Approach to a child with Portal Hhpertension

1. in Children
• Dr.C.S.N.Vittal

2. Portal Hypertension - Objectives
 To know the Etiopathology
 To study types
 Clinical features
 Diagnosis
 Management

4. Portal Hypertension
• Textbook of Pediatric Gastroenterology, Stefano Guandalini
 Normal portal pressure: 5 and 10 mm Hg (Avg 7 mm Hg)
 Portal hypertension is an increase in portal pressure of
> 10 mm Hg

5. Alternate Definitions
Elevation of Pressure Gradient
Between Portal Vein and IVC
(PPG) to ABOVE 10-12 mm of Hg
or 30 cm of Saline (normal =7 mm
of Hg)
• PPG > 10 mm Hg (varices)
• PPG >12 mm Hg (variceal bleed, ascites)
• PPG > 6 to 10 mm Hg (subclinical PHT)
• Intrasplenic pressure > 17
mm Hg
OR
• Hepatic vein pressure
gradient (HVPG) > 4 mm Hg
OR

6. • Cong hepatic fibrosis
• Liver cirrhosis
• Non cirrhotic portal
fibrosis (NCPF)
• Non-alcoholic fatty liver
disease
• Nodular regenerative
hyperplasia
• Schistosomiasis
• Glycogen storage
disease type IV
• Wilson disease
• Portal vein agenesis,
atresia, stenosis
• Portal venous
thrombosis or
cavernous
transformation
• Splenic vein thrombosis
• A-V fistula
• Budd Chiari
Syndrome (Hepatic vein
or IVC obstruction)
• Constrictive
pericarditis
• Veno-occlusive
disease
• Busulfan induced
• Hypercoagulable
states
Prehepatic Intrahepatic Posthepatic
Classification of Portal Hypertension

7. Pathophysiology
Increased
resistance
to portal
blood flow
Liver cell
injury
Stellate
cells –
change to
myofibrobl
asts
Expression of
specific smooth
muscle protein
a-actin,
Endothelin,
NO,
Prostaglandins
Fibrogenesis
Increased
resistance
leads to
portal
hypertension
Porto -
systemic
shunts

8. Normal venous flow through the portal and
systemic circulation.
Redirection of flow through the left gastric vein
secondary to portal hypertension or portal venous
occlusion.
Portal Hypertension Pathophysiology

9. CLINICAL FEATURES
 Upper GI Bleeding
 Hematemesis
 Esophageal, gastric and
 Portal gastropathy with bleeding
 Recurrent epistaxis, easy bruising
 Lower GI Bleeding,
 Rectal varices with bleeding
 Malena
 Ascites
 Malabsorption
 Protein-losing enteropathy
 Growth failure
 Anemia, thrombocytopenia
 Hepatosplenomegaly
 Hepatic encephalopathy

10. CLINICAL FEATURES OF
INTRAHEPATIC
PORTAL HYPERTENSION
JAUNDICE
PALMAR ERYTHEMA
CLUBBING
RECTAL VARICES
SPIDER ANGIOMA
ASCITES WITH DILATED
ANT. ABDOMINAL VEINS
GYNAECOMASTIA

11. Presence of PSE
(Portosystemic encephalopathy)
4 forms
1. Minimal Encephalopathy (> 50%)
2. Recurrent
3. Persistent
4. Acute
 Neuropsychological tests +ve
 Asterixis
 Fetor Hepaticus

12. Diagnosis
 Clinical
 Upper GI Endoscopy
 Ultrasound/Doppler
 Other Labs

13. Diagnosis - GI Endoscopy
 Upper GI Endoscopy
 Can reveal varices in
esophagus, stomach and
congestive gastropathy
 Colonoscopy
 Useful in children with lower
GI bleeding as it can show
presence of rectal varices or
polyps
Esophageal varices

14. Diagnosis - Imaging
 Ultrasound and Doppler study,
 Any block in portal, splenic or hepatic veins can be
detected,
 Increased size of portal vein is suggestive of IHPA
 Presence of collaterals, ascites, splenomegaly and
liver abnormalities (altered echotexture, size and
space occupying lesions) are also seen.
 Cirrhosis: Portal Vein dilated >10mm
 Loss of respiratory variation in flow
 Decreased flow velocity or bidirectional flow in portal
vein
Selective CT and MR
portovenography
useful for delineation
of vascular anatomy.

15. Diagnosis – Other Labs
 Liver function tests – in cirrhosis patients
 Hemogram – anemia, leukopenia and thrombocytopenia – suggest
hypersplenism
 Circulating endothelial cells (CECs): markers of vascular injury:

16. Diagnosis – Newer Serological Tests
 Serum laminin levels correlate with HVPG
 Serum levels of soluble vascular adhesion molecule (SVCAM-1): marker of
the hyperkinetic circulation
 Serum endothelin-1 (ET-1): elevated in portopulmonary hypertension and
associated with a poor outcome
• Urotensin II (U-II): a somatostatin-like cyclic peptide important marker of the
severity of PHT in children with chronic liver disease
• correlated with Child-Pugh score, paediatric end-stage liver disease score,
and long-term clinical outcome
• Von Willebrand factor (vWF), p-selectin, and 8-iso-PGF2a:
• surrogate markers of endothelial dysfunction and levels increased

17. Complications
 GI bleeding secondary to esophageal varices
 Hypersplenism - prone to splenic infarcts and accidental rupture with
trauma
 Ascites
 Hepatic encephalopathy
 Portal hypertensive biliopathy:
 Portal vein obstruction occurs as a result of external compression of the bile
ducts by cavernous transformation of the portal vein

18. Complications
 Renal Dysfunction –
 HRS 1: associated with rapid kidney failure and an overproduction of
creatinine.
 HRS 2: associated with more gradual kidney damage. Symptoms are
generally subtler.
 Portopulmonary hypertension (PP-HTN)
 Pulmonary arterial pressure > 25 mm Hg at rest or a left-ventricular end-diastolic
pressure of <15 mm Hg.
 Hepatic encephalopathy (HE)
 Hepatocellular carcinoma (HCC)

19. Hepatopulmonary syndrome (HPS)
in ≥10% of patients
 Release of a number of endogenous vasoactive molecules, including
endothelin-1 and nitric oxide into the venous circulation
 Present with dyspnoea, cyanosis, clubbing, and spider nevi
 Platypnea (dyspnea induced in upright position and relieved by recumbency)
 Orthodeoxia (arterial deoxygenation accentuated in upright position and relieved by
recumbency).
 Triad of hepatopulmonary syndrome.
1. Chronic liver disease or portal hypertension,
2. Alteration of arterial oxygenation (widened age corrected alveolar arterial oxygen
gradient with or without arterial hypoxemia)
3. Evidence of intrapulmonary vascular dilatations

20. Management
 Treatment of life threatening hemorrhage
 Fluid resuscitation with crystalloids and RBC replacement
 Correction of coagulopathy – Vitamin K / FFP / platelet infusion
 H2 blockers of proton pump inhibitors – to reduce risk of bleeding from
gastric erosions
 To reduce portal pressure with continued bleeding
 Vasopressin
 Nitroglycerine
 Somatostatin analog – octreotide
 Endoscopic sclerosis or
 Endoscopic elastic band ligation of esophageal varices

21. Endoscopic Sclerotherapy

22. TREATMENT contd.
If bleeding still persists
• Sengstaken-Blakemore tube - stops hemorrhage by mechanically
compressing esophageal and gastric varices
• TIPS(Trans jugular Intra hepatic Porto Systemic Shunt)
 Shunt surgeries- portosystemic shunts to divert portal blood and
decrease portal pressure –portocaval, mesocaval, splenorenal shunts

24. Endoscopic Procedures
 Endoscopic Sclerotherapy
 Intra and paravariceal injection of sclerosant –
causes thrombosis & obliteration of vessels.
 Common Complications- bleeding,
oesophageal ulcerations & stricture,
perforation, bacteraemia.
 Endoscopic variceal ligation
 Better option than sclerotherapy.

25. BALLOON TAMPONADE - Sengstaken Blakemore Tube
A tube made of 3 smaller tubes with 2
balloon located distally for the
oesophagus and the stomach, 3
proximal and 1 distal opening
Complications
 Asphyxia by pressure of the
oesophageal balloon on the trachea
 A ruptured balloon can set of an air
embolism

27. PREVENTION OF VARICEAL BLEED
 Long term Tt by nonselective ß- blocker
(↓ cardiac output , lowers portal perfusion, cause splanchnic vasoconstriction)
 Propranolol (0.5- 2 mg/kg 3-4 div. doses titrated upwards till 25% ↓ in HR from
baseline and ↓ BP by 15 mm Hg )

28.  Dr.C.S.N.Vittal