Gastrointest Radiol 16: 85-86 (1991)
Gastrointestinal
Radiology 9 Springer-VerlagNewYorkInc.1991
Letter to the Editor
Comments About Glisson's Capsule Phleboliths and Portal Vein Thrombosis In a recent issue of this Journal, we described Glisson's capsule pheboliths resulting in "porcelain liver" appearance in a 29-year-old woman, diagnosed as having portal vein obstruction at age 3 [1]. In this patient, we suggested that the hemodynamic changes were responsible for hepatic siderosis leading to cirrhosis [2, 3]. However, we were unable to determine if liver capsular phleboliths and portal thrombosis and/or hypertension were really asso.ciated or coincidental [1]. We have recently reviewed three personal cases of portal venous system calcifications, as well as the 123 previously reported ones [4]. This condition is associated with portal vein thrombosis in 65% of cases, and with portal hypertension in all the patients [4]. The calcifications, affecting thrombus and/or venous wall, involve preferentially the portal trunk but also may affect, in association or not, many tributaries as splenic, superior mesenteric, inferior mesenteric, coronary, pancreaticoduodenal, umbilical and appendicular veins, intrahepatic and intrasplenic venous branches, and ductus venosus [4]. Glisson's capsule veins are tributaries of the portal vein system, and in this respect, liver capsular phleboliths may represent a variant of portal venous system calcifications in our patient who presented with portal hypertension and thrombosis. Moreover, peripheral liver calcifications have been described in selected cases of infantile portal vein thromboembolism. In a neonate, who died at 26 h of age, autopsy revealed hepatic subcapsular venous wall calcifications [5]. In a 4month-old girl presenting with a Pierre Robin syndrome and arthroglyposis multiplex congenita, who died by asphyxia, abdominal plain film revealed hepatic calcifications [6]. Autopsy findings were compatible with phleboliths in the subcapsular veins. In 21 autopsied cases of calcified portal vein thromboemboli in new born and stillborn in-
rants, abdominal films demonstrated, in two cases, zig-zag or linear densities in an intrahepatic location [7]. At autopsy, the calcified venous thrombi were usually subcapsular and appeared as nodules or branching lines frequently running to the liver capsule. Friedman et al. reported six other infantile cases in which calcic density overlying the liver was an incidental observation on abdominal radiographs [8]. All lesions appeared to be peripheral. US studies performed in one case localized the calcification in the periphery of the liver. Autopsy performed in another case, revealed a calcification on the hepatic surface associated with the venous wall and thrombus calcifications in a main branch of the portal vein. Recently, in a neonate, who died at 36 h of age of hyaline membrane disease, abdominal radiography showed linear and hieroglyphic-like calcifications outlining the hepatic right lobe [9]. Postmortem liver radiographs provided further evidence of the peripheral location of the calcifications. Histologically, totally calcified thrombi into the subcapsular and the interlobular branches of the portal vein were demonstrated. Furthermore, in all these patients in which an abdominal plain film was done, radiological findings were grossly similar to those observed in our reported case. Finally, all these considerations support that Glisson's capsule phleboliths in our patient, who was diagnosed as having portal vein obstruction at age 3, were due to calcified portal vein thromboembolism. This hitherto undescribed condition seems to realize the ultimate variant, one in which calcifications are located in the intrahepatic portal vein extremities. Similar findings affecting the spleen have been described in an alcoholic cirrhotic patient presenting with portal, splenic, and intrasplenic veins calcifications associated with splenic capsule curvilinear calcifications on plain film and
86
Letter to the Editor
C T s t u d i e s [10]. T h e a u t h o r s p o s t u l a t e d t h a t s p l e n i c c a p s u l a r c a l c i f i c a t i o n s p r o b a b l y r e s u l t e d in p o r t a l h y p e r t e n s i o n . T h e f i n d i n g s in o u r p a t i e n t a p p e a r t o a g r e e w i t h t h i s o p i n i o n o n this " p o r c e l a i n s p l e e n " a p p e a r a n c e , a c o n d i t i o n a l r e a d y rep o r t e d in 1848, in a n o t h e r a u t o p s i e d p a t i e n t p r e s e n t i n g w i t h p o r t a l v e i n c a l c i f i c a t i o n s [11]. Jacques Delamarre, M.D. Vbronique Fabre, M.D. Henri Sevestre, M.D. Catherine Jouet-Gondry, M.D. Brigitte Deschepper, M.D. Jean-Pierre Capron, M.D. Service de Radiologie A CHU-Hopital Nord Amiens, France
References 1. Delamarre J, Capron JP, Sevestre H, Davion T, Deschepper B, Jouet-Gondry C. "Porcelain liver" appearance due t o Glisson's capsule phleboliths. Gastrointest Radiol 1989; 14: 339-340 2. Capron JP, R6mond A, Dupas JL, Mancheron H, CapronChivrac D, Braillon A. Hepatic siderosis in extrahepatic
portal vein obstruction. Role of spontaneous portosystemic shunting? Dig Dis Sci 1986; 31:889-892 3. Capron JP, Sevestre H, Delamarre J. Long-term follow up of hepatic siderosis associated with spontaneous portosystemic shunting [Letter]. Dig Dis Sci 1988; 34:804-805 4. Delamarre J, Fabre V, R6mond A, S6venet F, Tossou H, Capron JP. Calcifications du syst+me porte. Etude de 3 cas et revue de la litt6rature. Gastroenterol Clin Biol (in press) 5. Rossier A, Caldera R, Sarrut S. Les calcifications intrah6patiques chez le nouveau-n6. Cab Coll Med H6p Paris 1962; 10:717-725 6. Cinque NA, Ena J. Un taro caso di sindrome di Pierre Robin, con artrogriposi congenita e calcificazioni intraepatiche. Minerva Pediatr 1965; 17:1809-1814 7. Blanc WA, Berdon WE, Baker DH, Wigger HJ. Calcified portal vein thromboemboli in newborn and stillborn infants. Radiology 1967; 88:287-292 8. Friedman AP, Haller JO, Boyer B, Cooper R. Calcified portal vein thromboemboli in infants: radiography and ultrasonology. Radiology 1981 ; 140: 381-382 9. de Filippi G, Betta PG. Vascular liver calcifications in infants. A case report with some pathogenetic considerations. Pediatr Radiol 1988; 18:413-415 10. Hadar H, Sommer R. Calcification in the portal venous system demonstrated by computed tomography. Eur J Radiol 1983; 3:187-188 11. Raikem M. Observations, r6flexions et apergus sur quelques affections morbides de la veine porte et notamment sur l'intlammation, l'oblit~ration et l'ossification de ce tronc vasculaire. Mem Ac Roy Med Belg 1848; 1 : 38-63
Gastrointestinal
Radiology 9 Springer-VerlagNewYorkInc.1991
Letter to the Editor
Comments About Glisson's Capsule Phleboliths and Portal Vein Thrombosis In a recent issue of this Journal, we described Glisson's capsule pheboliths resulting in "porcelain liver" appearance in a 29-year-old woman, diagnosed as having portal vein obstruction at age 3 [1]. In this patient, we suggested that the hemodynamic changes were responsible for hepatic siderosis leading to cirrhosis [2, 3]. However, we were unable to determine if liver capsular phleboliths and portal thrombosis and/or hypertension were really asso.ciated or coincidental [1]. We have recently reviewed three personal cases of portal venous system calcifications, as well as the 123 previously reported ones [4]. This condition is associated with portal vein thrombosis in 65% of cases, and with portal hypertension in all the patients [4]. The calcifications, affecting thrombus and/or venous wall, involve preferentially the portal trunk but also may affect, in association or not, many tributaries as splenic, superior mesenteric, inferior mesenteric, coronary, pancreaticoduodenal, umbilical and appendicular veins, intrahepatic and intrasplenic venous branches, and ductus venosus [4]. Glisson's capsule veins are tributaries of the portal vein system, and in this respect, liver capsular phleboliths may represent a variant of portal venous system calcifications in our patient who presented with portal hypertension and thrombosis. Moreover, peripheral liver calcifications have been described in selected cases of infantile portal vein thromboembolism. In a neonate, who died at 26 h of age, autopsy revealed hepatic subcapsular venous wall calcifications [5]. In a 4month-old girl presenting with a Pierre Robin syndrome and arthroglyposis multiplex congenita, who died by asphyxia, abdominal plain film revealed hepatic calcifications [6]. Autopsy findings were compatible with phleboliths in the subcapsular veins. In 21 autopsied cases of calcified portal vein thromboemboli in new born and stillborn in-
