ANEURYSMAL DILATATION OF AORTIC SINUSES IN ARACHNODACTYLY: DIAGNOSIS DURING LIFE IN THREE CASES

Abstract

Article1 July 1955ANEURYSMAL DILATATION OF AORTIC SINUSES IN ARACHNODACTYLY: DIAGNOSIS DURING LIFE IN THREE CASESISRAEL STEINBERG, M.D., F.A.C.P., WILLIAM GELLER, M.D.ISRAEL STEINBERG, M.D., F.A.C.P., WILLIAM GELLER, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-43-1-120 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptArachnodactyly (Marfan's syndrome) is a rare hereditary disease of unknown etiology. It is characterized by widespread malformations of the skeletal, cardiovascular and ophthalmic systems. Marfan in 18961 described the first case and called attention to the long, thin, spider-like appearance of the extended extremities. Soon arachnodactyly2 became established as the name of the disorder.The association of cardiovascular anomalies with arachnodactyly became evident with complete autopsies. Forty-one necropsies of Marfan's disease have been reported; eight were under 28 months of age and had no aortic lesions; the remaining 33 were adults (14 to 55 years); all had lesions of the...Bibliography1. Marfan AB: Un cas de déformation congénitale des quatre membres, plus prononcée aux extremités, characterisée par l'allongement des os avec un certain degré d'amincissement, Bull. et mém. Soc. méd. d. hôp. de Paris 13: 220-226, 1896. Google Scholar2. Archard MC: Arachnodactylie, Bull. et mém. Soc. med. d. hôp. de Paris 19: 834-840, 1902. Google Scholar3. BaerTaussigOppenheimer RWHBEH: Congenital aneurysmal dilatation of the aorta with arachnodactyly, Bull. Johns Hopkins Hosp. 72: 309-317, 1943. Google Scholar4. EtterGlover LELP: Arachnodactyly complicated by dislocated lens and death from rupture of dissecting aneurysm, J. A. M. A. 123: 88-89, 1943. CrossrefGoogle Scholar5. TobinBayHumphreys JREBEM: Marfan's syndrome in the adult (dissecting aneurysm of the aorta associated with arachnodactyly), Arch. Int. Med. 80: 475-490, 1946. CrossrefGoogle Scholar6. UyeyamaKondoKamins HBM: Arachnodactyly and cardiovascular disease: report of an autopsied case with a summary of previously autopsied cases, Am. Heart J. 34: 580-591, 1947. CrossrefMedlineGoogle Scholar7. ThomasBrothersAndersonCuff JTMORSJR: Marfan's syndrome: a report of three cases with aneurysm of the aorta, Am. J. Med. 12: 613-618, 1952. CrossrefMedlineGoogle Scholar8. FischlRuthberg AAJ: Clinical implications of Marfan's syndrome, J. A. M. A. 146: 704-707, 1951. CrossrefMedlineGoogle Scholar9. MarvelGenovese RJPD: Cardiovascular disease in Marfan's syndrome, Am. Heart J. 42: 814-825, 1951. CrossrefMedlineGoogle Scholar10. Moses MF: Aortic lesions associated with arachnodactyly, Brit. M. J. 2: 81-84, 1951. CrossrefMedlineGoogle Scholar11. Gore I: Dissecting aneurysms of the aorta in persons under forty years of age, Arch. Path. 55: 1-13, 1953. Google Scholar12. GoyettePalmer EMPW: Cardiovascular lesions in arachnodactyly, Circulation 7: 373-379, 1953. CrossrefMedlineGoogle Scholar13. Pygott F: Arachnodactyly (Marfan's syndrome) with a report of two cases, Brit. J. Radiol. 