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Congenital Bronchial Atresia Associated With Spontaneous Pneumothorax
Congenital Bronchial Atresia Associated With Spontaneous Pneumothorax Kotaro Kameyama, MD, Norihito Okumura, MD, Yujiro Kokado, MD, Kentaro Miyoshi, MD, Tomoaki Matsuoka, MD, and Tatsuo Nakagawa, MD Department of Thoracic Surgery, Kurashiki Central Hospital, Okayama, Japan
A 36-year-old woman presented with left chest pain and frequent symptoms of upper respiratory infection. Chest roentgenograms revealed a left pneumothorax and apical bulla, and hyperlucency in the left pulmonary field. She was diagnosed with congenital bronchial atresia associated with a left spontaneous pneumothorax. A thoracoscopy-assisted left superior segmentectomy was performed. There was no recurrence of the pneumothorax or symptoms of recurrent upper respiratory infection at the 1-year follow-up examination. Bulla formation was believed to have resulted from emphysematous changes in the peripheral lung due to congenital bronchial atresia. The pneumothorax may have occurred due to rupture of the bulla. (Ann Thorac Surg 2006;82:1497–9) © 2006 by The Society of Thoracic Surgeons
C
ongenital bronchial atresia is a rare disorder. Obstruction in the bronchial origin results in enlargement of the peripheral bronchus, mucous accumulation, and emphysematous changes in the peripheral lung. We describe a case of congenital bronchial atresia associated with spontaneous pneumothorax and treated with thoracoscopy-assisted segmentectomy. The patient was a 36-year-old woman who experienced left chest pain while at work. She also experienced frequent symptoms of an upper respiratory infection, which occurred repeatedly at a rate of approximately once every 1 to 2 months, beginning approximately at the age of 20. These symptoms reappeared 3 weeks prior to the time the patient presented at our clinic for chest roentgenograms and continued for 2 days afterward. She was diagnosed with a left spontaneous pneumothorax and was admitted at our hospital. The patient’s medical history showed indications of a bulla in the left pulmonary apex on chest roentgenograms from school physical examinations since the age of 12. Results from blood tests and arterial blood gas analysis were noncontributory. On admission a left spontaneous pneumothorax and apical bulla were observed in chest Accepted for publication Nov 10, 2005. Address correspondence to Dr Kameyama, Department of Thoracic Surgery, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki, Okayama, 710-8602, Japan; e-mail: [email protected].
© 2006 by The Society of Thoracic Surgeons Published by Elsevier Inc
roentgenograms (Fig 1). In addition, radiolucency was increased in the left pulmonary field in spite of left pulmonary collapse. Hyperinflation of the left superior segment was observed in computed tomographic imaging of the chest (Fig 2). The periphery of the superior segmental bronchus was patent. The lumen of the central area was obstructed, and findings were observed that were consistent with mucoid impaction. The most central area located lateral to the pulmonary artery was cord-like (Fig 3A). A defect of the left superior segmental bronchus was observed in 3-dimensional computed tomographic reconstructed images of the respiratory tract (Fig 3B). Under bronchoscopy, the entire left upper lobar bronchus was continuous with the lingular segmental bronchus, but the orifice of the superior segmental bronchus was completely absent. In addition, both ventilation and perfusion scintigraphy showed a defect in the left superior segment. The patient was diagnosed with congenital bronchial atresia with associated spontaneous pneumothorax. Thoracoscopy-assisted left superior segmentectomy was performed with a 5.0-cm axillary thoracotomy. Highly incomplete lobulation between the superior and inferior lobes was observed perioperatively. However, no abnormality in vascular branching was observed. After control of vascular inflow to the superior segment was established, a preparation between the superior and the lingular segments was made, and the superior segment was removed. The surface of the resected left superior segment showed ischemic and generalized emphysematous changes. A 4-cm bulla and small bullae were seen in the pulmonary apex (Fig 4A). The left superior segmental bronchus was enlarged, and an accumulation of secretion was observed in the lumen (Fig 4B). Histopathologic examination did not show any inflammatory changes of the bronchial walls, and findings were inconsistent with bronchiectasis. In addition, the emphysematous lung tissues showed enlarged alveoli without inflammatory changes. The enlarged bronchus and emphysematous changes were believed to be due to physical changes caused by obstruction of the respiratory tract and was diagnosed as bronchial atresia. A follow-up examination approximately 1 year after surgery showed no recurrence of the pneumothorax, and the patient’s frequent cold-like symptoms had disappeared.
