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Vol. 8. Issue 4.
Pages 329-340 (July - August 2002)
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Vol. 8. Issue 4.
Pages 329-340 (July - August 2002)
ARTIGO ORIGINAL/ORIGINAL ARTICLE
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Traqueobroncopatia osteocondroplástica
Tracheobronchopathia osteochondroplastica
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João Moura e Sá*, José Almeida**, Joana Amado***, Beatriz Fernandes***, José Caminha*, José Miguel Ferraz****
* Assistente Hospitalar Graduado de Pneumologia
** Assistente Hospitalar de Pneumologia
*** Interna Complementar de Pneumologia
**** Chefe de Serviço de Pneumologia
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RESUMO

A traqueobroncopatia osteocondroplástica (TO) é uma doença rara e de etiopatogenia desconhecida. Caracteriza-se pela presença de múltiplos nódulos osteocartilagíneos na submucosa da árvore laringotraqueobrônquica, que se dispõem caracteristicamente na superfície luminal das suas paredes anteriores e laterais. A evolução é habitualmente benigna e silenciosa, pelo que a sua incidência in vivo (1:125 - 1:6000) poderá estar subestimada.

O aspecto broncoscópico típico de nódulos de consistência dura, tipo “grão de arroz” na submucosa e a confirmação histológica nos fragmentos biopsados são os elementos-chave para o diagnóstico.

Não se conhece terapêutica específica, sendo o tratamento, quando necessário, quase sempre exclusivamente sintomático. Numa minoria de casos cursa com complicações graves — infecção, hemoptises e obstrução—que poderão implicar o tratamento broncoscópico / cirúrgico das lesões.

Os autores procederam à análise retrospectiva da incidência, apresentação clínica e broncoscópica dos casos de TO diagnosticados na Unidade de Broncologia do Centro Hospitalar de Vila Nova de Gaia durante 14 anos, de 1987 a 2001.

Neste período foram realizadas 16 888 broncoscopias. Em 13 doentes (11 homens, 2 mulheres; idade média - 59,5±12,4 anos) foi diagnosticada acidentalmente traqueobroncopatia osteocondroplástica. Todos os casos, confirmados histologicamente, apresentaram o aspecto broncoscópico típico de TO e em nenhum foi necessário tratamento. Em quatro doentes identificaram-se simultaneamente neoplasias malignas do pulmão.

Na série descrita a incidência estimada de TO (1:1299 broncoscopias) sobrepôs-se à publicada na literatura e, tal como documentado por alguns autores, identificaram-se simultaneamente vários casos de neoplasias malignas do pulmão.

REV PORT PNEUMOL 2002; VIII(4): 329-339

Palavras-chave:
traqueobroncopatia osteocondroplástica
broncoscopia rígida
broncofibroscopia
traqueia
brônquios
ABSTRACT

Tracheobronchopathia osteochondroplastica (TO) is a rare disease of unknown pathogenesis and etiology. It is characterized by the presence of multiple submucosal osteocartilaginous nodules that involve luminal surfaces of anterior and lateral walls of the laryngotracheobronchial tree. In most cases the dis ease is benign and silent and its incidence in vivo (1:125 - 1:6000) is probably underestimated.

Typical bronchoscopic examination of submucosal hard nodules and histologic confirmation in biopsy specimens are essential in establishing the diagnosis. In the absence of specific treatment, management is aimed at symptoms relieve. A minority of patients develop serious complications — infection, hemoptysis and obstruction — and surgical or endoscopic excision may be necessary.

Incidence, clinical and bronchoscopic presentation of TO cases diagnosed in the Bronchology Unit of Centro Hospitalar de Vila Nova de Gaia during 14 years (since 1987 to 2001) were reviewed.

In this period, 16 888 bronchoscopies were performed. TO was incidentally diagnosed in 13 patients (11 men, 2 women; mean age - 59,5±12,4years). Bronchoscopy showed the characteristic endoscopic findings and all cases were histopathologically proven. None of the patients received treatment. Four patients presented with concomitant malignant lung cancer.

The incidence of TO in our series (1: 1299 bronchoscopies) is similar to that reported in the literature and several cases of malignant neoplasms associated with TO were identified as has been observed by other authors.

REV PORT PNEUMOL 2002; VIII(4): 329-339

Key-words:
tracheobronchopathia osteochondroplastica
rigid bronchoscopy
flexible bronchoscopy
trachea
bronchi
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