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Extrahepatic portal hypertension: A review of 70 cases
Extrahepatic Portal Hypertension: A Review of 70 Cases By S. K. Mitra, V. Kumar, D. V. Datta, P. N. Rao, K. Sandhu, G. K. Singh, J. S. Sodhi, and I. C. Pathak 9 Among 70 children with extrahepatic portal hypertension, more than 3 5 0 episodes of bleeding occurred. Of the 32 children who were not operated upon, six (19%) died of bleeding, Twelve children in the nonoperated group are thriving and well, although six of them have rebled 1 - 2 times. The operated group of 38 children had a total of 43 procedures, Central splenorenal and cavomesenteric anastomosis prevented further bleeding in 10 of 12 cases in which follow-up is available. Operative mortality was 24%, the majority of which were in emergency procedures. I N D E X W O R D S : Extrahepatic portal hypertension; splenorenal shunt; cavomesenteric shunt.
URING a period of 8 yr, from 1969 to 1977, 70 children (56 males, 14 females) were treated for extrahepatic portal hypertension (EHPH). Eighty percent of the children presented to us after the age of 6 yr (Table 1). The y o u n g e s t was 3 mo old when first diagnosed. Ten children (14%) had a definite history of neonatal infection.
D
MATERIALS AND METHODS The most frequent initial clinical manifestation of portal hypertension was variceal bleeding, which occurred in 57 children (81%). Of these, the parents of 25 children were aware of a mass in the left hypochondrium since early childhood, and in 23 children the spleen was palpable at the time of admission (Table 2). Fifty children (70%) had their first bleed after the age of 6 yr, while 28 children (40%) had their first bleed after the age of 10 yr. Three girls aged 7 yr, 11 yr, and 14 yr, died during their first attack in our hospital. Two boys and one girl died of repeated bleeds in remote villages, two of which were cut off by snow in the hills. The average total number of bleeds per patient was 5.1; one patient experienced 15 episodes of hemorrhage. Transfusions were given during approximately one-half of the bleeding episodes, and the majority required 1 1.5 liters of blood for resuscitation. Only 10 children required more than 2.5 liters of blood. Splenomegaly alone was the second m o s t common initial manifestation of E H P H and occurred in 13 cases (18.6%). At least three of these children have been followed up to 60 mo and have not bled yet. One child, first seen at the age of 14 yr, subsequently bled massively at the age of 19 yr. It might be of interest to state here that there are a n u m b e r of adults with E H P H in our institution series, which shows that a significant n u m b e r of patients who bleed the first time after the age of 20 were in fact aware of a m a s s in the left hypochondrium since early childhood.
Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978
Routine blood examination and liver function tests were carried out in all cases. Esophagogram was performed in 61 cases. Splenoportal venogram was done in 50 cases. Splenic pulp pressure was recorded in 38 cases. Liver biopsy was done in 42 cases. Liver function tests were normal in all. Anemia (hemoglobin < 10 g/100ml) was recorded in more than 80% of the cases. Children who had had a bleed in the recent past, had hemoglobin (Hb) as low as 2 5 g/100 ml at the time of admission. Some also had mild ascites. Evidence of hypersplenism was present in 30 cases, manifested by a leukocyte count of less than 2 0 0 0 / m m 3 and a platelet count of less than 100,000/mm a. There has been no case of splenectomy for the sake of hypersp|enism alone in this series. Table 3 shows that the portal venous pressure values ranged from 180 to 520 m m of saline; the majority however, were more than 300 m m of saline. There was no correlation between age and portal pressure, portal pressure and number and severity of bleeds, size of spleen and number of bleeds, or size of spleen and portal pressure. Splenoportovenogram showed a block in the splenic vein in seven cases, and a block in the portal vein region in 43 cases, of which 22 had classic cavernomatous malformation. There was no set pattern of collateral circulation, nor could they be correlated to either portal pressure and age or size of the spleen. Esophageal and g a s t r i c varices were demonstrated in 73% of cases, whereas 14% were reported as normal. Another important finding in this series is that at least seven children definitely had a history of jaundice after 3-4 blood transfusions, which could probably be attributed to serum hepatitis, Australia antigen being positive in three cases.
