- Email: [email protected]
Portal hypertension in children
23.
O r l o f f , M . l . , e t a l . : E x p e r i m e n t a l ascites. V I . T h e effects o f h e p a t i c v e n o u s o u t f l o w o b s t r u c t i o n a n d asciEes o n a l d o s t e r o n e s e c r e t i o n , S u r g e r y 5 6 : 8 3 , 1964. 24. P e s k i n , (.3. W . , et a l . : C o m p a r a t i v e effects o f p o r t o s y s t e m i c s h u n t i n g o n l i v e r f u n c t i o n , use o f a t e c h n i q u e : h a l f - a n d - h a l f s h u n t , S u r g e r y 54 : 67, ! 963, 25. P o p p e r , H., E l i a s , H., a n d p e t t y , D . E.: V a s c u l a r p a t t e r n of the c i r r h o t i c liver, A m . J. C l i n . P a t h . 2 2 : 7 1 7 , 1952. 26. R a t h e r , L. J., a n d C o h n , R . : S o m e effects u p o n t h e liver o f c o m p l e t e a r t e r i a l i z a t i o n o f its b l o o d s u p p l y : l I l . A c u t e v a s c u l a r n e u r o s i s , S u r g e r y 3 4 : 2 0 7 , 1953. 27. R e d e k e r , A. G., G e l l e r , H . M . , a n d R e y n o l d s , T. B.: H e p a t i c w e d g e p r e s s u r e , b l o o d flow, v a s c u l a r r e s i s t a n c e a n d o x y g e n c o n s u m p t i o n in c i r r h o s i s b e f o r e a n d a f t e r e n d - t o - s i d e p o r t a c a v a l s h u n t , J. C / i n . I n v e s t . 3 7 : 6 0 6 , 11958. 28. R e d e k e r , A. G., e t a l . : A s s e s s m e n t of p o r t a l a n d h e p a t i c h e m o d y n a m i c s a f t e r s i d e - t o - s i d e p o r t a c a v a l s h u n t in p a t i e n t s w i t h c i r r h o s i s , J. C l i n . I n v e s t . 43: 1464, 1964. 29. R o u s s e l o t , L. M . , e t a l . : E x p e r i e n c e s w i t h p o r t a c a v a l a n a s t o m o s i s : A n a l y s i s of I04 elective end-to-side shunts for prevention of recurrent hemorrhage f r o m e s o p h a g o g a s t r i c v a r i c e s ( 1 9 5 2 t h r o u g h 1961 ), A m . J. M e d . 3 4 : 2 9 7 , I963. 30. S c h i l l i n g , J. A . , M e K e e , F . W . , a n d W i l t , W . : E x p e r i m e n t a l h e p a t i c - p o r t a l a r t e r i o v e n o u s a n a s t o m o s e s , S u r g . , G y n e c . & O b s t . 9 0 : 4 7 3 , 1950. 31. S i l e n , W . , e t a l . : S t u d i e s o f h e p a t i c f u n c t i o n in d o g s w i t h E c k fistula or p o t t a c a v a l t r a n s p o s i t i o n , A r c h . S u r g . 7 4 : 9 6 4 , 1957. 32. W a r r e n , W . D., a n d M u l l e r , W . H . , Jr.: A c l a r i f i c a t i o n o f s o m e h e m o d y n a m i c c h a n g e s in c i r r h o s i s a n d t h e i r s u r g i c a l s i g n i f i c a n c e , A n n . S u r g . 1 5 0 : 4 1 3 , 1959. 33. W a r r e n , W . D., e t a l . : T h e i m p o r t a n c e o f h e m o d y n a m i c s t u d i e s in m a n a g e m e n t of p o r t a l h y p e r t e n s i o n , A n n . S u r g . I 5 8 : 3 8 7 , 1963. 34. W a r r e n , W . D . : C i r r h o s i s a n d p o r t a l h y p e r t e n s i o n : S e l e c t i o n o f p a t i e n t s f o r s h u n t a n d s h u n t f o r t h e p a t i e n t , A m . J. D i g e s t . Dis. 9 : 9 0 6 , 1964. 35. W e l c h , C. S., W e l c h , t-1. F., a n d C a r t e r , J. H . : T h e t r e a t m e n t o f ascites b y s i d e - t o - s i d e p o r t a c a v a l s h u n t , A n n . S u r g . 1 5 0 : 4 2 8 , 1959. 36. W e l c h , H . F., W e l c h , C . S., a n d C a r t e r , J. I t . : P r o g n o s i s a f t e r s u r g i c a l t r e a t m e n t o f a s c i t e s : R e s u l t s o f s i d e - t o - s i d e s h u n t in 4 0 p a t i e n t s , S u r g e r y 5 6 : 7 5 , 1964. 37. Z u i d e m a , G . D., a n d K i r s h . M . M . : H e p a t i c e n c e p h a l o p a t h y f o l l o w i n g p o r t a l decompression: Evaluation of end-to-side and side-to-side anastomosis, Am. S u r g e o n 3 1 : 5 6 7 , 1965.
Portal Hypertension in Children ERIC W .
FONKALSRUD
IN HYPERTENSION of the portal venous system in infants and children, both etiology and management are different than in the same condition in adults. Portal hypertension is o n e o f t h e m o r e d a n g e r o u s causes of upper gastrointestinal hemorrhage in childhood a n d it m u s t be seriously considered in the differential diagnosis of splenomegaly. The diminutive size of the inferior vena cava and mesenteric venous channels in young children makes the operative treatment of portal 70
h y p e r t e n s i o n a f r u s t r a t i n g experience, since s h u n t p r o c e d u r e s are freq u e n t l y f o l l o w e d by t h r o m b o t i c occlusion a n d r e c u r r e n t s y m p t o m s . M a n y ingenious o p e r a t i v e technics h a v e b e e n devised b y W h i p p l e ( 2 7 ) , Blakemore ( 2 ) , Rousselot ( 2 1 ) , Child ( 5 ) , Linton ( 1 8 ) , Clatw o r t h y ( 8 ) , K o o p ( 1 7 ) a n d m a n y o t h e r investigators to p r o v i d e m e t h o d s of p o r t a l d e c o m p r e s s i o n even in these s m a l l patients. W h i p p l e ' s classilication of p o r t a l h y p e r t e n s i o n into t w o g r o u p s b a s e d on the location of the v e n o u s o b s t r u c t i o n has a d d e d i m m e a s u r a b l y to the u n d e r s t a n d i n g of the p a t h o l o g y a n d c o n s e q u e n t l y to the s u c c e s s f u l t r e a t m e n t ( 2 6 ) . T h e v e n o u s b l o c k m a y be in the i n t r a h e p a t i c or m o r e c o m m o n l y in the e x t r a h e p a t i o p o r t a l veins, i n a d d i t i o n , p o r t a l h y p e r tension m a y o c c a s i o n a l l y be p r o d u c e d by s u p r a h e p a t i c o b s t r u c t i o n of the h e p a t i c veins. P r o g n o s i s a n d operative c o n s i d e r a t i o n s d e p e n d on the l o c a t i o n a n d the etiology of the venous o b s t r u c t i o n . %
EXTRAHEPATIC PORTAL V E N O U S
OBSTRUCTION
P o r t a l h y p e r t e n s i o n s e c o n d a r y to e x t r a h e p a t i c b l o c k is a l m o s t a l w a y s d u e to t h r o m b o s i s of the p o r t a l vein which m a y extend to include m a j o r t r i b u t a r i e s such as the s u p e r i o r m e s e n t e r i c a n d splenic veins ( F i g . 1 ). A l t h o u g h the c a u s e of the t h r o m b o s i s is u s u a l l y o b s c u r e , n e o n a t a l o m p h a l i t i s , i n t r a - a b d o m i n a l infections, d e h y d r a t i o n a n d u m b i l i c a l vein c a t h e t e r i z a t i o n have been r e c o g n i z e d as etiologic m e c h a n i s m s . C o n Fro. l . - - l i l u s i r a l i o n showing various types of portal venous obstruction. Extrahepatic portal Mock may be caused by thrombosis, cavernomatous transformation, stenosis or congenital valves or webs. lntrahepatic block in children is most commonly caused by toxic or postinfectious cirrhosis, biliary atresia or fibrocystic disease.
l~rmh~pat~c
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5te.nosks Sptem~o v. tI~rornbosks Thrombosis
5uI) mes¢,nteri.c v.
