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Portal hypertension paediatrics
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Transcript of 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.
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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.
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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)
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Features Normal(mm hg)
portal hypertension
Portal venous pressure
7-10 >12
Intrasplenic pressure
<17 >17
Wedge hepatic pressure
<6 >6
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Classification of PHT
Pre Sinusoidal:Extrahepatic(1) Intrahepatic(2)
Sinusoidal(3)
Post sinusoidal:intrahepatic(4) extrahepatic(5)
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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
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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.
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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:
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Pot belly & smiling umbilicus:
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Distended engorged veins:
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• 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
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III Evaluate: Presence of PSE•Neuropsychological tests
•Asterixis
•Foetor Hepaticus
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• 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
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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)
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Jean cruhveiller & paul caumen von baumgarten:
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Dilated Veins
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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
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• 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
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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%.
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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
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• Hypersplenism
• Thrombocytopenia - NCPF > EHPVO > Cirrhosis
• Anemia
• Anemia could also be secondary to GI Bleed
Hematological changes
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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).
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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
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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
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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