Treatment Acute bleeding episodes have by and large been controlled by deep sedation, ice cold gastric lavage, oral antacids, and vitamin K administration. Intravenous pitressin has also been used in a few. Esophagogastric baloon tamponade was rarely needed; in fact, it was used in two of the
From the Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Presented before the X X I V Annual International Congress of the British Association of Paediatric Surgeons, Oslo, Norway, August 2-6, 1977. Address reprint requests to S. K. Mitra, F.R.C.S., Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 9 1978 by Grune & Stratton, Inc. 0022-3468/78/1301-0012501.00/0 51
52
MITRA ET AL.
Table 1. Age and Sex Distribution of Cases of EHPH Male
Table 3. Portal Vein Pressure Measurement in 38 Cases
Percent
Salines Pressure (mm)
Cases
Group
Age
Female
Total Cases
1
0-2
2
1
3
4,3
0-199
1
2
2-4
3
1
4
5.7
200-300
7 21
3
4-6
5
2
7
10.1
301-400
4
6-10
22
1
23
32.8
401-500
7
5
10-14
24
9
33
47.1
500
2
56
14
70
Total
100
Total
three children who died in their first attack of hematemesis in the hospital because of shortage of blood. Earlier in the series we tried emergency shunt surgery and various other temporary surgical procedures. A total of 13 operations were performed with a prohibitive mortality of 60%. Our present policy is to treat the patient medically and offer surgery only if he has bled on more than three occasions, is coming from far away, or does not have easy access to a blood transfusion center. A variety of methods was used (Table 4). The average interval between the first bleed and surgery has been 1.5 yr. Of the 25 children who underwent elective procedures there have been no operative mortalities. As our children are of an older age group, we have attempted a portal systemic shunt whenever possible, and a central splenorenal' or side-to-side lienorenal without splenectomy z has been our first choice. Mesocaval shunt is next in the order of preference, but on at least four occasions the disease process involved the main superior mesenteric vein, and a shunt procedure could not be carried out. The side-to-side lienorenal shunt has been performed whenever the size of the spleen has been moderate and it was possible to retract the spleen laterally and cephalad to expose the pancreatic region easily at the time of operation. It was possible to construct a shunt more than 1.5 cm in diameter even if the splenic vessel was less than 1 cm in diameter. Three of the six patients were under the age of 5 yr.
Splenectomy and devascularization of the stomach was carried out earlier in the series when no adequate vein was available for a shunt. Currently, we prefer to do only devascularization of the stomach without splenectomy because 0f the reported infection after splenectomy in children 8 and also because an adequate vein may be found at a iater age. We, however, have not had any major infection attributable to splenectomy. Table 2. Presenting Features in Cases of EHPH Obstruction Age (yr) Symptoms
O-1
2-5
5-10
10-14
Total
Splenomegaly + GI hemorrhage
1
5
22
28
56
Splenomegaly
--
2
7
4
13
GI hemorrhage
--
1
--
--
Anemia
1
7
24
29
61
Ascites
--
3
5
12
Abdominal veins
--
--
--
2
2
Palpable liver only
--
5
10
10
25
Hypersplenism
--
3
9
14
26
4
1
38
RESULTS (TABLE 4) O f t h e 13 c e n t r a l lienorenal shunts, two w e r e done as e m e r g e n c y p r o c e d u r e s . Both patients died in the i m m e d i a t e p o s t o p e r a t i v e period with m a s s i v e bleeding. O f 11 e l e c t i v e p r o c e d u r e s , o n e bled in the i m m e d i a t e p o s t o p e r a t i v e period and has s u b s e q u e n t l y u n d e r g o n e B o e r e m a - C r i l e esophageal ligation o f varices. F o u r h a v e been lost to follow-up. O f t h e r e m a i n i n g seven, one bled after 3 yr and has also u n d e r g o n e a B o e r e m a - C r i l e operation. T h e r e m a i n i n g six h a v e done well, h a v e not bled, and a r e showing a normal growth pattern. W e h a v e been p a r t i c u l a r l y i m p r e s s e d by the s i d e - t o - s i d e l i e n o r e n a l s h u n t w i t h o u t splen e c t o m y . A l t h o u g h the n u m b e r is only six and t h e longest follow-up is only 4 yr, none o f the children h a v e bled so far. T h e i r spleens have cer t ai n l y shrunk. T h e only case t h a t we have restudied after 4 yr shows a p a t e n t shunt. This child was 3 ~ yr old when he u n d e r w e n t surgery. Tw o o f t h e s e six children h ad a p e r s i s t e n t discharging sinus for m o r e than 6 m o from the left flank drain site. W e w e r e u n a b l e to find a c a u s e for this but cer t ai n l y could n o t label this as o f p a n c r e a t i c origin. T h e only case o f e m e r g e n c y m e s o c a v a l shunt died on the table. O f the five who a r e available for follow-up, o n e bled after 2 yr and has und e r g o n e a B o e r e m a - C r i l e o p e r a t i o n . This child and also a n o t h e r child had f e a t u r e s a t t r i b u t a b l e to s u b a c u t e intestinal o b s t r u c t i o n , although no s u r g e r y was r e q u i r e d to relieve t h e m . T h e only case who had a 22 m m b o r e woven D a c r o n g r a f t i n t e r p o s e d b et w een inferior cava and superior m e s e n t e r i c vein 4 r e b l e d massively within 3 mo and u n d e r w e n t d e v a s c u l a r i z a t i o n of the s t o m a c h , is only 1 yr p o s t o p e r a t i v e , and has not bled so far.
DISCUSSION T h e n a t u r a l h i st o r y o f this d i s e a s e is still confusing. 3,5 In c o n t r a s t to o t h e r r e p o r t s , 3,~ m o r e
EXTRAHEPATIC PORTAL HYPERTENSION
53 Table 4. Overall Results of 70 Cases
Procedure
No. N
Immediate Death
Follow-up Available
Rebleed
Reoperated
Late Death
End-to-side central lienorenal shunt Side-to-side lienorenal shunt
13
2
7
4
2
0
6
0
6
0
0
0
Cavomesenteric shunt Splenectomy and devascularization of stomach Devascularization of stomach Boerema-crile transthoracic ligation of esophageal varices Mesocaval graft interposition Nonoperated
8 8
1 2
5 4
1 2
1 1
0 2
4 3
1 1
2 2
0 1
0 0
0 0
1
0
1
1
1
32
3
12
6
--
0 3
than 70% of the children in this series had their first bleed after the sixth yr of life. The explanation is not clear. The incidence of neonatal infection seems to be the same as in other reports. 3,~ The high incidence of infective diarrhea may result in endophlebitis in the portal circulation due to absorption of toxin and bacteria that could lead to thrombosis and portal hypertension) We are in agreement that a significant n u m b e r of children, if managed medically, would eventually stop having bleeding episodes. 3,7 We have also seen a considerable proportion of adult patients with this disease, who, in spite of the fact that they were aware of the enlarged spleen since early childhood, have bled for the first time after their late teens and have continued to bleed after that, eventually requiring surgery. A bout of chest infection or fever due to any other cause seems to trigger off a bleed. Chronic anemia and some degree of malnutrition may be the reason that in many of the bleeding episodes, blood transfusion was necessary to maintain vital signs, c o n t r a r y to the r e p o r t s of others. 3'7's The parents were of course warned not to give aspirin. In our unoperated series, at least six children succumbed directly to bleeding, a mortality of 20%. The operative mortality has been 24%, but one has to keep in mind that many of these have been emergency operations done earlier in the series. We have not done any emergency operations in the last 4 yr. We agree with Shaldon and Sherlock r that on most occasions it is possible to control bleeding with nono p e r a t i v e m e a s u r e s . H o w e v e r , if bleeding continues even after i.v. pitressin and baloon tamponade, temporary gastric devasculariza-
Other Complications Subdiaphragmatic abscess (1 case) wound sepsis (2 cases) Chronic discharging abdominal drain site (2) Subacute intestinal obstruction (2)
Viral hepatitis (6)
tion through an abdominal approach is our operation of choice at present, and three of the four we have done so far have stopped bleeding, at least temporarily. One died on the operation table, probably because it was too late to take him for surgery. Currently, the following criteria have been used as indications for elective surgery: (1) A child who has bled more than three times. (2) A child who has bled on more than one occasion and also has features of severe hypersplenism. (3) A child who is unlikely to reach a blood transfusion service in time of need. Wherever possible, we have attempted to do a s h u n t p r o c e d u r e . Mainly b e c a u s e of nonavailability of portal vein and also because of the fear of encephalopathy, 3'~ we have never attempted portacaval shunt. If the spleen is huge and the child also had evidence of hypersplenism, central end-to-side lienorenal shunt t was performed. If the spleen was not big and hypersplenism has not been a major problem, we performed side-to-side lienorenal shuntJ With both p r o c e d u r e s it was possible to c o n s t r u c t a shunt g r e a t e r than 1 cm in diameter. The t h r e e end-to-side lienorenal shunts that bled in the immediate postoperative period were done earlier in the series, and due to lack of experience were probably not true central shunts. At least two of these were done as emergency procedures. We have been particularly impressed by the side-to-side lienorenal shunt without splen e c t o m y ? This has a great advantage in that it is possible to construct a shunt more than 1.5
54
MITRA ET AL.
