Annals of the Royal College of Surgeons of England (1975) vol 57
ASPECTS OF TREATMENT*
Emergency and elective surgical treatment of portal hypertension A review of 23 years' experience R Kirby FRCS MSt F D Burke FRCS J D T Jones MS FRCS
University Department of Surgery, Royal Victoria Infirmary, Newcastle upon Tyne
Summary A retrospective review of surgical treatment for portal hypertension durinig a 23-year period in a regional unit is reported and the immediate and subsequent mnanagement of patients with; bleeding oesophageal varices is discussed. Fifty-four patients with recurrent varix haemorrhage uncontrolled by conservative methods have been treated by oesophageal transection with a mortality of 22.2% (26.66% for cirrhotic patients). Thirty-two per cent of the cirrhotics were alive after 2 years. Only a minority (I2%/,) of the survivors were considered suitable for a subsequent shunt proced ure. Therapeutic portacaval anastomosis has been performed on 65 patients with a 5I.2%/ 5-year survival (43.5% for cirrhotic patients). Further haemorrhage due to shunt thrombosis occurred in. 5.3%° of cases. The frequent occurrence of portal-systemic encephalopathy,
f Present
address: General Hospital, Hartlepool
increasing with duration of time following a shunt, is emphasized. The high morbidity and mortality in the poor-risk cirrhotic indicates that this type of patient is unsuitable for a portal-systemic shunt and is better treated by medical means.
Introduction Bleeding from gastro-oesophageal varices is a relatively uncommon but highly lethal cause of upper gastrointestinal tract haemorrhage, accounting for about 3% of such hospital admissions. Since it indicates portal hypertension, usually due to advanced hepatic disease, the mortality is high with all forms of treatment. Of 471 patients with cirrhosis and varices treated by non-operative means by the Boston Interhospital Liver Group over a 3-year period, 51 % experienced bleeding from varices and in 34% haemorrhage was the major cause of death. Only 31 % were alive i year after initial demonstration of varices and 5.5% after 5 years'. Mortality with the first large haemorrhage, treated by
Fellows interested in submitting papers for consideration with a v-cw to scrics should first wvrite to the Editor.
publicatiorn
in this
Emergentcy and elective surgical treatment of Portal hypertension medical to
means,
is high, varying from
30%
83%24.
Surgical treatment may be necessary to arrest uncontrollable varix haemorrhage or to provide portal decompression in a quiescent phase. Acute uncontrollable haemorrhage has been treated by direct ligation of varices5'7, transection of the oesophagus8, or emergency portal-systemic shunt9-". Since Blakemore and Lord first described the portacaval shunt in Ig945s a large number of reports have confirmed the effectiveness of this operation in preventing further haemorrhage from varices13'7. In recent years the side cffects of this procedure have been more fully appreciated and its use critically appraised"8. The clinical aspects of the management of severe variceal haemorrhage and the advisability or otherwise of a portal-systemic shunt remain difficult problems. In this retrospective review we illustrate the variety of cases presenting, formulate a plan of management, and analyse the results obtained in the surgical treatment of portal hypertension over a 23-year period in a regional centre. Clinical material During the period I 949-72 i6o patients have undergone surgical treatment for portal hypertension with oesophageal varices under the care of the senior author (J D T J) at the Royal Victoria Infirmary, Newcastle upon Tyne. Information has been gained from the patients' hospital records, from regional consultant staff in other hospitals, from general practitioners, and, when possible, by interviewing the survivors. Adequacy of follow-up data was variable in the earlier cases, and when the patients' subsequent fate was unknown they were presumed dead as from their last recorded outpatient visit in calculating survival figures. In all cases varices were demonstrated radiographically by barium swallow or splenic venography, by oesoph-
I49
agoscopy, or subsequently at operation or autopsy. Table I gives the underlying pathology in the i6o patients treated. In the majority (77 %) of the I43 in whom the pathology was recorded portal hypertension was secondary to portal cirrhosis. Of these, i8 patients (i 6%) were chronic alcoholics and the cirrhosis was presumed to be of alcoholic actiology, the remainder suffering from posthepatic or crvptogenic cirrhosis; this group included 3 cases of chronic active hepatitis progressing to cirrhosis. There were 2 cases of primary biliary cirrhosis, both in women, i of whom exhibited in addition Raynaud's phenomenon, digital calcinosis, and telangiectasia (CRSI syndrome). In i patient the portal hypertension was secondary to haemochromatosis and in another to haemosiderosis due to a long-standing haemolytic process (thalassaemia minor). There was i instance of thrombosis of the portal vein following splenectomy (for acholuric jaundice) and i patient had a relatively benign reticulosis (BrillSymmers disease), although the cause of the portal hypertension was never fully elucidated. Portal hypertension in childhood is most frequently due to an extrahepatic venous obstruction or congenital hepatic fibrosis. Ihere were I5 cases of extrahepatic venous obstruction in this series, of which a definite TABLE i Pathology in i6o patients treated surgically for portal hypertension, I949-72 Portal cirrhosis Cavernomatous transformation Congenital hepatic fibrosis Biliary cirrhosis Haemochromatosis Haemosiderosis (thalassaemia) Reticulosis Splenic vein thrombosis Nil abnormal
Unrecorded
I
o 15 1o 2 I I I I 2
I7
150
R Kirby, F D Burke, and J D T Jones
history of omphalitis was obtained in only I and neonatal exchange transfusion was a possible aetiological factor in 2 others. Some of these cases have been reported elsewhere". Congenital hepatic fibrosis accounted for of cases, 6 presenting in childhood, and these also had the associated renal cystic disease known to occur with this condition20. In 2 cases no apparent underlying cause was demonstrable to explain the undoubted portal hypertension. Both showed no evidence of extrahepatic obstruction on venography and the hepatic parenchyma was normal on gross and microscopic examination, although it is appreciated that biopsy specimens taken from the free edge of the liver may not be representative of the liver as a whole. Medical management With acute haemorrhage treatment consisted of blood transfusions, parenteral vitamin K, and the administration of enemas in an attempt to evacuate blood from the gastrointestinal tract. Neomycin or lactulose was given by mouth. In the absence of contraindication continued bleeding was treated with an intravenous infusion of vasopressin (20 U in I00 ml 5°% dextrose given over 30 min), repeated if necessary. If bleeding persisted or recurred balloon tamponade of the varices with the Sengstaken-Blakemore tube was applied for not more than 24 h. If bleeding recurred on deflating the balloons, as often happened, the balloons were reinflated and urgent surgery was performed. Blood loss of units, repeated severe haemmore than orrhage, or a single massive haemorrhage requiring a Sengstaken tube for control were the indications for operation. If, as was frequently the case, medical management was successful in arresting variceal haemorrhage, consideration was then given to the possibility of carrying out a portal-systemic shunt within the next 2-3 months. The policy has been to admit these io
i
io
patients to hospital i o d before the proposed operation to assess the operative risk and the likelihood of development of post-shunt portal-systemic encephalopathy (PSE), which is the principal complication and the reason why a high degree of selection is desirable. The criteria which indicate an acceptable risk are a serum bilirubin level below 50 ilmol/l (3 mg/ioo ml), serum albumin greater than 30 g/l (3 g/Ioo ml), normal plasma ammonium, normal electroencephalograms on a standard and high-protein diet, absence of ascites, and an upper age limit of 6o years. Occasionally, particularly in the earlier years of this series, some of these rigid criteria were transgressed when the majority were satisfactory and the risk of haemorrhage appeared inordinately high. Trans-splenic portal venography was employed to ensure a patent portal vein of reasonable calibre showing no evidence of previous thrombosis, and a liver biopsy was usually available to indicate the nature of the portal obstruction. Surgical management During the 23-year period several surgical procedures have been used and, not unnaturally, many have since been discarded. Table II lists the operations performed. Mediastinal packing, hepatic artery ligation, and oesophagogastric resection are not now performed. Oesophagoscopic injection of varices has been TABLE II Operations performed, I949-72 (I70 on i6o patients) Portacaval anastomosis Oesophageal transection Splenectomy and devascularization Splenectomy Splenorenal anastomosis Splenectomy and gastric section Omentopexy Ligation of hepatic artery Oesophagogastrectomy Boerema-Crile operation
67 54 27 7 5 3 2 2 2 I
Emergency and elective surgical treatment of portal hypertension
I 5I
found to be most unreliable and in this unit is Dissatisfaction with the high incidence of shunt thrombosis following splenorenal anasno longer used. Emergency treatment of acute varix haemorrhage between I949 and I96I tomosis has led to the adoption of the portausually consisted of devascularization of the caval anastomosis as the operation of choice in the first instance, and an end-to-side shunt upper stomach and lower oesophagus combined with splenectomy, but although fre- has been used exclusively in the last years. quently effective, this procedure is no longer advised in this situation since it carries the Results risk of thrombosis of the splenic and portal In assessing the overall results the patients veins, which may preclude any form of shunt have been divided into 3 groups depending procedure at a later date. Since I962 all on their degree of hepatic reserve according emergency cases have been treated by porta- to criteria suggested by Child21. The relevant clinical details include patient nutrition, the azygos disconnection in the form of a transthoracic oesophageal transection. By an ap- presence and degree of PSE and ascites, and proach through the bed of the resected 8th biochemical measurement of serum bilirubin rib the lower third of the oesophagus is and serum albumin (Table III). In emermobilized and the muscular coat incised gency cases grading was based on clinical longitudinally after application of a Satinski observation and biochemical liver function clamp at the gastro-oesophageal junction. The tests performed on admission to hospital, mucosal tube is freed and transected as low which was usually immediately after the down as possible and resutured with a con- onset of haemorrhage. Prolonged gastrointesttinuous 30-silk stitch, thereby occluding the inal bleeding rapidly leads to an increase in varices. The oesophageal musculature is serum bilirubin level and deterioration in reapproximated with interrupted catgut and liver function, and assessment at this stage is the chest closed, leaving an intercostal drain unreliable. Operative deaths are defined as death down to the site of the transection, this being within 28 days of operation or during the retained for 5 days. The ultimate aim has been the construc- period of hospital confinement following surtion of a portal-systemic shunt where possible, gery regardless of duration. The results will be considered in two basic either portacaval, splenorenal, or mesentericocaval, preferably as an elective procedure. groups according to the mode of presentation 20
Classification of preoperative liver function after Child2"
TABLE III
Serum bilirubin (,umol/l) Serum albumin (g/l) Ascites
Grade A
Grade B
Grade C
< 25.7 > 35 Nil
> 51.3 < 30
Nil
25.7-51.3 30-35 Easily controlled Minimal
Excellent
Good
Encephalopathy Nutrition
Serum bilirubin,
Poorly controlled Advanced 'coma'
Poor, wasting
i
pmol/l
=
approximately o.o6 mg/Ioo ml
152
R Kirby, F D Burke, and J D T Jones
of the patient. Because of the variability of operative procedure and the inadequate documentation and follow-up in the first 13 % Mortality years of study the results of emergency surgery for acute variceal haemorrhage have been restricted to the period i962-72, during which time all such patients were treated A B C primarily by oesophageal transection, with the exception of I patient who had an emerFIG. I Mortality of oesogency shunt. The second group consists of phageal transection related the 67 patients who had a portacaval anasto grading of preoperative tomosis, all but 4 as an elective procedure, liver function. either after an earlier episode of haemorrhage controlled by medical management or after but mortality in the poor-risk Grade C an emergency transection. patients was 53% , and in fact, of the I2 postoperative deaths, 9 were in Grade C I) Acute uncontrollable bleeding: patients. Of the 8 survivors of this group, 7 Since I 962 had died within 6 months from liver failure oesophageal transection 54 patients, with an average age of 48 or further haemorrhage and all within i (3-79) years, have been treated by oeso- year, so that the mortality of the poor-risk phageal transection. Of these, I 2 died in the cirrhotic is I 00% in the first year. The I -year survival, inclusive of operative postoperative period, giving an overall mortality of 22.2%. There were 8 patients with mortality, was 50°/O overall and 42 % for conditions other than cirrhosis, and if these cirrhotics. Thirty-two per cent of cirrhotic are excluded the mortality for cirrhotics only patients were alive after 2 years. Despite the was 26.6%. Six died in liver failure 5-6o d fact that all the survivors were investigated after surgery, two of whom were comatose at with a view to a shunt procedure being the time of operation and never recovered carried out, only 4 (12%) of the 33 cirrhotic consciousness; pig liver perfusion improved patients who survived operation did in fact the biochemical status of i patient but did eventually have a portacaval shunt pernot affect the level of consciousness. Three formed. Three of these are doing well after I patients died from haemorrhage and i from year, 5 years, and io years respectively: the a combination of liver failure and haemor- fourth patient left the region and is untraced. rhage. There was I death from bronchopneu- This low figure emphasizes the fact that the monia and i patient died from sepsis result- majority of patients with severe bleeding who ing from breakdown of the oesophageal anas- require emergency operation are not, and tomosis and fistula formation with empyema. never will be, suitable candidates for a shunt Employing Child's criteria, it is seen that on the basis of the criteria for selection adoptthe mortality rose dramatically with the ed on this unit, and this is the main reason decrease in hepatic reserve (Fig. I). In the why an emergency shunt has not been pergood-risk Grade A patients mortality was formed except under extenuating circum5.2%, the single death being in a 74-year-old stances. man who developed bronchopneumonia. Oesophageal transection failed to control Grade B patients had a mortality of ii.i%, bleeding in only 4 cases out of 54 (7.4%).
Emergency and elective surgical treatment of portal hypertension One of these patients subsequently had an emergency portacaval shunt and survived, a second had a devascularization procedure and splenectomy and survived, and 2 died from continuing haemorrhage. There is therefore only a very low incidence of operation failure, and since the transection site may be above the site of varix bleeding its effectiveness presumably relies on the postoperative thrombosis of varices on both sides of the suture line. The incidence of rebleeding (after discharge from hospital) was 26% in the first year and in only a minority of these cases wvas further surgery feasible. The difficulties that may be encouintered are illustrated by the following 2 cases. This young woman was jaundiced for in 1958 when aged 20 years, and 3 years later had a small haematemesis which was treated conservatively without a definite diagnosis being made. In I963, when 37 weeks pregnant, she had repeated massive haematemeses, splenomegaly was noted for the first time, and a barium swallow demonstrated large oesophageal varices. Bleeding continued and an emergency Caesarean section secured a viable infant; 6 h later and after the transfusion of 42 units of blood an oesophageal transection was performed which succeeded in arresting haemorrhage. Subsequent investigations confirmed postnecrotic hepatic cirrhosis and she had an elective portacaval shunt 3 months after years later delivery. She survives in good health with no further episodes of gastroinitestinal bleeding.
