PORTAl HYPERTENSION in pediatrics cases ses

Portal hypertension can be caused by hepatic, extra hepatic causes. It is the increase in portal vein pressure which forms varices and might cause bleeding too

1. PORTAL HYPERTENSION I n CHILDREN

2. C ONTEN T S INTRODUCTION ETIOPATHOGENESIS CLINICAL FEATURES DIAGNOSIS TREATMENT

3. Introduction The normal portal venous pressure is about 5- 10mmhg. Portal hypertension is defined when portal venous pressure is more than 10 mmhg . Portosystemic collaterals start developing with portovenous pressure of 10 mmhg . But variceal bleeding occur when portovenous pressure exceeds 12 mmhg

4. AN A T OMY *Portal vein- 8cm long *collects blood from alimentary canal, GB, pancreas, spleen *Behind the neck of pancreas>>>splenic plus superior mesenteric vein joins and forms PV. In liver, PV breaks into sinusoidal which is drained by hepatic vein>>>IVC * TRIBUTARIES 1.Paraumbilical vein 2.Cystic vein 3. Right superior pancreaticoduodenal 4. Left Gastric vein 5. Right Gastric vein

5. Sites of portal systemic collateral circulation Portal vein Systemic vein 1.Left gastric vein >> Esophageal vein 2. Sup. Rectal vein >>inf & middle rectal vein 3.Paraumbilical v.>> superior & lat. epigastric, Inf & sup epigastric, posterior intercostal 4. Splenic vein >>> left renal vein 5. Paraumbilical >>> diaphragm 6. Left branches of portal vein >> IVC

6. CLASSIFICATION OF PORTAL HYPERTENSION

7. CLINICAL FEATURES

8. ASCITIS

9. PORTO-SYSTEMIC SHUNTS REGION CLINICAL CONDITION PORTAL CIRCULATION SYSTEMIC CIRCULATION ESOPHAGEAL Esophageal varices Left gastric vein Azygous vein RECTAL Rectal varices Superior rectal vein Middle and inferior rectal veins PARAUMBILICAL Caput medusae Paraumbilical veins Superficial epigastric vein

10. CAPUT MEDUSAE Backflow from the portal vein to the paraumbilical veins increases the pressure and increased blood volume distends the veins

11. OESOPHAGEAL VARICES

12. ASTERIX Slowed oscillations in the primary motor cortex. The net result of the pathology is the failure of diencephalic motor centres in regulating tone between the agonist and antagonist muscles needed to maintain position and posture. FETOR HEPATICUS Sour, musty odour in the breath, due to volatile substances. These mercaptans if not removed by the liver are excreted through the lungs and appear in the breath.

13. APPROACH

14. History: Relevant to cirrhosis or chronic hepatitis . Gastrointestinal bleeding: number, dates, amounts, Results of previous endoscopies Patient history: blood transfusion, hepatitis B, hepatitis C, intra - abdominal, neonatal or other sepsis, myeloproliferative disorder

15. Examination Signs of hepatocellular failure Abdominal wall veins: site direction of blood fl ow Splenomegaly Liver size and consistency Ascites Oedema of legs Rectal examination

16. Complete hemogram - anaemia, evidence of hypersplenism LFT, Coagulation profile , viral markers Radiology Non-invasive Ultrasound with duplex Doppler CT scan MRI Invasive Splenoportovenography, arterioportography, percutaneous transhepaticportography Upper GI endoscopy INVESTIGATIONS

17. Management General management Specific management • Pharmacological therapy • Mechanical therapy • Endoscopic therapy • Surgical therapy Management of associated problems

18. GENERAL MANAGEMENT Adequate venous access should be established and iv fluid and /or packed cells infused Vitamin K, infusion of fresh frozen plasma and/or platelets might be infused to correct coagulopathy Continuous aspiration of ongoing bleeding to clear stomach by placing NG tube PPI (Pantoprazole)/H2 receptor antagonist is administered IV to reduce the risk of gastric erosion Monitor vitals and urine output

19. PHARMACOLOGICAL THERAPY Vasoconstrictive drugs –vasopressin 20U IV over 15 min, glypressin and terlipressin (nitroglycerine can be added) Cessation of bleeding in 50 – 80 % of cases. Somatostatin analogues such as octreotide - 1 mcg/kg body wt (max. 100 mcg), followed by 1 mcg/kg/hr as a continuous IV infusion Used till a bleeding free interval of of 24-48 hours is achieved

20. MECHANICAL THERAPY

21. VARICEAL SCLEROTHERAPY LIGATION OR BANDING WITH RUBBER ENDOSCOPIC THERAPY

22. SURGICAL THERAPY Devascularization or transaction of oesophagus which blocks the blood flow to the varices Transjugular intrahepatic portalsystemic stent shunt

23. TIPSS

24. MANAGEMENT OF ASSOCIATED PROBLEMS Hypersplenism- may need splenectomy Liver transplantation –cirrhosis and hepatic failure PROPHYLAXIS OF VARICEAL BLEEDING Beta blocker(propranolol,1-2 mg/kg/day) Heart rate should be monitored

25. COMPLICATIONS OF PORTAL HYPERTENSION Variceal bleeding (oesophageal, gastric, other) Ascites Iron deficiency anaemia Renal failure Hepatic encephalopathy Congestive gastropathy Hypersplenism

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