cm in d i a m e t e r even if the p a r e n t vessels a r e less t h a n 1 cm in d i a m e t e r . T h e y o u n g e s t p a t i e n t on whom we h a v e done this p r o c e d u r e is a 3 - y r - o l d boy who bled r e p e a t e d l y and had vessels roughly 8 m m in d i a m e t e r , Two o t h e r children w e r e u n d e r t h e age of 5 yr. All six children on w h o m this p r o c e d u r e was p e r f o r m e d a r e doing well, and their spleens a r e not p a l p a ble. W h e n e v e r the splenic vein was not visualized on s p l e n o p o r t o g r a m , an i n t r a o p e r a t i v e s u p e r i o r m e s e n t e r i c o v e n o g r a m was p e r f o r m e d to m a k e sure t h a t we w e r e not dealing with isolated splenic vein t h r o m b o s i s . T h r e e o f the children who w e r e r e p o r t e d to have a block in t h e splenic vein on s p l e n o p o r t o v e n o g r a p h y at l a p a r o t o m y w e r e found to have extensive d i s e a s e o f the p o r t a l venous s y s t e m , w h e r e even m e s o c a v a l
shunt could not be c o n s t r u c t e d . T h e r e s t o f the t h r e e with r e p o r t e d splenic vein block have not u n d e r g o n e surgery; h e n c e we a r e u n a b l e to c o m m e n t on them. W e have not p e r f o r m e d celiac axis a n g i o g r a m on any o f these. ~ Since o u r follow-up is v e r y short, we c a n n o t c o m m e n t on e n c e p h a l o p a t h y . T h e biggest d r a w b a c k o f this series is t h a t 24% of the o p e r a t e d series and 23% o f t h e n o n o p e r a t e d series h a v e been lost for follow-up, but with the available d a t a the following policies w e r e p r o p o s e d : (1) E m e r g e n c y s u r g e r y is b e s t avoided. (2) W i t h p r o p e r m e d i c a l m a n a g e m e n t a child could grow to an o l d e r age g r o u p when a suita b l e vein m a y be available for shunt. (3) In a small child with r e p e a t e d bleeding, a side-to-side lienorenal shunt or central lienorenal shunt should be offered.
REFERENCES
1. Clatworthy HW, DeLorimer AA: Portal decompression procedures in children. Am J Surg 107:447, 1964 2. Cooley DA: Side-to-side spleno-renal anastomosis with splenic preservation for portal hypertension. Surg Gynecol Obstet 116:626, 1963 3. Fonkalsrud EW, Myers NA, Robinson M J: Management of extrahepatic portal hypertension in children. Ann Surg 180:487, 1974 4. Thompson BW, Read RC, Cassali RE: Interposition grafting for portal hypertension. Am J Surg 130:733, 1975 5. Malt RA: Shunts for hepatic disease, in Bouchier IAO
(ed): Recent Advances in Gastroenterology (ed 3). Edinburgh, Churchill Livinstone, 1976 6. Basu AK, Guharay BN: Profile on portal hypertension in India with or without cirrhosis of liver, in Ahuja MMS (ed): Progress in clinical medicine. New Delhi, ArnoldHeine Mann, 1976 7. Shaldon S, Sherlock S: Obstruction to the extrahepatic portal system in childhood. Lancet 1:63, 1962 8. Voorhees AB Jr, Price JB Jr: Extrahepatic portal hypertension. A retrospective analysis of 127 cases and associated clinical implications. Arch Surg 108:338, 1974
URING a period of 8 yr, from 1969 to 1977, 70 children (56 males, 14 females) were treated for extrahepatic portal hypertension (EHPH). Eighty percent of the children presented to us after the age of 6 yr (Table 1). The y o u n g e s t was 3 mo old when first diagnosed. Ten children (14%) had a definite history of neonatal infection.
