What is Bronchial Atresia?
Congenital bronchial atresia is a rare condition that causes the partial blockage of a bronchus, a passageway that brings air into the lungs. The condition most often affects the apicoposterior segmental bronchus of the left upper lung, followed by the right upper, middle, and lower lobes of the lungs. Despite its rarity, bronchial atresia is sometimes linked to other lung problems that are present from birth, such as congenital cystic adenomatoid malformation, bronchopulmonary sequestration, congenital lobar emphysema, and mixed-pathology lesions.
What Causes Bronchial Atresia?
The exact cause of bronchial atresia, a lung condition, is still not fully understood. However, there are two main theories about what could lead to it. One idea is that during the typical development of the lung, growing cells might lose their connection with the section of the lung that’s developing. Another theory is that repeated harm to lung tissue during early development in the womb could cause a fully formed bronchus (a large airway in the lung) to be closed off.
Risk Factors and Frequency for Bronchial Atresia
Bronchial atresia tends to be more common in males, with about 1.2 cases per 100,000 in the male population.
Signs and Symptoms of Bronchial Atresia
Bronchial atresia is often diagnosed incidentally from chest x-rays as many people don’t have symptoms. It’s typically diagnosed in young adults, in their 20s or 30s. However, around one-third of people with this condition do experience symptoms.
- Cough
- Shortness of breath
- Recurrent infections
- Wheezing (less common)
- Coughing up blood (less common)
- Chest pain (less common)
- Collapse of a lung, or pneumothorax (less common)
Testing for Bronchial Atresia
As previously explained, most cases of bronchial atresia – a condition where part of the airway in the lungs is blocked or abnormal – are usually discovered by accident during a chest X-ray. This abnormality takes the shape of a tubular, round, ovoid, or branching shadow near the center of the lungs, an area known as the hilum. Sometimes, if the lung area beyond the abnormality gets infected, it can look like pneumonia in the chest X-ray.
When diagnosing bronchial atresia, the preferred method is using a computed tomography scan, also known as a CT scan. This scan can show classic signs of bronchial atresia, like tubular or nodular shadows radiating from the hilum with a “finger-in-glove” appearance. This appearance is due to the accumulation of mucus in the blocked or abnormal airway, creating something called a mucocele. However, there are rare cases where there is no mucus accumulation, and instead, the abnormal airway is filled with air. The lung segment beyond the abnormal airway may appear overly bright and inflated due to low blood supply and trapped air. There are certain mechanisms in our lungs that allow air to come in but prevent it from escaping, which contributes to this phenomenon.
In the past, a procedure called bronchoscopy – a way to look inside the airways – was the preferred method before CT scans became widely used. If bronchoscopy revealed an airway that ends abruptly coupled with typical features seen in a chest X-ray, it was believed to confirm bronchial atresia. Today, the absence of common signs in X-ray and bronchoscopy, but the presence of an abruptly ending bronchus is considered a normal anatomical variation and doesn’t necessarily indicate bronchial atresia.
Treatment Options for Bronchial Atresia
At present, there aren’t any established guidelines for treating or managing bronchial atresia. In most cases, patients who show no symptoms won’t receive any active treatment. However, some experts suggest removing the affected area of the lung to prevent further complications. These complications might include infections within the lung tissue or issues affecting adjacent lung tissue.
What else can Bronchial Atresia be?
When it comes to diagnosing bronchial atresia, a medical condition of the lungs, the results from imaging scans can sometimes look similar to several other conditions. Some of these include:
- Bronchogenic cyst (a benign lung tumor)
- Lung aplasia (when a part or all of a lung fails to develop)
- Congenital lobar emphysema (a birth defect that causes over-inflated lungs)
- Congenital cystic adenomatoid malformation (a rare lung condition present from birth)
- Anomalous pulmonary venous return (a heart defect present at birth)
- Pulmonary sequestration (a non-functioning piece of lung tissue)
- Cystic fibrosis (a disease that affects the cells that produce mucus, sweat, and digestive juices)
- Allergic bronchopulmonary aspergillosis (a reaction to a fungus that can lead to swelling of the airways)
- Other benign (non-cancerous) and neoplastic (cancerous) processes.
It’s crucial for doctors to be able to distinguish between these conditions in order to establish the correct diagnosis.
What to expect with Bronchial Atresia
As noted previously, most cases of bronchial atresia don’t have symptoms and whether to treat them or not can be a matter of debate. However, surgery is usually only considered for the rare cases where severe symptoms occur, such as repeated infections or serious damage to the surrounding lung tissue.
Overall, the outlook is generally good. Most patients either don’t experience severe symptoms or, if they do need surgery, the results are typically excellent.
Possible Complications When Diagnosed with Bronchial Atresia
The main issues that arise from bronchial atresia, a lung condition, usually include infections, spontaneous pneumothorax (a condition where air leaks out from the lungs into the chest), and a long term breakdown of lung tissue.
Recovery from Bronchial Atresia
After a patient has surgery to remove a diseased segment, there could be some postoperative complications. These might include air leak and pneumothorax, conditions that might need drainage. After the operation, patients might need anything ranging from minimal care to complex support like mechanical ventilation. Also, it’s crucial to manage the patient’s pain effectively to ensure their comfort. Encouraging the patient to move around early is beneficial for quicker recovery.
Preventing Bronchial Atresia
Most patients with bronchial atresia don’t show symptoms and don’t need any treatment. However, it’s crucial that these patients understand what symptoms are linked to this condition. If the patient has any complications related to bronchial atresia, they must seek medical help. The doctor will talk them through the treatment options – both medical and surgical – as well as what to expect after treatment and during recovery. This way, patients can make informed decisions about the treatment they would like to pursue.
