Portal Hypertension as Portrayed
by
Marked
Hepatosplenomegaly: Case Report
Robin A. Greene
Yale
University Medical Center/Yale New Haven Hospital, New Haven, Connecticut
The liver is vulnerable
to
a host
of
disease processes, includ-
ing portal hypertension. This is a severe hepatic condition in
which the liver is subject to numerous imbalances: increased
hepatic blood
flow,
increased portal vein pressure due to extra-
hepatic portal vein obstruction, and/or increases in hepatic
blood flow resistance (J). Although many disease states may
be responsible for the development
of
portal hypertension, it
is most commonly associated with moderately severe
or
ad-
vanced cirrhosis (2). Advanced, untreated portal hypertension
may
cause additional complications such as hepatosplenomeg-
aly, gastrointestinal bleeding, and ascites {1).
CASE
REPORT
The patient was a 22-yr-old man with a long history
of
sar-
coidosis, a chronic granulomatous disease process
of
unknown
etiology. Sarcoidosis is characterized
by
the formation
of
tubercle-like lesions in affected organs, usually the skin, lymph
nodes, lungs, and bone marrow (3). This patient had confirmed
pulmonary, eye, and dermatologic involvement. Because
of
persistent elevation in liver function tests, the clinicians be-
lieved that sarcoid involvement
of
the liver was present as well.
An abdominal exam revealed possible hepatosplenomegaly,
and a liver/spleen scan was requested.
After the intravenous administration
of
6 mCi (222 MBq
of
technetium-99m (
99
mTc)
sulfur colloid, multiple planar
images
of
the liver and spleen were obtained with a scintillation
camera. The images were collected for 750,000 counts each,
employing a 20% window that was centered on the
140
keY
photopeak
of
99
mTc.
High resolution collimation was used.
Image evaluation (Fig.
1)
revealed that both the liver and
spleen were enlarged (the spleen measured 23 em in its longest
dimension). No focal defects were apparent in the images.
There was minimal shift
of
radiocolloid from the liver to the
spleen.
The patient did not agree to a liver biopsy so there was
no confirmed diagnosis, although infectious hepatitis was
suspected based on all
of
the other tests performed.
DISCUSSION
In normal persons, the distribution ratio
of
99
mTc
radio-
colloid between the liver and spleen is about 5.5:1. In the
presence
of
portal hypertension this ratio decreases
or
may
For reprints contact:
Robin
A.
Greene,
Yale
University School of Medicine,
TIMI Core
Lab,
Fitkin
2,
Room
206,
333
Cedar Street,
New
Haven,
Cf
06504.
VOLUME
15,
NUMBER
4,
DECEMBER 1987
be reversed in more severe cases (2). This individual's
99
mTc
sulfur colloid images demonstrated the combination
of
an
enlarged liver and spleen, with homogeneous distribution of
radiocolloid. A slight shift in activity to the spleen was also
noted. The clinician's diagnosis based on these findings sug-
gested diffuse hepatocellular disease. The confirmation
of
this
diagnosis will require subsequent testing for other processes
such as infectious hepatitis, which has been found to produce
TABLE
1.
Causes of Hepatosplenomegaly
Common
Metastatic tumors
Fatty infiltration
Hepatitis
Cirrhosis
Congestive heart failure
Abscess
Leukemia
Lymphoma
Normal variants-Reidel's lobe
Uncommon
Infection
Tuberculosis
Infectious mononucleosis
Hemochromatosis
Chronic passive congestion
Trauma
Hemolysis
Erythroblastosis fetalis
Chronic hemolytic anemia
Drugs
Phenobarbital
Diphenylhydantoin
Sulfonamides
Acetaminophen
Tetracycline
Corticosteroids
Methotrexate
Androgens
Primary tumors
Cysts (hydatid)
Rare
Inherited metabolic disorders
with hepatic involvement
Wolman's disease
Glycogen storage disease
Rare (continued)
Wilson's disease
Gaucher's disease
M ucopolysaccharidosis
Niemann-Pick disease
Gangliosidosis
Alpha 1-antitrypsin deficiency
Hepatic porphyrias
Cystic fibrosis
Histiocytosis X
Galactosemia
Acromegaly
Polycystic disease
Inflammatory noninfective
disorders
Sarcoidosis
Granulomatous hepatitis
Juvenile rheumatoid arthritis
Kwashiorkor
Biliary obstruction
Amyloidosis
Vascular disorders
Hereditary hemorrhagic
telangiectasia
Multinodular
hemangiomatosis
Cavernous hemangiomas
Budd-Chiari syndrome
Jamaican vomiting disease
Infection
Congenital
or
postnatal
syphilis
Schistosomiasis
Amebiasis
Actinomycosis
Hydatid cyst
Weil's disease
Proxysmal nocturnal
hemoglobinuria
187
