E) Surgery - porto-systemic shunts :

Endoscopic sclerotherapy with banding is an effective primary treatment modality of bleeding oesophageal varices in majority of children with reasonable liver function. However, surgical intervention is indicated for the following cases:

• Uncontrolled bleeding from the oesophageal varices not responding to at least 2 sessions of banding or sclerotherapy
  
  
• Bleeding gastric or ectopic varices not responding to endoscopic treatment
  
  
• Hypersplenism or massive symptomatic splenomegaly
  
  
• Lack of access to endoscopic treatment
  
  
• Symptomatic biliary obstruction due to choledochal varices
  
  
• Selected patients with Budd-Chiari syndrome
  
  

The great variety of surgical procedures advocated for the management of portal hypertension was reflected in a French multi-centre study of children treated both in European countries and North Africa . Thirty different operations, which included a range of porto-systemic shunts and various devascularization techniques, were reported from the treatment of 109 children. The variety of surgical techniques can be explained by both the varied pathology of the portal venous system and the preferences and experience of individual surgeons. Occlusion of the portal system in the extrahepatic portal hypertension, for example, may affect the portal vein alone or may involve either the splenic or the superior mesenteric veins. At least 20 percent of these patients are not suitable for any form of shunt surgery and may undergo some type of devascularization with or without oesophageal transection. Unfortunately, devascularisation procedures do have a high incidence of rebleeding of up to 23 percent.

The construction of a shunt between the superior mesenteric vein and the inferior vena cava using a segment of the internal jugular vein seems to offer the best combination of long term patency and the lowest incidence of rebleeding. The rebleed rate was only 6 percent in four series of cases published since 1981.

The recent introduction of the mesenterico-left portal (Rex) shunt is likely to broaden the indications once more for shunt surgery as the primary treatment for children with portal venous occlusion. This shunt utilizes an interposition graft between the superior mesenteric vein and the intra-hepatic portion of the left portal vein, which is identified in the Rex recessus adjacent to the falciform ligament. By restoring hepatic portal blood flow and correcting portal hypertension, this technique is more physiological and obviates the potential disadvantages of the porto-systemic shunts.

The complications of porto-systemic shunting are not only concerned with rebleeding. Deterioration of liver function and hepatic encephalopathy are further hazards, particularly in children with cirrhosis. In a recent series of 37 children, 1 died in the early postoperative period with encephalopathy and 9 out of 31 with a patent shunt showed deterioration of mental function during a mean follow-up period of 5 years.

Porto-systemic shunt surgery should be regarded as a complementary therapy to endoscopic treatment. Porto-mesenteric venous anatomy does not permit successful shunt surgery in every child, and shunt thrombosis has been recorded with all types of shunts especially in smaller children.

Liver transplantation is the treatment of choice for children with variceal bleeding complicating end-stage chronic liver disease.

The management of children with bleeding oesophageal varices is an extremely challenging task and demands a variety of complementary techniques, each of which may be limited by its applicability, efficacy and its complications. Endoscopic sclerotherapy and banding is highly effective and appears to be the treatment of choice for the initial management of oesophageal varices in children.

However, shunt surgery should be reserved for

• treatment of gastric or ectopic varices not accessible to sclerotherapy
• uncontrollable bleeding secondary to a complication of sclerotherapy
• treatment of severe hypersplenism or symptomatic splenomegaly
• treatment of children living in communities far away from adequate medical care and blood transfusion facilities.
  
  

Liver transplantation is the procedure of choice for patients with complications of portal hypertension associated with end-stage liver disease. The role of newer modalities like the TIPS and Rex shunt has yet to be conclusively proven.

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3. Fonkalsrud EW. Surgical management of portal hypertension in childhood. Archives of Surgery 1980;115: 1042 - 1045
4. Howard ER, Stringer MD, Mowat AP. Assessment of injection sclerotherapy in the management of 152 children with oesophageal varices. British Journal of Surgery1988; 75:404 - 408
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Redkar R.Recent Approach To Portal Hypertension In Children.Pediatric Oncall [serial online] 2006 [cited 2006 November 1];3. Art # 40. Available from:

http://www.pediatriconcall.com/fordoctor/diseasesandcondition/hepatobiliary_disorders_old/Recent_approach_portal_hypertension.asp