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<Case
Presentation>
A 28 year-old male is
admitted to the hospital due to poor appetite and jaundice for
about two months.
The patient,
who is a Tibetian, was otherwise healthy until two months
prior to this admission. He had been to many places including
India, Nepal, India and Singapore in the past years. He stayed
in Taipei most of the time in recent two years. He went to
Hong Kong for a short time two months prior to this admission.
During the stay in Hong Kong, he began to suffer from poor
appetite, malaise, generalized itching, icteric skin and
sclera. Tea-colored urine and clay color stool were also
noted. He visited hospital on Dec 23, 2002 and was admitted.
During physical examination, he appears thin and had deeply
icteric skin and sclera. The temperature was 37℃, the pulse
was 84, and the respirations were 18. The blood pressure was
120/70 mm Hg. The conjunctiva is not pale. Neither neck
stiffness, jugular vein engorgement nor neck lymphadenopathy
was found. The lungs were clear. The heart sounds were regular
without murmurs. The abdomen was ovoid and soft without
tenderness. Liver and spleen were impalpable. The legs had no
pitting edema. No joint pain or swelling was observed. A
neurologic examination showed no abnormalities. During the
period before admission, there was no abdominal pain,
diarrhea, nausea, vomiting, tarry stool, bowel habit change or
cold sweating. He had weight loss about 6kg in two months.
Blood check showed elevated ALP and hyperbilirubinemia
(Bil-T/D: 15.6/8.0 mg/dl). Abdominal plain film revealed a
calcified mass at RUQ. （Figure
1）Abdominal sonography on Dec 24 showed a huge
heterogeneous (up to 13cm), ill-defined mass at right lobe.
IHD and CBD were not dilated. （Figure 2
A & B）Both
HBsAg and Anti-HCV were negative and tumor markers such as
alpha-fetoprotein(<3.0) and CA19-9(28.86<35) were within
normal limits. Abdominal CT (Dec 26) showed a well-defined
lobulated low density soft tissue mass about 11x12x12cm with
central amorphous and extensive calcification. （Figure 3
A & B
） It directly
extended to right adrenal gland and caused left portal vein
compression, right portal vein total encasement, left lobe IHD
dilatation and IVC encasement with collateral circulation.
Ciprofloxacin, ethambutol and streptomycin were administered
empirically for 3 days under the impression of tuberculosis
but discontinued because of no obvious improvement. Liver
biopsy was performed and pathological finding revealed
necrotic tissue surrounded by cholestatic hepatic tissue with
eosinophil and lymphocyte infiltrations.（Figure 4
） AFS, silver stain, GMS, PAS were all
negative. TB-PCR was negative.
<Laboratory Data>
CBC
| |
RBC
M/μL |
Hb
g/dL |
Hct
% |
MCV
fL
|
PLT
K/μL |
WBC
K/μL
|
Band
% |
Neu
% |
Lym
% |
Bas
% |
Mon
% |
Eos
% |
Absolute Eos
|
| 2002/12/23 |
4.12 |
11.3 |
33.2 |
83.2 |
388 |
10.02 |
4 |
57.5 |
18.5 |
0.2 |
6.7 |
13.1 |
1312 |
| 2003/01/03 |
3.93 |
11.1 |
32.3 |
82.2 |
397 |
9.11 |
2 |
58.6 |
18.2 |
0 |
7.1 |
12.1 |
1102 |
| 2003/02/13 |
3.92 |
11.8 |
34.6 |
88.3 |
232 |
8.62 |
0 |
57.5 |
22.3 |
0.9 |
7.4 |
11.9 |
1025 |
| 2003/02/24 |
3.14 |
9.1 |
27.1 |
86.3 |
324 |
5.40 |
0 |
52.5 |
29.1 |
1.9 |
6.3 |
10.2 |
