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DOI: 10.1148/rg.262055130
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RadioGraphics 2006;26:465-480
© RSNA, 2006

EDUCATION EXHIBIT

Eponyms in Radiology of the Digestive Tract: Historical Perspectives and Imaging Appearances

Part 2. Liver, Biliary System, Pancreas, Peritoneum, and Systemic Disease1

Jeffrey P. Kanne, MD, Charles A. Rohrmann, Jr, MD and Joel E. Lichtenstein, MD

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{at}u.washington.edu).


   Abstract
Top
 Abstract
Introduction
 Liver and Biliary Tree
 Pancreas
 Systemic Disease
 Conclusions
 References
 Suggested Readings
 
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 instances, more than one individual may have contributed to the discovery or description of a particular structure or disease, whereas in others, 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 observations, and familiarity with these terms is important for proper reporting and accurate communication. Moreover, the acquisition of some historical knowledge about the individuals whose names are associated with various structures or diseases helps restore some humanity to the science of medicine.

© RSNA, 2006


   Introduction
Top
 Abstract
 Introduction
Liver and Biliary Tree
 Pancreas
 Systemic Disease
 Conclusions
 References
 Suggested Readings
 
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). Eponyms are frequently encountered in the field of radiology, particularly radiology of the digestive tract, and knowledge of these terms is important 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 miscommunication. Moreover, more than one person may have contributed to the discovery or description of a structure or disease. In other cases, an eponym may have been incorrectly applied and then propagated for years afterward in the medical literature.

In this article, the second of a two-part series, we discuss and illustrate the imaging manifestations of eponyms encountered in radiology of the liver and biliary tree (Caroli disease, Klatskin tumor, spiral valves of Heister, Rokitansky-Aschoff sinuses, sphincter of Oddi, ampulla and papilla of Vater), pancreas (duct of Wirsüng, duct of Santorini), and peritoneum (Rigler sign and triad). In addition, we describe the radiologic appearances of eponyms relating to systemic diseases (Crohn disease, Whipple disease, Behçet disease, Peutz-Jeghers syndrome, Kaposi sarcoma, Chagas disease). We also explore the historical background of the individuals for whom these radiologic eponyms were named.


   Liver and Biliary Tree
Top
 Abstract
 Introduction
 Liver and Biliary Tree
Pancreas
 Systemic Disease
 Conclusions
 References
 Suggested Readings
 
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 individuals. The extrahepatic bile ducts are rarely involved, and there is no biliary obstruction. The abnormality may affect only one hepatic segment or lobe, most commonly the left lobe. Caroli disease is associated with autosomal recessive polycystic kidney disease and medullary sponge kidney. Complications include cholangitis, hepatic abscess, and biliary stones. Recurrent inflammation leads to the development of cholangiocarcinoma in about 7% of cases. Imaging shows scattered hepatic cysts communicating with the intrahepatic bile ducts (2,3).


Figure 1
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  		Figure 1.  Caroli disease. T-tube cholangiogram shows focal areas of saccular ectasia of the intrahepatic bile ducts. The extrahepatic bile duct is normal.

 
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 biliary tract surgeon. Following World War II, Caroli joined the faculty at l’Hôpital Sainte-Antoine in 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 published a manuscript describing congenital polycystic dilatation of the intrahepatic bile ducts, the disease that now bears his name (4). He was awarded a medal and accorded the rank of commander in the French Legion of Honor in 1976. Caroli died 3 years later in Paris (5).


Figure 2
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  		Figure 2.  Jacques Caroli (1902–1979). (From the National Library of Medicine, Washington, DC.)

 
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. Typically, Klatskin tumors are small, poorly differentiated, exhibit aggressive biologic behavior, and obstruct the intrahepatic bile ducts (6).


Figure 3
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  		Figure 3.  Klatskin tumor (cholangiocarcinoma). Endoscopic retrograde cholangiopancreatogram shows mild dilatation of the intrahepatic bile ducts and an irregular stricture (arrow) at the bifurcation of the main intrahepatic bile ducts.

 
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 practiced clinical hepatology. While serving as a medical 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.


Figure 4
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  		Figure 4.  Gerald Klatskin (1910–1986). (From the National Library of Medicine.)

 
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 Cincinnati (8,9), a fact that Klatskin acknowledged in his original manuscript. Although Klatskin’s dream of creating a histologic atlas of hepatic diseases 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 tortuous (11).


Figure 5
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  		Figure 5.  Spiral valves of Heister. Endoscopic retrograde cholangiopancreatogram shows normal cystic duct mucosal folds (arrow). * = gallbladder.

 
Lorenz Heister (1683–1758) (Fig 6) was born in Frankfurt am Main, Germany, and received his education at the University of Giessen and in Leiden, 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.


Figure 6
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  		Figure 6.  Lorenz Heister (1683–1758). (From the Universitätsbibliothek Erlangen-Nürnberg, Germany.)

