Cirrhosis of liver and

Portal Hypertension

Dr Manoj K Ghoda M.D., M.R.C.P.

Consultant Gastroenterologist

Visiting faculty, GCS hospital

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gujarat gastro group

35 yrs old Female

Presented with hematemesis

No p.h/o any illnessNo h/o drugs like NSAIDs

O/E : Pallor ++No jaundiceNo edema or

ascitesP/A: Liver not palpable

Spleen ++Visible veins on abdominal wall

CBC: normochromic normocytic anemia

LFT: Bil: 0.8 mg ALT: 27 i.u. ALP: 123 i.u.S. Alb.: 2.9S.Glob: 3.2PT: 18/15 secCreat: 1.0 mgWhat are the common causes of GI bleed in India?What will you do now?

In Pediatric pts

• Variceal bleed• Ulcers

Common causes of UGI bleed in India

In adults

• Ulcers, including NSAID induced

• Variceal bleed• Mellory-Weiss tear• Dieulafoy lesion.

What would do you now?

Irrespective of etiology first management step is to stabilize the patient

A: Airway protectionB: BreathingC: Circulation

Your next step?

Next step is to establish the cause of bleeding

Only UGI endoscopy could establish the cause of bleeding, even when there is established portal HT as nearly 3rd of patients with portal HT could bleed from other causes, mainly peptic ulcers.

While waiting to optimize the patient for endoscopy you may want to obtain evidence of portal HT by doing USG

• What is portal hypertension ?

• How does it present ?• How do we diagnose and manage it ?

Superior mesenteric vein

Splenic vein

Portal vein is not a true vein

20 %

80 %

Blood supply to Liver

Measuring Portal vein pressure

HEPATIC VEIN PRESSURE GRADIENTThe HVPG is the difference between the wedged hepatic venous pressure (WHVP) and free hepatic vein pressure (FHVP). The HVPG has been used to assess portal hypertension since its first description in 1951,and has been validated as the best predictor for the development of complications of portal hypertension.

Normally, the HVPG does not exceed 5 mm Hg. Portal hypertension exists if the HVPG exceeds this value.

Etiology of portal HT

EHPO

Splenic vein thrombosis

Non Cirrhotic Portal Fibrosis

Hepatic- Cirrhosis of Liver

Cirrhosis of Liver:

It is a group of signs and symptoms resulting from altered architecture of the liver resulting from unregulated fibrosis in the liver.

It is more of a pathological diagnosis than clinical one as most, if not all the signs and symptoms produced by cirrhosis may also be found in chronic liver diseases without cirrhosis.

Pathology and pathogenesis: Because of repeated insults to hepatic parenchyma and specific promotion of fibrogenesis in certain conditions, there is unregulated fibrosis.

Macroscopically, The liver progressively shrinks in size and nodular cirrhosis develops with occasional malignant transformation..Because of fibrosis, there is a loss of elasticity and the liver becomes hard.Associated with these, there is progressive portal hypertension.Microscopically,There is widespread fibrosis in the liver, which may encircle the lobule to form distinct nodules.Vascular arrangement of portal vein and hepatic vein radicals is disturbed in such a way that there is a mismatch.Telltale sign of the underlying pathology like hepatitis B or alcoholic hepatitis may be present.Malignant transformation may be seen.

Clinical features of Cirrhosis: They vary according to the stage of liver involvement but it is not uncommon to have practically no specific symptoms or signs even in advanced cirrhosis and it is not uncommon to see the patient presenting for the first time with one of its complication like GI bleed or ascites or encephalopathy.Tiredness, lethargy, depression may be present.Jaundice may be present. The skin of the exposed part may become black.There may be ascites and ankle edema.There may be spider nevi Liver palms, an erythematous skin in the periphery of the palms may also be seen.Gynecomastia and soft testis in males, together with reduced pubic hair may be seen because of failure to metabolise estrogen.There may be muscle wasting.Umbilical hernia and visible veins radiating from umbilicus or on the flanks with flow away from the centre may be seen.Liver may be enlarged in early cirrhosis and spleen may be enlarged, sometimes grossly.Flapping tremors and signs of encephalopathy may be present.Esophageal, gastric and rectal varices may be seen at endoscopy.

Grading of Cirrhosis

Management:It is two fold; supportive and specific.

Supportive include use of salt and fluid restriction, high protein diet and albumin supplement, use of diuretics, frusemide up to 160 mg. and aldesterone antagonists Spironolactone up to 400 mg. for edema. Non selective B-blockers, like propranolol may be used for reducing portal hypertension. Lactulose may be used for relieving constipation.

