portal hypertension paediatrics

By Dr Liza Bulsara

Under Guidance of :-Dr. Sunil Mhaske sir (Professor & Head)

In Presence of :-Dr. Kothari sir (Associate Professor)And all residents.

smalathi 2

S.GL.G

Portal venous System

Portal HypertensionNormal range of portal venous pressure is 5

to 10 mm of Hg or 10-15 cm saline above the pressure present in the IVC.

Portal hypertension is defined as elevation of this pressure gradient to values above 10 to 12 mm Hg.

smalathi 3

Portal HypertensionDefinition:PHT is a pathologic increase in

portal pressure in which the pressure gradient between the portal vein and the IVC (Portal pressure gradient or PPG)is increased above the upper limit of 10 mm of Hg.

PPG > 10 mmHg(varices)PPG > 12mmHg(variceal bleed,ascites)PPG > 6 to 10 mmHg(subclinical PHT)

smalathi 4

Features Normal(mm hg)

portal hypertension

Portal venous pressure

7-10 >12

Intrasplenic pressure

<17 >17

Wedge hepatic pressure

<6 >6

smalathi 5

Classification of PHT

Pre Sinusoidal:Extrahepatic(1) Intrahepatic(2)

Sinusoidal(3)

Post sinusoidal:intrahepatic(4) extrahepatic(5)

smalathi 6

1

2 34 5

Pathophysiology of PHCirrhosis results in scarring

(perisinusoidal deposition of collagen)Scarring narrows and compresses hepatic

sinusoids (fibrosis)Progressive increase in resistance to

portal venous blood flow results in PHPortal vein thrombosis, or hepatic venous

obstruction also cause PH by increasing the resistance to portal blood flow

Pathophysiology of PHAs pressure increases, blood flow decreases

and the pressure in the portal system is transmitted to its branches

Results in dilation of venous tributariesIncreased blood flow through collaterals and

subsequently increased venous return cause an increase in cardiac output and total blood volume and a decrease in systemic vascular resistance

With progression of disease, blood pressure usually falls

smalathi 9

Portal Vein CollateralsCoronary vein and short gastric veins -> veins of the

lesser curve of the stomach and the esophagus, leading to the formation of varices

Inferior mesenteric vein -> rectal branches which, when distended, form hemorrhoids

Umbilical vein ->epigastric venous system around the umbilicus (caput medusae)

Retroperitoneal collaterals ->gastrointestinal veins through the bare areas of the liver

Posterior abdominal wall:veins of posterior abdominal wall,subdiaphragmatic veins,retroperitoneal veins.

Splenorenal collateralsSplenoperitoneal collaterals.

Etiology of PH

Causes of PH can be divided into1. Pre-hepatic2. Intra-hepatic3. Post-hepatic

Pre-hepatic PH

Caused by obstruction to blood flow at the level of portal vein

Examples: congenital atresia, extrinsic compression, s, portal, superior mesenteric, or splenic vein thrombosis,cirrohsis.

Intrahepatic:Infections:cirrohsis due to hepatitis B,C.Metabolic disorders: wilson’s diseases.Congenitial hepatic fibrosisChronic active hepatitisChronic myeloid luekemiaLymphomaSarcoidosisLuekemic infiltrationToxins:arsenic,vinyl chloride,hyperviayminosis A.

smalathi 13

Post-hepatic

Caused by obstruction to blood flow at the level of hepatic vein

Examples: Budd-Chiari syndrome, chronic heart failure, constrictive pericarditis, vena cava webs

Budd-Chiari SyndromeCaused by hepatic venous obstructionAt the level of the inferior vena cava, the

hepatic veins, or the central veins within the liver itself

result of congenital webs , acute or chronic thrombosis and malignancy

Budd-Chiari SyndromeAcute symptoms include hepatomegaly, RUQ

abdominal pain, nausea, vomiting, ascitesChronic form present with the sequelae of

cirrhosis and portal hypertension, including variceal bleeding, ascites, spontaneous bacterial peritonitis, fatigue, and encephalopathy

Diagnosis is most often made by US evaluation of the liver and its vasculature

Cross-sectional imaging using contrast-enhanced CT or MRI

Budd-Chiari SyndromeGold standard for the diagnosis has been

angiographyManagement has traditionally been surgical

intervention (surgical decompression with a side-to-side portosystemic shunt)

