Eponyms in Cardiothoracic Radiology—Part II: Vascular Tan-Lucien H. Mohammed, MD, FCCP,a Megan R. Saettele, MD,b Timothy Saettele, MD,c Vikas Patel, MD,b and Jeffrey P. Kanne, MDd

Eponyms serve the purpose of honoring individuals who have made important observations and discoveries. As with other fields of medicine, eponyms are frequently encountered in radiology, particularly in chest radiology. However, inappropriate use of an eponym may lead to potentially dangerous miscommunication. Moreover, an eponym may honor the incorrect person or a person who falls into disrepute. Despite their limitations, eponyms are still widespread in the medical literature. Furthermore, in some circumstances, more than one individual may have contributed to the description or discovery of a particular anatomical structure or disease, whereas in others, an eponym may have been incorrectly applied initially and propagated for years in the medical literature. Nevertheless, radiologic eponyms are a means of honoring those who have made lasting contributions to the field of radiology, and familiarity with these eponyms is critical for proper reporting and accurate communication. In addition, the acquisition of some historical knowledge about those whose names are associated with various structures or pathologic conditions conveys a sense of humanity in the science of medicine. In this second part of a multipart series, the authors discuss a number of chest radiology eponyms as they relate to the pulmonary vasculature, including relevant clinical and imaging features, as well biographic information of the respective eponym's namesake.

From the aDepartment of Radiology, Virginia Mason Medical Center, Seattle, WA; bDepartment of Radiology, University of Missouri-Kansas City, Kansas City, MO; cDepartment of Pulmonary Medicine, MD Anderson Cancer Center, Houston, TX; and dDepartment of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, WI. Reprint requests: Tan-Lucien H. Mohammed, MD, FCCP, Department of Radiology, Virginia Mason Medical Center, Mail code C5-XR, 1100 Ninth St, Seattle, WA 98101. E-mail: [email protected]. Curr Probl Diagn Radiol 2014;43:219–226. & 2014 Mosby, Inc. All rights reserved. 0363-0188/$36.00 + 0 http://dx.doi.org/10.1067/j.cpradiol.2014.02.004

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Introduction In the field 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 radiology, particularly that of the cardiopulmonary system, and knowledge of these terms is important for appropriate reporting and communication. Eponyms are a means of honoring individuals who have made important contributions to medicine, but use of these terms may fail to convey a precise meaning or definition and could lead to dangerous miscommunication. Furthermore, it may be that more than one individual contributed to the discovery or description of a structure or disease. In other cases, an eponym may have been incorrectly applied initially and propagated for years in the medical literature. In this article, the second of a multipart series, we discuss and illustrate the imaging manifestations of eponyms commonly encountered in chest radiology as they relate to vascular diseases affecting the great vessels of the chest (Rasmussen aneurysm, Behçet disease, Marfan syndrome [MFS], and Takayasu arteritis [TA]), as well as vascular diseases afflicting the lungs (Osler-Weber-Rendu syndrome [OWRS]) and the coronary vessels (Kawasaki disease [KD]). We also explore the historical background of the individuals for whom these eponyms were named.

Great Vessels of the Chest Rasmussen Aneurysm Rasmussen aneurysm (Fig 1) is considered an uncommon complication of pulmonary tuberculosis and represents a pulmonary artery pseudoaneurysm adjacent to or within a tuberculous cavity.2,3 It occurs in up to 5% of patients with such lesions and may lead to rupture and hemorrhage.


Copenhagen in 1862, he worked as an assistant at the newly established Communehospitalet and was a prosector in the pathologic-anatomical museum. During the war with Germany over Schleswig-Holstein in 1864, Rasmussen was the head physician in a military hospital in the castle of Frederiksborg. The following year, in 1865, he was appointed as the prosector in the Communehospitalet. Subsequently, he obtained his doctorate in 1866. From 1866, he was the coeditor with Karl Georg Lange (1834-1900) of the Hospitals-Tidende, and from 1868-1874 was a privatdocent of pathologic anatomy department at the University of Copenhagen. During the years 1870-1875, on an initiative by Carl Emil Fenger (1814-1884), he headed a mixed department of medicine and surgery at the Communehospital and, in 1874, with Karl Georg Lange, established a medico-pneumatic institution as well as a clinic for diseases of the chest. From 1874 until his untimely death in 1877, he held the chair of pathologic anatomy and general pathology departments at the University of Copenhagen.4 However, owing to illness, he was never active in this position.

