イントラペリカルディア・エクストラロバル肺封じ込め: 症例報告と独特の胚学的変異の体系的レビュー

Margherita Roveri^1,2, Giada Pedroni^1,2, Alessandra Preziosi¹

¹Pediatric Surgery, ASST Grande Ospedale Metropolitano Niguarda, 20162 Milan, Italy.

Journal of clinical medicine

|February 13, 2026

まとめ

心臓内外肺部封じ込め (ELPS) は,珍しい先天性異常である. 早期診断と手術による摘出は,心臓タンポネードなどの重篤な合併症を予防するために極めて重要です.

科学分野:

生まれながらの変形
胸部外科手術について
ペディアトリック・カルディオロジー

背景:

心臓内外肺部封じ込め (ELPS) は,非常にまれな先天性異常である.
その位置は腫瘍を模倣し,診断および手術の困難を呈する可能性があります.
この研究は,新しいケースと体系的な文献レビューを提示しています.

研究の目的:

イントラペリカルディアルELPSの症例を記述する.
イントラペリカルディアルELPSに関する文献を体系的にレビューする.
この希少な疾患の診断および外科的検討を強調するためです.

主な方法:

PRISMAのガイドラインを使用して,体系的な文献レビューが行われました.
検索したデータベースには,PubMed,Scopus,Embaseが含まれる.
含有基準は,組織学的に確認された心内ペリカルダ・ELPSでした.

主要な成果:

現在の症例を含む11件の心内ペリカルダ・ELPS症例が報告されています.
胎児の検知は54%の症例で発生し,2つの症例では心臓タンポネードによる胎児死亡が発生した.
肺動脈からの異常な動脈供給 (54%) と右心房/上 vena cava (45%) への静脈排水が一般的な発見でした.

結論:

イントラペリカルディアルELPSはまれですが,手術で治療可能な状態です.
生命を脅かす合併症を避けるには,早期発見と完全な切除が不可欠です.
一貫した血管パターンは,ELPSがCPAM-シーケストレーションスペクトル内のユニークな胚学的変種であることを示唆しています.

キーワード:

生まれながらの肺の不形成症生まれながらのテラトーマ.ハイブリッド・レシオン・レシオン・ハイブリッド・レシオンイントラペリカルディアル・エクストラロバル肺封じ込めイントラペリカルディアル病変小児の胸腔外科手術について

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