D
MATERIALS AND METHODS The most frequent initial clinical manifestation of portal hypertension was variceal bleeding, which occurred in 57 children (81%). Of these, the parents of 25 children were aware of a mass in the left hypochondrium since early childhood, and in 23 children the spleen was palpable at the time of admission (Table 2). Fifty children (70%) had their first bleed after the age of 6 yr, while 28 children (40%) had their first bleed after the age of 10 yr. Three girls aged 7 yr, 11 yr, and 14 yr, died during their first attack in our hospital. Two boys and one girl died of repeated bleeds in remote villages, two of which were cut off by snow in the hills. The average total number of bleeds per patient was 5.1; one patient experienced 15 episodes of hemorrhage. Transfusions were given during approximately one-half of the bleeding episodes, and the majority required 1 1.5 liters of blood for resuscitation. Only 10 children required more than 2.5 liters of blood. Splenomegaly alone was the second m o s t common initial manifestation of E H P H and occurred in 13 cases (18.6%). At least three of these children have been followed up to 60 mo and have not bled yet. One child, first seen at the age of 14 yr, subsequently bled massively at the age of 19 yr. It might be of interest to state here that there are a n u m b e r of adults with E H P H in our institution series, which shows that a significant n u m b e r of patients who bleed the first time after the age of 20 were in fact aware of a m a s s in the left hypochondrium since early childhood.
Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978
Routine blood examination and liver function tests were carried out in all cases. Esophagogram was performed in 61 cases. Splenoportal venogram was done in 50 cases. Splenic pulp pressure was recorded in 38 cases. Liver biopsy was done in 42 cases. Liver function tests were normal in all. Anemia (hemoglobin < 10 g/100ml) was recorded in more than 80% of the cases. Children who had had a bleed in the recent past, had hemoglobin (Hb) as low as 2 5 g/100 ml at the time of admission. Some also had mild ascites. Evidence of hypersplenism was present in 30 cases, manifested by a leukocyte count of less than 2 0 0 0 / m m 3 and a platelet count of less than 100,000/mm a. There has been no case of splenectomy for the sake of hypersp|enism alone in this series. Table 3 shows that the portal venous pressure values ranged from 180 to 520 m m of saline; the majority however, were more than 300 m m of saline. There was no correlation between age and portal pressure, portal pressure and number and severity of bleeds, size of spleen and number of bleeds, or size of spleen and portal pressure. Splenoportovenogram showed a block in the splenic vein in seven cases, and a block in the portal vein region in 43 cases, of which 22 had classic cavernomatous malformation. There was no set pattern of collateral circulation, nor could they be correlated to either portal pressure and age or size of the spleen. Esophageal and g a s t r i c varices were demonstrated in 73% of cases, whereas 14% were reported as normal. Another important finding in this series is that at least seven children definitely had a history of jaundice after 3-4 blood transfusions, which could probably be attributed to serum hepatitis, Australia antigen being positive in three cases.
Treatment Acute bleeding episodes have by and large been controlled by deep sedation, ice cold gastric lavage, oral antacids, and vitamin K administration. Intravenous pitressin has also been used in a few. Esophagogastric baloon tamponade was rarely needed; in fact, it was used in two of the
From the Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Presented before the X X I V Annual International Congress of the British Association of Paediatric Surgeons, Oslo, Norway, August 2-6, 1977. Address reprint requests to S. K. Mitra, F.R.C.S., Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 9 1978 by Grune & Stratton, Inc. 0022-3468/78/1301-0012501.00/0 51
52
MITRA ET AL.
