Portal HTN and stigmata of liver diseases.pptx

Portal hypertension

1. Portal Hypertension Presenter: Dr. Unnat Hamal Sapkota 1st year resident, MD GP and EM Moderator: Maj. Dr. Bishwo Kunwar

2. CONTENTS Anatomy of the Portal System and Introduction to Portal Hypertension Causes of Portal Hypertension Classification of Portal Hypertension Hemodynamics in Portal Hypertension Clinical Presentation Diagnosis and Investigations Management of Portal Hypertension Recent Advances

3. Anatomy of Portal System drains blood from the GI tract, pancreas, spleen, and gallbladder to the liver. i t facilitates nutrient absorption and detoxification before systemic circulation. b lood flows into the liver sinusoids for filtration and exits via the hepatic veins into the inferior vena cava

4. Anatomy of Portal System Portal Vein – Formed by the splenic vein and superior mesenteric vein. Tributaries: Splenic vein – Drains the spleen. Superior mesenteric vein – Drains the small intestine and ascending colon. Inferior mesenteric vein – Drains the descending colon and rectum. Hepatic Sinusoids – Capillary-like vessels in the liver for filtration.

5. Introduction-Portal Hypertension The normal portal venous pressure is about 5-10mmhg.  Portal hypertension is defined when portal venous pressure is 10-12mmhg.  P ortal venous pressure builds up when their is an obstruction to portal venous flow

6. Introduction-Portal Hypertension Portosystemic collaterals start developing with porovenous pressure of 10 mmhg.  But variceal bleeding occur when portovenous pressure exceeds 12 mmhg

7. Causes of Portal HTN Pre-hepatic Causes Portal vein thrombosis Splenic vein thrombosis Congenital anomalies (e.g., atresia) Intra-hepatic Causes Cirrhosis (most common cause) Schistosomiasis Granulomatous diseases (e.g., sarcoidosis) Hepatic fibrosis Post-hepatic Causes Budd-Chiari syndrome (hepatic vein obstruction) Right heart failure Constrictive pericarditis

8. Classification of Portal HTN Pre-hepatic PHTN Pre sinusoidal Sinusoidal Intra-hepatic PHTN Post-hepatic PHTN Mixed PHTN

9. Category Sub-classification Causes 1. Pre-hepatic Pre-sinusoidal - Portal vein thrombosis - Congenital atresia or stenosis - Splenic vein thrombosis Sinusoidal - Rare in pre-hepatic PHTN. Typically due to microvascular involvement. 2. Intra-hepatic Pre-sinusoidal - Schistosomiasis - Primary biliary cholangitis - Granulomatous diseases Sinusoidal - Cirrhosis (most common cause) - Alcoholic liver disease - Hepatitis Post-sinusoidal - Veno-occlusive disease - Toxic injury 3. Post-hepatic Intra-hepatic (within liver) - Budd-Chiari syndrome (hepatic vein obstruction) - Sinusoidal obstruction syndrome Post-hepatic (beyond liver) - Right heart failure - Constrictive pericarditis - Inferior vena cava obstruction

10. Hemodynamics in Portal HTN Increased Intrahepatic Resistance Splanchnic Vasodilation Collateral Circulation Formation Systemic Effects

11. Clinical Presentation of Portal HTN Fig. Classical Triad of Portal HTN

12. Clinical Presentation of Portal HTN FIg. Dilated veins on flanks seen in Budd Chiari Sndrome Fig. Caput Medusae

13. G I HEMORRHAGE: Fig. GI bleeding as a presentation in Portal HTN

14. Hypersplenism:

15. Encephalopathy:

16. Bleeding from Nongut Sites: Severe thrombocytopenia can lead to hematuria, menorrhagia in adolescent girls, epistaxis, and hematochezia.  In cases of advanced liver disease, hemorrhagic complications in the lungs may cause severe respiratory compromise.

17. Ascites: seen in advanced liver disease with synthetic failure. accompanied by a low serum albumin level , low POP . also because of increase in renal tubular absorption of sodium and water in patients with decompensated cirrhosis

18. Stigmata of Liver Disease Fig. Clinical features/stigmatas associated with CLD

19. Diagnosis diagnosis depends on the synthesis of the clinical information gathered from the parents and the child and the results of imaging tests and lab investigations.

20. Approaching a Patient with Portal HTN Through and elaboratibe history taking General and systemic examination Investigations Management of etiology and complications

21. History Taking Relevant to cirrhosis or chronic hepatitis. GI bleed  Results of previous endoscopies  Patient history Family History Exposure to any herbal medications Personal Hisotry including exposure to pets, drinking water etc.

22. 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

23. Investigations CBC  LFT Infection screening  Coagulation profile S. ammonia S. albumin PT/INR Viral serology markers Metabolic and autoimmune tests: A S MA, ANA .Copper and cerruloplasmin le vel , lactate and pyruvate, alpha1 antitrypsin level Genetic test

24. Upper GI endoscopy Is used in establishing the cause of Gl bleeding and to confirm the presence of varices in the esophagus and stomach.  Varices are small (≤ 5 mm diameter) or large ( > 5 mm diameter) when assessed with full insufflation.  The larger the varix the more likely it is to bleed.

25. White nipple sign Red wale markings (longitudinal red streaks) Cherry Red Spots Spurting or oozing blood from varices. Mucosal changes like mosaic or snakeskin patterns, with or without bleeding.

26. Casas M, Vergara M, Brullet E, Junquera F, Martínez-Bauer E, Miquel M, Sánchez-Delgado J, Dalmau B, Campo R, Calvet X. Inter and intra-observer concordance for the diagnosis of portal hypertension gastropathy. Rev Esp Enferm Dig. 2018 Mar;110(3):166-171. doi: 10.17235/reed.2018.5169/2017. PMID: 29320862

27. Ultrasonography Fig. Hepatobilliary USG

28. Computed tomography (CT) and magnetic resonance (MR) angiography Are excellent diagnostic tools and have supplanted conventional digital angiography for most purposes.  Both modalities provide excellent information about all the intra-abdominal vessels and detailed information about the liver anatomy including the bile ducts. CT-angiography is more quickly and  Is less prone to image degradation from motion artifact than in MR angiography

29. Liver Biopsy Diagnosis: Confirms underlying liver pathology, such as cirrhosis, fibrosis, or congenital hepatic fibrosis, causing portal hypertension. Severity Assessment: Evaluates the extent of fibrosis and inflammation, helping to stage liver disease and guide prognosis. Etiology Identification: Detects metabolic, autoimmune, or infiltrative liver diseases contributing to portal hypertension.  For this patient should not have ascites and should have corrected coagulation parameters.

30. Portal Pressure Measurement Measurements are taken in the wedged hepatic venous pressure (WHVP) and free hepatic venous pressure (FHVP).  The hepatic venous pressure gradient (HVPG) is the difference between WHVP and FHVP.

31. A catheter is inserted into the jugular or femoral vein and advanced to the hepatic vein under fluoroscopic guidance. Free hepatic venous pressure (FHVP) is measured first. The catheter is then wedged into a smaller hepatic vein branch to measure the wedged hepatic venous pressure (WHVP), which reflects sinusoidal pressure and correlates with portal venous pressure.HVPG is calculated as:

32. MANAGEMENT OF COMPLICATIONS OF PORTAL HTN

33. Management of Ascites Sodium Restriction: <2 g/day Fluid Restriction - severe hyponatremia Diuretics:Spironolactone + Furosemide (monitor electrolytes and renal function) If ascites is very tense, intra - abdominal pressure may be reduced by a cautious paracentesis

34. Management of acute variceal bleeding Management of acute variceal bleeding include:  1. General measures 2. V asoactive drugs 3. Sengstaken - Blakemore tube and self - expanding oesophageal stent  4. Endoscopic banding ligation and injection of varices.  5. Emergency transjugular intrahepatic stent shunt

35. General Measures Immediate Resuscitation: A cute variceal hemorrhage require intense resuscitation with blood and crystalloids, replacement of coagulation factor deficiencies with fresh frozen plasma.

36. Vasoactive Drugs Vasoactive drugs lower portal venous pressure and should be started even before diagnostic and therapeutic endoscopy. Octreotide Terlipressin Midodrine Norepinephrine

37. Romano C, Oliva S, Martellossi S, Miele E, Arrigo S, Graziani MG, et al. Pediatric gastrointestinal bleeding: Perspectives from the Italian Society of Pediatric Gastroenterology. World J Gastroenterol. 2017 Feb 28;23(8):1328-1337. doi: 10.3748/wjg.v23.i8.1328. PMID: 28293079. Terlipressin Initial Dose: Administer 10–20 µg/kg IV every 4–6 hours. Maximum Dose: Do not exceed 1 mg per dose. Duration: The optimal duration of therapy in children is not well established; however, in adults, treatment is often continued for up to 5 days. Octreotide Loading Dose: Administer 1–2 µg/kg intravenously (IV) as a bolus. Continuous Infusion: Follow with a continuous IV infusion at a rate of 1–5 µg/kg per hour. Duration: Treatment is typically continued for up to 48 hours, with careful monitoring for efficacy and adverse effects

38. Long term Management use of nonselective beta blockers such as propranolol or nadolol, Initial Dose: Start with 0.5–1 mg/kg/day, administered orally in divided doses two to three times daily. Titration: Gradually increase the dose every few days by 0.5 mg/kg/day, monitoring the patient's heart rate and blood pressure closely. Maintenance Dose: The effective dose often ranges from 2 to 4 mg/kg/day, divided into two or three doses. Some studies have used doses up to 5.25 mg/kg/day to achieve the desired heart rate reduction. Maximum Dose: Do not exceed 6 mg/kg/day or 240 mg/day, whichever is lower.

