Successful
Splenorenal Shunt for Emergent Control of Variceal Bleeding in a Small Child
By Louis M. Marmon,
Stephen P. Dunn, Charles D. Vinocur, Deborah Billmire, and William H. Weintraub Washington, DC and Philadelphia, Pennsylvania
l A 8.8-kg, lo-month-old boy with idiopathic cavernous transformation of the portal vein developed variceal hemorrhage refractory to nonoperative management. A distal splenorenal shunt was performed while the bleeding was controlled with balloon tamponade. Hemorrhage was successfully controlled with no recurrent bleeding and the shunt remains patent at 1 year of follow-up. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Splenorenal shunt, infant; cavernous transformation of portal vein; esophageal varices.
T
HE OCCASIONAL pediatric patient with portal hypertension will develop gastrointestinal hemorrhage that is refractory to nonoperative management. Portosystemic shunts have been used successfully to manage refractory hemorrhage in many patients and a number of efficacious procedures have been described. The distal splenorenal shunt is an effective method of controlling hemorrhage that provides the additional benefit of preserving the spleen and is associated with long-term patency and a low complication rate. The successful use of a distal splenorenal shunt to control actively bleeding varices in a child weighing less than 9 kg has not been previously reported. This case demonstrates that the distal splenorenal shunt can be used successfully to control actively bleeding varices in a small child. CASE REPORT A lo-month-old boy weighing 8.6 kg with idiopathic cavernous transformation of the portal vein developed life-threateningvariceal hemorrhage refractory to two attempts at endosclerosis, maximal intravenous pitressen (0.4 Uiminil.73 m*), Hz blockers (3.5 mgikgid of ranitidine). and blood component therapy to correct his coagulation profile to normal laboratory values. Hemorrhage was controlled with the placement of a Sengstaken-Blakemore (SB) tube and inflation of the gastric balloon. After 24 hours the balloon was deflated but attempts to wean the pitressin resulted in recurrent bleeding. The gastric balloon was reinflated and the patient underwent mesenteric digital subtraction angiography to delineate portal venous anatomy. With the gastric tube inflated and while receiving maximal pharmacological therapy, the patient underwent an emergent distal end-to-side splenorenal shunt. The splenic vein measured 5 mm in diameter. The preshunt portal pressure measured via an omental vein was 41 mm Hg and decreased to I1 mm Hg after completion of the shunt. The variceal bleeding was controlled immediately. The SB tube was deflated in the operating room and was removed at 24 hours after surgery. The intravenous pitressin was tapered over the next 48 hours and then was discontinued. The patient did not require any additional blood transfusions and his postoperative course was remarkable only for the development of a distal esophageal stricture requiring dilatation. At 1 year of follow-up he has had no further bleeding. His Journalof Pediatric Surgery, Vol 27, No 8 (August), 1992: pp 1049-1050
splenomegaly has resolved and his liver function studies remain normal. He does not require any medications and his shunt remains patent as demonstrated by Doppler ultrasound.
DISCUSSION Extrahepatic portal hypertension in a child can be complicated by the development of esophageal variceal bleeding that is frequently self-limited or responds to endoscopic sclerotherapy, vasoconstrictor infusions, and occasionally balloon compression.1-3 On the other hand, patients with refractory serious liver disease and symptomatic portal hypertension should undergo orthotopic liver transplantation. However, refractory gastrointestinal bleeding due to portal hypertension in the child with normal liver function remains a clinically challenging event requiring surgical intervention. A number of different surgical procedures have been used to decrease the portal pressure in these children with generally good results reported. These procedures include the central splenorenal shunt,4 the mesocaval shunt,” and the distal splenorenal (Warren) shunt (DSRS). The DSRS has the theoretical advantage of selectively shunting the blood from the stomach and distal esophagus via the splenic vein while preserving hepatic blood flow. In adults, regardless of the patient’s preoperative Child’s classification, the use of the DSRS in both emergency and elective situations is associated with excellent control of hemorrhage and a low incidence of complications.6s7 Survival in these adult patients is dependent on the progression of the underlying liver disease. The successful use of the DSRS for the treatment of symptomatic portal hypertension in children has been reported by a number of different institutions.8-10These children ranged in age from 21/2years
From the Division of Pediatric Sutgety, Department of Surgery, Georgetown University School of Medicine, Washington, DC; and the Division of Pediatric Surgery, St Christopher’s Hospital ,for Children, Department of Sutgety, Temple Universitv School of Medicine, PhiladeLphia, PA. Presented at the 43rd Annual Meeting of the Surgical Section of the American Academy of Pediatn’cs. New Orleans. Louisiana, October 26-27, 1991. Address reprint requests to Stephen P. Dunn, MD, Division of Pediatric Surgey, St Christopher’s Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134-1095. Copyright Q 1992 by W B. Saunders Company 0022-3468192/2708-0022$03.0010 1049
1050
MARMON ET AL
to 14 years and the majority had intrinsic liver disease as the etiology of their portal hypertension. There were no operative mortalities and the shunts remained patent in 23 of the 27 children (85%). In addition, the DSRS maintains splenic function and is associated with a low incidence of postshunt encephalopathy, qualities that are of particular importance in the pediatric patient. In a long-term study of psychometric parameters in children who underwent portosystemic shunts for portal vein obstruction without intrinsic liver disease, Alagille et al documented that there was no compromise of intellectual capacity.l’ The performance of a successful DSRS is dependent on the patency of the splenic vein. Rodgers and Talbert9 have noted the favorable anatomy of the splenic vein in the young patient; the vein appears to be more caudal in the child as compared with the adult, thereby minimizing the amount of dissection that is required for mobilization of the vein. In most cases of intrinsic liver disease or portal vein thrombosis the splenic vein is spared but this anatomy must be confirmed preoperatively. The venous phase of a mesenteric arteriogram can define the portal anatomy. However, we prefer a digital subtraction mesenteric angiogram which provides clear and detailed
anatomic information regarding portal venous anatomy. Postoperatively the patency of the shunt can be determined safely and accurately by duplex Doppler ultrasound.12J3 Experience with portal vein reconstruction has been extensive in the pediatric liver transplant population at St Christopher’s Hospital for Children. This has led us to feel at ease with the vessel size involved in this case. Suture techniques for portal vein reconstruction, including the “growth factor” technique reported by Starzl et al,r5 are easily adapted to the splenic vein-to-renal vein anastomosis. Emergency operations to treat variceal bleeding in the child with portal vein obstruction are uncommon and have rarely been reported in infants.14 This case illustrates that the DSRS can be used to successfully treat refractory esophageal hemorrhage in a small infant with a splenic vein as small as 5 mm. The DSRS maintains hepatofugal blood flow, allows preservation of the spleen, provides excellent control of bleeding, and can be performed safely with minimal long-term morbidity. These characteristics make the DSRS the preferred operative procedure for portal decompression in children when the splenic vein is patent.
REFERENCES 1. Alvarez F, Bernard 0, Brunelle F, et al: Portal obstruction in children. I. Clinical investigation and hemorrhage risk. J Pediatr 103696702,1983 2. Fonkalsrud EW: Treatment of variceal hemorrhage in children. Surg Clin North Am 70:475-487,199O 3. Hassall E, Berquist WE, Ament ME, et al: Sclerotherapy for extrahepatic portal hypertension in childhood. J Pediatr 11569-74, 1989 4. Coran AG, Wesley JR, Weintraub WH: The central splenorenal shunt for portal hypertension in children: Experience with eight consecutive patent anastomoses. .I Pediatr Surg 15:827-834,198O 5. Gauthier F, De Dreuzy 0, Valayer J, et al: H-type shunt with an autologous venous graft for treatment of portal hypertension in children. J Pediatr Surg 24:1041-1043, 1989 6. Mitchell RL, Ignatius JA: Distal splenorenal shunt: Standard procedure for elective and emergency treatment of bleeding esophageal varices. Am J Surg 156:169-172,1988 7. Millikan WJ Jr, Warren WD, Henderson JM, et al: The Emory prospective randomized trial: Selective versus nonselective shunt to control variceal bleeding-ten year follow-up. Ann Surg 201:712-722,1985
8. Maksoud JG, Miles S, Pinto VC: Distal splenorenal shunt in children. J Pediatr Surg 13:335-340, 1978 9. Rodgers BM, Talbert JL: Distal spleno-renal shunt for portal decompression in childhood. J Pediatr Surg 14:33-37, 1979 10. Van Vroonhoven TJ, Molenaar JC: Distal splenorenal shunt for decompression of portal hypertension in children with cystic fibrosis. Surg Gynecol Obstet 149:559-560,1979 11. Alagille D, Carlier JC, Chiva M, et al: Long-term neuropsychological outcome in children undergoing portal-systemic shunts for portal vein obstruction without liver disease. J Pediatr Gastroenter01 Nutr 5:861-866,1986 12. Patriquin H, Lafortune M, Weber A, et al: Surgical portosystemic shunts in children: Assessment with duplex Doppler US. Radiology 165:25-28,1987 13. Rodgers BM, Kaude JV: Real-time ultrasound in determination of portasystemic shunt patency in children. J Pediatr Surg 16:968-971,198l 14. Alvarez F, Bernard 0, Brunelle F? et al: Portal obstruction in children. II. Results of surgical portosystemic shunts. J Pediatr 103:703-707.1983 15. Starzl TE, Iwatsuki S, Shaw BW Jr: A “growth factor” in fine vascular anastomoses. Surg Gynecol Obstet 159:164-165, 1984
Splenorenal Shunt for Emergent Control of Variceal Bleeding in a Small Child
By Louis M. Marmon,
Stephen P. Dunn, Charles D. Vinocur, Deborah Billmire, and William H. Weintraub Washington, DC and Philadelphia, Pennsylvania
l A 8.8-kg, lo-month-old boy with idiopathic cavernous transformation of the portal vein developed variceal hemorrhage refractory to nonoperative management. A distal splenorenal shunt was performed while the bleeding was controlled with balloon tamponade. Hemorrhage was successfully controlled with no recurrent bleeding and the shunt remains patent at 1 year of follow-up. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Splenorenal shunt, infant; cavernous transformation of portal vein; esophageal varices.
T
HE OCCASIONAL pediatric patient with portal hypertension will develop gastrointestinal hemorrhage that is refractory to nonoperative management. Portosystemic shunts have been used successfully to manage refractory hemorrhage in many patients and a number of efficacious procedures have been described. The distal splenorenal shunt is an effective method of controlling hemorrhage that provides the additional benefit of preserving the spleen and is associated with long-term patency and a low complication rate. The successful use of a distal splenorenal shunt to control actively bleeding varices in a child weighing less than 9 kg has not been previously reported. This case demonstrates that the distal splenorenal shunt can be used successfully to control actively bleeding varices in a small child. CASE REPORT A lo-month-old boy weighing 8.6 kg with idiopathic cavernous transformation of the portal vein developed life-threateningvariceal hemorrhage refractory to two attempts at endosclerosis, maximal intravenous pitressen (0.4 Uiminil.73 m*), Hz blockers (3.5 mgikgid of ranitidine). and blood component therapy to correct his coagulation profile to normal laboratory values. Hemorrhage was controlled with the placement of a Sengstaken-Blakemore (SB) tube and inflation of the gastric balloon. After 24 hours the balloon was deflated but attempts to wean the pitressin resulted in recurrent bleeding. The gastric balloon was reinflated and the patient underwent mesenteric digital subtraction angiography to delineate portal venous anatomy. With the gastric tube inflated and while receiving maximal pharmacological therapy, the patient underwent an emergent distal end-to-side splenorenal shunt. The splenic vein measured 5 mm in diameter. The preshunt portal pressure measured via an omental vein was 41 mm Hg and decreased to I1 mm Hg after completion of the shunt. The variceal bleeding was controlled immediately. The SB tube was deflated in the operating room and was removed at 24 hours after surgery. The intravenous pitressin was tapered over the next 48 hours and then was discontinued. The patient did not require any additional blood transfusions and his postoperative course was remarkable only for the development of a distal esophageal stricture requiring dilatation. At 1 year of follow-up he has had no further bleeding. His Journalof Pediatric Surgery, Vol 27, No 8 (August), 1992: pp 1049-1050
splenomegaly has resolved and his liver function studies remain normal. He does not require any medications and his shunt remains patent as demonstrated by Doppler ultrasound.
DISCUSSION Extrahepatic portal hypertension in a child can be complicated by the development of esophageal variceal bleeding that is frequently self-limited or responds to endoscopic sclerotherapy, vasoconstrictor infusions, and occasionally balloon compression.1-3 On the other hand, patients with refractory serious liver disease and symptomatic portal hypertension should undergo orthotopic liver transplantation. However, refractory gastrointestinal bleeding due to portal hypertension in the child with normal liver function remains a clinically challenging event requiring surgical intervention. A number of different surgical procedures have been used to decrease the portal pressure in these children with generally good results reported. These procedures include the central splenorenal shunt,4 the mesocaval shunt,” and the distal splenorenal (Warren) shunt (DSRS). The DSRS has the theoretical advantage of selectively shunting the blood from the stomach and distal esophagus via the splenic vein while preserving hepatic blood flow. In adults, regardless of the patient’s preoperative Child’s classification, the use of the DSRS in both emergency and elective situations is associated with excellent control of hemorrhage and a low incidence of complications.6s7 Survival in these adult patients is dependent on the progression of the underlying liver disease. The successful use of the DSRS for the treatment of symptomatic portal hypertension in children has been reported by a number of different institutions.8-10These children ranged in age from 21/2years
From the Division of Pediatric Sutgety, Department of Surgery, Georgetown University School of Medicine, Washington, DC; and the Division of Pediatric Surgery, St Christopher’s Hospital ,for Children, Department of Sutgety, Temple Universitv School of Medicine, PhiladeLphia, PA. Presented at the 43rd Annual Meeting of the Surgical Section of the American Academy of Pediatn’cs. New Orleans. Louisiana, October 26-27, 1991. Address reprint requests to Stephen P. Dunn, MD, Division of Pediatric Surgey, St Christopher’s Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134-1095. Copyright Q 1992 by W B. Saunders Company 0022-3468192/2708-0022$03.0010 1049
1050
MARMON ET AL
to 14 years and the majority had intrinsic liver disease as the etiology of their portal hypertension. There were no operative mortalities and the shunts remained patent in 23 of the 27 children (85%). In addition, the DSRS maintains splenic function and is associated with a low incidence of postshunt encephalopathy, qualities that are of particular importance in the pediatric patient. In a long-term study of psychometric parameters in children who underwent portosystemic shunts for portal vein obstruction without intrinsic liver disease, Alagille et al documented that there was no compromise of intellectual capacity.l’ The performance of a successful DSRS is dependent on the patency of the splenic vein. Rodgers and Talbert9 have noted the favorable anatomy of the splenic vein in the young patient; the vein appears to be more caudal in the child as compared with the adult, thereby minimizing the amount of dissection that is required for mobilization of the vein. In most cases of intrinsic liver disease or portal vein thrombosis the splenic vein is spared but this anatomy must be confirmed preoperatively. The venous phase of a mesenteric arteriogram can define the portal anatomy. However, we prefer a digital subtraction mesenteric angiogram which provides clear and detailed
anatomic information regarding portal venous anatomy. Postoperatively the patency of the shunt can be determined safely and accurately by duplex Doppler ultrasound.12J3 Experience with portal vein reconstruction has been extensive in the pediatric liver transplant population at St Christopher’s Hospital for Children. This has led us to feel at ease with the vessel size involved in this case. Suture techniques for portal vein reconstruction, including the “growth factor” technique reported by Starzl et al,r5 are easily adapted to the splenic vein-to-renal vein anastomosis. Emergency operations to treat variceal bleeding in the child with portal vein obstruction are uncommon and have rarely been reported in infants.14 This case illustrates that the DSRS can be used to successfully treat refractory esophageal hemorrhage in a small infant with a splenic vein as small as 5 mm. The DSRS maintains hepatofugal blood flow, allows preservation of the spleen, provides excellent control of bleeding, and can be performed safely with minimal long-term morbidity. These characteristics make the DSRS the preferred operative procedure for portal decompression in children when the splenic vein is patent.
REFERENCES 1. Alvarez F, Bernard 0, Brunelle F, et al: Portal obstruction in children. I. Clinical investigation and hemorrhage risk. J Pediatr 103696702,1983 2. Fonkalsrud EW: Treatment of variceal hemorrhage in children. Surg Clin North Am 70:475-487,199O 3. Hassall E, Berquist WE, Ament ME, et al: Sclerotherapy for extrahepatic portal hypertension in childhood. J Pediatr 11569-74, 1989 4. Coran AG, Wesley JR, Weintraub WH: The central splenorenal shunt for portal hypertension in children: Experience with eight consecutive patent anastomoses. .I Pediatr Surg 15:827-834,198O 5. Gauthier F, De Dreuzy 0, Valayer J, et al: H-type shunt with an autologous venous graft for treatment of portal hypertension in children. J Pediatr Surg 24:1041-1043, 1989 6. Mitchell RL, Ignatius JA: Distal splenorenal shunt: Standard procedure for elective and emergency treatment of bleeding esophageal varices. Am J Surg 156:169-172,1988 7. Millikan WJ Jr, Warren WD, Henderson JM, et al: The Emory prospective randomized trial: Selective versus nonselective shunt to control variceal bleeding-ten year follow-up. Ann Surg 201:712-722,1985
8. Maksoud JG, Miles S, Pinto VC: Distal splenorenal shunt in children. J Pediatr Surg 13:335-340, 1978 9. Rodgers BM, Talbert JL: Distal spleno-renal shunt for portal decompression in childhood. J Pediatr Surg 14:33-37, 1979 10. Van Vroonhoven TJ, Molenaar JC: Distal splenorenal shunt for decompression of portal hypertension in children with cystic fibrosis. Surg Gynecol Obstet 149:559-560,1979 11. Alagille D, Carlier JC, Chiva M, et al: Long-term neuropsychological outcome in children undergoing portal-systemic shunts for portal vein obstruction without liver disease. J Pediatr Gastroenter01 Nutr 5:861-866,1986 12. Patriquin H, Lafortune M, Weber A, et al: Surgical portosystemic shunts in children: Assessment with duplex Doppler US. Radiology 165:25-28,1987 13. Rodgers BM, Kaude JV: Real-time ultrasound in determination of portasystemic shunt patency in children. J Pediatr Surg 16:968-971,198l 14. Alvarez F, Bernard 0, Brunelle F? et al: Portal obstruction in children. II. Results of surgical portosystemic shunts. J Pediatr 103:703-707.1983 15. Starzl TE, Iwatsuki S, Shaw BW Jr: A “growth factor” in fine vascular anastomoses. Surg Gynecol Obstet 159:164-165, 1984
