O1050: Variation in Management of Extralobar Sequestration: A Multicenter Retrospective Study.

Friday, September 26, 2025

4:15 PM - 4:18 PM MDT

Location: Colorado Convention Center, Mile High Ballroom 2B-3B

Background: Extralobar sequestration (ELS) is a congenital lung malformation (CLM) characterized by non-aerated lung tissue covered by its own visceral pleura, supplied by an anomalous systemic vessel, and isolated from normal lung parenchyma. The role of non-operative management in ELS remains controversial. This study seeks to elucidate management patterns of ELS, focusing on those with intrathoracic lesions.

Methods: A retrospective cohort study of all CLMs diagnosed between 2016 to 2024 was conducted within a new consortium of 17 Children’s Hospitals. We focused on isolated ELS confirmed by postnatal imaging and/or intraoperative findings at resection. Patients with other thoracic anomalies (e.g., CDH) or additional congenital lung lesions were excluded.

Results: Of 1600 CLMs identified during the study period, 141 (9%) were isolated ELS. Of these, 106 (95%) were prenatally diagnosed and 86 (61%) were male. ELS were intrathoracic in 112 (81%), abdominal in 18 (13%), and intra-diaphragmatic in 8 (6%). Intrathoracic ELS were located on the left in 80 (71%). CVR was recorded in 71 (63%) with intrathoracic ELS, with a median last recorded CVR of 0.34 (IQR: 0.21, 0.7). Ten (9%) mothers received steroids prenatally for large lesions; however, only 3 had a CVR above 1.6. Four underwent fetal procedures: 2 thoracoamniotic shunting and 2 thoracenteses. Gestational age at birth was 39 weeks (IQR:37.7,39.2). All patients had at least one CT scan, with 64 (57%) reporting three-dimensional measurements. During the neonatal period, five (4%) patients had respiratory symptoms and underwent resection. Their median CT lesion size was 86 cm³ (IQR: 47, 161). Post-neonatal (n=107), intrathoracic ELS were managed operatively in 96 (90%) [median CT lesion size of 5.1 cm³ (IQR: 2.9, 11] and non-operatively in 11 (10%) [median CT lesion size of 6.63 cm³ (IQR: 1.87, 8.92)]; p=0.64. In the operative group, the median age at resection was 7 months (IQR: 5,9). Ninety-one (94%) remained asymptomatic and had elective resection, while 3 developed respiratory symptoms, but none had pneumonia. Thoracoscopic excision was performed in 90 patients (93%) with a median hospital stay of 1 day (IQR: 1,1). Only one patient developed a postoperative chylothorax. ELS was not associated with malignancy on pathology. The median age at last follow-up was 25 months (Range: 2, 87) in non-operative patients, all remained asymptomatic.

Conclusion: Large ELS on postnatal CT scans can present with respiratory symptoms at birth, often evoking surgical resection. No ELS became infected. The majority of intrathoracic ELS remain asymptomatic and are resected via thoracoscopy in the first year of life due to concern for potential future symptoms. However expectant management, over an average 2-year follow-up has not led to complications. Intrathoracic ELS require ongoing multicenter, ideally prospective study, to personalize the optimal treatment of these benign developmental lesions.

Presenting Author(s)

Tayana Jean Pierre, MD,MPH

Research fellow
Boston children’s Hospital
Boston Children's Hospital, Massachusetts

Senior Author(s)

Terry Buchmiller, MD

Attending Surgeon
Boston Children's Hospital
Boston Children's Hospital/ Harvard Medical School

Co-Author(s)

Christopher Derderian, MD

Assistant Professor of Surgery
Children's Hospital Colorado
Children's Hospital Colorado
Anne M. Sescleifer, MD

Postdoctorate Research Fellow
Johns Hopkins University
CC

Casey M. Calkins, MD

Professor at the Medical College of Wisconsin - Pediatric surgeon
Children’s Wisconsin
The Medical College of Wisconsin, Wisconsin
Benjamin Carr, MD (he/him/his)

Assistant Professor of Surgery
Oregon Health & Science University
Portland, Oregon
DC

Darrell Cass, MD

Director of Fetal Surgery
Cleveland Clinic
JC

Joseph Church, MD

Pediatric surgeon
University of Michigan, CS Mott Children's Hospital
VD

Vincent Duron, MD

Associate Professor of Surgery (Pediatrics)
Columbia University Irving Medical Center
SF

Stephen J. Fenton, MD

Associate Professor of Pediatric Surgery
University of Utah School of Medicine, Utah
Anthony Ferrantella, MD

Pediatric Surgeon
Children's Healthcare of Atlanta
Emory University
BG

Brian Gray, MD

Pediatric surgeon
Indiana University School of Medicine, Riley Hospital for Children
AK

Aimee Kim, MD

Attending Physician
Children's Hospital Los Angeles
IL

Inna Lobeck, MD, FACS, FAAP

Surgical Director, Fetal Health Center. Assistant Professor of Surgery
Children's Mercy Hospital - Kansas City
Jason O. Robertson, MD, MS

Pediatric Surgeon
Cleveland Clinic Children's Hospital
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University
Highland Heights, Ohio
MR

Marissa Ray, MD, MS

post-doctoral research fellow
Cincinnati Children’s Hospital Medical Center
Cincinnati, Ohio
Raphael C. Sun, MD

Associate Professor of Surgery, Pediatric Surgeon
Oregon Health & Science University Hospital
Oregon Health & Science University Hospital
Portland, Oregon
Peter T. Yu, MD MPH

Pediatric Surgeon
Division of Pediatric General and Thoracic Surgery, Children’s Hospital of Orange County, Orange, CA; Department of Surgery, University of California Irvine Medical Center, Orange, CA
University of California - Irvine
Orange, California
Irving J. Zamora, MD, MPH

Director, Advanced Minimally Invasive Surgery, Associate Program Director, Pediatric Surgery Fellowship
Vanderbilt University Medical Center
Vanderbilt University School of Medicine
Nashville, Tennessee
Shaun M. Kunisaki, MD, MSc

Professor of Surgery
Johns Hopkins University, Maryland