Extrahepatic Portal Hypertension: A Retrospective Analysis SHEILA E. GRAUER, M.D., SEYMOUR 1. SCHWARTZ, M.D., F.A.C.S.
A retrospective analysis of 19 patients with esophagogastric or intestinal varices secondary to extrahepatic portal hypertension was conducted. Nine patients had onset of bleeding during childhood. In six of eight pediatric patients in whom a shunt was performed no major bleeding occurred during the follow-up period. In seven adults with pure extrahepatic portal hypertension the four shunting procedures effected permanent control of bleeding. In two of three adults in whom portal vein thrombosis was associated with liver disease, shunting was successful. An emergency procedure was infrequently required. Multiple recurrences of bleeding episodes prior to decompression was the rule. Postshunt encephalopathy was never seen. In those patients in whom a shunt thrombosed or could not be performed, recurrent bleeding occurred uncommonly and was readily managed by bedrest and transfusion.
E XTRAHEPATIC portal hypertension, usually caused by thrombosis or cavernomatous transformation of the portal vein, is a relatively uncommon cause of bleeding gastroesophageal or intestinal varices, but it is one which merits special consideraton. The salient questions concerning this disorder relate to the general prognosis, including the course of the nonshunted patient, the applicability of decompressive shunting procedures and the risk of consequent encephalopathy. Nineteen patients with extrahepatic portal hypertension were managed at Strong Memorial Hospital between 1955 and 1978. During the same period approximately 260 portal- systemic shunts were performed for portal hypertension secondary to cirrhosis or other liver diseases. The patients' charts and radiographs were reviewed, and an assessment of their course was brought up to date whenever possible. Presented at the Annual Meeting of the Southern Surgical Association, December 4-6, 1978, Hot Springs, Virginia. Reprint requests: Seymour I. Schwartz, M.D., Department of Surgery; University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, New York 14642. Submitted for publication: December 7, 1978.
From The University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital, Rochester, New York
Clinical Data
It is appropriate to categorize the patients in three groups: 1) patients whose first bleeding episode occurred during childhood, 2) patients who first bled during adult life and 3) patients in whom the bleeding varices and extrahepatic portal hypertension were associated with significant intrahepatic pathology.
Childhood Onset of Bleeding Nine patients experienced their first bleeding episode early in life, before the age of six (Table 1). Seven patients bled during the first two years of life. Two of these (R.Y. and L.T.) were known to have had neonatal omphalitis, and two (S.C. and C.W.) were the product of difficult deliveries and may have sustained umbilical cord trauma. The liver function was normal in all patients. Five of the eight patients in whom platelet counts were performed had thrombocytopenia below 80,000/cu mm. Six patients with esophagogastric varices, all of whom bled by age two, had successful decompression during childhood. This group had a mean of three and one half bleeds prior to the definitive procedure. Three patients had undergone previous operations to control bleeding. In one patient, transesophageal ligation of varices at age one was effective for 3 years. In another patient distal esophagectomy-proximal gastrectomy controlled bleeding for eight years. Significant rebleeding in these patients necessitated the shunting procedures. The third patient had undergone a splenorenal shunt in another hospital but continued to bleed massively as a consequence of thrombosis of that shunt, and this led to an emergency mesocaval shunt.
0003-4932/79/0500/0566 $00.95 © J. B. Lippincott Company
566
EXTRAHEPATIC PORTAL HYPERTENSION
Vol. 189 * No. 5
567
TABLE 1. Extrahepatic Portal Hypertension Onset of Bleeding During Childhood in Nine Cases
Pt./Sex Esophagogastric varices
S.C./F J.L./F R.C./M R.Y./M
Age 1st Bleed
Previous Procedures (Age)
# Bleeds
S.B./M S.C./M L.T./F
2 1 1 1 2 2 4
2 6 6
R.H./M
2
7
C.W./F
5
2
4 2 2 4
T. E. ligation (1)
Splenorenal (19) Esophagogastrectomy (2) Splenectomy (12) T. E. ligation (14) Splenectomy (12) 3 Attempted shunts (13-14)
Treatment (Age) Mesocaval (5) Mesocaval (5)
Splenorenal (12) Mesocaval (4) Mesocaval (19) Splenorenal (10) Mesocaval attempted
Course 8 yr. 0 bleed 4 yr. 0 bleed 8 yr. 0 bleed 14 yr. 0 bleed 16 yr. 0 bleed 14 yr. 0 bleed 2 yr. 1 bleed
(24)
(27)
5 yr. 0 bleed
T. E. ligation (19)
Colon varices
Inf. mesocaval (19)
6 yr. I Minor bleed
The diagnosis was confirmed angiographically in these patients (Figs. I and 2). In all six patients a significant reduction in superior mesenteric pressure was demonstrated intraoperatively. The mean preshunt mesenteric venous pressure was 44 cm saline, while the postshunt pressures had a mean of 16 cm saline. There has been no bleeding subsequent to the operation in any of these patients for periods ranging between four and 16 years (Fig. 3). There has not been a single episode of postshunt encephalopathy in these patients. One patient who has been followed to adulthood is a college student, while another is a history professor. Two patients have had psychologic and social prob-
lems. Two patients (L.T. and R.H.) were first seen at our hospital with a history of multiple bleeds, each having been subjected to several surgical procedures. In both instances the most recent bleeding episode, which led to their admission was associated with ingestion of aspirin. One patient received a transfusion and has not bled over the ensuing five years. The other patient was explored, but extensive peritoneal adhesions precluded a proposed mesocaval shunt. She has bled once, not requiring transfusion, over the past two years. One patient with varices of the rectosigmoid colon, but no esophagogastric varices, first noted rectal
FIG. 1. Splenoportogram demonstrating cavernomatous transformation of portal vein, collaterals and large esophagogastric
FIG. 2. Venous phase of superior mesenteric artery angiogram demonstrating thrombosis of the portal vein and veins of Sappey coursing to the'liver.
varices.
568
GRAUER AND SCHWARTZ
FIG. 3. Venous phase of superior mesenteric angiogram showing patent end-to-side mesocaval shunt and absence of collaterals including varices (same patient as in Fig. 2).
FIG. 4. Thrombosis ofthe portal vein causing marked dilatation of the inferior mesenteric vein and associated varices of the colon. years
later during
lowing a term bleeding at age five. A hemorroidectomy performed for recurrent bleeding at age 19 was accompanied by a major blood loss, which led to the consideration of portal hypertension. A splenoportogram demonstrating a distended inferior mesenteric vein and obstruction of the portal vein (Fig. 4) confirmed the diagnosis. At operation the inferior mesenteric venous pressure was determined to be 54 cm saline. A side-toside inferior mesenteric-inferior venacaval shunt reduced this pressure to 14 cm saline. She has had one minor bleed since this procedure, and shunt patency and persistent pressure reduction were confirmed two
Ann. Surg. X May 1979
laparotomy for tubal ligation
fol-
pregnancy.
Adult Onset of Bleeding
Seven patients with portal vein thrombosis had consequent esophagogastric varices which became
manifest during adult life (Table 2). Liver function tests were normal in all, and five of the six patients in whom measurements were made had platelet counts
less than 80,000/cu mm. A 21-year-old man being evaluated for thrombocytopenia had a splenoportogram demonstrating esophagogastric varices (Fig. 5) which were confirmed endoscopically. These had not bled, and the patient has
TABLE 2. Extrahepatic Portal Hypertension Onset of Manifestations During Adult Life in Seven Cases
Predisposing
Previous Procedures (Age)
Treatment (Age)
Course
-(21) Splenorenal (22)
Mesenteric-ovarian (? patent) (26)
4 yr. 0 bleed 1 yr. 0 bleed
P.T./Sex
Age 1st Bleed
# Bleeds
S.O./M L.N./F
19
3
J.R./F
40
1
Cholecystectomy
Splenorenal (41)
4 yr. 0 bleed
Splenorenal (57)
6 yr. 0 bleed
Mesocaval H graft (62)
3 yr. 0 bleed
Esophago-gastrectomy jejunal-interpos. (28) LAP.-unshuntable (42)
8 yr. 0 bleed
Conditions
A.K./F
57
4
5 mo. P.T. bleed CBD exploration 4 mo. P.T. bleed
E.W./F
59
2
Felty's splenectomy sepsis 2 yr P./T. bleed Sepsis splenectomy talc peritonitis Cord trauma
R. E./M
26
2
A.T./F
39
3
LAP.-(24) unshuntable
6 yr. 3 bleed
VOl. 189.o NO. 5
EXTRAHEPATIC PORTAL HYPERTENSION
FIG. 5. Splenoportogram of a 21-year-old who has not bled. Note portal vein collaterals and coronary vein.
been followed seven years with no evidence of bleeding. The remaining six patients bled from varices. In two patients (L.N. and A.T.) the portal hypertension probably developed in childhood, but bleeding did not become apparent for years. One patient, L.N., first bled at age 19 and was shunted with a splenorenal anastomosis three years later because of repeated bleeding. This shunt thrombosed, and postoperatively the patient developed deep venous thrombosis requiring an inferior venacaval clip. Four years later because of persistently large varices, a mesentericleft ovarian shunt was performed. Angiography carried out one year later suggested that this too may have clotted, but the patient has not rebled. The other patient, A.T., sustained trauma to her umbilical cord at birth but did not bleed from varices until she was 39. Laparotomy three years later revealed the portal vein, the superior mesenteric vein and the splenic vein to be thrombosed, thus precluding a decompressive procedure. The patient has bled on three occasions, twice requiring transfusion, over the ensuing six years. At one time she underwent a
569 cholecystectomy for acute cholecystitis, but otherwise she has continued to work. The remaining four patients had extrahepatic portal hypertension which could be related to events which occurred during their adult life. Etiologic factors included sepsis, splenectomy and biliary tract operations. The two patients whose esophagogastric varices developed and bled within six months of an operation on the biliary tract had successful decompression with central splenorenal shunts. Neither has developed postshunt encephalopathy. The one patient, E.W., whose varices bled two years after splenectomy complicated by postoperative sepsis was successfully managed with a mesocaval shunt employing a No. 21 Dacron® conduit as an H graft. The final patient in this category also had a splenectomy followed by sepsis, plus talc granulomatous peritonitis, and underwent esophagogastrectomy with jejunal interposition, since the portal vein and superior mesenteric veins were thrombosed. He has not rebled during the eight year follow-up. Portal Vein Thrombosis Associated with Liver Disease
In three patients portal vein thrombosis was associated with hepatic pathology (Table 3). A 50-year-old alcoholic cirrhotic with bleeding esophagogastric varices temporarily controlled by intravenous Pitressin® was explored in anticipation of performing a portacaval shunt. The portal vein was totally occluded by a fresh thrombus, which was extracted. After adequate portal flow was demonstrated, an end-to-side portacaval shunt was effected, reducing splanchnic venous pressure from 39 to 9 cm saline. He has abstained from drinking and remains well after 7 years. Another male with known congenital hepatic fibrosis and normal liver function had two bleeding episodes related to esophagogastric varices. A splenectomy had been performed at another hospital for splenomegaly, and this led to subsequent development of a subphrenic abscess. The venous phase of a superior mesenteric artery angiogram (Fig. 6) revealed a thrombus within the portal vein. A superior mesenteric-inferior venacaval anastomosis was effected with a Dacron conduit, and the patient has continued to do well postoperatively.
TABLE 3. Portal Vein Thrombosis Associated with Liver Disease in Three Cases
P.T./Sex
Age
J.L./M
51
Cirrhosis
P.D./M D.D./M
41 46
Congenital fibrosis Intrahepatic A-V fistula
Hepatic Pathology
Treatment
Course
ThrombectomyPortacaval shunt Mesocaval-H graft Attempted shunt left hepatic lobectomy
7 yr. 0 bleed
I yr. 0 bleed DIED
570
GRAUER AND SCHWARTZ
Ann. Surg. * May 1979
subsided. He was readmitted two months later, and the fistula was removed by performing a left hepatic lobectomy. This procedure, unfortunately, was complicated by uncontrollable bleeding from portal collaterals in the hepatoduodenal region, and the patient expired during the immediate postoperative period.
FIG.
6.
Venous phase of superior mesenteric angiogram demon-
strating thrombus in the portal vein and patent superior mesenteric vein.
The third
patient
in this group
was
most unusual.
This 44-year-old man was admitted in May,
1978 with
In history 1956-58 he had undergone total colectomy and ileostomy for ulcerative colitis. In 1965 he had a subtotal gastrectomy for massive upper gastrointestinal bleeding. Two weeks prior to his admission to Strong Memorial Hospital he again bled massively, and large varices were noted. Angiography revealed thrombosis of the portal and splenic veins and a patent superior mesenteric vein. His bilirubin at that time was 8.2, and needle biopsy of the liver showed normal architecture and cells. One week later a repeat angiogram demonstrated a large hepatic artery and a questionable A-V fistula within the liver. He continued passing winecolored stools after transfer to Strong Memorial Hospital. Repeat angiography showed a large intrahepatic A-V fistula with retrograde flow through the veins of Sappey into the esophageal vein (Fig. 7 A and B). At operation the portal vein was noted to be thrombosed, and a No. 19 Dacron H graft mesocaval shunt was attempted. It clotted immediately after release of the occlusive clamps, and reconstruction was met with the same result. The conduit was therefore removed, and the venotomies closed. Postoperatively he developed intermittent jaundice, which
a
of bleeding esophagogastric
varices.
Discussion The pattern and consequences of bleeding associated with varices secondary to extrahepatic portal hypertension are distinctly different from those in patients in whom the varices are secondary to hepatic disease. Although bleeding was alarming in many of our patients, in only two did it necessitate an emergency operation. This feature is in concert with many previous reports.3'4"' In the large series of Voorhees and Price" death could be attributed to exsanguination in seven patients (5%). In the experience of Fonkalsrud et al.4 none of the 69 pediatric cases required an emergency shunt. However, others' 6"13 report that death associated with hemorrhage occurred more frequently, i.e., 13-31%. Recurrent bleeding episodes are common. In our series nine pediatric cases had a total of 35 bleeds, while the nine adults who bled did so 21 times. In two published reports3'4 children averaged five bleeding episodes. Therefore, although bleeding is usually well tolerated, patients must live with a realization that recurrence is a distinct possibility. Surgical procedures to stop hemorrhage from varices or obviate future bleeding in these patients have included direct attacks such as interruption of the esophagogastric veins and esophagogastrectomy, splenectomy, makeshift shunts and two classic shunting procedures, i.e., mesocaval and splenorenal. In most series" 3-5'89"11 the overwhelming majority of patients have rebled when treated by any procedure other than a mesocaval or splenorenal shunt. It has been suggested3'4 that a shunt under 1 cm in diameter is unlikely to remain patent or to effectively prevent subsequent bleeding. In reference to the mesocaval and splenorenal shunts, Clatworthy3 reported 12 successes and nine failures, while seven of 15 patients in the series of Fonkalsrud et al. had thrombosis of their shunts and rebled. Our experience is dissimilar and parallels that of Foster5 and Bismuth and Franco,2 who achieved patency in 87% of children, using veins between 2 and 10 mm in diameter. In the presently reported series seven of eight shunting procedures in the pediatric patients were successful, while four of five adult shunts maintained their patency. This leads us to believe that a major shunt can be constructed, antici-
Vol. 189 * No. 5
EXTRAHEPATIC PORTAL HYPERTENSION
571
FIG. 7. A) Selective hepatic arteriogram demonstrating A-V fistula within the liver. B) Venous phase demonstrating reversed flow
through veins of Sappey and into the esophageal varices.
_
pating patency and prolonged relief from bleeding both in children and in adults. Three of the presently reported patients in whom a mesocaval or splenorenal shunt failed or could not be performed continue to do well, and only one of these patients has experienced multiple major bleeding episodes. This parallels the report of pediatric4 and adult10 series. By contrast, Voorhees and Price'1 indicate that 87% of their patients in whom a shunt failed required a subsequent operation for significant
bleeding. The report of Voorhees et al.'2 was the first to focus on postshunt encephalopathy in patients with extrahepatic portal hypertension. Major psychologic and psychiatric problems were noted in the small group of patients, and most of these had accompanying hyperammonemia. In the large review from the same institution, central nervous system disorders occurred in 22% and 29% of patients with extrahepatic portal hypertension subjected to the splenorenal or mesocaval operations, respectively. However, the incidence of neuropsychiatric disorders was not correlated with patency, and a 19% incidence of emotional disorders occurred in patients undergoing esophagogstrectomy, making it difficult to ascribe an etiologic factor to the
4:
shunt per se. It is quite possible that the chronicity of illness and repeated hospitalization were contributory. In none of our patients has encephalopathy been a problem, nor has this occurred in the experience of others.4.7 The summation of factors, which include 1) a low incidence of exsanguination as a consequence of initial and recurrent bleeding varices, 2) a high incidence of recurrent bleeding, 3) reasonable success with classic shunting procedures both in children over five and in adults and 4) a negligible incidence of true encephalopathy, suggests that a mesocaval or splenorenal shunt should be performed on an elective basis in patients with extrahepatic portal hypertension, if patency of a large splenic or mesenteric vein has been defined. Failure to maintain patency of a portalsystemic shunt or a pathologic situation which precludes performance of such a shunt in this category of patients may be attended by recurrent bleeding, but the overall prognosis is good. References 1. Arcari, F. A. and Lynn, H. B.: Bleeding Esophageal Varices in Children. Surg. Gynecol. Obstet., 112:101, 1961. 2. Bismuth, H. and Franco, D.: Portal Diversion for Portal Hypertension in Early Childhood. Ann. Surg., 183:439, 1976.
GRAUER AND SCHWARTZ
572
Ann. Surg. * May 1979
3. Clatworthy, H. W. Jr.: Extrahepatic Portal Hypertension. In Child, C. G. (ed.): Portal Hypertension. 3rd edition. Philadelphia, W. B. Saunders Co., p. 243, 1974. 4. Fonkalsrud, E. W., Myers, N. A. and Robinson, M. J.: Management of Extrahepatic Portal Hypertension in Children. Ann. Surg., 180:487, 1974. 5. Foster, J. H.: Discussion in Fonkalsrud, E. W. et al.: Management of Extrahepatic Portal Hypertension in Children. Ann. Surg., 180:487, 1974. 6. Foster, J. H., Holcomb, G. W. and Kirtley, J. A.: Results of Surgical Treatment of Portal Hypertension in Children. Ann. Surg., 157:868, 1963. 7. Lambert, M. J. III., Tank, E. S. and Turcotte, J. G.: Late Sequelae of Mesocaval Shunts in Children. Am. J. Surg., 127: 19, 1974.
8. Mikkelsen, W. P.: Extrahepatic Portal Hypertension in Children. Am. J. Surg., 111:333, 1966. 9. Stathers, G. M., Ma, M. H. and Blackburn, C. R. B.: Extrahepatic Portal Hypertension: The Clinical Evaluation, Investigation and Results of Treatment of 28 patients. Aust. Ann. Med., 17:12, 1968. 10. Turcotte, J. G. and Child, C. G. III.: Idiopathic Extrahepatic Portal Hypertension in Adults. Am. J. Surg., 123:35, 1972. 11. Voorhees, A. B. Jr. and Price, J. B. Jr.: Extrahepatic Portal Hypertension. Arch. Surg. 108:338, 1974. 12. Voorhees, A. B. Jr., Chaitman, E., Schneider, S., et al.: Portalsystemic Encephalopathy in the Noncirrhotic Patient. Effect of Portal-systemic Shunting. Arch. Surg., 107:659, 1973. 13. Walker, R. M.: Treatment of Portal Hypertension in Children. Proc. Roy. Soc. Med., 55:770, 1972.
DISCUSSION
performed, the first on the left side bringing the cardiac end of the stomach above the aortic arch and in the second patient anastomosis was carried out on the right side, bringing the cardia to near the apex of the thorax. The first of these two patients was followed for 11 years and was then lost to follow-up. She never had another hemorrhage. The other one was followed for 21 years, never bled and died three years ago from an unrelated
DR. JERE W. LORD, JR. (New York, New York): I do not recall a paper on this subject being presented in the last decade at a major surgical meeting. A recent operation for portal hypertension in October represented the one hundredth patient that I have operated upon from January, 1947 till October, 1978. Of these 100 patients, five represented an extrahepatic block, and they bear out two or three of the points that Dr. Schwartz made. These patients are excellent risks. All five were operated upon electively and none of the five experienced an encephalopathic episode since their procedure. The thrust of my comment is directed towards the selection of an appropriate operation for the individual patient and not to try to do the same procedure for everyone. Dr. Schwartz has described a variety of procedures. Of the five patients, two of them had not been previously operated upon. One was a woman of 39, the other a lad of 15. They bore out the principle which the late Dr. Blakemore emphasized so often: Never to remove a spleen for Banti's syndrome, unless the surgeon was prepared to do a splenorenal shunt if indicated. These two patients experienced a massive hemorrhage from an extrahepatic block. One was operated upon in 1952 and 26 years later she is in excellent health, her only problem being a tendency towards obesity. The boy, a diabetic for eight years, was operated upon at the age of 15, some 22 months ago. In both cases the spleen was very large and the splenorenal shunt was easily constructed. He also has been well and has gained from 113 to 143 lbs. (slide) This preoperative angiogram showed the arterial phase with the celiac axis demonstrated and the next slide (slide) shows the venous phase. This is the splenic vein, the large mesenteric vein, and the large portal vein which is blocked as it enters the liver. The second type of operation which we carried out was in a patient who had had a splenectomy at the age of 6. I saw him at 32, some eight years following a subtotal colectomy for ulcerative colitis. He had experienced multiple variceal hemorrhages over many years, some of which were mild and some major. We carried out, following angiography, a mesocaval H-graft with a Teflon prosthesis. He lived 4 ½2 years and never bled again, but died from carcinoma of the rectum secondary to his ulcerative colitis. And the last two patients with extrahepatic blocks represent individuals in whom the superior mesenteric vein was not available because of its small size. Their spleens had been removed many years earlier. In each patient a subtotal esophagectomy was
cause. So I think if one is going to do an operation on the gastrointestinal tract then a subtotal esophagectomy is a better procedure than an
esophagogastrectomy. Recently we have been using a Gore-Tex® prosthesis for the mesocaval H-grafts. (slide) This is a postoperative study with the catheter placed in the vena cava from the femoral vein. One sees the graft, the vena cava and the superior mesenteric vein. I think this offers some advantages over the Teflon and Dacrong because of its thinner neointima and hence allows the surgeon to use a smaller size, i.e. 14-16 mm, instead of a 20-22 mm Dacron or Teflon prosthesis. DR. ARTHUR B. VOORHEES, JR. (New York, New York): Dr. Schwartz's paper has brought into focus points relevant to the natural history and therapy of extrahepatic portal hypertension, and has occasioned us in New York to review our most recent experience with 131 patients undergoing approximately 230 operations, often in a vain attempt to control recurrent variceal hemorrhage over the past 35 years. We agree that the mesocaval shunt is an excellent procedure for reducing portal pressure, particularly in the young. We also agree that rebleeding is relatively frequent in the nonfunctioning shunts, and that death from exsanguination is very rare. Dr. Schwartz has pointed out the incidence in various series. We agree that conservative therapy is associated with a relatively good prognosis in cases where an intolerable bleeding pattern has not been established. Where we sharply disagree with this report is that which contends that the successfully shunted extrahepatic patient need not fear neuropsychiatric disorders. We believe that we have identified a serious manifestation of portal systemic encephalopathy, primarily psychiatric in form, most significantly concentrated in the young, with a peak in incidence between five and ten years following the shunt. The attack rate after this interval is about 60% where the shunt was performed in the 10-year-old age group, 43% in the under 30 group and 25% in the over 30 years of age group. Concerned by these findings, we asked a cooperating team of
A retrospective analysis of 19 patients with esophagogastric or intestinal varices secondary to extrahepatic portal hypertension was conducted. Nine patients had onset of bleeding during childhood. In six of eight pediatric patients in whom a shunt was performed no major bleeding occurred during the follow-up period. In seven adults with pure extrahepatic portal hypertension the four shunting procedures effected permanent control of bleeding. In two of three adults in whom portal vein thrombosis was associated with liver disease, shunting was successful. An emergency procedure was infrequently required. Multiple recurrences of bleeding episodes prior to decompression was the rule. Postshunt encephalopathy was never seen. In those patients in whom a shunt thrombosed or could not be performed, recurrent bleeding occurred uncommonly and was readily managed by bedrest and transfusion.
