Intralobar pulmonary sequestration in situs inversus: A rare presentation with recurrent hemoptysis

Abstract

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 exceedingly rare [9].

Case presentation

We present a 51-year-old woman with SIT and BPS who developed massive hemoptysis. She had undergone prior embolization for intralobar sequestration (ILS) three years earlier. CT angiography revealed ILS in the medial left upper lobe (anatomic right middle lobe) with aberrant supply from the left phrenic artery. Emergent embolization achieved temporary control, but recurrent bleeding necessitated thoracotomy, wedge resection, and subsequent lobectomy.

Conclusion

This case highlights the diagnostic and therapeutic challenges of ILS in SIT. 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.

1. Background

Bronchopulmonary sequestration (BPS) represents 6.4% of congenital pulmonary malformations [1,2]. Two types exist: extralobar sequestration (ELS) and intralobar sequestration (ILS) [3,4]. In ILS, systemic arterial supply arises from the thoracic aorta in 74% of cases, with multiple anomalous arteries in 14.8% [1].

Situs inversus totalis (SIT) occurs in approximately 1:10,000 individuals, with a male-to-female ratio of 1.5:1 [5]. It is defined by mirror-image reversal of thoracic and abdominal viscera. The coexistence of SIT and BPS is extremely rare, with few cases reported in the literature.

We report a patient with SIT presenting with recurrent life-threatening hemoptysis due to ILS, managed successfully with lobectomy after failed embolization.

2. Case Presentation

A 51-year-old Hispanic 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, followed by bronchoscopic intervention.

2.1. Diagnostic studies

• •Chest X-ray: Dextrocardia, right-sided aortic arch, and chronic consolidation at the medial left lung base (Fig. 1).
• •Chest CTA: Intralobar sequestration in the medial left upper lobe (anatomic right middle lobe) supplied by an aberrant artery from the left phrenic artery (Fig. 2).
• •Bronchoscopy: Localized active bleeding controlled with bronchial blocker.
• •Angiography: Previously placed embolization pellets had migrated distally. Extravasation persisted from the left phrenic artery. Repeat embolization with glue was performed. An additional supply from the left internal mammary artery was identified and embolized (Fig. 3).
• •Histopathology: Confirmed ILS with cystic degeneration, fibrosis, and chronic inflammation [4] (Fig. 4).

Fig. 1.

Chest X-ray showing dextrocardia and right-sided aortic arch in situs inversus totalis.

Fig. 2.

CT angiography demonstrating ILS in medial left upper lobe (anatomic right middle lobe).

Fig. 3.

Angiography showing aberrant systemic supply from left phrenic artery before embolization.

Fig. 4.

Histopathology of resected specimen with cystic degeneration, fibrosis, and chronic inflammation.

2.2. Clinical course

Initial embolization controlled the bleeding temporarily. Two days later, hemoptysis recurred (∼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.

3. Discussion

Pulmonary sequestration is a non-functional lung segment supplied by systemic arteries without bronchial connection [[1], [2], [3],6]. ILS accounts for ∼75% of cases and is typically located within the visceral pleura of functional lung [4].

In adults, hemoptysis is the most common complication due to systemic arterial pressures [2]. Preoperative imaging with CT or MR angiography is critical for diagnosis [1,6]. Situs inversus complicates both anatomical assessment and surgical planning [5,7].

Endovascular embolization offers temporary control in select patients but is not always definitive [8]. In this case, recurrence highlighted the limitations of embolization. Surgical resection remains the gold standard, particularly in patients with life-threatening hemoptysis or failed embolization [[7], [8], [9]]. Thoracotomy was chosen over VATS due to aberrant vascularity and mirror-image anatomy [9].

Few cases of ILS in SIT have been reported, usually presenting with infection rather than hemoptysis [7]. Our case demonstrates the unique challenges of massive hemoptysis requiring repeat embolization and lobectomy in this rare dual anomaly.

4. Conclusion

The combination of intralobar pulmonary sequestration and situs inversus totalis presents extraordinary rarity and clinical management difficulties. Several key lessons emerge from this case; Situs inversus flips out standard anatomical reference points necessitating meticulous modifications to both diagnostic and therapeutic procedures. This case highlights that endovascular embolization may not serve as an adequate permanent solution for pulmonary sequestration with large aberrant vessels or aneurysmal changes even though it is becoming a popular less invasive alternative to surgery. The treatment of rare congenital anomaly combinations requires a multidisciplinary team approach that combines skills from interventional radiology, pulmonology, thoracic surgery, and intensive care medicine.

CRediT authorship contribution statement

Nawras Ibrahim: Data curation, Formal analysis, Project administration, Resources, Validation, Writing – original draft, Writing – review & editing, Conceptualization. Rahaf Jereisat: Writing – review & editing. Melissa Korb: Investigation, Resources, Visualization. Maher Dahdel: Data curation, Funding acquisition, Project administration, Supervision, Validation.

Ethical compliance

Written informed consent was obtained from the patient for the publication of this case report and any accompanying images.

Disclaimer/funding declaration

This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare-affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Abbreviations

BPS

Bronchopulmonary Sequestration

CTA

Computed Tomography Angiography

ELS

Extralobar Sequestration

ICU

Intensive Care Unit

ILS

Intralobar Sequestration

SIT

Situs Inversus Totalis

VATS

Video-Assisted Thoracoscopic Surgery

Appendix A. Supplementary data

The following is the Supplementary data to this article: