WOFAPS 2025 8th World Congress of Pediatric Surgery

Anete Rozentalberga, Janis Karlsons, Zane Abola

Riga Stradins University & Children's Clinical University Hospital, Department of Paediatric Surgery

Introduction: Pulmonary sequestration accounts for 0.15% to 6.40% of all congenital lung malformations.

Pulmonary sequestration is a condition in which a segment or lobe of dysplastic lung tissue exists with no communication with the rest of the tracheobronchial tree and receives an anomalous systemic vascular supply, separate from the rest of the lung. It is, therefore, a nonfunctional tissue.

This is a rare clinical case – a patient with 3 rare congenital lung malformations – congenital emphysema of the lower lobe of the left lung, left-sided extrapulmonary intrathoracic sequestration and bronchogenic cyst on the diafragm.

Case presentation: The child was born from IV pregnancy, IV delivery, at 39+1 weeks of gestation. Indicated delivery. Antenatal suspicion of cystic adenomatous malformation of the left lung. CT scan of the lungs performed, revealing lobar emphysema of the lower lobe of the left lung.

The child has no respiratory insufficiency. After 6 months, an MRI was performed, which revealed hyperventilation of the S6 and S9 segments of the left lung, against which elongated cystic structures were visible - a finding consistent with the CT scan. A benign cystic formation was also detected on the left side - differential diagnosis between a diaphragmatic cyst and an intradiaphragmatic bronchogenic cyst.

On 8 April 2025, surgical treatment was performed – left-sided thoracoscopy – lower lobe resection; left-sided thoracoscopic extrapulmonary sequestration resection and video-assisted diaphragmatic formation extirpation.

Histological response - lung tissue with emphysematous and atelectatic changes; lung tissue sequestration and bronchogenic cyst.

Conclusions: Despite its rarity, some radiological features are sufficiently suggestive of diagnosis of pulmonary sequestration. Advanced imaging modalities, such as CT and MRI, play a crucial role in accurately diagnosing this condition by identifying the aberrant arterial supply and the sequestered lung tissue. Surgical resection remains the definitive treatment for extrapulmonary sequestration, with the potential for excellent outcomes when appropriately managed.