 
Heister’s treatise on surgery, Chirugia, written in German, was the most popular surgical text of the 18th century. Heister performed the first autopsy for appendicitis and introduced a procedure for tracheotomy that was very similar to the technique 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 condition called adenomyomatous hyperplasia (adenomyomatosis), which may be focal, segmental, or diffuse (Fig 7).


Figure 7
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  		Figure 7a.   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 demonstrates gallbladder wall thickening with intramural echogenic foci (long arrow) and associated "ring-down" artifact (short arrows).

 

Figure 7
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  		Figure 7b.   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 demonstrates gallbladder wall thickening with intramural echogenic foci (long arrow) and associated "ring-down" artifact (short arrows).

 
Ultrasonographic (US) findings in adenomyomatous hyperplasia include focal or diffuse gall-bladder wall thickening and anechoic or echogenic foci in the gallbladder wall. Ring-down reverberation artifact may be identified arising from the echogenic foci. Rokitansky-Aschoff sinuses are best seen at magnetic resonance imaging, helping to distinguish adenomyomatous hyperplasia from gallbladder carcinoma (14).

Karl Rokitansky (1804–1878) (Fig 8) was born in Königgratz, Bohemia (now Hradec Králové, Czech Republic) and began his medical training in 1822, studying first at Charles University in Prague and then in Vienna. While a student, 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 became 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.


Figure 8
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  		Figure 8.  Karl von Rokitansky (1804–1878). (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 diagnoses, working closely with the famed internist Joseph Skoda. Rokitansky published a three-volume work entitled Handbook of Pathologic Anatomy. In 1842, he observed the cryptic sinuses in the gallbladder wall associated with chronic cholecystitis (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 understanding 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 is credited with describing the atrioventricular node and rheumatoid nodules in the myocardium (now known as Aschoff bodies). In 1905, Aschoff redescribed the sinuses in the gallbladder wall that had been identified initially by Rokitansky (18,19).


Figure 9
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  		Figure 9.  Carl Albert Ludwig Aschoff (1866–1942). (From the National Library of Medicine.)

 
Sphincter of Oddi
The sphincter of Oddi is the common muscular sphincter of the pancreatobiliary tree.

Ruggero Oddi (1864–1913) (Fig 10) was born in Perugia, Italy, and began his medical studies there. In his 4th year of medical studies, in Bologna, Oddi rediscovered the sphincter of the outlet of the common bile duct, previously described by Francis Glisson (1597–1677), a finding that became the basis for his doctoral thesis (20). Oddi was the first to measure the resistance of the sphincter and to show that the bile ducts dilated following cholecystectomy, a finding that has since been shown to be nonphysiologic. At 29 years of age, Oddi was named director of the Physiology Institute in Genoa, where he studied the physiology of the peripheral and central nervous systems.


Figure 10
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  		Figure 10.  Ruggero Oddi (1864–1913). (From the National Library of Medicine.)

 
Oddi’s career took a turn for the worse when he became associated with a nonphysician aristocrat, Capranica, who established a biochemistry laboratory in his palace. Capranica began practicing fanatical spiritualism and became addicted to morphine following the death of his mistress. Litigation surrounding Capranica’s laboratory forced Oddi to resign his position at the University of Genoa, so he went to the Congo for 3 years as a physician, only to return to Belgium because of ill health. He suffered a similar fate as his friend, Capranica, becoming involved in a panacea called Vitalina and being brought to trial in Italy several times for "abusive commerce of medicinal products." He suffered financially as well as physically, experiencing complications from an appendectomy as well as intestinal obstruction. Like his friend, Oddi became addicted to morphine and died a broken man (12,21).

Ampulla and Papilla of Vater
The ampulla of Vater refers to the common channel of the common bile and pancreatic ducts. However, exactly what it was that Vater described is the subject of debate. The papilla of Vater is the structure that projects into the duodenal lumen and drains the pancreaticobiliary tree.

Abraham Vater (1684–1751) (Fig 11) was born in Wittenberg, Germany, and received his doctorate in philosophy from the University of Wittenberg. He went on to study medicine in Leipzig, then in Meresbourg, ultimately earning his medical degree from the University of Wittenberg. Initially, he was Professor of Medicine, and then was appointed Professor of Anatomy and Botany, at that institution. In 1746, Vater was named Chairman of Therapeutics, which was considered to be the most important post at the university because it included the position of dean. He established a museum, gave anatomic demonstrations for women, and used the corpses of those who had committed suicide for anatomic investigations.


Figure 11
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  		Figure 11.  Abraham Vater (1684–1751). (From the Portraitsammlungen der Herzogs August Bibliothek, Wolfenbüttel, Germany.)

 
The initial description of what is now referred to as the ampulla of Vater remains controversial. In 1722, Vater gave a lecture on a bile diverticulum at the opening of the choledochal duct, a finding that was published soon thereafter (22). It has been suggested that what Vater actually described was what is now referred to as a perivaterian diverticulum, later described by Le Tulle in 1899 as an elevation of the mucosa in the duodenum. Suárez argues that the publication and widespread dissemination of Mémoire sur le Pancréas, by Claude Bernard (23), who erroneously described the ampulla at the terminal common bile duct as the "ampulla of Vater," led to the ready acceptance of this terminology for a structure not described by Vater. It is likely that Santorini was truly the first to describe the ampulla, and that Vater described the diverticulum that occurs immediately adjacent to it (12,24,25).