Minimally invasive treatment using TIPS may be first-line therapy now

Response rates to medical therapy are poor

Portal Vein ThrombosisMost common cause in children (fewer

than 10% of adult pts.)Normal liver function and not as

susceptible to the development of complications, such as encephalopathy

Diagnosis by sonography, CT and MRIOften, the initial manifestation of portal

vein thrombosis is variceal bleeding in a noncirrhotic patient with normal liver function

Portal Vein Thrombosis - CausesUmbilical vein infection (the most common

cause in children)Coagulopathies (protein C and antithrombin

III deficiency),Hepatic malignancy, myeloproliferative

disordersInflammatory bowel diseasepancreatitistraumaMost cases in adults are idiopathic

Portal Vein ThrombosisTherapeutic options are esophageal variceal

ligation and sclerotherapyDistal splenorenal shunt Rex shunt in patients whose intrahepatic

portal vein is patent (most commonly children)

Splenic Vein ThrombosisMost often caused by disorders of the

pancreas (acute and chronic pancreatitis, trauma, pancreatic malignancy, and pseudocysts)

Related to the location of the splenic vein Gastric varices are present in 80% of patientsOccurs in the setting of normal liver functionReadily cured with splenectomy (variceal

hemorrhage), although observation for asymptomatic patients is acceptable.

Clinical Evaluation

• Splenomegaly • Abdominal veins •Ascites

• Varices

Ascites:

smalathi 23

Pot belly & smiling umbilicus:

smalathi 24

Distended engorged veins:

smalathi 25

smalathi 26

• Splenomegaly

• Ascites• PS Collaterals – abd. veins &

varices

• Hyper dynamic circulation

• Porto Systemic Encephalopathy

I Evaluation of PHT: type and consequences of PHT

II Evaluate: Physical signs of chronic liver disease

smalathi 28

III Evaluate: Presence of PSE•Neuropsychological tests

•Asterixis

•Foetor Hepaticus

smalathi 29

• An enlarged spleen is the single most important diagnostic sign of PHT

•Does not correlate with height of portal pressure, size of varices or age of pt.

•Correlates with type of PHT (*NCPF 12cm, * * EHPVO 6cm)

•Spleen may not be palpable soon after a bleed

Splenomegaly

smalathi 30

Dilated Abdominal Veins• Presence supports the diagnosis of PHT (Cirrhosis, BCS)

• Absence does not exclude PHT (EHPVO)

• Periumbilical veins indicate intrahep PHT, (murmur – Cruvellier Baumgarten)

• Back veins – indicates HVOO (Classical BCS/IVC)

smalathi 31

Jean cruhveiller & paul caumen von baumgarten:

smalathi 32

Dilated Veins

smalathi 33

Ascites• Presence of ascites supports diag of PHT

• Present in sinusoidal/post-sinusoidal

• Sudden accumulation of ascites – HVOO

• “Frog belly” – IVC obstruction

• Ascites in EHPVO (0-36%), NCPF (5-10%) transient

smalathi 34

• Consistency more significant than size• Size correlates poorly with height of pp. • Normal, soft or small liver EHPVO• Firm, nodular , vertical span or

enlarged,L .lobe palpable- cirrhosis• Left lobe liver enlarged - CHF• Firm liver – NCPF (10-15% nodular)

Liver Size & Consistency

smalathi 35

Presentation:GI Bleed• GI Bleed usually is the first

presentation in EHPVO/NCPF.

• Bleeds well tolerated in presinusoidal PHT.

• Bleeds occur night / morning (Peaks at 10 P.M, 9A.M).

• Mortality following variceal bleed in cirrhosis 20% to 30%.

smalathi 36

Porto Systemic Hepatic Encephalopathy• Minimal Encephalopathy (>50%)• Recurrent • Persistent• Acute

All 4 forms seen in cirrhosis. In NCPF / EHPVO, this may follow GI bleed but majority recover

smalathi 37

• Hypersplenism

• Thrombocytopenia - NCPF > EHPVO > Cirrhosis

• Anemia

• Anemia could also be secondary to GI Bleed

Hematological changes

smalathi 38

Clinical Features• Growth Retardation – Resistance to the action

of growth hormone (EHPVO)

• Portopulmonary hypertension – non-embolic pulmonary vasoconstriction in the presence of PHT. (4% of cirrhosis, 9% of NCPF))

.

