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Surgical Treatment of Esophageal Varices in Children
Surgical Treatment of Esophageal Varices in Children HUGH B. LYNN, M.D., F.A.C.S.*
Knowledge of esophageal varices is still woefully meager, but physicians are becoming increasingly aware of the hemodynamics and the etiologies which create this condition. Esophageal varices, insofar as is known, are usually the result of portal hypertension. In the pediatric age group portal hypertension is a frustrating condition to handle. The lesion may be an intrahepatic portal obstruction due to hepatitis and cirrhosis or other fibrosing processes4 producing a dilated portal vein; more commonly, it may be an alteration of the extrahepatic portal system with obliteration of the main vein and attempted replacement by multiple varicosities and collateral channels. It is the lack of a satisfactory portal vein which limits the surgical therapy in most children. For years, authors have pointed out the relationship of omphalitis to portal hypertension. Recently, the number of patients presenting with a history of exchange transfusions by way of the umbilical vein has been striking. The validity of many of the histories of omphalitis is questionable, since a dried-up umbilical cord is a repulsive sight to many inexperienced parents. No close correlation has been noted between the degree of portal hypertension, the magnitude of the varicosities and the severity or frequency of hemorrhage. At the time of this study the surgical treatment of portal hypertension is directed toward decompression of the portal system. When this fails or is not feasible, obliteration or elimination of the dilated venous channels around the esophagogastric junction seems to offer the most satisfactory "last resort." Actually the large number of surgical procedures which have been advocated is testimony to their lack of efficacy. Comments on only the major categories of decompression, obliteration of varices and elimination of varices will be included in this presentation. From the Mayo Clinic, Rochester, Minnesota * Head of Section of Pediatric Surgery, Mayo Clinic; Assistant Professor of Surgery, Mayo Foundation for Medical Education and Research, Rochester, Minnesota
1423
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HUGH
B.
LYNN
No category has been created for the decrease in portal system flow brought about by elimination of the spleen. The all too frequent association of hypersplenism is well known and presents yet another decision which is complicated by the age and size of.the patient and, thus, by the size of the shunting vessels. Without any encouragement from this source, splenectomies will continue to be done at an early age without associated shunting. In fact, in many of these cases failure would result at this early stage, even if the operation were anatomically successful, just because of the low pressure gradient. Ligation of the splenic artery with preservation of the spleen has been employed by Everson and Cole. I have not had any personal experience with this procedure. Thrombosis of the splenic vein would almost certainly follow such a procedure. While hypersplenism is a real entity, undoubtedly low-grade intestinal bleeding or oozing accounts for anemia in many patients long before a true hemorrhage is encountered. Although general agreement is lacking, it is my feeling that esophageal varicosities in children should not be treated until there is real evidence of hemorrhage. The chance of a fatal first hemorrhage in a child is sufficiently remote to justify delaying any surgical therapy at least this long. In fact, I have tried to avoid operation until after the second true hemorrhage, although I have usually outlined future plans with great care after the first episode of bleeding. Certainly x-ray evidence of esophageal varices alone would not appear to justify surgical intervention before bleeding occurred. The definite corroboration of the x-ray findings by esophagoscopy seems only reasonable, and it has been pointed out by Conn and associates that both of these procedures have limitations. Where doubt exists, a splenoportogram is most helpful if the spleen is still present. Failing this, an operative portogram through a mesenteric vein with pressure readings is an essential step in approaching the operation. During the "delaying action," when an attempt is made to gain time, it has been advocated that antacids be given to avoid peptic esophagitis with erosion of the varices. While varices are present throughout the gastrointestinal tract, those around the esophagogastric junction have some partially unexplained characteristics which make them more available to stress, engorgement and abuse. It would seem reasonable that gastric juices would not be beneficial (despite the report of Orloff and Thomas) and that antacids might well be given. From a realistic point of view, however, these children are usually great consumers of milk and milk products, and it is doubtful if any child who is not actually having symptoms would be bothered by such medications over any protracted period. While I prescribe antacids during hospitalization for bleeding varices, inclusion of such treatment in long-term plans seems pointless since there is no valid evidence of theiefficacy of such medications.
SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
1425
DECOMPRESSION
Basic Criteria In children, the presence of ascites and a low value for serum protein have not proved to be more than temporary deterrents. Intensive treatment with a high protein and high carbohydrate diet and daily intravenous infusions of albumin and blood have been rewarding in most cases; bed rest and supplemental vitamins also have seemed worthwhile. A value for serum albumin of less than 3 gm. per 100 ml. has been accepted rather generally as a dangerous level. Before attempting surgical measures it is essential that the prothrombin time be within reasonably normal limits. With vitamin K therapy, any deviation of more than 3 to 4 seconds above the control would be a poor prognostic sign. The achievement of a successful shunt requires a pressure gradielllt sufficient to prevent thrombosis of the stoma. It is impossible to know how valid the figure of 300 mm. of water really is. Certainly, the higher the pressure the more likely is the possibility of a functioning shunt. Many surgeons have obtained successful results with lower pressure readings; however, when the pressure approaches the upper limit of normal variation (150 to 225 mm. of water) the chance of a successful shunt fades and at the same time the question of its need or advisability arises.
Direct Shunts PORTACAVAL AND MESENTERIC CAVAL SHUNTS. Once the patient has reached the point where an operation is required, there is remarkably universal agreement as to the operation of choice. A portacaval shunt would seem to give the best results. In my own experience, patients in the pediatric age group who qualify for this procedure are remarkably few. The last two portacaval shunts in the Mayo Clinic series under study actually were anastomoses between the proximal end of the transected inferior vena cava and a terminal dilatation of the portal vein just proximal to the junction of the splenic remnant and the superior mesenteric vein. These were both intended to be mesenteric-caval shunts but the dilated region was exposed after persistent dissection because in each instance the mesenteric vessel alone appeared rather small. SPLENORENAL SHUNT. In the absence of a suitable portal vein the splenorenal anastomosis seems most desirable. Technically, a portacaval shunt can be accomplished at almost any age, but the splenorenal end-toside anastomosis appears to cause great difficulty and has little chance of success in young children. In general, there have been few successes among children less than seven or eight years of age. It has been pointed out by
1426
HUGH
B.
LYNN
Boles and Clatworthy that dissection of the splenic vein behind, or into the substance of, the pancreas will facilitate the procedure and does seem to give a larger shunt without kinking or angulation. Cooley has pointed out the feasibility of performing this shunt as a side-to-side anastomosis and of preserving the spleen. Such a procedure might make the shunt available to a younger age group. When neither of these long-accepted procedures is possible, transection of the inferior vena cava and end-to-side anastomosis with a suitable mesenteric vessel is often possible. In the small Mayo Clinic series, ligation of the inferior vena cava has not produced any of the complications which might be anticipated. The success of shunting procedures can be judged only on the basis of prolonged freedom from hemorrhage. Certainly the natural history of patients who receive any form of therapy is so variable as to be temporarily encouraging. A single specific test for the patency of a shunt has long been needed. While angiography may demonstrate the functioning shunt, it is a fairly formidable procedure. Tests have been described by Eiseman and co-workers and by Schwartz in which attempts have been made to establish the patency of the shunt. These are not without some risk, and Schwartz' isotopic evaluation is possible only when the spleen is present. Quite possibly the fructose test described by Martin and Bryant will prove to be the solution. Certainly every surgeon would like to have some confirmation of the success of the operation he has performed, and the fructose excretion test on the urine seems, thus far, to provide a reliable, simple and safe method of checking.
Indirect Shunting The methods of indirect shunting are less appealing from a clinical standpoint and seem to be more theoretical than practical. However, when one reviews the results of past therapy in children, and particularly the group less than eight years of age, it is obvious that any form of treatment which offers any promise whatsoever must be explored and evaluated. SUPRADIAPHRAGMATIC TRANSPOSITION OF SPLEEN. Supradiaphragmatic transposition of the spleen for relief of bleeding of esophageal varices was reported by Nylander and Turunen in 1955. The presence of a dense collateral network of veins surrounding such a displaced spleen in a war casualty led them to this field of research. Clinical reports concerning this method, which deserves further trial, are encouraging and greater effort should be made to promote the use of this procedure prior to the inevitable splenectomy. OMENTOCAVOPEXY AND THORACIC SUBCUTANEOUS ILEOPEXY. Berman and associates have recently advocated and performed omentocavopexy with some success although follow-up periods are still too short to be significant. More recently Bader and associates have described a method, which is employed as yet only in the laboratory, of establishing
SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
1427
a portosystemic shunting system by thoracic subcutaneous ileopexy. This procedure is intriguing and one which I intend to investigate with the hope of applyi~g it clinically. Other indirect shunts, including poudrage, have been attempted, and undoubtedly still other methods will be devised. All procedures will require prolonged and cautious review. Unless the actual volume of shunted blood represents a major portion of the portal circulation, it is not likely that indirect shunts will produce long-term relief. OBLITERATION OF VARICES
By Injection Many attempts have been made to obliterate varices in the esophagogastric region. The direct injection of sclerosing substances through an esophagoscope has been advocated. The nature of this procedure is such that there is little chance that it will ever become a commonly used method in the pediatric age group.