Behçet Disease FIG 1. Selective right pulmonary angiogram shows rounded right upper lobe mass representing a Rasmussen aneurysm in a 39-year-old man with established history of Tb. Tb, tuberculosis.

Fritz Valdemar Rasmussen (August 28, 1837 to February 22, 1877) was a Danish physician who, in 1860, completed his basic schooling in Copenhagen and subsequently studied abroad, under Rudolf Virchow (1821-1902) in Berlin, Germany. Upon returning to

Behçet disease is a multisystemic and chronic inflammatory vasculitis of unknown etiology. The mean age at which Behçet disease occurs is 20-30 years. Thoracic manifestations of Behçet disease affect 1%-8% of patients. Pulmonary arterial aneurysms are the most common and can be fusiform or saccular (Fig 2A). These aneurysms are commonly multiple and bilateral and located in the lower lobe or main pulmonary arteries. They occur almost exclusively in men and are associated

FIG 2. (A) Axial maximum intensity projection from a contrast-enhanced CT scan of a 28-year-old man with Behçet disease shows aneurysms of the pulmonary arteries with irregular wall thickening (arrows). (B) Hulusi Behçet (1889-1948). (Image courtesy: the History of Medicine [NLM]). NLM, National Library of Medicine; CT, computed tomography.


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with a poor prognosis. Other imaging manifestations in the lungs include subpleural alveolar consolidation and wedge-shaped or rounded areas of increased attenuation, which represent focal vasculitis and thrombosis resulting in infarction, hemorrhage, and focal atelectasis.5,6 Hulusi Behçet (February 20, 1889 to March 8, 1948) was born in Istanbul, Turkey (Fig 2B). Behçet lost his mother during his childhood and was raised by his grandmother. Following the establishment of the Turkish Republic and the “Family Name Law” was accepted, his father Ahmet Behçet, a businessman who was a friend of Mustafa Kemal Atatürk, the founder of Turkish Republic, received private permission to use his father's name, Behçet. Hulusi Behçet pursued his education at Gülhane Military Medical Academy. After he became a medical doctor, he specialized in dermatology and venereal disease at Gülhane Military Medical Academy and completed his training in 1914.7 Behçet was appointed as a professor to the clinic of dermatology and syphilis in 1933, receiving the degree of ordinarius in 1939. He held his professorship for 14 years, until 1947. He was the first person in Turkish academic life that received the title of Professor.7 His first observations on the disease that would ultimately bear his name began with a patient he initially encountered in 1924. Subsequently, Dr Behçet followed the symptoms of 3 patients whom he had cared for over many years and then concluded that they were all related to a previously undescribed disease (1936). He published these cases in the Archives of

Dermatology and Venereal Disease. He was a heavy smoker and died of a sudden myocardial infarction on March 8, 1948.

Marfan Syndrome MFS is a multisystem hereditary connective tissue disease caused by a mutation of the fibrillin-1 gene. MFS is characterized by a wide range of clinical manifestations.8 Common cardiovascular manifestations, most of which are substantial contributors to mortality, include annuloaortic ectasia with or without aortic valve insufficiency, aortic dissection, aortic aneurysm, pulmonary artery dilatation, and mitral valve prolapse (Fig 3A). Scoliosis, pectus excavatum and carinatum, arachnodactyly, and acetabular protrusion are common musculoskeletal manifestations.8,9 Antoine Bernard-Jean Marfan was a French pediatrician born June 23, 1858 in Castelnaudary (Fig 3B). In 1887, he enrolled in Toulouse School of Medicine and then transferred to Paris in 1879 to complete his medical training.10,11 Dr Marfan then joined the French military, and in 1886, he graduated from his service with a silver medal. In 1892, he began working at the Hôpital des Enfants Malades in France as an assistant professor of pediatrics. In 1896, he published an article describing a case of a 5-year-old girl with disproportionately long limbs and fingers.10 The patient eventually died; however, after subsequent study of the same patient by later physicians, it was determined that Dr Marfan's patient

FIG 3. (A) Coronal maximum intensity projection from a contrast-enhanced CT scan of a 32-year-old man with Marfan syndrome shows aortic annuloaortic ectasia (arrows). (B) Bernard-Jean Antoine Marfan (1858-1942). (Portrait by Emile Charles Wauters. Paris, Musée d’Histoire de la Medecine.)

