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Z Gastroenterol 2012; 50(12): 1296-1301
DOI: 10.1055/s-0032-1325340
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© Georg Thieme Verlag KG Stuttgart · New York

Spontaneous Hepatic Rupture in Amyloidosis – Report of a Case

Spontane Leberruptur bei Amyloidose
T. G. Lehmann
1   Department of General-, Visceral- and Transplant Surgery, University of Tübingen, Germany
,
V. Scheble
2   Institute of Pathology, University of Tübingen, Germany
,
S. Miller
3   Department of Radiology, University of Tübingen, Germany
,
A. Heininger
4   Department of Anaesthesiology and Intensive Care Medicine, University of Tübingen, Germany
,
F. Fend
2   Institute of Pathology, University of Tübingen, Germany
,
A. Koenigsrainer
1   Department of General-, Visceral- and Transplant Surgery, University of Tübingen, Germany
› Author Affiliations
Further Information

Publication History

22 April 2012

18 August 2012

Publication Date:
07 December 2012 (online)

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Abstract

In systemic amyloidosis spontaneous rupture of the liver is a rare complication. Here we report on a patient with unrecognized systemic amyloidosis who presented to an outside hospital with unspecific abdominal pain. Under the signs of a hemorrhagic shock, distended abdomen and intraabdominal bleeding a laparotomy was performed. Due to uncontrollable hemorrhage of the ruptured right liver lobe a packing was performed. After being transferred to the reporting institution a CT scan was performed showing a grade IV laceration of the right liver. Therefore a transarterial embolization of the right hepatic artery was carried out. The following day an infection of the abdominal cavity and the abdominal wall with gas-producing bacteria was noticed and a relaparotomy, necrectomy and repacking due to diffuse bleeding were performed. The situation deteriorated and at the second relaparotomy the following day an almost completely necrotic liver was found. The patient deceased the following day. Life-threatening spontaneous liver rupture due to systemic amyloidosis might only successfully be cured by high urgency liver transplantation as presented in the literature. However, in two published cases interventional therapy by embolization of bleeding vessels has saved patients’ life.

Zusammenfassung

Die spontane Leberruptur aufgrund einer systemischen Amyloidose stellt eine sehr seltene Komplikation dar. Wir berichten von einem Patienten mit einer bis dato unbekannten Amyloidose, der in einem auswärtigen Krankenhaus mit unspezifischen abdominellen Beschwerden aufgenommen wurde. Es zeigte sich im stationären Verlauf ein sich rasch entwickelnder hämorrhagischer Schock bei einer massiven intraabdominellen Blutung, sodass der Patient unter Notfallbedingungen laparotomiert wurde. Intraoperativ fand sich eine unkontrollierbare Blutung aus einem rupturierten rechten Leberlappen, sodass als Ultima Ratio ein Packing der Leber durchgeführt wurde. Anschließend wurde der Patient bei instabilen Kreislaufverhältnissen und kontinuierlicher Blutung in unser Klinikum verlegt. Eine bei Aufnahme durchgeführte CT-Untersuchung zeigte weiterhin eine massive Blutung aus der rechten Leber. Daraufhin erfolgte eine transarterielle Embolisation der A. hepatica dextra, welche primär erfolgreich war. Im weiteren Verlauf entwickelte sich eine intraabdominelle Infektion mit gasbildenden Keimen, sodass eine operative Revision erfolgte. Am Folgetag war bei progredienter Sepsis eine erneute Revision indiziert, bei der eine nahezu komplette Nekrose der Leber diagnostiziert wurde. Bei infauster Prognose verstarb der Patient am folgenden Tag. Die Therapie der Wahl in der beschriebenen, hochdramatischen Situation der spontanen Leberruptur bei Amyloidose besteht im Wesentlichen nur aus dem Organersatz im Sinne einer Notfall-Lebertransplantation. Jedoch wurden zweimal in der Literatur auch erfolgreich Embolisationsverfahren beschrieben, sodass die interventionelle Therapie als eine sinnvolle therapeutische Option bei erhaltener Organfunktion in Betracht gezogen werden sollte.

Key words

hepatic rupture - amyloidosis - liver embolization - liver packing

Schlüsselwörter

Leberruptur - Amyloidosis - arterielle Embolisation - Packing der Leber
 

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