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Liver Biopsy Findings in Children with Obliterative Portal Hypertension
Beitr. Path. Bd. 149,307-310 (1973)
Short Communications
Department of Pathology (Director: Prof. Dr. M. EDER) and the Surgical Section of the Department of Pediatrics (Director: Prof. Dr. C. HECKER), University of Munich
Liver Biopsy Findings in Children with Obliterative Portal Hypertension Leberbiopsien bei Kindern mit obliterativer portaler Hypertension P. MEISTER and J. ENGERT With 2 Figures· Received March 28, 1973 . Accepted April 25, 1973
Summary Centrolobular hepatic changes similar to those frequently seen in adults with cardiac congestion (= post hepatic block) were observed in 8 consecutive cases of children with portal vein thrombosis (= prehepatic block), unassociated with hepatic lesions compatible with intrahepatic block or evidence of posthepatic block.
This short communication deals with liver biopsy findings in children with portal hypertension showing only slight hepatic changes which could not serve as explanation for portal hypertension. Within the past 3 years we not only did receive biopsies from patients with intrahepatic block, e. g., with congenital hepatic fibrosis (KERR et aI., 196 I; MACMAHON et aI., 1929), but also 10 needle biopsies from children (age I to I I years) with portal hypertension, bleeding esophageal varices and splenoportographical findings compatible with prehepatic block (ENGERT et aI., in press). 2 of these 10 cases showed congenital hepatic fibrosis. In addition to prehepatic block, an intrahepatic factor for portal hypertension became evident later by splenoportal venography in these 2 cases. Therefore, portal thrombosis could have been secondary. The remaining 8 cases showed neither fibrosis with destruction of hepatic architecture, nor changes of the intrahepatic portal vein branches or signs of
308 .
P.
MEISTER
and
J. ENGERT
~
.
.,....I /,
Fig. 1. Silver impregnation (Gomori) with distinct accentuation of perivenous reticulin frame-work. Note increased density and thickness of fibrils. x 400.
venoocclusive disease as possible morphological correlates of portal hypertension caused by liver disease. However, in all of these 8 cases, sometimes only minimal peculiar centrolobular perivenous changes were found: one case exhibited only slight sinus ectasia and hardly recognizable collagenization, in the remaining cases slight to distinct centrolobular collagenization, usually associated with some fatty change and atrophy of liver cells were seen. The collagen fibers could well be demonstrated by van Gieson stains. However, most impressive were increased numbers and calibers of fibrillary structures after silver impregnation (fig. 1 and 2). These findings reminded of changes otherwise seen with congestion, i. e., posthepatic block (CASTBERG, 1952). However, there was no evidence for cardiac failure and congestion in the children examined (ENGERT et al., in press). Revision of the literature did not yield any larger series of liver biopsy findings in patients with portal vein thrombosis, as usually only with impressive macroscopical liver changes tissue is obtained for microscopical examination (MIKKELSEN, 1966; VOORHEES et al., 1964). BAGGENSTOSS and WOLLAEGER (1956) reported an autopsy series of 15 adults with prehepatic block showing centrolobular fibrosis, comparable to our findings, and additional slight portal inflammatory reaction with fibrosis.
Liver in Portal Block . 309
r>:. ..
.'
:i"..,. 1.1.t .. ~, J
). '•
~ ".. f
..... ,\ Fig. 1. Survey micrograph showing prominence of perivenous fibrils in central location. x 160.
These observations bring up the question how strongly liver changes, especially centro lobular ectasia and sinusoidal fibrosis, can suggest prehepatic or portal block in the absence of clinical signs of congestion and posthepatic block. It seems possible that the centrolobular changes are a consequence of the disturbance of portal circulation with diminution of volume and velocity of intra sinusoidal blood. Long-term studies are needed to clearly separate late changes after primary portal vein thrombosis with secondary hepatic fibrosis and obliteration of intrahepatic portal vein from primary, i. e., congenital hepatic fibrosis with hypoplasia of intrahepatic portal vein branches and secondary portal vein thrombosis after portal hypertension. In the latter case additional malformation of extrahepatic portal veins also has to be considered. It ist the purpose of this communication to draw attention to histological liver changes in patients with prehepatic block, e. g., portal vein thrombosis and hypertension. To elucidate the significance of these liver changes, larger numbers of cases are to be studied. 21 Beitr. Path. Bd. 149
31o . P. MEISTER and]. ENGERT
Bibliography BAGGENSTOSS, A. and WOLLAEGER, E.: Amer.]. Med. 21,16 (1956) CASTBERG, T.: Acta path. microbiol. scand. 30, 358 (1952) ENGERT, J., MEISTER, P., HECKER, W., and STRASSBURG, R.: Z. Kinderchirurgie (im Druck) KERR, D., HARRISON, c., SHERLOCK, S., and WALKER, R.: Quart.]' Med. n. s. 30, 91 (1961) MACMAHON, H.: Amer. Arch. Path. 5,499 (1929) MIKKELSEN, W.: Amer. ]. Surg. 1I I , 333 (1966) VOORHEES, A., HARRIS, R., BRITTON, R., PRICE, ]., and SANTULLI, T.: Ped. Surg. 58, 540 (19 64) Dozent Dr. PETER MEISTER, Pathologisches Institut, D - 8 Miinchen
2, Thalkirchner
Str. 36
Short Communications
Department of Pathology (Director: Prof. Dr. M. EDER) and the Surgical Section of the Department of Pediatrics (Director: Prof. Dr. C. HECKER), University of Munich
Liver Biopsy Findings in Children with Obliterative Portal Hypertension Leberbiopsien bei Kindern mit obliterativer portaler Hypertension P. MEISTER and J. ENGERT With 2 Figures· Received March 28, 1973 . Accepted April 25, 1973
Summary Centrolobular hepatic changes similar to those frequently seen in adults with cardiac congestion (= post hepatic block) were observed in 8 consecutive cases of children with portal vein thrombosis (= prehepatic block), unassociated with hepatic lesions compatible with intrahepatic block or evidence of posthepatic block.
