Intra-diaphragmatic Extralobar Pulmonary Sequestration: Surgical Approaches and Outcome

jpedsurg.org

Intra-diaphragmatic extralobar pulmonary sequestration (IDEPS) is an extremely rare congenital lung malformation characterized by non-functional lung tissue within the diaphragm. Although surgical resection is frequently performed, management strategies vary significantly between centers and remain controversial due to difficulties in accurately locating the lesion intraoperatively and the absence of large case series.This retrospective multicenter study analyzes the experience in the treatment of IDEPS and compares the findings with those reported in the existing literature.

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Article · Dec 2025 · 1 min read

In brief

This multicenter retrospective study examines surgical management of intra-diaphragmatic extralobar pulmonary sequestration, a rare congenital condition where non-functional lung tissue develops within the diaphragm. The research addresses controversies in treatment approaches and intraoperative localization challenges due to limited published case data.

IDEPS is an extremely rare congenital malformation with non-functional lung tissue embedded within the diaphragm itself.
Intraoperative localization of IDEPS lesions remains technically challenging, contributing to variability in surgical management.
No standardized treatment protocol exists due to limited case series and lack of consensus among pediatric surgery centers.
Surgical resection is the most common intervention, though optimal approach and timing remain controversial.
Multicenter collaboration is essential to build evidence base for this rare condition and refine management strategies.

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