Comment Congenital bronchial atresia is a rare disorder first reported by Ramsay and Byron [1] in 1953. Obstruction in the bronchial origin results in enlargement of the peripheral bronchus, mucous accumulation, and emphysematous changes in the peripheral lung. Causes of emphysematous changes are believed to be similar to those for emphysema, resulting from collateral ventilation through the pores of Kohn and canals of Lambert in the surround0003-4975/06/$32.00 doi:10.1016/j.athoracsur.2005.11.033
FEATURE ARTICLES
CASE REPORTS
1498
CASE REPORT KAMEYAMA ET AL BRONCHIAL ATRESIA WITH PNEUMOTHORAX
Ann Thorac Surg 2006;82:1497–9
FEATURE ARTICLES
Fig 1. Chest roentgenogram of a spontaneous pneumothorax that was observed on the left side. A bulla (arrows) was observed in the apex of the left lung. Radiolucency was increased in the left field in spite of left lung collapse.
ing lung and bronchi, and check valve mechanism [2]. In this study, emphysematous changes resulted in bulla formation, and it is speculated that the pneumothorax occurred from rupture of the bulla. The cause of the bronchial obstruction remains unknown. One explanation is a disturbance in bronchial
Fig 3. High-resolution computed tomographic images of the thorax. (A) Two-dimensional coronal reformation of the lungs. The central portion of the left superior segmental bronchus (arrow) was obstructed and cord-like. This area was located lateral to the pulmonary artery. (B) Three-dimensional reconstructed image of the respiratory tract. The left main stem bronchus branches into the lingular segmental and inferior lobar bronchi.
Fig 2. Chest computed tomography. Hyperinflation of the left superior segment was observed. The periphery of the superior segmental bronchus (arrow) was patent.
branching at approximately the fifth week of the embryonic period [3]. However, peripheral bronchi and alveolar structures in the obstructed area remain pre-
CASE REPORT KAMEYAMA ET AL BRONCHIAL ATRESIA WITH PNEUMOTHORAX
1499
pression on the bronchus resulting in bronchial stricture and obstruction [6]. In this study, the superior segmental bronchus, which was obstructed and cordlike on chest computed tomography, also seemed to have an abnormality located lateral to the pulmonary artery. A well-known positional abnormality of the superior lobe of the bronchus is a tracheal bronchus, which is a superior transposition. However, this abnormality is seen less often on the left side than on the right [7]. In bronchial atresia, 64% of cases occur in the left superior lobe [4]. When a superior transposition of the left superior lobar bronchus occurs, bronchial atresia can result from compression by the pulmonary artery. Bronchial atresia is asymptomatic in 58% of cases at the time of evaluation. For symptomatic cases, 21% have recurrent infection, 14% have dyspnea, and 6% have coughing [4]. Surgical resection is generally performed in symptomatic cases. We believe that only 2 cases have been reported with concurrent pneumothorax. One case received lobectomy by thoracotomy, and another case was treated by drainage [8, 9]. The subject in our study received thoracoscopy-assisted left superior segmentectomy for congenital bronchial atresia with associated spontaneous pneumothorax. Since ventilation and perfusion in the left superior segment had already been decreased, loss of respiratory function from the segmentectomy was believed to be minimal. No recurrence of the pneumothorax was observed, and the patient’s frequent symptoms of upper respiratory infection also disappeared. From this report, thoracoscopy-assisted left superior segmentectomy is considered to be an effective treatment for congenital bronchial atresia with associated spontaneous pneumothorax.