Table 1. Age and Sex Distribution of Cases of EHPH Male
Table 3. Portal Vein Pressure Measurement in 38 Cases
Percent
Salines Pressure (mm)
Cases
Group
Age
Female
Total Cases
1
0-2
2
1
3
4,3
0-199
1
2
2-4
3
1
4
5.7
200-300
7 21
3
4-6
5
2
7
10.1
301-400
4
6-10
22
1
23
32.8
401-500
7
5
10-14
24
9
33
47.1
500
2
56
14
70
Total
100
Total
three children who died in their first attack of hematemesis in the hospital because of shortage of blood. Earlier in the series we tried emergency shunt surgery and various other temporary surgical procedures. A total of 13 operations were performed with a prohibitive mortality of 60%. Our present policy is to treat the patient medically and offer surgery only if he has bled on more than three occasions, is coming from far away, or does not have easy access to a blood transfusion center. A variety of methods was used (Table 4). The average interval between the first bleed and surgery has been 1.5 yr. Of the 25 children who underwent elective procedures there have been no operative mortalities. As our children are of an older age group, we have attempted a portal systemic shunt whenever possible, and a central splenorenal' or side-to-side lienorenal without splenectomy z has been our first choice. Mesocaval shunt is next in the order of preference, but on at least four occasions the disease process involved the main superior mesenteric vein, and a shunt procedure could not be carried out. The side-to-side lienorenal shunt has been performed whenever the size of the spleen has been moderate and it was possible to retract the spleen laterally and cephalad to expose the pancreatic region easily at the time of operation. It was possible to construct a shunt more than 1.5 cm in diameter even if the splenic vessel was less than 1 cm in diameter. Three of the six patients were under the age of 5 yr.
Splenectomy and devascularization of the stomach was carried out earlier in the series when no adequate vein was available for a shunt. Currently, we prefer to do only devascularization of the stomach without splenectomy because 0f the reported infection after splenectomy in children 8 and also because an adequate vein may be found at a iater age. We, however, have not had any major infection attributable to splenectomy. Table 2. Presenting Features in Cases of EHPH Obstruction Age (yr) Symptoms
O-1
2-5
5-10
10-14
Total
Splenomegaly + GI hemorrhage
1
5
22
28
56
Splenomegaly
--
2
7
4
13
GI hemorrhage
--
1
--
--
Anemia
1
7
24
29
61
Ascites
--
3
5
12
Abdominal veins
--
--
--
2
2
Palpable liver only
--
5
10
10
25
Hypersplenism
--
3
9
14
26
4
1
38
RESULTS (TABLE 4) O f t h e 13 c e n t r a l lienorenal shunts, two w e r e done as e m e r g e n c y p r o c e d u r e s . Both patients died in the i m m e d i a t e p o s t o p e r a t i v e period with m a s s i v e bleeding. O f 11 e l e c t i v e p r o c e d u r e s , o n e bled in the i m m e d i a t e p o s t o p e r a t i v e period and has s u b s e q u e n t l y u n d e r g o n e B o e r e m a - C r i l e esophageal ligation o f varices. F o u r h a v e been lost to follow-up. O f t h e r e m a i n i n g seven, one bled after 3 yr and has also u n d e r g o n e a B o e r e m a - C r i l e operation. T h e r e m a i n i n g six h a v e done well, h a v e not bled, and a r e showing a normal growth pattern. W e h a v e been p a r t i c u l a r l y i m p r e s s e d by the s i d e - t o - s i d e l i e n o r e n a l s h u n t w i t h o u t splen e c t o m y . A l t h o u g h the n u m b e r is only six and t h e longest follow-up is only 4 yr, none o f the children h a v e bled so far. T h e i r spleens have cer t ai n l y shrunk. T h e only case t h a t we have restudied after 4 yr shows a p a t e n t shunt. This child was 3 ~ yr old when he u n d e r w e n t surgery. Tw o o f t h e s e six children h ad a p e r s i s t e n t discharging sinus for m o r e than 6 m o from the left flank drain site. W e w e r e u n a b l e to find a c a u s e for this but cer t ai n l y could n o t label this as o f p a n c r e a t i c origin. T h e only case o f e m e r g e n c y m e s o c a v a l shunt died on the table. O f the five who a r e available for follow-up, o n e bled after 2 yr and has und e r g o n e a B o e r e m a - C r i l e o p e r a t i o n . This child and also a n o t h e r child had f e a t u r e s a t t r i b u t a b l e to s u b a c u t e intestinal o b s t r u c t i o n , although no s u r g e r y was r e q u i r e d to relieve t h e m . T h e only case who had a 22 m m b o r e woven D a c r o n g r a f t i n t e r p o s e d b et w een inferior cava and superior m e s e n t e r i c vein 4 r e b l e d massively within 3 mo and u n d e r w e n t d e v a s c u l a r i z a t i o n of the s t o m a c h , is only 1 yr p o s t o p e r a t i v e , and has not bled so far.
