Doença cística traqueobrônquica

Abstract

Cistos aéreos que emergem anormalmente da traquéia e/ou brônquios de maior calibre são doença extremamente rara. Na literatura encontram-se poucas descrições de casos isolados de cistos paratraqueais associados a pneumopatias crônicas. Relatamos dois casos dessa entidade clínica, rara, em membros de uma mesma família: o pai das pacientes faleceu dessa doença com 43 anos de idade e as duas filhas encontram-se em tratamento. O quadro clínico é semelhante ao da supuração pulmonar crônica. O diagnóstico foi firmado através de broncovideoscopia e tomografia computadorizada de tórax com reconstrução em 3D e broncoscopia virtual. As pacientes estão sob observação e tratamento clínico paliativo.

Infected tracheal diverticulum: a rare association with alpha-1 antitrypsin deficiency

“…A tracheal diverticulum is a benign outpouching of the tracheal wall that is rarely diagnosed in routine clinical practice, and there are few reports of cases in the literature. Tracheal diverticula can be single or multiple ( 1 - 9 ) and can present either as ovoid pedunculated cysts or sessile formations, their longest axis measuring up to 3 cm, communicating with the trachea by channels. ( 9 ) Concerning etiology, tracheal diverticula can represent congenital anomalies of the tracheobronchial tree or acquired outpouchings from a weakened tracheal wall, ( 1 , 2 , 4 , 5 , 8 - 10 ) the two conditions differing mainly in relation to the site of implantation and the histological features of the wall.…”

“…Tracheal diverticula can be single or multiple ( 1 - 9 ) and can present either as ovoid pedunculated cysts or sessile formations, their longest axis measuring up to 3 cm, communicating with the trachea by channels. ( 9 ) Concerning etiology, tracheal diverticula can represent congenital anomalies of the tracheobronchial tree or acquired outpouchings from a weakened tracheal wall, ( 1 , 2 , 4 , 5 , 8 - 10 ) the two conditions differing mainly in relation to the site of implantation and the histological features of the wall. ( 3 , 6 , 11 ) Congenital tracheal diverticula are usually smaller and feature a narrower communication with the trachea than do those that are acquired.…”

“…( 3 , 6 , 11 ) Congenital tracheal diverticula are usually smaller and feature a narrower communication with the trachea than do those that are acquired. ( 1 - 3 , 5 , 8 , 9 , 11 - 13 ) The former are frequently right-sided, occurring 4-5 cm below the vocal cords or a few centimeters above the carina, ( 3 , 4 , 13 - 15 ) and are histologically similar to the tracheal wall, comprising respiratory epithelia, smooth muscle, and cartilage, whereas acquired tracheal diverticula are composed exclusively of respiratory epithelia. ( 3 , 11 ) The lack of cartilage in the acquired form is thought to be due to its origin, because it is caused by increased intra-luminal pressure or by the weakening of the structures after surgical procedures, ( 10 , 13 ) resulting in evagination of the mucous membrane through vulnerable points in the trachea.…”

“…( 3 , 11 ) The lack of cartilage in the acquired form is thought to be due to its origin, because it is caused by increased intra-luminal pressure or by the weakening of the structures after surgical procedures, ( 10 , 13 ) resulting in evagination of the mucous membrane through vulnerable points in the trachea. ( 2 , 3 , 9 , 11 , 12 ) Therefore, acquired tracheal diverticula can appear at any level, although they typically occur along the right posterolateral wall ( 6 , 16 , 17 ) near the thoracic inlet. ( 1 , 2 , 8 )…”

“…( 1 - 3 , 5 - 8 , 11 , 12 , 19 ) Consequently, the reported incidence varies depending on the diagnostic tool used. For example, in a postmortem study, the incidence was estimated to be approximately 1%, ( 1 , 4 , 6 - 9 , 11 , 13 , 14 ) compared with 0.3% in children diagnosed through bronchoscopy. ( 7 , 8 , 14 ) However, in a study employing cervical CT, Buterbaugh & Erly estimated that paratracheal air cyst (i.e., tracheal diverticulum) occurs in approximately 3.7% of the population.…”