Characteristics of congenital bronchial atresia with cystic lesions: a single-center retrospective study

S Yoshimura, C Hisamatsu, W Sasaki, K Fukui, T Nakatani, Y Tomioka, S Murakami, A Yokoi, T Hatakeyama

Department of Pediatric Surgery, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan

Purpose

Congenital bronchial atresia (CBA) is a rare lung abnormality and often associated with cystic lesions (CLs) mimicking congenital pulmonary airway malformation (CPAM). The management of CBA remains uncertain; however, surgery is the preferred option, particularly for symptomatic cases. This study aimed to characterize CBA with CLs and investigate the association between CLs and the symptomatic rate.

Method

Patients with prenatally detected lung lesions who were born between April 2015 and March 2024 were collected. Among them, patients with radiologically or pathologically diagnosed CBA in our hospital were included. Clinical, radiological, surgical, and pathological data were retrospectively reviewed and analyzed. Continuous variables were presented as median [range], and p<0.05 was considered statistically significant.

Results

Twenty-three patients (13 female; 15 left-sided) were diagnosed with CBA. Computed tomography (CT) revealed that CLs were detected in 18 patients (78%) with a maximum size of 16 [4-28] mm. Thirteen patients with CLs required radiological differentiation between CBA and CPAM; however, CBA was finally diagnosed based on pathological findings of peripheral dilated bronchiole due to bronchial obstruction. Conversely, all five patients without CLs could be diagnosed with CBA based on the CT findings of hyperlucency in the lung field. All patients remained asymptomatic immediately after birth regardless of CLs. Only two patients without CLs chose conservative management, and the other patients underwent lobectomy or segmental lobectomy at 329 [187-1152] days of age. Six patients (26%) experienced lower respiratory tract infection, and the symptomatic rate of CBA with or without CLs was not statistically significant (28% vs. 20%, p=1.00).

Conclusion

CBA with CLs required differentiation from CPAM, and all patients with CLs underwent surgery. Conservative management was an option for CBA without CLs; however, CLs were not associated with symptomatic rate.

Keywords: Congenital bronchial atresia, congenital pulmonary airway malformation, symptomatic rate

S Yoshimura, C Hisamatsu, W Sasaki, K Fukui, T Nakatani, Y Tomioka, S Murakami, A Yokoi, T Hatakeyama

Department of Pediatric Surgery, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan

Anahtar Kelimeler: