Pulmonale Sequestration

Pulmonale Sequestration ist eine Masse abnormalen Lungengewebes ohne Verbindung zum normalen Bronchialbaum und mit systemischer arterieller Versorgung (meist von der Aorta). Zwei Typen: intralobäre Sequestration (ILS, 75%) und extralobäre Sequestration (ELS, 25%). Der diagnostische Schlüssel ist die Darstellung der aberranten Speisungsarterie von der Aorta in der CT-Angiographie. Behandlung ist chirurgische Resektion.

Synonyme: Pulmonary sequestration · Bronchopulmonary sequestration · BPS · Intralobar sequestration · Extralobar sequestration · Pulmoner sekestrasyon · Pulmonale Sequestration

Altersbereich

0-18

Häufigkeitsalter

0.5

Geschlecht

Mannlich dominant

Prävalenz

Sehr selten

◆Diagnostische Schlüsselhinweise

1Aberrante Speisungsarterie von Aorta in CT-Angiographie — pathognomonischer Befund
2Solide oder zystische Masse im linken Unterlappen posterobasal — häufigste Lokalisation (60%+ ILS)
3Rezidivierende Pneumonie gleicher Lokalisation — typische klinische Präsentation der ILS
4Fehlende normale Bronchialverbindung
5ELS: Separate pleuragekleidete Masse in linkem unterem Thorax oder subdiaphragmatisch — CDH-Assoziation häufig

♦Pathophysiologie

Pulmonale Sequestration entsteht aus einer abnormalen Lungenknospe, die sich während der Embryonalentwicklung vom Vorderdarm trennt und unabhängig entwickelt. Dieses anomale Gewebe behält systemische arterielle Versorgung (von Aorta). Die intralobäre Sequestration entwickelt möglicherweise über die poröse Grenze partielle Atemwegsverbindungen — daher anfällig für rezidivierende Infektionen. Die aberrante Arterie in der CT-Angiographie reflektiert direkt die Persistenz der embryologischen systemischen vaskulären Verbindung.

★

SchlusselzeichenCTarterial

Aberrante Speisungsarterie von der Aorta

Der Signaturbefund der pulmonalen Sequestration ist die Darstellung einer aberranten Speisungsarterie von der Aorta in der CT-Angiographie. Dieser Befund ist pathognomonisch und differenziert Sequestration definitiv von anderen kongenitalen Lungenläsionen.

Bildgebungsmerkmale

CTarterialpathognomonic

Aberrant Systemic Arterial Supply From Aorta (Pathognomonic)

Der diagnostischste Befund in der CT-Angiographie ist die Darstellung einer aberranten Speisungsarterie von der Aorta. Diese Arterie ist meist einzeln und großkalibrig (5-10 mm). MIP- und VR-Rekonstruktionen zeigen Arterienursprung, Verlauf und Verzweigung detailliert.

Berichtssatz

In der CT-Angiographie wird eine aberrante Speisungsarterie von ___ mm aus der thorakalen/abdominalen Aorta auf Höhe ___ beobachtet, die die Masse im linken/rechten Unterlappen-Posterobasalsegment versorgt; pathognomonisch für pulmonale Sequestration.

CTnon-contrasthighly-suggestive

Solid Or Cystic Mass In Left Lower Lobe Posterobasal Segment

Sequestration erscheint im CT als solide, zystische oder gemischte Masse im linken Unterlappen-Posterobasalsegment. Subdiaphragmatische ELS kann mit Neuroblastom verwechselt werden.

Berichtssatz

Eine solide/zystische Masse von ___ × ___ cm wird im linken/rechten Unterlappen-Posterobasalsegment beobachtet.

CTarterialhighly-suggestive

Venous Drainage Assessment (Pulmonary Vs Systemic)

Venöse Drainage der Sequestration wird in der venösen Phase der CT-Angiographie beurteilt. ILS drainiert über normale Pulmonalvenen zum linken Vorhof. ELS drainiert über systemische Venen — meist Azygos, Hemiazygos oder VCI.

Berichtssatz

Die venöse Drainage der Sequestration erfolgt über Pulmonalvenen/systemische Venen (Azygos/VCI), vereinbar mit ILS/ELS.

USdopplerhighly-suggestive

Prenatal/Postnatal US: Echogenic Mass With Feeding Artery On Doppler

Im pränatalen US erscheint Sequestration als homogene echogene Masse — meist im linken unteren Thorax oder subdiaphragmatisch. Farb-Doppler kann Speisungsarterie von Aorta zeigen.

Berichtssatz

Im US wird eine echogene Masse im linken/rechten unteren Thorax/subdiaphragmatisch mit Speisungsarterie von Aorta im Doppler beobachtet; vereinbar mit pulmonaler Sequestration.