   Pancreas
Top
 Abstract
 Introduction
 Liver and Biliary Tree
 Pancreas
Systemic Disease
 Conclusions
 References
 Suggested Readings
 
Duct of Wirsüng
The term duct of Wirsüng refers to the main pancreatic duct that drains to the major papilla (Fig 12).


Figure 12
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  		Figure 12.  Pancreatic ducts of Wirsüng and Santorini. Endoscopic retrograde cholangiopancreatogram demonstrates normal filling of the ducts of Wirsüng (arrowhead) and Santorini (arrow).

 
Johann Georg Wirsüng (1589–1643) was born in the Imperial Free City of Augsburg, Germany, on July 3, 1589, but declared himself to be from the Catholic city of Munich when he matriculated to the University of Padua, Italy, in 1629. On March 2, 1642, Wirsüng discovered the main pancreatic duct in an autopsy of an executed murderer. However, he never published his finding because the function of this duct was not yet known. Instead, he secretly engraved his finding on a copper plate (Fig 13) and made seven impressions, which he sent to famous European anatomists. Doing so not only allowed him to seek advice as to the function of the duct, but also ensured him recognition for its discovery. Wirsüng was assassinated on August 22, 1643, by Jacques Cambier, a Belgian student. The murder was thought to be an act of revenge for Cambier’s forced resignation, based on suspected unethical behavior, from his newly elected post as Procurator of the German Nation of Artists (26).


Figure 13
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  		Figure 13.  Copper plate engraving of Wirsüng’s original description of the main pancreatic duct (Padova, Italy, 1642). (From the library of the Università deglis Studi di Padova.)

 
Duct of Santorini
The duct of Santorini, or accessory pancreatic duct, derives from portions of the dorsal pancreatic bud of the foregut. When present, it drains into the duodenum through the minor papilla. It may communicate with the main pancreatic duct (duct of Wirsüng), or it may drain the body and tail of the pancreas, with the duct of Wirsüng separately draining portions of the pancreatic head and uncinate process, a condition known as pancreas divisum.

Giovanni Domenico Santorini (1681–1737) (Fig 14) was born in Venice and studied medicine at Bologna and Padua. In 1701, he received his medical degree from the University of Pisa, and, upon his return to Venice, he was named Professor of Medicine at the Physico-Medical College there, where his duties included instruction in anatomy. Santorini had a reputation as a meticulous dissector, and his anatomic illustrations, published posthumously in 1765 (27), were regarded as among the masterpieces of that century. In addition to delineating the accessory pancreatic duct, Santorini was the first to describe the emissary veins that drain the dura mater covering the brain. For a time, discovery of the accessory pancreatic duct was credited to Claude Bernard (1813–1878), the great French physiologist, who was the first to describe the digestive function of the pancreas (28,29).


Figure 14
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  		Figure 14.  Giovanni Domenico Santorini (1681–1737). (From the National Library of Medicine.)

 
Peritoneum
The Rigler sign, or "double wall sign," refers to the presence of gas on both sides of the bowel wall on an abdominal radiograph, a finding that is indicative of pneumoperitoneum (Fig 15) (30). The Rigler triad consists of dilated small bowel, pneumobilia, and calcified ectopic gallstone and is indicative of gallstone ileus (31).


Figure 15
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  		Figure 15.  Rigler sign (double wall sign). Supine radiograph of the abdomen shows loops of bowel outlined by free intraperitoneal gas (arrows).

 
Leo George Rigler (1896–1979) (Fig 16) was born in Minneapolis, Minnesota, and completed his undergraduate, graduate, and medical education at the University of Minnesota. After a 1-year internship at St Louis (Missouri) City Hospital, he worked briefly as a general practitioner in New England and North Dakota, later returning to Minneapolis. He served as a radiologist at Minneapolis General Hospital from 1923 to 1926, and, while serving in this capacity, he received formal training in radiology at Battle Creek (Michigan) Sanatorium under the supervision of J. T. Case and at the University of Michigan under P. J. Hickey.


Figure 16
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  		Figure 16.  Leo G. Rigler (1896–1979). (From the National Library of Medicine.)

 
In 1924, Rigler spent a year at the Karolinska Institute in Stockholm, Sweden, with Gosta Forssel. Upon his return to Minneapolis, he received a full-time faculty appointment at the University of Minnesota. He was promoted to professor 2 years later and was asked to be the first chairman of the Department of Radiology, a position that he held until 1957. In 1934, he became the 68th candidate to be certified by the American Board of Radiology.

In 1957, Rigler moved to California, serving as Executive Director of the Cedars of Lebanon–Mount Sinai Hospitals. In 1963, he returned to academic medicine at the University of California, Los Angeles, serving as the director of the postgraduate training program in diagnostic radiology there until his death in 1979.