rants, abdominal films demonstrated, in two cases, zig-zag or linear densities in an intrahepatic location [7]. At autopsy, the calcified venous thrombi were usually subcapsular and appeared as nodules or branching lines frequently running to the liver capsule. Friedman et al. reported six other infantile cases in which calcic density overlying the liver was an incidental observation on abdominal radiographs [8]. All lesions appeared to be peripheral. US studies performed in one case localized the calcification in the periphery of the liver. Autopsy performed in another case, revealed a calcification on the hepatic surface associated with the venous wall and thrombus calcifications in a main branch of the portal vein. Recently, in a neonate, who died at 36 h of age of hyaline membrane disease, abdominal radiography showed linear and hieroglyphic-like calcifications outlining the hepatic right lobe [9]. Postmortem liver radiographs provided further evidence of the peripheral location of the calcifications. Histologically, totally calcified thrombi into the subcapsular and the interlobular branches of the portal vein were demonstrated. Furthermore, in all these patients in which an abdominal plain film was done, radiological findings were grossly similar to those observed in our reported case. Finally, all these considerations support that Glisson's capsule phleboliths in our patient, who was diagnosed as having portal vein obstruction at age 3, were due to calcified portal vein thromboembolism. This hitherto undescribed condition seems to realize the ultimate variant, one in which calcifications are located in the intrahepatic portal vein extremities. Similar findings affecting the spleen have been described in an alcoholic cirrhotic patient presenting with portal, splenic, and intrasplenic veins calcifications associated with splenic capsule curvilinear calcifications on plain film and
86
Letter to the Editor
C T s t u d i e s [10]. T h e a u t h o r s p o s t u l a t e d t h a t s p l e n i c c a p s u l a r c a l c i f i c a t i o n s p r o b a b l y r e s u l t e d in p o r t a l h y p e r t e n s i o n . T h e f i n d i n g s in o u r p a t i e n t a p p e a r t o a g r e e w i t h t h i s o p i n i o n o n this " p o r c e l a i n s p l e e n " a p p e a r a n c e , a c o n d i t i o n a l r e a d y rep o r t e d in 1848, in a n o t h e r a u t o p s i e d p a t i e n t p r e s e n t i n g w i t h p o r t a l v e i n c a l c i f i c a t i o n s [11]. Jacques Delamarre, M.D. Vbronique Fabre, M.D. Henri Sevestre, M.D. Catherine Jouet-Gondry, M.D. Brigitte Deschepper, M.D. Jean-Pierre Capron, M.D. Service de Radiologie A CHU-Hopital Nord Amiens, France
References 1. Delamarre J, Capron JP, Sevestre H, Davion T, Deschepper B, Jouet-Gondry C. "Porcelain liver" appearance due t o Glisson's capsule phleboliths. Gastrointest Radiol 1989; 14: 339-340 2. Capron JP, R6mond A, Dupas JL, Mancheron H, CapronChivrac D, Braillon A. Hepatic siderosis in extrahepatic
portal vein obstruction. Role of spontaneous portosystemic shunting? Dig Dis Sci 1986; 31:889-892 3. Capron JP, Sevestre H, Delamarre J. Long-term follow up of hepatic siderosis associated with spontaneous portosystemic shunting [Letter]. Dig Dis Sci 1988; 34:804-805 4. Delamarre J, Fabre V, R6mond A, S6venet F, Tossou H, Capron JP. Calcifications du syst+me porte. Etude de 3 cas et revue de la litt6rature. Gastroenterol Clin Biol (in press) 5. Rossier A, Caldera R, Sarrut S. Les calcifications intrah6patiques chez le nouveau-n6. Cab Coll Med H6p Paris 1962; 10:717-725 6. Cinque NA, Ena J. Un taro caso di sindrome di Pierre Robin, con artrogriposi congenita e calcificazioni intraepatiche. Minerva Pediatr 1965; 17:1809-1814 7. Blanc WA, Berdon WE, Baker DH, Wigger HJ. Calcified portal vein thromboemboli in newborn and stillborn infants. Radiology 1967; 88:287-292 8. Friedman AP, Haller JO, Boyer B, Cooper R. Calcified portal vein thromboemboli in infants: radiography and ultrasonology. Radiology 1981 ; 140: 381-382 9. de Filippi G, Betta PG. Vascular liver calcifications in infants. A case report with some pathogenetic considerations. Pediatr Radiol 1988; 18:413-415 10. Hadar H, Sommer R. Calcification in the portal venous system demonstrated by computed tomography. Eur J Radiol 1983; 3:187-188 11. Raikem M. Observations, r6flexions et apergus sur quelques affections morbides de la veine porte et notamment sur l'intlammation, l'oblit~ration et l'ossification de ce tronc vasculaire. Mem Ac Roy Med Belg 1848; 1 : 38-63