551 |
BCS+electrolytes
| |
Bil(T)
mg/dL |
Bil(D)
mg/dL |
AST
U/L |
ALT
U/L |
ALP
U/L |
GGT
U/L |
BUN
mg/dL
|
CRE
mg/dL |
CRP
mg/dL |
| 2002/12/23 |
15.6 |
8.0 |
65 |
57 |
844 |
|
8 |
1.1 |
|
| 2003/01/07 |
22.49 |
|
71 |
44 |
1040 |
45 |
10.4 |
0.73 |
|
| 2003/01/13 |
14.0 |
11.4 |
63 |
45 |
645 |
30 |
9.0 |
0.6 |
2.66 |
| 2003/01/20 |
9.0 |
7.4 |
58 |
39 |
571 |
62 |
6.5 |
0.7 |
2.90 |
| 2003/01/27 |
5.7 |
5.0 |
55 |
35 |
590 |
81 |
6.9 |
0.8 |
|
| 2003/02/24 |
1.8 |
1.2 |
30 |
14 |
637 |
92 |
7.2 |
0.8 |
|
| |
Na
mmol/L |
K
mmol/L |
Ca
mmol/L |
Albumin
g/dL |
Globulin
g/dL |
LDH
U/L |
Glucose
mg/dL |
| 2002/12/23 |
137 |
3.4 |
9.2 |
4.3 |
4.7 |
675 |
101 |
| 2003/01/07 |
143 |
3.5 |
2.22 |
4.01 |
5.72 |
466 |
77 |
| 2003/01/13 |
138 |
3.7 |
2.36 |
3.6 |
4.6 |
371 |
|
| 2003/01/27 |
139 |
4.2 |
2.15 |
3.6 |
4.9 |
403 |
|
| 2003/02/24 |
138 |
3.8 |
2.07 |
3.9 |
4.1 |
|
106 |
Coagulation profiles
| |
PT
sec |
PT Cont.
sec |
INR
|
PTT
sec |
PTT Cont.
sec
|
| 2002/12/23 |
13.1 |
11.5 |
1.3 |
34.3 |
27.9 |
| 2003/01/07 |
19.2 |
11.4 |
1.6 |
61.6 |
36.5 |
| 2003/01/16 |
13.7 |
11.0 |
1.2 |
47.4 |
35.8 |
| 2003/02/17 |
17.7 |
10.4 |
1.5 |
54.6 |
36.3 |
Stool exams
|
Stool |
Outlook |
Prarsite ova |
OB |
Pus cell |
|
01/07 |
White, formed |
negative |
4+ |
negative |
| |
CA19-9
<37(U/mL) |
CEA
<3(ng/mL) |
| 1/9 |
47.9 |
0.92 |
| |
Ferritin
10.2~265
(ng/mL) |
Iron
33 ~ 167
(μg/dL) |
TIBC
275 ~ 332
(μg/dL) |
| 1/9 |
983 |
56 |
177 |
<Course and Treatment>
Percutaneous
transhepatic cholangiodrainage（PTCD） was performed smoothly on
Jan 9 and his jaundice and pruritis improved gradually. Stool
and bile were sent for parasite and ovum study and all were
negative. After angiography of abdomen on Jan 25 for detailed
anatomy, he was then transferred to general surgical ward on
Jan 28, 2003. Extended right lobectomy with partial tumor
excision was performed on Feb 5. A huge tumor (12x15 cm in
size), yellowish and hard with partial caseous necrosis-like
substance located at right lobe of liver with encasement of
IVC with diaphragm adhesion was observed during
operation.
Post-operative course was rather smooth and pathology favored
Echinococcal infection. Albendazole 400mg bid for 6 courses (6
weeks, then rest for 2 weeks) was administered and he was
discharged smoothly on March 1, 2003 and will be regularly
followed up at outpatient
clinic.
<Case Analysis>
This is a
case of Echonococcus multilocularis infection
with local invasion. It causes multilocular alveolar lesions
that are locally invasive and is found in sub-Arctic or Arctic
regions, including Canada, the United States, and northern
Europe and Asia. The definitive hosts are dogs that pass eggs
in their feces. Cysts develop in the intermediate host-mice
and other rodents after the ingestion of eggs. When a dog
ingests beef or lamb containing cysts, the life cycle is
completed. Slowly enlarging echinococcal cysts generally
remain asymptomatic until their expanding size or their
space-occupying effect in an involved organ elicits symptoms.
The liver and the lungs are the most common sites of these
cysts. Since a period of years elapses before cysts enlarge
sufficiently to cause symptoms, they may be discovered
incidentally on a routine x-ray or ultrasound
study.
