Surg Today (2015) 45:1326–1329 DOI 10.1007/s00595-014-1016-z
CASE REPORT
Recurrent pneumothorax associated with bronchial atresia: report of a case Kazuhisa Tanaka · Hidemi Suzuki · Takahiro Nakajima · Tetsuzo Tagawa · Takekazu Iwata · Teruaki Mizobuchi · Shigetoshi Yoshida · Ichiro Yoshino
Received: 14 March 2014 / Accepted: 6 August 2014 / Published online: 26 August 2014 © Springer Japan 2014
Abstract We herein report a case of recurrent pneumothorax associated with congenital bronchial atresia. A 26-year-old male presented with chest pain. Chest roentgenograms showed left pneumothorax, a left apical bulla and an area of hyperlucency in the left upper lung field, and chest computed tomography revealed a discontinuation of the left superior bronchus. Additionally, both ventilation and perfusion scintigraphy showed a defect in the left superior segment. A thoracoscopy-assisted left superior segmentectomy was performed, and a pathological examination indicated left superior segmental bronchial atresia, which might have predisposed the peripheral lung to emphysematous conditions. No relapse was observed 6 months after the operation. Although this entity is rare, congenital bronchial atresia should be considered in the differential diagnosis when a patient has suffered from a recurrent spontaneous pneumothorax. Keywords Recurrent pneumothorax · Congenital bronchial atresia · Surgery
Introduction Congenital bronchial atresia is a rare disorder that is characterized by an emphysematous condition of the involved peripheral lung, rarely resulting in pneumothorax [1, 2]. The emphysematous lung is suggested to be caused by
K. Tanaka (*) · H. Suzuki · T. Nakajima · T. Tagawa · T. Iwata · T. Mizobuchi · S. Yoshida · I. Yoshino Department of General Thoracic Surgery, Chiba University Graduate School of Medicine, 1‑8‑1 Inohana, Chuo‑ku, Chiba 260‑8670, Japan e-mail: kaztanaka‑
[email protected]
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an air-trapping mechanism through collateral ventilation [1, 2]. We herein describe a case of recurrent pneumothorax associated with congenital bronchial atresia that was successfully treated with thoracoscopy-assisted segmentectomy.
Case report The patient was a 26-year-old male who experienced chest pain. His past medical history included pneumonia during childhood. On admission, a left spontaneous pneumothorax and a left apical bulla were observed on the chest X-ray (Fig. 1a). In addition, the radiolucency was increased in the left upper lung field. Hyperinflation of the left superior segment was observed on chest computed tomography (CT) (Fig. 1b). The peripheral superior segmental bronchus was patent. The lumen of the central area was obstructed, and the findings were consistent with mucoid impaction. A defect of the branch of the left superior segmental bronchus was confirmed on bronchoscopy. Additionally, both ventilation and perfusion scintigraphy showed a defect in the left superior segment (Fig. 1c). The patient was diagnosed with left superior bronchial atresia and an emphysematous condition of the peripheral lung, and it was considered that this entity might have been the cause of the bullous formation and the pneumothorax. The left lung expanded fully after tube thoracostomy, and the tube was removed after the cessation of air leakage. One month later, the patient had a second spontaneous pneumothorax. After tube thoracostomy, thoracoscopyassisted left superior segmentectomy was performed with an 8.0 cm axillary thoracotomy. No abnormality in the vascular branching of the region was observed. The border of
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Fig. 1 a Chest X-ray showing a left spontaneous pneumothorax and left apical bulla. b Hyperinflation of the left superior segment was observed in computed tomographic imaging of the chest (filled arrowhead). The periphery of the superior segmental bronchus was
patent (open arrow). The lumen of the central area was obstructed, and the findings were observed that were consistent with mucoid impaction (arrow). c Both ventilation and perfusion scintigraphy showed a defect in the left superior segment
Fig. 2 a A 3.0 cm bulla and smaller bullae were seen in the pulmonary apex (filled arrowhead). b The left superior segmental bronchus was enlarged, and an accumulation of secretions was observed in the
lumen. c The microscopic examination did not show any inflammatory changes of the bronchial walls around bronchial atresia (H&E stain, ×100)
the left superior segment was clearly distinguished as an emphysematous area. We did not find the left superior segmental bronchus from the left central bronchus. We found the stump of the left superior segmental bronchus in the abnormal lung. Therefore, we performed a left superior segmentectomy without managing the bronchial stump. A 3.0 cm bulla and smaller bullae were seen in the pulmonary apex (Fig. 2a). The left superior segmental
bronchus was enlarged, and an accumulation of secretions was observed in the lumen (Fig. 2b). A histopathological examination did not show any inflammatory changes in the bronchial walls (Fig. 2c). In addition, the emphysematous lung tissues showed enlarged alveoli without any inflammation or scarring, suggesting congenital abnormalities. The number of alveoli was small and the ordered fibrosis around the bronchiole was also suggestive of congenital
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1328 Table 1 Cases of adult congenital bronchial atresia associated with spontaneous pneumothorax reported in the literature
Surg Today (2015) 45:1326–1329 References
Age/sex
Year
Location
Treatment
Bulla
Berkman et al. [10] Okamoto and Okubo [11] Yoon et al. [12] Kameyama et al. [13] Niimi and Gotoh [14] Igai et al. [15]
20/M 25/M 36/F 36/F 25/F 18/F
1996 2002 2004 2006 2010 2012
Left B6 Left superior Right B2 Left superior Right B4 Right B2
Drainage Drainage Right upper lobectomy Left superior segmentectomy Right S4 segmentectomy Right S2 segmentectomy
Tanaka (current case)
26/M
2013
Left superior
Left superior segmentectomy
Unknown Unknown + + + +
disease. The postoperative clinical course was uneventful, and the patient was discharged on the 13th day after surgery. No relapse has been observed 6 months after the surgery.
