PEDIATRICS Vol. 100 No. 2 August 1997,
p. e10
Copyright ©1997 by the American Academy of Pediatrics
ELECTRONIC ARTICLE:
Auxiliary Liver Transplant in Fulminant Failure
Philip Rosenthal,
John P. Roberts,
Nancy L. Ascher, and
John C. Emond
Pediatric Liver Transplant Program
University of California, San Francisco Medical Center
San Francisco, CA 94143
ABSTRACT
- CASE REPORT
- DISCUSSION
- ACKNOWLEDGMENT
- ABBREVIATIONS
- REFERENCES
ABSTRACT 
Fulminant hepatic failure (FHF) is defined as a
syndrome of acute liver failure with the development of hepatic
encephalopathy and severe hypoprothrombinemia occurring within 2 months
of the onset of symptoms or jaundice in a person without preexisting liver disease.1 Total orthotopic liver transplantation
(OLTX) is a lifesaving therapeutic option for patients with FHF, but currently requires lifelong immunosuppression to maintain the graft.2 Auxiliary partial orthotopic liver transplantation (A-OLTX) is a procedure whereby only a portion of the native liver is
removed, and the remainder of the native liver is left in
situ.3 A-OLTX provides temporary support until the native
liver recovers and immunosuppression can be withdrawn.
We describe the successful application of emergency A-OLTX in a
young girl who accidentally ingested Amanita phalloides
mushrooms and developed FHF.
Key words:
auxiliary liver
transplant,
fulminant hepatic failure,
emergency,
Amanita phalloides.
CASE REPORT
A13-year-old girl went wild mushroom picking with her
family in a local reservoir and inadvertently harvested several
Amanita phalloides mushrooms (death cap). Upon their return
home, her family prepared a meal consisting of the olive green-brown
capped mushrooms with white gills and a white stem in a sauce with
spaghetti. Within 12 hours, the family sought medical attention because
of symptoms of abdominal cramping, nausea, vomiting, and watery
diarrhea. The patient and her family were seen in a local emergency
facility and sent home with prochlorperazine and advised to maintain
good fluid intake. However, because of persistent symptoms, the patient returned for evaluation. Initial laboratory studies 2 days after ingestion revealed serum aspartate aminotransferase (AST) 281 IU/L
(normal: 12-42), alanine aminotransferase (ALT) 392 IU/L (9-50), and
alkaline phosphatase 222 IU/L (150-420). She was treated with
intravenous fluids, prochlorperazine, and intravenous penicillin G (250 mg/kg/day). Her physical exam was notable for asterixis and
hepatomegaly. Within 72 hours of ingestion, her AST increased to 1290 IU/L, ALT 2094 IU/L, and prothrombin time 37 seconds (control 10.3-14.1 seconds). She was transferred to the University of
California, San Francisco Medical Center for management of FHF and
listed for liver transplantation. Laboratory studies performed there revealed serum AST >4500 IU/L, ALT >6000 IU/L, total bilirubin 3.4 mg/dL [(58.1 µmol/L) normal: .1-1.2 mg/dL], prothrombin time 56 seconds, and ammonia 80 mmol/L (11-35). She became progressively lethargic and encephalopathic. On day 4 after ingestion, a donor organ
became available. The donor cadaveric liver was split using standard
techniques such that an adult received the right lobe, and the girl
received a left A-OLTX. She was begun on immunosuppression with
cyclosporine, mycophenolate mofetil, and prednisone. Her explanted liver section demonstrated extensive diffuse
centrilobular necrosis consistent with Amanitin poisoning (Fig
1). Her coagulopathy and serum transaminases rapidly
corrected after A-OLTX. A nuclear hepatobiliary scan at 5 days after
A-OLTX revealed equal functioning of the native liver and grafted liver
with visualization of the biliary tree and gallbladder by 20 minutes
(Fig 2A). By 8 days after A-OLTX, hepatobiliary scan showed
the native liver had improved function with excretion of tracer into
the intestine by 10 minutes but the grafted liver had less function
(Fig 2B). The A-OLTX was surgically removed and all immunosuppression
was stopped. Repeat histology of the native liver revealed only 10% to
20% of the parenchyma necrotic (Fig 3). She recovered
rapidly and was discharged home in 16 days. Follow-up at 4 months
revealed a normally functioning regenerated native liver with normal
biochemical parameters that continues 9 months after ingestion.
Fig. 1.
Sample from the liver at time of auxiliary transplant shows extensive
necrosis in zone 2 and 3 (mid-zonal and central, center of photo to
right lower) with focal involvement of zone 1 (periportal, upper left).
This view shows some viable periportal hepatocytes. Congestion of the
necrotic zones is also present (center) (hematoxylin and eosin, 50X).
[View Larger Version of this Image (154K GIF file)]
Fig. 2.
A, Nuclear hepatobiliary scan obtained 5 days after A-OLTX reveals
equal functioning of the native liver (on the left) and grafted liver
(on the right) with visualization of the biliary tree and gallbladder
by 20 minutes. B, Nuclear hepatobiliary scan obtained 8 days after
A-OLTX reveals the native liver has improved function with excretion of
tracer into the intestine by 10 minutes but the grafted liver has
negligible function.
[View Larger Version of this Image (61K GIF file)]
Fig. 3.
Liver biopsy from the right lobe 8 days after transplantation shows
residual zone 3 (centrilobular) necrosis and congestion (center), with
viable cells in other zones (hematoxylin and eosin, 50X).
[View Larger Version of this Image (152K GIF file)]
DISCUSSION
Liver transplantation for FHF has been well-accepted in
recent years, accounting for 5% to 10% of indications for
OLTX.4 Survival rates have varied substantially
between centers ranging from 50% to over 90%.4 This
variation has been due in large part to the difficulty in finding
donors within the short time span before irreversible brain injury, and
the use of marginal donors with poor initial graft function in this
context. Progress in recent years has included the development of
improved prognostic scores for the selection of patients for OLTX and
the use of intracranial pressure monitoring for selection of viable
candidates and perioperative monitoring.
Despite improvements in transplantation therapy, recovery of the
native liver obviating the need for lifetime immunosuppression is the
optimum outcome for patients with acute liver failure. To achieve this
end, artificial liver support and the use of auxiliary liver grafts
with preservation of the native liver have been
introduced.3 Although artificial liver devices have had
limited success to date, up to 40% of patients supported with
auxiliary liver transplants may have recovery of their own liver with
subsequent withdrawal of immunosuppression.
FHF caused by acute toxic ingestions offer an ideal setting in which to
perform A-OLTX. The insult is discrete, structural damage may be
minimal, and no infectious agent is involved.
Emergency A-OLTX for FHF has the distinct advantages of
permanently stopping immunosuppressive therapy with withdrawal of the
auxiliary graft and allowing recovery of full native liver function as
demonstrated in the present case. Obviously, A-OLTX is not appropriate
therapy for all patients. Criteria for prediction of those patients who
are expected to have complete native liver regeneration and who could
benefit from A-OLTX are not established. For FHF, young age, viral
hepatitis or acetaminophen overdose as etiologies of FHF, and a delay
between the onset of jaundice and encephalopathy less than 7 days are
recognized predictors of a favorable outcome for patients with
FHF.1,5 Amanita phalloides ingestion is a rare
enough condition such that prognostic criteria are not well recognized.
The extent of hepatic injury might be expected to influence outcome
because liver regeneration is required for ultimate recovery. Our
patient had extensive diffuse centrilobular necrosis in her native
liver at the time of emergency A-OLTX, and her native liver recovered
completely. This finding is in agreement with other reports indicating
that the extent of hepatocyte necrosis on liver biopsy is of no value
in predicting the survival of patients with FHF.3,6
Surviving hepatocytes, even if few in number but if relatively
damage-free, have the potential for extensive regeneration with time
and the potential for ultimate complete recovery of the native liver.
Situations where there is injury from a limited single hepatotoxin
exposure, as in the current case, may theoretically be the most
appropriate candidates for emergency A-OLTX. Based on our successful
case, application of emergency A-OLTX should be considered a
therapeutic option for children with FHF from Amanita
phalloides ingestion or other hepatotoxins when the native liver
has the potential to regenerate and recover.
FOOTNOTES
Received for publication Jan 17, 1997; accepted Apr 1, 1997.
Address correspondence to: Philip Rosenthal, MD, University of
California, San Francisco Medical Center, 500 Parnassus Avenue, MU
4-East, San Francisco, CA 94143-0136.
ACKNOWLEDGMENT
The authors thank Dr Linda Ferrell for her help with the
histology in this case.
ABBREVIATIONS
FHF, fulminant hepatic failure.
OLTX, orthotopic
liver transplantation.
A-OLTX, auxiliary partial orthotopic liver
transplantation.
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