Case
i
3 weeks
I 53
uneventful pregnancy had a sizeable haematemesis and was transfused. The following day she was delivered of a normal infant and had a further
haematemesis; barium swallow showed oesophageal varices. No further haemorrhages occurred, but I week after delivery she developed peritonitis, and laparotomy showed a venous infarction involving 90 cm (3 ft) of upper jejunum, which was resected. The liver was normal as noted at the previous operation. The platelet count at this time was 940 X 1o9/l (940 ooo/ud). It appeared highly probable that she had developed portal hypertension secondary to splenic and portal vein thrombosis following splenectomy, but she refused further investigation and rejected the possibility of operation. Nine months later she was admitted to her local hospital with very severe haematemesis and melaena and was eventually transferred after transfusion of 40 units of blood. An oesophageal transection was performed shortly after admission and arrested the haemorrhage. Further investigation was again refused, but fortunately she has not had any further bleeding episodes in the 7 years since operation.
In the period 2) Portacaval shunt I952-72 67 end-to-side portacaval shunts were performed. The records of 2 cases are not available, leaving 65 for study. This group consists of patients who had had at least one haemorrhage from oesophageal varices, the majority having initially responaed to medical treatment. Four patients had previously had an oesophageal transection for acute bleeding. Two of these had a shunt performed as an emergency after failure of the transection to control haemorrhage and 2 A woman aged 22 with hereditary Case spherocytosis underwent splenectomy and removal others had an emergency shunt for additional of a pigment stone from the gallbladder in I964. reasons. The remaining 6I patients underThere was a strong family history of the disease went elective operation after a period of inand the patient had had symptoms since the age tensive medical treatment. of years. She recovered well from operation The age distribution was 9-70 (average 43) with no specific complaints, but her platelet count increased and she was given anticoagulant therapy years and the sex distribution was approxiwith heparin and phenindione (Dindevan) when mately equal (33 male, 32 female). this reached IOOO X io9/1 (i 000 ooo/ul). Shortly Of the 65 patients, 7 died in the postoperaafter discharge from hospital she became pregnant tive period, an overall mortality of io.8/./ but aborted at 8 weeks and at this time she had a of the study small haematemcsis. She immediately became preg- Mortality in the first decade of 28 shunts) and nant again ancl in the 39th week of an otherwisc was 17.90/0 (5 deaths out io
2
ii
154
R Kirby, F D Burke, and J D T Jones
in the second decade 5.4% (2 deaths out of 37 shunts). Liver failure accounted for 4 deaths 2-I9 days after operation. One young patient with chronic active hepatitis on steroids for 3 years before operation developed breakdown of the thoracoabdominal incision and died from sepsis and liver failure 8 weeks after a shunt. Two patients died from haemorrhage; I had a fatal haematemesis while convalescing and autopsy showed a thrombosed shunt, while the other had an unidentified bleeding diathesis and died on the day of operation. Using Child's classification of the degree of hepatic reserve the operative mortality is seen to be very high in Grade C patients (Fig. 2). There were 38 good-risk Grade A patients with 2 operative deaths (5.5s%), i8 Grade B patients of whom 2 died (ii.i%), and 9 poor-risk Grade C patients with 3 deaths (33.30/O) Of 4 patients undergoing emergency shunts, 2 died, and both of these were poorrisk cases. Both the survivors were good risks in whom a transection had failed to control
100 80 60
In .
_
40 20 [ I]IL
..
1
I
I
I
I
2
3
4
I
5 Years
I
I
I
I
I
6
7
8
9
10
FIG. 3 Survival of patients treated by portacaval shunt.
