aorta. Except in emergencies, replacement of the ascending aorta by a composite graft must be envisaged in subjects with Marfan's syndrome presenting with aortic regurgitation and dilatation of the aortic root.
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A case of idiopathic aortic thrombosis in a term neonate is reported. Ultrasound examination led to diagnosis and an embolectomy was carried out. Literature data concerning etiology and treatment of neonatal aortic thrombosis are reviewed.
A case of solitary plasmacytoma of the thyroid is reported. A monoclonal component (IgG lambda) was identified in the serum of the patient and disappeared after total thyroidectomy and radiotherapy. The use of the immunoperoxidase technique on thyroid, bone marrow, and lymph node specimens allowed us to demonstrate the production of IgG lambda by the tumor, and to rule out the possibility of multiple myeloma. Previously reported cases are reviewed (15 cases). The diagnostic criteria, the association with thyroiditis, and the treatment of this rare disorder are discussed.
A 55-year-old patient with primary pulmonary leiomyosarcoma was treated by pneumonectomy. The diagnosis was established by histology of the operative piece. Bronchoscopy and cytology of expectorations were negative. This rare tumor (fewer than 100 cases described) differs from bronchial carcinoma by its method of spread, mainly hematogenic, and its better prognosis. clinical and radiographic data do not provide differential diagnoses. Treatment is essentially surgical since the tumor is only weakly radio-sensitive and responds poorly to chemotherapy.
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