Diagnostic ultrasound ( PDFDrive )

182 PART II Abdominal and Pelvic Sonography
efectively rules out the disease. he indings include the multimodality assessment of patients with this disease. he high
following:
spatial resolution of ultrasound allows for detection of minute
• Bile duct wall thickening of the intrahepatic and extrahepatic
biliary tree.
include irregular, circumferential bile duct wall thickening of
early changes that can be missed with MRCP. Sonographic indings
• Focal strictures and dilations identical to those of primary varying degree, encroaching on and narrowing the lumen
sclerosing cholangitis.
(Fig. 6.22, Videos 6.5 and 6.6). Focal strictures and dilations of
• Dilation of the CBD caused by an inlamed and stenosed the bile ducts ensue. he extrahepatic disease is more easily
papilla of Vater (papillary stenosis). he inlamed papilla itself visible. A high degree of suspicion and careful examination of
may be seen as an echogenic nodule protruding into the distal the portal triads in all hepatic segments is required to detect
duct. 40
intrahepatic ductal involvement. Irregularity of the thickened
• Difuse gallbladder wall thickening. 41
bile duct mucosa is a key feature that should be sought. An
earlier study suggested false normal appearance of the intrahepatic
bile ducts in 25% of patients. 39 he gallbladder and cystic duct
Immune-Related Diseases of the
are involved in 15% to 20% of patients. 40 Choledocholithiasis,
Biliary Tree
once thought to exclude the disease, is now recognized as a
Primary Biliary Cirrhosis and
complication and is more frequently seen in symptomatic
Autoimmune Cholangitis
patients. 41 hey are most oten nonshadowing (Fig. 6.22D). he
Primary biliary cirrhosis and autoimmune cholangitis (also called stones are easily overlooked and should be speciically sought
autoimmune cholangiopathy) afect biliary ductules that are especially with patients with deteriorating cholestatic liver
too small to resolve by imaging. Only gross changes in the liver enzymes. Patients with primary sclerosing cholangitis also have
architecture resulting from biliary cirrhosis are detected. Biliary hepatic indings of biliary cirrhosis, namely hepatomegaly, and
cirrhosis, resulting from any cause of chronic difuse biliary enlarged periportal lymph nodes. In advanced cases, indings
obstruction, appears as difusely enlarged liver unless the liver of cirrhosis are present, oten with difuse atrophy of the liver
disease is end-stage.
but occasionally manifest as peripheral atrophy with central
hypertrophy of the liver. 44
Primary Sclerosing Cholangitis
Cholangiocarcinoma develops in 7% to 30% of patients with
Sclerosing cholangitis is a chronic inlammatory disease process primary sclerosing cholangitis and is particularly a diicult
afecting the biliary tree. If the cause of the disease is unknown, diagnosis to make in this setting. 43 Rapid progression of the
the term primary sclerosing cholangitis is used.
disease or development of a visible mass is a clue to this complication
(Fig. 6.23, Videos 6.7 and 6.8). Hepatic transplantation is
Primary sclerosing cholangitis is a chronic disease afecting
the entire biliary tree. he process involves a ibrosing inlammation
of the small and large bile ducts, leading to biliary disease may recur in the transplanted organ in 1% to 20% of
required in the latter stages of the disease. Unfortunately, the
strictures and cholestasis and eventually biliary cirrhosis, portal patients. 45
hypertension, and hepatic failure. 42,43 It occurs more frequently
in men, with median age of 39 years at diagnosis. 38 About 80% IgG4-Related Cholangitis
of the patients have concomitant inlammatory bowel disease, Immunoglobulin G subtype 4 (IgG4)–related cholangitis is the
typically ulcerative colitis, but this association occurs less oten biliary manifestation of IgG4-related disease or multifocal
in a non-Western population. It may also occur with other systemic ibrosclerosis. he exact cause of the disease is unknown
autoimmune disorders. 36
but is associated with elevation of serum immunoglobulin G
Most patients diagnosed with primary sclerosing cholangitis and speciically IgG subtype 4 levels in a majority of patients.
are asymptomatic. Ultrasound is a key component of the In the abdomen, the most commonly afected organ is the
pancreas, resulting in autoimmune pancreatitis, followed by
Causes of Secondary Sclerosing Cholangitis the biliary tree and gallbladder, the kidneys (interstitial nephritis),
and the retroperitoneum (retroperitoneal ibrosis). 46 A
IgG4-related sclerosing cholangitis
history of salivary or lacrimal gland disease is also common.
AIDS cholangiopathy
he disease predominates in older patients and is more common
Bile duct neoplasm a
in males. Unlike primary sclerosing cholangitis, the disease tends
Biliary tract surgery, trauma
to be steroid responsive. IgG4-related cholangitis afects both
Choledocholithiasis
large and small ducts. here is a lymphoplasmacytic iniltrate
Congenital abnormalities of biliary tract
about the ducts with associated ibrosis causing strictures; if
Ischemic stricturing of bile ducts
untreated it can lead to cirrhosis. Given the similarity in imaging
Toxic strictures related to intraarterial infusion of loxuridine appearance to primary sclerosing cholangitis, until recently it
Posttreatment for hydatid cyst
has been mistaken for this disease.
Several features help the diferentiation or at least direct the
a Primary sclerosing cholangitis not previously established.
referring clinicians to further investigations. In IgG4-related
Modiied from Narayanan Menon KV, Wiesner RH. Etiology and natural
history of primary sclerosing cholangitis. J Hepatobiliary Pancreat Surg. disease, the patient is usually older and the disease may spontaneously
resolve or improve. Bile duct wall thickening can be 1999;6(4):343-351. 43 much