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Intrapericardial Extralobar Pulmonary Sequestration: A Case Report and Systematic Review of a Unique Embryologic
Margherita Roveri1,2, Giada Pedroni1,2, Alessandra Preziosi1
1Pediatric Surgery, ASST Grande Ospedale Metropolitano Niguarda, 20162 Milan, Italy.
Journal of Clinical Medicine
|February 13, 2026
Summary
Intrapericardial extralobar pulmonary sequestration (ELPS) is a rare congenital anomaly. Early diagnosis and surgical removal are crucial for preventing severe complications like cardiac tamponade.
Area of Science:
- Congenital Malformations
- Thoracic Surgery
- Pediatric Cardiology
Background:
- Intrapericardial extralobar pulmonary sequestration (ELPS) is an extremely rare congenital malformation.
- Its location can mimic tumors, presenting diagnostic and surgical difficulties.
- This study presents a new case and a systematic literature review.
Purpose of the Study:
- To describe a case of intrapericardial ELPS.
- To systematically review the literature on intrapericardial ELPS.
- To highlight diagnostic and surgical considerations for this rare condition.
Main Methods:
- A systematic literature review was performed using PRISMA guidelines.
- Databases searched included PubMed, Scopus, and Embase.
- Inclusion criteria were histologically confirmed intrapericardial ELPS.
Main Results:
- Eleven cases of intrapericardial ELPS, including the current one, have been reported.
- Prenatal detection occurred in 54% of cases; two cases resulted in fetal demise due to cardiac tamponade.
- Aberrant arterial supply from pulmonary arteries (54%) and venous drainage to the right atrium/superior vena cava (45%) were common findings.
Conclusions:
- Intrapericardial ELPS is a rare but surgically treatable condition.
- Early recognition and complete resection are vital to avoid life-threatening complications.
- A consistent vascular pattern suggests ELPS is a unique embryologic variant within the CPAM-sequestration spectrum.
Keywords:
congenital lung malformationscongenital teratomahybrid lesionintrapericardial extralobar pulmonary sequestrationintrapericardial lesionpediatric thoracic surgeryMore Related Videos
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