Downloaded from www.ajronline.org by 209.197.169.221 on 11/07/15 from IP address 209.197.169.221. Copyright ARRS. For personal use only; all rights reserved
1277
Case Report 1;
#{149}
‘
. .
.
.
.
.
.
.
Transjugular Intrahepatic with Cystic Fibrosis Scott
R. Kerns1
and
Irvin
F. Hawkins,
.
.
portal
sive
congenital
surgical
due
to
portal
hypertension
had failed numerous illness
portosystemic
and shunt
her
from
times.
The nature
mother’s
reluctance
or liver
modified
cirrhosis,
and
of this progresprevented
I
Ross needle was used to direct a sharp 21-
April 20, 1992: accepted
Both authors: Department
AJR 159:1277-1278,
December
,
. )
#{149}:, .
.
#{149}‘-..:
- .
“:
.
.;
Shunt in a Child
on the anterior
by using sonographic
abdominal
guidance
wall with
to provide
venous system was entered initially which an 8-French catheter and 12advanced into the portal vein. Initial mm
Hg/36
mm
a 6-mm-diameter and stented with
Hg.
The
track
1992 0361 -803X/92/1596-1277
C American
balloon
angioplasty balloon a 42-mm Wallstent
Hg. Portography showed brisk flow through the shunt without filling of esophageal varices (Fig. 1 B). The child has been followed up for the past 6 months both clinically
flow sonography.
The shunt has remained
widely patent, and no further bleeding has occurred. A single episode ofencephalopathy was treated easily by altering the lactulose dosage
and dietary restrictions.
Discussion Cystic fibrosis is an inherited
disorder
ofthe exocrine
glands
that results most prominently in abnormalities of the alimentary tract, pancreas, and lungs. Biliary cirrhosis may develop in the liver as a result of mucous plugging of the bile ducts
followed by portal fibrosis. Advanced changes in the liver, as portrayed in this case, occur in less than 5% of patients with cystic fibrosis, but when present they can lead to portal hypertension and bleeding varices. Because of coexistent lung disease, poor nutritional status, and a propensity to infections, these patients are poor candidates for surgical portosystemic shunts or liver transplantation.
after revision June 5, 1992.
of Radiology. University of Florida, Box 100374,
an
a target
self-expanding metallic prosthesis (Schneider, Minneapolis, MN; Fig. 1A), which lowered the portal venous pressure to 36 mm Hg/31 mm
and with color Doppler a
transplantation.
gauge Hawkins needle guide (Cook, Bloomington, IN) anteriorly through the liver parenchyma under fluoroscopic guidance. The main Received
, . , .‘
during fluoroscopy. The portal with the needle guide, over French guiding catheter were pressures measured were 50 dilated with a 5-mm- and then (Medi-tech, Watertown, MA)
Physical examination showed that she was small for her age, had hepatosplenomegaly, and had clubbing of the fingers. No jaundice or ascites was present. Laboratory values included a low hematocrit of 30% (0.30) and mildly elevated prothrombin time (1 3.2 sec) and partial thromboplastin time (42 sec.) Total protein and albumin levels were normal at 7.1 g/dl (71 g/I) and 3.8 g/dI (38 g/I), respectively. However, results of liver function tests were abnormal: the aspartate aminotransferase level was 1 21 U/I, alanine aminotransferase level was 63 U/I, and total bilirubin level was 1 .6 mg/dI (27 ,moI/l). Her condition was considered Child class A. A transjugular intrahepatic portosystemic shunt was requested to alleviate the portal hypertension. The right internal jugular vein was punctured under sonographic guidance, and after serial dilatation, a 1 2-French guiding catheter was placed into the right hepatic vein. A blunt-tipped
..
. . ...
vein had been marked
ECG electrode
A 13-year-old girl with cystic fibrosis was referred to our service after multiple episodes of upper gastrointestinal hemorrhage. Pulmonary manifestations were mild, consisting only of shortness of breath with increased activity. She had had six to seven episodes of bleeding
.
Jr.
Case Report
sclerotherapy
,
#{149}
Portosystemic
We report a case of a 1 3-year-old girl with cystic fibrosis that manifested as cirrhosis, portal hypertension, and variceal bleeding. A transjugular intrahepatic portosystemic shunt was performed successfully with no recurrent hemorrhages.
variceal
:‘
JHMHC, Roentgen
Gainesville, Ray Society
FL 32610.
