ABSTRACT
Pulmonary artery aneurysms are extremely rate and are seldom diagnosed during life. In true aneurysms, dilatation tends to be confined to the main trunk of the artery and degenerative changes in the wall are found. In 1971, Williams et al reported the first successful excision and replacement of the main pulmonary artery, including the bifurcation, with a dacron graft. Since then there have been few cases of main pulmonary artery aneurysm treated surgically. However, aneurysm of the main pulmonary artery treated surgically was not reported in our country till now. Recently we have experienced a case of the mainpulmonary artery aneurysm in a 10 years old boy and it was confirmed by echocardiogram, ultrasonogram of the chest, computed tomogram of the chest, radioisotope heart scan, cardiac catheterization and cine-angiogram of main pulmonary artery, aorta & right ventricle etc and patent ductus arteriosus was noted at the indifferent site from aneurysmal sac. Thereafter he underwent surgical correction at our hospital with the aid of total cardiopulmonary by-pass.
Subject(s)
Child , Humans , Male , Aneurysm , Aorta , Arteries , Cardiac Catheterization , Cardiac Catheters , Dilatation , Ductus Arteriosus, Patent , Heart , Heart Ventricles , Polyethylene Terephthalates , Pulmonary Artery , Thorax , Transplants , UltrasonographyABSTRACT
Histiocytic Medullary Reticulosis(H.M.R.) is a rapidly progressing fatal disease seen most often in adults, but it has been reported in children younger than 15 years of age. H.M.R. is clinically characterized by fever, wasting generalized lymphadenopathy and hepatosplenomegaly. In the terminal stage, jaundice, purpura, anemia and pancytopenia are all present with or without skin involvement. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the lymphoreticular tissues. Four cases of H.M.R. occurring in pediatric age group, i.e., 5 years, 6 years, 12 years and 14 years of age, respectively, are presented with discussion of the clinico-pathologic characteristics and management. Cases 1,2 and 3 died within 6 months after the onset of illness. Case 1 of our series was particularly younger than in previously reported cases. This was the case that the diagnosis was confirmed by postmortem examination. Analysis of previously reported 25 cases occurring in ages younger than 20 years of age was done.