Case Report: A rare cause of neonatal respiratory distress: extralobar pulmonary sequestration

Pulmonary sequestration (PS) is a rare congenital malformation characterized by a mass of non-functioning, dysplastic lung tissue that lacks normal tracheobronchial communication and receives its blood supply from an aberrant systemic artery. Although advances in prenatal ultrasonography have significantly improved early detection rates, a subset of cases remains undiagnosed until postnatal clinical symptoms emerge. Extralobar sequestration (ELS) in particular typically presents during the neonatal period, frequently manifesting as acute respiratory distress. We present the case of a term male neonate who developed progressive respiratory failure within the first hours of life, despite a completely unremarkable prenatal ultrasound history. Initially managed with non-invasive positive airway pressure, his clinical condition rapidly deteriorated, necessitating endotracheal intubation and invasive mechanical ventilation. Concurrently, the patient was diagnosed with severe congenital pneumonia caused by extended-spectrum β-lactamase (ESBL)-producing Klebsiella pneumoniae. While early chest radiographs showed bilateral diffuse opacities without definitive structural anomalies, follow-up imaging on the fifth day of life revealed a large, persistent consolidation in the right lower lobe. Subsequent contrast-enhanced computed tomography angiography (CTA) confirmed the presence of an extralobar pulmonary sequestration measuring approximately 37.5 × 20 × 53 mm. Notably, the CTA identified a rare anatomical variant: the aberrant feeding artery originated directly from the right renal artery, rather than the more typical thoracic or abdominal aorta. Following targeted antibiotic therapy and stabilization of his respiratory status, the patient underwent successful surgical resection of the sequestered lobe on the tenth day of life. Histopathological examination definitively confirmed the ELS diagnosis. The neonate was successfully extubated on the day of surgery, demonstrated an excellent postoperative recovery without requiring supplemental oxygen, and was discharged home on day 22. This case emphasizes the critical need to include congenital lung malformations in the differential diagnosis of unexplained neonatal respiratory failure, even in the context of normal prenatal imaging. Furthermore, the presence of an atypical feeding artery originating from the renal artery underscores the absolute necessity of detailed preoperative vascular mapping with CTA to optimize surgical planning, minimize intraoperative risks, and ensure a favorable clinical outcome.