Variceal Bleeding

Whenever a patient starts to bleed from varices, he/she should be admitted and included in a program of variceal sclerosis or ligation in order to eradicate the varices and avoid the risk of recurrent and fatal bleeding. It is not so far recommended to start sclerotherapy in patients who have never bled . Prophylactic banding is similarly not recommended, unless in high risk patients who cannot benefit form B-Blockers treatment for first bleeding prophylaxis.

If varices are not treated appropriately, recurrent bleeding is nearly systematic and will cause decompensation of the liver disease, ascitis, sepsis , renal failure, and eventually patient's death . Sclerotherapy is able to control acute bleeding in 90 % of the cases. However, the endoscopy should not necessarily be performed immediately, since about 40% of the patients will spontaneously stop bleeding . Bed rest, correction -but not overcorrection - of hypovolemia and use of vasoconstrictors will promote arrest of bleeding . Transfusions should be limited to the minimum required , since they may reactivate bleeding by expanding intravascular volume. Central venous pressure must be monitored .

Delaying endoscopy until ressucitation completed allows to avoid difficult procedure during acute bleeding . Endoscopy aims to detect the site of bleeding, and therefore should include gastric and duodenal inspection for ulcus , hypertensive gastropathy, and or distal varices. When no site of bleeding is detected in children with biliary atresia, distal bleeding at the level of the intestinal Y loop should be suspected.

Although the material is still not appropriate for small children, variceal ligation should now be preferred to injection sclerotherapy. There is less complications after this procedure, less rebleeding, less ulcus, less oesophageal stenosis, and improved survival ^(22,23) . If injection sclerotherapy is used, all varices should be injected near to the cardia, and a second injection made more distally. A second session is programmed after 5 to 7 days, and repeated endoscopies are programmed to reach variceal eradication, which necessitates usually 4 to 5 sessions. Band ligation leads more rapidly to complete variceal eradication. When severe hypertensive gastritis dominates and no sclerotherapy nor banding is feasible. (Fig)

If sclerotherapy and or ligation fail to control bleeding, or if bleeding site can not be reached by endoscopy, the patient should be evaluated for shunt surgery or transjugular portosystemic shunt (TIPSS). Again, although feasible in young patients, the material is not adapted to infants ^(24,25). The procedure is efficient to control bleeding, but increases the risk of encephalopathy and is contraindicated in advanced cirrhosis and jaundiced patients. TIPPS may also help to control ascitis.

Surgical porto caval shunt should be avoided if the child is candidate for liver transplantation.

In case of extra-hepatic portal hypertension, due to portal thrombosis and cavernoma, without underlying liver disease, it is now possible to revasularize the portal system by bridging the thrombosed segment of the portal vein. This " mesenterico-Rex " shunt has been succesfully performed in both post liver transplant portal vein thrombosis and in children with portal cavernoma( ^26,27).

Acute bleeding
Pharmacological approach following acute bleeding include vasopressin, somatostatin or octreotide. Vasopressin is no more used in view of its marked peripheral vasoconstrictive effect, and is now replaced by somatostatin ⁽²⁸⁾ . The synthetic analogue of somatostatin, octreotide, although shown efficient against endocrine releases (glucagon, growth hormone), has a more controversial efficacy on variceal bleeding and sustained decrease in portal pressure.
Somatostatin can be infused continuously or following a repeated bolus scheme (25 to 50 µgr of Somatostatin IV bolus) , which may be more efficient to decrease variceal pressure and stop bleeding. The vasoconstrictive drugs act by reducing the splanchnic blood flow and portal flow ^{and are given for a period of five days following acute bleeding}( ²⁹⁾

Primary & secondary prophylaxis
Similarly, reduction of splanchnic blood flow and portal pressure can be obtained with B Blockers, preferentially the non selective ones . There is no data concerning the use of B blockers for primary or secondary prophylaxis of bleeding in children with portal hypertension, but this approach has been demonstrated efficient in adults and is widely used in children. B Blockers such as propanolol , 1 mg/kg 4 times daily, are indicated whenever the risk of bleeding becomes significant: large varices, presence of red wale markings, severe cirrhosis, high hepatic venous pressure gradient (> 12 mm Hg). Propanolol may reduce the portal venous pressure gradient by 15 to 25%.

Splanchnic fow can further be reduced by reducing the expanded plasma volume by a low Sodium diet and spironolactone.

Vasodilators such as isosorbide dinitrate or prazosin may decrease splanchnic flow, but are likely to increase secondary sodium and water retention in cirrhotic patients: they should be used in conjunction with vasoconstrictors in acute bleeing, and not alone nor as a chronic treatment. Practically, these drugs are rarely used and combination treatment is not clearly superior to monotherapy.

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