References
Fig 4. (A) Removal of the left superior segment. The lung surface was whitish and poorly vascularized. It was also generally emphysematous. A 4-cm bulla and small bullae were observed at the pulmonary apex area. (B) Horizontal section of the left superior segment. The arrow shows the enlarged superior segment with an accumulation of secretion in the lumen.
served. Therefore it is highly probable that the cause was a local disturbance occurring on or after the 16th week of the fetal period when bronchial branching is complete [4]. Another common theory is a perfusion disturbance in the bronchial artery [5]. Furthermore, one theory states that an anomaly of the pulmonary artery or bronchus can lead to pulmonary artery com-
1. Ramsay BH, Byron FX. Mucocele, congenital bronchiectasis, and bronchiogenic cyst. J Thorac Surg 1953;26:21–30. 2. Meng RL, Jensik RJ, Faber LP, Matthew GR, Kittle CF. Bronchial atresia. Ann Thorac Surg 1978;25:184 –92. 3. Williams AJ, Schuster SR. Bronchial atresia associated with a bronchogenic cyst. Evidence of early appearance of atretic segments. Chest 1985;87:396 – 8. 4. Jederlinic PJ, Sicilian LS, Baigelman W, Gaensler EA. Congenital bronchial atresia. A report of 4 cases and a review of the literature. Medicine (Baltimore) 1987;66:73– 83. 5. Lacquet LK, Fornhoff M, Dierickx R, Buyssens N. Bronchial atresia with corresponding segmental pulmonary emphysema. Thorax 1971;26:68 –73. 6. Ko SF, Lee TY, Kao CL, et al. Bronchial atresia associated with epibronchial right pulmonary artery and aberrant right middle lobe artery. Br J Radiol 1998;71:217–20. 7. Remy J, Smith M, Marache P, Nuyts JP. Pathogenetic left tracheal bronchus. A review of the literature in connection with four cases (author’s translation). J Radiol Electrol Med Nucl 1977;58:621–30. 8. Berkman N, Bar-Ziv J, Breuer R. Recurrent spontaneous pneumothorax associated with bronchial atresia. Respir Med 1996;90:307–9. 9. Yoon YH, Son KH, Kim JT, et al. Bronchial atresia associated with spontaneous pneumothorax: report of a case. J Korean Med Sci 2004;19:142– 4.
FEATURE ARTICLES
Ann Thorac Surg 2006;82:1497–9
A 36-year-old woman presented with left chest pain and frequent symptoms of upper respiratory infection. Chest roentgenograms revealed a left pneumothorax and apical bulla, and hyperlucency in the left pulmonary field. She was diagnosed with congenital bronchial atresia associated with a left spontaneous pneumothorax. A thoracoscopy-assisted left superior segmentectomy was performed. There was no recurrence of the pneumothorax or symptoms of recurrent upper respiratory infection at the 1-year follow-up examination. Bulla formation was believed to have resulted from emphysematous changes in the peripheral lung due to congenital bronchial atresia. The pneumothorax may have occurred due to rupture of the bulla. (Ann Thorac Surg 2006;82:1497–9) © 2006 by The Society of Thoracic Surgeons
C
ongenital bronchial atresia is a rare disorder. Obstruction in the bronchial origin results in enlargement of the peripheral bronchus, mucous accumulation, and emphysematous changes in the peripheral lung. We describe a case of congenital bronchial atresia associated with spontaneous pneumothorax and treated with thoracoscopy-assisted segmentectomy. The patient was a 36-year-old woman who experienced left chest pain while at work. She also experienced frequent symptoms of an upper respiratory infection, which occurred repeatedly at a rate of approximately once every 1 to 2 months, beginning approximately at the age of 20. These symptoms reappeared 3 weeks prior to the time the patient presented at our clinic for chest roentgenograms and continued for 2 days afterward. She was diagnosed with a left spontaneous pneumothorax and was admitted at our hospital. The patient’s medical history showed indications of a bulla in the left pulmonary apex on chest roentgenograms from school physical examinations since the age of 12. Results from blood tests and arterial blood gas analysis were noncontributory. On admission a left spontaneous pneumothorax and apical bulla were observed in chest Accepted for publication Nov 10, 2005. Address correspondence to Dr Kameyama, Department of Thoracic Surgery, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki, Okayama, 710-8602, Japan; e-mail: [email protected].
