PORTAL HYPERTENSION OUTLINE: Definition Causes Pathogenesis Clinical features Investigations Management Complications Prognosis Approach Definition: Defined as: Portal Pressure > 10-12 mm Hg, with diameter >10mm Or Hepatic Venous Pressure Gradient > 4 mm Hg increased portal resistance or increased portal venous blood flow major cause of morbidity and mortality in chronic liver diseases Portal Vein: Causes of Portal HTN: Extrahepatic/Pre-hepatic Hepatic Pre-Sinusoidal Sinusoidal Post-Sinusoidal Post-hepatic A. Extra-hepatic: Portal Vein Thrombosis- Most common Neonates: Omphalitis, Umbilical Vein Catheterization, Dehydration, Sepsis Older Children: Intra-abdominal infections e.g., Appendicitis, IBD, PSC Hypercoagulable states: Deficiencies of factor V Leiden, protein C, S Blunt Abdominal Trauma Portal vein agenesis, atresia, stenosis Splenic vein thrombosis Biliary tract disease Extrahepatic biliary atresia Choledochal cyst B. Intra-hepatic: C. Post-hepatic: Budd-Chiari Syndrome IVC Webs Chronic Constrictive Pericarditis Pathogenesis And Consequence of Portal HTN Portosystemic collaterals: Sites: Lower part of esophagus Lower part of rectum Around Umbilicus Clinical Features: Bleeding: Most common presentation risk of first bleed in cirrhosis is 22% rises to 38% in with known varices >5-yr period Pattern of bleeding Hematemesis/Malena: Most common worsened by Stress / Intercurrent illness Size of varices → Bleeding Splenomegaly: 2nd Most common presentation asymptomatic or associated with cytopenia Ascites: Seen in 7-21% patients Less common but important manifestations Portal Hypertensive Biliopathy Growth Failure Hepatopulmonary Syndrome Porto-pulmonary HTN Caput Medusae: Abnormal, dilated venous network on anterior abdominal wall, radiating from the umbilicus Not seen in extra-hepatic portal HTN Seen in intra-hepatic portal HTN Continuous murmur between umbilicus and lower sternum Cruveilhier-Baumgarten Murmur Investigations USG with Doppler portal vein diameter > 10 mm hepatic diseases, masses, presence of varices and ascites ascertain pattern of flow Reversal of portal blood flow (Hepatofugal flow) - Associated with bleeding varices Cavernous transformation of the portal vein in EHPVO Increased thickness of lesser omentum CECT and MRA: Needed in selective cases Selective Arteriography: When surgical decompression is being planned GIT Endoscopy: Most reliable to detect varices Other investigations: CBC LFT Barium swallow Portal angiogram Percutaneous intrasplenic measurement of portal pressure Venography A. Emergency Management of Bleeding Varices 1st Step (Initial resuscitation): airway protection Obtain I/V Access Restoration of IV volume: fluid and BT PRBC: Target Hb: 7-9 g/dL Correction of coagulopathy: vitamin K, FFP/PC NG

1. PORTAL HYPERTENSION
PRESENTER:
DR. PRABIN PAUDYAL
1ST YEAR RESIDENT
DEPARTMENT OF PEDIATRICS AND
ADOLESCENCE MEDICINE
MODERATOR:
DR. DHIRENDRA PRASAD YADAV
ASSISTANT PROFESSOR
DEPARTMENT OF PEDIATRICS AND
ADOLESCENCE MEDICINE

2.  Definition
 Causes
 Pathogenesis
 Clinical features
 Investigations
 Management
 Complications
 Prognosis
 Approach
OUTLINE:

3. Definition:
 Defined as:
 Portal Pressure > 10-12 mm Hg, with diameter >10mm Or
 Hepatic Venous Pressure Gradient > 4 mm Hg
 increased portal resistance or increased portal venous blood flow
 major cause of morbidity and mortality in chronic liver diseases

