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EDUCATION EXHIBIT
465
RadioGraphics
Eponyms in Radiology
of the Digestive Tract:
Historical Perspectives
and Imaging Appear-
ances
Part 2. Liver, Biliary System, Pancreas,
Peritoneum, and Systemic Disease
1
TEACHING
POINTS
See last page
Jeffrey P. Kanne, MD
●
Charles A. Rohrmann, Jr, MD
●
Joel E.
Lichtenstein, MD
Eponyms are a means of honoring individuals who have made lasting
contributions to medicine. Eponyms are frequently encountered in the
field of radiology, especially radiology of the digestive tract. However,
the use of eponyms may fail to convey a precise meaning or definition
and could result in miscommunication. Furthermore, in some in-
stances, more than one individual may have contributed to the discov-
ery or description of a particular structure or disease, whereas in oth-
ers, an eponym may have been incorrectly applied and then propagated
for years thereafter in the medical literature. Still, eponyms are a means
of honoring those who have made important discoveries and observa-
tions, and familiarity with these terms is important for proper reporting
and accurate communication. Moreover, the acquisition of some his-
torical knowledge about the individuals whose names are associated
with various structures or diseases helps restore some humanity to the
science of medicine.
©
RSNA, 2006
Abbreviation:
AIDS
acquired immunodeficiency syndrome
RadioGraphics 2006;
26:465– 480
●
Published online
10.1148/rg.262055130
●
Content Code:
1
From
the Department of Radiology, University of Washington, Box 357115, 1959 NE Pacific, Seattle, WA 98195-7115. Presented as an education
exhibit at the 2004 RSNA Annual Meeting. Received April 8, 2005; revision requested May 4 and received June 15; accepted June 17. All authors have
no financial relationships to disclose.
Address correspondence to
C.A.R. (e-mail:
rohrmann@u.washington.edu).
See Kanne et al in the January 2006 issue (pp 129 –142) for Part 1 of this two-part series.
©
RSNA, 2006
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Figures 1, 2.
(1)
Caroli disease.
T-tube cholangio-
gram shows focal
areas of saccular ec-
tasia of the intrahe-
patic bile ducts. The
extrahepatic bile
duct is normal.
(2)
Jacques Caroli
(1902–1979). (From
the National Library
of Medicine, Wash-
ington, DC.)
Introduction
In the context of medicine, an eponym is defined
as “a name of a drug, structure, or disease based
on or derived from the name of a person” (1).
Teaching
Eponyms are frequently encountered in the field
Point
of radiology, particularly radiology of the diges-
tive tract, and knowledge of these terms is impor-
tant for proper reporting and communication.
Eponyms are a means of honoring individuals
who have made lasting contributions to medicine,
but use of these terms may fail to convey a precise
meaning or definition and could lead to miscom-
munication. Moreover, more than one person
may have contributed to the discovery or descrip-
tion of a structure or disease. In other cases, an
eponym may have been incorrectly applied and
then propagated for years afterward in the medi-
cal literature.
In this article, the second of a two-part series,
we discuss and illustrate the imaging manifesta-
tions of eponyms encountered in radiology of the
liver and biliary tree (Caroli disease, Klatskin tu-
mor, spiral valves of Heister, Rokitansky-Aschoff
sinuses, sphincter of Oddi, ampulla and papilla of
Vater), pancreas (duct of Wirsung, duct of San-
¨
torini), and peritoneum (Rigler sign and triad). In
addition, we describe the radiologic appearances
of eponyms relating to systemic diseases (Crohn
disease, Whipple disease, Behcet disease, Peutz-
¸
Jeghers syndrome, Kaposi sarcoma, Chagas dis-
ease). We also explore the historical background
of the individuals for whom these radiologic ep-
onyms were named.