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may not have suffered from what is now known as MFS, and her case seemed more typical of a congenital contractural disorder. Despite the controversy, disorders of fibrillin resulting in the characteristic phenotype of long limbs, aortic wall laxity, and lens dislocation were designated MFS. In 1897, he coauthored the Treatise on Diseases of Children, and he was recognized with an award by the French Academy of Sciences.11 In 1910, he was appointed head of the diphtheria service and held that position at Hôpital des Enfants Malades until 1928 when he retired. In 1932, Marfan was elected honorary member of the Royal Society of Medicine. He died in Paris in 1942. Dr Marfan was a pioneer of pediatrics during his lifetime and remains so today.

Takayasu Arteritis TA (also known as idiopathic medial aortopathy or pulseless disease) is a granulomatous large-vessel

vasculitis predominantly affecting the aorta and its major branches. It may also affect the pulmonary arteries.12 The exact cause is not known but pathology is similar to giant cell arteritis. There is a strong female predominance (F:M  9:1), increased prevalence in Asian populations, and it typically affects younger patients (o50 years of age). The typical age of onset is approximately 15-30 years of age. Computed tomography and magnetic resonance imaging (MRI) imaging features of TA include vessel wall thickening and enhancement early in the disease, and stenoses, occlusions, and aneurysms later in the course of the disease13 (Fig 4A and B). Cross-sectional imaging, particularly MRI, is useful for establishing the diagnosis of TA and for showing response to nonsurgical therapy or for planning a surgical intervention. Mikito Takayasu (September 4, 1860 to November 20, 1938) was a Japanese ophthalmologist (Fig 4C).

FIG 4. (A) T1-weighted black-blood axial MR image of a 26-year-old woman with Takayasu arteritis shows thickening of the descending thoracic aortic wall. (B) Fat-suppressed T2-weighted axial MR image shows high signal intensity of the descending thoracic aortic wall, reflecting active inflammation. (C) Image courtesy: Mikito Takayasu and Jokichi Takamine, two famous Japanese doctors who were from Kanazawa, Japan. Jpn J Vasc Surg. 563-569, 2003. MR, magnetic resonance.


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He graduated from Tokyo Imperial University in 1887, and from 1888, he worked as a teacher of ophthalmology at the Fourth High School, Faculty of Medicine, in Kanazawa, Ishikawa, which was the predecessor of the present Kanazawa University School of Medicine.14 At the 12th Congress of the Japanese Society for Ophthalmology in Fukuoka on April 1, 1908, he reported a case of a 22-year-old woman with peculiar arteriovenous anastomosis and aneurysms. The patient experienced visual disturbance for approximately 1 year and eventually lost visual acuity in both eyes. Later, this disease was summarized and has been called Takayasu disease or pulseless disease, distinguished by no radial pulse, flower-ring– like vascular anastomoses and hypersensitivity of the carotid body. Takayasu lived for 46 years in Kanazawa and moved to Beppu, Kyushu, where he died in November 1938.15

Lungs Osler-Weber-Rendu Syndrome Also known as hereditary hemorrhagic telangiectasia and Rendu-Osler-Weber syndrome, OWRS is an autosomal dominant disease characterized by mucosal and cutaneous telangiectases and arteriovenous malformations (AVMs).16 OWRS affects both the sexes evenly. It may present at any age, but most commonly in the third decade of life. Typically, OWRS will present as a triad of telangiectasias (typically smaller than 5 mm), recurrent epistaxis (most common presentation), and a positive family history. Pulmonary AVMs may appear as a solitary or multiple masses with thick, ropelike vessels leading back to the hilum16,17 (Fig 5A and B). Computed tomography is best at demonstrating these lesions. Hepatic AVMs appear as large pools of

FIG 5. Axial (A) and coronal (B) maximum intensity projections from contrast-enhanced CT scans of a 38-year-old woman with Osler-Weber-Rendu syndrome show multiple pulmonary arteriovenous malformations (arrows). (C) Sir William Osler (1849-1919). Images from the History of Medicine (NLM). (D) Frederick Parkes Weber (1863-1962) The library at Wellcome Collection. Parkes Weber Archive, Medical World ca 1913. (E) Henri Jules Louis Marie Rendu (1844-1902) (Image courtesy: http://commons.wikimedia.org/wiki/File:Henri_Rendu.jpg. Accessed January 14, 2014.)

