C63-30 Intralobar Pulmonary Sequestration In Situs Inversus: A Rare Presentation With Recurrent Hemoptysis

Background Bronchopulmonary sequestration (BPS) is a rare congenital malformation of lung tissue characterized by the absence of normal bronchial connection and systemic arterial supply [1,2]. Situs inversus totalis (SIT) is an independent congenital anomaly in which thoracic and abdominal organs are reversed in mirror-image fashion [4]. The coexistence of BPS and SIT is extremely rare [9]. Case Presentation A 51-year-old female with a history of situs inversus totalis, diabetes mellitus, hypertension, and previously treated pulmonary sequestration (via endovascular embolization three years prior) presented with acute, recurrent, life-threatening hemoptysis and required intubation for airway protection. Chest X-ray done showed dextrocardia, right-sided aortic arch, and chronic consolidation at the medial left lung base. Chest CTA showed intralobar sequestration in the medial left upper lobe (anatomic right middle lobe) supplied by an aberrant artery from the left phrenic artery. Initial embolization controlled the bleeding temporarily. Two days later, hemoptysis recurred (about 500 mL). A bronchial blocker was reinserted for airway management. Due to continued bleeding, the patient underwent thoracotomy with partial wedge resection of the affected lobe. Recurrent bleeding necessitated a total lobectomy of the left upper (anatomic right middle) lobe. Postoperative recovery was uneventful; the patient was stabilized in the ICU and later transferred to inpatient rehabilitation. Histopathological analysis of the resected specimen confirmed intralobar pulmonary sequestration, characterized by cystic degeneration, fibrosis, and chronic inflammatory changes. Discussion Intralobar lung sequestration (ILS) accounts for 75% of cases of pulmonary sequestration and is typically located within the visceral pleura of functional lung [7]. Hemoptysis is the most common complication due to systemic arterial pressures [3]. Preoperative imaging with CT or MR angiography is critical for diagnosis [1,5]. Situs inversus complicates both anatomical assessment and surgical planning [4,9]. Endovascular embolization offers temporary control in select patients but is not always definitive [8] similar to our case. Surgical resection remains the gold standard, particularly in patients with life-threatening hemoptysis or failed embolization [6,8,9]. Thoracotomy was chosen over VATS due to aberrant vascularity and mirror-image anatomy [6]. Few cases of ILS in SIT have been reported, usually presenting with infection rather than hemoptysis [9]. Conclusion The coexistence of ILS and SIT is extremely rare and challenging. In this case, embolization provided temporary control, but definitive management was achieved with surgical resection [6,8]. Clinicians should be aware of the potential for recurrent hemoptysis in ILS with complex vascular anatomy, especially in patients with SIT.