io years (Fig. 3). Eight patients in this group had a shunt for portal hypertension due to congenital hepatic fibrosis, and if these are excluded the 5-year survival for cirrhotics only is 43.5%/,. The longest survivor had a shunt I 9 years ago and remained in good health for I7 years, since when she has had mild symptoms of PSE. Shunt thrombosis was demonstrated in 6 bleeding. patients (9°%). Two of these are still alive, Survival figures include operative mortality although both have had further haemorrhages but are uncorrected. Seventy-nine per cent of from varices and i has recently had a sucpatients survived i year after shunt, 5I.2% cessful mesentericocaval shunt. Two others survived 5 years, and i8% were alive after died from haemorrhage, I in the postoperative period and the other 2 years after operation. 60 One girl with congenital hepatic fibrosis died 7 years after a shunt with peritonitis second50 to mesenteric venous infarction, and the ary t 40 t shunt was thrombosed at autopsy. It is not2 30 able that of the 4 children (I 4 years or less
ASPECTS OF TREATMENT*
Emergency and elective surgical treatment of portal hypertension A review of 23 years' experience R Kirby FRCS MSt F D Burke FRCS J D T Jones MS FRCS
University Department of Surgery, Royal Victoria Infirmary, Newcastle upon Tyne
Summary A retrospective review of surgical treatment for portal hypertension durinig a 23-year period in a regional unit is reported and the immediate and subsequent mnanagement of patients with; bleeding oesophageal varices is discussed. Fifty-four patients with recurrent varix haemorrhage uncontrolled by conservative methods have been treated by oesophageal transection with a mortality of 22.2% (26.66% for cirrhotic patients). Thirty-two per cent of the cirrhotics were alive after 2 years. Only a minority (I2%/,) of the survivors were considered suitable for a subsequent shunt proced ure. Therapeutic portacaval anastomosis has been performed on 65 patients with a 5I.2%/ 5-year survival (43.5% for cirrhotic patients). Further haemorrhage due to shunt thrombosis occurred in. 5.3%° of cases. The frequent occurrence of portal-systemic encephalopathy,
f Present
address: General Hospital, Hartlepool
increasing with duration of time following a shunt, is emphasized. The high morbidity and mortality in the poor-risk cirrhotic indicates that this type of patient is unsuitable for a portal-systemic shunt and is better treated by medical means.
Introduction Bleeding from gastro-oesophageal varices is a relatively uncommon but highly lethal cause of upper gastrointestinal tract haemorrhage, accounting for about 3% of such hospital admissions. Since it indicates portal hypertension, usually due to advanced hepatic disease, the mortality is high with all forms of treatment. Of 471 patients with cirrhosis and varices treated by non-operative means by the Boston Interhospital Liver Group over a 3-year period, 51 % experienced bleeding from varices and in 34% haemorrhage was the major cause of death. Only 31 % were alive i year after initial demonstration of varices and 5.5% after 5 years'. Mortality with the first large haemorrhage, treated by
Fellows interested in submitting papers for consideration with a v-cw to scrics should first wvrite to the Editor.
publicatiorn
in this
Emergentcy and elective surgical treatment of Portal hypertension medical to
means,
is high, varying from
30%
83%24.
Surgical treatment may be necessary to arrest uncontrollable varix haemorrhage or to provide portal decompression in a quiescent phase. Acute uncontrollable haemorrhage has been treated by direct ligation of varices5'7, transection of the oesophagus8, or emergency portal-systemic shunt9-". Since Blakemore and Lord first described the portacaval shunt in Ig945s a large number of reports have confirmed the effectiveness of this operation in preventing further haemorrhage from varices13'7. In recent years the side cffects of this procedure have been more fully appreciated and its use critically appraised"8. The clinical aspects of the management of severe variceal haemorrhage and the advisability or otherwise of a portal-systemic shunt remain difficult problems. In this retrospective review we illustrate the variety of cases presenting, formulate a plan of management, and analyse the results obtained in the surgical treatment of portal hypertension over a 23-year period in a regional centre. Clinical material During the period I 949-72 i6o patients have undergone surgical treatment for portal hypertension with oesophageal varices under the care of the senior author (J D T J) at the Royal Victoria Infirmary, Newcastle upon Tyne. Information has been gained from the patients' hospital records, from regional consultant staff in other hospitals, from general practitioners, and, when possible, by interviewing the survivors. Adequacy of follow-up data was variable in the earlier cases, and when the patients' subsequent fate was unknown they were presumed dead as from their last recorded outpatient visit in calculating survival figures. In all cases varices were demonstrated radiographically by barium swallow or splenic venography, by oesoph-
I49
agoscopy, or subsequently at operation or autopsy. Table I gives the underlying pathology in the i6o patients treated. In the majority (77 %) of the I43 in whom the pathology was recorded portal hypertension was secondary to portal cirrhosis. Of these, i8 patients (i 6%) were chronic alcoholics and the cirrhosis was presumed to be of alcoholic actiology, the remainder suffering from posthepatic or crvptogenic cirrhosis; this group included 3 cases of chronic active hepatitis progressing to cirrhosis. There were 2 cases of primary biliary cirrhosis, both in women, i of whom exhibited in addition Raynaud's phenomenon, digital calcinosis, and telangiectasia (CRSI syndrome). In i patient the portal hypertension was secondary to haemochromatosis and in another to haemosiderosis due to a long-standing haemolytic process (thalassaemia minor). There was i instance of thrombosis of the portal vein following splenectomy (for acholuric jaundice) and i patient had a relatively benign reticulosis (BrillSymmers disease), although the cause of the portal hypertension was never fully elucidated. Portal hypertension in childhood is most frequently due to an extrahepatic venous obstruction or congenital hepatic fibrosis. Ihere were I5 cases of extrahepatic venous obstruction in this series, of which a definite TABLE i Pathology in i6o patients treated surgically for portal hypertension, I949-72 Portal cirrhosis Cavernomatous transformation Congenital hepatic fibrosis Biliary cirrhosis Haemochromatosis Haemosiderosis (thalassaemia) Reticulosis Splenic vein thrombosis Nil abnormal