• Hepatorenal syndrome – renal insufficiency in patients with severe liver failure in the absence of any other cause of renal pathology (cirrhosis).

smalathi 39

Clinical Features•Hepato pulmonary syndrome – triad of PHT, intrapulmonary vascular dilatation and arterial hypoxemia (PaO2 < 70mm of Hg) In the absence of primary cardio pulmonary disease. (17.5% cirrhotics, 13.3% NCPF, 10% EHPVO) Anand A C IJ Gastro 2001.

•Foetor Hepaticus – results from porto systemic shunting of blood, allows mercaptans to pass directly to the lungs.

•Portal Biliopathy

smalathi 40

Evaluation of various forms of portal hypertension Parameter EHPVO NCPF Cirrhosis HVOOMean age (years)

Children & occ. adults

18-25 All ages All ages

GI Bleed ++ Well tolerated

++ Well tolerated

+ + / -

Ascites 5% - 10% 5% - 10% + + + + +Pedal oedema - - ++ +++Encephalopathy - - + + / -Spleen + + + ++ + +Liver Normal or

Small volume

Firm Decreased vol / firm / nodular

Enlarged / firm / nodular

Anterior Abdominal Veins

- / few veins on lumbar region

+ / - ++ + + + Back vein

T. Protein A/G ratio

Normal Normal T.P decreased Glob increased

T P decreased Glob increased (Chronic)

US PV thrombosis Cavernoma CollateralsSplenomegaly

Patent dilated PV splenomegaly collaterals

Liver coarse echoes Collaterals dilated PVascitesSplenomegaly

Liver enlarged Hepatic vein thrombosis or IVC obstruction

Liver biopsy

Normal Normal / Peri Portal fibrosis

Necrosis, nodules fibrosis

Centrilobular necrosis, fibrosis Reversed lobulation

Features EHPVO NCPF CIRRHOSIS HVOO

Diagnosis of PHTClinical

Upper GI Endoscopy

Ultrasound/Doppler

smalathi 42

Complications of PH

GI bleeding due to gastric and esophageal varices

AscitesHepatic encephalopathy

VaricesMost life threatening complication is

bleeding from esophageal varicesDistal 5 cm of esophagusUsually the portal vein-hepatic vein pressure

gradient >12 mm HgBleeding occurs in 25-35% of pts. With

varices and risk is highest in 1st yr.

Prevention of VaricesPrimary prophylaxis: prevent 1st episode of

bleedingSecondary prophylaxis: prevent recurrent

episodes of bleedingInclude control of underlying cause of

cirrhosis and pharmacological, surgical interventions to lower portal pressure

Prevention of VaricesBeta blockade: Beta blockade (Nadolol,

Propranolol)Nitrates:Organic nitratesSurgery: No longer performed*

Endoscopy: Sclerotherapy (no longer used*) and variceal ligation

* Refers to primary prophylaxis

Treatment of Varices Initial Management:

1. Airway control2. Hemodynamic monitoring3. Placement of large bore IV lines4. Full lab investigation (Hct, Coags, LFTs,)5. Administration of blood products6. ICU monitoring

Pharmacologic Treatment of VaricesDecreases the rate of bleedingEnhances the endoscopic ability to

visualize the site of bleedingVasopressin - potent splanchnic

vasoconstrictor; decreases portal venous blood flow and pressure

Somatostatin: decrease splanchnic blood flow indirectly; fewer side effects

Octreotide: Initial drug of choice for acute variceal bleeding

Endoscopic Therapy for VaricesEndoscopic Sclerotherapy: complications

occur in 10-30% and include fever, retrosternal chest pain, dysphagia, perforation

Endoscopic variceal ligation: becoming the initial intervention of choice; success rates range from 80-100%

Balloon TamponadeSengstaken-Blakemore tubeMinnesota tubeAlternative therapy for pts. who fail

pharmacologic or endoscopic therapyComplications: aspiration, perforation,

necrosisLimited to 24 hrs; works in 70-80%

TIPSTransjugular intrahepatic

portasystemic shunt1st line treatment for bleeding

esophageal varices when earlier-mentioned methods fail

Success rates 90-100%Significant complication is hepatic

encephalopathy

Surgical InterventionLiver transplantation: only definitive

procedure for PH caused by cirrhosisShunts

Totally diverting (end-side portacaval)Partially diverting (side-side portacaval)Selective (distal splenorenal shunt)

Devascularization