- Email: [email protected]
Invited commentary
728
Orloff et al
Bleeding Esophagogastric Varices from EHPH
40. Auvert J, Weisgerber G. Immediate and long-term results of superior mesenteric vein—inferior vena cava shunt for portal hypertension in children. J Pediatr Surg 1975;10:901–908. 41. Lambert MJ III, Tank ES, Turcotte JG. Late sequelae of mesocaval shunts in children. Am J Surg 1974;127:19–24. 42. Reynolds JT, Southwick HW. Portal hypertension: use of venous grafts when side to side anastomosis is impossible. Arch Surg 1951;62:789–800. 43. Rousselot LM. Autogenous vein graft in spleno-renal anastomosis: description of technique and its clinical application in 7 patients. Surgery 1952;31:403–410. 44. Bismuth H, Sherlock DJ. Portosystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg 1991; 214:581–589. 45. Vons C, Bourtstyn E, Bonnet P, et al. Results of portal systemic shunts in Budd-Chiari syndrome. Ann Surg 1986;203:366–370. 46. Kahn D, Terblanche J, Kitano S, Bornman P. Injection sclerotherapy in adult patients with extrahepatic portal venous obstruction. Br J Surg 1987;74:600–602. 47. Howard ER, Stringer MD, Mowat AP. Assessment of injection sclerotherapy in the management of 152 children with oesophageal varices. Br J Surg 1988;75:404–408. 48. Bhargava DK, Dwivedi M, Dasarathy S, Arora A. Endoscopic sclerotherapy for portal hypertension due to extrahepatic obstruction: long-term follow-up. Gastrointest Endosc 1989;35: 309–311. 49. Thatcher BS, Sivak MV Jr, Petrini JL. Endoscopic sclerotherapy for bleeding esophageal varices secondary to extrahepatic portal vein obstruction. Gastrointest Endosc 1987;33:214–219. 50. Dilawari JB, Chawla YK, Ramesh GN, et al. Endoscopic sclerotherapy in children. J Gastroenterol Hepatol 1989;4:155–160. 51. Szczepanik AB, Rudowski WJ. Extrahepatic portal hypertension: long-term results of surgical treatment. Ann R Coll Surg Engl 1989;71:222–225. 52. Belli L, Romani F, Riolo F, et al. Thrombosis of portal vein in absence of hepatic disease. Surg Gynecol Obstet 1969;169:46–49. 53. Cello JP, Grendell JH, Crass RA, et al. Endoscopic sclerotherapy versus portacaval shunt in patients with severe cirrhosis and acute variceal hemorrhage. N Engl J Med 1987;316:11–16. 54. Rikkers LF, Burnett DA, Volentine GD, et al. Shunt surgery versus endoscopic sclerotherapy for long-term treatment of variceal bleeding. Early results of a randomized trial. Ann Surg 1987;206:262–271. 55. Kjaergaard J, Fischer A, Miskowiask J, et al. Sclerotherapy of bleeding esophageal varices. Long-term results. Scand J Gastroenterol 1982;17:363–367. 56. MacDougall BRD, Theodossi A, Westaby D, et al. Increased long-term survival in variceal haemorrhage using injection sclerotherapy. Results of a controlled trial. Lancet 1982;1:124–127. 57. Terblanche J, Kahn D, Campbell JAH, et al. Failure of repeated injection sclerotherapy to improve long-term survival after oesophageal variceal bleeding. Lancet 1983;1:1328–1332. 58. The Copenhagen Esophageal Varices Sclerotherapy Project. Sclerotherapy after first variceal hemorrhage in cirrhosis. A randomized multicenter trial. N Engl J Med 1984;311:1594–1600. 59. Korula J, Balart LA, Radvin G, et al. A prospective, randomized controlled trial of chronic esophageal variceal sclerotherapy. Hepatology 1985;5:584–589. 60. So¨ derlund C, Ihre T. Endoscopic sclerotherapy v. conservative management of bleeding oesophageal varices. Acta Chir Scand 1985;151:449–456.
J Am Coll Surg
61. Fonkalsrud EW. Surgical management of portal hypertension in children. Arch Surg 1980;115:1042–1045. 62. Hamilton DW, Hunt AH. Extrahepatic portal obstruction. Med J Aust 1970;1:493–499. 63. Boles ET Jr, Wise WE Jr, Birken G. Extrahepatic portal hypertension in children: long-term evaluation. Am J Surg 1986;151: 734–739.
Invited Commentary Layton F Rikkers, MD Madison, WI Dr Orloff and his colleagues make a strong case for portal-systemic shunts as primary therapy for patients with bleeding varices secondary to extrahepatic portal hypertension. Their prospective series of 200 patients is remarkable with respect to its size, the length and completeness of followup, and the excellent results achieved. To my knowledge, this is the largest such experience ever reported and the highest shunt patency ever achieved in this challenging group of patients. I agree with most of the concepts put forth by Dr Orloff regarding the pathophysiology and treatment of portal hypertension in these patients. Although pharmacotherapy and endoscopic sclerosis or banding of varices are useful for the management of acutely bleeding patients, a portal-systemic shunt, when possible, is the preferred definitive treatment for this population. The risk of surgery is low and the longterm benefit is substantial. The type of shunt done is dictated by which veins are available and their size. Longterm shunt patency is a more important consideration than the type of shunt performed, because nearly all patients with normal livers do not develop neuropsychiatric sequelae. In patients with isolated portal vein thrombosis, I have preferred a distal splenorenal shunt because it does preserve hepatic portal perfusion, which might be of benefit in the longterm, and the patency rate in most series is similar to that reported here by Dr Orloff. The concept that patients with portal vein thrombosis have no portal flow to their livers is erroneous. In fact, when this has been studied by a variety of techniques, patients with portal vein thrombosis tend to have better nutrient perfusion of their hepatic sinusoids by portal flow through hepatopetal collaterals than do patients with cirrhosis and open portal veins. In contrast to patients with cirrhosis, intrahepatic vascular resistance in
Vol. 194, No. 6, June 2002
Orloff et al
these patients is low, and hepatic portal perfusion following a distal splenorenal shunt can be preserved indefinitely. Although I agree that splenectomies are often performed inappropriately for clinically insignificant hypersplenism in children with extrahepatic portal hypertension, I believe that a splenectomy should be a component of the operation when splenic vein thrombosis is present. Therefore, we have preferred the combination of splenectomy and interposition mesocaval or mesorenal shunt when splenic vein thrombosis is associated with portal vein thrombosis. I have a number of questions for Dr Orloff. In my experience, a number of patients with either isolated superior mesenteric vein thrombosis or superior mesenteric vein thrombosis in association with portal vein thrombosis eventually experience bleeding from small bowel or large bowel varices whether or not they have had a portal-systemic shunt. A splenorenal shunt of any variety does not decompress the superior mesenteric venous circulation when the superior mesenteric vein is occluded. Did any of the patients with superior mesenteric vein thrombosis in this series experience unexplained lower gastrointestinal bleeding that might have been from small bowel or large bowel varices? In analyzing my own series of shunts done for either cirrhotic or noncirrhotic portal hypertension, shunt thrombosis has been more common when the splanchnic vein utilized is 6 mm or less in diameter. What is the minimal size of vein the authors would use in constructing a shunt in patients with extrahepatic portal hypertension? Because there is an extremely low mortality associated with bleeding in these patients, does it make sense in infants and young children to delay surgery and allow growth of these veins before constructing a portalsystemic shunt? Do the authors always perform a splenectomy when the splenic vein is thrombosed? If not, why not? What makes this situation different from isolated splenic vein thrombosis, which often causes bleeding from gastric varices in patients without generalized portal hypertension? I would agree that prophylactic shunts in patients that have not bled are not indicated in this population of patients. A vexing problem I have faced occasionally is the patient with massive splenomegaly, no bleeding, and clinically significant hypersplenism with platelet counts persistently less than 25,000 or white blood cell counts
Bleeding Esophagogastric Varices from EHPH
729
less than 1200, or both. I have been reluctant to perform a splenectomy in such patients because it eliminates future shunt options. What does Dr Orloff prefer in this situation? Would he perform a prophylactic portalsystemic shunt to relieve hypersplenism and to prevent future variceal bleeding? Finally, with very few portal-systemic shunts currently being done in surgical training programs throughout our country, who will perform these operations in future years?
Reply Marshall J Orloff, MD, FACS San Diego, CA In response to the insightful commentary of Dr Rikkers, I would like first to answer the specific questions that he posed, 1. None of the 200 patients in our series had lower gastrointestinal bleeding, either before or after portal-systemic shunt (PSS). In our experience with extrahepatic portal hypertension, bleeding from small bowel or large bowel varices is rare. 2. As indicated in our manuscript, French surgeons have obtained consistent longterm success in children as young as 16 months of age with veins 6 mm or less in diameter. The French surgeons have reported substantial data to support their recommendation that PSS be done after only one episode of variceal bleeding, regardless of the child’s age or size of the veins. We concur with that recommendation. Our series contains a number of children aged 4 to 9 years who, with the use of magnification technique, underwent successful PSS using veins smaller than 10 mm in diameter. 3. When the splenic vein was thrombosed and the spleen was intact, we performed a mesocaval shunt without splenectomy. After mesocaval shunt in such patients, esophageal and gastric varices invariably disappeared, the enlarged spleen decreased in size, and hypersplenism with cytopenia was corrected or markedly improved. It is clear that reduction of the pressure in the superior mesenteric vein resulted in a reduction of pressure in the portal-systemic collateral veins. Our experience indicates that splenectomy is not indicated. 4. I agree with the reluctance of Dr Rikkers to perform a splenectomy in the patient with marked splenomegaly and pronounced hypersplenism who has never bled from esophagogastric varices. We have not performed a prophy-
Orloff et al
Bleeding Esophagogastric Varices from EHPH
40. Auvert J, Weisgerber G. Immediate and long-term results of superior mesenteric vein—inferior vena cava shunt for portal hypertension in children. J Pediatr Surg 1975;10:901–908. 41. Lambert MJ III, Tank ES, Turcotte JG. Late sequelae of mesocaval shunts in children. Am J Surg 1974;127:19–24. 42. Reynolds JT, Southwick HW. Portal hypertension: use of venous grafts when side to side anastomosis is impossible. Arch Surg 1951;62:789–800. 43. Rousselot LM. Autogenous vein graft in spleno-renal anastomosis: description of technique and its clinical application in 7 patients. Surgery 1952;31:403–410. 44. Bismuth H, Sherlock DJ. Portosystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg 1991; 214:581–589. 45. Vons C, Bourtstyn E, Bonnet P, et al. Results of portal systemic shunts in Budd-Chiari syndrome. Ann Surg 1986;203:366–370. 46. Kahn D, Terblanche J, Kitano S, Bornman P. Injection sclerotherapy in adult patients with extrahepatic portal venous obstruction. Br J Surg 1987;74:600–602. 47. Howard ER, Stringer MD, Mowat AP. Assessment of injection sclerotherapy in the management of 152 children with oesophageal varices. Br J Surg 1988;75:404–408. 48. Bhargava DK, Dwivedi M, Dasarathy S, Arora A. Endoscopic sclerotherapy for portal hypertension due to extrahepatic obstruction: long-term follow-up. Gastrointest Endosc 1989;35: 309–311. 49. Thatcher BS, Sivak MV Jr, Petrini JL. Endoscopic sclerotherapy for bleeding esophageal varices secondary to extrahepatic portal vein obstruction. Gastrointest Endosc 1987;33:214–219. 50. Dilawari JB, Chawla YK, Ramesh GN, et al. Endoscopic sclerotherapy in children. J Gastroenterol Hepatol 1989;4:155–160. 51. Szczepanik AB, Rudowski WJ. Extrahepatic portal hypertension: long-term results of surgical treatment. Ann R Coll Surg Engl 1989;71:222–225. 52. Belli L, Romani F, Riolo F, et al. Thrombosis of portal vein in absence of hepatic disease. Surg Gynecol Obstet 1969;169:46–49. 53. Cello JP, Grendell JH, Crass RA, et al. Endoscopic sclerotherapy versus portacaval shunt in patients with severe cirrhosis and acute variceal hemorrhage. N Engl J Med 1987;316:11–16. 54. Rikkers LF, Burnett DA, Volentine GD, et al. Shunt surgery versus endoscopic sclerotherapy for long-term treatment of variceal bleeding. Early results of a randomized trial. Ann Surg 1987;206:262–271. 55. Kjaergaard J, Fischer A, Miskowiask J, et al. Sclerotherapy of bleeding esophageal varices. Long-term results. Scand J Gastroenterol 1982;17:363–367. 56. MacDougall BRD, Theodossi A, Westaby D, et al. Increased long-term survival in variceal haemorrhage using injection sclerotherapy. Results of a controlled trial. Lancet 1982;1:124–127. 57. Terblanche J, Kahn D, Campbell JAH, et al. Failure of repeated injection sclerotherapy to improve long-term survival after oesophageal variceal bleeding. Lancet 1983;1:1328–1332. 58. The Copenhagen Esophageal Varices Sclerotherapy Project. Sclerotherapy after first variceal hemorrhage in cirrhosis. A randomized multicenter trial. N Engl J Med 1984;311:1594–1600. 59. Korula J, Balart LA, Radvin G, et al. A prospective, randomized controlled trial of chronic esophageal variceal sclerotherapy. Hepatology 1985;5:584–589. 60. So¨ derlund C, Ihre T. Endoscopic sclerotherapy v. conservative management of bleeding oesophageal varices. Acta Chir Scand 1985;151:449–456.
J Am Coll Surg
61. Fonkalsrud EW. Surgical management of portal hypertension in children. Arch Surg 1980;115:1042–1045. 62. Hamilton DW, Hunt AH. Extrahepatic portal obstruction. Med J Aust 1970;1:493–499. 63. Boles ET Jr, Wise WE Jr, Birken G. Extrahepatic portal hypertension in children: long-term evaluation. Am J Surg 1986;151: 734–739.
Invited Commentary Layton F Rikkers, MD Madison, WI Dr Orloff and his colleagues make a strong case for portal-systemic shunts as primary therapy for patients with bleeding varices secondary to extrahepatic portal hypertension. Their prospective series of 200 patients is remarkable with respect to its size, the length and completeness of followup, and the excellent results achieved. To my knowledge, this is the largest such experience ever reported and the highest shunt patency ever achieved in this challenging group of patients. I agree with most of the concepts put forth by Dr Orloff regarding the pathophysiology and treatment of portal hypertension in these patients. Although pharmacotherapy and endoscopic sclerosis or banding of varices are useful for the management of acutely bleeding patients, a portal-systemic shunt, when possible, is the preferred definitive treatment for this population. The risk of surgery is low and the longterm benefit is substantial. The type of shunt done is dictated by which veins are available and their size. Longterm shunt patency is a more important consideration than the type of shunt performed, because nearly all patients with normal livers do not develop neuropsychiatric sequelae. In patients with isolated portal vein thrombosis, I have preferred a distal splenorenal shunt because it does preserve hepatic portal perfusion, which might be of benefit in the longterm, and the patency rate in most series is similar to that reported here by Dr Orloff. The concept that patients with portal vein thrombosis have no portal flow to their livers is erroneous. In fact, when this has been studied by a variety of techniques, patients with portal vein thrombosis tend to have better nutrient perfusion of their hepatic sinusoids by portal flow through hepatopetal collaterals than do patients with cirrhosis and open portal veins. In contrast to patients with cirrhosis, intrahepatic vascular resistance in
Vol. 194, No. 6, June 2002
Orloff et al
these patients is low, and hepatic portal perfusion following a distal splenorenal shunt can be preserved indefinitely. Although I agree that splenectomies are often performed inappropriately for clinically insignificant hypersplenism in children with extrahepatic portal hypertension, I believe that a splenectomy should be a component of the operation when splenic vein thrombosis is present. Therefore, we have preferred the combination of splenectomy and interposition mesocaval or mesorenal shunt when splenic vein thrombosis is associated with portal vein thrombosis. I have a number of questions for Dr Orloff. In my experience, a