28: 26-29, 1955. CrossrefMedlineGoogle Scholar14. SloperStorey JCG: Aneurysms of the ascending aorta due to medial degeneration associated with arachnodactyly (Marfan's disease), J. Clin. Path. 6: 299-303, 1953. CrossrefMedlineGoogle Scholar15. AndersonPratt-Thomas MHR: Marfan's syndrome, Am. Heart J. 46: 911-917, 1953. CrossrefMedlineGoogle Scholar16. Lillian M: Multiple pulmonary artery aneurysms, Am. J. Med. 7: 280-287, 1949. CrossrefMedlineGoogle Scholar17. Weill G: Ectopie des cristallins and malformations generales, Ann. d'ocul. 169: 21, 1932. Google Scholar18. PonsetiShepard IVRS: Lesions of the skeleton and of other mesodermal tissues in rats fed sweet-pea (Lathyrus odoratus) seeds, J. Bone and Joint Surg. 36-A: 1031-1058, 1954. CrossrefGoogle Scholar19. Rados A: Marfan's syndrome (arachnodactyly coupled with dislocations of the lens), Arch. Ophth. 27: 477-538, 1942. CrossrefGoogle Scholar20. SchorrBraunWildman SKJ: Congenital aneurysmal dilatation of the ascending aorta associated with arachnodactyly: an angiocardiographic study, Am. Heart J. 42: 610-616, 1951. CrossrefMedlineGoogle Scholar21. Morgan JonesLangley AFA: Aortic sinus aneurysm, Brit. Heart J. 11: 325-341, 1949. CrossrefMedlineGoogle Scholar22. Micks RH: Congenital aneurysms of all three sinuses of Valsalva, Brit. Heart J. 2: 63-78, 1940. CrossrefMedlineGoogle Scholar23. FalholtThomsen WG: Congenital aneurysm of the right sinus of Valsalva: diagnosed by aortography, Circulation 8: 549-553, 1953. CrossrefMedlineGoogle Scholar24. RobbSteinberg GPI: Visualization of the chambers of the heart, the pulmonary circulation, and the great blood vessels in man: a practical method, Am. J. Roentgenol. 41: 1-14, 1939. Google Scholar25. SteinbergDotterPeabodyReaderHeimoffWebster ICTGEGGLB: The angiocardiographic diagnosis of syphilitic aortitis, Am. J. Roentgenol. 62: 655-660, 1949. Google Scholar26. PeabodyReaderDotterSteinbergWebster GEGGCTIB: Angiocardiography in the diagnosis of cardiovascular syphilis, Am. J. M. Sc. 219: 242-248, 1950. CrossrefMedlineGoogle Scholar27. DotterSteinberg CTI: Angiocardiography, 1951, Paul B. Hoeber, Inc., New York. Google Scholar28. DubilierTaylorSteinberg WHTLI: Aortic sinus aneurysm associated with coarctation of the aorta, Am. J. Roentgenol. 73: 10-14, 1955. Google Scholar29. Taussig HB: Congenital malformations of the heart, 1947, The Commonwealth Fund, New York. Google Scholar30. Brown JW: Congenital heart disease, 2nd Ed., 1950, Staples Press, London and New York. Google Scholar31. McKusick VA: The cardiovascular aspects of Marfan's syndrome: a heritable disorder of connective tissue, Circulation 11: 321-342, 1955. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New York, N. Y.*Received for publication February 7, 1955.From the Departments of Medicine and Radiology, The New York Hospital-Cornell Medical Center.Aided by a grant from the Mallinckrodt Chemical Works. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byRuptured sinus of valsalva aneurysm after lung resectionThe Marfan syndrome: Cardiovascular physical findings and diagnostic correlatesHeritable Disorders of Connective TissueAnasthesia in Marfan's syndromeAnasthesia in Marfan's syndromeRepair of Sinus of Valsalva Aneurysm Associated with Aortoventricular DiscontinuityComparison of echocardiography and radiology in the diagnosis of aortic root dilatation in Marfan's syndrome and in syphilis.Echocardiographic Recognition of Silent Aortic Root Dilatation in Marfan's SyndromeKardiovascul�re Manifestationsformen des Marfan SyndromsMarfan's syndrome: A reviewAortic regurgitation and aneurysm of sinus of Valsalva associated with osteogenesis imperfectaSinus of Valsalva AbnormalitiesAortic Sinus Aneurysm:Report of an Unusual CaseAneurysm of the aortic sinus of valsalva communicating with a fistulous cavity in the interventricular septum: Report of a caseAortic root aneurysm—Radiographic and pathologic featuresCongenital arterial diseasesAneurysms of two sinuses of Valsalva with a ventricular septal defect and aortic regurgitation.Surgical correction of aneurysm of the sinus of ValsalvaDarstellung der Herzhöhlen, der Gefäßlumina und des BlutstromesIatrogenic aortico—right ventricular shuntDystrophia mesodermalis congenita (Typ Marfan), Arachnodaktylie, "Marfan-Syndrom"Angeborene Herz- und GefäßfehlerCardiovascular SystemCongenital aneurysms of all three sinuses of Valsalva with rupture in an asymptomatic patientUnusual manifestations of ruptured aneurysm of the aortic sinusKurzschlußverbindungen des großen und kleinen KreislaufsOral manifestations of Marfan's syndrome in twinsCongenital aneurysms of all three sinuses of ValsalvaCine-aortographic Evaluation of Aortic Insufficiency Unsuspected Idiopathic Aneurysmal Dilatation of the Aortic Root as a Possible Indication of the Marfan SyndromeJOHN S. BARRETT, M.D., JOHN HELWIG JR., M.D., F.A.C.P., CALVIN F. KAY, M.D., F.A.C.P., JULIAN JOHNSON, M.D., F.A.C.S.DISSECTING ANEURYSM OF AORTA ASSOCIATED WITH TURNER'S SYNDROMECongenital Aneurysm of Aortic Root with Fistula to Left VentricleRupture of a sinus of Valsalva aneurysm in an infantThe Marfan SyndromeCongenital aneurysm of the sinus of Valsalva Anatomy and classificationSome Unusual Features of the Marfan SyndromeRuptured aneurysms of the aortic sinuses of ValsalvaSUBSIDENCE OF DILATATION OF ASCENDING AORTA AND AORTIC SINUS AFTER RESECTION OF COARCTATIONCongenital Aneurysms of the Aortic Sinuses with Cardioaortic Fistula*Angeborene Herz- und GefäßmißbildungenKrankheiten der GefäßeKrankheiten der GefäßeMarfan's syndromeRupture of an aneurysm of the posterior sinus of valsalva into the right atriumMedionecrosis of the Aorta in Marfan's Syndrome.Das "Marfan-Syndrom"The syndrome of ventricular septal defect with aortic insufficiency∗ANEURYSMAL DILATATION OF THE AORTIC SINUSES IN COARCTATION OF THE AORTA: REPORT OF TWO NEW CASES AND REVIEW OF THE LITERATURE*†ISRAEL STEINBERG, M.D., F.A.C.P., B. P. SAMMONSMarfan's syndrome associated with pregnancyUnruptured congenital aortic sinus aneurysm with aneurysmal dilatation of the aorta and aortic regurgitation without arachnodactylySEVERE AORTIC INSUFFICIENCY IN MARFAN'S SYNDROME*JOHN F. GRIFFIN, M.D., GERALD M. KOMAN, M.D.Marfan's syndrome: Description of a familyAneurysmal Dilatation of the Aortic Sinuses in Marfan's SyndromeCONGENITAL ANEURYSM OF THE NONCORONARY SINUS OF VALSALVA LEADING TO COMPLETE HEART BLOCK: CASE REPORT*EDMON B. LEE, M.D., OSCAR J. KRIEGER, M.D., NORMAN K. LEE, M.D.The antemortem diagnosis of syphilitic aneurysm of the aortic sinusesANEURYSM OF THE AORTIC SINUSES WITH PSEUDOCOARCTATION OF THE AORTACurrent pediatric referencesCongenital Aneurysm of the Right Aortic Sinus Associated with Coarctation of the Aorta and Subacute Bacterial Endocarditis 1 July 1955Volume 43, Issue 1Page: 120-132KeywordsAortaAutopsyCardiovascular diseasesDiagnostic radiologyEtiologyGenetic diseasesLesionsMarfan syndromeVision ePublished: 1 December 2008 Issue Published: 1 July 1955 PDF downloadLoading ...