Liver and Biliary Tree
Caroli Disease
Caroli disease (Fig 1) is a congenital anomaly of
the biliary tree that is characterized by saccular
dilatation of the intrahepatic bile ducts and may
be an autosomal recessive trait in some individu-
als. The extrahepatic bile ducts are rarely in-
volved, and there is no biliary obstruction. The
abnormality may affect only one hepatic segment
or lobe, most commonly the left lobe. Caroli dis-
ease is associated with autosomal recessive poly-
cystic kidney disease and medullary sponge kid-
ney. Complications include cholangitis, hepatic
abscess, and biliary stones. Recurrent inflamma-
tion leads to the development of cholangiocarci-
noma in about 7% of cases. Imaging shows scat-
tered hepatic cysts communicating with the intra-
hepatic bile ducts (2,3).
Jacques Caroli (1902–1979) (Fig 2) was born
near Versailles, France. He began his medical
training in Angers, completing it in Paris under
Henri Hartmann (1860 –1952), the famous bili-
ary tract surgeon. Following World War II, Caroli
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Figures 3, 4.
(3)
Klatskin tumor
(cholangiocarci-
noma). Endoscopic
retrograde cholan-
giopancreatogram
shows mild dilatation
of the intrahepatic
bile ducts and an
irregular stricture
(arrow) at the bifur-
cation of the main
intrahepatic bile
ducts.
(4)
Gerald
Klatskin (1910 –
1986). (From the
National Library of
Medicine.)
joined the faculty at l’Hopital Sainte-Antoine in
ˆ
Paris, where he was chief of service for 30 years.
In conjunction with his colleagues in surgery and
radiology, he devised a system to study the biliary
tree with a combination of contrast radiography
and pressure measurement. In 1958, Caroli pub-
lished a manuscript describing congenital poly-
cystic dilatation of the intrahepatic bile ducts,
the disease that now bears his name (4). He was
awarded a medal and accorded the rank of com-
mander in the French Legion of Honor in 1976.
Caroli died 3 years later in Paris (5).
Klatskin Tumor
Cholangiocarcinoma occurring at the bifurcation
of the common hepatic duct is referred to as a
Klatskin tumor (Fig 3). This neoplasm accounts
for about 25% of all cholangiocarcinomas.
Typi-
Teaching
cally, Klatskin tumors are small, poorly differenti-
ated, exhibit aggressive biologic behavior, and
Point
obstruct the intrahepatic bile ducts (6).
Gerald Klatskin (1910 –1986) (Fig 4) was born
in New York City. He was first in his medical
school class at Cornell University in Ithaca, New
York, receiving his MD degree in 1933. Klatskin
trained as a pathologist at Yale–New Haven
(Connecticut) Hospital and Strong Memorial
Hospital in Rochester, New York, but also prac-
ticed clinical hepatology. While serving as a medi-
cal officer in Calcutta, India, during World War
II, Klatskin developed an interest in hepatitis and
hepatic amebic abscesses. Following the war, he
returned to Yale and established a laboratory.
Klatskin’s contributions include linking the
Australia antigen to hepatitis B and recognizing
that hyperlipidemia resulting from alcohol intake
could lead to pancreatitis. In 1965, he described
the unique features of the tumor that now bears
his name (7). Interestingly, three cases of this
unique tumor had been described 8 years earlier
by William A. Altemeier at the University of Cin-
cinnati (8,9), a fact that Klatskin acknowledged in
his original manuscript. Although Klatskin’s
dream of creating a histologic atlas of hepatic dis-
eases never came to fruition during his lifetime,
his first fellow and long-time colleague, Harold
Conn, produced such an atlas in 1993 (10).
Spiral Valves of Heister
The spiral valves of Heister (Fig 5) are the normal
mucosal folds in the cystic duct. The duct and
spiral folds contain muscle fibers that respond to
pharmacologic, neural, and hormonal stimuli.
However, no convincing evidence of a discrete
muscular sphincter within the duct has yet been
adduced. In humans, the principal function of the
internal spiral folds may be to maintain patency of
the cystic duct, which is quite narrow and tortu-
ous (11).
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Figures 5, 6. (5)
Spiral valves of Heister. Endoscopic retrograde cholangiopancreatogram shows nor-
mal cystic duct mucosal folds (arrow).
*
gallbladder.
(6)
Lorenz Heister (1683–1758). (From the Uni-
versitatsbibliothek Erlangen-Nurnberg, Germany.)
