ABSTRACT
A traqueopatia osteocondroplástica (TO) é uma desordem idiopática, incomum, caracterizada pela presença de nódulos osteocartilaginosos na submucosa das vias aéreas, causando rigidez e estreitamento da árvore respiratória. Afeta principalmente homens acima dos 50 anos com manifestações clínicas devidas à obstrução e/ou a infecções locais. Sua patogênese é desconhecida. Relatamos um caso de TO encontrada acidentalmente em autopsia de mulher com 73 anos de idade, que apresentava carcinoma ductal biliar extra hepático (tumor de Klatskin).
Tracheopathia osteochondroplastica (TO) is an unusual idiopathic disorder, characterized by osteocartilaginous nodules in the submucosa of the respiratory airway, which causes rigidity and narrowing of the respiratory tree. It affects mainly men over 50 and clinical manifestations are due to obstruction and/or local infections. The pathogenesis is uncertain. We report a case of TO found incidentally in an autopsy of a 73 year-old woman, who had an extrahepatic biliary ductal carcinoma (Klatskin tumor).
Subject(s)
Humans , Female , Aged , Bronchial Diseases , Tracheal Diseases/etiology , Tracheal Diseases/pathology , Osteochondrodysplasias/pathology , Autopsy , Case Reports , Chondrogenesis , Tracheal Diseases/diagnosis , Klatskin Tumor , Ossification, HeterotopicABSTRACT
We treated a rare case of TO that presented with recurrent massive hemoptysis that resulted in total obstruction of the bronchus intermedius by very large blood clots. Bronchoscopic intervention resulted in a full recovery from the atelectasis. However, there are no guidelines for preventing recurrence of the hemoptysis or disease progression. Conservative and expectant management are used to treat these patients and most do well.