E XTRAHEPATIC portal hypertension, usually caused by thrombosis or cavernomatous transformation of the portal vein, is a relatively uncommon cause of bleeding gastroesophageal or intestinal varices, but it is one which merits special consideraton. The salient questions concerning this disorder relate to the general prognosis, including the course of the nonshunted patient, the applicability of decompressive shunting procedures and the risk of consequent encephalopathy. Nineteen patients with extrahepatic portal hypertension were managed at Strong Memorial Hospital between 1955 and 1978. During the same period approximately 260 portal- systemic shunts were performed for portal hypertension secondary to cirrhosis or other liver diseases. The patients' charts and radiographs were reviewed, and an assessment of their course was brought up to date whenever possible. Presented at the Annual Meeting of the Southern Surgical Association, December 4-6, 1978, Hot Springs, Virginia. Reprint requests: Seymour I. Schwartz, M.D., Department of Surgery; University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, New York 14642. Submitted for publication: December 7, 1978.
From The University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital, Rochester, New York
Clinical Data
It is appropriate to categorize the patients in three groups: 1) patients whose first bleeding episode occurred during childhood, 2) patients who first bled during adult life and 3) patients in whom the bleeding varices and extrahepatic portal hypertension were associated with significant intrahepatic pathology.
Childhood Onset of Bleeding Nine patients experienced their first bleeding episode early in life, before the age of six (Table 1). Seven patients bled during the first two years of life. Two of these (R.Y. and L.T.) were known to have had neonatal omphalitis, and two (S.C. and C.W.) were the product of difficult deliveries and may have sustained umbilical cord trauma. The liver function was normal in all patients. Five of the eight patients in whom platelet counts were performed had thrombocytopenia below 80,000/cu mm. Six patients with esophagogastric varices, all of whom bled by age two, had successful decompression during childhood. This group had a mean of three and one half bleeds prior to the definitive procedure. Three patients had undergone previous operations to control bleeding. In one patient, transesophageal ligation of varices at age one was effective for 3 years. In another patient distal esophagectomy-proximal gastrectomy controlled bleeding for eight years. Significant rebleeding in these patients necessitated the shunting procedures. The third patient had undergone a splenorenal shunt in another hospital but continued to bleed massively as a consequence of thrombosis of that shunt, and this led to an emergency mesocaval shunt.
0003-4932/79/0500/0566 $00.95 © J. B. Lippincott Company
566
EXTRAHEPATIC PORTAL HYPERTENSION
Vol. 189 * No. 5
567
TABLE 1. Extrahepatic Portal Hypertension Onset of Bleeding During Childhood in Nine Cases
Pt./Sex Esophagogastric varices
S.C./F J.L./F R.C./M R.Y./M
Age 1st Bleed
Previous Procedures (Age)
# Bleeds
S.B./M S.C./M L.T./F
2 1 1 1 2 2 4
2 6 6
R.H./M
2
7
C.W./F
5
2
4 2 2 4
T. E. ligation (1)
Splenorenal (19) Esophagogastrectomy (2) Splenectomy (12) T. E. ligation (14) Splenectomy (12) 3 Attempted shunts (13-14)
Treatment (Age) Mesocaval (5) Mesocaval (5)
Splenorenal (12) Mesocaval (4) Mesocaval (19) Splenorenal (10) Mesocaval attempted
Course 8 yr. 0 bleed 4 yr. 0 bleed 8 yr. 0 bleed 14 yr. 0 bleed 16 yr. 0 bleed 14 yr. 0 bleed 2 yr. 1 bleed
(24)
(27)
5 yr. 0 bleed
T. E. ligation (19)
Colon varices
Inf. mesocaval (19)
6 yr. I Minor bleed
The diagnosis was confirmed angiographically in these patients (Figs. I and 2). In all six patients a significant reduction in superior mesenteric pressure was demonstrated intraoperatively. The mean preshunt mesenteric venous pressure was 44 cm saline, while the postshunt pressures had a mean of 16 cm saline. There has been no bleeding subsequent to the operation in any of these patients for periods ranging between four and 16 years (Fig. 3). There has not been a single episode of postshunt encephalopathy in these patients. One patient who has been followed to adulthood is a college student, while another is a history professor. Two patients have had psychologic and social prob-
lems. Two patients (L.T. and R.H.) were first seen at our hospital with a history of multiple bleeds, each having been subjected to several surgical procedures. In both instances the most recent bleeding episode, which led to their admission was associated with ingestion of aspirin. One patient received a transfusion and has not bled over the ensuing five years. The other patient was explored, but extensive peritoneal adhesions precluded a proposed mesocaval shunt. She has bled once, not requiring transfusion, over the past two years. One patient with varices of the rectosigmoid colon, but no esophagogastric varices, first noted rectal
FIG. 1. Splenoportogram demonstrating cavernomatous transformation of portal vein, collaterals and large esophagogastric
FIG. 2. Venous phase of superior mesenteric artery angiogram demonstrating thrombosis of the portal vein and veins of Sappey coursing to the'liver.
varices.
568
GRAUER AND SCHWARTZ
FIG. 3. Venous phase of superior mesenteric angiogram showing patent end-to-side mesocaval shunt and absence of collaterals including varices (same patient as in Fig. 2).