In 1941, Rigler described the sign of pneumoperitoneum that now bears his name. However, he made other, even greater contributions to radiology. Rigler built a legacy of radiologic teaching, instituting the interdepartmental radiology conference at the University of Minnesota and establishing the first postgraduate course in radiology. He traveled with the World Health Organization after World War II to help strengthen clinical radiology in other nations. A large number of his trainees became leaders in academic radiology (32,33).


   Systemic Disease
Top
 Abstract
 Introduction
 Liver and Biliary Tree
 Pancreas
 Systemic Disease
Conclusions
 References
 Suggested Readings
 
Crohn Disease
Crohn disease is an inflammatory disorder of unknown cause that can involve any part of the alimentary tract (Fig 17). Inflammation of the bowel wall, ranging from mucosal erosions to full-thickness inflammation, and formation of noncaseating granulomas characterize the disease. Radiographic manifestations include aphthous lesions; deep, confluent ulcerations; thickened, nodular, or distorted mucosal folds; bowel wall fibrosis with stricture formation; loop separation caused by mesenteric involvement; areas of normal bowel interposed between diseased segments; and formation of fistulas and sinus tracts (34,35).


Figure 17
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  		Figure 17.  Crohn disease. Image from a small bowel barium examination shows a long stricture (arrow) and an intramural tract (arrowhead).

 
Burrill B. Crohn (1884–1983) (Fig 18) was one of 12 children in a German-Jewish immigrant family living in New York City. He enrolled at the City University of New York at 13 years of age and graduated at age 18 years. He then pursued studies in medicine at the College of Physicians and Surgeons at Columbia University. Crohn completed a 2-year rotating internship and a year-long fellowship at Mount Sinai Hospital in New York City. His career as a gastroenterologist was highlighted by his election as President of the American Gastroenterological Association in 1935.


Figure 18
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  		Figure 18.  Burrill Bernard Crohn (1884–1983). (Courtesy of the Mount Sinai Archives, New York, NY.)

 
His first description of regional ileitis, which would later become known as Crohn disease, was published in JAMA in 1932 (36). Richard Marshak, Crohn’s radiologist, was unable to convince him that regional enteritis could also occur in the colon, a concept that was not accepted by Crohn and other American investigators until much later, following the reports of Lockhart-Mummery et al (37). For a time, Crohn believed that both ulcerative colitis and regional ileitis were actually infections, likely viral in origin, and was a strong proponent of using steroids to treat them.

Whipple Disease
Whipple disease (Fig 19) is a rare systemic disorder that can affect the gastrointestinal tract, joints, and central nervous system. It most commonly occurs in older men, who often present with arthritis, neurologic dysfunction, or steatorrhea. The etiologic agent has been identified as the Tropheryma whippelii bacillus, which is found within macrophages in various tissues. At radiography, small bowel folds may be thickened and distorted, particularly in the jejunum. The presence of 1–2-mm nodules in the small bowel mucosa, representing villi enlarged by distended lymphatic vessels, as well as of periodic acid-Schiff–positive macrophages deposited in the submucosa and lamina propria, is highly suggestive of the disease. At computed tomography, enlarged low-attenuation mesenteric lymph nodes may be present.


Figure 19
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  		Figure 19.  Whipple disease. Image from a small bowel barium examination shows mildly thickened and nodular folds.

 
George Hoyt Whipple (1878–1976) (Fig 20) was born in New Hampshire into a family of rural physicians. He received his medical training at Johns Hopkins University in Baltimore, Maryland, joining the faculty afterwards and rising to the rank of Associate Professor of Pathology. In 1909, Whipple meticulously described a previously unrecognized lipodystrophy in a young physician, a condition now known as Whipple disease (38). Later, he took the position of Professor of Research Medicine at the University of California. In 1921, he was appointed Professor of Pathology and dean at the newly founded medical school at the University of Rochester (New York). It was there that Whipple focused on the pathophysiologic features of anemia, and, in 1934, he shared the Nobel Prize in medicine for discoveries leading to the treatment of primary pernicious anemia with liver extract (39).


Figure 20
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  		Figure 20.  George Hoyt Whipple (1878–1976). (From the National Library of Medicine.)

 
Behçet Disease
Behçet disease (Fig 21) is a multisystemic condition resulting from a small vessel vasculitis. It primarily affects the skin, eyes, joints, central nervous system, and intestinal tract (40). Intestinal disease most commonly occurs in the ileocecal region, typically manifesting as aphthous lesions, deep ulcerations, strictures, and development of fistulas. These findings closely mimic Crohn disease.


Figure 21
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  		Figure 21.  Behçet disease involving the colon. Magnified image from an air-contrast barium enema examination shows segmental areas with ulcers (arrowhead) and inflammatory polyps (arrow).

 
Hulusi Behçet (1889–1948) (Fig 22) was born in Istanbul, which at that time was part of the Ottoman Empire. His father was a prominent businessman, which allowed him to get an excellent education in Damascus, Syria. Behçet received his medical training at the Gülhane Military Medical Academy in Ankara, Turkey, since there was no civilian medical school there at the time, graduating at 21 years of age in 1910. He specialized in dermatology and venereal diseases, serving at Edirne Military Hospital during World War I.


Figure 22
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  		Figure 22.  Hulusi Behçet (1889–1948).

 
Following the war, Behçet went first to Budapest and then to Berlin to further his medical knowledge. In 1923, he returned to the newly established Turkish Republic and was appointed head medical doctor at Hasköy Venereal Disease Hospital. Shortly thereafter, he moved to Guraba Hospital, now part of Istanbul Medical College.

Behçet wrote extensively on syphilis and leishmaniasis, publishing his paramount monograph, Clinical and Practical Syphilis, Diagnosis and Related Dermatoses, in 1940. Behçet’s first observations of the disease that would eventually bear his name began in 1924. He described three cases of the disease in 1936, first at a meeting and then in publication (41). However, it was not until around 1947 that this entity was universally accepted, since many dermatologists had been skeptical of its cutaneous manifestations.

Peutz-Jeghers Syndrome
Peutz-Jeghers syndrome is an autosomal dominant disorder that is characterized by formation of multiple hamartomatous polyps in the digestive tract. It most commonly involves the small intestine but can also affect the stomach (Fig 23) and colon. Characteristic pigmented macules develop on the face, the ventral aspects of the digits, and the mucous membranes. At radiography, the polyps vary in size and are most commonly seen in the jejunum, with areas of normal bowel interspersed among affected segments. Patients are at risk for intussusception because polyps can serve as a lead point. Although the polyps themselves rarely undergo malignant degeneration, patients with Peutz-Jeghers syndrome are at risk for developing adenocarcinoma of the gastrointestinal tract as well as other malignancies, including adenocarcinoma of the breast, pancreas, and ovary (42).


Figure 23
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  		Figure 23.  Peutz-Jeghers syndrome. Image from a small bowel barium examination shows two large filling defects (arrows) representing hamartomatous polyps.

 
Johannes L. A. Peutz (1886–1957) was a Dutch internist who, in 1921, described a familial condition characterized by pigmentation of the skin and mucous membranes and intestinal polyposis (43). Dermal involvement had previously been described by J. T. Connor in 1895, when he reported the cutaneous manifestations in identical twin girls, who subsequently died of complications of intussusception and of carcinoma of the breast, respectively (44).

Harold J. Jeghers (1904–1990) (Fig 24) was born in New Jersey and received his medical degree from Western Reserve University in Cleveland. He trained in internal medicine at Boston City Hospital and was appointed Chairman of the Department of Medicine at Georgetown University in Washington, DC, in 1946. In 1966, Jeghers returned to Boston, where he was appointed Professor of Medicine at Tufts University Medical School. In 1949, he published a detailed account of the syndrome that now bears his name (44–46).


Figure 24
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  		Figure 24.  Harold J. Jeghers (1904–1990). (From the National Library of Medicine.)

 
Kaposi Sarcoma
Kaposi sarcoma (Fig 25) is the most common AIDS-associated malignancy and is the second most common AIDS-defining condition following Pneumocystis carinii pneumonia (47). Kaposi sarcoma is caused by a subtype of herpes virus, and now occurs almost exclusively in homosexual or bisexual men infected by human immunodeficiency virus (48). The neoplasm is multicentric and can involve the skin, solid viscera, gastrointestinal tract, and mucous membranes. Kaposi sarcoma involves the gastrointestinal tract in about 40%–50% of affected individuals, causing nodules, plaques, polyps, and fold thickening. Bulky lymphadenopathy may develop in the mesentery and retroperitoneum, and intense lymph node enhancement at computed tomography is highly suggestive of the disease in the appropriate clinical context (49).


Figure 25
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  		Figure 25.  Esophageal Kaposi sarcoma in a man with acquired immunodeficiency syndrome (AIDS). Air-contrast esophagogram shows a 2-cm smooth filling defect (arrow).

 
Moritz Kaposi (1837–1902) (Fig 26) was born Moritz Kohn in Kaposvar, Hungary, officially changing his surname to Kaposi (for his birthplace) in 1871. The exact reason for changing his name is unknown, but the change may reflect his conversion from Judaism to Catholicism. Alternatively, Kaposi suggested that he had changed his name because Kohn was a very common name and he wanted to ensure that he received proper credit for several of his important papers.


Figure 26
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  		Figure 26.  Moritz Kaposi (1837–1902). (From the National Library of Medicine.)

 
Kaposi was an accomplished clinician and histopathologist, publishing over 100 papers on various aspects of dermatology. He was the first to describe several diseases, including disseminated lupus erythematosis, xeroderma pigmentosum, eczema herpeticum, and dermatitis papillaris capillitii. However, it is the angiosarcoma that bears his name for which he is remembered. Kaposi’s initial paper describing this neoplasm, published in 1872, discusses the cases of five men, all of whom were over 40 years old and had a rapidly lethal multicentric angiosarcoma involving the skin, upper aerodigestive tract, and gastrointestinal tract (50,51). He immediately recognized that the disease was systemic, unlike cutaneous metastasis from sarcomas located elsewhere in the body.

In 1880, Kaposi was appointed Professor and Chairman of the Department of Dermatology at the University of Vienna, where he trained an entire generation of dermatologists until his death in 1902. His Pathologie und Therapie der Hautkrankheiten in Vorlesungen (ie, Lectures on the Pathology and Treatment of Skin Diseases) was published in 1880. It was translated into many languages and widely read for many years (51).

Chagas Disease
Chagas disease results from infection by Trypanosoma cruzi, a parasite found in the hindgut of reduviid bugs. Chagas disease afflicts approximately 12 million people, primarily in Central and South America, and results in about 13,000 deaths per year. Because 15%–30% of individuals with Chagas disease develop chronic forms of the disease, including damage to the heart, digestive tract, and nervous system, morbidity is quite extensive.

The main manifestation of chronic Chagas disease in the digestive tract is megaviscera, primarily megaesophagus (Fig 27) and megacolon, resulting from destruction of the myenteric plexus by T cruzi. Megaesophagus usually manifests as dysphagia and can be diagnosed with esophagography. Megacolon leads to varying degrees of constipation and may be detected with contrast enema examination. An emergent complication of megacolon is torsion of the dilated segment, leading to obstruction and possibly ischemia with subsequent perforation (52).


Figure 27
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  		Figure 27.  Megaesophagus from chronic Chagas disease. Single-contrast esophagogram shows a markedly dilated esophagus with mucosal irregularity. The esophageal lumen tapers at the esophagogastric junction.

 
Carlos Justiniano Ribeiro Chagas (1879–1934) (Fig 28) was born in the small town of Oliveira, Brazil. His father, who died when Chagas was 4 years old, owned a small coffee plantation. His mother continued to run the coffee plantation while raising her four children, and encouraged Chagas to study mining engineering. However, his uncle, a physician, encouraged him to study medicine, arguing that eradication of endemic diseases was crucial to Brazil’s development. When Chagas began his studies at the Faculty of Medicine in Rio de Janeiro, yellow fever, smallpox, and bubonic plague were rampant. He received his degree in 1902, writing his doctoral thesis on the hematologic aspects of malaria. During that year, Chagas met Oswaldo Cruz (1871–1917), who was working at Manguinhos, an institute that was dedicated to the production of vaccines and sera and that would later be named after Cruz. The two men became friends at once, and Chagas eventually accepted Cruz’s invitation to join the institute.


Figure 28
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  		Figure 28.  Carlos Justiniano Ribeiro Chagas (1879–1934). (From the National Library of Medicine.)

 
In 1907, while working on an antimalaria campaign at the site of expansion of the Central Railway, Chagas became aware of the "barbeiro," a blood-sucking insect that had infested the huts of the region and had a predilection for biting victims on the face. In the hindgut of this insect, Chagas found a new species of trypanosome, which he named cruzi in honor of his mentor. He first found the trypanosome in the blood of a cat, and then in a girl whom he had seen earlier in the same hut as the infected cat. Chagas carried out several experiments, reproducing the infection in small animals and recovering the trypanosome in them. However, whether the infection was pathogenic in vertebrates was yet to be determined. In 1909, 2 years after identifying the new parasite, Chagas published several reports on the disease in humans (53,54). In 1911, he established the definitive description of the disease, including its clinical, etiologic, pathologic, and epidemiologic features. Thus, within 2 short years, the young man identified an unknown single-cell organism and described an entire disease from its cause to its epidemiologic features, confirming all of Koch’s postulates in the process (55,56).


   Conclusions
Top
 Abstract
 Introduction
 Liver and Biliary Tree
 Pancreas
 Systemic Disease
 Conclusions
References
 Suggested Readings
 
Numerous eponyms are encountered in radiology of the digestive tract, and this two-part series is by no means comprehensive. However, familiarity with many of these eponyms is important for accurate communication. Although some purists may argue against their use, eponyms serve as a means of honoring those individuals who have made important discoveries and observations. Acquiring a little historical knowledge about these individuals brings some humanity back into the science of medicine.


   Footnotes
 

Abbreviations: AIDS = acquired immunodeficiency syndrome

See Kanne et al in the January 2006 issue (pp 129–142) for Part 1 of this two-part series.


