Portal hypertension: Surgical management in infants and children

Portal Hypertension : Surgical Management in Infants and Children By F. Ehrlich, S. Pipatanagul, W. K. Sieber, and W. B. Kiesewetter

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t O R T A L H Y P E R T E N S I O N with secondary esophageal varices persists a s a major cause for upper gastrointestinal hemorrhage in infants and children. The management of this problem continues to concern surgeons responsible for the care of these patients. F r o m 1949 through July, 1972, 59 cases of portal hypertension with esophageal varices were seen at the Children's Hospital of Pittsburgh. This report will present a discussion of the etiological, clinical, and therapeutic parameters of the problem in this group. ETIOLOGY

Utilizing the division of patients suggested by Whipple, l there were 47 cases of extrahepatic portal hypertension and 12 cases of intrahepatic portal hypertension in our series; this is an exaggerated incidence of the extrahepatic type which usually comprises 50%-70% of cases as reported by others, z-4 Thirteen patients (28%) out of the extrahepatic group had a history consistent with omphalitis in the newborn period; three additional patients (6~o) gave a neonatal history of diarrhea and fever which could have led to portal thrombosis. The remaining two-thirds of the patients had no apparent etiology for their extrahepatic portal hypertension. It should be noted that none of the extrahepatic cases had umbilical vein catheterization. However, since the increased usage of umbilical vein catheters has occurred only in the past few years, one would not expect to see many cases yet in which this factor might be a possible etiology. O f the 12 patients who had intrahepatic portal hypertension, ten cases were secondary to cirrhosis and two to congenital hepatic fibrosis. Of the ten patients with cirrhosis, seven cases were of the postnecrotic variety and three were secondary to biliary atresia. CLINICAL PICTURE

The male to female ratio was 34 to 25, which is similar to other series. 2-4 O f interest is the fact that only two cases occurred in blacks; Santulli z also reported a similar low incidence of this problem in nonwhites. From the Surgical Clinic of the Children's Hospital of Pittsburgh and the Department of Surgery, University of Pittsburgh School of Medicine. Pittsburgh, Pa. Presented at the Annual Meeting of the American Academy of Pediatrics, Surgical Section, Chicago, HI., October 20-24, 1973. F. Ehrlich, M.D.: Former Teaching Fellow in Pediatric Surgery, University of Pittsburgh School of Medicine. S. Pipatanagu|, M.D.: Former Teaching Fellow in Pediatric Surger.,, University of Pittsburgh School of Medicine. V~. K. Sieber, M.D.: Clinical Associate Professor of Surgery, University of Pittsburgh School of Medicine. W . B. Kiesewetter, M.D.: Professor of Pediatric Surgery, University of Pittsburgh School of Medicine. Address for reprint requests." IV. B. Kiesewetter, M.D., Children's Hospital, 125 DeSoto Street, Pittsburgh, Pa. 15213. 9 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 9, No. 3 (June), 1974

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EHRLICH ET AL.

The onset of symptoms was highest in the first year of life. Those symptoms most commonly seen at this time were ascites and hepatosplenomegaly. Ascites was the initial symptom in 11 of our 59 cases (19~o). The role of ascites in the pathophysiology of extrahepatic portal hypertension has been alluded to by others, 5,6 and we concur that it is probably present more often than noted in this series. Bleeding from the gastrointestinal tract did not become a prominent sign until between ages 2 and 6. The patients' age at the time of diagnosis closely correlated with the peak onset of symptoms and the first bleeding episode. Varices were present in all of our patients and were confirmed by either esophagoscopy, esopbagram, splenoportogram, selective angiography, or some combination thereof. SURGICAL MANAGEMENT

A variety of operations were performed on the 38 patients who underwent 60 procedures for their portal hypertension (Table 1). Thirty-six might be classified as temporizing surgery and included splenectomy, ligation of the varices, and division of the stomach (Tanner operation). The remaining 24 operations were what might be called definitive surgery, i.e., removal of the bleeding area or creation of a decompressive portosystemic shunt.

Temporizing Splenectomy alone was used in the early 1950's. Since then, it has only been used in conjunction with an unsuccessful attempt to create a splenorenal shunt due to small vessels. There were 19 such splenectomies performed. All of the patients had extrahepatic portal hypertension. Eight rebled after splenectomy. This is a high rate (42~o) of rebleeding per se but low when one considers that no therapy was actually applied to the hemodynamic situation. Three patients rebled within the first postoperative year, one in the second year, and the other four cases before the fifth year after surgery. This suggests that if there is no Table 1. Surgical Therapy Number of Temporizing Splenectomy Ligation of varices Transthoracic 3 Transgastric 12

Transection of stomach (Tanner procedure) Definitive Subtotal gastrectomy and pyloroplasty F:sophagogastrectomy and interposition Colon 3 Jejunum 2 Portosystemic shunt Splenorenal 11 Portocaval 4 Mesocaval 3 *Death from cause unrelated to portal hypertension.

Cases

Rebled

No Bleeding

19 15

8

11

0

2 9 0

1 3 2

0 1 0

1

0

0

0 0

3 2

1 0

4 1 1

7 3 2

1 0 1*

2

1 5

Deaths

18

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rebleeding in the first year postsplenectomy, a long remission may result. It should be noted that in the 11 patients who did not rebleed, two had not bled preoperatively. Eight successful secondary operations were carried out upon the patients who rebled. F o u r had transgastric varix ligations, two had colon interpositions, one had a stomach division and anastomosis, and one had a mesocaval shunt. A final indication for splenectomy in a child with portal hypertension is the presence of severe hypersplenism. This is not a frequent finding but may have to be considered; it should be combined with a shunting procedure if at all possible. There were no deaths among the patients undergoing splenectomy. Fifteen patients underwent ligation of varices. Three of four of these rebled. There was one death among these patients, a child with extrahepatic portal hypertension, who previously had a transgastric ligation. Of the 15 patients, three had a transthoracic ligation with two instances of rebleeding and 12 patients had a transgastric ligation with nine who rebled. It would appear from these figures that ligation of varices is of use only in the emergency situation to " b u y time" because the child is too young a n d / o r the vessels too small for a shunt; long-term results for controlling bleeding are unsatisfactory by this method. It is of interest that one patient who underwent a transthoracic ligation had a previous mesocaval shunt. The patient was readmitted 5 days after discharge from mesocaval shunting with another massive gastrointestinal hemorrhage. It was felt that his shunt was clotted, and a retrograde X-ray and pressure study was done of his vena cava and shunt. The latter proved to be patient with acceptable pressures on the mesenteric side. His varices and coronary vein were ligated and he has done well since. Warren 7 has advocated such ligation of the coronary vein as part of the definitive procedure. This case would seem to lend credence to that idea, since p r o o f has not been offered that a patent, functioning shunt will promptly lower the flow in varices. There were two cases in which the stomach was divided and then sutured back together. Neither patient rebled and there was no mortality. Since the varices usually recur, this is not a definitive procedure, but it may be of value in gaining time and allowing the child to develop vessels large enough to shunt.

Definitive Subtotal gastrectomy and pyloroplasty was carried out in one patient. The child rebled 1 yr later and had a splenorenal shunt. While it would seem that decreased gastric acid output might help in the problem of bleeding varices, it does not provide an answer to the basic hemodynamic problem. There were five children who underwent esophagogastrectomy and intestinal interposition. In three patients, colon was used, and in two jejunum. One patient with extrahepatic disease died in the postoperative period of sepsis secondary to disruption of the cologastrostomy. N o n e of the surviving patients rebled. The reason for choosing this procedure was failure of previous surgery for gastrointestinal bleeding in each case. However, K o o p 8 has advocated its use as a primary procedure when a shunt is not feasible. In reviewing numerous other series of patients treated with esophagogastrectomy and interposition, Bernstein 9 found a rebleeding incidence of 25~o-30% when they were averaged together.

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In 18 patients, some form of portosystemic shunt was created. It is our feeling, along with other authors, that the definitive treatment for gastrointestinal bleeding from esophageal varices is such a shunt. Other procedures temporize while varices re-form and rebleed. The problem with creating a successful shunt in children is having large enough vessels to maintain the shunt patency. O f the 18 shunts, six of the patients rebled (33~o). There were two deaths, one in a patient with a splenorenal shunt and one in a patient with a mesocaval shunt. However, the latter death was due to causes unrelated to the portal hypertension. Eleven patients had a splenorenal shunt. These were all of the distal variety as opposed to the central type advocated by Clatworthy. ~~ Eight of these patients had extrahepatic disease and three had intrahepatic block. F o u r patients rebled (36~), three in the extrahepatic group and one in the intrahepatic group. Two of the three patients with extrahepatic portal hypertension who rebled had selective angiography and the shunt did not visualize, suggesting that it had thrombosed. The one death was in the patient with intrahepatic disease; death occurred from heaptic failure 289 yr after the shunt, without any rebleeding. It would appear that this is a good operation if the shunt remains patent. However, the small size of the vessels involved in the shunt often makes this difficult to achieve. Some authors 4,~ believed that there is little hope for a successful splenorenal shunt in patients under the age of 10. Our data are not large enough to draw any conclusion about this. There were four portacaval shunts done. One patient rebled and there were no deaths. This procedure, when done in a side-to-side manner, remains an excellent choice for those patients with intrahepatic disease. It is often a difficult procedure in those patients with an extrahepatic block, although Martin's t2 recent report offers encouragement for this group. There were three patients who had mesocaval shunts. One patient died 189 yr postshunt of unrelated causes without having rebled. One patient did rebleed and was discussed above; his shunt was open and functioning. Since the initial description of this type of shunt, much controversy regarding its necessity a n d / o r efficacy has appeared in the literature. However, a recent report 13 concerning the use of a synthetic graft between the vena cava and the superior mesenteric vein has added new hope for such a shunt with a low rate of rebleeding. Furthermore, the incidence of postshunt morbidity is reported to be very low. MORTALITY

The overall mortality rate in this series was 12~o (7/59); six of the deaths were related to portal hypertension and one occurred from a totally unrelated cause. The mortality from portal hypertension alone was, therefore, 10~o (6/59). Of the six deaths, four patients had intrahepatic disease; two had extrahepatic disease. Three of the six deaths directly related to portal hypertension occurred in the 21 patients who did not undergo surgery. The remaining three deaths were in 38 patients undergoing 60 operative procedures for a mortality of 8~o.

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SUMMARY

A series of 59 cases of portal hypertension with esophageal varices has been reviewed. Thirty-eight patients underwent 60 procedures, with three deaths; among the 21 patients treated expectantly there were three deaths. Splenectomy, division of the stomach, and ligation of varices are temporizing procedures only; rebleeding is c o m m o n and further surgery frequently necessary. Esophagogastrectomy with interposition is acceptable when other more definitive procedures have failed or cannot be done. Portalsystemic shunts offer the best chance for a cure; their success or failure is related to the patency of the shunt, not the occurrence of new varices. REFERENCES

1. Whipple AW: Problem of portal hypertension in relation to hepatosplenopathies Ann Surg 122:449, 1945 2. Voorhees AB Jr, Harris RC, Britton RC, Price JB, Santulli TV: Portal hypertension in children: 98 cases. Surgery 58:540, 1965 3. Foster JH, Holcomb GW, Kirtley JA: l~esuits of surgical treatment of portal hypertension in children. Ann Surg 157:868, 1963 4. Trusler GA, Morris FR, Mustard WT: Portal hypertension in childhood. Surgery 52:664, 1962 5. Mikkelsen WP: Extrahepatic portal hypertension in children. Am J Surg 111:333, 1966 6. Clatworthy HW Jr, Boles ET, Jr: Extrahepatic portal bed block in children: Pathogenesis and treatment. Ann Surg 150:371, 1959 7. Warren WD, Zeppa R, Fomon J J: Selective trans-splenic decompression of gastroesophageal varices by distal splenorenal shunt. Ann Surg 166"437, 1967

8. Koop CE, Kavianian A: Reappraisal of colonic replacement of distal esophagus and proximal stomach in the management of bleeding varices in children. Surgery 57:454, 1965 9. Bernstein EF, Varco RL, Wangensteen OH: Treatment of bleeding esophageal varices in portal-systemic shunt failures. Arch Surg 99:171, 1969 10. Clatworthy HW Jr, Wall T, Watman RN: A new type of portal to systemic venous shunt for portal hypertension. Arch Surg 71:588, 1955 11. Arcari FA, Lynn HB: Bleeding esophageal varices in children. Surg Gynecol Obstet 112:101, 1961 12. Martin LW: Changing concepts of management of portal hypertension in children; J Ped Pediatr Surg 7:559, 1972 13. Drapanas T: Interposition mesocaval shunt for treatment of portal hypertension; Ann Surg 176:435, 1972