71
g e n i t a l valves, . w e b s o r s t e n o s e s of t h e p o r t a l vein r a r e l y c a u s e e x t r a hepatic obstruction (3).
INFANTS A s c i t e s h a s b e e n - o b s e r v e d by C l a t w o r t h y a n d Boles to be a c o m m o n e a r l y m a n i f e s t a t i o n of p o r t a l h y p e r t e n s i o n d u e t o e x t r a h e p a t i c b l o c k in young infants (6). Anemia, gastrointestinal hemorrhage from diapedesis of e r y t h r o c y t e s a c r o s s the i n t e s t i n a l m u c o s a o r f r o m e s o p h a g e a l varices, malnutrition, and respiratory infections resulting from phrenic e l e v a t i o n are also f r e q u e n t s y m p t o m s in t h e s e infants. T h e o c c a s i o n a l l y e n c o u n t e r e d c a v e r n o m a t o u s t r a n s f o r m a t i o n of t h e p o r t a l v e i n a n d its t r i b u t a r i e s is b e l i e v e d to r e s u l t e i t h e r f r o m t h e r e c a n a l i z a t i o n of an old t h r o m b u s o r to the d e v e l o p m e n t of s m a l l e x t r a l u m i n a I v e n o u s c o l l a t e r als. T h e s e f r u s t r a t i n g a n a t o m i c s i t u a t i o n s m a y m a k e it a l m o s t i m p o s sible t o p e r f o r m a n y t y p e of p o r t a l d e c o m p r e s s i o n p r o c e d u r e , since t h e o n l y m e s e n t e r i c v e n o u s c h a n n e l s t h a t a r e p a t e n t are u s u a l l y so s m a l l t h a t flow is. insufficient t o p r e v e n t t h r o m b o s i s . I n f a n t s with a c u t e e x t r a h e p a t i c p o r t a l v e n o u s o b s t r u c t i o n m a y be t r e a t e d m o r e s u c c e s s f u l l y b y r e p e a t e d i n f u s i o n s of b l o o d a n d a l b u m i n to increase the intravascular osmotic colloid pressure, and repeated p a r a c e n t e s e s w h e n t h e ascitic fluid c a u s e s r e s p i r a t o r y d i s t r e s s o r n a u sea. D i u r e t i c s , a n d s p i r o n o l a c t o n e s , h a v e o c c a s i o n a l l y b e e n h e l p f u l in t h e t r e a t m e n t of t h e s e s m a l l infants. U s i n g t h e s e n o n o p e r a t i v e m e a s ures, B o l e s a n d C l a t w o r t h y ( 3 ) h a v e o b s e r v e d s p o n t a n e o u s c e s s a t i o n of ascites a n d r e s u m p t i o n of a n o r m a l g r o w t h p a t t e r n in s e v e r a l i n f a n t s w i t h e x t r a h e p a t i c p o r t a l o b s t r u c t i o n . Since t h e liver is a l m o s t a l w a y s n o r m a l b o t h f u n c t i o n a l l y a,~d h i s t o l o g i c a l l y , a n d b e c a u s e m a s s i v e h e m o r r h a g e is r a r e in t h e s e infants, c o n s e r v a t i v e t r e a t m e n t h a s m u c h t o r e c o m m e n d it until t h e p a t i e n t is large e n o u g h so that a s h u n t is m o r e l i k e l y t o r e m a i n p a t e n t . If m a s s i v e v a r i c e a l h e m o r r h a g e o c c u r s , b l o o d replacement, intravenous posterior pituitary extract (Pituitrin), bed rest a n d s e d a t i o n u s u a l l y will suffice to tide t h e i n f a n t t h r o u g h t h e a c u t e episode. F o r persistent bleeding, gastroesophageal t a m p o n a d e and t r a n s e s o p h a g e a l v a r i c e a l l i g a t i o n m a y be n e c e s s a r y , a l t h o u g h t h e b e n e fit is u s u a l l y t e m p o r a r y a n d t h e s e t e c h n i c s u s e d in e m e r g e n c y s i t u a t i o n s in s m a l l c h i l d r e n h a v e b e e n g e n e r a l l y d i s a p p o i n t i n g . W h e n r e p e a t e d h e m o r r h a g e occurs, r e s e c t i o n of t h e v a r i x - b e a r i n g a r e a b y e s o p h a g o gastrectomy s u p p l e m e n t e d by pyloroplasty has been used with some s u c c e s s as a palliative p r o c e d u r e . T h e r e s e c t e d p o r t i o n Of t h e s t o m a c h m a y b e r e p l a c e d by j e j u n u m ( 2 0 ) or c o l o n ( 1 7 ) or a d i r e c t e s 0 p h a g o g a s t r o s t o m y . S p l e n e c t o m y a l o n e is n o t a n effective m e t h o d of t r e a t i n g p o r t a l h y p e r t e n s i o n , e x c e p t in p a t i e n t s w i t h i s o l a t e d splenic vein t h r o m bosis. 72
OLDER CHILDREN Massive and recurring gastrointestinal h e m o r r h a g e from varices due to e x t r a h e p a t i c p o r t a l b l o c k is a s e r i o u s p r o b l e m in c h i l d r e n , a l t h o u g h it is r a r e l y fatal o n t h e first t w o e p i s o d e s ( 1 ). T h u s ; p r o p h y l a c t i c s h u n t i n g h a s little p t a c e in t h e m a n a g e m e n t of p o r t a l h y p e r t e n s i o n in child r e n . S p l e n o m e g a l y itself is a f r e q u e n t c o n c o m i t a n t of p o r t a l h y p e r t e n Sion a n d m a y p r o d u c e f u r t h e r u n p l e a s a n t s y m p t o m s . I n c o n t r a s t t o i n f a n t s w i t h e x t r a h e p a t i c b l o c k , c h i l d r e n do n o t c o m m o n l y d e v e l o p ascites as an e a r l y s y m p t o m . T h e n o n o p e r a t i v e t r e a t m e n t of h e m o r r h a g e f r o m e s o p h a g e a l v a r i c e s a d v o c a t e d f o r i n f a n t s m a y also be a d v i s a b l e in c h i l d r e n u n d e r 10 y e a r s of age. W h e n r e p e a t e d h e m o r r h a g e o c c u r s , it is i m p o r t a n t to define t h e l o c a t i o n a n d t h e e x t e n t of t h e p o r t a l vein o b s t r u c t i o n by v e n o g r a p h y b e f o r e p l a n n i n g the o p e r a t i v e a p p r o a c h . S p l e n o p o r t o g r a p h y is t h e m o s t c o m m o n l y u s e d t e c h n i c a n d is m o s t safely p e r f o r m e d u n d e r light general a n e s t h e s i a in c h i l d r e n w h o s e m o v e m e n t s are f r e q u e n t l y u n p r e d i c t a b l e (Fig. 2 ) . If this m e t h o d is n o t p o s s i b l e b e c a u s e of p r e v i o u s s p l e n e c t o m y or t h r o m b o s i s of t h e s p l e n i c vein, it m a y be n e c e s s a r y to c a n n u l a t e a m e s e n t e r i c vein at the t i m e of l a p a r o t o m y . H e m o r r h o i d a l c o n t r a s t studies h a v e o c c a s i o n a l l y p r o v i d e d s a t i s f a c t o r y v i s u a l i z a t i o n of the p o r t a l v e n o u s s y s t e m . W h e n t h e e x t r a h e p a t i c p o r t a l o b s t r u c t i o n is l i m i t e d to t h e p o r t a l vein, a c e n t r a l l y p l a c e d s p l e n o r e n a l s h u n t close to the v e n a c a v a t h r o u g h a left t h o r a c o a b d o m i n a l incision, as a d v o c a t e d by C l a t w o r t h y FrG. 2 . I P e r c u t a n e o u s splenoportogram, from patient with portal vein th~.-ombosis, showing dilated mesenteric venous channels extending up to esophagus.