number of patients with either isolated superior mesenteric vein thrombosis or superior mesenteric vein thrombosis in association with portal vein thrombosis eventually experience bleeding from small bowel or large bowel varices whether or not they have had a portal-systemic shunt. A splenorenal shunt of any variety does not decompress the superior mesenteric venous circulation when the superior mesenteric vein is occluded. Did any of the patients with superior mesenteric vein thrombosis in this series experience unexplained lower gastrointestinal bleeding that might have been from small bowel or large bowel varices? In analyzing my own series of shunts done for either cirrhotic or noncirrhotic portal hypertension, shunt thrombosis has been more common when the splanchnic vein utilized is 6 mm or less in diameter. What is the minimal size of vein the authors would use in constructing a shunt in patients with extrahepatic portal hypertension? Because there is an extremely low mortality associated with bleeding in these patients, does it make sense in infants and young children to delay surgery and allow growth of these veins before constructing a portalsystemic shunt? Do the authors always perform a splenectomy when the splenic vein is thrombosed? If not, why not? What makes this situation different from isolated splenic vein thrombosis, which often causes bleeding from gastric varices in patients without generalized portal hypertension? I would agree that prophylactic shunts in patients that have not bled are not indicated in this population of patients. A vexing problem I have faced occasionally is the patient with massive splenomegaly, no bleeding, and clinically significant hypersplenism with platelet counts persistently less than 25,000 or white blood cell counts
Bleeding Esophagogastric Varices from EHPH
729
less than 1200, or both. I have been reluctant to perform a splenectomy in such patients because it eliminates future shunt options. What does Dr Orloff prefer in this situation? Would he perform a prophylactic portalsystemic shunt to relieve hypersplenism and to prevent future variceal bleeding? Finally, with very few portal-systemic shunts currently being done in surgical training programs throughout our country, who will perform these operations in future years?
Reply Marshall J Orloff, MD, FACS San Diego, CA In response to the insightful commentary of Dr Rikkers, I would like first to answer the specific questions that he posed, 1. None of the 200 patients in our series had lower gastrointestinal bleeding, either before or after portal-systemic shunt (PSS). In our experience with extrahepatic portal hypertension, bleeding from small bowel or large bowel varices is rare. 2. As indicated in our manuscript, French surgeons have obtained consistent longterm success in children as young as 16 months of age with veins 6 mm or less in diameter. The French surgeons have reported substantial data to support their recommendation that PSS be done after only one episode of variceal bleeding, regardless of the child’s age or size of the veins. We concur with that recommendation. Our series contains a number of children aged 4 to 9 years who, with the use of magnification technique, underwent successful PSS using veins smaller than 10 mm in diameter. 3. When the splenic vein was thrombosed and the spleen was intact, we performed a mesocaval shunt without splenectomy. After mesocaval shunt in such patients, esophageal and gastric varices invariably disappeared, the enlarged spleen decreased in size, and hypersplenism with cytopenia was corrected or markedly improved. It is clear that reduction of the pressure in the superior mesenteric vein resulted in a reduction of pressure in the portal-systemic collateral veins. Our experience indicates that splenectomy is not indicated. 4. I agree with the reluctance of Dr Rikkers to perform a splenectomy in the patient with marked splenomegaly and pronounced hypersplenism who has never bled from esophagogastric varices. We have not performed a prophy-