Good outcome following liver transplantation using pericardial-peritoneum window for hepato-atrial anastomosis to overcome advanced hepatic alveolar echinococcosis and secondary Budd-Chiari Syndrome - a case report
Kobryń et al. BMC Surgery
Good outcome following liver transplantation using pericardial- peritoneum window for hepato-atrial anastomosis to overcome advanced hepatic alveolar echinococcosis and secondary Budd-Chiari Syndrome - a case report
Konrad Kobryń 0
Rafał Paluszkiewicz 0
Krzysztof Dudek 0
Urszula Ołdakowska-Jedynak 1
Michał Korba 0
Joanna Raszeja-Wyszomirska 1
Piotr Remiszewski 0
Michał Grąt 0
Piotr Milkiewicz 1
Waldemar Patkowski 0
Marek Krawczyk 0
0 Department of General, Transplant and Liver Surgery, Medical University of Warsaw , Poland. Banacha Street 1a, 02-097 Warsaw , Poland
1 Department of Hepatology and Internal Diseases, Medical University of Warsaw , Warsaw , Poland
Background: This report presents a case of a 57- year old female with advanced Hepatic Alveolar Echinococcosis causing a secondary Budd-Chiari Syndrome due to infiltration of the suprahepatic inferior vena cava treated successfully by liver transplantation. Case presentation: A temporary veno-venous bypass was introduced, but a typical end to end cavo-caval anastomosis wasn't possible in this case. In order to access a disease free part of the inferior vena cava, an oval window of the diaphragm was excised, providing communication between the peritoneum and pericardium. A vascular clamp was placed onto the right atrium which allowed for an atrial-caval anastomosis. The remainder of hepatectomy was performed in a conventional manner. In the post-operative period and during the 18 month follow-up there were no complications. The patient remains in good general condition with optimal graft function. Conclusions: A hepato-atrial anastomosis with a pericardial-peritoneum window during liver transplantation is feasible and extends the curability potential for patients with advanced Hepatic Alveolar Echinococcosis considered for liver transplantation.
Atrium-IVC anastomosis; Budd-Chiari Syndrome; Hepatic alveolar Echinococcosis; Liver transplantation; Pericardial-peritoneum window; Case report
Advanced hepatic alveococcosis due to its volume may
interfere with the hepatic veins and inferior vena cava
(IVC) causing Budd-Chiari syndrome (BCS) which is an
indication for liver transplantation (LTx). The
thrombosis and stricture may be found at any point, ranging
from the hepatic veins to the right atrium along the IVC
[1, 2]. When this is the case, hepatectomy of the
diseased liver requires supra hepatic IVC clamping at
the right atrium, with later end-to-end anastomosis
with the donor liver IVC cuff [3–5]. Our report
presents a rare approach during hepatectomy,
perioperative management and excellent postoperative results
of the patient.
A 57 year old female patient was qualified for LTx due
to BCS caused by an advanced Echinococcus
multilocularis infection. The patient received Albendazole
treatment for several years which was terminated two years
prior to transplantation due to a developed post-drug
pancytopenia. No other anthelminthic drugs were
administered until LTx. Furthermore, portal hypertension,
esophageal and gastric varices, portal hypertensive
gastropathy, and ascites were present at time of transplant
qualification. Celiac disease, gastro-esophageal reflux
disease (GERD), and L1 vertebral fracture were
The size of the alveolar lesion in segments III–VIII
was 110 × 75 × 70 mm (Fig. 1a) and involved the hepatic
vein confluence, causing narrowing of the IVC lumen
with mild but disturbed blood flow. The intrahepatic
IVC was free of abnormalities. The lesion occluded the
left and middle hepatic veins, extending towards the
confluence of the right hepatic vein with the IVC
(Fig. 1b). The lumen was narrowed, with an organized
thrombus visible on ultrasound, but blood flow was
The donor liver was retrieved from a 43 year old dead
brain donor (DBD) matching the recipient’s blood type
and similar weight and height values. The LTx began on
the 16th September, 2014 with the use of an
extracorporeal veno-venous bypass. The portal vein and
hepatoduodenal ligament was difficult to dissect as it
was embodied in post-inflammatory scar tissue. The
suprahepatic IVC along with the surrounding diaphragm
was involved in the alveolar lesion. A 10 × 10 cm
window of the diaphragm was excised creating an opening
of the right pleural cavity and pericardium (Fig. 2). Thus,
with the exposure of the pericardial part of the IVC, the
vascular clamp was placed on the atrium because the
whole IVC was infiltrated by the lesion. The infrahepatic
part of the IVC was clamped in a standard procedure.
Both ends of the IVC were then cut, and the liver was
explanted along with its intrahepatic IVC (Fig. 3a). The
clamping of the right atrium caused cardiac arrhythmia
and the electrocardiogram showed signs of myocardial
ischemia. This was managed by the anesthesiologist and
hemodynamic stability was achieved throughout the
Two 24 fr pleural drains were placed into the apex
and base of the right pleural space. The supra hepatic
IVC of the donor graft was sutured directly to the right
atrium using an end-to-end anastomosis. The excised
window in the diaphragm was minimized by suturing its
free ends together without the use of a Prolene patch,
and without complete closure of the diaphragm in order
to avoid stenosis of the IVC–atrium anastomosis.
Communication between the pericardium and right pleural
cavity was intentionally sustained to allow for fluid
drainage. After completion of the portal anastomosis
clamps from the IVC and portal vein were removed. A
Fig. 1 Pre-Transplant CT-scans presenting: a Computed Tomography of a 110 × 75 × 70 mm hepatic alveolar echinoccocal cyst located in
segments III–VIII; b Hepatic vein thrombosis – Budd-Chiari Syndrome
mild, 1-min reperfusion syndrome with a systolic
pressure of 60 mmHg was observed after 8 h 6 min of cold
ischemia. At that point, pleural drainage was enabled.
Further steps during transplantation were performed
in a standard procedure. Intraoperative blood loss was
estimated at 1000 ml; thus, 2 units of red blood
concentrate and 4 units of fresh frozen plasma were
transfused. The anhepatic phase was 136 min. Total
operation time was 6.5 h.
An abdominal ultrasound was performed on the first
post-operative day (POD), showing good vascular flow.
An echocardiography performed on the second POD
found no abnormalities. The pleural drain was removed
on the third POD and a chest X-ray confirmed no signs
of pneumothorax or effusion in the right pleural space.
Time in the intensive transplant care unit was 3.5 days.
The immunosuppressive regimen consisted of
tacrolimus 0.05 mg/kg × 2 and prednisolone 20 mg × 1. The
patient’s liver tests recovered gradually. Computed
tomography showed satisfactory and functioning
hepatoatrial anastomosis (Fig. 4a and b). She was discharged on
POD 25 and continued with tacrolimus 7 mg × 2,
mycophenolate mofetil 500 mg × 2 and albendazole treatment
400 mg × once daily. Intolerance of the latter drug was
not observed during hospitalization. At an 18-month
follow-up and recurrence free period, the patient is
doing well with good overall outcome and graft
Hepatic alveolar echinococcosis (HAE) is a parasitic
disease that develops slowly. Very often, the first symptoms
manifest, as in our case of E. multilocularis (Fig. 3b), at
a very advanced, mature stage. In the first imaging
examinations, HAE can resemble a tumor like
hepatocellular carcinoma. Clinical signs, such as mechanical
jaundice, repetitive cholangitis, liver abscess, portal
hypertension, esophageal varices, and sepsis are
secondary to the disease. Additionally, Budd-Chiari Syndrome
might be present [3–6].
Surgical resection usually is the main treatment for
this disease; however, because alveolar symptoms tend to
be absent for a long period of time, the cysts tend to be
very advanced at diagnosis. Large lesions without
response to drug treatment and with invasion of the
vascular tree (Fig. 3c) of the liver will eventually develop
BCS, thus should be directly qualified for LTx. This
seems to be the only final treatment for HAE, and 90%
of patients die within 10 years without surgery [4–7].
LTx due to HAE has been reported to have
increased morbidity and mortality. A thorough
examination is compulsory with MRI and three-dimensional
computed tomography reconstruction in order to
minimize intraoperative risk . An early remark on
the use of veno-venous bypass allows for better
intraoperative preparation and greatly minimizes the risk
of bowel congestion, shortens the hepatectomy time
and limits the need for excessive maneuvers with the
diseased liver, reducing the risk of tissue impairment and
excessive bleeding [8–12]. A pericardial-peritoneum window
as a procedure has been reported many times, but is
performed due to malignant diseases or pericardial effusion
and not during LTx [8–10, 12–14].
Several authors state that a pericardial-peritoneum
window acts as a collection chamber for pericardial
fluid, which is later absorbed through the peritoneum
Fig. 3 a Explanted diseased liver; b Alveolar Cyst with E. multilocularis in the explanted liver; c Strictured lumen of the supra hepatic inferior vena
cava, which was invaded by an alveolar lesion (marked by needle). Dissected diaphragm measured 10 × 10 cm
Fig. 4 Post-transplant computed tomography scans: a Anterior CT image of hepato-atrial (atrial-caval) anastomosis; b 3-Dimensional computed
tomography reconstruction of the vascular image presenting the hepato-atrial anastomosis
[9, 10]. In our opinion, the implantation of the donor
liver through such a suprahepatic atrium-IVC cuff
end-to-end anastomosis allows both lobes of the liver
to firmly press against the diaphragm. This creates a
continuation of the thoracic-abdominal barrier,
simultaneously avoiding future diaphragmatic hernia. Thus,
leaving a 20-40-mm opening around the IVC
anastomosis avoids its occlusion in the future, provides
prompt drainage of fluid, and leaves the diaphragm
under less tension. There has been several
publications, mainly for initial BCS, presenting different
solutions of cavoplasty in order to perform an atrial-caval
anastomosis [15, 16]. Though principles are similar,
our report presents how to manage a rare case of
advanced HAE and in result secondary BCS.
LTx is feasible when performing an IVC hepato-atrial
anastomosis. Patient’s outcome is not impaired, and the
post-operative results, follow up, and quality of life is
good. Therefor even advanced cases of HAE may be
successfully treated with liver transplantation.
BCS: Budd-Chiari Syndrome; CD: Celiac Disease; EV: Esophageal Varices;
GERD: Gastro-Esophageal Reflux Disease; HAE: Hepatic Alveolar
Echinococcosis; IVC: Inferior vena cava; LTx: Liver transplantation; PDPS:
Post-drug pancytopenia syndrome; PHG: Portal Hypertensive Gastropathy;
POD: Post-operative day
Availability of data and materials
The datasets generated and/or analysed during the current study are
available from the corresponding author on reasonable request.
KK designed the report; KK, RP, KD, MKo performed the surgical
procedure; PM, JR-W, UO-J post-operatively treated the patient; KK
collected the patient’s clinical data; KK, RP, JR-W, PR, MG, PM, WP,
MKo analyzed the data; KK, RP, PM, MKr wrote and revised the paper.
All authors read and approved the final manuscript.
Ethics approval and consent to participate
The authors did attain the permission of the Medical University of Warsaw
Bioethics Committee (reference # AKBE157/16) to publish the data and
written, informed consent to participate was obtained from the patient.
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