73
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FIG. 3. Centrally placed splenorenal shunt as r e c o m m e n d e d by C l a t w o r t h y and Boles. Shunt is made with as short a length of splenic vein as possible and as close to the confluence with the mesenteric vein as feasible, and is directed into the renal vein without angulation. and Boles (6), has a good chance of remaining patent (Fig. 3). Conv e n t i o n a l s p l e n o r e n a l s h u n t s in c h i l d r e n u n d e r t h e a g e o f 10 h a v e a n 82% incidence of failure (7). Although portacaval shunts are rarely p o s s i b l e in c h i l d r e n w i t h p o r t a l v e i n t h r o m b o s i s , S h a l d o n a n d S h e r l o c k ( 2 3 ) r e p o r t e d s u c c e s s in 5 s u c h p a t i e n t s . W h e n t h e s p l e n i c v e i n is o b s t r u c t e d o r is u n a v a i l a b l e b e c a u s e o f p r e v i o u s s p l e n e c t o m y , t h e s i d e - t o end superior mesenteric to vena cava shunt has been of considerable v a l u e ( 8 , 14, I 9 ) ( F i g . 4 ) . T h i s p r o c e d u r e p e r m i t s c r e a t i o n o f t h e largest possible shunt between venous lakes or anomalous drainage a r e a s in t h e p o r t a l v e n o u s s y s t e m a n d t h e s y s t e m i c v e n o u s c i r c u l a t i o n . S u b s e q u e n t e d e m a o f t h e l o w e r l i m b s is a r a r e a n d t r a n s i e n t o c c u r rence. Makeshift shunts using mesenteric venous tributaries have been f o l l o w e d b y r e p e a t e d h e m o r r h a g e in e a c h o f 1 l r e p o r t e d c a s e s ( 7 ) . Portal venous anastomoses are susceptible to thrombosis if a c t i v e t h r o n a b o p h l e b i t i s is p r e s e n t . Even though the technical difficulties of successfully constructing a n E c k f i s t u l a in s m a l l p a t i e n t s a r e g r e a t , s u c h p r o c e d u r e s in s e l e c t e d c h i l d r e n m a y b e l i f e s a v i n g . A s u c c e s s f u l s h u n t is t h e o n l y p r o c e d u r e w h i c h is l i k e l y to b e c u r a t i v e in p a t i e n t s w i t h e x t r a h e p a t i c b l o c k . Experimental evidence indicates that venous anastomoses performed i n s m a l l a n i m a l s will g r o w if h a l f o f t h e s u t u r e l i n e c o n s i s t s o f i n t e r r u p t e d s t i t c h e s of fine s i l k ( 2 2 ) . I t s h o u l d b e e m p h a s i z e d t h a t e v e n though Eck fistulae may obviate further variceal hemorrhage, the longterm effects on growth and development and the ultimate prognosis are not yet known. 74
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FIG. 4.1Side-to-end superior ~nesenteric vein to inferior vena cava anastomosis, for patients with extrahepatic venous obstruction in whom portacaval and sptenorenal shunts are not possible.
W h e n t h e v e n o u s o b s t r u c t i o n is so e x t e n s i v e as to p r e c l u d e a s h u n t ing p r o c e d u r e , o r if s e v e r e h e m o r r h a g e r e c u r s f o l l o w i n g a s h u n t , a d i r e c t o p e r a t i v e a p p r o a c h on the v a r i c e s m a y b e n e c e s s a r y . . E n d o s c o p i c i n j e c t i o n of t h e v a r i c e s w i t h s c l e r o s i n g s o l u t i o n s h a s b e e n s u c c e s s f u l in c o n t r o l l i n g the h e m o r r h a g e t e m p o r a r i l y in a f e w s u c h i n s t a n c e s a n d m a y be s a f e r t h a n v a r i c e a l l i g a t i o n ( 9 , 1 0 ) . F r e q u e n t b l a n d f e e d i n g s a n d a n t a c i d s a r e also h e l p f u l in s u c h p a t i e n t s .
INTRAHEPATIC
PORTAL V E N O U S
OBSTRUCTION
P o r t a l h y p e r t e n s i o n d u e to i n t r a h e p a t i c v e n o u s b l o c k c a r r i e s a m u c h less f a v o r a b l e o u t l o o k t h a n e x t r a h e p a t i c o b s t r u c t i o n b e c a u s e of t h e s e r i o u s a n d p r o g r e s s i v e n a t u r e of the p r i m a r y h e p a t i c d i s e a s e . I n t r a 75
h e p a t i c p o r t a l o b s t r u c t i o n is a l m o s t always a s e q u e l a of cirrhosis of the liver w h i c h m a y follow n e o n a t a l hepatitis, e x p o s u r e to toxic a g e n t s or c o n g e n i t a l biliary atresia. G l y c o g e n s t o r a g e diseases m a y o c c a s i o n a l l y p r o d u c e v e n o u s c o m p r e s s i o n with r e s u l t a n t p o r t a l h y p e r t e n s i o n . It is g e n e r a l l y a s s u m e d t h a t p e r i p o r t a l fibrosis, r e g a r d l e s s of the etiology, may produce venous hypertension. T h e o p e r a t i v e m a n a g e m e n t of p o r t a l h y p e r t e n s i o n d u e to i n t r a h e p a t i c b l o c k d e p e n d s o n the p r o g n o s i s in the u n d e r l y i n g liver disease. G a s t r o i n t e s t i n a l h e m o r r h a g e f r o m varices in s u c h p a t i e n t s is m o r e likely to be p r o t r a c t e d and fatal t h a n in t h o s e with e x t r a h e p a t i c block. P o o r liver f u n c t i o n results in m a l n u t r i t i o n a n d h y p o p r o t h r o m b i n e m i a w h i c h c o m p l i c a t e s t r e a t m e n t of a c u t e h e m o r r h a g e . T o x i c i t y f r o m h y p e r a m m o n e m i a , d u e to a b s o r p t i o n of p r o t e i n f r o m b l o o d in t h e large bowel, also c o m p l i c a t e s the c a r e d u r i n g an episode of bleeding. T h e c o u r s e of p o r t a l h y p e r t e n s i o n in p a t i e n t s with biliary c i r r h o s i s c a u s e d by e x t r a h e p a t i c bile d u c t o b s t r u c t i o n , e.g., injury to the c o m m o n bile d u c t or c h o l e d o c h a l cyst When the bite d u c t block is relieved, is n o t c l e a r ( 4 ) . If p o r t a l v e n o u s d e c o m p r e s s i o n is a c c o m p a n i e d b y s u b s e q u e n t i m p r o v e m e n t in h e p a t i c f u n c t i o n as s u g g e s t e d by B l a k e m o r e ( 2 ) , t h e n a s h u n t s h o u l d logically be p e r f o r m e d along w i t h c o r rection of c o m m o n d u c t o b s t r u c t i o n . T h i s has b e e n o u r e x p e r i e n c e in a child with a c h o l e d o c h a l cyst w h o s e initial c o m p l a i n t was v a r i c e a l h e m o r r h a g e ( I ! ). T h e r e is little e v i d e n c e to suggest t h a t c i r r h o s i s a n d p o r t a l h y p e r t e n s i o n due to p o s t i n f e c t i o u s necrosis will s p o n t a n e o u s l y regress. T h e m o s t effective relief of p o r t a l h y p e r t e n s i o n in c h i l d r e n o v e r 4 years of age with i n t r a h e p a t i c o b s t r u c t i o n is o b t a i n e d f r o m a side-toside p o r t a c a v a l a n a s t o m o s i s or f r o m a c e n t r a l l y p l a c e d s p l e n o r e n a t s h u n t ( 6 ) . Y o u n g e r c h i l d r e n s h o u l d be t r e a t e d n o n o p e r a t i v e l y o r by v a r i c e a l injection o r ligation. C a r e f u l p r e o p e r a t i v e p r e p a r a t i o n with diuresis of ascitic fluid, a d m i n i s t r a t i o n of blood, colloid a n d v i t a m i n K a n d r e m o v a l of b l o o d f r o m the g a s t r o i n t e s t i n a l t r a c t are i m p o r t a n t adj u n c t s to successful s u r g e r y in t h e s e p a t i e n t s . It is wise to avoid h e p a t o toxic a n e s t h e t i c a g e n t s a n d to h a v e fresh blood available. In c h i l d r e n with p o r t a l h y p e r t e n s i o n , d u e to p o s t n e c r o t i c or posth e p a t i t i c cirrhosis, or h e p a t i c i n v o l v e m e n t f r o m fibrocystic disease of the p a n c r e a s , i m p r o v e m e n t in n u t r i t i o n , r e g r e s s i o n of e s o p h a g e a l varices a n d d i s a p p e a r a n c e of ascites and h y p e r s p l e n i s m h a v e f r e q u e n t l y f o l l o w e d successful s h u n t p r o c e d u r e s ( 3 ) . C h i l d r e n with p o r t a l h y p e r t e n s i o n d u e to c o n g e n i t a l biliary at r esi a h a v e the least f a v o r a b l e o u t l o o k . S h u n t i n g p r o c e d u r e s Or o t h e r h e r o i c lifesaving o p e r a t i v e m e a s u r e s are g e n e r a l l y n o t advised in these patients w h o s e disease r a r e l y p e r m i t s s u r v i v a l b e y o n d 2 y e a r s of age ( 1 5 ) . T o t a l h e p a t i c h o m o t r a n s p l a n t a t i o n a p p e a r s to be the only p r o m ising a p p r o a c h to the m a n a g e m e n t of p a t i e n t s with this m a l f o r m a t i o n 76
when the technical, immunologic and procurement surmounted.
obstacles have been
SUPRAHI~PATIC PORTAL V E N O U S OBSTRUCTION H y p e r t e n s i o n of t h e p o r t a l v e n o u s s y s t e m is o c c a s i o n a l l y p r o d u c e d by suprahepatic venous obstruction. Gastrointestinal hemorrhage from varices, ascites and hypersplenism with relatively normal liver function may be sequelae of this condition. Hepatic vein obstruction by granulomatosis has occurred in children i n S o u t h A m e r i c a , b u t is o f l i t t l e s i g n i f i c a n c e in t h e U n i t e d S t a t e s . I d i o p a t h i c r e t r o p e r i t o n e a l fibrosis p r o d u c e d c o m p l e t e o c c l u s i o n of the entire i n f e r i o r vena cava a n d o b s t r u c t i o n of the h e p a t i c veins in a 1 4 - y e a r - o l d b o y t r e a t e d in o u r h o s p i t a l ( 1 2 ) . W e w e r e a b l e to d e c o m press the portal v e n o u s s y s t e m s u c c e s s f u l l y in this p a t i e n t by a n a s t o m o s i n g t h e s p l e n i c v e i n to t h e i s o l a t e d p u l m o n a r y a r t e r y o f t h e l e f t l o w e r l o b e o f t h e l u n g b y m e a n s of a n i n t e r v e n i n g T e f l o n g r a f t . Congestive heart failure or constrictiv e pericarditis may occasionally p r o d u c e s u p r a h e p a t i c o b s t r u c t i o n , w h i c h u s u a l l y r e s p o n d s well-'to a p propriate cardiac therapy.
REFERENCES 1. Arcari, F. A., and Lynn, H. B,: Bleeding esophageal varices in children, Surg., Gynec. & Obst. I 12: 101, I961. 2. Blakemore, A. H.: Portacaval anastomosis: A report on fourteen cases, Bull. New York Acad. Med. 22: 254, 1946. 3. Boles, E. T., Jr., and Clatworthy, H. W., Jr.: Diseases of the Spleen and Portal Circulation, in Benson, C. D., e/ ai. (eds.): Pediatric S u r g e r y (Chicago: Year Book Medical Publishers, Inc., 1962), p. 648. 4. Carnishion, R. C., and Ballinger, W. F., 1I: Portal hypertension in biliar3e cirrhosis, Surg., Gynec. & Obst. 1I 1:91. 1960. 5. Child, C. G., III, a~d Callow, A. D.: The general problem of portal hypertension, Bull. Tufts-New England Med. Center I :1, 1955. 6. Clatworthy, I-I. W., Jr,, and Boles, E. T., Jr.: Extrahepatic portal bed block in children: Pathogenesis and treatment, Ann. Surg. 150:371, 1959. 7. Clatworthy, H. W., Jr., and DeLorimier, A. A.: Portal decompression procedures in children, A m. J. Surg. 107:447, 1964. 8. Clatworthy, H. W., Jr., Wail, T., and W a t m a n , R. N.: A new type of portal to systemic venous shunt for portal hypertension, Arch. Surg. 7 i :588, I955. 9. Crafoord, C., and Frenckner, P.: New surgical treatment of varicose veins of oesophagus, Acta oto-laryng. 27:422, 1939. 10. Fearon, B., and Sass-Kortsak: The m a n a g e m e n t of esophageal varices in children by injection of sclerosing agents, Ann. 0~ol. Rhin. & Laryng. 68: 906, 1959. t 1. Fonkalsrud, E. W., and Boles. E. T., Jr.: Choledochal cysts in infancy and childhood, Surg., Gynec. & Obst. 12I :733. 1965. 12. Fonkalsrud, E. W., Linde, I., and Longnaire. W. P., Jr.: Portal hypertension from idiopathic superior vena caval obstruction. J.A.M.A. I96: 129, 1966. 77
t3.
F o s t e r . J. H . , H o t c o m b , G . W . , a n d K i r t l e y , J. A . : R e s u l t s o f s u r g i c a l t r e a t m e n t o f p o r t a l h y p e r t e n s i o n in c h i l d r e n , A n n . S u r g . 1 5 7 : 8 6 8 , 1963. 14. G l i e d m a n , M . L., a n d M a r g u l i e s , M . : T h e s i d e - t o - e n d s u p e r i o r m e s e n t e r i c vein-inferior vena cava shunt for portal decompression, Surgery 56:473, 1964. 15. H a y s , D . M . , a n d S n y d e r , W . H . , J r . : L i f e s p a n in u n t r e a t e d b i l i a r y a t r e s i a , S u r g e r y 5 4 : 3 7 3 , 1963. 16. K o o p , C . E., a n d K a v i a n i a n , A . : R e a p p r a i s a l o f c o l o n i c r e p l a c e m e n t o f distal e s o p h a g u s a n d p r o x i m a l s t o m a c h in t h e m a n a g e m e n t o f b l e e d i n g v a r i c e s in c h i l d r e n , S u r g e r y 5 7 : 4 5 4 , 1965. 17. K o o p , C. E., a n d R o d d y , S. R-: C o l o n i c r e p l a c e m e n t o f d i s t a l e s o p h a g u s a n d p r o x i m a l s t o m a c h in t h e m a n a g e m e n t o f b l e e d i n g v a r i c e s in c h i l d r e n , A n n . S u r g . 147: 17, 1958. 18. L i n t o n , R . R . , H a r d y , I. B., a n d W o l w i l e r , W . : P o r t a c a v a l s h u n t s irt t r e a t m e n t o f p o r t a l h y p e r t e n s i o n , S u r g . , G y n e c . & O b s t . 8 7 : 1 2 9 , 1948. 19. M a r i o n , P . : L e s O b s t r u c t i o n s P o r t a l e s , S e m a i n e h 6 p . P a r i s 2 9 : 2 7 8 ] , 1953. 20. M e r e n d i n o , K . A . , a n d D i l l a r d , D. H . : T h e c o n c e p t o f s p h i n c t e r s u b s t i t u t i o n by an interposed jejunal segment for anatomic and physiologic abnormalities a t t h e e s o p h a g o g a s t r i c j u n c t i o n , w i t h s p e c i a l r e f e r e n c e to r e f l u x e s o p h a g i t i s , c a r d i o s p a s m , a n d e s o p h a g e a l v a r i c e s , A n n . S u r g . 1 4 2 : 4 8 6 , 1955. 21. R o u s s e l o t , L. M . : T h e p r e s e n t s t a t u s o f s u r g e r y f o r p o r t a l h y p e r t e n s i o n , A m . J. M e d . 1 6 : 8 7 4 , 1954. 22. S a u v a g e , L. R., a n d W e s o l o w s k i , S. A . : A n a s t o m o s e s a n d g r a f t s in t h e v e n o u s s y s t e m w i t h s p e c i a l r e f e r e n c e to g r o w t h c h a n g e s , S u r g e r y 3 7 : 7 1 4 , 1955. 23. S h a l d o n , S., a n d S h e r l o c k , S.: O b s t r u c t i o n to t h e e x t r a h e p a t i c p o r t a l s y s t e m in c h i l d h o o d , L a n c e t 1 : 63, 1962. 24. T r u s l e r , G . A., M o r r i s , F . R., a n d M u s t a r d , W . T . : P o r t a l h y p e r t e n s i o n in c h i l d h o o d ; S u r g e r y 52: 6 6 4 , 196225. Y o o r h e e s , A. B., Jr., e t a l . : P o r t a l h y p e r t e n s i o n in c h i l d r e n : 98 cases, S u r g e r y 5 8 : 5 4 0 , 1965. 26. W h i p p l e , A. O . : P r o b l e m o f p o r t a l h y p e r t e n s i o n in r e / a t i o n to h e p a t o s p l e n o p a t h i e s , A n n . S u r g . 1 2 2 : 4 4 9 , 1945. 27. W h i p p l e , A . O . : T h e r a t i o n a l e o f p o r t a c a v a l a n a s t o m o s i s , Bull. N e w Y o r k A c a d . M e d . 2 2 : 2 5 1 , 1946.
Surgical Treatment of Portal Hypertension-Long-Term Results WILLIAM
P. L O N G M I R E ,
JR,
THE SURGICAL TREATMENT OF PORTAL HYPERTENSION has b e e n directed primarily t o w a r d the c o n t r o l of bleeding f r o m e s o p h a g e a l v a r i c e s , o n e of t h e s e c o n d a r y effects of t h e e l e v a t e d v e n o u s p r e s s u r e . It is of n o d i r e c t b e n e f i t in t h e t r e a t m e n t of t h e l i v e r d i s e a s e p e r se, a l t h o u g h t h e p r e v e n t i o n of h e m o r r h a g e i n t o t h e g a s t r o i n t e s t i n a l t r a c t will d e c r e a s e t h e l i k e l i h o o d of f u r t h e r d a m a g e to t h e h e p a t i c cells f r o m t h e a b s o r p t i o n i n t o the p o r t a l v e n o u s s y s t e m of t h e p r o d u c t s of b l o o d m e t a b o l i z e d in t h e i n t e s t i n a l t r a c t . A n u m b e r of s t u d i e s ( 1 - 8 ) 78
O r l o f f , M . l . , e t a l . : E x p e r i m e n t a l ascites. V I . T h e effects o f h e p a t i c v e n o u s o u t f l o w o b s t r u c t i o n a n d asciEes o n a l d o s t e r o n e s e c r e t i o n , S u r g e r y 5 6 : 8 3 , 1964. 24. P e s k i n , (.3. W . , et a l . : C o m p a r a t i v e effects o f p o r t o s y s t e m i c s h u n t i n g o n l i v e r f u n c t i o n , use o f a t e c h n i q u e : h a l f - a n d - h a l f s h u n t , S u r g e r y 54 : 67, ! 963, 25. P o p p e r , H., E l i a s , H., a n d p e t t y , D . E.: V a s c u l a r p a t t e r n of the c i r r h o t i c liver, A m . J. C l i n . P a t h . 2 2 : 7 1 7 , 1952. 26. R a t h e r , L. J., a n d C o h n , R . : S o m e effects u p o n t h e liver o f c o m p l e t e a r t e r i a l i z a t i o n o f its b l o o d s u p p l y : l I l . A c u t e v a s c u l a r n e u r o s i s , S u r g e r y 3 4 : 2 0 7 , 1953. 27. R e d e k e r , A. G., G e l l e r , H . M . , a n d R e y n o l d s , T. B.: H e p a t i c w e d g e p r e s s u r e , b l o o d flow, v a s c u l a r r e s i s t a n c e a n d o x y g e n c o n s u m p t i o n in c i r r h o s i s b e f o r e a n d a f t e r e n d - t o - s i d e p o r t a c a v a l s h u n t , J. C / i n . I n v e s t . 3 7 : 6 0 6 , 11958. 28. R e d e k e r , A. G., e t a l . : A s s e s s m e n t of p o r t a l a n d h e p a t i c h e m o d y n a m i c s a f t e r s i d e - t o - s i d e p o r t a c a v a l s h u n t in p a t i e n t s w i t h c i r r h o s i s , J. C l i n . I n v e s t . 43: 1464, 1964. 29. R o u s s e l o t , L. M . , e t a l . : E x p e r i e n c e s w i t h p o r t a c a v a l a n a s t o m o s i s : A n a l y s i s of I04 elective end-to-side shunts for prevention of recurrent hemorrhage f r o m e s o p h a g o g a s t r i c v a r i c e s ( 1 9 5 2 t h r o u g h 1961 ), A m . J. M e d . 3 4 : 2 9 7 , I963. 30. S c h i l l i n g , J. A . , M e K e e , F . W . , a n d W i l t , W . : E x p e r i m e n t a l h e p a t i c - p o r t a l a r t e r i o v e n o u s a n a s t o m o s e s , S u r g . , G y n e c . & O b s t . 9 0 : 4 7 3 , 1950. 31. S i l e n , W . , e t a l . : S t u d i e s o f h e p a t i c f u n c t i o n in d o g s w i t h E c k fistula or p o t t a c a v a l t r a n s p o s i t i o n , A r c h . S u r g . 7 4 : 9 6 4 , 1957. 32. W a r r e n , W . D., a n d M u l l e r , W . H . , Jr.: A c l a r i f i c a t i o n o f s o m e h e m o d y n a m i c c h a n g e s in c i r r h o s i s a n d t h e i r s u r g i c a l s i g n i f i c a n c e , A n n . S u r g . 1 5 0 : 4 1 3 , 1959. 33. W a r r e n , W . D., e t a l . : T h e i m p o r t a n c e o f h e m o d y n a m i c s t u d i e s in m a n a g e m e n t of p o r t a l h y p e r t e n s i o n , A n n . S u r g . I 5 8 : 3 8 7 , 1963. 34. W a r r e n , W . D . : C i r r h o s i s a n d p o r t a l h y p e r t e n s i o n : S e l e c t i o n o f p a t i e n t s f o r s h u n t a n d s h u n t f o r t h e p a t i e n t , A m . J. D i g e s t . Dis. 9 : 9 0 6 , 1964. 35. W e l c h , C. S., W e l c h , t-1. F., a n d C a r t e r , J. H . : T h e t r e a t m e n t o f ascites b y s i d e - t o - s i d e p o r t a c a v a l s h u n t , A n n . S u r g . 1 5 0 : 4 2 8 , 1959. 36. W e l c h , H . F., W e l c h , C . S., a n d C a r t e r , J. I t . : P r o g n o s i s a f t e r s u r g i c a l t r e a t m e n t o f a s c i t e s : R e s u l t s o f s i d e - t o - s i d e s h u n t in 4 0 p a t i e n t s , S u r g e r y 5 6 : 7 5 , 1964. 37. Z u i d e m a , G . D., a n d K i r s h . M . M . : H e p a t i c e n c e p h a l o p a t h y f o l l o w i n g p o r t a l decompression: Evaluation of end-to-side and side-to-side anastomosis, Am. S u r g e o n 3 1 : 5 6 7 , 1965.
Portal Hypertension in Children ERIC W .
FONKALSRUD
IN HYPERTENSION of the portal venous system in infants and children, both etiology and management are different than in the same condition in adults. Portal hypertension is o n e o f t h e m o r e d a n g e r o u s causes of upper gastrointestinal hemorrhage in childhood a n d it m u s t be seriously considered in the differential diagnosis of splenomegaly. The diminutive size of the inferior vena cava and mesenteric venous channels in young children makes the operative treatment of portal 70
h y p e r t e n s i o n a f r u s t r a t i n g experience, since s h u n t p r o c e d u r e s are freq u e n t l y f o l l o w e d by t h r o m b o t i c occlusion a n d r e c u r r e n t s y m p t o m s . M a n y ingenious o p e r a t i v e technics h a v e b e e n devised b y W h i p p l e ( 2 7 ) , Blakemore ( 2 ) , Rousselot ( 2 1 ) , Child ( 5 ) , Linton ( 1 8 ) , Clatw o r t h y ( 8 ) , K o o p ( 1 7 ) a n d m a n y o t h e r investigators to p r o v i d e m e t h o d s of p o r t a l d e c o m p r e s s i o n even in these s m a l l patients. W h i p p l e ' s classilication of p o r t a l h y p e r t e n s i o n into t w o g r o u p s b a s e d on the location of the v e n o u s o b s t r u c t i o n has a d d e d i m m e a s u r a b l y to the u n d e r s t a n d i n g of the p a t h o l o g y a n d c o n s e q u e n t l y to the s u c c e s s f u l t r e a t m e n t ( 2 6 ) . T h e v e n o u s b l o c k m a y be in the i n t r a h e p a t i c or m o r e c o m m o n l y in the e x t r a h e p a t i o p o r t a l veins, i n a d d i t i o n , p o r t a l h y p e r tension m a y o c c a s i o n a l l y be p r o d u c e d by s u p r a h e p a t i c o b s t r u c t i o n of the h e p a t i c veins. P r o g n o s i s a n d operative c o n s i d e r a t i o n s d e p e n d on the l o c a t i o n a n d the etiology of the venous o b s t r u c t i o n . %
EXTRAHEPATIC PORTAL V E N O U S
OBSTRUCTION
P o r t a l h y p e r t e n s i o n s e c o n d a r y to e x t r a h e p a t i c b l o c k is a l m o s t a l w a y s d u e to t h r o m b o s i s of the p o r t a l vein which m a y extend to include m a j o r t r i b u t a r i e s such as the s u p e r i o r m e s e n t e r i c a n d splenic veins ( F i g . 1 ). A l t h o u g h the c a u s e of the t h r o m b o s i s is u s u a l l y o b s c u r e , n e o n a t a l o m p h a l i t i s , i n t r a - a b d o m i n a l infections, d e h y d r a t i o n a n d u m b i l i c a l vein c a t h e t e r i z a t i o n have been r e c o g n i z e d as etiologic m e c h a n i s m s . C o n Fro. l . - - l i l u s i r a l i o n showing various types of portal venous obstruction. Extrahepatic portal Mock may be caused by thrombosis, cavernomatous transformation, stenosis or congenital valves or webs. lntrahepatic block in children is most commonly caused by toxic or postinfectious cirrhosis, biliary atresia or fibrocystic disease.
l~rmh~pat~c
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5te.nosks Sptem~o v. tI~rornbosks Thrombosis
5uI) mes¢,nteri.c v.
71
g e n i t a l valves, . w e b s o r s t e n o s e s of t h e p o r t a l vein r a r e l y c a u s e e x t r a hepatic obstruction (3).
INFANTS A s c i t e s h a s b e e n - o b s e r v e d by C l a t w o r t h y a n d Boles to be a c o m m o n e a r l y m a n i f e s t a t i o n of p o r t a l h y p e r t e n s i o n d u e t o e x t r a h e p a t i c b l o c k in young infants (6). Anemia, gastrointestinal hemorrhage from diapedesis of e r y t h r o c y t e s a c r o s s the i n t e s t i n a l m u c o s a o r f r o m e s o p h a g e a l varices, malnutrition, and respiratory infections resulting from phrenic e l e v a t i o n are also f r e q u e n t s y m p t o m s in t h e s e infants. T h e o c c a s i o n a l l y e n c o u n t e r e d c a v e r n o m a t o u s t r a n s f o r m a t i o n of t h e p o r t a l v e i n a n d its t r i b u t a r i e s is b e l i e v e d to r e s u l t e i t h e r f r o m t h e r e c a n a l i z a t i o n of an old t h r o m b u s o r to the d e v e l o p m e n t of s m a l l e x t r a l u m i n a I v e n o u s c o l l a t e r als. T h e s e f r u s t r a t i n g a n a t o m i c s i t u a t i o n s m a y m a k e it a l m o s t i m p o s sible t o p e r f o r m a n y t y p e of p o r t a l d e c o m p r e s s i o n p r o c e d u r e , since t h e o n l y m e s e n t e r i c v e n o u s c h a n n e l s t h a t a r e p a t e n t are u s u a l l y so s m a l l t h a t flow is. insufficient t o p r e v e n t t h r o m b o s i s . I n f a n t s with a c u t e e x t r a h e p a t i c p o r t a l v e n o u s o b s t r u c t i o n m a y be t r e a t e d m o r e s u c c e s s f u l l y b y r e p e a t e d i n f u s i o n s of b l o o d a n d a l b u m i n to increase the intravascular osmotic colloid pressure, and repeated p a r a c e n t e s e s w h e n t h e ascitic fluid c a u s e s r e s p i r a t o r y d i s t r e s s o r n a u sea. D i u r e t i c s , a n d s p i r o n o l a c t o n e s , h a v e o c c a s i o n a l l y b e e n h e l p f u l in t h e t r e a t m e n t of t h e s e s m a l l infants. U s i n g t h e s e n o n o p e r a t i v e m e a s ures, B o l e s a n d C l a t w o r t h y ( 3 ) h a v e o b s e r v e d s p o n t a n e o u s c e s s a t i o n of ascites a n d r e s u m p t i o n of a n o r m a l g r o w t h p a t t e r n in s e v e r a l i n f a n t s w i t h e x t r a h e p a t i c p o r t a l o b s t r u c t i o n . Since t h e liver is a l m o s t a l w a y s n o r m a l b o t h f u n c t i o n a l l y a,~d h i s t o l o g i c a l l y , a n d b e c a u s e m a s s i v e h e m o r r h a g e is r a r e in t h e s e infants, c o n s e r v a t i v e t r e a t m e n t h a s m u c h t o r e c o m m e n d it until t h e p a t i e n t is large e n o u g h so that a s h u n t is m o r e l i k e l y t o r e m a i n p a t e n t . If m a s s i v e v a r i c e a l h e m o r r h a g e o c c u r s , b l o o d replacement, intravenous posterior pituitary extract (Pituitrin), bed rest a n d s e d a t i o n u s u a l l y will suffice to tide t h e i n f a n t t h r o u g h t h e a c u t e episode. F o r persistent bleeding, gastroesophageal t a m p o n a d e and t r a n s e s o p h a g e a l v a r i c e a l l i g a t i o n m a y be n e c e s s a r y , a l t h o u g h t h e b e n e fit is u s u a l l y t e m p o r a r y a n d t h e s e t e c h n i c s u s e d in e m e r g e n c y s i t u a t i o n s in s m a l l c h i l d r e n h a v e b e e n g e n e r a l l y d i s a p p o i n t i n g . W h e n r e p e a t e d h e m o r r h a g e occurs, r e s e c t i o n of t h e v a r i x - b e a r i n g a r e a b y e s o p h a g o gastrectomy s u p p l e m e n t e d by pyloroplasty has been used with some s u c c e s s as a palliative p r o c e d u r e . T h e r e s e c t e d p o r t i o n Of t h e s t o m a c h m a y b e r e p l a c e d by j e j u n u m ( 2 0 ) or c o l o n ( 1 7 ) or a d i r e c t e s 0 p h a g o g a s t r o s t o m y . S p l e n e c t o m y a l o n e is n o t a n effective m e t h o d of t r e a t i n g p o r t a l h y p e r t e n s i o n , e x c e p t in p a t i e n t s w i t h i s o l a t e d splenic vein t h r o m bosis. 72
OLDER CHILDREN Massive and recurring gastrointestinal h e m o r r h a g e from varices due to e x t r a h e p a t i c p o r t a l b l o c k is a s e r i o u s p r o b l e m in c h i l d r e n , a l t h o u g h it is r a r e l y fatal o n t h e first t w o e p i s o d e s ( 1 ). T h u s ; p r o p h y l a c t i c s h u n t i n g h a s little p t a c e in t h e m a n a g e m e n t of p o r t a l h y p e r t e n s i o n in child r e n . S p l e n o m e g a l y itself is a f r e q u e n t c o n c o m i t a n t of p o r t a l h y p e r t e n Sion a n d m a y p r o d u c e f u r t h e r u n p l e a s a n t s y m p t o m s . I n c o n t r a s t t o i n f a n t s w i t h e x t r a h e p a t i c b l o c k , c h i l d r e n do n o t c o m m o n l y d e v e l o p ascites as an e a r l y s y m p t o m . T h e n o n o p e r a t i v e t r e a t m e n t of h e m o r r h a g e f r o m e s o p h a g e a l v a r i c e s a d v o c a t e d f o r i n f a n t s m a y also be a d v i s a b l e in c h i l d r e n u n d e r 10 y e a r s of age. W h e n r e p e a t e d h e m o r r h a g e o c c u r s , it is i m p o r t a n t to define t h e l o c a t i o n a n d t h e e x t e n t of t h e p o r t a l vein o b s t r u c t i o n by v e n o g r a p h y b e f o r e p l a n n i n g the o p e r a t i v e a p p r o a c h . S p l e n o p o r t o g r a p h y is t h e m o s t c o m m o n l y u s e d t e c h n i c a n d is m o s t safely p e r f o r m e d u n d e r light general a n e s t h e s i a in c h i l d r e n w h o s e m o v e m e n t s are f r e q u e n t l y u n p r e d i c t a b l e (Fig. 2 ) . If this m e t h o d is n o t p o s s i b l e b e c a u s e of p r e v i o u s s p l e n e c t o m y or t h r o m b o s i s of t h e s p l e n i c vein, it m a y be n e c e s s a r y to c a n n u l a t e a m e s e n t e r i c vein at the t i m e of l a p a r o t o m y . H e m o r r h o i d a l c o n t r a s t studies h a v e o c c a s i o n a l l y p r o v i d e d s a t i s f a c t o r y v i s u a l i z a t i o n of the p o r t a l v e n o u s s y s t e m . W h e n t h e e x t r a h e p a t i c p o r t a l o b s t r u c t i o n is l i m i t e d to t h e p o r t a l vein, a c e n t r a l l y p l a c e d s p l e n o r e n a l s h u n t close to the v e n a c a v a t h r o u g h a left t h o r a c o a b d o m i n a l incision, as a d v o c a t e d by C l a t w o r t h y FrG. 2 . I P e r c u t a n e o u s splenoportogram, from patient with portal vein th~.-ombosis, showing dilated mesenteric venous channels extending up to esophagus.
73
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FIG. 3. Centrally placed splenorenal shunt as r e c o m m e n d e d by C l a t w o r t h y and Boles. Shunt is made with as short a length of splenic vein as possible and as close to the confluence with the mesenteric vein as feasible, and is directed into the renal vein without angulation. and Boles (6), has a good chance of remaining patent (Fig. 3). Conv e n t i o n a l s p l e n o r e n a l s h u n t s in c h i l d r e n u n d e r t h e a g e o f 10 h a v e a n 82% incidence of failure (7). Although portacaval shunts are rarely p o s s i b l e in c h i l d r e n w i t h p o r t a l v e i n t h r o m b o s i s , S h a l d o n a n d S h e r l o c k ( 2 3 ) r e p o r t e d s u c c e s s in 5 s u c h p a t i e n t s . W h e n t h e s p l e n i c v e i n is o b s t r u c t e d o r is u n a v a i l a b l e b e c a u s e o f p r e v i o u s s p l e n e c t o m y , t h e s i d e - t o end superior mesenteric to vena cava shunt has been of considerable v a l u e ( 8 , 14, I 9 ) ( F i g . 4 ) . T h i s p r o c e d u r e p e r m i t s c r e a t i o n o f t h e largest possible shunt between venous lakes or anomalous drainage a r e a s in t h e p o r t a l v e n o u s s y s t e m a n d t h e s y s t e m i c v e n o u s c i r c u l a t i o n . S u b s e q u e n t e d e m a o f t h e l o w e r l i m b s is a r a r e a n d t r a n s i e n t o c c u r rence. Makeshift shunts using mesenteric venous tributaries have been f o l l o w e d b y r e p e a t e d h e m o r r h a g e in e a c h o f 1 l r e p o r t e d c a s e s ( 7 ) . Portal venous anastomoses are susceptible to thrombosis if a c t i v e t h r o n a b o p h l e b i t i s is p r e s e n t . Even though the technical difficulties of successfully constructing a n E c k f i s t u l a in s m a l l p a t i e n t s a r e g r e a t , s u c h p r o c e d u r e s in s e l e c t e d c h i l d r e n m a y b e l i f e s a v i n g . A s u c c e s s f u l s h u n t is t h e o n l y p r o c e d u r e w h i c h is l i k e l y to b e c u r a t i v e in p a t i e n t s w i t h e x t r a h e p a t i c b l o c k . Experimental evidence indicates that venous anastomoses performed i n s m a l l a n i m a l s will g r o w if h a l f o f t h e s u t u r e l i n e c o n s i s t s o f i n t e r r u p t e d s t i t c h e s of fine s i l k ( 2 2 ) . I t s h o u l d b e e m p h a s i z e d t h a t e v e n though Eck fistulae may obviate further variceal hemorrhage, the longterm effects on growth and development and the ultimate prognosis are not yet known. 74
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FIG. 4.1Side-to-end superior ~nesenteric vein to inferior vena cava anastomosis, for patients with extrahepatic venous obstruction in whom portacaval and sptenorenal shunts are not possible.
W h e n t h e v e n o u s o b s t r u c t i o n is so e x t e n s i v e as to p r e c l u d e a s h u n t ing p r o c e d u r e , o r if s e v e r e h e m o r r h a g e r e c u r s f o l l o w i n g a s h u n t , a d i r e c t o p e r a t i v e a p p r o a c h on the v a r i c e s m a y b e n e c e s s a r y . . E n d o s c o p i c i n j e c t i o n of t h e v a r i c e s w i t h s c l e r o s i n g s o l u t i o n s h a s b e e n s u c c e s s f u l in c o n t r o l l i n g the h e m o r r h a g e t e m p o r a r i l y in a f e w s u c h i n s t a n c e s a n d m a y be s a f e r t h a n v a r i c e a l l i g a t i o n ( 9 , 1 0 ) . F r e q u e n t b l a n d f e e d i n g s a n d a n t a c i d s a r e also h e l p f u l in s u c h p a t i e n t s .