Patients with
hepatic echinococcosis who are symptomatic most often present
with abdominal pain or a palpable mass in the right upper
quadrant. Compression of a bile duct or leakage of cyst fluid
into the biliary tree may mimic recurrent cholelithiasis, and
biliary obstruction can result in jaundice. Rupture of or
episodic leakage from a hydatid cyst may produce fever,
pruritus, urticaria, eosinophilia, or anaphylaxis. Pulmonary
hydatid cysts may rupture into the bronchial tree or
peritoneal cavity and produce cough, chest pain, or
hemoptysis. Rupture of hydatid cysts may lead to multifocal
dissemination of protoscolices, which can form additional
cysts. Rupture can occur spontaneously or at surgery. Other
presentations are due to the involvement of bone (invasion of
the medullary cavity with slow bone erosion producing
pathologic fractures), the central nervous system
(space-occupying lesions), and the heart (conduction defects,
pericarditis).
The
cysts of E. multilocularis characteristically
present as a slowly growing hepatic tumor, with progressive
destruction of the liver and extension into vital structures.
Patients commonly complain of upper quadrant and epigastric
pain, and obstructive jaundice may be apparent. A minority of
patients experience the metastasis of lesions to the lung and
brain. Radiographic and related imaging studies are important
in detecting and evaluating echinococcal cysts. Plain films
will define pulmonary cysts-usually as rounded irregular
masses of uniform density-but may miss cysts in other organs
unless there is cyst wall calcification (as occurs in the
liver). MRI, CT and ultrasound reveal well-defined cysts with
thick or thin walls. When older cysts contain a layer of
hydatid sand that is rich in accumulated scolices, these
imaging methods may detect this fluid layer of different
density. However, the most pathognomonic finding, if
demonstrable, is that of daughter cysts within the larger
cyst.
A specific
diagnosis can be made by the examination of aspirated fluids
for scoliceal hooklets, but diagnostic aspiration is not
usually recommended because of the risk of fluid leakage
resulting in either dissemination of infection or anaphylactic
reactions. Serodiagnostic assays can be useful, although a
negative test does not exclude the diagnosis of
echinococcosis. Cysts in the liver elicit positive antibody
responses in ~90% of cases, whereas up to 50% of individuals
with cysts in the lungs are seronegative. Detection of
antibody to specific echinococcal antigens by immunoblotting
specificity.
Therapy for
echinococcosis is based on considerations of the size,
location, and manifestations of cysts and the overall health
of the patient. Surgery has traditionally been the principal
definitive method of treatment; tissue containing E.
multilocularis cysts is resected. Risks at surgery
from leakage of fluid include anaphylaxis and dissemination of
infectious scolices. The latter complication has been
minimized by the instillation of scolicidal solutions such as
hypertonic saline or ethanol, which may cause hypernatremia,
intoxication, or sclerosing cholangitis. Albendazole, which is
active against Echinococcus, should be administered
adjunctively, beginning before resection and continuing for 2
years for E. multilocularis. Percutaneous
aspiration, infusion of scolicidal agents, and reaspiration
(PAIR) can be used instead of surgery in many cases of cystic
echinococcosis. PAIR is contraindicated for superficially
located cysts (because of the risk of rupture), for cysts with
multiple thick internal septal divisions (honeycombing
pattern), and for cysts communicating with the biliary tree.
Therapy with albendazole (15 mg/kg daily in two divided doses)
should be initiated at least 4 days before the procedure and
continued for at least 4 weeks afterward. Ultrasound- or
CT-guided aspiration allows confirmation of the diagnosis by
demonstration of protoscolices in the aspirate. Either alcohol
or hypertonic saline should then be infused. Daughter cysts
within the primary cyst may need to be punctured separately.
In experienced hands, this approach yields rates of cure and
relapse equivalent to those following surgery, with less
perioperative morbidity and shorter hospitalization. Medical
therapy with albendazole alone for 12 weeks to 6 months
results in cure in ~30% of cases and improvement in another
50%. Many of the failures are subsequently treated
successfully with PAIR or additional courses of medical
therapy. Response to treatment is best assessed by serial
imaging studies with attention to cyst size and
consistency.
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Case Discussion
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