Doppler Ultrasonographic Grading of Hepatic Vascular Malformations in Hereditary Hemorrhagic Telangiectasia - Results of Extensive Screening

 

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Zusammenfassung

Studienziel: In früheren Arbeiten wurde die Häufigkeit von vaskulären Missbildungen (VMs) in der Leber bei einer Familie mit hereditärer hämorrhagischer Teleangiektasie (HHT) mit der Doppler-Sonographie untersucht. Dabei wurden die entscheidenden Doppler-sonographischen Befunde beschrieben und klassifiziert. Seitdem wird die Doppler-Sonographie routinemäßig als Suchmethode für eine Leberbeteiligung bei Familien mit HHT eingesetzt und die VMs in der Leber Doppler-sonographisch beurteilt und klassifiziert. Methode: 346 Personen aus 64 Familien wurden auf Hinweise einer HHT geprüft und mit abdomineller Doppler-Sonographie auf VMs untersucht. Die vaskulären Veränderungen wurden wie folgt eingeteilt: minimal (bei einer auf den extrahepatischen Verlauf beschränkten Erweiterung der A. hepatica von mehr als 6 mm); mäßig (bei einer Erweiterung der A. hepatica im intra- und extrahepatischen Verlauf); und schwer (bei komplexen Veränderungen des Gefäßsystems der A. hepatica zusammen mit Erweiterung der Lebervenen und/oder Pfortader). Ferner wurden sowohl qualitative (Blutflussrichtung, Turbulenz) als auch quantitative (maximale Blutflussgeschwindigkeit und Widerstandsindex in der A. hepatica, durchschnittliche Blutflussgeschwindigkeit in der Pfortader, maximale distolische Blutflussgeschwindigkeit in den Lebevenen) Doppler-Werte in unserer Klassifikation der VMs berücksichtigt. Ergebnisse: Eine HHT wurde bei 222 Personen festgestellt. Die Doppler-Sonographie konnte bei 92 (41 %) Personen (24 Männer, 68 Frauen, Durschschnittsalter 52,2 Jahre) VMs in der Leber nachweisen. Die VMs waren bei 11 Personen minimal, bei 70 mäßig und bei 11 schwer. Schlussfolgerung: Mithilfe unserer vorgeschlagenen Doppler-sonographischen Einteilung können VMs in der Leber bei Personen mit HTT einfach klassifiziert werden. Entsprechende Folgeuntersuchungen und Behandlungen, die das Ausmaß der Gefäßveränderungen in der Leber berücksichtigen, können entwickelt werden.

Abstract

Aims: In previous studies, the prevalence of hepatic vascular malformations (VMs) in a large Italian family with hereditary hemorrhagic telangiectasia (HHT) was examined by Doppler ultrasonography (US) as screening technique, and the relevant Doppler US findings were described and classified. Thereafter, Doppler US has been routinely used to screen HHT families for liver involvement. Hepatic VMs were evaluated and classified on the basis of Doppler US findings. Methods: Three hundred and forty-six subjects belonging to 64 pedigrees were checked for the presence of signs of HHT. All of them underwent abdominal Doppler US screening for hepatic VMs. Vascular abnormalities were classified as minimal if the hepatic artery was dilated in extrahepatic tract only and measured > 6 mm; as moderate if the hepatic artery was dilated in both intra and extrahepatic tract; and as severe if complex changes of the arterial hepatic branches were associated with hepatic and/or portal vein dilatation. Furthermore, Doppler parameters, both qualitative (flow direction, turbulence) and quantitative (peak flow velocity and resistivity index in hepatic artery, mean velocity in portal vein, diastolic peak flow velocity in hepatic veins), were entered into our VM classification. Results: HHT was found in 222 subjects, with hepatic VMs detected by Doppler US in 92 (41.4 %) (24 males, 68 females, mean age 52.2). Hepatic VMs were minimal in 11 subjects, moderate in 70, and severe in 11. Conclusions: On the basis of our proposed grading, hepatic VMs can be easily classified in subjects with HHT by Doppler US. Depending on the degree of hepatic vascular derangement, appropriate programs for follow up and/or therapy can be designed.

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Elisabetta Buscarini

Gastroenterology Department

Ospedale Maggiore

Via Macallé 1

26013 Crema

Italy

Phone: ++ 39/03 73/28 03 20

Fax: ++ 39/03 73/28 06 54

Email: ebuscarini@rim.it