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contrast with intervening enlarged hepatic vessels. Central nervous system AVMs may be shown better on MRI. Oddly enough, OWRS was first described by Sutton in 1864 but his name is not included as part of the eponym. The disease is named after Henri Rendu (1896), Sir William Osler (1901), and Frederick Parkes Weber (1907), who described various aspects of the syndrome in the years cited. Sir William Osler (July 12, 1849 to December 29, 1919) was born in Bond Head, Canada West (now Ontario). Educated at the original Trinity College School in Weston, Ontario, as a teenager Osler's aim was to follow his father into the Anglican ministry, and to that end, he entered Trinity College, Toronto (now part of the University of Toronto), in the autumn of 1867. However, his chief interest proved to be medicine and, forgoing his original intention, he enrolled in the Toronto School of Medicine, a privately owned institution, and not part of the Medical Faculty of the University of Toronto. Osler (Fig 5C) left the Toronto School of Medicine after being accepted to the medical program at McGill University Faculty of Medicine in Montreal. He received his medical degree in 1872. There, he created the first formal journal club. In 1884, he was appointed Chair of Clinical Medicine at the University of Pennsylvania in Philadelphia and, in 1885, was 1 of the 7 founding members of the Association of American Physicians. In addition, he was 1 of the 4 founding professors of Johns Hopkins Hospital. Osler created the first residency program for specialty

training of physicians, and he was the first to bring medical students out of the lecture hall for bedside clinical training. He died at the age of 70 years, on December 29, 1919 in Oxford, England, during the Spanish influenza epidemic.18 Frederick Parkes Weber (May 8, 1863 to June 2, 1962) was born in London, England, in 1863 (Fig 5D). His father, Sir Hermann David Weber (1823-1918), was a personal physician to Queen Victoria. Weber was educated at Charterhouse and Trinity College, Cambridge. He subsequently studied medicine at St Bartholomew's Hospital and abroad at Vienna and Paris. Returning to England, he became House Physician and House Surgeon at St Bartholomew's Hospital. He was subsequently House Physician at Brompton Hospital and Physician at Mount Vernon Hospital. Weber contributed more than 1200 medical articles and wrote 23 books over a period of 50 years. In 1922, he, along with his wife, published a philosophical medical tome called Aspects of Death and Correlated Aspects of Life in Art, Epigram, and Poetry. His final years were clouded by blindness, and he died at 99 years of age in June 1962.19 Henri Jules Louis Marie Rendu (July 24, 1844 to April 16, 1902) was a French physician born to an ancient family of the high French bourgeoisie in Saint-Germain-des Prés (Fig 5E). He initially received an education in sciences at the school of agronomy in Rennes (passing his baccalauréat at the age of 16 years) and first intended to study for a degree in natural history.20 In October 1865, on the

FIG 6. (A) Contrast-enhanced image from coronary CT angiogram shows a fusiform aneurysm of the left anterior descending coronary artery (arrow). (B) V. Kenrick (May 26, 2007). (Image courtesy: Tomisaku Kawasaki. The Japan Times. http://www.japantimes.co.jp/life.)


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advice of his father, Rendu registered in the School of Medicine in Paris, becoming an intern in 1868 at the Hôpital Saint-Antoine. He served as a military surgeon during the Franco-Prussian War, and a few years later worked in the department of Pierre Potain (1825-1901) at the Hôpital Necker in Paris. In 1877, he became médecin des hôpitaux, earning his certification the following year with a dissertation on chronic nephritis called Etude comparative des néphrites chroniques. In 1885, Rendu was appointed head of the department of medicine at Hôpital Necker, a position he maintained for the rest of his career. In 1897, Rendu was elected to the Académie Nationale de Médecine. He was a prolific writer, with many of his medical articles being published in the Bulletin de la Société anatomique de Paris, of which he was editor from 1873-1874. Throughout his career, Rendu held an avid interest in natural sciences and spent considerable time as a botanical collector. This remained true until his death in 1902.20,21

Coronary Vessels Kawasaki Disease KD is an acute, small- to medium-size vessel vasculitis of childhood that predominantly affects the coronary arteries. The etiology of KD remains unknown, although an infectious agent is strongly suspected. It can affect any body organ, but there is a predilection for the coronary vessels (Fig 6A). Children with KD develop coronary artery aneurysms or ectasia, ischemic heart disease, and sudden death. KD is the leading cause of acquired heart disease among children in developed countries.22 Angiography is the most sensitive and specific for vascular assessment. Tomisaku Kawasaki (born February 7, 1925) is a Japanese pediatrician who was born in Tokyo, Japan (Fig 6B). He graduated from Chiba University Medical School. Following his internships, he received an appointment in the pediatrics department at the Red Cross Hospital in Hiroo, Tokyo, where he became director and remained for 40 years. He first observed the condition that now bears his name in 1960 and published the first English-language report of 50 patients with KD in 1974.23

Conclusions There are many eponyms that can be encountered in cardiopulmonary imaging, and this multipart series

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should not be considered comprehensive. However, familiarity with many of these eponyms is important for accurate communication. Although some may argue against their use, eponyms serve as a means of honoring those who have made important discoveries and observations. Acquiring some historical background about these individuals helps to bring some humanity back into the science of medicine.