Unrecorded
I
o 15 1o 2 I I I I 2
I7
150
R Kirby, F D Burke, and J D T Jones
history of omphalitis was obtained in only I and neonatal exchange transfusion was a possible aetiological factor in 2 others. Some of these cases have been reported elsewhere". Congenital hepatic fibrosis accounted for of cases, 6 presenting in childhood, and these also had the associated renal cystic disease known to occur with this condition20. In 2 cases no apparent underlying cause was demonstrable to explain the undoubted portal hypertension. Both showed no evidence of extrahepatic obstruction on venography and the hepatic parenchyma was normal on gross and microscopic examination, although it is appreciated that biopsy specimens taken from the free edge of the liver may not be representative of the liver as a whole. Medical management With acute haemorrhage treatment consisted of blood transfusions, parenteral vitamin K, and the administration of enemas in an attempt to evacuate blood from the gastrointestinal tract. Neomycin or lactulose was given by mouth. In the absence of contraindication continued bleeding was treated with an intravenous infusion of vasopressin (20 U in I00 ml 5°% dextrose given over 30 min), repeated if necessary. If bleeding persisted or recurred balloon tamponade of the varices with the Sengstaken-Blakemore tube was applied for not more than 24 h. If bleeding recurred on deflating the balloons, as often happened, the balloons were reinflated and urgent surgery was performed. Blood loss of units, repeated severe haemmore than orrhage, or a single massive haemorrhage requiring a Sengstaken tube for control were the indications for operation. If, as was frequently the case, medical management was successful in arresting variceal haemorrhage, consideration was then given to the possibility of carrying out a portal-systemic shunt within the next 2-3 months. The policy has been to admit these io
i
io
patients to hospital i o d before the proposed operation to assess the operative risk and the likelihood of development of post-shunt portal-systemic encephalopathy (PSE), which is the principal complication and the reason why a high degree of selection is desirable. The criteria which indicate an acceptable risk are a serum bilirubin level below 50 ilmol/l (3 mg/ioo ml), serum albumin greater than 30 g/l (3 g/Ioo ml), normal plasma ammonium, normal electroencephalograms on a standard and high-protein diet, absence of ascites, and an upper age limit of 6o years. Occasionally, particularly in the earlier years of this series, some of these rigid criteria were transgressed when the majority were satisfactory and the risk of haemorrhage appeared inordinately high. Trans-splenic portal venography was employed to ensure a patent portal vein of reasonable calibre showing no evidence of previous thrombosis, and a liver biopsy was usually available to indicate the nature of the portal obstruction. Surgical management During the 23-year period several surgical procedures have been used and, not unnaturally, many have since been discarded. Table II lists the operations performed. Mediastinal packing, hepatic artery ligation, and oesophagogastric resection are not now performed. Oesophagoscopic injection of varices has been TABLE II Operations performed, I949-72 (I70 on i6o patients) Portacaval anastomosis Oesophageal transection Splenectomy and devascularization Splenectomy Splenorenal anastomosis Splenectomy and gastric section Omentopexy Ligation of hepatic artery Oesophagogastrectomy Boerema-Crile operation
67 54 27 7 5 3 2 2 2 I
Emergency and elective surgical treatment of portal hypertension
I 5I
found to be most unreliable and in this unit is Dissatisfaction with the high incidence of shunt thrombosis following splenorenal anasno longer used. Emergency treatment of acute varix haemorrhage between I949 and I96I tomosis has led to the adoption of the portausually consisted of devascularization of the caval anastomosis as the operation of choice in the first instance, and an end-to-side shunt upper stomach and lower oesophagus combined with splenectomy, but although fre- has been used exclusively in the last years. quently effective, this procedure is no longer advised in this situation since it carries the Results risk of thrombosis of the splenic and portal In assessing the overall results the patients veins, which may preclude any form of shunt have been divided into 3 groups depending procedure at a later date. Since I962 all on their degree of hepatic reserve according emergency cases have been treated by porta- to criteria suggested by Child21. The relevant clinical details include patient nutrition, the azygos disconnection in the form of a transthoracic oesophageal transection. By an ap- presence and degree of PSE and ascites, and proach through the bed of the resected 8th biochemical measurement of serum bilirubin rib the lower third of the oesophagus is and serum albumin (Table III). In emermobilized and the muscular coat incised gency cases grading was based on clinical longitudinally after application of a Satinski observation and biochemical liver function clamp at the gastro-oesophageal junction. The tests performed on admission to hospital, mucosal tube is freed and transected as low which was usually immediately after the down as possible and resutured with a con- onset of haemorrhage. Prolonged gastrointesttinuous 30-silk stitch, thereby occluding the inal bleeding rapidly leads to an increase in varices. The oesophageal musculature is serum bilirubin level and deterioration in reapproximated with interrupted catgut and liver function, and assessment at this stage is the chest closed, leaving an intercostal drain unreliable. Operative deaths are defined as death down to the site of the transection, this being within 28 days of operation or during the retained for 5 days. The ultimate aim has been the construc- period of hospital confinement following surtion of a portal-systemic shunt where possible, gery regardless of duration. The results will be considered in two basic either portacaval, splenorenal, or mesentericocaval, preferably as an elective procedure. groups according to the mode of presentation 20
