Single-center study: evaluation of sonography in Budd-Chiari syndrome

Abstract

Purpose Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction. The study aimed to evaluate the diagnostic utility of ultrasound in confirming the diagnosis of BCS and to provide an overview of the clinical picture.

Materials and method In this retrospective single-center study, patients with an initial diagnosis of BCS were included. The files were analyzed concerning the ultrasound images and compared to computed tomography (CT) and magnetic resonance imaging (MRI). Main clinical signs of BCS were collected.

Results Data of 25 patients were analyzed. Doppler sonography showed the highest sensitivity (78.9%) with the highest specificity 97.4 (%) in confirming the correct diagnosis of BCS. Main imaging signs were obstruction in the hepatic veins (68.0%, 17/25 thrombotic), collaterals (91.7%, 11/12 intrahepatic), inhomogeneous liver parenchyma (7/21), and a hypertrophied lobus caudatus (18/21) (p < 0.01). All imaging signs could be detected with sonography. Hypertrophied lobus caudatus was seen exclusively in BCS. Furthermore, portal hypertension (9/25), liver cirrhosis (9/25), and ascites (19/25) can be diagnosed as non-specific signs of BCS (p < 0.01).

The main clinical findings were elevated γ-GT levels in the laboratory (92.0%, 23/25, p < 0.01) and esophageal varices in endoscopy (12/25 p < 0.01). An association with myeloproliferative neoplasia (MPN) was frequently seen (10/25) (p < 0.01).

Conclusion The present study demonstrates that sonography is an appropriate tool for the diagnosis of BCS and should be used as the first imaging procedure.

Zusammenfassung

Hintergrund Die Diagnostik mittels Ultraschalls eignet sich besonders gut für Organe im Oberbauch wie z.B. die Leber. Das Budd-Chiari-Syndrom (BCS) ist eine seltene Erkrankung, bei der der venöse Abfluss der Lebervenen behindert ist.

Zielsetzung Ziel dieser retrospektiven Single-Center-Studie ist es, den diagnostischen Nutzen und die Leistungsfähigkeit des Ultraschalls bei der Diagnosesicherung des BCS zu evaluieren.

Methoden Insgesamt 25 BCS-Fälle mit Erstdiagnose im Universitätskrankenhaus Tübingen (UKT) aus dem Zeitraum 2005–2018 wurden in diese Studie eingeschlossen. Die Akten wurden auf sonografische (bildgebende) und klinische Zeichen des BCS und im Vergleich von Ultraschall (B-Bild-farbkodierte Dopplersonografie) und Computertomografie (CT) und Magnetresonanztomografie (MRT) analysiert.

Ergebnisse Als Zeichen in der Bildgebung (Sonografie/CT/MRT) wurden Obstruktionen in den Lebervenen (68,0%, 17/25 thrombotisch), Kollateralen (zu 91,7%, 11/12 intrahepatisch), eine inhomogene Leber (7/21) und ein hypertrophierter Lobus caudatus (18/21) beobachtet (p<0,01). Portale Hypertension (9/25), Leberzirrhose (9/25) und Aszites (19/25 wurden als unspezifische Zeichen eines BCS gefunden (p<0,01). Im Labor fanden sich erhöhte γ-GT-Werte (92,0%, 23/25, p<0,01) und in der Endoskopie wurden Ösophagusvarizen nachgewiesen (12/25 p<0,01). Ein Zusammenhang mit der Myeloproliferativen Neoplasie (MPN) ist typisch für BCS (10/25) (p<0,01). Die Sonografie zeigte die höchste Sensitivität 78,9% bei der höchsten Spezifität 97,4%.

Schlussfolgerung Die Sonografie ist ein geeignetes Instrument für die Diagnose des BCS. Sie sollte als erstes bildgebendes Verfahren eingesetzt werden. Alle in der Bildgebung auftretenden Zeichen können mit der Sonografie erkannt werden. Der kontrastmittelverstärkte Ultraschall (CEUS) könnte die Sensitivitäten und Spezifitäten erhöhen und möglicherweise eine weitere Bildgebung überflüssig machen.

Schlüsselwörter

Budd-Chiari-Syndrom - Leber - Ultraschall

Keywords

Budd-Chiari-Syndrome - Liver - Ultrasound

Publication History

Received: 19 May 2021

Accepted after revision: 08 July 2021

Article published online:
15 November 2021

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