   References
Top
 Abstract
 Introduction
 Liver and Biliary Tree
 Pancreas
 Systemic Disease
 Conclusions
 References
Suggested Readings
 

  1. The American Heritage Stedman’s Medical Dictionary. Boston, Mass: Houghton Mifflin, 2002.
  2. Miller WJ, Sechtin AG, Campbell WL, Pieters PC. Imaging findings in Caroli’s disease. AJR Am J Roentgenol 1995;165:333–337.[Abstract/Free Full Text]
  3. Levy AD, Rohrmann CA Jr, Murakata LA, Lonergan GJ. Caroli’s disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol 2002;179:1053–1057.[Abstract/Free Full Text]
  4. Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska M. La dilatation polykystigul congenitale desvoies biliares intrahepatigues: essai de classification. Sem Hop 1958;34:488–495.[Medline]
  5. Haubrich WS. Caroli of Caroli’s disease. Gastroenterology 2000;118:486.[CrossRef]
  6. Soyer P, Bluemke DA, Reichle R, et al. Imaging of intrahepatic cholangiocarcinoma. II. Hilar cholangiocarcinoma. AJR Am J Roentgenol 1995;165: 1433–1436.[Abstract/Free Full Text]
  7. Klatskin G. Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis: an unusual tumor with distinctive clinical and pathological features. Am J Med 1965;38:241–256.[CrossRef][Medline]
  8. Altemeier WA, Gall EA, Zinninger MM, Hoxworth PI. Sclerosing carcinoma of the major intrahepatic bile ducts. AMA Arch Surg 1957;75:450–461.[Medline]
  9. Diekhoff EJ. Altemeier tumors? [letter]. Am J Surg 1993;166:570–571.[Medline]
  10. Haubrich WS. Klatskin of the Klatskin tumor. Gastroenterology 1999;116:1309.
  11. Dasgupta D, Stringer MD. Cystic duct and Heister’s "valves". Clin Anat 2005;18:81–87.[CrossRef][Medline]
  12. Wood M. Presidential address: eponyms in biliary tract surgery. Am J Surg 1979;138:746–754.[CrossRef][Medline]
  13. Heister L. Compendium anatomica. Amsterdam, the Netherlands: 1720.
  14. Levy AD, Murakata LA, Abbott RM, Rohrmann CA Jr. From the archives of the AFIP: benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts—radiologicpathologic correlation. RadioGraphics 2002;22:387–413.[Abstract/Free Full Text]
  15. Rokitansky C. Handbuch der speziellen pathogischen anatomie. Vienna, Austria: Braumuller & Seidel, 1842.
  16. Davies MK, Hollman A. Karl Freiherr von Rokitansky (1804–78). Heart 1997;78:425.[Free Full Text]
  17. Tesar V, Kazderova M, Hlavackova L. Rokitansky and his first description of polyarteritis nodosa. J Nephrol 2004;17:172–174.[Medline]
  18. Haubrich WS. Rokitansky and Aschoff of the Rokitansky-Aschoff sinuses. Gastroenterology 2001; 120:1598.
  19. Aschoff CAL. Bemerkungen sur pathologischen anatomie der cholelithiasis und cholecystitis. Verhandl Deutsch Path Gesellsch 1905;9:41.
  20. Oddi R. Di una disposizione a sfinter allo abocco del coledoco. Arch Ital Biol 1887;8:317–322.
  21. Haubrich WS. Oddi of the sphincter of Oddi. Gastroenterology 1999;116:542.
  22. Vater A. Novum bilis diverticulum: dissertatio IV. In: Wittenbergae: Lit Gerdesianus, 4to, 1720.
  23. Bernard C. Memoire sur le pancréas. Paris, France: 1856.
  24. Stern CD. A historical perspective on the discovery of the accessory duct of the pancreas, the ampulla ‘of Vater’ and pancreas divisum. Gut 1986; 27:203–212.[Abstract/Free Full Text]
  25. Suarez CV. Ampulla of Vater: a misnomer. Mt Sinai J Med 1980;47:373–385.[Medline]
  26. Howard JM, Hess W, Traverso W. Johann Georg Wirsung (1589–1643) and the pancreatic duct: the prosector of Padua, Italy. J Am Coll Surg 1998;187:201–211.[CrossRef][Medline]
  27. Santorini GD. Observationes anatomicae. Venice, Italy: 1775.
  28. Busnardo AC, DiDio LJ, Tidrick RT, Thomford NR. History of the pancreas. Am J Surg 1983;146: 539–550.[CrossRef][Medline]
  29. Haubrich WS. Biographical sketch: Bernard of the duct of Bernard. Gastroenterology 2000;119:828.[CrossRef][Medline]
  30. Rigler LG. Spontaneous pneumoperitoneum: a roentgenologic sign found in the supine position. Radiology 1941;37:604–607.
  31. Rigler LG, Borman CN, Noble JF. Gallstone obstruction: pathogenesis and roentgen manifestations. JAMA 1941;117:1753–1759.
  32. Lewicki AM. The Rigler sign and Leo G. Rigler. Radiology 2004;233:7–12.[Free Full Text]
  33. Heitzman ER. Leo G. Rigler, MD: a personal perspective. Radiology 2004;233:13–14.[Free Full Text]