FIG. 4. Thrombosis ofthe portal vein causing marked dilatation of the inferior mesenteric vein and associated varices of the colon. years
later during
lowing a term bleeding at age five. A hemorroidectomy performed for recurrent bleeding at age 19 was accompanied by a major blood loss, which led to the consideration of portal hypertension. A splenoportogram demonstrating a distended inferior mesenteric vein and obstruction of the portal vein (Fig. 4) confirmed the diagnosis. At operation the inferior mesenteric venous pressure was determined to be 54 cm saline. A side-toside inferior mesenteric-inferior venacaval shunt reduced this pressure to 14 cm saline. She has had one minor bleed since this procedure, and shunt patency and persistent pressure reduction were confirmed two
Ann. Surg. X May 1979
laparotomy for tubal ligation
fol-
pregnancy.
Adult Onset of Bleeding
Seven patients with portal vein thrombosis had consequent esophagogastric varices which became
manifest during adult life (Table 2). Liver function tests were normal in all, and five of the six patients in whom measurements were made had platelet counts
less than 80,000/cu mm. A 21-year-old man being evaluated for thrombocytopenia had a splenoportogram demonstrating esophagogastric varices (Fig. 5) which were confirmed endoscopically. These had not bled, and the patient has
TABLE 2. Extrahepatic Portal Hypertension Onset of Manifestations During Adult Life in Seven Cases
Predisposing
Previous Procedures (Age)
Treatment (Age)
Course
-(21) Splenorenal (22)
Mesenteric-ovarian (? patent) (26)
4 yr. 0 bleed 1 yr. 0 bleed
P.T./Sex
Age 1st Bleed
# Bleeds
S.O./M L.N./F
19
3
J.R./F
40
1
Cholecystectomy
Splenorenal (41)
4 yr. 0 bleed
Splenorenal (57)
6 yr. 0 bleed
Mesocaval H graft (62)
3 yr. 0 bleed
Esophago-gastrectomy jejunal-interpos. (28) LAP.-unshuntable (42)
8 yr. 0 bleed
Conditions
A.K./F
57
4
5 mo. P.T. bleed CBD exploration 4 mo. P.T. bleed
E.W./F
59
2
Felty's splenectomy sepsis 2 yr P./T. bleed Sepsis splenectomy talc peritonitis Cord trauma
R. E./M
26
2
A.T./F
39
3
LAP.-(24) unshuntable
6 yr. 3 bleed
VOl. 189.o NO. 5
EXTRAHEPATIC PORTAL HYPERTENSION
FIG. 5. Splenoportogram of a 21-year-old who has not bled. Note portal vein collaterals and coronary vein.
been followed seven years with no evidence of bleeding. The remaining six patients bled from varices. In two patients (L.N. and A.T.) the portal hypertension probably developed in childhood, but bleeding did not become apparent for years. One patient, L.N., first bled at age 19 and was shunted with a splenorenal anastomosis three years later because of repeated bleeding. This shunt thrombosed, and postoperatively the patient developed deep venous thrombosis requiring an inferior venacaval clip. Four years later because of persistently large varices, a mesentericleft ovarian shunt was performed. Angiography carried out one year later suggested that this too may have clotted, but the patient has not rebled. The other patient, A.T., sustained trauma to her umbilical cord at birth but did not bleed from varices until she was 39. Laparotomy three years later revealed the portal vein, the superior mesenteric vein and the splenic vein to be thrombosed, thus precluding a decompressive procedure. The patient has bled on three occasions, twice requiring transfusion, over the ensuing six years. At one time she underwent a
569 cholecystectomy for acute cholecystitis, but otherwise she has continued to work. The remaining four patients had extrahepatic portal hypertension which could be related to events which occurred during their adult life. Etiologic factors included sepsis, splenectomy and biliary tract operations. The two patients whose esophagogastric varices developed and bled within six months of an operation on the biliary tract had successful decompression with central splenorenal shunts. Neither has developed postshunt encephalopathy. The one patient, E.W., whose varices bled two years after splenectomy complicated by postoperative sepsis was successfully managed with a mesocaval shunt employing a No. 21 Dacron® conduit as an H graft. The final patient in this category also had a splenectomy followed by sepsis, plus talc granulomatous peritonitis, and underwent esophagogastrectomy with jejunal interposition, since the portal vein and superior mesenteric veins were thrombosed. He has not rebled during the eight year follow-up. Portal Vein Thrombosis Associated with Liver Disease
In three patients portal vein thrombosis was associated with hepatic pathology (Table 3). A 50-year-old alcoholic cirrhotic with bleeding esophagogastric varices temporarily controlled by intravenous Pitressin® was explored in anticipation of performing a portacaval shunt. The portal vein was totally occluded by a fresh thrombus, which was extracted. After adequate portal flow was demonstrated, an end-to-side portacaval shunt was effected, reducing splanchnic venous pressure from 39 to 9 cm saline. He has abstained from drinking and remains well after 7 years. Another male with known congenital hepatic fibrosis and normal liver function had two bleeding episodes related to esophagogastric varices. A splenectomy had been performed at another hospital for splenomegaly, and this led to subsequent development of a subphrenic abscess. The venous phase of a superior mesenteric artery angiogram (Fig. 6) revealed a thrombus within the portal vein. A superior mesenteric-inferior venacaval anastomosis was effected with a Dacron conduit, and the patient has continued to do well postoperatively.
TABLE 3. Portal Vein Thrombosis Associated with Liver Disease in Three Cases
P.T./Sex
Age
J.L./M
51
Cirrhosis
P.D./M D.D./M
41 46
Congenital fibrosis Intrahepatic A-V fistula
Hepatic Pathology
Treatment
Course
ThrombectomyPortacaval shunt Mesocaval-H graft Attempted shunt left hepatic lobectomy
7 yr. 0 bleed
I yr. 0 bleed DIED
570
GRAUER AND SCHWARTZ
Ann. Surg. * May 1979
subsided. He was readmitted two months later, and the fistula was removed by performing a left hepatic lobectomy. This procedure, unfortunately, was complicated by uncontrollable bleeding from portal collaterals in the hepatoduodenal region, and the patient expired during the immediate postoperative period.
