Original articleThe diagnosis and management of intradiaphragmatic extralobar pulmonary sequestrations: a report of 4 cases☆
Section snippets
Methods
We reviewed all cases of fetal and pediatric BPS at University of California, San Francisco, from 1995 to 2010 and classified each patient based on the location of the lung lesion at the time of operative resection. Information was obtained from hospital and pediatric surgery clinic records. Patients with congenital pulmonary airway malformations were not included in this series. We identified a small cohort of patients with IDEPS, each of whom underwent operative resection by 1 of 4
Results
From 1995 to 2010, we identified 27 patients who underwent operative resection for BPS at our institution. We stratified the location of these lesions based on their intraoperative findings. Most cases were found within the thoracic cavity (n = 18, or 66.7%). Of the 5 cases that were located within the abdominal cavity (18.5%), 3 were densely adherent to the diaphragm. An additional 4 sequestrations (14.8%) were located within the diaphragm itself (Table 1).
The prospective diagnosis of IDEPS
Discussion
Bronchopulmonary sequestration is a type of bronchopulmonary foregut malformation that is thought to arise either from anomalous budding of the primitive bronchial tree and gastrointestinal tract or from in utero bronchial obstruction [6], [7]. Although the location (intrathoracic vs intraabdominal) of BPS is often accurately identified on prenatal imaging, our experience indicates that some lesions may be completely hidden within the diaphragm and, therefore, pose a unique diagnostic
Acknowledgments
We thank Drs Doug Miniati and Diana Lee Farmer for helpful discussions and Liz Gress and Vilma Zarate for their assistance in coordinating the study.
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Cited by (25)
Infra-diaphragmatic extralobar pulmonary sequestration: Radiologic features
2023, Journal of Pediatric Surgery Case ReportsCitation Excerpt :The arterial supply to these lesions is systemic, rather than pulmonary. Twenty-five percent (25%) of sequestrations are extrapulmonary, occurring outside the normal lung parenchyma, of these, 8% are infra-diaphragmatic [1,3]. Intra-abdominal extralobar pulmonary sequestrations frequently present as a cystic mass diagnosed on ultrasound or CT [1].
Revising the classification of lung sequestrations
2021, Clinical ImagingCitation Excerpt :In the last decades, the increasing detection of congenital lung lesions in utero has increased both the number of postnatal imaging exams and of surgical resections in pediatric patients. The obtained data evidence a wide variability in the anatomical structures involved in lung sequestrations: pleura, arterial supply and venous drainage, airways and lung parenchyma [10–16]. The aims of this article are to identify the atypical anatomical findings detected on imaging studies, during surgery and at pathology in a series of children with confirmed lung sequestrations and to quantify their frequency.
Concurrent intrathoracic and extrathoracic congenital lung malformations
2021, Journal of Pediatric Surgery Case ReportsUndiagnosed Pulmonary Sequestration Causing Systemic Circulation Hypoperfusion During Ventricular Septal Defect Repair in an Infant
2020, Journal of Cardiothoracic and Vascular AnesthesiaCitation Excerpt :PS involves an independent blood supply from the systemic circulation rather than a pulmonary arterial branch and drainage by pulmonary or systemic veins. Typically, the arterial supply in PS arises from both the thoracic aorta and the abdominal aorta (very infrequently from the phrenic artery or the intercostal artery).7,8 In this case, neither the type (intra- or extralobar) nor the venous drainage source could be confirmed by DSA because of the limitation of the DSA technique.
Congenital lung lesions
2019, Seminars in Pediatric SurgeryCitation Excerpt :The most common location of intralobar BPS is the lower lobe, predominantly the posterior aspect of the left lower lobe. Similarly, extralobar BPS also arise within the lower aspect of the left hemithorax, but may occasionally be located below the diaphragm, classically in the area of the adrenal gland, or rarely within the diaphragm musculature.27–29 Congenital lobar emphysema (CLE), which represents approximately 10% of congenital lung malformations, is a fetal lung pathology of debated etiology.
Undiagnosed pulmonary sequestration results in an unexplained hemorrhagic shock in thoracoscopic pulmonary lobectomy
2016, Journal of Clinical Anesthesia
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Presented at the 2010 American College of Surgeons Clinical Congress, Washington, DC.