- What is Bronchial Atresia?
- Bronchial Atresia — Causes
- Bronchial Atresia — Symptoms
- Bronchial Atresia — CT & Radiology Findings
- Bronchial Atresia — ICD-10
- Bronchial Atresia — Treatment & Management
What is Bronchial Atresia?
Bronchial atresia is a congenital airway anomaly in which a segmental or subsegmental bronchus ends blindly before reaching the normal bronchial tree. Distal to the atretic segment, mucus secreted by viable bronchial glands accumulates and dilates the obstructed airway, forming a mucocele or bronchocele. Because there is no patent proximal airway, the affected lung tissue receives air by collateral pathways (pores of Kohn, canals of Lambert), which can lead to hyperinflation and air trapping. Bronchial atresia is most often discovered incidentally in adolescents or young adults during imaging for unrelated reasons, though it is present from birth. The classic location is the apicoposterior segment of the left upper lobe, but any lobe can be involved. Importantly, it is usually an isolated finding, yet it may coexist with other congenital pulmonary malformations such as congenital lobar overinflation or bronchogenic cysts. Understanding the anatomy and physiology behind this condition helps clinicians distinguish it from acquired airway obstruction caused by foreign bodies, tumors, or mucus plugging.
Clinically, many patients remain asymptomatic throughout life, and the condition is often benign. When symptoms occur, they are generally related to regional overinflation or secondary infection of distal bronchi that cannot drain well. Because the proximal bronchus is atretic, bronchoscopy may not demonstrate a visible lumen to the involved segment, which can complicate direct diagnosis. Radiology therefore plays a central role in recognizing the triad of mucocele/bronchocele, hyperinflated adjacent lung, and absent/stenotic proximal airway. Awareness of bronchial atresia prevents unnecessary interventions for suspected “mass lesions” and guides appropriate follow-up. In modern practice, management is individualized: observation for asymptomatic cases, and targeted treatment if recurrent infections, significant dyspnea, or compression of normal lung parenchyma develops. Patient education about warning signs—fever, persistent cough, or new shortness of breath—supports timely reassessment and reduces the risk of complications.
Bronchial Atresia — Causes
The cause of bronchial atresia is developmental. During embryogenesis, the bronchial tree branches from the foregut in a highly ordered process. Focal disruption in this branching, vascular supply, or epithelial recanalization can leave a segmental bronchus incompletely formed or sealed at its origin. The distal bronchial structures (mucous glands, smooth muscle, alveoli) continue to develop, which explains why mucus production persists beyond the atretic segment. Over time, retained secretions distend the distal airway into a mucocele, while collateral ventilation maintains aeration of the adjacent parenchyma. Unlike acquired obstruction, there is no foreign body, tumor, or inflammatory stricture causing the blockage. The atresia is present from birth even if not recognized until later imaging.
Risk factors at the individual level are not well defined, and most cases appear sporadic. Bronchial atresia can, however, be seen alongside other congenital lung anomalies, suggesting shared developmental pathways may be affected. It is not typically linked to environmental exposures after birth. Importantly, clinicians should distinguish congenital bronchial atresia from conditions that mimic it radiographically, such as impacted mucus from asthma or cystic fibrosis, allergic bronchopulmonary aspergillosis with “finger-in-glove” mucus impaction, or endobronchial neoplasms. These mimics have different etiologies and treatments. A careful review of patient age, symptoms, history of infections or atopy, and detailed CT imaging features (including the presence of a blind-ending bronchus) helps confirm the congenital nature and avoid misclassification.
Bronchial Atresia — Symptoms
Symptoms range from none to mild and are driven by the degree of air trapping and the propensity for infection distal to the atresia. Many individuals are asymptomatic and learn of the condition after a chest X-ray taken for employment or preoperative evaluation reveals an unusual lucency or mass-like opacity. When symptomatic, patients may report chronic cough, episodic wheeze, chest tightness, or exertional dyspnea related to regional hyperinflation. Recurrent or persistent localized infections can occur because mucus in the blind segment drains poorly, predisposing to bacterial growth. Low-grade fever, sputum production, and pleuritic discomfort during episodes may bring patients to care. In infants and young children—where recognition is rarer but possible—tachypnea and feeding difficulties during respiratory illnesses may be clues.