© 2006 by The Society of Thoracic Surgeons Published by Elsevier Inc
roentgenograms (Fig 1). In addition, radiolucency was increased in the left pulmonary field in spite of left pulmonary collapse. Hyperinflation of the left superior segment was observed in computed tomographic imaging of the chest (Fig 2). The periphery of the superior segmental bronchus was patent. The lumen of the central area was obstructed, and findings were observed that were consistent with mucoid impaction. The most central area located lateral to the pulmonary artery was cord-like (Fig 3A). A defect of the left superior segmental bronchus was observed in 3-dimensional computed tomographic reconstructed images of the respiratory tract (Fig 3B). Under bronchoscopy, the entire left upper lobar bronchus was continuous with the lingular segmental bronchus, but the orifice of the superior segmental bronchus was completely absent. In addition, both ventilation and perfusion scintigraphy showed a defect in the left superior segment. The patient was diagnosed with congenital bronchial atresia with associated spontaneous pneumothorax. Thoracoscopy-assisted left superior segmentectomy was performed with a 5.0-cm axillary thoracotomy. Highly incomplete lobulation between the superior and inferior lobes was observed perioperatively. However, no abnormality in vascular branching was observed. After control of vascular inflow to the superior segment was established, a preparation between the superior and the lingular segments was made, and the superior segment was removed. The surface of the resected left superior segment showed ischemic and generalized emphysematous changes. A 4-cm bulla and small bullae were seen in the pulmonary apex (Fig 4A). The left superior segmental bronchus was enlarged, and an accumulation of secretion was observed in the lumen (Fig 4B). Histopathologic examination did not show any inflammatory changes of the bronchial walls, and findings were inconsistent with bronchiectasis. In addition, the emphysematous lung tissues showed enlarged alveoli without inflammatory changes. The enlarged bronchus and emphysematous changes were believed to be due to physical changes caused by obstruction of the respiratory tract and was diagnosed as bronchial atresia. A follow-up examination approximately 1 year after surgery showed no recurrence of the pneumothorax, and the patient’s frequent cold-like symptoms had disappeared.
Comment Congenital bronchial atresia is a rare disorder first reported by Ramsay and Byron [1] in 1953. Obstruction in the bronchial origin results in enlargement of the peripheral bronchus, mucous accumulation, and emphysematous changes in the peripheral lung. Causes of emphysematous changes are believed to be similar to those for emphysema, resulting from collateral ventilation through the pores of Kohn and canals of Lambert in the surround0003-4975/06/$32.00 doi:10.1016/j.athoracsur.2005.11.033
FEATURE ARTICLES
CASE REPORTS
1498
CASE REPORT KAMEYAMA ET AL BRONCHIAL ATRESIA WITH PNEUMOTHORAX
Ann Thorac Surg 2006;82:1497–9
FEATURE ARTICLES
Fig 1. Chest roentgenogram of a spontaneous pneumothorax that was observed on the left side. A bulla (arrows) was observed in the apex of the left lung. Radiolucency was increased in the left field in spite of left lung collapse.
ing lung and bronchi, and check valve mechanism [2]. In this study, emphysematous changes resulted in bulla formation, and it is speculated that the pneumothorax occurred from rupture of the bulla. The cause of the bronchial obstruction remains unknown. One explanation is a disturbance in bronchial
Fig 3. High-resolution computed tomographic images of the thorax. (A) Two-dimensional coronal reformation of the lungs. The central portion of the left superior segmental bronchus (arrow) was obstructed and cord-like. This area was located lateral to the pulmonary artery. (B) Three-dimensional reconstructed image of the respiratory tract. The left main stem bronchus branches into the lingular segmental and inferior lobar bronchi.