4. Portal Vein:

5. Causes of Portal HTN:
1) Extrahepatic/Pre-hepatic
2) Hepatic
a) Pre-Sinusoidal
b) Sinusoidal
c) Post-Sinusoidal
3) Post-hepatic

6. A. Extra-hepatic:
 Portal Vein Thrombosis- Most common
 Neonates: Omphalitis, Umbilical Vein Catheterization, Dehydration, Sepsis
 Older Children: Intra-abdominal infections e.g., Appendicitis, IBD, PSC
 Hypercoagulable states: Deficiencies of factor V Leiden, protein C, S
 Blunt Abdominal Trauma
 Portal vein agenesis, atresia, stenosis
 Splenic vein thrombosis
 Biliary tract disease
 Extrahepatic biliary atresia
 Choledochal cyst

7. Pre-sinusoidal: Sinusoidal: Post sinusoidal:
 Schistosomiasis
 Congenital hepatic fibrosis
 Drugs: methotrexate, arsenic
 Parenteral nutrition
 Hepatocellular disease
 viral hepatitis (acute/chronic)
 Cirrhosis
 Wilson disease
 Alfa-1 antitrypsin deficiency
 GSD type IV
 Veno-occlusive
disease
B. Intra-hepatic:

8. C. Post-hepatic:
 Budd-Chiari Syndrome
 IVC Webs
 Chronic Constrictive Pericarditis

9. Pathogenesis And Consequence of Portal HTN
Portal HTN
Development of Collaterals
Junction between absorptive epithelium
& squamous epithelium
↑ Pressure
VARICES
Prone to
bleeding
Porto-Systemic Shunting
Abnormal mediators
reaching circulation
Development of ectasias
in stomach
Congestive
Gastropathy
Bleeding
Splenomegaly
↓ Blood counts
(Rarely)
↓ Flow to liver
Hepatocyte
dysfunction

10. Portosystemic collaterals:
Sites:
 Lower part of esophagus
 Lower part of rectum
 Around Umbilicus

11. Clinical Features:
1) Bleeding:
 Most common presentation
 risk of first bleed in cirrhosis is 22%
 rises to 38% in with known varices >5-yr period
 Pattern of bleeding
 Hematemesis/Malena: Most common
 worsened by Stress / Intercurrent illness
 Size of varices → Bleeding

12. 2) Splenomegaly:
 2nd Most common presentation
 asymptomatic or associated with cytopenia
3) Ascites:
 Seen in 7-21% patients
4) Less common but important manifestations
 Portal Hypertensive Biliopathy
 Growth Failure
 Hepatopulmonary Syndrome
 Porto-pulmonary HTN

13. Caput Medusae:
 Abnormal, dilated venous network on anterior abdominal wall, radiating from
the umbilicus
 Not seen in extra-hepatic portal HTN
 Seen in intra-hepatic portal HTN
 Continuous murmur between umbilicus and lower sternum
 Cruveilhier-Baumgarten Murmur

14. Investigations
1) USG with Doppler
 portal vein diameter > 10 mm
 hepatic diseases, masses, presence of varices and ascites
 ascertain pattern of flow
 Reversal of portal blood flow (Hepatofugal flow) - Associated with
bleeding varices
 Cavernous transformation of the portal vein in EHPVO
 Increased thickness of lesser omentum

15. 2) CECT and MRA: Needed in selective cases
3) Selective Arteriography: When surgical decompression is being planned
4) GIT Endoscopy: Most reliable to detect varices

16. Other investigations:
 CBC
 LFT
 Barium swallow
 Portal angiogram
 Percutaneous intrasplenic measurement of portal pressure
 Venography

17. A. Emergency Management of Bleeding Varices
1st Step (Initial resuscitation):
• airway protection
• Obtain I/V Access
• Restoration of IV volume: fluid and BT
 PRBC: Target Hb: 7-9 g/dL
• Correction of coagulopathy: vitamin K, FFP/PC
• NG tube: Active Bleeding
• PPI/H2 blocker: reduce bleeding from gastric erosion
• Broad spectrum Empirical I/V antibiotics