FIG.
6.
Venous phase of superior mesenteric angiogram demon-
strating thrombus in the portal vein and patent superior mesenteric vein.
The third
patient
in this group
was
most unusual.
This 44-year-old man was admitted in May,
1978 with
In history 1956-58 he had undergone total colectomy and ileostomy for ulcerative colitis. In 1965 he had a subtotal gastrectomy for massive upper gastrointestinal bleeding. Two weeks prior to his admission to Strong Memorial Hospital he again bled massively, and large varices were noted. Angiography revealed thrombosis of the portal and splenic veins and a patent superior mesenteric vein. His bilirubin at that time was 8.2, and needle biopsy of the liver showed normal architecture and cells. One week later a repeat angiogram demonstrated a large hepatic artery and a questionable A-V fistula within the liver. He continued passing winecolored stools after transfer to Strong Memorial Hospital. Repeat angiography showed a large intrahepatic A-V fistula with retrograde flow through the veins of Sappey into the esophageal vein (Fig. 7 A and B). At operation the portal vein was noted to be thrombosed, and a No. 19 Dacron H graft mesocaval shunt was attempted. It clotted immediately after release of the occlusive clamps, and reconstruction was met with the same result. The conduit was therefore removed, and the venotomies closed. Postoperatively he developed intermittent jaundice, which
a
of bleeding esophagogastric
varices.
Discussion The pattern and consequences of bleeding associated with varices secondary to extrahepatic portal hypertension are distinctly different from those in patients in whom the varices are secondary to hepatic disease. Although bleeding was alarming in many of our patients, in only two did it necessitate an emergency operation. This feature is in concert with many previous reports.3'4"' In the large series of Voorhees and Price" death could be attributed to exsanguination in seven patients (5%). In the experience of Fonkalsrud et al.4 none of the 69 pediatric cases required an emergency shunt. However, others' 6"13 report that death associated with hemorrhage occurred more frequently, i.e., 13-31%. Recurrent bleeding episodes are common. In our series nine pediatric cases had a total of 35 bleeds, while the nine adults who bled did so 21 times. In two published reports3'4 children averaged five bleeding episodes. Therefore, although bleeding is usually well tolerated, patients must live with a realization that recurrence is a distinct possibility. Surgical procedures to stop hemorrhage from varices or obviate future bleeding in these patients have included direct attacks such as interruption of the esophagogastric veins and esophagogastrectomy, splenectomy, makeshift shunts and two classic shunting procedures, i.e., mesocaval and splenorenal. In most series" 3-5'89"11 the overwhelming majority of patients have rebled when treated by any procedure other than a mesocaval or splenorenal shunt. It has been suggested3'4 that a shunt under 1 cm in diameter is unlikely to remain patent or to effectively prevent subsequent bleeding. In reference to the mesocaval and splenorenal shunts, Clatworthy3 reported 12 successes and nine failures, while seven of 15 patients in the series of Fonkalsrud et al. had thrombosis of their shunts and rebled. Our experience is dissimilar and parallels that of Foster5 and Bismuth and Franco,2 who achieved patency in 87% of children, using veins between 2 and 10 mm in diameter. In the presently reported series seven of eight shunting procedures in the pediatric patients were successful, while four of five adult shunts maintained their patency. This leads us to believe that a major shunt can be constructed, antici-
Vol. 189 * No. 5
EXTRAHEPATIC PORTAL HYPERTENSION
571
FIG. 7. A) Selective hepatic arteriogram demonstrating A-V fistula within the liver. B) Venous phase demonstrating reversed flow
through veins of Sappey and into the esophageal varices.
_
pating patency and prolonged relief from bleeding both in children and in adults. Three of the presently reported patients in whom a mesocaval or splenorenal shunt failed or could not be performed continue to do well, and only one of these patients has experienced multiple major bleeding episodes. This parallels the report of pediatric4 and adult10 series. By contrast, Voorhees and Price'1 indicate that 87% of their patients in whom a shunt failed required a subsequent operation for significant
bleeding. The report of Voorhees et al.'2 was the first to focus on postshunt encephalopathy in patients with extrahepatic portal hypertension. Major psychologic and psychiatric problems were noted in the small group of patients, and most of these had accompanying hyperammonemia. In the large review from the same institution, central nervous system disorders occurred in 22% and 29% of patients with extrahepatic portal hypertension subjected to the splenorenal or mesocaval operations, respectively. However, the incidence of neuropsychiatric disorders was not correlated with patency, and a 19% incidence of emotional disorders occurred in patients undergoing esophagogstrectomy, making it difficult to ascribe an etiologic factor to the
4:
shunt per se. It is quite possible that the chronicity of illness and repeated hospitalization were contributory. In none of our patients has encephalopathy been a problem, nor has this occurred in the experience of others.4.7 The summation of factors, which include 1) a low incidence of exsanguination as a consequence of initial and recurrent bleeding varices, 2) a high incidence of recurrent bleeding, 3) reasonable success with classic shunting procedures both in children over five and in adults and 4) a negligible incidence of true encephalopathy, suggests that a mesocaval or splenorenal shunt should be performed on an elective basis in patients with extrahepatic portal hypertension, if patency of a large splenic or mesenteric vein has been defined. Failure to maintain patency of a portalsystemic shunt or a pathologic situation which precludes performance of such a shunt in this category of patients may be attended by recurrent bleeding, but the overall prognosis is good. References 1. Arcari, F. A. and Lynn, H. B.: Bleeding Esophageal Varices in Children. Surg. Gynecol. Obstet., 112:101, 1961. 2. Bismuth, H. and Franco, D.: Portal Diversion for Portal Hypertension in Early Childhood. Ann. Surg., 183:439, 1976.