Address
reprint
requests to S. A. Kerns.
1278
KERNS
AND
HAWKINS
AJR:159,
Fig. 1.-A,
Radiograph
December
1992
shows Wallstent
pros-
Downloaded from www.ajronline.org by 209.197.169.221 on 11/07/15 from IP address 209.197.169.221. Copyright ARRS. For personal use only; all rights reserved
thesis (arrows) connecting hepatic and portal venous systems. B, Portogram shows brisk flow through shunt from portal vein (arrow) to hepatic vein and right atrium (arrowheads).
A
B
Transjugular intrahepatic portosystemic shunts have recently become a practical alternative to surgical portosystemic shunting for decompression of portal hypertension and treatment of variceal bleeding refractory to medical management and sclerotherapy. Although Colapinto et al. [1 ] first described creating a portosystemic shunt in humans by a nonsurgical approach in 1 982, the technique of percutaneous transjugular shunting has only recently been widely accepted. The use of metallic stents to maintain patency has vastly improved the efficacy of the procedure [2]. Although the long-term outcome of transjugular intrahepatic portosystemic shunts has yet to be determined, the shortterm effects appear promising. Shunt patency appears high, and if the shunt thromboses, it can be reopened percutaneously via a transjugular approach. Even though most investigators have performed this procedure on more critically ill
patients in Child mortality of the portosystemic option for those conditions that
B or C condition, the observed morbidity and procedure are lower than those for surgical shunts [3]. This case demonstrates a new caring for children with cystic fibrosis or other lead to cirrhosis and portal hypertension.
REFERENCES 1 . Colapinto RF, Stronell AD, Birch SJ, et al. Creation of an intrahepatic portosystemic shunt with a Gruentzig balloon catheter. Can Med Assoc J 1982;126:267-268
2. Palmaz JC, Garcia F, Sibbitt AR, et at. Expandable shunt
stents
in dogs
with
chronic
portal
intrahepatic
hypertension.
portacaval AJR
1986:
147:1251 -1 254 3. Richter GM, Noeldge G, Palmaz JC, Roessle M. The transjugular intrahepatic portosystemic stent-shunt (TIPSS): results of a pilot study. Cardiovasc Intervent Radiol 1990:13:200-207
1277
Case Report 1;
#{149}
‘
. .
.
.
.
.
.
.
Transjugular Intrahepatic with Cystic Fibrosis Scott
R. Kerns1
and
Irvin
F. Hawkins,
.
.
portal
sive
congenital
surgical
due
to
portal
hypertension
had failed numerous illness
portosystemic
and shunt
her
from
times.
The nature
mother’s
reluctance
or liver
modified
cirrhosis,
and
of this progresprevented
I
Ross needle was used to direct a sharp 21-
April 20, 1992: accepted
Both authors: Department
AJR 159:1277-1278,
December
,
. )
#{149}:, .
.
#{149}‘-..:
- .
“:
.
.;
Shunt in a Child
on the anterior
by using sonographic
abdominal
guidance
wall with
to provide
venous system was entered initially which an 8-French catheter and 12advanced into the portal vein. Initial mm
Hg/36
mm
a 6-mm-diameter and stented with
Hg.
The
track
1992 0361 -803X/92/1596-1277
C American
balloon
angioplasty balloon a 42-mm Wallstent
Hg. Portography showed brisk flow through the shunt without filling of esophageal varices (Fig. 1 B). The child has been followed up for the past 6 months both clinically
flow sonography.
The shunt has remained
widely patent, and no further bleeding has occurred. A single episode ofencephalopathy was treated easily by altering the lactulose dosage
and dietary restrictions.
Discussion Cystic fibrosis is an inherited
disorder
ofthe exocrine
glands
that results most prominently in abnormalities of the alimentary tract, pancreas, and lungs. Biliary cirrhosis may develop in the liver as a result of mucous plugging of the bile ducts
followed by portal fibrosis. Advanced changes in the liver, as portrayed in this case, occur in less than 5% of patients with cystic fibrosis, but when present they can lead to portal hypertension and bleeding varices. Because of coexistent lung disease, poor nutritional status, and a propensity to infections, these patients are poor candidates for surgical portosystemic shunts or liver transplantation.
after revision June 5, 1992.
of Radiology. University of Florida, Box 100374,
an
a target
self-expanding metallic prosthesis (Schneider, Minneapolis, MN; Fig. 1A), which lowered the portal venous pressure to 36 mm Hg/31 mm
and with color Doppler a
transplantation.
gauge Hawkins needle guide (Cook, Bloomington, IN) anteriorly through the liver parenchyma under fluoroscopic guidance. The main Received
, . , .‘
during fluoroscopy. The portal with the needle guide, over French guiding catheter were pressures measured were 50 dilated with a 5-mm- and then (Medi-tech, Watertown, MA)
Physical examination showed that she was small for her age, had hepatosplenomegaly, and had clubbing of the fingers. No jaundice or ascites was present. Laboratory values included a low hematocrit of 30% (0.30) and mildly elevated prothrombin time (1 3.2 sec) and partial thromboplastin time (42 sec.) Total protein and albumin levels were normal at 7.1 g/dl (71 g/I) and 3.8 g/dI (38 g/I), respectively. However, results of liver function tests were abnormal: the aspartate aminotransferase level was 1 21 U/I, alanine aminotransferase level was 63 U/I, and total bilirubin level was 1 .6 mg/dI (27 ,moI/l). Her condition was considered Child class A. A transjugular intrahepatic portosystemic shunt was requested to alleviate the portal hypertension. The right internal jugular vein was punctured under sonographic guidance, and after serial dilatation, a 1 2-French guiding catheter was placed into the right hepatic vein. A blunt-tipped
..
. . ...
vein had been marked
ECG electrode
A 13-year-old girl with cystic fibrosis was referred to our service after multiple episodes of upper gastrointestinal hemorrhage. Pulmonary manifestations were mild, consisting only of shortness of breath with increased activity. She had had six to seven episodes of bleeding
.
Jr.
Case Report
sclerotherapy
,
#{149}
Portosystemic
We report a case of a 1 3-year-old girl with cystic fibrosis that manifested as cirrhosis, portal hypertension, and variceal bleeding. A transjugular intrahepatic portosystemic shunt was performed successfully with no recurrent hemorrhages.
variceal
:‘
JHMHC, Roentgen
Gainesville, Ray Society
FL 32610.
Address
reprint
requests to S. A. Kerns.
1278
KERNS
AND
HAWKINS
AJR:159,
Fig. 1.-A,
Radiograph
December
1992
shows Wallstent
pros-
Downloaded from www.ajronline.org by 209.197.169.221 on 11/07/15 from IP address 209.197.169.221. Copyright ARRS. For personal use only; all rights reserved
thesis (arrows) connecting hepatic and portal venous systems. B, Portogram shows brisk flow through shunt from portal vein (arrow) to hepatic vein and right atrium (arrowheads).
A
B
Transjugular intrahepatic portosystemic shunts have recently become a practical alternative to surgical portosystemic shunting for decompression of portal hypertension and treatment of variceal bleeding refractory to medical management and sclerotherapy. Although Colapinto et al. [1 ] first described creating a portosystemic shunt in humans by a nonsurgical approach in 1 982, the technique of percutaneous transjugular shunting has only recently been widely accepted. The use of metallic stents to maintain patency has vastly improved the efficacy of the procedure [2]. Although the long-term outcome of transjugular intrahepatic portosystemic shunts has yet to be determined, the shortterm effects appear promising. Shunt patency appears high, and if the shunt thromboses, it can be reopened percutaneously via a transjugular approach. Even though most investigators have performed this procedure on more critically ill
patients in Child mortality of the portosystemic option for those conditions that
B or C condition, the observed morbidity and procedure are lower than those for surgical shunts [3]. This case demonstrates a new caring for children with cystic fibrosis or other lead to cirrhosis and portal hypertension.
REFERENCES 1 . Colapinto RF, Stronell AD, Birch SJ, et al. Creation of an intrahepatic portosystemic shunt with a Gruentzig balloon catheter. Can Med Assoc J 1982;126:267-268
2. Palmaz JC, Garcia F, Sibbitt AR, et at. Expandable shunt
stents
in dogs
with
chronic
portal
intrahepatic
hypertension.
portacaval AJR
1986:
147:1251 -1 254 3. Richter GM, Noeldge G, Palmaz JC, Roessle M. The transjugular intrahepatic portosystemic stent-shunt (TIPSS): results of a pilot study. Cardiovasc Intervent Radiol 1990:13:200-207