Physical examination is often normal between infections. During exacerbations, focal decreased breath sounds or wheeze over the affected lobe may be heard. Routine pulmonary function testing can be normal in mild disease or show an obstructive pattern in those with broader airway hyperreactivity. The symptom pattern is typically regional rather than diffuse, which helps separate it from asthma or COPD. Because bronchial atresia often involves the upper lobes, symptoms might be posture- or exertion-dependent. Education about prompt evaluation of new fevers, persistent cough, or hemoptysis is important, though significant bleeding is uncommon. Overall, recognizing the usually benign natural history while staying alert for treatable complications leads to balanced, patient-centered care.
Bronchial Atresia — CT & Radiology Findings
Radiology is central to diagnosis. On chest X-ray, a tubular or branching opacity representing a mucus-filled bronchus (bronchocele) may project from the hilum, sometimes with a surrounding area of increased lucency due to hyperinflation. The silhouette can mimic an endobronchial mass or vascular structure. Because plain radiographs are limited, CT of the chest is the modality of choice. Classic CT features include a blind-ending or abruptly tapering segmental bronchus, a mucocele/bronchocele often with soft-tissue attenuation or air–mucus level, and adjacent hyperinflated lung with decreased vascular markings from air trapping via collateral ventilation. On expiratory imaging, air trapping is accentuated. The well-known “finger-in-glove” sign describes the branching, glove-like appearance of mucoid impaction extending into the distal airway.
Additional CT clues include displacement of fissures from regional hyperexpansion, and the absence of a proximal obstructing lesion. CT angiography can clarify relationships to vessels when anatomy is complex or when other congenital malformations are suspected. Bronchoscopy may show no visible orifice to the affected segment, supporting atresia, but its main value is to exclude acquired intraluminal obstruction in atypical cases. Differential diagnoses encompass allergic bronchopulmonary aspergillosis, cystic fibrosis with mucus impaction, endobronchial carcinoid or other tumors, congenital lobar overinflation, and bronchogenic cysts. Radiologists emphasize the constellation—mucocele, distal hyperinflation, and absent proximal lumen—to favor bronchial atresia and guide conservative versus interventional management.
Bronchial Atresia — ICD-10
There is no single, universally mandated stand-alone ICD-10 code labeled “bronchial atresia.” In many billing and documentation workflows, cases are classified under the chapter for congenital malformations of the respiratory system. A commonly used option is Q33.8 — Other congenital malformations of lung, with additional codes applied for associated conditions such as localized infection, hemoptysis, or respiratory symptoms as appropriate. Some institutions may instead choose a code for other specified congenital malformations of bronchus/lung depending on the local code set and payer guidance. Because coding conventions and payer policies vary, clinicians and coders should confirm the preferred code description for their jurisdiction and update practices as code sets are revised.
Best practice is to document clearly in the chart that the finding is a congenital, segmental bronchial atresia with typical radiologic features, and to add problem-list entries for any sequelae (e.g., recurrent localized pneumonia) or interventions (e.g., segmentectomy). When imaging is the basis of diagnosis, including the CT report language in the encounter note supports accurate coding and audit readiness. If bronchial atresia coexists with another congenital pulmonary anomaly, the primary code may reflect the dominant diagnosis, with bronchial atresia referenced in the narrative and secondary codes as needed.
Bronchial Atresia — Treatment & Management
Management is tailored to symptoms and complications. Asymptomatic patients with typical imaging and normal lung function are frequently managed with observation, periodic clinical review, and education about warning signs of infection. Vaccination updates (influenza, pneumococcal as indicated) and general lung health measures are sensible supportive steps. When infections occur distal to the atresia, treatment is directed at the episode—culture-guided antibiotics when appropriate, airway clearance strategies, and follow-up to ensure resolution. Routine bronchoscopy has limited benefit because there is no patent proximal lumen; it may be reserved for atypical or complicated cases to exclude acquired obstruction. For individuals with recurrent localized infections, significant dyspnea, or compressive hyperinflation affecting adjacent healthy lung, segmentectomy or lobectomy can be curative with excellent outcomes in experienced centers.
Shared decision-making is key. Surgeons, pulmonologists, and radiologists should review imaging together to confirm that symptoms correlate with the anatomic abnormality before recommending resection. Minimally invasive approaches (video-assisted thoracoscopic surgery) are often feasible. Postoperative prognosis is generally good, with symptom relief and preservation of global lung function, especially when disease is localized. For patients who are not surgical candidates or prefer conservative care, a plan emphasizing prompt treatment of infections, pulmonary rehabilitation where beneficial, and periodic reassessment works well. Because bronchial atresia is congenital, long-term follow-up focuses on monitoring rather than “curing” the anomaly in those who remain well. Clear documentation, patient education, and a low threshold to re-image if the clinical picture changes help maintain safety and quality of life.
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