DISCUSSION T h e n a t u r a l h i st o r y o f this d i s e a s e is still confusing. 3,5 In c o n t r a s t to o t h e r r e p o r t s , 3,~ m o r e
EXTRAHEPATIC PORTAL HYPERTENSION
53 Table 4. Overall Results of 70 Cases
Procedure
No. N
Immediate Death
Follow-up Available
Rebleed
Reoperated
Late Death
End-to-side central lienorenal shunt Side-to-side lienorenal shunt
13
2
7
4
2
0
6
0
6
0
0
0
Cavomesenteric shunt Splenectomy and devascularization of stomach Devascularization of stomach Boerema-crile transthoracic ligation of esophageal varices Mesocaval graft interposition Nonoperated
8 8
1 2
5 4
1 2
1 1
0 2
4 3
1 1
2 2
0 1
0 0
0 0
1
0
1
1
1
32
3
12
6
--
0 3
than 70% of the children in this series had their first bleed after the sixth yr of life. The explanation is not clear. The incidence of neonatal infection seems to be the same as in other reports. 3,~ The high incidence of infective diarrhea may result in endophlebitis in the portal circulation due to absorption of toxin and bacteria that could lead to thrombosis and portal hypertension) We are in agreement that a significant n u m b e r of children, if managed medically, would eventually stop having bleeding episodes. 3,7 We have also seen a considerable proportion of adult patients with this disease, who, in spite of the fact that they were aware of the enlarged spleen since early childhood, have bled for the first time after their late teens and have continued to bleed after that, eventually requiring surgery. A bout of chest infection or fever due to any other cause seems to trigger off a bleed. Chronic anemia and some degree of malnutrition may be the reason that in many of the bleeding episodes, blood transfusion was necessary to maintain vital signs, c o n t r a r y to the r e p o r t s of others. 3'7's The parents were of course warned not to give aspirin. In our unoperated series, at least six children succumbed directly to bleeding, a mortality of 20%. The operative mortality has been 24%, but one has to keep in mind that many of these have been emergency operations done earlier in the series. We have not done any emergency operations in the last 4 yr. We agree with Shaldon and Sherlock r that on most occasions it is possible to control bleeding with nono p e r a t i v e m e a s u r e s . H o w e v e r , if bleeding continues even after i.v. pitressin and baloon tamponade, temporary gastric devasculariza-
Other Complications Subdiaphragmatic abscess (1 case) wound sepsis (2 cases) Chronic discharging abdominal drain site (2) Subacute intestinal obstruction (2)
Viral hepatitis (6)
tion through an abdominal approach is our operation of choice at present, and three of the four we have done so far have stopped bleeding, at least temporarily. One died on the operation table, probably because it was too late to take him for surgery. Currently, the following criteria have been used as indications for elective surgery: (1) A child who has bled more than three times. (2) A child who has bled on more than one occasion and also has features of severe hypersplenism. (3) A child who is unlikely to reach a blood transfusion service in time of need. Wherever possible, we have attempted to do a s h u n t p r o c e d u r e . Mainly b e c a u s e of nonavailability of portal vein and also because of the fear of encephalopathy, 3'~ we have never attempted portacaval shunt. If the spleen is huge and the child also had evidence of hypersplenism, central end-to-side lienorenal shunt t was performed. If the spleen was not big and hypersplenism has not been a major problem, we performed side-to-side lienorenal shuntJ With both p r o c e d u r e s it was possible to c o n s t r u c t a shunt g r e a t e r than 1 cm in diameter. The t h r e e end-to-side lienorenal shunts that bled in the immediate postoperative period were done earlier in the series, and due to lack of experience were probably not true central shunts. At least two of these were done as emergency procedures. We have been particularly impressed by the side-to-side lienorenal shunt without splen e c t o m y ? This has a great advantage in that it is possible to construct a shunt more than 1.5
54
MITRA ET AL.