Specific treatment for encephalopathy, hepatorenal syndrome, Hepatopulmonary syndrome and GI bleed .Specific treatment for viral hepatitides, Wilson’s, autoimmune hepatitis and hemochromatosis as appropriate.In end sage cirrhosis liver transplant is the treatment of choice.

Prognosis:

Unless the underlying etiology could be effectively treated and that too, at not a very late stage, the prognosis is dismal and once the decompensation sets in, mortality could be as high as 75% at 2 years.

Etiology of Portal HT continued….

Post Hepatic

Important side effect of portal HT is development of varices.

If there was no development of Esophageal or Gastric varices, portal HT would be of little consequences

Main sites of development of varices:

• The squamocolumnar epithelial junctions of the gastrointestinal tract. These sites are gastroesophageal junction and anorectal junction.

• Esophageal varices are the most important. They are supplied mainly by an enlarged coronary (left gastric) vein and by the short gastric veins arising from the splenic bed and drain into the azygous vein. They are largest 2-3cm above the gastroesophageal junction

• The next most common site for significant varices is the stomach, either in obvious continuity with esophageal varices or isolated gastric varices.

Other sites of development of varices

The recanalized umbilical vein, which communicates with the paraumbilical plexus in the abdominal wall. This gives rise to caput medusae and a Cruveilhier-Baumgarten venous hum in the epigastrium.

The retroperitoneum. The veins of abdominal viscera are commonly in contact with the abdominal wall. Retroperitoneal collaterals frequently communicate with the left renal vein. Sites of previous abdominal surgery or intra-abdominal trauma.

Ectopic sites in the gastrointestinal tract. These are, the duodenum, ileum, cecum, and rectum especially in patients who have had previous abdominal surgery.

Portal hypertension could remain silent for ever in many cases.

Upper or lower GI bleeding are the most commonly recognizable and dreaded symptoms.

If there is advanced decompensated liver diseases accompanying, then there may be jaundice, ascites, edema and encephalopathy.

Collaterals may be visible on the abdomen.

Spleen may be enlarged, sometimes grossly.

Clinical features:

Investigations and diagnosis:

•USG may show dilated portal vein, splenomegaly and at times collaterals in splenic hilum and lower end of esophagus. Underlying etiology like cirrhosis, portal vein or splenic vein thrombosis and Budd-Chiari syndrome could be identified. Doppler study may shoe thrombosis as mentioned above and reversal of blood flow and collaterals.

•Upper GI endoscopy may show varices and portal hypertensive gastropathy. Colopathy changes could be seen on sigmoidoscopy. •LFTs•CBC

Ascites

Shrunken Liver

Portal vein thrombosis Splenomegaly

Portal Pressure Measurement

• Wedged hepatic venous pressure

• Splenic pulp pressure

• Catheterization of umbilical vein

Management of Portal HT and its complications:

If there is no cirrhosis/ liver parenchymal damage, esophageal and gastric varices are the only dreaded complication of portal HT

• Non selective B-blocker propranolol starting with a dose of 10 mg. twice a day and then titrated to reduce the pulse rate buy 25% is used as primary prophylaxis against bleeding

• In a high risk patients, patients living in remote area or in selected patients with large esophageal varices of grade 3 or more could be offered prophylactic endoscopic band ligation(EVL).

• Bleeding is managed as mentioned in the upper GI bleeding.

• Underlying etiology may be corrected with appropriate treatment

Principles of UGI bleed

• Resuscitate• While waiting for endoscopy start IV PPI and if portal HT is suspected Somatostatin or Octreotide.

• Clean stomach with help of a large bore Ryle's tube. Rule of 18; 18 G Venflon, 18 G Ryle’s tube, and endoscopy in 18 hours

• Once the diagnosis is confirmed stop one of the two as per the findings

Variceal bleed

• Band Ligation• Cyanoacrylate glue

• Sclerotherapy

Ulcer bleed

• Adrenalin Injection• Heater prob• Hemoclip application

Prognosis in portal HT:

Depends upon bleeding and underlying liver status.

In NCPF, the prognosis is excellent if the bleeding episodes are well managed.

Prognosis is guarded if there is accompanying liver disease.

Any Questions?Dr Manoj K Ghoda M.D., M.R.C.P.Consultant GastroenterologistVisiting faculty, GCS Hospital

gujarat gastro group