18. Drugs:
Vasopressin:
 increases splanchnic vascular tone and decreases portal blood flow
 Continuous iv infusion:
• initial: 2-5 milliunits/kg/min
• Titrate dose as required
• Max dose: 10 milliunits/kg/min
 decreased perfusion of vital organs
 Compromise of Cardiac function + ↓ blood supply to GIT
 Nitroglycerin: to reduce side effects

19. Octreotide:
 decreases splanchnic blood flow
 Dose: 1-2 mcg/kg iv bolus
• followed by continuous iv infusion of 1-2 mcg/kg/hr
• Titrate infusion rate to response
• Taper dose by 50% every 12 hr when no active bleeding occurs for 24 hrs
• Discontinue when dose is 25% of initial dose
 fewer side effects
 Better tolerated
Ref: Al-Hussaini A et. al. Therapeutic applications of octeotride in pediatric patients. Saudi J Gastroenterol.2012

20.  Endoscopic treatment:
• not responding to medical therapy
• using endoscopic band ligation
 Endoscopic sclerotherapy:
• sodium tetradecyl sulphate, polidocanol, hypertonic saline, chromated
glycerin
• Side effects: further bleeding, bacteremia, esophageal ulceration, stricture

21.  Insertion of Sangstaken-Blakemore Tube
• Compression - Stops bleeding
• Less well tolerated
• sedation
• ↑ complication
• Last Resort

22. Surgical Options in Portal HTN
Indications for surgical management
1) Recurrent variceal bleeds
2) Hypersplenism
3) Presinusoidal types (extrahepatic/ intrahepatic)

23. 1) Porta-Caval Shunt:
 Portal vein to IVC
 Reduces Pulmonary HTN but also reduces hepatic blood flow
 Can precipitate hepatic encephalopathy
Surgical treatments:

24. 2) Meso-Caval shunt
3) Distal splenorenal shunt
Reduce Portal HTN and less effects on hepatic blood flow
Difficult due to small vessel size in children

25. 4) Meso-Rex Shunt:
 Superior mesenteric vein with left portal vein
 Best for children with EHPVO

26. 4) TIPSS
 Transjugular Intrahepatic Porto-Systemic Shunt
 Portal Vein and Rt hepatic vein
 Temporary relief, increases risk of thrombosis and hepatic
encephalopathy
5) DIPS
• Direct intrahepatic portocaval shunt
• Preferred over TIPSS

27. 6) OLT: - Orthotopic Liver Transplantation

28. B. Role of Prophylactic Therapy
• Nonselective beta blocker:
 lower CO (β-blocker) and
 Lower portal pressure (β2 blockade)
o in Adolescents and Adults
o Not well-defined in children
• Treat the correctable cause

29. 1) Hypersplenism
Sequestration of RBCs, WBCs and platelets
Pancytopenia
2) Ascitis
3) Hepatorenal syndrome
4) Hepatic encephalopathy
5) Variceal bleeding
6) SBP
Complications:

30. Unfavourable prognostic signs:
 Jaundice
 Ascitis
 Hypoalbuminemia
 Encephalopathy
Prognosis:

31. EHPVO
 Most common cause
 Portal Vein replaced by cavernoma
 shunts blood across the obstruction with portosystemic collaterals
 Mostly idiopathic
 some cases related to umbilical catheterization/sepsis

32.  features of portal HTN with prominent splenomegaly
 Caput medusa: absent
 associated with poor growth and GIT bleeding
 Managed similar to other cases
 Meso-Rex shunt: best surgical procedure
 OLT may be needed

33. Budd-Chiari Syndrome (BCS)
 hepatic vein obstruction with/without occlusion of suprahepatic part of IVC
 Primary BCS: Endoluminal obstruction due to thrombus or web
 Secondary BCS: Obstruction due to lesion outside the venous system

