Congenital lobar emphysema (CLA) is a rare congenital pulmonary malformation. It is a disease of early childhood. It presents with hyperinflation/swelling of a lobe or certain regions/segments of the lung.
The etiology is hyperinflation/swelling of a part of the lung due to abnormal cartilage structure in the bronchial wall, an infection causing alveolar damage, and obstruction/closure of the bronchus due to mucus plug formation
The lung's most common locations are the left upper lobe, middle lobe, and right upper and lower lobes.
The most common symptoms are dyspnea, intercostal retractions, wheezing, cyanosis, and feeding difficulties. These symptoms appear immediately after birth in 30% of cases, while others appear in childhood.
Very few patients are discovered incidentally in adulthood without any symptoms. It can cause acute respiratory distress in newborns. Recurrent lung infections are also common in these patients.
Chest radiography usually helps us diagnose, but thoracic tomography is better for a definitive diagnosis.
CLA may be accompanied by some congenital diseases (cardiovascular abnormalities, renal agenesis, renal cyst, pectus excavatum, diaphragmatic hernia, etc.).
Congenital Lobar Emphysema Treatment and Follow-up
Close clinical follow-up is essential in asymptomatic patients with congenital lobar emphysema. In these cases, the ideal approach is to perform surgery before the pneumonia attack. Considering the risks, surgery is recommended at 3-12 months. The surgery is a lobectomy, and it is life-saving in these patients.
Surgical Technique
We generally prefer closed surgery methods (Thoracoscopic Surgery/VATS) in Congenital Lobar Emphysema and Pediatric lung diseases. With the development of technology, we prefer closed surgery methods even for children aged 8-12 months. The closed surgery method uses one or two incisions, as in open surgery, by placing an intercostal retractor. One of these incisions is 1 cm, and the other is a two or 3-cm incision. Surgical instruments are advanced through these incisions. The diseased area is removed. Closed surgery; has many advantages, such as less pain, shorter recovery time, and more aesthetics.
Even a tiny incision looks very big in open surgery. Patients generally stay in the intensive care unit for one day. Patients are discharged in 3-4 days.
These closed surgeries in children require special high-tech devices and need a highly experienced center.