  34. Gore RM, Balthazar EJ, Ghahremani GG, Miller FH. CT features of ulcerative colitis and Crohn’s disease. AJR Am J Roentgenol 1996;167:3–15.[Free Full Text]
  35. Wills JS, Lobis IF, Denstman FJ. Crohn disease: state of the art. Radiology 1997;202:597–610.[Free Full Text]
  36. Crohn BB, Ginzburg L, Oppenheimer GD. Regional ileitis: a pathologic and clinical entity. JAMA 1932;35:1323–1329.
  37. Janowitz HD. Burrill B. Crohn (1884–1983). Mt Sinai J Med 2000;67:12–13.[Medline]
  38. Whipple GH. A hitherto undescribed disease characterised anatomically by deposits of fat and fatty acids in the intestinal mesenteric lymphatic tissue. Johns Hopkins Hosp Bull 1907;18:382–391.
  39. Haubrich WS. Whipple of Whipple’s disease. Gastroenterology 1999;117:576.[CrossRef]
  40. Iida M, Kobayashi H, Matsumoto T, et al. Intestinal Behçet disease: serial changes at radiography. Radiology 1993;188:65–69.[Abstract/Free Full Text]
  41. Behçet H. Ü ber rezidivierende aphtösedurch ein virus verursachte gesehwüre am mund, am auge, und an der genitalien. Derm Wschr 1937;105: 1152–1163.
  42. Buck JL, Harned RK, Lichtenstein JE, Sobin LH. Peutz-Jeghers syndrome. RadioGraphics 1992;12: 365–378.[Abstract]
  43. Peutz JLA. Over een zeer merkvaardige gecombineerde familiaire polyposis van de slijmvliezen, van den tractus intestinalis met die van de neuskeelholte en gepard met eigenaardige pigmentaties van huid en slijmvliezen. Nederlandsch Maandschrift voor Geneeskunde 1921;10:134–136.
  44. Keller JJ, Offerhaus GJ, Giardiello FM, Menko FH. Jan Peutz, Harold Jeghers and a remarkable combination of polyposis and pigmentation of the skin and mucous membranes. Fam Cancer 2001; 1:181–185.[CrossRef][Medline]
  45. Haubrich WS. Jeghers of the Peutz-Jeghers syndrome. Gastroenterology 1999;117:1068.[CrossRef]
  46. Jeghers H, McKusick VA, Katz KH. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits: a syndrome of diagnostic significance. N Engl J Med 1949;241:993–1005.[Medline]
  47. Herndier BG, Friedman SL. Neoplasms of the gastrointestinal tract and hepatobiliary system in acquired immunodeficiency syndrome. Semin Liver Dis 1992;12:128–141.[Medline]
  48. Moore PS, Chang Y. Detection of herpesvirus-like DNA sequences in Kaposi’s sarcoma in patients with and without HIV infection. N Engl J Med 1995;332:1181–1185.[Abstract/Free Full Text]
  49. Herts BR, Megibow AJ, Birnbaum BA, Kanzer GK, Noz ME. High-attenuation lymphadenopathy in AIDS patients: significance of findings at CT. Radiology 1992;185:777–781.[Abstract/Free Full Text]
  50. Kaposi M. Idiopathisches multiples pigmentsarcom der haut. Arch Derm Syph 1872;4:265–278.[CrossRef]
  51. Oriel JD. Moritz Kaposi (1837–1902). Int J STD AIDS 1997;8:715–717.[Free Full Text]
  52. Barrett MP, Burchmore RJS, Stich A, et al. The trypanosomiases. Lancet 2003;362:1469–1480.[CrossRef][Medline]
  53. Chagas C. Nouvelle espèce de trypanosomiase humaine. Bull Soc Pathol Exotique 1909;2:304–307.
  54. Chagas C. Nova trypanozomiaze humana: estudos sobre a morfolojia e cíclo evolutivo do schizotripanum cruzi n gen n sp, ajente etiolòjico de nova entidade morbida do homem. Mem Inst Oswaldo Cruz 1909;1:159–218.
  55. Haubrich WS. Chagas of Chagas’ disease. Gastroenterology 2003;125:1031.[CrossRef][Medline]
  56. Lewinsohn R. Carlos Chagas and the discovery of Chagas’ disease (American trypanosomiasis). J R Soc Med 1981;74:451–455.[Medline]

   Suggested Readings
Top
 Abstract
 Introduction
 Liver and Biliary Tree
 Pancreas
 Systemic Disease
 Conclusions
 References
 Suggested Readings
 
Delong MF. Medical acronyms, eponyms & abbreviations. 4th ed. Los Angeles, Calif: Practice Management Information Corporation, 2002.

Firkin BG, Whitworth JA. Dictionary of medical eponyms. 2nd ed. New York, NY: Parthenon, 1996.

Forbis P, Bartolucci SL. Stedman’s medical eponyms. Philadelphia, Pa: Lippincott Williams & Wilkins, 1998.

Marcucci L. Marcucci’s handbook of medical eponyms. Philadelphia, Pa: Lippincott Williams & Wilkins, 2001.

Morton LT. Morton’s medical bibliography: an annotated check-list of texts illustrating the history of medicine. 5th ed. Scolar Press/Ashgate, 1991.

Skinner HA. The origin of medical terms. 2nd ed. Baltimore, Md: Williams & Wilkins, 1961.

Sloane SB. Medical abbreviations & eponyms. 2nd ed. Philadelphia, Pa: Saunders, 1997.





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