34.  Risk factors:
 Hypercoagulable states: AT-III deficiency, Deficiency of Protein C, S
 PNH
 OCPs
 Behcet Syndrome
 IBD
 Aspergillosis
 Dacarbazine therapy
 Nephrotic Syndrome

35. Clinical Presentation:
 Acute BCS: Severe Abdominal Pain, Vomiting, Ascites, Hepatomegaly and
rapid-onset ALF
 Chronic BCS: Hepatomegaly, Portal HTN and Bleeding Varices
• In IVC block, back veins are prominent, dilated, tortuous with flow from
below upwards

36.  Doppler ultrasound and venography confirm the diagnosis
 Gold standard: Angiography
 MRA is a useful non-invasive test
 Rx:
Angioplasty/TIPSS
followed by Shunts.
OLT in ESLD

37. Sinusoidal Obstruction Syndrome
 Veno-Occlusive Disease / Stuart-Bras Syndrome
 occlusion / thrombosis of centrilobular venules or sublobular hepatic veins
 Seen in BMT patients after radiation/cytotoxic therapy
 associated with Azathioprine, 6-MP and Pyrrolizidine Alkaloids

38.  Clinically behaves similar to BCS
 needs liver biopsy for definite diagnosis
 Rx:
Supportive
Shunts
OLT in non-responding cases

39.  Most common presentation : UGI bleeding
 Hematemesis or malena:
• rupture of esophageal varices,
• portal gastropathy,
• gastric antral ectasias or stomal,
• intestinal or anorectal varices
Approach to a case of Portal Hypertension:

40. Jaundice:
 Waxing and waning:
• choledochal cyst, chronic active hepatitis, cholestasis
 Progressive:
• intrahepatic causes, malignancy
 Absent:
• extrahepatic portal vein obstruction

41. Age:
 Neonatal: EHBA, alpha-1 antitrypsin deficiency
 Infancy: Cystic fibrosis, GSD type IV, choledochal cysts, alagille
syndrome
 Childhood: alpha-1 antitrypsin deficiency, congenital hepatic fibrosis,
Wilson disease, choledochal cyst, alagille syndrome

42.  Pruritus:
• sclerosing cholangitis and other causes of obstructive jaundice
 Colour of urine and stool:
• persistently acholic stool and dark urine is suggestive of obstructive jaundice
 Abdominal distension:
• ascites or tumor
 Fever:
• cholangitis, chronic hepatic fibrosis, viral hepatitis, sepsis due to gram negative
bacteria in CLD

43.  Pain abdomen:
• upper right quadrant
• dull aching type
• generalized in case of peritonitis
 Mass per abdomen: choledochal cyst
 Vomiting:
• viral hepatitis, ingestion of hepatotoxins, inborn errors of metabolism
 Steatorrhea
 History of blood transfusion

44.  Features suggestive of IBD
 Hypercoagulable state: calf pain, seizures, blurring of vision
 History of recurrent genital or oral ulcerations: Behcet’s syndrome
 Pinched facies with sallow complexion: cirrhosis
 Dysmorphic face: Alagille syndrome

45.  Growth retardation: cirrhosis
 KF ring: Wilson disease
 Peripheral stigmata of cirrhosis:
• alopecia, parotid swelling, fetor hepaticus, gynecomastia, spider angioma,
palmar erythema, testicular atrophy, flapping tremors

46.  Hepatomegaly:
• congenital hepatic fibrosis (hard liver with minimal hepatic dysfunction),
biliary atresia, budd chiari syndrome, choledochal cyst
• Consistency of liver is more significant than size of liver
o Normal, soft or small liver: extrahepatic portal vein obstruction
o Firm nodular and decreased vertical span: cirrhosis

47.  Splenomegaly
• most important diagnostic sign
• size of spleen does not correlate with the height of portal pressure and
size varies with the age of patient