This short communication deals with liver biopsy findings in children with portal hypertension showing only slight hepatic changes which could not serve as explanation for portal hypertension. Within the past 3 years we not only did receive biopsies from patients with intrahepatic block, e. g., with congenital hepatic fibrosis (KERR et aI., 196 I; MACMAHON et aI., 1929), but also 10 needle biopsies from children (age I to I I years) with portal hypertension, bleeding esophageal varices and splenoportographical findings compatible with prehepatic block (ENGERT et aI., in press). 2 of these 10 cases showed congenital hepatic fibrosis. In addition to prehepatic block, an intrahepatic factor for portal hypertension became evident later by splenoportal venography in these 2 cases. Therefore, portal thrombosis could have been secondary. The remaining 8 cases showed neither fibrosis with destruction of hepatic architecture, nor changes of the intrahepatic portal vein branches or signs of
308 .
P.
MEISTER
and
J. ENGERT
~
.
.,....I /,
Fig. 1. Silver impregnation (Gomori) with distinct accentuation of perivenous reticulin frame-work. Note increased density and thickness of fibrils. x 400.
venoocclusive disease as possible morphological correlates of portal hypertension caused by liver disease. However, in all of these 8 cases, sometimes only minimal peculiar centrolobular perivenous changes were found: one case exhibited only slight sinus ectasia and hardly recognizable collagenization, in the remaining cases slight to distinct centrolobular collagenization, usually associated with some fatty change and atrophy of liver cells were seen. The collagen fibers could well be demonstrated by van Gieson stains. However, most impressive were increased numbers and calibers of fibrillary structures after silver impregnation (fig. 1 and 2). These findings reminded of changes otherwise seen with congestion, i. e., posthepatic block (CASTBERG, 1952). However, there was no evidence for cardiac failure and congestion in the children examined (ENGERT et al., in press). Revision of the literature did not yield any larger series of liver biopsy findings in patients with portal vein thrombosis, as usually only with impressive macroscopical liver changes tissue is obtained for microscopical examination (MIKKELSEN, 1966; VOORHEES et al., 1964). BAGGENSTOSS and WOLLAEGER (1956) reported an autopsy series of 15 adults with prehepatic block showing centrolobular fibrosis, comparable to our findings, and additional slight portal inflammatory reaction with fibrosis.
Liver in Portal Block . 309
r>:. ..
.'
:i"..,. 1.1.t .. ~, J
). '•
~ ".. f
..... ,\ Fig. 1. Survey micrograph showing prominence of perivenous fibrils in central location. x 160.
These observations bring up the question how strongly liver changes, especially centro lobular ectasia and sinusoidal fibrosis, can suggest prehepatic or portal block in the absence of clinical signs of congestion and posthepatic block. It seems possible that the centrolobular changes are a consequence of the disturbance of portal circulation with diminution of volume and velocity of intra sinusoidal blood. Long-term studies are needed to clearly separate late changes after primary portal vein thrombosis with secondary hepatic fibrosis and obliteration of intrahepatic portal vein from primary, i. e., congenital hepatic fibrosis with hypoplasia of intrahepatic portal vein branches and secondary portal vein thrombosis after portal hypertension. In the latter case additional malformation of extrahepatic portal veins also has to be considered. It ist the purpose of this communication to draw attention to histological liver changes in patients with prehepatic block, e. g., portal vein thrombosis and hypertension. To elucidate the significance of these liver changes, larger numbers of cases are to be studied. 21 Beitr. Path. Bd. 149
31o . P. MEISTER and]. ENGERT
Bibliography BAGGENSTOSS, A. and WOLLAEGER, E.: Amer.]. Med. 21,16 (1956) CASTBERG, T.: Acta path. microbiol. scand. 30, 358 (1952) ENGERT, J., MEISTER, P., HECKER, W., and STRASSBURG, R.: Z. Kinderchirurgie (im Druck) KERR, D., HARRISON, c., SHERLOCK, S., and WALKER, R.: Quart.]' Med. n. s. 30, 91 (1961) MACMAHON, H.: Amer. Arch. Path. 5,499 (1929) MIKKELSEN, W.: Amer. ]. Surg. 1I I , 333 (1966) VOORHEES, A., HARRIS, R., BRITTON, R., PRICE, ]., and SANTULLI, T.: Ped. Surg. 58, 540 (19 64) Dozent Dr. PETER MEISTER, Pathologisches Institut, D - 8 Miinchen
2, Thalkirchner
Str. 36