CASE REPORTS Extrahepatic Portal Hypertension Due
to Congenital Obstruction of the Portal Vein and Associated
Gross Hepatic Lobulation Akinsanya O. Obisesan,
Abiodun O. Johnson, M.D., MRCP,DCH,*
M.B.,
A. Olufemi Williams, M.D.,
DMRD, FRCR. FRCP, MRCPath†
A 10-week old girl presented with splenomegaly and recurrent hematemesis from esophageal varices. Splenoportography revealed a dilated extrahepatic portion of the portal vein with nonvisualization of its intrahepatic tributaries. The child died following an episode of hematemesis and was found to have a dilated portal vein which ended blindly. In addition, there was abnormal lobulation of the inferior surface of the liver which was not cirrhotic. The portal vascular anomaly, which presumably was responsible for the portal hypertension, was probably due to failure of communication between the embryonic vitelline veins or to atresia of the portal vein secondary to pressure from the abnormal hepatic lobulation in utero. It would appear that congenital factors may be significant in the etiology and pathogenesis of some cases of extrahepatic portal hypertension in early life and recognition of such developmental anomalies is of importance in management.
I N MANY tNSTANCES, the cause of extrahc-. patic portal hypertension in cht!dhood remains tzt~s~ut‘~.’ Neonatal omplialitis, perittMtitis, infection
~:c~ll~~~ ~~~~a~t~~l, ~’~~4~~~~~~. Nifreria with the complaint that the child had vomited, approximately
with dehydration and umbilical vein catheterizatiori arc acquired factors which, are widely believed to predispose, to thrombosis ~~’ ~tz~ poi-tal vein or its tnbutanes,~!tim.at€!y resulting ~zz a raised portal venous pressure. However, only in the. minority of cases is the exact, cause o~cxtrahepa.Ht: portal ~y_ ’..’’.’ pertension i(lentified.2 .. ’ ’ Extrahepatic portal hypertension may also b~ c~n~enit~~~, r~sz~~tizz~ f’r~z~z failure of anastot-n6sis of the vitelline veins in the embryo,3 or atresia of ’ some ~~.rt z~f‘ t~~ portal ’venous’ system,-’ but doctimented instances of* these developinentat anomalies B’ .~. B’/:~ ~ .~’’~.’ are relatively rare. This is a report of the unusual ending of portal vein, occlusion and gross hepatic lobulation ~n a
.
..
..
50 mt of* red blood that aftcrntxt’n. No. blood had been z~~~r~~~.*c~’i~a ~h~ s~c~c~~, ~~~~’~v~~ ~~c~r~rz at ta~~zr~~.~ after.a’M! term uneventful pregnancy- There was no.’history ..d’ nponat.ai jaundice-or infection. Since birth, the child had t.hrivect. C:3r~ ~c~t~~~~~a~~~~, t~~~ c~a~~~ was afebriie’ and amctfnc. She was pale but well nourished, weighing 4.5 kg. Her artery pulse’was &dquo;t58/mmute and blood pressure was 90~55 mm Hg. The sp!€cn, enlarged to 6 cni below the left costal .’ ~~~x~ir~ ~~c~~~ t~~ ~:nt~ric~~ a~ci~l;~~-y li~~, was firm -but/not tender. The liver was’just palpable and There was no ascites. Investigations revealed that.
her~~~c~~r~~ was, ~t~~~~ WBC- l4i3OO/#l (neutrophils
13%, lyi-nphocvtes 81%, monocytes 6%). HemoIglo4in electropboresis w~..AA~’phte!€’t count,, clotting and bleeding t~~~~, ~~~~n ’eteetro!yte’s,~Mood.’
’.&dquo; ;.
~’
~ ~’~~rz~orz~~=cz~~ ~~il~ w~~ ~~r~~~z~t~~ ~i~h ~’~~tzares ~, urea: and liver function tests were all within normal ~ ’’ ’ limits. Stool , examination ’revealed presence of ’of’extrahepatic’ port,41 hypertension. ~~~~~~~t blood. The child. ..was. thought to have ../: portal hypertension probably secondary to neo~ & d q u o ; ~’’’ Ca~:~$p~~’:.;.,&dquo;:;~~~ ,:; ’~Jr~:~./’’./~;~:~:. ’~’~.~~.:.’~ ~~~~ B ;~.~.’:&dquo; ~’&dquo;’;/&dquo;~.&dquo; .f~~~’’ ~.’ /~ ’ ’~ ~:~~~’ ~~~~~~~~~~* . &dquo;
’
’’
’&dquo;
~
’
~~-~rz~~z~~~Z4~~~ female child
~~~ ~~~~~~~~~~$~ ~z~~~~~z~~~ z-~~~~
was. admitted to of the Uni-ier,,sif~,,v
~
~
~.
’
~~~~~ ~~~~ ~,~~~~~‘~~~~~ ~~=i~ ~ ~~ rr~~I of whole blood,
and a,iititit,&’Lari-,
wab,’’adr4linist,-ered ~,
~~ ~. ~~°~p~~~~~.~~* ~aer~~~ ~~~~~~~ ~~‘ the upper gas~~~~~t~~~~~~~ tract , showed
,.