48.  Dilated abdominal veins:
• periumbilical veins: intrahepatic portal hypertension

49.  Ascites:
• present in sinusoidal/post sinusoidal
• Portosystemic hepatic encephalopathy and ALF
 Hematological changes: thrombocytopenia, anemia
 Portopulmonary hypertension

50.  Hepatorenal syndrome:
• renal insufficieny in patients with severe liver failure in absence of any
other cause of renal pathology
 Hepatopulmonary syndrome:
• triad of:
o portal hypertension,
o intrapulmonary vascular dilation and
o arterial hypoxemia (PaO2 <70 mmHg) in absence of primary
cardiopulmonary disease

51. Parameters EHPVO Cirrhosis HVOO (BCS)
Age (mean) Children and adults All ages All ages
GI bleeding ++ + +/-
Ascites 5-10% ++ +++
Pedal edema - ++ +++
Encephalopathy - + +/-
Spleen ++ + +
Liver Normal to small Decreased vol/ firm, nodular Enlarged/firm/nodular
Ant abdominal veins -/few lumbar veins ++ +++ back veins
Total protein/A:G ratio Normal Total protein decreased,
globulin increased
Total protein decreased,
globulin increased
US PV thrombosis, cavernoma,
collaterals, splenomegaly
Liver coarse echotexure,
collaterals, dilated portal
vein, ascites, splenomegaly
Liver enlarged, hepatic vein
fibrosis or IVC obstruction
Liver biopsy Normal Necrosis, nodules, fibrosis Centrilobular necrosis,
fibrosis, reversed lobulation

52. 1. CBC:
 anemia due to chronic disease or acute blood loss
 pancytopenia due to hypersplenism
2. LFT:
 AST and ALT: 1000 fold rise in acute hepatocellular injury due to viral
hepatitis, drugs or toxin induced, shock hypoxemia, metabolic disease
 AST and ALT elevations less marked in case of CLD, biliary obstruction
 In cholestasis: TSB and DSB elevated, ALP, 5’nucleotidase, GGT increases
 Acute hepatitis: rise in ALT > AST
 AST elevation > ALT in alcohol mediated injury, fulminant echo virus infection
and metabolic disease
Investigations:

53. 3. Viral markers:
 HBsAg, anti HCV, IgM anti HAV, IgM anti HEV
4. Metabolic screening:
 serum ceruloplasmin, 24 hour urinary copper excretion
5. UGI endoscopy:
 detects esophageal varices, varices usually appear white and opaque
6. Doppler USG of abdomen:
 patency of portal vein, direction of flow

54. 7. Liver biopsy :
 determine the precise histologic diagnosis,
 for enzyme analysis in case of inborn error of metabolism and
 analysis of stored materials e.g. Iron, copper
8. Hepatic scintigraphy
9. Cholangiography
10. Contrast CT and MR angiography

55. 1. Emergency management of variceal bleeding
 Initial resuscitation
 Restoration of IV volume
 Correction of coagulopathy
 PPI/H2 blocker
Treatment:

56.  Drugs:
• Vasopression
• Octeotride
• Nonselective beta blocker
 Endoscopic treatment
 Sengstaken blackmore tube

57. 2. Surgical procedures:
 Portocaval shunt
 Mesocaval shunt
 Rex shunt
 Trans-jugular intrahepatic portosystemic shunt

58. 3) Prophylaxis to prevent subsequent bleeding (medical, endoscopic, surgical)
4) Management of ascites
5) Salt restriction, diuresis, V2 receptor antagonists
6) Therapeutic paracentesis (for each liter of fluid removed 5 gm albumin to
be transfused)
7) Orthotopic liver transplantation

59.  Nelson Textbook of Pediatrics: 21st Edition
 Ghai Essentials of Prdiatrics: 9th Edition
 Clinical Pediatrics, Aruchamy Lakshmanaswamy: 3rd Edition
 Gray’s Anatomy, International Edition
 Al-Hussaini A et. al. Therapeutic applications of octeotride in pediatric
patients. Saudi J Gastroenterol.2012
References:

60. THANK YOU