cm in d i a m e t e r even if the p a r e n t vessels a r e less t h a n 1 cm in d i a m e t e r . T h e y o u n g e s t p a t i e n t on whom we h a v e done this p r o c e d u r e is a 3 - y r - o l d boy who bled r e p e a t e d l y and had vessels roughly 8 m m in d i a m e t e r , Two o t h e r children w e r e u n d e r t h e age of 5 yr. All six children on w h o m this p r o c e d u r e was p e r f o r m e d a r e doing well, and their spleens a r e not p a l p a ble. W h e n e v e r the splenic vein was not visualized on s p l e n o p o r t o g r a m , an i n t r a o p e r a t i v e s u p e r i o r m e s e n t e r i c o v e n o g r a m was p e r f o r m e d to m a k e sure t h a t we w e r e not dealing with isolated splenic vein t h r o m b o s i s . T h r e e o f the children who w e r e r e p o r t e d to have a block in t h e splenic vein on s p l e n o p o r t o v e n o g r a p h y at l a p a r o t o m y w e r e found to have extensive d i s e a s e o f the p o r t a l venous s y s t e m , w h e r e even m e s o c a v a l
shunt could not be c o n s t r u c t e d . T h e r e s t o f the t h r e e with r e p o r t e d splenic vein block have not u n d e r g o n e surgery; h e n c e we a r e u n a b l e to c o m m e n t on them. W e have not p e r f o r m e d celiac axis a n g i o g r a m on any o f these. ~ Since o u r follow-up is v e r y short, we c a n n o t c o m m e n t on e n c e p h a l o p a t h y . T h e biggest d r a w b a c k o f this series is t h a t 24% of the o p e r a t e d series and 23% o f t h e n o n o p e r a t e d series h a v e been lost for follow-up, but with the available d a t a the following policies w e r e p r o p o s e d : (1) E m e r g e n c y s u r g e r y is b e s t avoided. (2) W i t h p r o p e r m e d i c a l m a n a g e m e n t a child could grow to an o l d e r age g r o u p when a suita b l e vein m a y be available for shunt. (3) In a small child with r e p e a t e d bleeding, a side-to-side lienorenal shunt or central lienorenal shunt should be offered.
REFERENCES
1. Clatworthy HW, DeLorimer AA: Portal decompression procedures in children. Am J Surg 107:447, 1964 2. Cooley DA: Side-to-side spleno-renal anastomosis with splenic preservation for portal hypertension. Surg Gynecol Obstet 116:626, 1963 3. Fonkalsrud EW, Myers NA, Robinson M J: Management of extrahepatic portal hypertension in children. Ann Surg 180:487, 1974 4. Thompson BW, Read RC, Cassali RE: Interposition grafting for portal hypertension. Am J Surg 130:733, 1975 5. Malt RA: Shunts for hepatic disease, in Bouchier IAO
(ed): Recent Advances in Gastroenterology (ed 3). Edinburgh, Churchill Livinstone, 1976 6. Basu AK, Guharay BN: Profile on portal hypertension in India with or without cirrhosis of liver, in Ahuja MMS (ed): Progress in clinical medicine. New Delhi, ArnoldHeine Mann, 1976 7. Shaldon S, Sherlock S: Obstruction to the extrahepatic portal system in childhood. Lancet 1:63, 1962 8. Voorhees AB Jr, Price JB Jr: Extrahepatic portal hypertension. A retrospective analysis of 127 cases and associated clinical implications. Arch Surg 108:338, 1974