Escolar Documentos
Profissional Documentos
Cultura Documentos
and
portal hypertension
in Pediatrics
Overview
• INTRODUCTION
• ANATOMY
• ETIOLOGY/CLASSIFICATION
• CLINICAL MANIFESTATION
• DIAGNOSIS
• TREATMENT
• SUMMARY
• Portal hypertension, defined as an elevation of
portal pressure >10-12 mm Hg(normal value
being 7 mmHg).
10 mmHg(varices)
12mmHg(variceal bleed,ascites)
Anatomy
• Dual blood supply
Portal vein 75 % and Hepatic artery 25%
• Portal circulation
• high compliance,
low-resistance
system
• SMA and Splenic vein
• IMA
• Coronary vein
• Umbilical vein
• The portal vein enters the liver at the porta
hepatis in two main branches, one to each
lobe and has a segmental intrahepatic
distribution, accompanying the hepatic artery.
• Portal hypertension occurs as a result of
increased portal resistance, increased portal
blood flow, or both.
Hepatic Venous Pressure Gradient (HVPG)
• Prehepatic
• Intrahepatic
• Post-hepatic
EHPVO
• Infections
• Hypercoagulable states
• Trauma
• Invasion and compression
• Intrahepatic causes of PH
• Idiopathic
Intrahepatic Causes of PH
• Noncirrhotic portal fibrosis
– obliterative portal venopathy resulting in PH, well
tolerated episodes of variceal bleeding and preserved
liver function.
• Chronic liver disease
– Cirrhosis and chronic hepatitis.
– The major cause of cirrhosis in children are viral
hepatitis, neonatal cholestasis syndrome and
metabolic liver diseases like Wilson’s disease, glycogen
storage disease, α1 antitrypsin deficiency, etc.
Post sinusoidal block
• Veno-occlusive disease (VOD) or endophlebitis
obliterans
(1) Plant alkaloids,
(2) Irradition,
(3) Drug-immunosuppresants,
antineoplastics and indigenous system of
therapy
• Budd-Chiari syndrome or hepatic venous
outflow tract obstruction (HVOO
• In patients with underlying hepatic disease,
physical examination might show jaundice and
stigmata of cirrhosis such as palmar erythema
and vascular telangiectasias.
EPHVO
• Baveno V workshop consensus statement defined
EHPVO as (De Franchis, 2010):
– EHPVO is defined by obstruction of the extra-hepatic
portal vein with or without involvement of the intra-
hepatic portal veins and does not include isolated
thrombosis of splenic vein or superior mesenteric vein
(SMV).
– EHPVO is characterized by features of recent
thrombosis or of portal hypertension with portal
cavernoma as a sequel of portal vein obstruction
– Presence of cirrhosis and/or malignancy should be
stated.
Epidemiology
• EHPVO is the most common cause of upper
gastrointestinal bleeding in children.
• EHPVO is responsible for 54% of portal
hypertension.
• Most (85-92%) of the upper gastrointestinal
bleeding in Indian children was result of portal
hypertension due to EHPVO
Etiology
• Infection
• Trauma and surgery
• Congenital anomaly
• Prothrombotic state
• Idiopathic
Pathogenesis
• Portal vein changes
– usually the entire length of the portal vein is
occluded with extension into the splenic vein and
sometimes into the superior mesenteric vein.]
– On gross examination, the original portal vein is
difficult to identify as it is usually replaced by a
cluster of variable-sized vessels arranged
haphazardly within a connective tissue support,
called as the Portal Cavernoma.
– intrahepatic venous pressure is normal
– intrasplenic pressure is increased.
– The hepatic blood flow is relatively normal
– hyperkinetic circulatory state
• Liver changes
– liver is normal and the architectural pattern is
preserved.
Clinical presentation
• Recent Or Acute And Chronic
Recent or acute EHPVO
• This can present with abdominal pain, ascites or fever.
• there is no evidence of porto-systemic collaterals and portal
cavernoma.
• The extent of obstruction in portal vein and speed of
evolution of thrombosis predicts the clinical manifestation.
• If associated with infection, features of sepsis may be there.
• may be passed as episodes of acute abdomen or sepsis.
Chronic EHPVO
• typical presenting symptoms like
– episodes of variceal or gastrointestinal bleeding,
– lump in the abdomen and
– hypersplenism.
• EHPVO in childhood is most often chronic and
presents with features of variceal bleeding
and splenomegaly, whereas in adults it could
be either acute or chronic.
Natural history of EHPVO
• Recurrent well tolerated major GI bleeds.
Endosopy-
EST,EVL.
Surgery
smalathi
Management of EHPVO
• Without bleed
• Following bleed.
EHPVO :without bleed.
• Medical:Primary prophylaxis:Non selective
B blockers 1 mg/kg/day.
• Medical:Resuscitation,vasoactives,vasodil
a tors.
• Octreotide and somatostatin
recommended.
• Endoscopic:Endoscopic sclerotherapy
or Endoscopic variceal ligation.
EHPVO following bleed.
• Medical:Continue b blockers.
• Surgery:Shunt procedures
Indications for surgery in EHPVO
• Devascularisation and
decompression surgeries
• Failure of EST.
• Hypersplenism.
• Child living in remote areas or with rare
blood groups.
• ?Growth retardation.
Reasons for not advocating early
surgery in EHPVO
• Natural history of the disease.