INTRAHEPATIC
PORTAL V E N O U S
OBSTRUCTION
P o r t a l h y p e r t e n s i o n d u e to i n t r a h e p a t i c v e n o u s b l o c k c a r r i e s a m u c h less f a v o r a b l e o u t l o o k t h a n e x t r a h e p a t i c o b s t r u c t i o n b e c a u s e of t h e s e r i o u s a n d p r o g r e s s i v e n a t u r e of the p r i m a r y h e p a t i c d i s e a s e . I n t r a 75
h e p a t i c p o r t a l o b s t r u c t i o n is a l m o s t always a s e q u e l a of cirrhosis of the liver w h i c h m a y follow n e o n a t a l hepatitis, e x p o s u r e to toxic a g e n t s or c o n g e n i t a l biliary atresia. G l y c o g e n s t o r a g e diseases m a y o c c a s i o n a l l y p r o d u c e v e n o u s c o m p r e s s i o n with r e s u l t a n t p o r t a l h y p e r t e n s i o n . It is g e n e r a l l y a s s u m e d t h a t p e r i p o r t a l fibrosis, r e g a r d l e s s of the etiology, may produce venous hypertension. T h e o p e r a t i v e m a n a g e m e n t of p o r t a l h y p e r t e n s i o n d u e to i n t r a h e p a t i c b l o c k d e p e n d s o n the p r o g n o s i s in the u n d e r l y i n g liver disease. G a s t r o i n t e s t i n a l h e m o r r h a g e f r o m varices in s u c h p a t i e n t s is m o r e likely to be p r o t r a c t e d and fatal t h a n in t h o s e with e x t r a h e p a t i c block. P o o r liver f u n c t i o n results in m a l n u t r i t i o n a n d h y p o p r o t h r o m b i n e m i a w h i c h c o m p l i c a t e s t r e a t m e n t of a c u t e h e m o r r h a g e . T o x i c i t y f r o m h y p e r a m m o n e m i a , d u e to a b s o r p t i o n of p r o t e i n f r o m b l o o d in t h e large bowel, also c o m p l i c a t e s the c a r e d u r i n g an episode of bleeding. T h e c o u r s e of p o r t a l h y p e r t e n s i o n in p a t i e n t s with biliary c i r r h o s i s c a u s e d by e x t r a h e p a t i c bile d u c t o b s t r u c t i o n , e.g., injury to the c o m m o n bile d u c t or c h o l e d o c h a l cyst When the bite d u c t block is relieved, is n o t c l e a r ( 4 ) . If p o r t a l v e n o u s d e c o m p r e s s i o n is a c c o m p a n i e d b y s u b s e q u e n t i m p r o v e m e n t in h e p a t i c f u n c t i o n as s u g g e s t e d by B l a k e m o r e ( 2 ) , t h e n a s h u n t s h o u l d logically be p e r f o r m e d along w i t h c o r rection of c o m m o n d u c t o b s t r u c t i o n . T h i s has b e e n o u r e x p e r i e n c e in a child with a c h o l e d o c h a l cyst w h o s e initial c o m p l a i n t was v a r i c e a l h e m o r r h a g e ( I ! ). T h e r e is little e v i d e n c e to suggest t h a t c i r r h o s i s a n d p o r t a l h y p e r t e n s i o n due to p o s t i n f e c t i o u s necrosis will s p o n t a n e o u s l y regress. T h e m o s t effective relief of p o r t a l h y p e r t e n s i o n in c h i l d r e n o v e r 4 years of age with i n t r a h e p a t i c o b s t r u c t i o n is o b t a i n e d f r o m a side-toside p o r t a c a v a l a n a s t o m o s i s or f r o m a c e n t r a l l y p l a c e d s p l e n o r e n a t s h u n t ( 6 ) . Y o u n g e r c h i l d r e n s h o u l d be t r e a t e d n o n o p e r a t i v e l y o r by v a r i c e a l injection o r ligation. C a r e f u l p r e o p e r a t i v e p r e p a r a t i o n with diuresis of ascitic fluid, a d m i n i s t r a t i o n of blood, colloid a n d v i t a m i n K a n d r e m o v a l of b l o o d f r o m the g a s t r o i n t e s t i n a l t r a c t are i m p o r t a n t adj u n c t s to successful s u r g e r y in t h e s e p a t i e n t s . It is wise to avoid h e p a t o toxic a n e s t h e t i c a g e n t s a n d to h a v e fresh blood available. In c h i l d r e n with p o r t a l h y p e r t e n s i o n , d u e to p o s t n e c r o t i c or posth e p a t i t i c cirrhosis, or h e p a t i c i n v o l v e m e n t f r o m fibrocystic disease of the p a n c r e a s , i m p r o v e m e n t in n u t r i t i o n , r e g r e s s i o n of e s o p h a g e a l varices a n d d i s a p p e a r a n c e of ascites and h y p e r s p l e n i s m h a v e f r e q u e n t l y f o l l o w e d successful s h u n t p r o c e d u r e s ( 3 ) . C h i l d r e n with p o r t a l h y p e r t e n s i o n d u e to c o n g e n i t a l biliary at r esi a h a v e the least f a v o r a b l e o u t l o o k . S h u n t i n g p r o c e d u r e s Or o t h e r h e r o i c lifesaving o p e r a t i v e m e a s u r e s are g e n e r a l l y n o t advised in these patients w h o s e disease r a r e l y p e r m i t s s u r v i v a l b e y o n d 2 y e a r s of age ( 1 5 ) . T o t a l h e p a t i c h o m o t r a n s p l a n t a t i o n a p p e a r s to be the only p r o m ising a p p r o a c h to the m a n a g e m e n t of p a t i e n t s with this m a l f o r m a t i o n 76
when the technical, immunologic and procurement surmounted.
obstacles have been
SUPRAHI~PATIC PORTAL V E N O U S OBSTRUCTION H y p e r t e n s i o n of t h e p o r t a l v e n o u s s y s t e m is o c c a s i o n a l l y p r o d u c e d by suprahepatic venous obstruction. Gastrointestinal hemorrhage from varices, ascites and hypersplenism with relatively normal liver function may be sequelae of this condition. Hepatic vein obstruction by granulomatosis has occurred in children i n S o u t h A m e r i c a , b u t is o f l i t t l e s i g n i f i c a n c e in t h e U n i t e d S t a t e s . I d i o p a t h i c r e t r o p e r i t o n e a l fibrosis p r o d u c e d c o m p l e t e o c c l u s i o n of the entire i n f e r i o r vena cava a n d o b s t r u c t i o n of the h e p a t i c veins in a 1 4 - y e a r - o l d b o y t r e a t e d in o u r h o s p i t a l ( 1 2 ) . W e w e r e a b l e to d e c o m press the portal v e n o u s s y s t e m s u c c e s s f u l l y in this p a t i e n t by a n a s t o m o s i n g t h e s p l e n i c v e i n to t h e i s o l a t e d p u l m o n a r y a r t e r y o f t h e l e f t l o w e r l o b e o f t h e l u n g b y m e a n s of a n i n t e r v e n i n g T e f l o n g r a f t . Congestive heart failure or constrictiv e pericarditis may occasionally p r o d u c e s u p r a h e p a t i c o b s t r u c t i o n , w h i c h u s u a l l y r e s p o n d s well-'to a p propriate cardiac therapy.
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Surgical Treatment of Portal Hypertension-Long-Term Results WILLIAM
P. L O N G M I R E ,
JR,
THE SURGICAL TREATMENT OF PORTAL HYPERTENSION has b e e n directed primarily t o w a r d the c o n t r o l of bleeding f r o m e s o p h a g e a l v a r i c e s , o n e of t h e s e c o n d a r y effects of t h e e l e v a t e d v e n o u s p r e s s u r e . It is of n o d i r e c t b e n e f i t in t h e t r e a t m e n t of t h e l i v e r d i s e a s e p e r se, a l t h o u g h t h e p r e v e n t i o n of h e m o r r h a g e i n t o t h e g a s t r o i n t e s t i n a l t r a c t will d e c r e a s e t h e l i k e l i h o o d of f u r t h e r d a m a g e to t h e h e p a t i c cells f r o m t h e a b s o r p t i o n i n t o the p o r t a l v e n o u s s y s t e m of t h e p r o d u c t s of b l o o d m e t a b o l i z e d in t h e i n t e s t i n a l t r a c t . A n u m b e r of s t u d i e s ( 1 - 8 ) 78