¨
¨
Lorenz Heister (1683–1758) (Fig 6) was born
in Frankfurt am Main, Germany, and received his
education at the University of Giessen and in Lei-
den, the Netherlands. He ultimately received his
medical degree in 1708 from the University of
Helmstedt in Germany. After first serving as chief
surgeon to the army of the Netherlands, Heister
was appointed Professor of Anatomy and Surgery
at the University of Helmstedt in 1719. He was
later given the additional position of Professor of
Botany and established a famous botanic garden.
Under his leadership, the University of Helmstedt
became a premier school of surgery.
Heister’s treatise on surgery,
Chirugia,
written
in German, was the most popular surgical text of
the 18th century. Heister performed the first au-
topsy for appendicitis and introduced a procedure
for tracheotomy that was very similar to the tech-
nique used today. The “valves” in the cystic duct
that bear his name were first described in 1720 in
his textbook
Compendium Anatomica
(12,13).
Rokitansky-Aschoff Sinuses
Rokitansky-Aschoff sinuses are outpouchings of
gallbladder mucosa into the muscularis that occur
with mucosal and smooth muscle hyperplasia.
These outpouchings are seen in a benign condi-
tion called adenomyomatous hyperplasia (adeno-
myomatosis), which may be focal, segmental, or
diffuse (Fig 7).
Ultrasonographic (US) findings in adenomyo-
matous hyperplasia include focal or diffuse gall-
bladder wall thickening and anechoic or echo-
genic foci in the gallbladder wall. Ring-down re-
verberation artifact may be identified arising from
the echogenic foci. Rokitansky-Aschoff sinuses
are best seen at magnetic resonance imaging,
helping to distinguish adenomyomatous hyper-
plasia from gallbladder carcinoma (14).
Karl Rokitansky (1804 –1878) (Fig 8) was
born in Koniggratz, Bohemia (now Hradec
¨
Kralove, Czech Republic) and began his medical
´ ´
training in 1822, studying first at Charles Univer-
sity in Prague and then in Vienna. While a stu-
dent, Rokitansky worked as a volunteer in the
dissection laboratory at Vienna General Hospital.
He was appointed Professor of Pathology at the
University of Vienna at 30 years of age and be-
came the department’s first chairman in 1834. He
served four terms as Dean of the Medical School
of the University of Vienna and became the first
elected rector of the institution. Rokitansky was
elected to and eventually served as president of
the Academy of Sciences in Vienna. In 1874, a
noble title was bestowed on him: Freiherr von
Rokitansky.
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Figures 7–9. (7)
Gallbladder adenomyomatous hyperplasia.
(a)
Abdominal radiograph shows round calcifications
(arrow) representing calculi in Rokitansky-Aschoff sinuses in the region of the gallbladder.
(b)
US image demon-
strates gallbladder wall thickening with intramural echogenic foci (long arrow) and associated “ring-down” artifact
(short arrows).
(8)
Karl von Rokitansky (1804 –1878). (From the National Library of Medicine.)
(9)
Carl Albert
Ludwig Aschoff (1866 –1942). (From the National Library of Medicine.)
Rokitansky convinced Emperor Joseph II to
decree that autopsy be performed on the corpses
of all Austrian citizens when they died, allowing
Rokitansky to officiate at approximately 60,000
postmortem examinations, or nearly 2000 a year.
He kept detailed records of each examination and
correlated pathologic findings with clinical diag-
noses, working closely with the famed internist
Joseph Skoda. Rokitansky published a three-vol-
ume work entitled
Handbook of Pathologic Anat-
omy.
In 1842, he observed the cryptic sinuses in
the gallbladder wall associated with chronic cho-
lecystitis (15). Rokitansky is also credited with
making the distinction between lobar pneumonia
and bronchopneumonia, describing polyarteritis
nodosa, coining the term “spondylolisthesis,” and
making significant contributions to the under-
standing of congenital heart disease. However, his
greatest accomplishment was likely establishing
pathology as a separate discipline in medicine
(16 –18).
Carl Albert Ludwig Aschoff (1866 –1942) (Fig
9) was born in Berlin, Germany, and received his
medical degree from the University of Bonn. He

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