The survival of the children with portal hypertension depends almost entirely on the etiology. Recent reports show that oesophageal varices in childhood are well controlled with either injection sclerotherapy or porto-systemic shunting and both methods have their advocates. Patients with portal vein obstruction and normal liver histology can be expected to live normal lives providing the oesophageal varices are under control.

A)Treatment of the acute bleed

Acute variceal bleeding, particularly in young infants, can pose problems in management. A delay in immediate management could prove fatal for a child. Medical measures include blood transfusion and the intravenous infusion of vasopressin (0.2 - 0.4 units/1.73 m / min) which may arrest the bleeding. Vasopressin or its precursor, glypressin may be used alone or in combination with nitrates to reduce the portal venous pressure. Unfortunately, these agents have side-effects related to systemic vaso-constriction like headache, nausea and abdominal cramps. Somatostatin reduces splanchnic blood flow and portal pressure with minimal side-effects, but it has a short half life of less than 3 minutes. Octreotide, a long acting analog of somatostatin, has a plasma half-life of more than 1 hour. Although the effectiveness of octreotide has been studied in a small number of children, its safety and side-effect profile have encouraged its use in cases of acute variceal bleeding.

Continued bleeding may be controlled with injection sclerotherapy but the small size of the paediatric endoscope channels can limit the clearance of blood from within the oesophagus. In addition to the above difficulties, there is an added risk of needing general anesthesia in a child with a compromised consciousness. The Sangstaken-Blackmore (S-B tube) compression balloon may be life saving when there is a failure of visualization of the varices due to overwhelming haemorrhage. However, the dangers of this instrument cannot be overemphasized. Correct placement of the gastric balloon must be checked with X-ray control in order to avoid the inflation within the lumen of the oesophagus. This accidental inflation with the oesophagus may result in oesophageal rupture or suffocation from airway obstruction. Inflation of the gastric balloon and moderate prolonged traction achieved by securing the S-B tube to the side of the face with an adhesive tape is usually sufficient to stop the bleeding. It is rarely necessary to inflate the oesophageal balloon present on the standard instrument. Balloon deflation is performed 18 to 24 hours later and this is followed immediately with endoscopic variceal injection.