Discussion Congenital bronchial atresia is a rare disorder first reported by Ramsay in 1953 [3]. The precise pathogenesis of this disorder is unclear. Obstruction in the bronchial origin results in enlargement of the peripheral bronchus, the accumulation of mucus and emphysematous changes in the peripheral lung. With occlusion of the bronchus, the aeration of the distal pulmonary tissue must await development of collateral air drifting through the pores of Kohn and bronchiolar–alveolar channels of Lambert [1, 2]. This mode of ventilation favors inspiration rather than expiration. The collateral routes are distended during inspiration, but collapsed during expirations, leading to air trapping and hyperinflation [4]. These emphysematous changes might result in bulla formation, and it is speculated that the pneumothorax in the present case may have occurred due to a rupture of the bulla [5]. The detailed process of the bronchial obstruction remains unknown. One explanation is a disturbance in the bronchial branching during the 5th week of the fetal period when the central airways are being formed [4]. However, the peripheral bronchi and alveolar structures in the obstructed area were preserved. Since the process of airway development is completed by the 16th week of fetal development, it has been said that the insult which leads to this localized abnormality is likely to have occurred after this [4, 6, 7]. Another common theory is a perfusion disturbance in the bronchial artery [8]. As in the present case, most cases of bronchial atresia occur in young adults, and in males more frequently than in females (60 vs. 40 %) [9]. In a review of 101 cases, 50 % of cases occurred in the left superior lobe, 49 % were asymptomatic and the diagnosis was made due to coincidental findings on routine plain chest X-rays in these cases [9]. Of the symptomatic cases, 21 % had recurrent infections, 14 % had dyspnea and 6 % had coughing
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+
[8]. The respiratory symptoms in patients with bronchial atresia are thought to be attributable to infection in the lung involved in the atresia, and to compression of the adjacent pulmonary tissue by the hyperinflation of lung [9]. Surgical resection is generally performed in symptomatic cases. However, the resection should be as limited as possible to preserve the normal pulmonary tissue, and thus, segmentectomy is preferable [9]. To the best of our knowledge, only six previous cases of bronchial atresia with concurrent pneumothorax have been reported in adults (Table 1). One case underwent lobectomy, three cases received segmentectomy and two cases were treated by only thoracic drainage [10–15]. The cause of pneumothorax was thought to be rupture of the bulla, and pathologically, a bulla was detected in all cases except the two cases treated by drainage. The present case received thoracoscopy-assisted left superior segmentectomy for a recurrent pneumothorax associated with congenital bronchial atresia, and multiple bullae were shown in the pulmonary apex. The pathological findings in the present case were different from those of spontaneous pneumothorax without bronchial atresia. The difference was the findings of lung hypoplasia. The number of alveoli was small, and the ordered fibrosis around the bronchiole was suggestive of congenital disease. When you manage pneumohtorax, if bronchial atresia is present, the treatment strategy for this disease entity should be changed so that the abnormal lung is completely resected, not only performing resection to manage the ruptured bulla. Since the ventilation and perfusion in the left superior segment had already been decreased preoperatively, the loss of respiratory function from the segmentectomy was minimal. After surgery, the patient has been well with no relapse for 6 months.
Conclusion In summary, we herein reported a rare case of recurrent pneumothorax associated with congenital bronchial atresia. Thoracoscopy-assisted segmentectomy was considered to be an effective treatment. Congenital bronchial atresia
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should be considered in the differential diagnosis when a patient suffers from a recurrent spontaneous pneumothorax. Conflict of interest Kazuhisa Tanaka and the co-authors have no conflicts of interest to declare in association with this study.
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