MRIT2highly-suggestive

MRI/MRA: Mass With Systemic Arterial Supply Confirmation

MRT kann für fetale Sequestrationsbewertung und als Alternative zur CT postnatal verwendet werden. In T2 erscheint Sequestration meist hyperintens. MR-Angiographie (MRA) kann aberrante Speisungsarterie ohne Strahlung darstellen.

Berichtssatz

Im MRT wird eine T2-hyperintense Masse im linken/rechten Unterlappen mit aberranter Speisungsarterie von der Aorta in der MRA beobachtet.

Subtypen

Intralobäre Sequestration (ILS)

Kriterien

75% der Fälle. Innerhalb des normalen Lungenparenchyms, kein eigener Pleuraüberzug. Drainage über Pulmonalvenen.

Unterscheidungsmerkmale

Präsentation mit rezidivierender Pneumonie. Assoziierte Anomalien selten. Lobektomie ist Behandlung.

Extralobäre Sequestration (ELS)

Kriterien

25% der Fälle. Eigener Pleuraüberzug. Drainage über systemische Venen. Linker unterer Thorax oder subdiaphragmatisch. 60% CDH-Assoziation.

Unterscheidungsmerkmale

Meist pränatale/neonatale Diagnose. Häufig assoziierte Anomalien (60%+ CDH). Einfache Resektion (separate Masse). Infektion selten.

Differentialdiagnosen

Kongenitale Pulmonale Atemwegsmalformation (CPAM)CT angiography

Unterscheidungsmerkmal

CPAM wird von Pulmonalarterie versorgt, Sequestration von Aorta. CT-Angiographie liefert definitive Differenzierung.

Kongenitale ZwerchfellhernieCXR + CT

Unterscheidungsmerkmal

CDH hat Zwerchfelldefekt mit abdominellen Organen im Thorax. Bei Sequestration ist Zwerchfell intakt. ELS koexistiert häufig mit CDH.

NeuroblastomCT angiography + lab

Unterscheidungsmerkmal

Subdiaphragmatische ELS kann mit Neuroblastom verwechselt werden. Neuroblastom erhöht Katecholamin-Metabolite, Verkalkung häufig. ELS zeigt direkte Aortenversorgung, Katecholamine normal.

Klinische Relevanz

Dringlichkeit

routine

Management

surgical

Biopsie

Nicht erforderlich

Nachsorge

specialist-referral

Pulmonale Sequestration wird mit chirurgischer Resektion behandelt. Präoperative CT-Angiographie für genaue Arterienlokalisation obligat. Prognose nach Operation exzellent.

Differentialdiagnostische Tipps

→CPAM: pulmonale arterielle Versorgung (Sequestration hat systemische Arterie), zystisch dominante Struktur
→Bronchogene Zyste: solitär, unilokulär, Wasserdichte, kann bronchiale Verbindung haben
→CDH: Zwerchfelldefekt, Darmschlingen im Thorax
→Unterlapppneumonie: infektiöse Zeichen, kein systemisches Zuführungsgefäß, Rückbildung in der Verlaufskontrolle

●Klinische Bedeutung

Zweithäufigste kongenitale Lungenanomalie nach CPAM. Der kritischste diagnostische Schritt ist die Darstellung des systemischen arteriellen Zuführungsgefäßes — Sequestration kann ohne diesen Befund nicht diagnostiziert werden. CTA ist der Goldstandard für die präoperative Gefäßkartierung. Der intralobare Typ präsentiert sich mit rezidivierenden Pneumonien, der extralobare Typ wird meist pränatal oder in der Neugeborenenperiode erkannt. Die chirurgische Resektion ist die definitive Behandlung. Hybridläsionen (CPAM + Sequestration) können auftreten — können sowohl pulmonale als auch systemische arterielle Versorgung aufweisen.

Referenzen

Wei Y, Li F. Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40(1):e39-42.
Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Savic B, et al. Lung sequestration: report of seven cases and review of 540 published cases. Thorax. 1979;34(1):96-101.
ACR Appropriateness Criteria: Neonatal Respiratory Distress. 2020.
Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am. 1994;41(3):453-72.
Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Gezer S, et al. Pulmonary sequestration: a single-institutional series of 25 cases. J Thorac Cardiovasc Surg. 2007;133(4):955-9.

Verwandte Erkrankungen

Kongenitale Pulmonale Atemwegsmalformation (CPAM)Kongenitale Zwerchfellhernie Neuroblastom

← Zurück zur Liste

Pulmonale Sequestration

◆Diagnostische Schlüsselhinweise

1Aberrante Speisungsarterie von Aorta in CT-Angiographie — pathognomonischer Befund

2Solide oder zystische Masse im linken Unterlappen posterobasal — häufigste Lokalisation (60%+ ILS)

3Rezidivierende Pneumonie gleicher Lokalisation — typische klinische Präsentation der ILS

4Fehlende normale Bronchialverbindung

5ELS: Separate pleuragekleidete Masse in linkem unterem Thorax oder subdiaphragmatisch — CDH-Assoziation häufig

♦Pathophysiologie

Bildgebungsmerkmale

CTarterialpathognomonic

Aberrant Systemic Arterial Supply From Aorta (Pathognomonic)

Berichtssatz

CTnon-contrasthighly-suggestive

Solid Or Cystic Mass In Left Lower Lobe Posterobasal Segment

Sequestration erscheint im CT als solide, zystische oder gemischte Masse im linken Unterlappen-Posterobasalsegment. Subdiaphragmatische ELS kann mit Neuroblastom verwechselt werden.

Berichtssatz

Eine solide/zystische Masse von ___ × ___ cm wird im linken/rechten Unterlappen-Posterobasalsegment beobachtet.

CTarterialhighly-suggestive

Venous Drainage Assessment (Pulmonary Vs Systemic)

Berichtssatz

Die venöse Drainage der Sequestration erfolgt über Pulmonalvenen/systemische Venen (Azygos/VCI), vereinbar mit ILS/ELS.

USdopplerhighly-suggestive

Prenatal/Postnatal US: Echogenic Mass With Feeding Artery On Doppler

Im pränatalen US erscheint Sequestration als homogene echogene Masse — meist im linken unteren Thorax oder subdiaphragmatisch. Farb-Doppler kann Speisungsarterie von Aorta zeigen.

Berichtssatz

Im US wird eine echogene Masse im linken/rechten unteren Thorax/subdiaphragmatisch mit Speisungsarterie von Aorta im Doppler beobachtet; vereinbar mit pulmonaler Sequestration.

MRIT2highly-suggestive

MRI/MRA: Mass With Systemic Arterial Supply Confirmation

Berichtssatz

Im MRT wird eine T2-hyperintense Masse im linken/rechten Unterlappen mit aberranter Speisungsarterie von der Aorta in der MRA beobachtet.

Subtypen

Intralobäre Sequestration (ILS)

Kriterien

75% der Fälle. Innerhalb des normalen Lungenparenchyms, kein eigener Pleuraüberzug. Drainage über Pulmonalvenen.

Unterscheidungsmerkmale

Präsentation mit rezidivierender Pneumonie. Assoziierte Anomalien selten. Lobektomie ist Behandlung.

Extralobäre Sequestration (ELS)

Kriterien

25% der Fälle. Eigener Pleuraüberzug. Drainage über systemische Venen. Linker unterer Thorax oder subdiaphragmatisch. 60% CDH-Assoziation.

Unterscheidungsmerkmale

Meist pränatale/neonatale Diagnose. Häufig assoziierte Anomalien (60%+ CDH). Einfache Resektion (separate Masse). Infektion selten.

Differentialdiagnosen

Kongenitale Pulmonale Atemwegsmalformation (CPAM)CT angiography

Unterscheidungsmerkmal

CPAM wird von Pulmonalarterie versorgt, Sequestration von Aorta. CT-Angiographie liefert definitive Differenzierung.

Kongenitale ZwerchfellhernieCXR + CT

Unterscheidungsmerkmal

CDH hat Zwerchfelldefekt mit abdominellen Organen im Thorax. Bei Sequestration ist Zwerchfell intakt. ELS koexistiert häufig mit CDH.

NeuroblastomCT angiography + lab

Unterscheidungsmerkmal

Subdiaphragmatische ELS kann mit Neuroblastom verwechselt werden. Neuroblastom erhöht Katecholamin-Metabolite, Verkalkung häufig. ELS zeigt direkte Aortenversorgung, Katecholamine normal.

Differentialdiagnostische Tipps

→CPAM: pulmonale arterielle Versorgung (Sequestration hat systemische Arterie), zystisch dominante Struktur

→Bronchogene Zyste: solitär, unilokulär, Wasserdichte, kann bronchiale Verbindung haben

→CDH: Zwerchfelldefekt, Darmschlingen im Thorax

→Unterlapppneumonie: infektiöse Zeichen, kein systemisches Zuführungsgefäß, Rückbildung in der Verlaufskontrolle

●Klinische Bedeutung

Referenzen

Wei Y, Li F. Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40(1):e39-42.

ACR Appropriateness Criteria: Neonatal Respiratory Distress. 2020.

Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am. 1994;41(3):453-72.

Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Gezer S, et al. Pulmonary sequestration: a single-institutional series of 25 cases. J Thorac Cardiovasc Surg. 2007;133(4):955-9.