~s~~a~~;~~~1 varices. ~; ~
From the Departments of Paediatrics,* Radiology and ~ ~~5~~~~~~~-~~~~;~~~~ ~-e~~~~~~ a dilated splenic vein Pathology, † University of Ibadan and University College ~~~i~~ ~~~~ ~ dilated coronary vein. Th4~,~, Hospital, Ibadan, Nigeria. ~ ~’~~r~ ,:p.r~.tt!mt~:poRma~:
to Congenital Obstruction of the Portal Vein and Associated
Gross Hepatic Lobulation Akinsanya O. Obisesan,
Abiodun O. Johnson, M.D., MRCP,DCH,*
M.B.,
A. Olufemi Williams, M.D.,
DMRD, FRCR. FRCP, MRCPath†
A 10-week old girl presented with splenomegaly and recurrent hematemesis from esophageal varices. Splenoportography revealed a dilated extrahepatic portion of the portal vein with nonvisualization of its intrahepatic tributaries. The child died following an episode of hematemesis and was found to have a dilated portal vein which ended blindly. In addition, there was abnormal lobulation of the inferior surface of the liver which was not cirrhotic. The portal vascular anomaly, which presumably was responsible for the portal hypertension, was probably due to failure of communication between the embryonic vitelline veins or to atresia of the portal vein secondary to pressure from the abnormal hepatic lobulation in utero. It would appear that congenital factors may be significant in the etiology and pathogenesis of some cases of extrahepatic portal hypertension in early life and recognition of such developmental anomalies is of importance in management.
I N MANY tNSTANCES, the cause of extrahc-. patic portal hypertension in cht!dhood remains tzt~s~ut‘~.’ Neonatal omplialitis, perittMtitis, infection
~:c~ll~~~ ~~~~a~t~~l, ~’~~4~~~~~~. Nifreria with the complaint that the child had vomited, approximately
with dehydration and umbilical vein catheterizatiori arc acquired factors which, are widely believed to predispose, to thrombosis ~~’ ~tz~ poi-tal vein or its tnbutanes,~!tim.at€!y resulting ~zz a raised portal venous pressure. However, only in the. minority of cases is the exact, cause o~cxtrahepa.Ht: portal ~y_ ’..’’.’ pertension i(lentified.2 .. ’ ’ Extrahepatic portal hypertension may also b~ c~n~enit~~~, r~sz~~tizz~ f’r~z~z failure of anastot-n6sis of the vitelline veins in the embryo,3 or atresia of ’ some ~~.rt z~f‘ t~~ portal ’venous’ system,-’ but doctimented instances of* these developinentat anomalies B’ .~. B’/:~ ~ .~’’~.’ are relatively rare. This is a report of the unusual ending of portal vein, occlusion and gross hepatic lobulation ~n a
.
..
..
50 mt of* red blood that aftcrntxt’n. No. blood had been z~~~r~~~.*c~’i~a ~h~ s~c~c~~, ~~~~’~v~~ ~~c~r~rz at ta~~zr~~.~ after.a’M! term uneventful pregnancy- There was no.’history ..d’ nponat.ai jaundice-or infection. Since birth, the child had t.hrivect. C:3r~ ~c~t~~~~~a~~~~, t~~~ c~a~~~ was afebriie’ and amctfnc. She was pale but well nourished, weighing 4.5 kg. Her artery pulse’was &dquo;t58/mmute and blood pressure was 90~55 mm Hg. The sp!€cn, enlarged to 6 cni below the left costal .’ ~~~x~ir~ ~~c~~~ t~~ ~:nt~ric~~ a~ci~l;~~-y li~~, was firm -but/not tender. The liver was’just palpable and There was no ascites. Investigations revealed that.
her~~~c~~r~~ was, ~t~~~~ WBC- l4i3OO/#l (neutrophils
13%, lyi-nphocvtes 81%, monocytes 6%). HemoIglo4in electropboresis w~..AA~’phte!€’t count,, clotting and bleeding t~~~~, ~~~~n ’eteetro!yte’s,~Mood.’
’.&dquo; ;.
~’
~ ~’~~rz~orz~~=cz~~ ~~il~ w~~ ~~r~~~z~t~~ ~i~h ~’~~tzares ~, urea: and liver function tests were all within normal ~ ’’ ’ limits. Stool , examination ’revealed presence of ’of’extrahepatic’ port,41 hypertension. ~~~~~~~t blood. The child. ..was. thought to have ../: portal hypertension probably secondary to neo~ & d q u o ; ~’’’ Ca~:~$p~~’:.;.,&dquo;:;~~~ ,:; ’~Jr~:~./’’./~;~:~:. ’~’~.~~.:.’~ ~~~~ B ;~.~.’:&dquo; ~’&dquo;’;/&dquo;~.&dquo; .f~~~’’ ~.’ /~ ’ ’~ ~:~~~’ ~~~~~~~~~~* . &dquo;
’
’’
’&dquo;
~
’
~~-~rz~~z~~~Z4~~~ female child
~~~ ~~~~~~~~~~$~ ~z~~~~~z~~~ z-~~~~
was. admitted to of the Uni-ier,,sif~,,v
~
~
~.
’
~~~~~ ~~~~ ~,~~~~~‘~~~~~ ~~=i~ ~ ~~ rr~~I of whole blood,
and a,iititit,&’Lari-,
wab,’’adr4linist,-ered ~,
~~ ~. ~~°~p~~~~~.~~* ~aer~~~ ~~~~~~~ ~~‘ the upper gas~~~~~t~~~~~~~ tract , showed
,.
~s~~a~~;~~~1 varices. ~; ~
From the Departments of Paediatrics,* Radiology and ~ ~~5~~~~~~~-~~~~;~~~~ ~-e~~~~~~ a dilated splenic vein Pathology, † University of Ibadan and University College ~~~i~~ ~~~~ ~ dilated coronary vein. Th4~,~, Hospital, Ibadan, Nigeria. ~ ~’~~r~ ,:p.r~.tt!mt~:poRma~:
