The Symmetrical Liver as a Roentgen Sign of Asplenia By RUSSELL V. LUCAS, JR., M.D., HENRY N. NEUFELD, M.D., RICHARD G. LESTER, M.D., AND JESSE E. EDWARDS, M.D. Downloaded from http://circ.ahajournals.org/ by guest on September 30, 2016 THE YOUNG INFANT with cyanotic congenital cardiac disease presents an acute and difficult diagnostic problem. The anomalies responsible for eyanosis in some such infants are now curable surgically while, on the other hand, there remains a group of eyanotic newborn infants in whom the congenital cardiac anomalies are so complex that no surgical procedures for their correction have yet been devised. A method for early separation of these two groups of patients, that is, operable and inoperable, would be of value. Among the so-called inoperable group of patients are individuals with asplenia and congenital cardiac disease, since in these the cardiac anomalies are of such a complex nature that they usually defy surgical correction.' Among a group of cases of asplenia with congenital cardiac diseases the pathologic manifestations include persistent truncus arteriosus, transposition of the great vessels, pulmonary atresia or stenosis, common atrioventricular canal, anomalies of pulmonary veins, and cor biloculare. Moreover, several of these complex malformations usually coexist in the same patient. Visceral symmetry is common also. One step in the direction of separating by clinical means the operable from the inoperable group is to determine whether the spleen is absent. Evidence favoring asplenia includes certain changes in the peripheral blood and findings of visceral symmetry. The hematologic findings present in the peripheral blood in pativnts with asplenia have been well described.2 4 These consist of the presence of Howell-Jolly bodies and Heinz bodies in the erythrocytes as well as numerous erythroblasts. Putschar and Manion5 as well as Ivemark0 noted the general tendency for visceral symmetry as indicated by the frequent occurrence of three lobes in each lung, a large symmetrical liver, wherein both lobes are of about equal size, persistence of the dorsal mesentery to the duodenum and colon, an anatomic twochambered heart, and the absence of the only strictly unilateral mesenchymal organ of the body, namely, the spleen. To our knowledge the organ simplest to identify clinically from the point of view of visceral symmetry is the liver. This is done by roentgenographic examination of the upper abdomen, a procedure that usually is a by-product of the routine posteroanterior roentgenogram of the thorax in the cardiac patient (figs. 1 and 2). In the normal, the lower edge of the liver lies obliquely, as the right lobe is noticeably larger than the left. In hepatic symmetry, as seen in asplenia, the lower edge of the liver lies horizontally. It is the purpose of this communication to call attention to the sign of hepatic symmetry, as a probable sign of asplenia. Addendum Since preparation of this manuscript the sign was also described in the following referenee: Forde, W. J., and Finby, N.: Roentgenograpbic features of asplenia, a teratologic syndrome of visceral symmetry. Am. J. Roentgenol. 86: 523, 1961. From the Departments of Pediatrics, Radiology, and Pathology, University of Minnesota, Minneapolis, Minnesota, and the Department of Pathology, Charles T. Miller Hospital, St. Paul, Minnesota. Supported in part by research grant no. H5694 of the National Heart Institute, United States Public Health Service. Circulation, Volume XXV, June 1962 References 1. LYONS, W. S., HANLON, D. G., HELMHOLZ, H. F., JR., DUSHANE, J. W., AND EDWARDS, J. E.: Cardiac clinics CXLVIII. Congenital cardiac disease and asplenia: Report of seven cases. 973 F Downloaded from http://circ.ahajournals.org/ by guest on September 30, 2016 Figure 1 Posterooanterior roen tgenoygrams frnom three patients wcho manifested neonatal cyanosis an(1d death in early infaincy. In each instaince at necropsy the spleen was absent, the liver wllas neearly symmetrical, and various combiniations of complicated intracardiae inalforornaction s were observed. Upper Left. Patie at with dcxt rocardia. Upp(er Right. Patjent with levocardia. Fullness of the shadow caused bqf the left lobe of the liver is evident in both. Lower. Two illustrations from the same patient. Lower Left. Roentgenogram miade during venous angiocardiography. The lower edge of the lef t lobc of the liver lies prominently at a considerably more inferior level than normal. Lowver Rlight. Film made during intravcnious pyelography in the same patient whose roentgenogram is shown in Lower Left bringing out the features of the symmetrical liver. 974 Circulation, Volume XXV, June 1.962 SYMMETRiCALi LTVER 9 T"' Downloaded from http://circ.ahajournals.org/ by guest on September 30, 2016 rigure 2 Thoracic organs and liver from two eyanotic infants with asplenia and multiple intracardiac anomalies. In each instance, the two lobes of the liver are of about equal size. In the case illustrated in Left the lower edge of the left lobe is slightly superior to that of the right lobe, but is considerably louer than the normal. In the specimen illustrated in Right the reverse regarding the lowcer edge of the liver is trlcn in that the lowver edge of the left lobe is slightly inferior to that on the right stde. In each instance there were three lobes in both lungs. Proc. Staff Meet., Mayo Clin. 32: 277, 1957. 2. WmILi, H., AND GASSER, C.: The cliniical dliagiiosis of the triad spleen agenesis, defects of the lhear t and vessels and situs inver sus. Et. neonatal 4: 25, 1955. 3. Busis, J. A., AND AINaIrc, L. E.: Congernital absenice of the spleen w-ith congenital heart disease: Report of a ca. se -with antemortem diagnosis on the basis of hematologic morphology. Pediatrics 15: 93, 1955. 4. POLHEMUS, D. W., NxD SCHAFER, W. B.: Coln- Circulation, Volume XXV, June 1962 genital absence of the spleeni. Pediatrics 16: 495, 1955. 5. PUTSCHAR, W. G. J., AND MANION, W. C.: Congenital absence of the spleen and associated abnormalities. Am. J. Chini. Path. 26: 429, 1956. 6. IVEMARK, B. I.: Implicationis of agenesis of the spleen oni the pathogeniesis of cono-truneus aIionoalies in childhood: An analvsis of the heart malfornmations in the spleniic agenesis syndrome, with fourteeni niew cases. Acta Paediat. (Uppsala), Suppl. 104, Vol. 44, 1955. The Symmetrical Liver as a Roentgen Sign of Asplenia RUSSELL V. LUCAS, JR., HENRY N. NEUFELD, RICHARD G. LESTER and JESSE E. EDWARDS Downloaded from http://circ.ahajournals.org/ by guest on September 30, 2016 Circulation. 1962;25:973-975 doi: 10.1161/01.CIR.25.6.973 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1962 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/25/6/973.citation Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. 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