Fig 2. Chest computed tomography. Hyperinflation of the left superior segment was observed. The periphery of the superior segmental bronchus (arrow) was patent.
branching at approximately the fifth week of the embryonic period [3]. However, peripheral bronchi and alveolar structures in the obstructed area remain pre-
CASE REPORT KAMEYAMA ET AL BRONCHIAL ATRESIA WITH PNEUMOTHORAX
1499
pression on the bronchus resulting in bronchial stricture and obstruction [6]. In this study, the superior segmental bronchus, which was obstructed and cordlike on chest computed tomography, also seemed to have an abnormality located lateral to the pulmonary artery. A well-known positional abnormality of the superior lobe of the bronchus is a tracheal bronchus, which is a superior transposition. However, this abnormality is seen less often on the left side than on the right [7]. In bronchial atresia, 64% of cases occur in the left superior lobe [4]. When a superior transposition of the left superior lobar bronchus occurs, bronchial atresia can result from compression by the pulmonary artery. Bronchial atresia is asymptomatic in 58% of cases at the time of evaluation. For symptomatic cases, 21% have recurrent infection, 14% have dyspnea, and 6% have coughing [4]. Surgical resection is generally performed in symptomatic cases. We believe that only 2 cases have been reported with concurrent pneumothorax. One case received lobectomy by thoracotomy, and another case was treated by drainage [8, 9]. The subject in our study received thoracoscopy-assisted left superior segmentectomy for congenital bronchial atresia with associated spontaneous pneumothorax. Since ventilation and perfusion in the left superior segment had already been decreased, loss of respiratory function from the segmentectomy was believed to be minimal. No recurrence of the pneumothorax was observed, and the patient’s frequent symptoms of upper respiratory infection also disappeared. From this report, thoracoscopy-assisted left superior segmentectomy is considered to be an effective treatment for congenital bronchial atresia with associated spontaneous pneumothorax.
References
Fig 4. (A) Removal of the left superior segment. The lung surface was whitish and poorly vascularized. It was also generally emphysematous. A 4-cm bulla and small bullae were observed at the pulmonary apex area. (B) Horizontal section of the left superior segment. The arrow shows the enlarged superior segment with an accumulation of secretion in the lumen.
served. Therefore it is highly probable that the cause was a local disturbance occurring on or after the 16th week of the fetal period when bronchial branching is complete [4]. Another common theory is a perfusion disturbance in the bronchial artery [5]. Furthermore, one theory states that an anomaly of the pulmonary artery or bronchus can lead to pulmonary artery com-
1. Ramsay BH, Byron FX. Mucocele, congenital bronchiectasis, and bronchiogenic cyst. J Thorac Surg 1953;26:21–30. 2. Meng RL, Jensik RJ, Faber LP, Matthew GR, Kittle CF. Bronchial atresia. Ann Thorac Surg 1978;25:184 –92. 3. Williams AJ, Schuster SR. Bronchial atresia associated with a bronchogenic cyst. Evidence of early appearance of atretic segments. Chest 1985;87:396 – 8. 4. Jederlinic PJ, Sicilian LS, Baigelman W, Gaensler EA. Congenital bronchial atresia. A report of 4 cases and a review of the literature. Medicine (Baltimore) 1987;66:73– 83. 5. Lacquet LK, Fornhoff M, Dierickx R, Buyssens N. Bronchial atresia with corresponding segmental pulmonary emphysema. Thorax 1971;26:68 –73. 6. Ko SF, Lee TY, Kao CL, et al. Bronchial atresia associated with epibronchial right pulmonary artery and aberrant right middle lobe artery. Br J Radiol 1998;71:217–20. 7. Remy J, Smith M, Marache P, Nuyts JP. Pathogenetic left tracheal bronchus. A review of the literature in connection with four cases (author’s translation). J Radiol Electrol Med Nucl 1977;58:621–30. 8. Berkman N, Bar-Ziv J, Breuer R. Recurrent spontaneous pneumothorax associated with bronchial atresia. Respir Med 1996;90:307–9. 9. Yoon YH, Son KH, Kim JT, et al. Bronchial atresia associated with spontaneous pneumothorax: report of a case. J Korean Med Sci 2004;19:142– 4.
FEATURE ARTICLES
Ann Thorac Surg 2006;82:1497–9