39. S engstaken black more tube  The gastric balloon is inflated with 250 mL of air, oesophageal tube is then inflated to a pressure of 40 mmHg, The sengstaken black more tube is kept in place until emergency therapeutic endoscopy or tips can be performed.

40. Complications include:  obstruction to upper airways.  Ulceration of the lower oesophagus complicates prolonged or repeated use.  Oesophagel rupture can occur, usually when the gastric balloon is wrongly inflated in the oesophagus

41. Endoscopic Band Ligation Procedure: Elastic bands are placed around the varices during endoscopy to cause vascular thrombosis and ligation. Effect: Reduces bleeding risk by eliminating or controlling varices. Complications: Rare but may include ulceration, stricture formation, and bleeding.

42. Endoscopic Injection Sclerotherapy Sclerosing solutions include sodium morrhuate, ethanolamine, sodium tetradecyl sulfate, and polidocanol are used . Generally, two to three injections at 1 mL per injection are required for each varix, up to a maximum of 10 to 15 mL per session Effect: Reduces bleeding risk by scarifying and collapsing varices. Complications: Possible esophageal ulceration, stricture, and bleeding.

43. TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNTS(TIPS)

44. used in children with cystic fibrosis, biliary atresia,and congenital hepatic fibrosis Its primary limitation is the high rate of shunt thrombosis. Vigilance is required to monitor shunt patency and to declot the shunt when it is thrombosed  The use of PTFE stents has greatly reduced the rate of occlusion compared to bare metal stents

45. Recent Advances Use of WIreless Endoscopy

46. Recent Advances Meso Rex Shunt

47. Take Home Message Definition and Causes: Portal hypertension results from increased pressure in the portal venous system, most commonly caused by liver cirrhosis, thrombosis, or congenital anomalies. Clinical Signs: Key presentations include variceal bleeding, ascites, splenomegaly, and encephalopathy. Early diagnosis is crucial for effective management. Diagnostic Tools: Imaging (ultrasound, CT, MRI) and endoscopy are vital for confirming diagnosis and assessing severity.

48. Acute Management: Use vasoactive drugs such as octreotide (1–2 mcg/kg bolus, then 1–5 mcg/kg/hour infusion) and terlipressin (10–20 mcg/kg every 4–6 hours). Endoscopic therapies (band ligation, sclerotherapy) and emergency procedures (Sengstaken-Blakemore tube, TIPS) may be needed. Long-term Management: Non-selective beta-blockers like propranolol (initial 0.5–1 mg/kg/day, titrate up to 2–4 mg/kg/day) to reduce variceal rebleeding risk. Regular monitoring and preventive strategies can improve prognosis. Multidisciplinary Approach: Effective treatment involves gastroenterologists, hepatologists, and intensivists for comprehensive care.

49. REFRENCES Mehta SK. Portal hypertension in children. Indian Pediatr. 1992 Jul;29(7):945-53 de Kolster CC, Rapa de Higuera M, Carvajal A, Castro J, Callegari C, Kolster J. Propranolol en niños y adolescentes con hipertensión portal: dosis, efectos clínicos, cardiovasculares y bioquímicos [Propranolol in children and adolescents with portal hypertension: its dosage and the clinical, cardiovascular and biochemical effects]. G E N. 1992 Jul-Sep;46(3):199-207. Spanish. PMID: 1340825. Casas M, Vergara M, Brullet E, Junquera F, Martínez-Bauer E, Miquel M, Sánchez-Delgado J, Dalmau B, Campo R, Calvet X. Inter and intra-observer concordance for the diagnosis of portal hypertension gastropathy. Rev Esp Enferm Dig. 2018 Mar;110(3):166-171. doi: 10.17235/reed.2018.5169/2017. PMID: 29320862 Zhang J, Li L. Rex Shunt for Extra-Hepatic Portal Venous Obstruction in Children. Children (Basel). 2022 Feb 21;9(2):297. doi: 10.3390/children9020297. PMID: 35205017; PMCID: PMC8870553.

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