Portal hypertension

K76.6

DESCRIPTION

Increased portal venous pressure above vena cava pressure. Most commonly secondary to cirrhosis, but causes without cirrhosis may be divided into prehepatic portal vein obstruction, intra-hepatic (pre-or post-sinusoidal) and post-hepatic causes.

DIAGNOSTIC CRITERIA

Clinical

  • Splenomegaly with ascites, variceal haemorrhage or hypersplenism.

Investigations

  • FBC may show hypersplenism.
  • Doppler assisted ultrasound and angiography.
  • Investigations as listed under cirrhosis.

GENERAL AND SUPPORTIVE MEASURES

  • Determine and manage underlying cause.

REFERRAL

  • All children with portal hypertension should be referred.

BLEEDING OESOPHAGEAL VARICES

I85.0

DESCRIPTION

Presentation with haematemesis (fresh blood) or melaena in a patient who has a spontaneous bleed from varices at the oesophageal-gastric junction. The patient may or may not have been known to have chronic liver disease and portal hypertension. This bleeding may be hard to control and be life threatening.

GENERAL AND SUPPORTIVE MEASURES

  • Resuscitation and blood transfusion as required.
  • For local control of acute bleeds that are not controlled with medicine treatment: Sengstaken tube.
  • For secondary prophylaxis after a bleed: refer for endoscopic injection sclerotherapy or variceal banding every 2 weeks until eradicated.
  • If either or both treatments fail: surgical over-sewing.

MEDICINE TREATMENT

  • Octreotide, IV, bolus, 1–2 mcg/kg immediately then 1–5 mcg/kg/hour by infusion. (Specialist initiated).

LoE: III[10]

Post bleed prophylactic management

  • Proton pump inhibitor, e.g.:
  • Omeprazole, oral, 0.7-1.4 mg/kg/day once daily. Specialist initiated.
  • Maximum dose: 20-40 mg/dose.
    If 1 month–2 years: 5 mg once daily.
    If > 2–6 years: 10 mg once daily.
    If > 7–12 years: 20 mg once daily.

LoE: III[8]

AND

  • Propranolol, oral, 2 mg/kg daily in 3 divided doses.
    • If needed, increase dose to 8 mg/kg/24 hours.
    • Aim to reduce the resting pulse rate by 25%.

REFERRAL

  • All, to establish diagnosis and initiate treatment.
  • Bleeding varices: only after commencement of resuscitation and octreotide, if available.

ASCITES DUE TO PORTAL HYPERTENSION

R18

GENERAL AND SUPPORTIVE MEASURES

  • Restrict sodium intake, 1-2 mmol/kg/24 hours.
  • Restrict fluids if serum sodium < 130 mmol/L.

MEDICINE TREATMENT

  • Spironolactone, oral, 1–3 mg/kg as a single daily dose. Can increase dosage slowly to 4-6 mg/kg/day.
    • Continue for as long as needed to control ascites.
    • Monitor serum potassium.

If insufficient response, add:

  • Furosemide, oral, 1-3 mg/kg as a single daily dose.
  • Spironolactone to furosemide ratio should be 2.5:1.

OR (do not give furosemide and hydrochlorothiazide together)

  • Hydrochlorothiazide, oral, 1 mg/kg/dose 12–24 hourly.
  • Maximum dose: 25 mg daily.

Therapeutic paracentesis may be performed to relieve the cardiorespiratory and gastrointestinal manifestations of tense ascites. The upper abdomen, surgical scars, the bladder and collateral vessels should be avoided when inserting the paracentesis needle. 50 mL/kg ascites can be tapped over an hour with IV albumin 1 g/kg to prevent circulatory dysfunction.

LoE: III[11]

REFERRAL

  • Urgent: Refractory ascites interfering with respiration.
  • For determination of the underlying cause of the cirrhosis, portal hypertension and initiation of treatment.
  • Cirrhosis, portal hypertension and/or liver failure not responding to adequate therapy.
  • Hepatic encephalopathy.