GRAUER AND SCHWARTZ
572
Ann. Surg. * May 1979
3. Clatworthy, H. W. Jr.: Extrahepatic Portal Hypertension. In Child, C. G. (ed.): Portal Hypertension. 3rd edition. Philadelphia, W. B. Saunders Co., p. 243, 1974. 4. Fonkalsrud, E. W., Myers, N. A. and Robinson, M. J.: Management of Extrahepatic Portal Hypertension in Children. Ann. Surg., 180:487, 1974. 5. Foster, J. H.: Discussion in Fonkalsrud, E. W. et al.: Management of Extrahepatic Portal Hypertension in Children. Ann. Surg., 180:487, 1974. 6. Foster, J. H., Holcomb, G. W. and Kirtley, J. A.: Results of Surgical Treatment of Portal Hypertension in Children. Ann. Surg., 157:868, 1963. 7. Lambert, M. J. III., Tank, E. S. and Turcotte, J. G.: Late Sequelae of Mesocaval Shunts in Children. Am. J. Surg., 127: 19, 1974.
8. Mikkelsen, W. P.: Extrahepatic Portal Hypertension in Children. Am. J. Surg., 111:333, 1966. 9. Stathers, G. M., Ma, M. H. and Blackburn, C. R. B.: Extrahepatic Portal Hypertension: The Clinical Evaluation, Investigation and Results of Treatment of 28 patients. Aust. Ann. Med., 17:12, 1968. 10. Turcotte, J. G. and Child, C. G. III.: Idiopathic Extrahepatic Portal Hypertension in Adults. Am. J. Surg., 123:35, 1972. 11. Voorhees, A. B. Jr. and Price, J. B. Jr.: Extrahepatic Portal Hypertension. Arch. Surg. 108:338, 1974. 12. Voorhees, A. B. Jr., Chaitman, E., Schneider, S., et al.: Portalsystemic Encephalopathy in the Noncirrhotic Patient. Effect of Portal-systemic Shunting. Arch. Surg., 107:659, 1973. 13. Walker, R. M.: Treatment of Portal Hypertension in Children. Proc. Roy. Soc. Med., 55:770, 1972.
DISCUSSION
performed, the first on the left side bringing the cardiac end of the stomach above the aortic arch and in the second patient anastomosis was carried out on the right side, bringing the cardia to near the apex of the thorax. The first of these two patients was followed for 11 years and was then lost to follow-up. She never had another hemorrhage. The other one was followed for 21 years, never bled and died three years ago from an unrelated
DR. JERE W. LORD, JR. (New York, New York): I do not recall a paper on this subject being presented in the last decade at a major surgical meeting. A recent operation for portal hypertension in October represented the one hundredth patient that I have operated upon from January, 1947 till October, 1978. Of these 100 patients, five represented an extrahepatic block, and they bear out two or three of the points that Dr. Schwartz made. These patients are excellent risks. All five were operated upon electively and none of the five experienced an encephalopathic episode since their procedure. The thrust of my comment is directed towards the selection of an appropriate operation for the individual patient and not to try to do the same procedure for everyone. Dr. Schwartz has described a variety of procedures. Of the five patients, two of them had not been previously operated upon. One was a woman of 39, the other a lad of 15. They bore out the principle which the late Dr. Blakemore emphasized so often: Never to remove a spleen for Banti's syndrome, unless the surgeon was prepared to do a splenorenal shunt if indicated. These two patients experienced a massive hemorrhage from an extrahepatic block. One was operated upon in 1952 and 26 years later she is in excellent health, her only problem being a tendency towards obesity. The boy, a diabetic for eight years, was operated upon at the age of 15, some 22 months ago. In both cases the spleen was very large and the splenorenal shunt was easily constructed. He also has been well and has gained from 113 to 143 lbs. (slide) This preoperative angiogram showed the arterial phase with the celiac axis demonstrated and the next slide (slide) shows the venous phase. This is the splenic vein, the large mesenteric vein, and the large portal vein which is blocked as it enters the liver. The second type of operation which we carried out was in a patient who had had a splenectomy at the age of 6. I saw him at 32, some eight years following a subtotal colectomy for ulcerative colitis. He had experienced multiple variceal hemorrhages over many years, some of which were mild and some major. We carried out, following angiography, a mesocaval H-graft with a Teflon prosthesis. He lived 4 ½2 years and never bled again, but died from carcinoma of the rectum secondary to his ulcerative colitis. And the last two patients with extrahepatic blocks represent individuals in whom the superior mesenteric vein was not available because of its small size. Their spleens had been removed many years earlier. In each patient a subtotal esophagectomy was
cause. So I think if one is going to do an operation on the gastrointestinal tract then a subtotal esophagectomy is a better procedure than an
esophagogastrectomy. Recently we have been using a Gore-Tex® prosthesis for the mesocaval H-grafts. (slide) This is a postoperative study with the catheter placed in the vena cava from the femoral vein. One sees the graft, the vena cava and the superior mesenteric vein. I think this offers some advantages over the Teflon and Dacrong because of its thinner neointima and hence allows the surgeon to use a smaller size, i.e. 14-16 mm, instead of a 20-22 mm Dacron or Teflon prosthesis. DR. ARTHUR B. VOORHEES, JR. (New York, New York): Dr. Schwartz's paper has brought into focus points relevant to the natural history and therapy of extrahepatic portal hypertension, and has occasioned us in New York to review our most recent experience with 131 patients undergoing approximately 230 operations, often in a vain attempt to control recurrent variceal hemorrhage over the past 35 years. We agree that the mesocaval shunt is an excellent procedure for reducing portal pressure, particularly in the young. We also agree that rebleeding is relatively frequent in the nonfunctioning shunts, and that death from exsanguination is very rare. Dr. Schwartz has pointed out the incidence in various series. We agree that conservative therapy is associated with a relatively good prognosis in cases where an intolerable bleeding pattern has not been established. Where we sharply disagree with this report is that which contends that the successfully shunted extrahepatic patient need not fear neuropsychiatric disorders. We believe that we have identified a serious manifestation of portal systemic encephalopathy, primarily psychiatric in form, most significantly concentrated in the young, with a peak in incidence between five and ten years following the shunt. The attack rate after this interval is about 60% where the shunt was performed in the 10-year-old age group, 43% in the under 30 group and 25% in the over 30 years of age group. Concerned by these findings, we asked a cooperating team of