Classification of preoperative liver function after Child2"
TABLE III
Serum bilirubin (,umol/l) Serum albumin (g/l) Ascites
Grade A
Grade B
Grade C
< 25.7 > 35 Nil
> 51.3 < 30
Nil
25.7-51.3 30-35 Easily controlled Minimal
Excellent
Good
Encephalopathy Nutrition
Serum bilirubin,
Poorly controlled Advanced 'coma'
Poor, wasting
i
pmol/l
=
approximately o.o6 mg/Ioo ml
152
R Kirby, F D Burke, and J D T Jones
of the patient. Because of the variability of operative procedure and the inadequate documentation and follow-up in the first 13 % Mortality years of study the results of emergency surgery for acute variceal haemorrhage have been restricted to the period i962-72, during which time all such patients were treated A B C primarily by oesophageal transection, with the exception of I patient who had an emerFIG. I Mortality of oesogency shunt. The second group consists of phageal transection related the 67 patients who had a portacaval anasto grading of preoperative tomosis, all but 4 as an elective procedure, liver function. either after an earlier episode of haemorrhage controlled by medical management or after but mortality in the poor-risk Grade C an emergency transection. patients was 53% , and in fact, of the I2 postoperative deaths, 9 were in Grade C I) Acute uncontrollable bleeding: patients. Of the 8 survivors of this group, 7 Since I 962 had died within 6 months from liver failure oesophageal transection 54 patients, with an average age of 48 or further haemorrhage and all within i (3-79) years, have been treated by oeso- year, so that the mortality of the poor-risk phageal transection. Of these, I 2 died in the cirrhotic is I 00% in the first year. The I -year survival, inclusive of operative postoperative period, giving an overall mortality of 22.2%. There were 8 patients with mortality, was 50°/O overall and 42 % for conditions other than cirrhosis, and if these cirrhotics. Thirty-two per cent of cirrhotic are excluded the mortality for cirrhotics only patients were alive after 2 years. Despite the was 26.6%. Six died in liver failure 5-6o d fact that all the survivors were investigated after surgery, two of whom were comatose at with a view to a shunt procedure being the time of operation and never recovered carried out, only 4 (12%) of the 33 cirrhotic consciousness; pig liver perfusion improved patients who survived operation did in fact the biochemical status of i patient but did eventually have a portacaval shunt pernot affect the level of consciousness. Three formed. Three of these are doing well after I patients died from haemorrhage and i from year, 5 years, and io years respectively: the a combination of liver failure and haemor- fourth patient left the region and is untraced. rhage. There was I death from bronchopneu- This low figure emphasizes the fact that the monia and i patient died from sepsis result- majority of patients with severe bleeding who ing from breakdown of the oesophageal anas- require emergency operation are not, and tomosis and fistula formation with empyema. never will be, suitable candidates for a shunt Employing Child's criteria, it is seen that on the basis of the criteria for selection adoptthe mortality rose dramatically with the ed on this unit, and this is the main reason decrease in hepatic reserve (Fig. I). In the why an emergency shunt has not been pergood-risk Grade A patients mortality was formed except under extenuating circum5.2%, the single death being in a 74-year-old stances. man who developed bronchopneumonia. Oesophageal transection failed to control Grade B patients had a mortality of ii.i%, bleeding in only 4 cases out of 54 (7.4%).
Emergency and elective surgical treatment of portal hypertension One of these patients subsequently had an emergency portacaval shunt and survived, a second had a devascularization procedure and splenectomy and survived, and 2 died from continuing haemorrhage. There is therefore only a very low incidence of operation failure, and since the transection site may be above the site of varix bleeding its effectiveness presumably relies on the postoperative thrombosis of varices on both sides of the suture line. The incidence of rebleeding (after discharge from hospital) was 26% in the first year and in only a minority of these cases wvas further surgery feasible. The difficulties that may be encouintered are illustrated by the following 2 cases. This young woman was jaundiced for in 1958 when aged 20 years, and 3 years later had a small haematemesis which was treated conservatively without a definite diagnosis being made. In I963, when 37 weeks pregnant, she had repeated massive haematemeses, splenomegaly was noted for the first time, and a barium swallow demonstrated large oesophageal varices. Bleeding continued and an emergency Caesarean section secured a viable infant; 6 h later and after the transfusion of 42 units of blood an oesophageal transection was performed which succeeded in arresting haemorrhage. Subsequent investigations confirmed postnecrotic hepatic cirrhosis and she had an elective portacaval shunt 3 months after years later delivery. She survives in good health with no further episodes of gastroinitestinal bleeding.
Case
i
3 weeks
I 53
uneventful pregnancy had a sizeable haematemesis and was transfused. The following day she was delivered of a normal infant and had a further
haematemesis; barium swallow showed oesophageal varices. No further haemorrhages occurred, but I week after delivery she developed peritonitis, and laparotomy showed a venous infarction involving 90 cm (3 ft) of upper jejunum, which was resected. The liver was normal as noted at the previous operation. The platelet count at this time was 940 X 1o9/l (940 ooo/ud). It appeared highly probable that she had developed portal hypertension secondary to splenic and portal vein thrombosis following splenectomy, but she refused further investigation and rejected the possibility of operation. Nine months later she was admitted to her local hospital with very severe haematemesis and melaena and was eventually transferred after transfusion of 40 units of blood. An oesophageal transection was performed shortly after admission and arrested the haemorrhage. Further investigation was again refused, but fortunately she has not had any further bleeding episodes in the 7 years since operation.
In the period 2) Portacaval shunt I952-72 67 end-to-side portacaval shunts were performed. The records of 2 cases are not available, leaving 65 for study. This group consists of patients who had had at least one haemorrhage from oesophageal varices, the majority having initially responaed to medical treatment. Four patients had previously had an oesophageal transection for acute bleeding. Two of these had a shunt performed as an emergency after failure of the transection to control haemorrhage and 2 A woman aged 22 with hereditary Case spherocytosis underwent splenectomy and removal others had an emergency shunt for additional of a pigment stone from the gallbladder in I964. reasons. The remaining 6I patients underThere was a strong family history of the disease went elective operation after a period of inand the patient had had symptoms since the age tensive medical treatment. of years. She recovered well from operation The age distribution was 9-70 (average 43) with no specific complaints, but her platelet count increased and she was given anticoagulant therapy years and the sex distribution was approxiwith heparin and phenindione (Dindevan) when mately equal (33 male, 32 female). this reached IOOO X io9/1 (i 000 ooo/ul). Shortly Of the 65 patients, 7 died in the postoperaafter discharge from hospital she became pregnant tive period, an overall mortality of io.8/./ but aborted at 8 weeks and at this time she had a of the study small haematemcsis. She immediately became preg- Mortality in the first decade of 28 shunts) and nant again ancl in the 39th week of an otherwisc was 17.90/0 (5 deaths out io
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in the second decade 5.4% (2 deaths out of 37 shunts). Liver failure accounted for 4 deaths 2-I9 days after operation. One young patient with chronic active hepatitis on steroids for 3 years before operation developed breakdown of the thoracoabdominal incision and died from sepsis and liver failure 8 weeks after a shunt. Two patients died from haemorrhage; I had a fatal haematemesis while convalescing and autopsy showed a thrombosed shunt, while the other had an unidentified bleeding diathesis and died on the day of operation. Using Child's classification of the degree of hepatic reserve the operative mortality is seen to be very high in Grade C patients (Fig. 2). There were 38 good-risk Grade A patients with 2 operative deaths (5.5s%), i8 Grade B patients of whom 2 died (ii.i%), and 9 poor-risk Grade C patients with 3 deaths (33.30/O) Of 4 patients undergoing emergency shunts, 2 died, and both of these were poorrisk cases. Both the survivors were good risks in whom a transection had failed to control
100 80 60
In .
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40 20 [ I]IL
..
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I
I
I
I
2
3
4
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5 Years
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6
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FIG. 3 Survival of patients treated by portacaval shunt.
io years (Fig. 3). Eight patients in this group had a shunt for portal hypertension due to congenital hepatic fibrosis, and if these are excluded the 5-year survival for cirrhotics only is 43.5%/,. The longest survivor had a shunt I 9 years ago and remained in good health for I7 years, since when she has had mild symptoms of PSE. Shunt thrombosis was demonstrated in 6 bleeding. patients (9°%). Two of these are still alive, Survival figures include operative mortality although both have had further haemorrhages but are uncorrected. Seventy-nine per cent of from varices and i has recently had a sucpatients survived i year after shunt, 5I.2% cessful mesentericocaval shunt. Two others survived 5 years, and i8% were alive after died from haemorrhage, I in the postoperative period and the other 2 years after operation. 60 One girl with congenital hepatic fibrosis died 7 years after a shunt with peritonitis second50 to mesenteric venous infarction, and the ary t 40 t shunt was thrombosed at autopsy. It is not2 30 able that of the 4 children (I 4 years or less
