Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)

Abstract Background Congenital diaphragmatic hernia (CDH) and bronchopulmonary sequestration (BPS) are rare congenital anomalies that can coexist, with studies suggesting that 25–40% of CDH cases are accompanied by BPS. The association between CDH and BPS is thought to arise from embryological disru...

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Main Authors: Sujin Gang, Yong Jae Kwon, Hyunhee Kwon, Suhyun Ha, Jueun Park, Byong Sop Lee, Euiseok Jung, Jiyoon Jeong, Soo Hyun Kim, Jung-Man Namgoong
Format: Article
Language:English
Published: BMC 2025-05-01
Series:BMC Pediatrics
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Online Access:https://doi.org/10.1186/s12887-025-05755-w
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author Sujin Gang
Yong Jae Kwon
Hyunhee Kwon
Suhyun Ha
Jueun Park
Byong Sop Lee
Euiseok Jung
Jiyoon Jeong
Soo Hyun Kim
Jung-Man Namgoong
author_facet Sujin Gang
Yong Jae Kwon
Hyunhee Kwon
Suhyun Ha
Jueun Park
Byong Sop Lee
Euiseok Jung
Jiyoon Jeong
Soo Hyun Kim
Jung-Man Namgoong
author_sort Sujin Gang
collection DOAJ
description Abstract Background Congenital diaphragmatic hernia (CDH) and bronchopulmonary sequestration (BPS) are rare congenital anomalies that can coexist, with studies suggesting that 25–40% of CDH cases are accompanied by BPS. The association between CDH and BPS is thought to arise from embryological disruptions during early gestation, with BPS potentially serving as an anatomical barrier. This study aimed to evaluate the incidence, clinical characteristics, and outcomes of patients with concurrent CDH and BPS at a single institution to improve therapeutic approaches. Methods This study retrospectively analyzed the medical records of neonates diagnosed and treated for BPS concurrent with CDH at Asan Medical Center from 1990 to 2021, identifying 15 cases (3.0%) among 493 CDH patients. Comprehensive data on demographics, treatments, outcomes, diagnostic imaging, and pathological findings were collected and analyzed to explore disease characteristics and evaluate clinical outcomes. Results This study analyzed 15 neonates with concurrent BPS and CDH, with a male predominance (10:5) and an average gestational age of 37.8 weeks, of whom 80% were diagnosed prenatally. CDH repair was performed at a median of 8 days, with no acute repair-related complications observed. In some cases, additional procedures such as hiatal hernia or re-do CDH repair was required, and BPS resection was performed either simultaneous or delayed. Follow-up revealed that most patients grew within the 50th percentile range, with thoracoscopic approaches feasible in over half of the cases, and recurrence-free outcomes achieved in those treated for hiatal hernia or BPS. Discussions No statistical difference in CDH severity was observed between the CDH and CDH + BPS groups, and the clinical outcome of CDH + BPS was better, although the high prevalence of sac-associated CDH types may have confounded the results. Our findings provide additional evidence supporting the protective effect of BPS. However, they also highlight the need for further studies in larger populations to clarify the causal relationship.
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spelling doaj-art-2e32ab128eea4c5ca648bcc924021c1a2025-08-20T01:53:25ZengBMCBMC Pediatrics1471-24312025-05-0125111010.1186/s12887-025-05755-wProtective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)Sujin Gang0Yong Jae Kwon1Hyunhee Kwon2Suhyun Ha3Jueun Park4Byong Sop Lee5Euiseok Jung6Jiyoon Jeong7Soo Hyun Kim8Jung-Man Namgoong9Division of Pediatric Surgery, Asan Medical Center Children’s Hospital, Asan Medical Center, University of Ulsan College of MedicineDivision of Pediatric Surgery, Asan Medical Center Children’s Hospital, Asan Medical Center, University of Ulsan College of MedicineDivision of Pediatric Surgery, Asan Medical Center Children’s Hospital, Asan Medical Center, University of Ulsan College of MedicineDivision of Pediatric Surgery, Asan Medical Center Children’s Hospital, Asan Medical Center, University of Ulsan College of MedicineDivision of Pediatric Surgery, Asan Medical Center Children’s Hospital, Asan Medical Center, University of Ulsan College of MedicineDepartment of Pediatrics, Asan Medical Center, University of Ulsan College of MedicineDepartment of Pediatrics, Asan Medical Center, University of Ulsan College of MedicineDepartment of Pediatrics, Asan Medical Center, University of Ulsan College of MedicineDepartment of Pediatrics, Asan Medical Center, University of Ulsan College of MedicineDivision of Pediatric Surgery, Asan Medical Center Children’s Hospital, Asan Medical Center, University of Ulsan College of MedicineAbstract Background Congenital diaphragmatic hernia (CDH) and bronchopulmonary sequestration (BPS) are rare congenital anomalies that can coexist, with studies suggesting that 25–40% of CDH cases are accompanied by BPS. The association between CDH and BPS is thought to arise from embryological disruptions during early gestation, with BPS potentially serving as an anatomical barrier. This study aimed to evaluate the incidence, clinical characteristics, and outcomes of patients with concurrent CDH and BPS at a single institution to improve therapeutic approaches. Methods This study retrospectively analyzed the medical records of neonates diagnosed and treated for BPS concurrent with CDH at Asan Medical Center from 1990 to 2021, identifying 15 cases (3.0%) among 493 CDH patients. Comprehensive data on demographics, treatments, outcomes, diagnostic imaging, and pathological findings were collected and analyzed to explore disease characteristics and evaluate clinical outcomes. Results This study analyzed 15 neonates with concurrent BPS and CDH, with a male predominance (10:5) and an average gestational age of 37.8 weeks, of whom 80% were diagnosed prenatally. CDH repair was performed at a median of 8 days, with no acute repair-related complications observed. In some cases, additional procedures such as hiatal hernia or re-do CDH repair was required, and BPS resection was performed either simultaneous or delayed. Follow-up revealed that most patients grew within the 50th percentile range, with thoracoscopic approaches feasible in over half of the cases, and recurrence-free outcomes achieved in those treated for hiatal hernia or BPS. Discussions No statistical difference in CDH severity was observed between the CDH and CDH + BPS groups, and the clinical outcome of CDH + BPS was better, although the high prevalence of sac-associated CDH types may have confounded the results. Our findings provide additional evidence supporting the protective effect of BPS. However, they also highlight the need for further studies in larger populations to clarify the causal relationship.https://doi.org/10.1186/s12887-025-05755-wCongenital diaphragmatic herniaBronchopulmonary sequestrationDiagnosisSurgeryOutcome
spellingShingle Sujin Gang
Yong Jae Kwon
Hyunhee Kwon
Suhyun Ha
Jueun Park
Byong Sop Lee
Euiseok Jung
Jiyoon Jeong
Soo Hyun Kim
Jung-Man Namgoong
Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)
BMC Pediatrics
Congenital diaphragmatic hernia
Bronchopulmonary sequestration
Diagnosis
Surgery
Outcome
title Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)
title_full Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)
title_fullStr Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)
title_full_unstemmed Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)
title_short Protective Effects of Bronchopulmonary Sequestration (BPS) on the Prognosis of Neonates with Congenital Diaphragmatic Hernia (CDH)
title_sort protective effects of bronchopulmonary sequestration bps on the prognosis of neonates with congenital diaphragmatic hernia cdh
topic Congenital diaphragmatic hernia
Bronchopulmonary sequestration
Diagnosis
Surgery
Outcome
url https://doi.org/10.1186/s12887-025-05755-w
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