By Suture Transthoracic ligation and oversewing of the varicosities have been somewhat successful. Almost every author has had some notable results with this procedure. In my experience it has been almost a complete failure, and whatever control of hemorrhage it provided has, in my opinion, been supplanted by intelligent use of the Sengstaken-Blakemore tube. While use of this tube is distasteful to me and its prolonged use in small children is both difficult and hazardous, it has served a purpose in controlling hemorrhage and has allowed opportunity to gain time and to regroup not only the demoralized resources of the patient but also those of the hospital team. Gentle traction on the inflated lower balloon has usually proved adequate to control bleeding, and it is my impression that the young patient tends to be more comfortable when the upper balloon is not inflated. ELIMINATION OF VARICES
Esopbagogastrectomy In the event that no direct shunt can be established and no satisfactory indirect shunt can be produced, elimination of the superficial varices around the esophagogastric junction seems to be the last resort. Excision of this critical portion, with reanastomosis of the esophagus to the stomach and usually with creation of a hiatal hernia, has had its advocates. In an attempt to avoid hiatal hernia I have tried to eliminate the varices by transecting the stomach just below the esophagogastric junction and, after excising a
1428
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LYNN
narrow segment of stomach, by reanastomosing the gastric segments. One patient bled severely from his varices while still convalescing from this procedure and none was relieved for a prolonged period.
Colon Interposition While esophagogastrectomy with interposition of the colon seems unphysiologic and cumbersome, it seems to be the most satisfactory method available at the time of this report. It certainly permits wide excision of the critical region and anastomosis without tension, herniation, or other adverse situations; it has been employed effectively both as a means of gaining time and growth and as a last resort. The exposure of the esophageal mucosa to gastric juices is reduced or eliminated by this method. To date, results at this clinic have been most encouraging and my own total experience has been most gratifying, considering the condition of the patients presenting for such therapy. Many surgeons have used the jejunum for interposition substitution. However, the sensitivity of the jejunal mucosa to gastric juices and the cumbersome sacculated nature of the segment necessary because of the blood supply have made it less desirable. My experience has been limited to those situations in which colon was not available, or to conditions other than portal hypertension in which the greater curvature could not be used to form an esophageal tube.
RESULTS IN MAYO CLINIC SERIES
From 1950 through 1963, 40 children have undergone surgical treatment for bleeding esophageal varices at the Mayo Clinic. A total of 72 surgical procedures have been performed on these patients. Of the 72 Table 1. Surgical Procedures Prior to Registration at Mayo Clinic PROCEDURE
NUMBER OF OPERATIONS
Splenectomy....................... . Extrahepatic ...................... Intrahepatic ..................... , Splenorreal shunt .................. . Extrahepatic ...................... Intrahepatic ...................... Portacaval shunt ................... . Ligation of varices (extrahepatic) .................... . Exploration-no shunt (extrahepatic) .................. . TOTAL . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
9 7 2 5 4 1 1
2 1
18
1429
SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
procedures, 18 were performed prior to patients' registration at this clinic (Table 1), 49 were performed at this clinic, and 5 were performed subsequent to patients leaving this clinic (Table 2). The list of surgical procedures performed on these 40 patients is impressive (Table 3). Of the 40 patients, 31 had extrahepatic obstruction and nine intrahepatic obstruction. There were 22 boys and nine girls with extrahepatic blockage and two boys and seven girls with intrahepatic blocks. Follow-up reports indicate that two of the 40 patients have not undergone splenectomy. One of the nine patients with intrahepatic obstruction had a portacaval shunt as an initial procedure and no subsequent operation has been
Table 2. Surgical Procedures Since Mayo Clinic Treatment EXTRAHEPATIC
NUMBER OF OPERATIONS
Esophagogastrectomy. . . . . . . . . . . .. 1 Ligation of varices ................ 1 Shunt (portamesenteric) ........... 1.. TOTAL . . . . . . . . . . . . • . . • • . . . • •
NUMBER OF OPERATIONS
INTRAHEPATIC
Interposition (colon). . . . . . . . . .. 1* Ligation of varices ............. It
3t
2
* Had splenorenal shunt at Mayo Clinic.
t Had portacaval shunt at Mayo Clinic. t Each had splenectomy at Mayo Clinic.
Table 3.
Operations Performed at the Mayo Clinic on 40 Pediatric Patients With Diagnosis of Portal Hypertension (1950-1963) TYPE OF PROCEDURE
NUMBER OF PATIENTS
Splenectomy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 24 Splenorenal shunt ..................................... , 14 Ligation of varices ..................................... 14 Portacaval shunt ...................................... , 7 Interposition (colon, 5; jejunum, 2). . . . . . . . . . . . . . . . . . . . . . . 7 Partial esophagogastrectomy With esophagogastrostomy. . . . . . . . . . . . . . . . . . . . . . . . . . .. 1 With esophagojejunostomy (end-to-end) and jejunojejunostomy (end-to-side). . . . . . . . . . . . . . . . . . . . . . . . . .. 1 With esophagogastrostomy and partial gastrectomy with Schoemaker type Billroth I gastroduodenostomy. . . 1 With esophagojejunostomy (end-to-side) Roux Y........... 1 Left nephrectomy, left renal artery obstruction after splenorenal shunt......... . . . . . . . . . . . . . . . . . . . . . . . . . . .. 1 Negative exploration for possible portacaval shunt. ... .... .. 1 TOTAL . • • • • • • . . . . . . . . . . . . . . . . • • . . . . . . . . . . . . . • • . . . • 72
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performed. One of the 31 patients having an extrahepatic obstruction had transthoraeic ligation of varices as the primary operation and had had no further procedures when last contacted.
SUMMARY
The surgical treatment of esophageal varices in children is a discouraging problem. In light of present knowledge, the longer such treatment can be delayed the better the chance of a functioning shunt. While a portacaval shunt is preferable and technically feasible in small children, this procedure cannot be carried out in most children with portal hypertension since it is more commonly due to extrahepatic blockage. A splenorenal shunt is desirable but not usually successful in children less than seven or eight years of age; in the event of failure, a mesenteric caval shunt is often satisfactory. Any temporizing procedure which can prevent a recurrence of the hemorrhage and still preserve the splenic vein for future use seems justifiable in small children.
REFERENCES 1. Bader, Karl, Jr., Roseman, D. L., Economou, B. G. and Beattie, E. J., Jr.: Portalsystemic venous shunting by thoracic subcutaneous ileopexy. Read at the meeting of the Central Surgical Association, Rochester, Minnesota, February 27 to 29, 1!)64. 2. Berman, E. J., Waite, Paul, Gerig, E. L. and Bakemier, R. E.: Omentocavopexy: Further analysis. Arch. Burg. 86: 1008-1013 (June) 1963. 3. Boles, E. T., Jr. and Clatworthy, H. W., Jr.: Diseases of the spleen and portal circulation. In Benson, C. D.: Pediatric Surgery, Chicago, Year Book Medical Publishers, Inc., 1962, Vol. 1, pp. 639-653. 4. Boley, B. J., Arlen, Myron, and Mogilner, L. J.: Congenital hepatic fibrosis causing portal hypertension in children. Burgery 54: 356-360 (Aug.) 1963. 5. Conn, H. 0., Mitchell, J. R. and Brodoff, M. G.: A comparison of the radiologic and esophagoscopic diagnosis of esophageal varices. New England J. Med. 265: 160-164 (July 27) 1961. 6. Cooley, D. A.: Discussion. Ann. Burg. 157: 880--881 (June) 1963. 7. Eiseman, B., Lindeman, G. M. and Clark, G. M.: Clinical evaluation of the ammonium citrate tolerance test for determining the patency of a portacaval shunt. J. Lab. & Clin. Med. 48: 579-588 (Oct.) 1956. 8. Everson, T. C. and Cole, W. H.: Ligation of the splenic artery in patients with portal hypertension. Arch. Burg. 56: 153-160 (Feb.) 1948. 9. Martin, L. W. and B'ryant, L. R.: Use of fructose to detc'rmine the patency of portalsystemic shunts. Arch. Burg. 85: 783-790 (Nov.) 1962. 10. Nylander, P. E. A. and Turunen, Martti: Transposition of the spleen into the thoracic cavity in cases of portal hypertension. Ann. Surg. 142: 954-956 (Dec.) 1955. 11. Orloff, M. J. and Thomas, H. B.: Pathogenesis of esophageal varix rupture: A study based on groBS and microscopic examination of the esophagus at thetime of bleeding. Arch. Burg. 87: 301-307 (Aug.) 1963. 12. Schwartz, B.!.:: Isotopic evaluation of portal circulation: Diagnosis of esophagogastric varices. Bull. Soc. Internat. Chir. 21: 290--303, 1962.
SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
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13. Sengstaken, R. W. and Blakemore, A. H.: Balloon tamponage for the control of hemorrhage from esophageal varices. Ann. Surg. 131: 781-789 (May) 1950. Mayo Clinic Rochester, Minnesota
Knowledge of esophageal varices is still woefully meager, but physicians are becoming increasingly aware of the hemodynamics and the etiologies which create this condition. Esophageal varices, insofar as is known, are usually the result of portal hypertension. In the pediatric age group portal hypertension is a frustrating condition to handle. The lesion may be an intrahepatic portal obstruction due to hepatitis and cirrhosis or other fibrosing processes4 producing a dilated portal vein; more commonly, it may be an alteration of the extrahepatic portal system with obliteration of the main vein and attempted replacement by multiple varicosities and collateral channels. It is the lack of a satisfactory portal vein which limits the surgical therapy in most children. For years, authors have pointed out the relationship of omphalitis to portal hypertension. Recently, the number of patients presenting with a history of exchange transfusions by way of the umbilical vein has been striking. The validity of many of the histories of omphalitis is questionable, since a dried-up umbilical cord is a repulsive sight to many inexperienced parents. No close correlation has been noted between the degree of portal hypertension, the magnitude of the varicosities and the severity or frequency of hemorrhage. At the time of this study the surgical treatment of portal hypertension is directed toward decompression of the portal system. When this fails or is not feasible, obliteration or elimination of the dilated venous channels around the esophagogastric junction seems to offer the most satisfactory "last resort." Actually the large number of surgical procedures which have been advocated is testimony to their lack of efficacy. Comments on only the major categories of decompression, obliteration of varices and elimination of varices will be included in this presentation. From the Mayo Clinic, Rochester, Minnesota * Head of Section of Pediatric Surgery, Mayo Clinic; Assistant Professor of Surgery, Mayo Foundation for Medical Education and Research, Rochester, Minnesota
1423
1424
HUGH
B.
LYNN
No category has been created for the decrease in portal system flow brought about by elimination of the spleen. The all too frequent association of hypersplenism is well known and presents yet another decision which is complicated by the age and size of.the patient and, thus, by the size of the shunting vessels. Without any encouragement from this source, splenectomies will continue to be done at an early age without associated shunting. In fact, in many of these cases failure would result at this early stage, even if the operation were anatomically successful, just because of the low pressure gradient. Ligation of the splenic artery with preservation of the spleen has been employed by Everson and Cole. I have not had any personal experience with this procedure. Thrombosis of the splenic vein would almost certainly follow such a procedure. While hypersplenism is a real entity, undoubtedly low-grade intestinal bleeding or oozing accounts for anemia in many patients long before a true hemorrhage is encountered. Although general agreement is lacking, it is my feeling that esophageal varicosities in children should not be treated until there is real evidence of hemorrhage. The chance of a fatal first hemorrhage in a child is sufficiently remote to justify delaying any surgical therapy at least this long. In fact, I have tried to avoid operation until after the second true hemorrhage, although I have usually outlined future plans with great care after the first episode of bleeding. Certainly x-ray evidence of esophageal varices alone would not appear to justify surgical intervention before bleeding occurred. The definite corroboration of the x-ray findings by esophagoscopy seems only reasonable, and it has been pointed out by Conn and associates that both of these procedures have limitations. Where doubt exists, a splenoportogram is most helpful if the spleen is still present. Failing this, an operative portogram through a mesenteric vein with pressure readings is an essential step in approaching the operation. During the "delaying action," when an attempt is made to gain time, it has been advocated that antacids be given to avoid peptic esophagitis with erosion of the varices. While varices are present throughout the gastrointestinal tract, those around the esophagogastric junction have some partially unexplained characteristics which make them more available to stress, engorgement and abuse. It would seem reasonable that gastric juices would not be beneficial (despite the report of Orloff and Thomas) and that antacids might well be given. From a realistic point of view, however, these children are usually great consumers of milk and milk products, and it is doubtful if any child who is not actually having symptoms would be bothered by such medications over any protracted period. While I prescribe antacids during hospitalization for bleeding varices, inclusion of such treatment in long-term plans seems pointless since there is no valid evidence of theiefficacy of such medications.
SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
1425
DECOMPRESSION
Basic Criteria In children, the presence of ascites and a low value for serum protein have not proved to be more than temporary deterrents. Intensive treatment with a high protein and high carbohydrate diet and daily intravenous infusions of albumin and blood have been rewarding in most cases; bed rest and supplemental vitamins also have seemed worthwhile. A value for serum albumin of less than 3 gm. per 100 ml. has been accepted rather generally as a dangerous level. Before attempting surgical measures it is essential that the prothrombin time be within reasonably normal limits. With vitamin K therapy, any deviation of more than 3 to 4 seconds above the control would be a poor prognostic sign. The achievement of a successful shunt requires a pressure gradielllt sufficient to prevent thrombosis of the stoma. It is impossible to know how valid the figure of 300 mm. of water really is. Certainly, the higher the pressure the more likely is the possibility of a functioning shunt. Many surgeons have obtained successful results with lower pressure readings; however, when the pressure approaches the upper limit of normal variation (150 to 225 mm. of water) the chance of a successful shunt fades and at the same time the question of its need or advisability arises.
Direct Shunts PORTACAVAL AND MESENTERIC CAVAL SHUNTS. Once the patient has reached the point where an operation is required, there is remarkably universal agreement as to the operation of choice. A portacaval shunt would seem to give the best results. In my own experience, patients in the pediatric age group who qualify for this procedure are remarkably few. The last two portacaval shunts in the Mayo Clinic series under study actually were anastomoses between the proximal end of the transected inferior vena cava and a terminal dilatation of the portal vein just proximal to the junction of the splenic remnant and the superior mesenteric vein. These were both intended to be mesenteric-caval shunts but the dilated region was exposed after persistent dissection because in each instance the mesenteric vessel alone appeared rather small. SPLENORENAL SHUNT. In the absence of a suitable portal vein the splenorenal anastomosis seems most desirable. Technically, a portacaval shunt can be accomplished at almost any age, but the splenorenal end-toside anastomosis appears to cause great difficulty and has little chance of success in young children. In general, there have been few successes among children less than seven or eight years of age. It has been pointed out by
1426
HUGH
B.
LYNN
Boles and Clatworthy that dissection of the splenic vein behind, or into the substance of, the pancreas will facilitate the procedure and does seem to give a larger shunt without kinking or angulation. Cooley has pointed out the feasibility of performing this shunt as a side-to-side anastomosis and of preserving the spleen. Such a procedure might make the shunt available to a younger age group. When neither of these long-accepted procedures is possible, transection of the inferior vena cava and end-to-side anastomosis with a suitable mesenteric vessel is often possible. In the small Mayo Clinic series, ligation of the inferior vena cava has not produced any of the complications which might be anticipated. The success of shunting procedures can be judged only on the basis of prolonged freedom from hemorrhage. Certainly the natural history of patients who receive any form of therapy is so variable as to be temporarily encouraging. A single specific test for the patency of a shunt has long been needed. While angiography may demonstrate the functioning shunt, it is a fairly formidable procedure. Tests have been described by Eiseman and co-workers and by Schwartz in which attempts have been made to establish the patency of the shunt. These are not without some risk, and Schwartz' isotopic evaluation is possible only when the spleen is present. Quite possibly the fructose test described by Martin and Bryant will prove to be the solution. Certainly every surgeon would like to have some confirmation of the success of the operation he has performed, and the fructose excretion test on the urine seems, thus far, to provide a reliable, simple and safe method of checking.
Indirect Shunting The methods of indirect shunting are less appealing from a clinical standpoint and seem to be more theoretical than practical. However, when one reviews the results of past therapy in children, and particularly the group less than eight years of age, it is obvious that any form of treatment which offers any promise whatsoever must be explored and evaluated. SUPRADIAPHRAGMATIC TRANSPOSITION OF SPLEEN. Supradiaphragmatic transposition of the spleen for relief of bleeding of esophageal varices was reported by Nylander and Turunen in 1955. The presence of a dense collateral network of veins surrounding such a displaced spleen in a war casualty led them to this field of research. Clinical reports concerning this method, which deserves further trial, are encouraging and greater effort should be made to promote the use of this procedure prior to the inevitable splenectomy. OMENTOCAVOPEXY AND THORACIC SUBCUTANEOUS ILEOPEXY. Berman and associates have recently advocated and performed omentocavopexy with some success although follow-up periods are still too short to be significant. More recently Bader and associates have described a method, which is employed as yet only in the laboratory, of establishing
SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
1427
a portosystemic shunting system by thoracic subcutaneous ileopexy. This procedure is intriguing and one which I intend to investigate with the hope of applyi~g it clinically. Other indirect shunts, including poudrage, have been attempted, and undoubtedly still other methods will be devised. All procedures will require prolonged and cautious review. Unless the actual volume of shunted blood represents a major portion of the portal circulation, it is not likely that indirect shunts will produce long-term relief. OBLITERATION OF VARICES
By Injection Many attempts have been made to obliterate varices in the esophagogastric region. The direct injection of sclerosing substances through an esophagoscope has been advocated. The nature of this procedure is such that there is little chance that it will ever become a commonly used method in the pediatric age group.
By Suture Transthoracic ligation and oversewing of the varicosities have been somewhat successful. Almost every author has had some notable results with this procedure. In my experience it has been almost a complete failure, and whatever control of hemorrhage it provided has, in my opinion, been supplanted by intelligent use of the Sengstaken-Blakemore tube. While use of this tube is distasteful to me and its prolonged use in small children is both difficult and hazardous, it has served a purpose in controlling hemorrhage and has allowed opportunity to gain time and to regroup not only the demoralized resources of the patient but also those of the hospital team. Gentle traction on the inflated lower balloon has usually proved adequate to control bleeding, and it is my impression that the young patient tends to be more comfortable when the upper balloon is not inflated. ELIMINATION OF VARICES
Esopbagogastrectomy In the event that no direct shunt can be established and no satisfactory indirect shunt can be produced, elimination of the superficial varices around the esophagogastric junction seems to be the last resort. Excision of this critical portion, with reanastomosis of the esophagus to the stomach and usually with creation of a hiatal hernia, has had its advocates. In an attempt to avoid hiatal hernia I have tried to eliminate the varices by transecting the stomach just below the esophagogastric junction and, after excising a
1428
HUGH
B.
LYNN
narrow segment of stomach, by reanastomosing the gastric segments. One patient bled severely from his varices while still convalescing from this procedure and none was relieved for a prolonged period.
Colon Interposition While esophagogastrectomy with interposition of the colon seems unphysiologic and cumbersome, it seems to be the most satisfactory method available at the time of this report. It certainly permits wide excision of the critical region and anastomosis without tension, herniation, or other adverse situations; it has been employed effectively both as a means of gaining time and growth and as a last resort. The exposure of the esophageal mucosa to gastric juices is reduced or eliminated by this method. To date, results at this clinic have been most encouraging and my own total experience has been most gratifying, considering the condition of the patients presenting for such therapy. Many surgeons have used the jejunum for interposition substitution. However, the sensitivity of the jejunal mucosa to gastric juices and the cumbersome sacculated nature of the segment necessary because of the blood supply have made it less desirable. My experience has been limited to those situations in which colon was not available, or to conditions other than portal hypertension in which the greater curvature could not be used to form an esophageal tube.
RESULTS IN MAYO CLINIC SERIES
From 1950 through 1963, 40 children have undergone surgical treatment for bleeding esophageal varices at the Mayo Clinic. A total of 72 surgical procedures have been performed on these patients. Of the 72 Table 1. Surgical Procedures Prior to Registration at Mayo Clinic PROCEDURE
NUMBER OF OPERATIONS
Splenectomy....................... . Extrahepatic ...................... Intrahepatic ..................... , Splenorreal shunt .................. . Extrahepatic ...................... Intrahepatic ...................... Portacaval shunt ................... . Ligation of varices (extrahepatic) .................... . Exploration-no shunt (extrahepatic) .................. . TOTAL . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
9 7 2 5 4 1 1
2 1
18
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SURGICAL TREATMENT OF ESOPHAGEAL VARICES IN CHILDREN
procedures, 18 were performed prior to patients' registration at this clinic (Table 1), 49 were performed at this clinic, and 5 were performed subsequent to patients leaving this clinic (Table 2). The list of surgical procedures performed on these 40 patients is impressive (Table 3). Of the 40 patients, 31 had extrahepatic obstruction and nine intrahepatic obstruction. There were 22 boys and nine girls with extrahepatic blockage and two boys and seven girls with intrahepatic blocks. Follow-up reports indicate that two of the 40 patients have not undergone splenectomy. One of the nine patients with intrahepatic obstruction had a portacaval shunt as an initial procedure and no subsequent operation has been
Table 2. Surgical Procedures Since Mayo Clinic Treatment EXTRAHEPATIC
NUMBER OF OPERATIONS
Esophagogastrectomy. . . . . . . . . . . .. 1 Ligation of varices ................ 1 Shunt (portamesenteric) ........... 1.. TOTAL . . . . . . . . . . . . • . . • • . . . • •
NUMBER OF OPERATIONS
INTRAHEPATIC
Interposition (colon). . . . . . . . . .. 1* Ligation of varices ............. It
3t
2
* Had splenorenal shunt at Mayo Clinic.
t Had portacaval shunt at Mayo Clinic. t Each had splenectomy at Mayo Clinic.
Table 3.
Operations Performed at the Mayo Clinic on 40 Pediatric Patients With Diagnosis of Portal Hypertension (1950-1963) TYPE OF PROCEDURE
NUMBER OF PATIENTS
Splenectomy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 24 Splenorenal shunt ..................................... , 14 Ligation of varices ..................................... 14 Portacaval shunt ...................................... , 7 Interposition (colon, 5; jejunum, 2). . . . . . . . . . . . . . . . . . . . . . . 7 Partial esophagogastrectomy With esophagogastrostomy. . . . . . . . . . . . . . . . . . . . . . . . . . .. 1 With esophagojejunostomy (end-to-end) and jejunojejunostomy (end-to-side). . . . . . . . . . . . . . . . . . . . . . . . . .. 1 With esophagogastrostomy and partial gastrectomy with Schoemaker type Billroth I gastroduodenostomy. . . 1 With esophagojejunostomy (end-to-side) Roux Y........... 1 Left nephrectomy, left renal artery obstruction after splenorenal shunt......... . . . . . . . . . . . . . . . . . . . . . . . . . . .. 1 Negative exploration for possible portacaval shunt. ... .... .. 1 TOTAL . • • • • • • . . . . . . . . . . . . . . . . • • . . . . . . . . . . . . . • • . . . • 72
HUGH
B.
LYNN
performed. One of the 31 patients having an extrahepatic obstruction had transthoraeic ligation of varices as the primary operation and had had no further procedures when last contacted.
SUMMARY
The surgical treatment of esophageal varices in children is a discouraging problem. In light of present knowledge, the longer such treatment can be delayed the better the chance of a functioning shunt. While a portacaval shunt is preferable and technically feasible in small children, this procedure cannot be carried out in most children with portal hypertension since it is more commonly due to extrahepatic blockage. A splenorenal shunt is desirable but not usually successful in children less than seven or eight years of age; in the event of failure, a mesenteric caval shunt is often satisfactory. Any temporizing procedure which can prevent a recurrence of the hemorrhage and still preserve the splenic vein for future use seems justifiable in small children.
REFERENCES 1. Bader, Karl, Jr., Roseman, D. L., Economou, B. G. and Beattie, E. J., Jr.: Portalsystemic venous shunting by thoracic subcutaneous ileopexy. Read at the meeting of the Central Surgical Association, Rochester, Minnesota, February 27 to 29, 1!)64. 2. Berman, E. J., Waite, Paul, Gerig, E. L. and Bakemier, R. E.: Omentocavopexy: Further analysis. Arch. Burg. 86: 1008-1013 (June) 1963. 3. Boles, E. T., Jr. and Clatworthy, H. W., Jr.: Diseases of the spleen and portal circulation. In Benson, C. D.: Pediatric Surgery, Chicago, Year Book Medical Publishers, Inc., 1962, Vol. 1, pp. 639-653. 4. Boley, B. J., Arlen, Myron, and Mogilner, L. J.: Congenital hepatic fibrosis causing portal hypertension in children. Burgery 54: 356-360 (Aug.) 1963. 5. Conn, H. 0., Mitchell, J. R. and Brodoff, M. G.: A comparison of the radiologic and esophagoscopic diagnosis of esophageal varices. New England J. Med. 265: 160-164 (July 27) 1961. 6. Cooley, D. A.: Discussion. Ann. Burg. 157: 880--881 (June) 1963. 7. Eiseman, B., Lindeman, G. M. and Clark, G. M.: Clinical evaluation of the ammonium citrate tolerance test for determining the patency of a portacaval shunt. J. Lab. & Clin. Med. 48: 579-588 (Oct.) 1956. 8. Everson, T. C. and Cole, W. H.: Ligation of the splenic artery in patients with portal hypertension. Arch. Burg. 56: 153-160 (Feb.) 1948. 9. Martin, L. W. and B'ryant, L. R.: Use of fructose to detc'rmine the patency of portalsystemic shunts. Arch. Burg. 85: 783-790 (Nov.) 1962. 10. Nylander, P. E. A. and Turunen, Martti: Transposition of the spleen into the thoracic cavity in cases of portal hypertension. Ann. Surg. 142: 954-956 (Dec.) 1955. 11. Orloff, M. J. and Thomas, H. B.: Pathogenesis of esophageal varix rupture: A study based on groBS and microscopic examination of the esophagus at thetime of bleeding. Arch. Burg. 87: 301-307 (Aug.) 1963. 12. Schwartz, B.!.:: Isotopic evaluation of portal circulation: Diagnosis of esophagogastric varices. Bull. Soc. Internat. Chir. 21: 290--303, 1962.
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13. Sengstaken, R. W. and Blakemore, A. H.: Balloon tamponage for the control of hemorrhage from esophageal varices. Ann. Surg. 131: 781-789 (May) 1950. Mayo Clinic Rochester, Minnesota