REFERENCES 1. The American Heritage Stedman's Medical Dictionary. Boston, MA: Houghton Mifflin, 2012. 2. Wang W, Gao L, Wang X. Rasmussen's aneurysm with aspergilloma in old, healed pulmonary tuberculosis. Clin Imaging 2013;37(3):580-2. 3. van den Heuvel MM, van Rensburg JJ. Images in clinical medicine. Rasmussen's aneurysm. N Engl J Med 2006;355 (16):e17. 4. http://www.whonamedit.com/doctor.cfm/2310.html. Accessed January 6, 2014. 5. Seyahi E, Melikoglu M, Akman C, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: A series of 47 patients. Medicine 2012;91(1):35-48. 6. Yilmaz S, Cimen KA. Pulmonary artery aneurysms in Behçet's disease. Rheumatol Int 2010;30(10):1401-3. 7. Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J 1997;38(6):327-32. 8. Waseem M, Ganti S. Chest pain in an adolescent with Marfan syndrome. J Emerg Med 2008;35(4):449-51. 9. Daeubler BF, Carrel T, Kujawski T, et al. Alterations of the thoracic spine in Marfan's syndrome. Am J Roentgenol 2006;186(5):1246-51. 10. Marfan A. Un cas de déformation congénitale des quartre membres, plus prononcée aux extrémitiés, caractérisée par l’allongement des os avec un certain degré d’amincissement. Bull Mem Soc Med Hop Paris 1896;13:220-6. 11. Febrer J. Bernard-Jean Antoine Marfan (1858-1942). Historiadelamedicina.org. Available at: http://historiadelamedicina.org/ marfan.html. 2011. Accessed December 19, 2013. 12. Castañer E, Alguersuari A, Andreu M, et al. Imaging findings in pulmonary vasculitis. Semin Ultrasound CT MR 2012;33 (6):567-79. 13. Papa M, De Cobelli F, Baldissera E, et al. Takayasu arteritis: Intravascular contrast medium for MR angiography in the evaluation of disease activity. Am J Roentgenol 2012;198(3): W279-W284. 14. Sugiyama K, Ijiri S, Tagawa S, et al. Takayasu disease on the centenary of its discovery. Jpn J Ophthalmol 2009;53 (2):81-91. 15. Matsubara J. Mikito Takayasu and Jokichi Takamine, two famous Japanese doctors who were from Kanazawa, Japan. Jpn J Vasc Surg 2003;12:563-9. 16. Narsinh KH, Ramaswamy R, Kinney TB. Management of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia patients. Semin Interv Radiol 2013;30 (4):408-12.


17. Lacombe P, Lacout A, Marcy PY, et al. Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagn Interv Imaging 2013;94(9):835-48. 18. McCall N, editor. The Portrait Collection of Johns Hopkins Medicine: A Catalog of Paintings and Photographs at the Johns Hopkins University School of Medicine and the Johns Hopkins Hospital. Baltimore: The Johns Hopkins University School of Medicine; 1993. 19. Hall LA. A “remarkable collection”; the papers of Frederick Parkes Weber (1863-1962). Med Hist 2001;45:523-32.


20. Henri Rendu (1844-1902). Rendu-Osler-Weber disease. J Am Med Assoc 1966;197(7):583. 21. Hirschberg R. What Benjamin Babington, William Osler, Frederick Weber, and Henri Rendu did not know. Cardiovasc Res 2005;68(2):180-2. 22. Inokuchi R, Kurata H, Harada M, et al. Coronary artery aneurysms after adult-onset Kawasaki disease. Circulation 2013;127(15):1636-7. 23. Kawasaki T. A conversation with Tomisaku Kawasaki, MD. Interviewed by Stanford T. Shulman. Pediatr Ann 2012;41 (5):212.

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Eponyms in cardiothoracic radiology--part II: vascular.

Eponyms serve the purpose of honoring individuals who have made important observations and discoveries. As with other fields of medicine, eponyms are ...
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