Congenital pulmonary sequestration

Congenital pulmonary sequestration (also known as bronchopulmonary sequestration or pulmonary sequestration) is a rare developmental malformation of the lower respiratory tract in which a mass of non-functioning lung tissue forms during fetal development. This abnormal tissue does not connect to the normal tracheobronchial tree and receives its blood supply from an anomalous systemic artery, most commonly arising from the thoracic or abdominal aorta. The condition primarily affects the pulmonary system but can also involve vascular structures and, in some cases, the gastrointestinal tract. There are two main types: intralobar sequestration (ILS) and extralobar sequestration (ELS). Intralobar sequestration, which accounts for approximately 75% of cases, is located within the visceral pleura of a normal lobe and typically presents later in childhood or adulthood with recurrent pneumonia, chronic cough, and hemoptysis. Extralobar sequestration has its own pleural covering, is more commonly diagnosed in the neonatal period or infancy, and may be associated with other congenital anomalies such as congenital diaphragmatic hernia, cardiac defects, or foregut malformations. ELS is more frequently found on the left side and may present with respiratory distress in newborns. Diagnosis is often made through imaging studies including chest X-ray, CT angiography, or prenatal ultrasound, which can identify the aberrant arterial blood supply. Treatment is primarily surgical, involving resection of the sequestered tissue either through lobectomy (for intralobar sequestrations) or excision of the mass (for extralobar sequestrations). Minimally invasive thoracoscopic approaches are increasingly used. In some prenatally diagnosed cases, particularly those with associated hydrops fetalis, fetal intervention may be considered. Prognosis after surgical resection is generally excellent.

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