Downloaded from www.ajronline.org by 161.0.63.233 on 11/03/15 from IP address 161.0.63.233. Copyright ARRS. For personal use only; all rights reserved
626
Case Report .
.
.
H
.
#{149}
AIDS-Related A. Rusin,1’2
Jerry
.
Cholangitis
Carlos
J. Sivit,1
Tamara
in Children:
A. Rakusan,2’3
Biliary tract disease is recognized as a complication in adults [1 -5]. Abnormalities of the biliany tract
and Roma
Case
and in whom
cholangitis
was
noted
diffuse
girl who had peninatally transmitted AIDS was admitted with recurrent varicella. Systemic manifestations of AIDS had been present for 2 years and included chronic Cryptosporidium enteritis and Candida albicans mucositis. On physical examination, the upper abdomen was tender, and the liver edge was palpable 2
margin.
Additionally,
cervical
and inguinal
lymph nodes were palpable. Blood chemistry profile showed increased levels of aspartate aminotransferase, 172 U/I (normal, 9-30 U/I), and alanine aminotransferase, 1 61 U/l(normal, 8-32 of all other laboratory tests, including serum bilirubin
U/I). Results level, were
normal. Abdominal sonography showed dilatation of the intra- and extrahepatic biliary tract and hyperechoic hepatic parenchyma (Figs. 1A and 1 B). The maximal diameter of the common bile duct was 8.5 mm.
Thickening of the gallbladder wall also was noted; between 4 and 7 mm. Abdominal CT performed
the
fatty
infiltration
of the liver was noted.
A second child who had peninatally transmitted AIDS was noted to have cholangitis at autopsy. Sonognaphic examination at 1 year of age showed normal caliber of the biliany tract. She died of cardionespiratony arrest following an episode of Pneuomocystis carinll pneumonia 4 months after sonographic evaluation. At autopsy, cholangiolan proliferation, bile plugging, and numerous peniductal neutrophils were noted. AIDS-related acalculous biliary tract disease is a newly recognized abnormality in children. No reports of cholangitis in this population have been reported. The condition may currently be underestimated, as clinical diagnosis is difficult [1 , 3, 4]. Clinical features in the cases reported were nonspecific. Hepatomegaly was noted; however, that is a frequent clinical finding in children who have AIDS [6]. Jaundice was not present in either of our cases. The liver function profile of these children was variable. Transaminase levels were elevated in one child, and results of liver function tests were normal in the other. A cholestatic pattern of liver function test abnormalities, characterized by disproportionate elevation of serum levels of alkaline phosphatase and total bilirubin levels relative to transaminase levels, has been reported in many adult patients who have AIDS-related cholangitis [i , 3-5]. This pattern was not observed in either child in this report, indicating that biliary obstruction was incomplete.
at autopsy.
Report
the right costal
S. Chandra2’4
Discussion
A 9-year-old
cm below
Findings
formation was noted (Fig. i C). The gallbladder and extrahepatic bile ducts were uniformly dilated owing to the cholangitis. Additionally,
of AIDS
in these patients include acalculous cholecystitis, papillary stenosis, and cholangitis [i , 3-5]. Biliary lesions have not been previously described in children who have AIDS. In this report, we describe the sonographic findings in two children who later died of AIDS
Sonographic
.
wall
1 week after sonography also showed biliary dilatation. Extrinsic masses were not identified at either sonography or CT. Abdominal sonography was repeated 3 and 8 weeks after the initial study. Follow-up scans continued to show biliary tract dilatation and gallbladder wall thickening. The child did not have jaundice. She died 8 months later of cardiorespiratory arrest. At autopsy, extensive involvement of the liver by suppurative cholangitis with bacterial overgrowth and abscess measured
Received March 9, 1992; accepted after revision April 13, 1992. I Department of Diagnostic Imaging and Radiology, Childrens National Medical Center and the George Washington University Schcol of Medicine and Health Sciences, 1 11 Michigan Ave., NW., Washington, DC 20010-2970. Address reprint requests to C. J. Sivit. 2 Department of Pediatrics, Children’s National Medical Center and the George Washington University Schcol of Medicine and Health Sciences, Washington, DC
2001 0-2970. 3 Department of Special Immunology, Washington, DC 200i0-2970. 4 Deportment of Pathology, Children’s
Children’s National
National Medical
Medical Center
Center
and the George
and the George
Washington
Washington University
DC 20010-2970. AJR 159:626-627,
September
1992 0361-803X/92/1
593-0626
0 American
Roentgen
Ray Society
University Schcol
Schcol
of Medicine
of Medicine
and Health
and
Health
Sciences,
Sciences,
Washington,
Downloaded from www.ajronline.org by 161.0.63.233 on 11/03/15 from IP address 161.0.63.233. Copyright ARRS. For personal use only; all rights reserved
AJR:159,
September
AIDS-RELATED
1992
Fig. 1.-9-year-old girl with suppurative cholangitis. A and B, Longitudinal sonograms through right upper hepatic parenchyma. C, Gross specimen of liver shows multiple abscesses
quadrant
CHOLANGITIS
show dilatation
with irregular
contours
IN CHILDREN
of common
bile duct(A)and
(arrowheads).
Dark
green
627
intrahepatic viscid
ducts(B).
bile was present
Also note hyperechoic in some.
Proposed causes of AIDS-related biliary tract disease in adults include the following: (1) biliary inflammation due to the immune
deficiency
itself;
(2) direct
infiltration
of the bile duct
mucosa by HIV; and (3) secondary infection by opportunistic organisms that frequently involve the gastrointestinal tract, such as Cryptosporidium, cytomegalovirus, on Candida albicans [1 -5]. Cryptosporidium was isolated from the stool in one of the cases presented. The underlying pathogenesis in the other child is unclear. Bile sampling was not obtained, and AIDS-specific pathogens were not isolated from the blood or stool. Histologic evidence of cytomegalovinus was not present at autopsy. A variety
of biliany
tract
abnormalities
seen
on imaging
studies have been reported in adult patients with AIDS. include dilatation, irregularity, stricture of the intra- and hepatic bile ducts, and dilatation and wall thickening gallbladder [1 , 5]. Biliary dilatation and gallbladder wall ening were noted in one of the two children in this report. The precise characterization
tract
abnormalities
usually
requires
These extraof the
thickwith cholangitis of these biliany
cholangiognaphy
[i,
which was not done in these two cases. The biliany tract has been reported to appear uniformly dilated in patients 3-5],
with papillary stenosis tures with intervening ductal
caliber
have
at sonography
been
noted
in patients
abnormalities in adults
who
who
have cholan-
also have
been ob-
have AIDS-related
biliary
tract disease [7, 8]. Sonognaphic abnormalities of the hepatic parenchyma were noted in both cases reported. Diffuse hyperechogenicity
of the hepatic
panenchyma
was noted
in one
child (Figs. iA and 1 B), while peniportal hyperechogenicity was observed in the other (Fig. 2). These changes may have been unrelated to cholangitis, however, as associated fatty infiltration of the liver in addition to cholangitis was noted at autopsy in both cases. Physicians
AIDS-related Noninvasive with
liver
who
care
for
children
should
be aware
that
cholangitis can occur in the pediatric population. imaging with sonography and CT, in conjunction function
Transverse
sonogram
of liver
Functional nuclear medicine studies of the biliary tree and serial examination with sonography or CT may also be helpful in
case management.
at cholangiography, while focal stnicsegments of normal and increased
gitis [1 , 3-5]. Hepatic parenchymal served
Fig. 2.-i-year-old girl with cholangitis. shows diffuse periportal hyperechogenicity.
studies,
may
suggest
the
abnormality.
REFERENCES 1 . Dolmatch BL, Laing FC, Federle MP, Jeffrey RB, Cello J. AIDS-related cholangitis: radiographic findings in nine patients. Radiology 1987; 163: 313-316 2. Viteri AL, Greene JF. Bile duct abnormalities in the acquired immune deficiency syndrome. Gastroenterology 1987:92:2014-2018 3. Cello JP. Acquired immunodeficiency syndrome cholangiopathy: spectrum of disease. Am J Med 1989;86:539-546 4. Teixidor HS, Godwin TA, Ramirez EA. Cryptosporidiosis ofthe biliary tract. Radiology 199i;i80:51-56 5. Schneiderman DJ, Cello JP, Laing FC. Papillary stenosis and scierosing cholangitis in the acquired immunodeficiency syndrome. Ann Intern Med
1987;106:546-549 6. Haney
PJ, YaIe-Loehr
AJ, Nussbaum
AP, Gellad FE. Imaging of infants 1033-1041 7. Defalque D, Menu Y, Girard PM, Couland JP. Sonographic diagnosis of cholangitis in AIDS patients. Gastrointest Radiol 1989;14: 143-i 47 8. McCarty M, Choudhri AH, Helbert M, Crofton ME. Radiological features of AIDS related cholangitis. Clln Radiol 1989;40:582-585
and children with AIDS. AJR 1989;152:
626
Case Report .
.
.
H
.
#{149}
AIDS-Related A. Rusin,1’2
Jerry
.
Cholangitis
Carlos
J. Sivit,1
Tamara
in Children:
A. Rakusan,2’3
Biliary tract disease is recognized as a complication in adults [1 -5]. Abnormalities of the biliany tract
and Roma
Case
and in whom
cholangitis
was
noted
diffuse
girl who had peninatally transmitted AIDS was admitted with recurrent varicella. Systemic manifestations of AIDS had been present for 2 years and included chronic Cryptosporidium enteritis and Candida albicans mucositis. On physical examination, the upper abdomen was tender, and the liver edge was palpable 2
margin.
Additionally,
cervical
and inguinal
lymph nodes were palpable. Blood chemistry profile showed increased levels of aspartate aminotransferase, 172 U/I (normal, 9-30 U/I), and alanine aminotransferase, 1 61 U/l(normal, 8-32 of all other laboratory tests, including serum bilirubin
U/I). Results level, were
normal. Abdominal sonography showed dilatation of the intra- and extrahepatic biliary tract and hyperechoic hepatic parenchyma (Figs. 1A and 1 B). The maximal diameter of the common bile duct was 8.5 mm.
Thickening of the gallbladder wall also was noted; between 4 and 7 mm. Abdominal CT performed
the
fatty
infiltration
of the liver was noted.
A second child who had peninatally transmitted AIDS was noted to have cholangitis at autopsy. Sonognaphic examination at 1 year of age showed normal caliber of the biliany tract. She died of cardionespiratony arrest following an episode of Pneuomocystis carinll pneumonia 4 months after sonographic evaluation. At autopsy, cholangiolan proliferation, bile plugging, and numerous peniductal neutrophils were noted. AIDS-related acalculous biliary tract disease is a newly recognized abnormality in children. No reports of cholangitis in this population have been reported. The condition may currently be underestimated, as clinical diagnosis is difficult [1 , 3, 4]. Clinical features in the cases reported were nonspecific. Hepatomegaly was noted; however, that is a frequent clinical finding in children who have AIDS [6]. Jaundice was not present in either of our cases. The liver function profile of these children was variable. Transaminase levels were elevated in one child, and results of liver function tests were normal in the other. A cholestatic pattern of liver function test abnormalities, characterized by disproportionate elevation of serum levels of alkaline phosphatase and total bilirubin levels relative to transaminase levels, has been reported in many adult patients who have AIDS-related cholangitis [i , 3-5]. This pattern was not observed in either child in this report, indicating that biliary obstruction was incomplete.
at autopsy.
Report
the right costal
S. Chandra2’4
Discussion
A 9-year-old
cm below
Findings
formation was noted (Fig. i C). The gallbladder and extrahepatic bile ducts were uniformly dilated owing to the cholangitis. Additionally,
of AIDS
in these patients include acalculous cholecystitis, papillary stenosis, and cholangitis [i , 3-5]. Biliary lesions have not been previously described in children who have AIDS. In this report, we describe the sonographic findings in two children who later died of AIDS
Sonographic
.
wall
1 week after sonography also showed biliary dilatation. Extrinsic masses were not identified at either sonography or CT. Abdominal sonography was repeated 3 and 8 weeks after the initial study. Follow-up scans continued to show biliary tract dilatation and gallbladder wall thickening. The child did not have jaundice. She died 8 months later of cardiorespiratory arrest. At autopsy, extensive involvement of the liver by suppurative cholangitis with bacterial overgrowth and abscess measured
Received March 9, 1992; accepted after revision April 13, 1992. I Department of Diagnostic Imaging and Radiology, Childrens National Medical Center and the George Washington University Schcol of Medicine and Health Sciences, 1 11 Michigan Ave., NW., Washington, DC 20010-2970. Address reprint requests to C. J. Sivit. 2 Department of Pediatrics, Children’s National Medical Center and the George Washington University Schcol of Medicine and Health Sciences, Washington, DC
2001 0-2970. 3 Department of Special Immunology, Washington, DC 200i0-2970. 4 Deportment of Pathology, Children’s
Children’s National
National Medical
Medical Center
Center
and the George
and the George
Washington
Washington University
DC 20010-2970. AJR 159:626-627,
September
1992 0361-803X/92/1
593-0626
0 American
Roentgen
Ray Society
University Schcol
Schcol
of Medicine
of Medicine
and Health
and
Health
Sciences,
Sciences,
Washington,
Downloaded from www.ajronline.org by 161.0.63.233 on 11/03/15 from IP address 161.0.63.233. Copyright ARRS. For personal use only; all rights reserved
AJR:159,
September
AIDS-RELATED
1992
Fig. 1.-9-year-old girl with suppurative cholangitis. A and B, Longitudinal sonograms through right upper hepatic parenchyma. C, Gross specimen of liver shows multiple abscesses
quadrant
CHOLANGITIS
show dilatation
with irregular
contours
IN CHILDREN
of common
bile duct(A)and
(arrowheads).
Dark
green
627
intrahepatic viscid
ducts(B).
bile was present
Also note hyperechoic in some.
Proposed causes of AIDS-related biliary tract disease in adults include the following: (1) biliary inflammation due to the immune
deficiency
itself;
(2) direct
infiltration
of the bile duct
mucosa by HIV; and (3) secondary infection by opportunistic organisms that frequently involve the gastrointestinal tract, such as Cryptosporidium, cytomegalovirus, on Candida albicans [1 -5]. Cryptosporidium was isolated from the stool in one of the cases presented. The underlying pathogenesis in the other child is unclear. Bile sampling was not obtained, and AIDS-specific pathogens were not isolated from the blood or stool. Histologic evidence of cytomegalovinus was not present at autopsy. A variety
of biliany
tract
abnormalities
seen
on imaging
studies have been reported in adult patients with AIDS. include dilatation, irregularity, stricture of the intra- and hepatic bile ducts, and dilatation and wall thickening gallbladder [1 , 5]. Biliary dilatation and gallbladder wall ening were noted in one of the two children in this report. The precise characterization
tract
abnormalities
usually
requires
These extraof the
thickwith cholangitis of these biliany
cholangiognaphy
[i,
which was not done in these two cases. The biliany tract has been reported to appear uniformly dilated in patients 3-5],
with papillary stenosis tures with intervening ductal
caliber
have
at sonography
been
noted
in patients
abnormalities in adults
who
who
have cholan-
also have
been ob-
have AIDS-related
biliary
tract disease [7, 8]. Sonognaphic abnormalities of the hepatic parenchyma were noted in both cases reported. Diffuse hyperechogenicity
of the hepatic
panenchyma
was noted
in one
child (Figs. iA and 1 B), while peniportal hyperechogenicity was observed in the other (Fig. 2). These changes may have been unrelated to cholangitis, however, as associated fatty infiltration of the liver in addition to cholangitis was noted at autopsy in both cases. Physicians
AIDS-related Noninvasive with
liver
who
care
for
children
should
be aware
that
cholangitis can occur in the pediatric population. imaging with sonography and CT, in conjunction function
Transverse
sonogram
of liver
Functional nuclear medicine studies of the biliary tree and serial examination with sonography or CT may also be helpful in
case management.
at cholangiography, while focal stnicsegments of normal and increased
gitis [1 , 3-5]. Hepatic parenchymal served
Fig. 2.-i-year-old girl with cholangitis. shows diffuse periportal hyperechogenicity.
studies,
may
suggest
the
abnormality.
REFERENCES 1 . Dolmatch BL, Laing FC, Federle MP, Jeffrey RB, Cello J. AIDS-related cholangitis: radiographic findings in nine patients. Radiology 1987; 163: 313-316 2. Viteri AL, Greene JF. Bile duct abnormalities in the acquired immune deficiency syndrome. Gastroenterology 1987:92:2014-2018 3. Cello JP. Acquired immunodeficiency syndrome cholangiopathy: spectrum of disease. Am J Med 1989;86:539-546 4. Teixidor HS, Godwin TA, Ramirez EA. Cryptosporidiosis ofthe biliary tract. Radiology 199i;i80:51-56 5. Schneiderman DJ, Cello JP, Laing FC. Papillary stenosis and scierosing cholangitis in the acquired immunodeficiency syndrome. Ann Intern Med
1987;106:546-549 6. Haney
PJ, YaIe-Loehr
AJ, Nussbaum
AP, Gellad FE. Imaging of infants 1033-1041 7. Defalque D, Menu Y, Girard PM, Couland JP. Sonographic diagnosis of cholangitis in AIDS patients. Gastrointest Radiol 1989;14: 143-i 47 8. McCarty M, Choudhri AH, Helbert M, Crofton ME. Radiological features of AIDS related cholangitis. Clln Radiol 1989;40:582-585
and children with AIDS. AJR 1989;152:
