I, caudate lobe; II-IV the I, caudate lobe; II-IV the left-; V-VIII the right left-; V-VIII the right hemiliverhemiliver

Functional unit: Functional unit: acinus acinus PT: PT: PV, arteriole, bile PV, arteriole, bile

ductuleductuleZone 1 Zone 1 is well O2; more is well O2; more resistant than zone 3. resistant than zone 3.

Sinusoids Sinusoids lack BM; have lack BM; have fenestrated endoth. & fenestrated endoth. &

Kupffer cells. Bile Kupffer cells. Bile canaliculi join to form canaliculi join to form

bile ductulesbile ductules

BM: basement membrane

THV: terminal hepatic venule

PT: Portal triad

Cirrhosis & Portal Cirrhosis & Portal HypertensionHypertension

At the end we will learnAt the end we will learn

CLD is rare in children (1/5000)CLD is rare in children (1/5000) CLD may be CLD may be idiopathicidiopathic Most CLD are preventableMost CLD are preventable Commonest c/of PH is cirrhosisCommonest c/of PH is cirrhosis Commonest c/of HCC is HBVCommonest c/of HCC is HBV HCV is curableHCV is curable

CLD: chr. liver d. PH: portal HTN. HCC: HC CaCLD: chr. liver d. PH: portal HTN. HCC: HC Ca

DefinitionDefinitionChr. liver d.: Chr. liver d.: progressive destruction & regen. of progressive destruction & regen. of

Liver parenchyma lasting Liver parenchyma lasting >6mo >6mo leading to cirrhosis. leading to cirrhosis. It includes: It includes: inf., metabolic, genetic, drugs, idiopathic, ., metabolic, genetic, drugs, idiopathic,

structural, HCC & AIDstructural, HCC & AID

Cirrhosis: Cirrhosis: scarring of Liver from injury/long-term d. scarring of Liver from injury/long-term d. blocking BF: blocking BF: HC failure & HC failure &

Portal HTN developPortal HTN develop Chr. Hepatitis: Chr. Hepatitis: chr. inflamchr. inflam

within L. leading to cirrhosiswithin L. leading to cirrhosis& liver failure; persistent & liver failure; persistent for a period of 6mofor a period of 6mo

BF: blood flow. D: disorder/diseaseBF: blood flow. D: disorder/disease

Etiology of Cirrhosis & PHEtiology of Cirrhosis & PH

Infections: Infections: Liver disorders: Liver disorders: Bile ductal blockages: Bile ductal blockages: Drugs & toxins: Drugs & toxins:

Liver disorders:Liver disorders: – Autoimmune hepatitis:Autoimmune hepatitis:– Chr. Chr. alcoholismalcoholism– Hereditary:Hereditary:

HemochromatosisHemochromatosis αα1-antitrypsin deficiency1-antitrypsin deficiency GSDGSD GalactosemiaGalactosemia CFCF Fructose intoleranceFructose intolerance TyrosinemiaTyrosinemia Wilson DWilson D

– Indian childhood cirrhosisIndian childhood cirrhosis

Autoimmune Liver DiseasesAutoimmune Liver DiseasesProgressive inflam. by autoantibodies; rare in children: Progressive inflam. by autoantibodies; rare in children: 2% 2% of LD in specialized pediatric L. centersof LD in specialized pediatric L. centersBroad clinical spectrum. Broad clinical spectrum. Quickly responds to steroids Quickly responds to steroids

& immunosuppressants& immunosuppressantsCommon Common SS: SS: tiredness & unwellness, anorexia, school tiredness & unwellness, anorexia, school deterioration. deterioration. HSM, abnormal LFT, HSM, abnormal LFT, autoantibodiesautoantibodies, , characteristic liver characteristic liver biopsybiopsy

Dx Dx is often by is often by exclusionexclusion. Anti-sm Ab, anti-liver kidney . Anti-sm Ab, anti-liver kidney microsome type I Ab, ANA. Biopsy may be necessary microsome type I Ab, ANA. Biopsy may be necessary for for type & severity of L. damage & inflamtype & severity of L. damage & inflam2 types: 2 types: AIH & AI sclerosing cholangitisAIH & AI sclerosing cholangitis

Inherited liver diseasesInherited liver diseases

>100 liver diseases; >100 liver diseases; some are inherited. 2 common are some are inherited. 2 common are haemochromatosis & alpha-1 antitrypsin d.haemochromatosis & alpha-1 antitrypsin d.

Haemochromatosis: Haemochromatosis: excess Fe is absorbed & deposited. The excess Fe is absorbed & deposited. The commonest is hereditary. 2y HC occurs in hemolytic a. commonest is hereditary. 2y HC occurs in hemolytic a. Genetic HC affects M x5. As F menstruate, are unlikely Genetic HC affects M x5. As F menstruate, are unlikely to show SS till >10y after MP. to show SS till >10y after MP. More in N Europe More in N Europe

SS: SS: Hepatomegaly, joint p, fatigue, unexplained wt loss, Hepatomegaly, joint p, fatigue, unexplained wt loss, darkening of skin ("bronzing“), AP, loss of libido. DM darkening of skin ("bronzing“), AP, loss of libido. DM & & heart d. may dev.; also cirrhosis, HCC, testicular heart d. may dev.; also cirrhosis, HCC, testicular atrophy, & chr AP. Blood iron is confirmatory. HC DNA atrophy, & chr AP. Blood iron is confirmatory. HC DNA test is done; also for screeningtest is done; also for screening

Rx.Rx. of haemochromatosis of haemochromatosis

to remove excess Fe, reduce SS or complications. Fe is to remove excess Fe, reduce SS or complications. Fe is removed by phlebotomy: ½L blood is removed/w for 2-3y removed by phlebotomy: ½L blood is removed/w for 2-3y

until Fe is reduced; then less frequently: based on until Fe is reduced; then less frequently: based on individual circumstancesindividual circumstances

Avoid dietary Fe, most commonly offal, red meat & Avoid dietary Fe, most commonly offal, red meat & fortified BF cereals, also multi-vitamin prepn.fortified BF cereals, also multi-vitamin prepn.

It is also a good idea to limit It is also a good idea to limit vitamin Cvitamin C No alcoholNo alcohol

If HC has caused cirrhosis, the risk of HCC is higher. As a If HC has caused cirrhosis, the risk of HCC is higher. As a result, screening should be performed/6moresult, screening should be performed/6mo

Alpha-1 antitrypsin d.Alpha-1 antitrypsin d.

an imp. Liver protein alpha-1 antitrypsin, an imp. Liver protein alpha-1 antitrypsin, is either lacking or is either lacking or low. Pts. are able to make it; but, the disease low. Pts. are able to make it; but, the disease

prevents prevents it from entering blood & accumulates in liverit from entering blood & accumulates in liver A1AT protects lungs from damage. A1AT protects lungs from damage. The d. causes the lungs The d. causes the lungs

become damaged: difficulty breathingbecome damaged: difficulty breathing 1% COPD have A1AT deficiency. Risk of cirrhosis is high 1% COPD have A1AT deficiency. Risk of cirrhosis is high

SS: SS: first symptoms appear usually in lungs: COPD: first symptoms appear usually in lungs: COPD: SoB/ SoB/ wheezing, unexplained wt loss & barrel-shaped chest wheezing, unexplained wt loss & barrel-shaped chest As it progresses, SS of cirrhosis may appear As it progresses, SS of cirrhosis may appear

A1AT in blood will confirm A1AT in blood will confirm

Rx.: Rx.: No cure. Replacement has been triedNo cure. Replacement has been tried

Rx COPD & cirrhosis: ABT, inhaled medications. DiureticsRx COPD & cirrhosis: ABT, inhaled medications. Diuretics& other measures to reduce edema& other measures to reduce edema

No alcohol, smokingNo alcohol, smoking Healthy dietHealthy diet Flu & pneumonia vax.Flu & pneumonia vax. Treat inf. Treat inf. asapasap. Occasionally the lungs or liver deteriorate . Occasionally the lungs or liver deteriorate

despite Rx. Lung/liver transplant may be neededdespite Rx. Lung/liver transplant may be needed With appropriate Rx A1AT is usually not fatal. But, With appropriate Rx A1AT is usually not fatal. But,

complications a/with them can be fatalcomplications a/with them can be fatal It is v. imp. that people with inherited LD do all they can It is v. imp. that people with inherited LD do all they can

to stay healthyto stay healthy

Bile ductal blockagesBile ductal blockages

– Cong.: biliary atresia Cong.: biliary atresia – Sclerosing cholangitis– Gallbladder surgeryGallbladder surgery

Drugs & toxinsDrugs & toxins– Paracetamol. arsenic, INH Paracetamol. arsenic, INH – MethotrexateMethotrexate– Excess vitamin A, ironExcess vitamin A, iron– Afla toxinAfla toxin

InfectionsInfections– Hepatitis B, C, DHepatitis B, C, D– Schistosomiasis, BrucellosisSchistosomiasis, Brucellosis– Echinococcosis, advanced or cong. syphilisEchinococcosis, advanced or cong. syphilis

Indian Childhood CirrhosisIndian Childhood Cirrhosis

a CLD of children (1–3y) due to deposition of Cu in liver. a CLD of children (1–3y) due to deposition of Cu in liver. It is It is a non-Wilsonian Cu overload by ingested Cu occurs a non-Wilsonian Cu overload by ingested Cu occurs

in in genetically susceptible infantsgenetically susceptible infants It had a v. high CFR before but has eventually become It had a v. high CFR before but has eventually become

preventable, treatablepreventable, treatable Now rareNow rare

CFR: case fatality rateCFR: case fatality rate

The commonest The commonest surgicalsurgical c/of cirrhosis in c/of cirrhosis in childhood is biliary atresiachildhood is biliary atresia

The commonest The commonest medicalmedical causes are causes are– infectionsinfections– alpha-1- antitrypsin Dalpha-1- antitrypsin D– metabolic LDmetabolic LD

C.F. of C.L.D.C.F. of C.L.D.Often asymptomatic in early! Often asymptomatic in early! Start with L. failure. Start with L. failure.

Severity depends on damageSeverity depends on damage– Early: Early: fatigue, weakness, AN, AP, fatigue, weakness, AN, AP, wt. losswt. loss– Later: Later: jaundice, jaundice, dark urinedark urine, itching, edema, ascites, , itching, edema, ascites,

poor healing, drug toxicity, testicular atrophy, poor healing, drug toxicity, testicular atrophy, poor poor hair, hair, hepatic facies, hepatic facies, easy bruising; esophageal, easy bruising; esophageal,

stomach & anal varices; stomach & anal varices; kidney failure, gall stoneskidney failure, gall stones

A small number get A small number get liver cancerliver cancer

CF have 2 componentsCF have 2 components

SS of Hepatocellular failureSS of Hepatocellular failure– pallor, edema, pallor, edema, fatigue, fatigue, jaundice,jaundice, itchy skin, dark urine, itchy skin, dark urine,

ANVANV, , wt. loss, palmar erythemawt. loss, palmar erythema– AP & swelling, pale stool, hepatic facies, spider nevi, AP & swelling, pale stool, hepatic facies, spider nevi,

pigmentationpigmentation– testicular atrophy, poor hair, gynecomazia, poor libidotesticular atrophy, poor hair, gynecomazia, poor libido

SS of Portal HypertensionSS of Portal Hypertension– ascites, distended vein (caput medusa), splenomegaly, ascites, distended vein (caput medusa), splenomegaly,

varices at PS junctions: hematemesis, varices at PS junctions: hematemesis, bloody/tar-bloody/tar-colored stoolcolored stool

Muehrcke's nails Muehrcke's nails

Terry’s nailsTerry’s nails

Kayser–Fleischer ring Kayser–Fleischer ring at limbus: deposition at limbus: deposition of Cu in Descemet m.: of Cu in Descemet m.: characteristic in most characteristic in most

neuro-Wilson& 50% of neuro-Wilson& 50% of hepatic WDhepatic WD

Arcus senilis

Mechanism of Ascites in CirrhosisMechanism of Ascites in Cirrhosis

Portal hypertension: Portal hypertension: commonestcommonest Hepatic arterial vasodilatationHepatic arterial vasodilatation HypoalbuminemiaHypoalbuminemia 2y hyperaldosteronism2y hyperaldosteronism Raised ADHRaised ADH

Complications of CirrhosisComplications of Cirrhosis

Palmar erythemaPalmar erythema HypogonadismHypogonadism Darkening of skinDarkening of skin GynecomaziaGynecomazia HypersplenismHypersplenism Spider neviSpider nevi Caput medusaCaput medusa Ascites, edemaAscites, edema

Varices: hgeVarices: hge Itching Itching Abdominal infx.Abdominal infx. EencephalopathyEencephalopathy Raised drug toxicityRaised drug toxicity Liver cancerLiver cancer

Spider naevi (arterial spider, vascular spider, naevus araneus, naevus

arachnoidius, spider angioma

DiagnosisDiagnosis

LFTLFT CT, USG, laparoscopy CT, USG, laparoscopy BiopsyBiopsy Others: Others:

– FibroscanFibroscan– Measuring the amount of caffeine in the salivaMeasuring the amount of caffeine in the saliva– Measuring the pressure within the liver vein Measuring the pressure within the liver vein – Ascitic fluid analysisAscitic fluid analysis

TreatmentTreatmentNothing cures scar tissue. Nothing cures scar tissue. Goals are:Goals are:

– Rx underlying causesRx underlying causes– Preventing further damagePreventing further damage– Rx symptoms & complicationsRx symptoms & complications

Fight inf.Fight inf. Cut down absorption of wastes/toxinsCut down absorption of wastes/toxins FEB. Salt restriction FEB. Salt restriction HemostasisHemostasis

Liver transplant SOSLiver transplant SOS Vax. against flu, pneumonia, hepatitisVax. against flu, pneumonia, hepatitis No hepatotoxic drugsNo hepatotoxic drugs No alcoholNo alcohol Diet: extra calories & a generous protein to help Diet: extra calories & a generous protein to help

liver regenerateliver regenerate If cirrhosis is advanced limit proteinIf cirrhosis is advanced limit protein

PORTAL PORTAL

HYPERTENSION IN HYPERTENSION IN

CHILDRENCHILDREN

IntroductionIntroduction Portal vein: Portal vein: SV with SMV: carries 70% of liver blood. SV with SMV: carries 70% of liver blood.

Ends in sinusoidsEnds in sinusoids Double capillariesDouble capillaries PH: PH: raised pressure in PV >raised pressure in PV >11mmHg 11mmHg or a splenic pulp or a splenic pulp

pressure of >16mmHg. Clinically this is not measured pressure of >16mmHg. Clinically this is not measured directly until a TIPS is decideddirectly until a TIPS is decided

SV: splenic vein. SMV: superior mesenteric vein. PH: portal hypertensionSV: splenic vein. SMV: superior mesenteric vein. PH: portal hypertensionTIPS: transjugular intrahepatic portosystemic shuntTIPS: transjugular intrahepatic portosystemic shunt

PH: PH: splenomegaly splenomegaly & & porto-systemic shunts at:porto-systemic shunts at:

Lower end of Lower end of eophagus eophagus via GE veins: via GE veins: hemat./malenahemat./malena Anal Anal veins: veins: hemorrhoidshemorrhoids Umbilical Umbilical veins: veins: caput medusacaput medusa Abdominal wall & retroperitoneum: Abdominal wall & retroperitoneum: distended veinsdistended veins

GE: gastroesophagealGE: gastroesophageal

Eesophageal varicesEesophageal varices• Gastroesophageal junction to 15cm upGastroesophageal junction to 15cm up

• Slow oozing or sudden severe hgeSlow oozing or sudden severe hge

Gastric varicesGastric varices

Extension of esophageal varices or varices in Extension of esophageal varices or varices in fundus & upper body. Can occur alonefundus & upper body. Can occur alone

Rectal varices:Rectal varices: hemorrhoidshemorrhoids

Gastric varicesGastric varices

Rectal varicesRectal varices

Aetiology of Portal HTNAetiology of Portal HTN• IntrahepaticIntrahepatic:: 80% 80%

• CirrhosisCirrhosis is the commonest; scar tissue blocks BF & is the commonest; scar tissue blocks BF & slows liver functionsslows liver functions

•Schistosomiasis, cong. hepatic fibrosis, etc.Schistosomiasis, cong. hepatic fibrosis, etc.

• PrehepaticPrehepatic:: 20%20%•PV thrombosis:PV thrombosis:

• extension of obliterative process of umb. veinextension of obliterative process of umb. vein• omphalitis (in newborn)omphalitis (in newborn)

• PosthepaticPosthepatic (rare): cons. pericarditis, tricuspid (rare): cons. pericarditis, tricuspid incompetence, incompetence, Budd-Chiari syn. Budd-Chiari syn. (occlusion of HV)(occlusion of HV)

Cong. hepatic fibrosis: Cong. hepatic fibrosis: hematemesis, normal LFTs, hematemesis, normal LFTs, hepatomegaly. May have polycystic disease& other hepatomegaly. May have polycystic disease& other renal D. Etiology is unknownrenal D. Etiology is unknown

Hepato-portal sclerosis: Hepato-portal sclerosis: thickening of intrahepatic PV thickening of intrahepatic PV obstructs BF: leads to collateral veins in porta obstructs BF: leads to collateral veins in porta

hepatishepatis

Suprahepatic obstruction Suprahepatic obstruction c/by a web in the IVC or c/by a web in the IVC or Budd-Chiari syn. Extremely rare. CF may mimic Budd-Chiari syn. Extremely rare. CF may mimic constrictive pericarditis. DD by echo-, venographyconstrictive pericarditis. DD by echo-, venography

CF of portal hypertensionCF of portal hypertensionmay not always be specificmay not always be specific Splenomegaly (hypersplenism: pancytopenia)Splenomegaly (hypersplenism: pancytopenia) Caput medusaCaput medusa Hematemesis, melena or PR bleedHematemesis, melena or PR bleed AscitesAscites Encephalopathy: poor liver function & diversion of blood Encephalopathy: poor liver function & diversion of blood

away from liveraway from liver

DD of uncommon CLDDD of uncommon CLD

ConditionsConditions PresentationPresentationBudd-Chiari SynBudd-Chiari Syn AscitesAscites

Primary sclerosing Primary sclerosing Cholangitis (PSC)Cholangitis (PSC)

Abnormal LFT/jaundice Abnormal LFT/jaundice

Primary bil. cirrhosis (PBC)Primary bil. cirrhosis (PBC) Abnormal LFT, malaise, Abnormal LFT, malaise, lethargy, itchinglethargy, itching

Caroli diseaseCaroli disease Abdominal pain/sepsisAbdominal pain/sepsis

Simple liver cystSimple liver cyst Pain/coincidental findingsPain/coincidental findings

Polycystic liver diseasePolycystic liver disease Pain/hepatomegalyPain/hepatomegaly

Diagnosis: Diagnosis: by detecting CLD, encephalopathy, ascites, by detecting CLD, encephalopathy, ascites,

varices.varices. Investigations: Investigations: esophagoscopy, Ba-swallow, esophagoscopy, Ba-swallow, USG, celiac axis angiogramUSG, celiac axis angiogram

PV Thrombosis: PV Thrombosis: Dx is difficultDx is difficult

may present within 5y age as hematemesis with only may present within 5y age as hematemesis with only splenomegaly & low plateletsplenomegaly & low platelet

• H/of umbilical V cannulation & abdo. sepsis (40%), or H/of umbilical V cannulation & abdo. sepsis (40%), or trauma/pancreatitis. PVT is mainly congenital. LFTs in trauma/pancreatitis. PVT is mainly congenital. LFTs in these these are normal. USG may confirm PVT: collateral veins are normal. USG may confirm PVT: collateral veins in the in the porta hepatis replacing the PVporta hepatis replacing the PV

TreatmentTreatmentEsophageal variceal bleeding:Esophageal variceal bleeding:

By the time pt. reaches ERBy the time pt. reaches ER

- 1/31/3rdrd – stopped bleeding – stopped bleeding

- 1/31/3rdrd – still oozing – still oozing

- 1/31/3rdrd – still bleeding heavily – still bleeding heavily

Initial measures: ICUInitial measures: ICU- BT: up to 10 unitsBT: up to 10 units- LFT: coagulation profileLFT: coagulation profile- If <50,000, platelet transfusion- If <50,000, platelet transfusion- Endoscopic evaluation & treatment- Endoscopic evaluation & treatment

• Encephalopathy: may need ventilatorEncephalopathy: may need ventilator

• Bronchial aspiration – a frequent complicationBronchial aspiration – a frequent complication

• If profuse bleed prohibits endoscopy:If profuse bleed prohibits endoscopy:• Insert Sengstaken – Blakemore tube & tamponade; Insert Sengstaken – Blakemore tube & tamponade;

deflate after 12h to avoid pressure necrosisdeflate after 12h to avoid pressure necrosis

Endoscopic sclerotherapyEndoscopic sclerotherapy

- Ethanolamine oleate- Ethanolamine oleate

- Sodium tetradesyl sulphate- Sodium tetradesyl sulphate

- 5% phenol in Almond oil- 5% phenol in Almond oil

- Butyl cyanoacrylate- Butyl cyanoacrylate

DrugsDrugs

• I/V Vasopressin. SE: Abdo. crampsI/V Vasopressin. SE: Abdo. cramps• I/V Octreotide (Somatostatin analogue): Equally I/V Octreotide (Somatostatin analogue): Equally

effective, safeeffective, safe

TIPSSTIPSS (transjugular intrahepatic portosystemic stent) (transjugular intrahepatic portosystemic stent) revolutionized the Rxrevolutionized the Rx

• Main Rx for drug resistant cases or in endoscopic Main Rx for drug resistant cases or in endoscopic therapy failuretherapy failure

Surgical shuntSurgical shunt

(Replaced by TIPSS & liver transplant)(Replaced by TIPSS & liver transplant)

Surgical shunts:Surgical shunts:

• splenorenalsplenorenal

• portocaval portocaval

SE: encephalopathy (40%)SE: encephalopathy (40%)

No benefit for pts. who have no bleedingNo benefit for pts. who have no bleeding

C.I. C.I. of Liver transplantof Liver transplant

• Age >65 yAge >65 y

• H/o IHD, HF, Chr. Respiratory diseaseH/o IHD, HF, Chr. Respiratory disease

• Previous Surgical shunts (relative C.I.)Previous Surgical shunts (relative C.I.)

Rx of Ascites of CLDRx of Ascites of CLD

• Salt restrictionSalt restriction

• DiureticsDiuretics

• ParacentesisParacentesis

• Peritoneovenous shuntingPeritoneovenous shunting

• TIPSSTIPSS

• Liver transplantLiver transplant

MCQMCQ

Hepatitis A & E are common c/of CLDHepatitis A & E are common c/of CLD Vaccines for HBV protects against HDVVaccines for HBV protects against HDV Haemorrhoids in cirrhosis are due to HC failureHaemorrhoids in cirrhosis are due to HC failure Palmer erythema is normal in pregnancyPalmer erythema is normal in pregnancy Commonest c/of PHT is cirrhosisCommonest c/of PHT is cirrhosis

MCQMCQ Commonest surgical c/of cirrhosis in children is Commonest surgical c/of cirrhosis in children is

biliary atresiabiliary atresia Edema in CLD starts in legsEdema in CLD starts in legs CLD may be idiopathicCLD may be idiopathic Autoimmune LD have good prognosisAutoimmune LD have good prognosis A1AT protects lungsA1AT protects lungs

Next lecture: Next lecture:

UTI in ChildrenUTI in Children

THANK YOU

Cirrhosis: OverviewCirrhosis: OverviewMany children are Dx with cirrhosisMany children are Dx with cirrhosis L. is the 2L. is the 2ndnd largest organ: processes nutrients. It is also a largest organ: processes nutrients. It is also a

big filter for blood: removing poisons & toxins as well as big filter for blood: removing poisons & toxins as well as byproducts from metabolism. It helps to control BGL & byproducts from metabolism. It helps to control BGL & cholesterol, & makes coagulantscholesterol, & makes coagulants

It has regenerative ability: large portion can be removed: It has regenerative ability: large portion can be removed: will grow back with no problemswill grow back with no problems

But this ability is not unlimited. If the damage is chr., the But this ability is not unlimited. If the damage is chr., the repair mechanisms fail & it begins to scar: when much of L repair mechanisms fail & it begins to scar: when much of L will be replaced with scar: will be replaced with scar: cirrhosiscirrhosis

As it's hard to reverse this scarring once started, the main As it's hard to reverse this scarring once started, the main goal of Rx is to keep it from advancing further, generally goal of Rx is to keep it from advancing further, generally by addressing whatever underlying medical condition has by addressing whatever underlying medical condition has caused the liver to scar. If uncontrolled, cirrhosis can have caused the liver to scar. If uncontrolled, cirrhosis can have serious complications like PH & hepatopulmonary syn. It serious complications like PH & hepatopulmonary syn. It can also increase risk of HCCcan also increase risk of HCC

While cirrhosis in adults is often c/by alcohol, in children While cirrhosis in adults is often c/by alcohol, in children d. of biliary tract & inherited or genetic conditions are the d. of biliary tract & inherited or genetic conditions are the most common reasons. It is important to stress that most common reasons. It is important to stress that no no amount of alcohol consumption by a parent, even during amount of alcohol consumption by a parent, even during pregnancy, can cause a child to develop cirrhosispregnancy, can cause a child to develop cirrhosis

Cirrhosis of the liver Symptoms & Cirrhosis of the liver Symptoms & CausesCauses

We understand that you may have a lot of questions when your child is diagnosed with cirrhosis. What exactly is it? What are the causes of cirrhosis? What are potential complications in my child’s case? What are the treatments? What are possible side effects from treatment? How will it affect my child long term? We’ve tried to provide some answers to those questions here, & when you meet with our experts, they can explain your child’s condition & treatment options fully. What is the liver, & what does it do? The liver is the body’s second largest organ, located in the right side of the abdominal cavity below the diaphragm & above the right kidney & intestines. The liver helps the body in hundreds of ways: All of the blood coming from the stomach & intestines passes through the liver through a large vein called the portal vein. The liver turns nutrients from the food we eat & chemicals from the medicines we take into forms that the rest of our bodies can use. The liver helps clean the bloodstream of harmful substances & poisons. The liver makes bile, which contains chemicals to help us digest the food we eat. The liver helps control blood sugar & cholesterol levels The liver makes the proteins that allow blood to clot normally. What is cirrhosis? While the liver has an amazing capacity to heal itself if damaged by illness or injury, in the case of long term damage the liver may become scarred. The most severe form of scarring is called cirrhosis. What happens to the liver in cirrhosis? The normal healthy liver has a smooth, shiny surface. Over time, as the liver tries to recover from long-term illness or injury, hard scar tissue can replace the liver’s healthy tissue. When this happens, blood cannot flow through the liver as easily & the liver cannot work as well. Once it has started, if the damage continues, the scar tissue in the liver will gradually replace the liver’s healthy tissue, & the organ can actually start to shrink & take on a shriveled appearance. When the scarring (also called

fibrosis) gets to the point of causing nodules instead of a soft, smooth liver, it is called cirrhosis. Causes What causes cirrhosis? Because cirrhosis is always linked to some kind of long-term disease or injury to the liver, it can be caused by a large number of disorders. In adults, chronic alcohol ingestion, viral hepatitis, & fatty liver are the most common causes. In children, biliary tract disorders & genetic conditions top the list of causes: Biliary atresia, a condition that occurs in the first month or two of life, a blockage of the ducts that bring bile from the liver to the gallbladder & intestines. Autoimmune hepatitis, in which the body’s immune system turns against the liver Cystic fibrosis, an inherited chronic disease in which the body produces unusually thick, sticky secretions. Alpha-1 antitrypsin deficiency, a genetic disease that prevents the liver from building a protein it normally releases into the bloodstream. Chronic viral hepatitis caused by the viruses hepatitis B or C. These viruses cause the liver to swell, which can encourage scar tissue to form. Primary sclerosing cholangitis, an acquired condition which causes inflam. & narrowing of the bile ducts inside and/or outside of the liver Wilson disease, an uncommon inherited disorder in which the body cannot rid itself of excess copper found in many foods; the copper then builds up in the liver. Non-alcoholic fatty liver disease (NAFLD), a buildup of fat in the liver most commonly caused by obesity. Many children with NAFLD have a more serious condition called non-alcoholic steatohepatitis (NASH), which can lead to severe liver scarring. Some congenital (inherited) heart defects Other less common disorders While in adults overconsumption of alcohol is the most common cause of cirrhosis, it is important to stress that no amount of alcohol consumption by a parent, even during pregnancy, can cause a child to develop cirrhosis . Signs & symptoms What are the symptoms of cirrhosis? Cirrhosis itself generally produces no symptoms early on, though your child may experience symptoms related to the underlying medical condition that caused the liver damage. As it worsens, cirrhosis may cause: Loss of appetite Nausea or vomiting Weight loss or difficulty gaining weight Weakness Abdominal pain or swelling The appearance of spider-like blood vessels on the skin With time, cirrhosis may also lead to additional serious problems, including: A yellowing of the skin or the whites of the eyes (called jaundice) Bruising or bleeding easily, or nosebleeds Swelling of the legs or abdomen from build up of fluid. In the legs this fluid buildup is called edema; in the abdomen it is called ascites. Confusion or difficulty thinking (encephalopathy), caused by a buildup in the bloodstream of waste products from food. Failure of the kidneys to work properly Because in cirrhosis blood cannot flow as easily through the liver, the pressure in the vein entering your child’s liver, called the portal vein, may increase, a condition called portal hypertension. This condition can cause its own symptoms & complications, & has its own treatments. The increased blood pressure in the portal vein can also affect how blood circulates in the lungs, causing conditions called hepatopulmonary syndrome & portopulmonary hypertension. How is cirrhosis diagnosed? Doctors usually make a Dx of cirrhosis based on a combination of symptoms, medical history, physical exam, & blood tests. In some cases, our doctors may order a liver biopsy to confirm the diagnosis, which will help them see the extent of the scarring in the liver. How do you treat cirrhosis? While in most cases there is no way to cure cirrhosis, our doctors will work with you & your child to control it & keep it from getting worse. They will also help understand & treat the underlying illness or damage that is causing the liver to scar. Cirrhosis can also raise your child’s risk of developing liver cancer. & in advanced cases, cirrhosis can cause the liver to start to fail altogether. If that should happen, we may refer your child for a liver transplant. How can cirrhosis affect my child in the long term? Because a damaged liver cannot break down medicines as quickly as if it were healthy, medicines – including over-the-counter medicines & vitamin or herbal supplements – may work more strongly than before, or may not be made into the active forms by the sick liver. For this reason, if your child has cirrhosis, you should always talk to his or her doctor before starting any new medications or supplements, even vitamins. Cirrhosis may make it harder for your child to gain or maintain his weight & healthy nutritional status. Cirrhosis may be an additional risk factor if your child has other medical problems or needs surgery. If your child has portal hypertension from cirrhosis, she may have more long-term effects. Cirrhosis is a chronic condition, & for this reason your child will likely have to seek care for it for the rest of his or her life. The Center for Childhood Liver Disease can help you & your child plan for the eventual transition from pediatric to adult care. Questions to ask your doctor You & your family play an essential role in your child’s care for cirrhosis. It’s important that you share your observations & ideas with your child’s treating physician, & that you have all the information you need to fully understand the treatment team’s explanations & recommendations. You’ve probably thought of many questions to ask about your child’s cirrhosis. It’s often very helpful to jot down your thoughts & questions ahead of time & bring them with you, along with a notebook, to your child’s appointment. That way, you’ll have all of your questions in front of you when you meet with your child’s treating clinician & can make notes to take home with you. Some questions to ask your doctor might include: How did you arrive at this diagnosis? Are there any other conditions my child might have instead? Does my child need further testing? What is causing my child's cirrhosis? What is the long-term outlook for my child? What medications will you prescribe, & what are the possible side effects? How should I talk to my child about this condition & her long-term health? Do I need to make any changes to my child’s home & school routines? Can you point me to educational & support service resources for children with cirrhosis in my area? What other resources can you point me to for more information?

Testing & Dx for Cirrhosis of the liver in ChildrenThe first step in treating your child is forming an accurate & complete diagnosis. Your child’s doctor will usually make a Dx of cirrhosis

based on a combination of symptoms, medical history, physical exam, & blood tests. In most cases, our doctors will order a liver biopsy to confirm the diagnosis, & help them see the extent of the scarring in your child’s liver.Your doctor may gauge the severity of your child’s cirrhosis using a system called the CTP or Child-Turcotte-Pugh (“Child”) score. This score

combines a set of three laboratory-based measures (bilirubin, albumin, & prothrombin time) with two bedside ones (the presence & severity of ascites, & the level of encephalopathy). While this scoring system was designed for adults & has its limitations, it draws a good picture of both the status of your child’s liver & the overall changes in the body caused by cirrhosis.

Additional monitoringRegardless of the underlying cause, once they make a Dx of cirrhosis our doctors will keep track of your child’s condition by several means,

including periodic:Blood tests, to check how well your child’s liver is working as well as his or her fluid balance.Ultrasounds, which use high frequency sound to help your child’s doctor to see his or her liver. Ultrasounds are painless, non-invasive, &

use no radiation.Physical exams. In addition, our doctors will want to check your child’s blood oxygen levels every year to see whether he or she is developing

hepatopulmonary syndrome, in which increased blood pressure in the liver’s portal vein causes changes in how blood circulates through the lungs. We will also monitor your child’s kidneys for signs of complications.

Our doctors may also want to occasionally conduct an endoscopic exam to see whether new, weakened blood vessels called varices are growing in your child’s esophagus in response to the irregular blood flow in the liver. To do this, they will either use either an endoscope – a small, flexible tube – to examine the inside of your child’s esophagus, or ask him or her to swallow a small capsule containing a camera, which will pass through your child’s digestive tract.

Because cirrhosis can raise your child’s risk of developing liver cancer, our doctors will want to conduct regular cancer surveillance.

Treatments for Cirrhosis of the liver in ChildrenAt Boston Children’s Hospital, we take a multidisciplinary approach to the care of children with cirrhosis. Our

Center for Childhood Liver Disease is one of the few centers with a dedicated team of specialists who are board-certified in pediatric hepatology & transplant.

Cirrhosis has no cure; once the liver starts to scar, in most cases the scarring will not go away. Therefore, our focus is to keep your child’s cirrhosis from becoming worse, largely by treating the underlying illness or damage that caused the scarring to start in the first place.

While the specific course of treatment will therefore be chosen largely based on your child’s underlying medical condition, there are some things that we do for all children with cirrhosis:

Encourage healthy eating. Good nutrition is key for all people with cirrhosis, young & old. It is especially important for children with cirrhosis or any other liver disease to eat well so as to maintain growth. For this reason, our doctors may prescribe caloric supplements or special formulas.

Monitor for complications. Cirrhosis can lead to several complications affecting many organs & systems in the body. Our doctors will keep a close eye on your child to catch any complications early.

Treat complications if they arise. For instance, our doctors may prescribe diuretics to reduce any swelling in your child’s legs or abdomen. If he or she develops varices & they start to bleed, our doctors will treat them, either endoscopically or surgically. They may also ask to conduct additional tests if your child starts to show signs of portal hypertension or hepatopulmonary syndrome, & treat him or her accordingly.

Because a damaged liver cannot break down medicines as quickly as if it were healthy, medicines – including over-the-counter medicines or vitamin or herbal supplements – will work more strongly than before. For this reason, if your child has cirrhosis, you should always talk to his or her doctor before starting any new medications or supplements, even vitamins.

If your child’s cirrhosis cannot be treated & his or her liver starts to fail, a liver transplant may become necessary.Because cirrhosis is a chronic disease, your child will likely have to seek care for it for the rest of his or her life. The Center for Childhood

Liver Disease can help you & your child plan for the eventual transition from pediatric to adult care.

Portal Hypertension Liver Disease in ChildrenContact the Center for Childhood Liver Disease1-617-355-5837While liver disease is rare in children, portal hypertension is even less common. For this reason, few centers are able to adequately manage the

care of children with this serious condition. Over the next few pages we will introduce you to the basics of portal hypertension, its causes, signs & symptoms, & how the physicians in the Center for Childhood Liver Disease at Boston Children's Hospital care for children with portal hypertension.

The liver is a large organ (the second largest in the body) responsible for processing nutrients from our food & medications in ways that allow the rest of the body to make use of them. It also acts as a big filter for the bloodstream, removing poisons & toxins as well as byproducts made by our cells & tissues. The liver helps to control our blood sugar & cholesterol, & it produces chemicals that help our blood to clot.

The liver is truly unique in how it receives its blood supply. Nearly every tissue & organ in the body receives nourishment & oxygen from blood delivered through an artery, which carry blood under high pressure due to the heart’s pumping action. The liver, however, is the only organ to receive the majority of its blood supply through a large vein. Called the portal vein, this vein carries blood to the liver from the intestines & the spleen.

Why would the liver work this way? The pressure in veins, including the portal vein, is much lower than in arteries. The reduced pressure allows blood to percolate through the liver & gives the liver’s cells the time they need to do their work.

Obstruction of the portal vein – caused primarily by clots in or narrowing of the vein before it reaches the liver, cirrhosis, or high pressure in the veins that drain the liver into the heart – can cause the pressure in the vein to build up, much as blocking the end of a hose causes the pressure in the hose to climb. Increased portal vein pressure – known medically as portal hypertension – causes blood to back up in the organs that send blood to the liver. The body tries to relieve the pressure by generating new blood vessels that bypass the blockage, but such vessels are often weak & twisted, & tend to bleed easily. These vessels, called varices, may also bypass the liver itself, allowing toxins & nutrients to travel through the bloodstream unprocessed.

How Boston Children's Hospital approaches portal hypertensionThe weak blood vessels produced by the body in response to portal hypertension put a child at risk for gastrointestinal bleeding (bleeding into the

intestine that causes vomiting of blood and/or passage of blood from the rectum). The major goals of the physicians, nurses, & staff in the Center for Childhood Liver Disease in treating children with portal hypertension are to reduce that risk and, ideally, to reduce the increased blood pressure in the portal vein by identifying its root cause. At every step, our specialists endeavor to provide compassionate care that respects the values of each family & addresses their hopes & concerns for their child’s present & future health.

Portal Hypertension Liver Disease Symptoms & Causes Contact the Center for Childhood Liver Disease 1-617-355-5837 What is the liver, & what does it do? The liver is the body’s second largest organ, located in the right side of the abdominal cavity below the diaphragm & above the right kidney & intestines. The liver helps the body in hundreds of ways. All of the blood coming from the stomach & intestines passes through the liver through a large vein called the portal vein. The liver turns nutrients from the food we eat & chemicals from the medicines we take into forms that the rest of our bodies

can use. The liver helps clean the bloodstream of harmful substances & poisons. The liver makes bile, which contains chemicals to help us digest the food we eat. The liver helps control blood sugar & cholesterol levels The liver makes the proteins that allow blood to clot normally. What is portal hypertension? Portal hypertension describes an increase in pressure within the portal vein, the vessel that carries blood from the organs of the digestive system (the intestines & the spleen) to the liver. What happens to the liver & other organs in portal hypertension? The increased blood pressure in the portal vein causes pressure in other blood vessels to increase as well. For instance, rising blood pressure can force blood to back up into the splenic vein, causing the spleen to swell. Pressure back into the veins

of the intestines can cause diarrhea. As the body senses the increased portal vein pressure, it tries to compensate by growing new veins that bypass the liver. These new veins, called varices, tend to be full of twists & turns, are much weaker than normal veins, & can bleed very easily.

The varices tend to grow around the esophagus, spleen, stomach, & colon. Because varices bypass the liver, toxins & nutrients that the liver would normally process can travel into the rest of the bloodstream. Causes What causes portal hypertension? Portal hypertension results from a blockage in the portal vein before (“prehepatic”), within (“hepatic”), or past (“posthepatic”) the liver. Prehepatic blockages, the most common cause of portal hypertension in children, stem from blood clots or narrowing of the portal vein before it reaches the liver. In response, the body grows varices that bypass the blockage but which, instead of

relieving the pressure, lead to what is called “cavernous transformation of the portal vein.” Cirrhosis is the most common cause of portal hypertension in adults, & the second most common in children. This progressive scarring of the liver is a result of long-term illness or damage to the liver caused by a wide range of conditions. In a child

with cirrhosis, the liver’s soft, healthy tissue is gradually replaced with hard, nodular tissue that blocks the flow of blood through the portal vein.

Signs & symptoms What are the symptoms of portal hypertension? Portal hypertension itself usually causes no symptoms, though its complications can. For instance, varices can rupture & bleed easily, causing gastrointestinal bleeding evidenced by black, tarry stools or vomiting of blood. Fluid buildup, called

ascites, can cause the abdomen to swell & enlarge. Some children feel vague discomfort in the upper left part of the abdomen, caused by enlargement of the spleen. Because portal hypertension is itself often a complication of advanced liver disease, children with it may also experience symptoms of poor liver function. They may experience poor weight gain or weight loss, jaundice & confusion or forgetfulness

due to the presence in the bloodstream of substances, such as toxins, that are normally filtered by the liver.

How is portal hypertension diagnosed? Portal hypertension is often diagnosed through a combination of imaging & endoscopic studies. These techniques give physicians a comprehensive picture of the state of the portal vein & the organs & vessels connected to it. How do you treat portal hypertension? With portal hypertension, the main goal of therapy is to prevent further complications & reduce the risk of variceal bleeding while, ideally, reducing the pressure in the portal vein. This is accomplished through a combination of medication,

endoscopic treatment (“banding” of varices), & surgical therapies (shunting of the blood flow in the portal venous system into the systemic circulation). Because portal hypertension can be a late complication of liver disease, such as cirrhosis, it is important to try to manage the conditions that caused damage to the organ in the first place. Should the liver begin to fail, a liver transplant may become

necessary. How can portal hypertension affect my child in the long term? Because portal hypertension is typically a chronic condition, your child may have to seek care for it for the rest of his or her life. The Center for Childhood Liver Disease can help you & your child plan for the eventual transition from pediatric to

adult care.

Testing & Dx for Portal Hypertension Liver Disease in ChildrenContact the Center for Childhood Liver Disease1-617-355-5837The first step in treating your child is forming an accurate & complete diagnosis. Because portal hypertension can

cause a variety of complications, your child’s doctor will likely already be on the lookout for signs such as gastrointestinal bleeding, enlargement of the spleen, the development of varices, & the presence of abdominal swelling (ascites).

Imaging technologies can provide a great deal of information on the anatomy & function of the portal vein & surrounding organs & vessels. Using ultrasound, a painless & non-invasive imaging technology, physicians can see the direction & speed of the blood flow through the portal vein. In addition, the technology lets them assess the state of the liver, spleen, & gallbladder, & also see whether varices have developed. Often, ultrasound is the first way in which “cavernous transformation of the portal vein” is diagnosed. The portal vein & related blood vessels can also be seen using other techniques, such as a special CT scan (called a “CTA” or “CT angiogram”) or magnetic resonance imaging (MRI).

If your child has portal hypertension, even if he or she has not yet had gastrointestinal bleeding, physicians may use an endoscope – a thin, flexible, lighted tube – to look for varices in the esophagus. If your child is old enough & can swallow a capsule, a wireless capsule endoscopy may be done instead. In this case, a tiny camera in a capsule sends digital pictures to a computer as the capsule itself goes down the esophagus.

If varices are bleeding, our physicians can also use an endoscope to deliver some forms of treatment aimed at controlling this complication.

Treatments for Portal Hypertension Liver Disease in ChildrenContact the Center for Childhood Liver Disease1-617-355-5837As there is no simple cure for portal hypertension, our Center for Childhood Liver Disease takes a multidisciplinary

approach to preventing the condition from becoming worse while addressing the risk of gastrointestinal bleeding.Physicians often prescribe treatment with a medication called a nonselective beta blocker, such as propranolol or

nadolol, which can help lower the blood pressure within the portal vein.Control or prevention of bleeding from varices is a high priority with portal hypertension. To do this, physicians

often use an endoscope to tie off varices using a rubber band (a procedure known as “banding”) or to deliver sclerosing therapy. In this kind of therapy, a physician injects a chemical into the varices directly, causing them to clot.

If your child develops significant ascites, our physicians may try to relieve the fluid load with diuretic medications or, if necessary, by draining the fluid from the abdomen with a needle (a non-surgical procedure called abdominal paracentesis).

Should your child experience continued internal bleeding, doctors may create a bypass or shunt between the portal vein & the rest of the bloodstream. Our physicians often use one of two types of shunting procedures, transjugular intrahepatic portal-systemic shunting (TIPSS, a non-surgical procedure involving use of a catheter) or surgical shunting. Both procedures relieve the pressure on the portal vein & redistribute it to the rest of the bloodstream.

Because portal hypertension is an advanced complication of other forms liver disease, such as cirrhosis, it is important to try to manage the conditions that caused damage to the organ in the first place. Should liver function begin to fail, a liver transplant may become necessary.

Surgery – porto-systemic shunts sclerotherapy with banding is effective control bleeding esophageal varices in majority of children. surgical intervention :

Failure of at least 2 sessions of banding/ sclerotherapy gastric/ectopic varices not responding to endoscopic RxHypersplenismLack of access to endoscopic Rxbiliary obstruction due to choledochal varices Selected patients with Budd-Chiari syn30 different operations were reported. Occlusion of the portal system in the extrahepatic portal hypertension, for example, may affect the portal vein alone or may involve either the splenic or the superior mesenteric veins. At

least 20 percent of these patients are not suitable for any form of shunt surgery& may undergo some type of devascularization with or without oesophageal transection. Unfortunately, devascularisation procedures do have a high incidence of rebleeding of up to 23 percent.

The construction of a shunt between the superior mesenteric vein& the inferior vena cava using a segment of the internal jugular vein seems to offer the best combination of long term patency& the lowest incidence of rebleeding. The rebleed rate was only 6 percent in four series of cases published since 1981.

The recent introduction of the mesenterico-left portal (Rex) shunt is likely to broaden the indications once more for shunt surgery as the primary treatment for children with portal venous occlusion. This shunt utilizes an interposition graft between the superior mesenteric vein& the intra-hepatic portion of the left portal vein, which is identified in the Rex recessus adjacent to the falciform ligament. By restoring hepatic portal blood flow& correcting portal hypertension, this technique is more physiological& obviates the potential disadvantages of the porto-systemic shunts.

The complications of porto-systemic shunting are not only concerned with rebleeding. Deterioration of liver function& hepatic encephalopathy are further hazards, particularly in children with cirrhosis. In a recent series of 37 children, 1 died in the early postoperative period with encephalopathy& 9 out of 31 with a patent shunt showed deterioration of mental function during a mean follow-up period of 5 years.

Porto-systemic shunt surgery should be regarded as a complementary therapy to endoscopic treatment. Porto-mesenteric venous anatomy does not permit successful shunt surgery in every child,& shunt thrombosis has been recorded with all types of shunts especially in smaller children.

Liver transplantation is the treatment of choice for children with variceal bleeding complicating end-stage chronic liver disease.

Conclusion:The management of children with bleeding oesophageal varices is an extremely challenging task& demands a variety of complementary techniques, each of which may be limited by its applicability, efficacy& its complications. Endoscopic sclerotherapy& banding is highly effective& appears to be the treatment of choice for the initial management of oesophageal varices in children.

However, shunt surgery should be reserved for

treatment of gastric or ectopic varices not accessible to sclerotherapy

uncontrollable bleeding secondary to a complication of sclerotherapy

treatment of severe hypersplenism or symptomatic splenomegaly

treatment of children living in communities far away from adequate medical care& blood transfusion facilities.

Liver transplantation is the procedure of choice for patients with complications of portal hypertension associated with end-stage liver disease. The role of newer modalities like the TIPS& Rex shunt has yet to be conclusively proven.

What tests are required before the TIPS& DSRS procedures? Evaluation of medical history A physical examination Blood tests Galactose LFT Angiogram Ultrasound Endoscopy Before either the TIPS or DSRS procedure, your physician may ask you to have other pre-operative tests, which may

include an electrocardiogram (also called an EKG), chest X-ray or additional blood tests. If your physician thinks you will need additional blood products (such as plasma), they will be ordered at this time.

More about the TIPS procedureDuring the TIPS procedure, a radiologist makes a tunnel through the liver with a needle, connecting the portal vein (the vein that carries blood from the digestive organs to the liver) to one of the hepatic veins (the three veins that carry blood from the liver). A metal stent is placed in this tunnel to keep the tunnel open.

The TIPS procedure reroutes blood flow in the liver& reduces pressure in all abnormal veins, not only in the stomach& esophagus, but also in the bowel& the liver.

The TIPS procedure is not a surgical procedure. The radiologist performs the procedure within the vessels under X-ray guidance. The procedure lasts 1 to 3 hours. You should expect to stay in the hospital 2 to 3 days after the procedure.

The TIPS procedure controls bleeding immediately in over 90 percent of patients. However, in about 20 percent of patients, the shunt may narrow, causing varices to rebleed at a later time.

Potential complications of the TIPS procedureShunt narrowing or occlusion (blockage) can occur within the first year after the procedure. Follow-up ultrasound examinations are performed frequently after the TIPS procedure to detect these complications. The signs of occlusion include increased ascites or rebleeding. This condition can be treated by a radiologist who re-expands the shunt with a balloon or repeats the procedure to place a new stent.

Encephalopathy, or mental changes caused by abnormal functioning of the brain that occur with severe liver disease. Encephalopathy can be worse when blood flow to the liver is reduced by TIPS, which may result in toxic substances reaching the brain without being metabolized first by the liver. This condition can be treated with medications, diet or by replacing the shunt.

More about the DSRS procedureThe DSRS is a surgical procedure. During the surgery, the vein from the spleen (called the splenic vein) is detached from the portal vein& attached to the left kidney (renal) vein. This surgery selectively reduces the pressure in your varices& controls the bleeding.

A general anesthetic is given to you before the surgery. The surgery lasts about about 4 hours. You should expect to stay in the hospital from 7 to 10 days.

DSRS controls bleeding in over 90 percent of patients, with the highest risk of any rebleeding in the first month. However, the DSRS procedure provides good long-term control of bleeding.

Potential complication of the DSRS surgery: Ascites, an accumulation of fluid in the abdomen. This can be treated with diuretics& restricted sodium intake.

FU after TIPS or DSRS

After 10 d: evaluate progress by Lab work 6 weeks& again 3 mo: do USG to check shunt. Do angiogram only

if it indicates a problem 6 mo after TIPS or DSRS: do an USG to see shunt. Lab work& a

galactose LFT also done 12 mo: USG. angiogram to check pressure within veins across the

shunt. Lab work& a galactose LFT done If the shunt is working, every 6 mo after the first yearOther treatment Liver transplant removes the varices when a TIPS or a surgical shunt is not

possible or fails in controlling bleeding ascites sometimes needs to be directly removed by paracentesis

Clinical features

PH mainly starts as splenomegaly at 8 y ageEncephalopathy& ascites may be presentGrowth failure (malabsorption, protein losing

enteropathy) is well-noted In PV occlusion: <5 y age with ac. GI hge.,

hemorrhoids seen in 2/3 In Budd-Chiari syn, intractable ascites with

hepatomegaly is the usual initial presentation. Jaundice is variable

InvestigationsInvestigations

Hypersplenism : anemia, low WBC, platelet. Plasma procoagulant& anti- may be reduced in PV occlusion or Budd-Chiari syn

LFT are abnormal in cirrhosis, but normal in PVO. Albumin is low in ac. variceal hge

USG: large collateral veins, portal cavernoma, splenomegaly. Doppler for direction, velocity& waveform characteristics of PV flow. In Budd-Chiari syn, Doppler of HV& IVC can be Dx. Ascites is a notable feature of BCS& cirrhosis can be definitely identified& quantified on USG

GI endoscopyUseful in Dx& Rx of varicesgrading of esophageal varices. Smaller Portal congestive gastropathy is characterized by

mucosal hyperemia with dilated submucosal veins

CT angiography& MRI increasingly used in Dx of BCS& to identify liver

lesions in PH (focal nodular hyperplasia)MR angiography is non-invasive. It delineates

porto-mesenteric venous anatomy

Angiography

Inferior vena cavography with pressure measurements is valuable in patients with Budd-Chiari syndrome in whom hepatic venography can be used to assess hepatic venous patency. Balloon dilatation can be undertaken of inferior vena caval membrane or short segment narrowing of the hepatic veins, which can prove therapeutic.

signs of cirrhosis. As noted before, these signs are not specific to cirrhosis, nor do they occur in all cases of cirrhosis: Spider angiomata or spider nevi, spider telangiectasias: Vascular lesions consisting of a central arteriole (the smallest branches of an artery, terminating in capillaries) surrounded by many smaller vessels commonly found on trunk, face,& upper

limbs, due to an increase in estradiol to free testosterone ratio. [3] These occur in about one third of cases. [4] Verify this finding by compressing it with a glass slide,& observe the pulsation of the central arteriole as blood fills the central arteriole, then travels to the peripheral tips with blanching. Spider angiomas may also be seen in pregnancy (high estrogen state), severe malnutrition, or occasionally in healthy individuals. Greater number& size of spider angioma are associated with more severe liver cirrhosis& risk of bleeding from varices (permanent abnormal dilation& lengthening of a vein). [5][6]

Palmar erythema: Exaggerations of normal speckled mottling of the palm, due to altered sex hormone metabolism. [7] Palmar erythema affects mainly the thenar& hypothenar prominences while sparing the central palm. It is also seen in pregnancy, rheumatoid arthritis, hyperthyroidism,& blood malignancies.

Nail changes:– Muehrcke's nails: Paired horizontal bands separated by normal color due to hypoalbuminemia,[8] from inadequate production of albumin, which is solely made by the liver. Muehrcke's nails are seen in other conditions associated with low serum albumin, such as malnutrition&

nephrotic syndrome (related to the kidney).– Terry's nails: Proximal two-thirds of the nail plate appears white& distal one-third red, also due to hypoalbuminemia.– Clubbing: Angle between the nail plate& proximal nail fold > 180 degrees,& may appear like a drumstick when severe. This is more commonly seen with biliary cirrhosis& is nonspecific.

Hypertrophic osteoarthropathy (HOA): Chronic proliferative periostitis of the long bones, which can be very painful. [9] Note that the most common cause of HOA is lung cancer, which must be ruled out. Dupuytren's contracture: Thickening& shortening of palmar fascia (connective tissue binding parts together) leading to flexion deformities of the fingers. It results from fibroblast proliferation& disorderly collagen deposition in the fascia, likely due

to free radical formation by oxidative metabolism of hypoxanthine. [10] Dupuytren's contracture is common in alcoholic cirrhosis, occurring in about one-third of cases. [11] A nonspecific finding, it is also seen in diabetes mellitus, cigarette smokers, alcohol users without cirrhosis, workers with repetitive hand motions,& Peyronie's disease.

Gynecomastia: Benign proliferation of glandular tissue of male breasts, presenting with a rubbery or firm mass extending concentrically from the nipples. This results from increased estradiol& is seen in up to two-third of cases. Gynecomastia must be distinguished from pseudogynecomastia, fat deposits without glandular proliferation, often seen in obese men. To tell them apart, lie on the back, place thumb& forefinger on each side of the breast,& slowly bring them together to appreciate a concentric, rubbery-to-firm disk of tissue directly under the nipple area in gynecomastia. No mass is felt in pseudogynecomastia,& other mass disorders, such as cancer, tend to be eccentrically located (not centered).

Hypogonadism: Manifested as impotence, infertility, loss of sexual drive,& testicular atrophy, due to primary gonadal injury or suppression of hypothalamic or pituitary function. It is most often seen in alcoholic cirrhosis& hemochromatosis, likely due to toxic effects of alcohol or iron, respectively.[12]

Change in liver size: The liver can be enlarged (hepatomegaly), or of normal size, or shrunken. When palpated, the cirrhotic liver tends to feel firm& nodular. Splenomegaly (increase in size of the spleen): Due to congestion of the splenic red pulp as a result of portal hypertension (increased pressure in the portal vein, as blood backs up from the cirrhotic liver with extensive fibrosis impeding blood flow,

resulting in pressure buildup). Ascites: Accumulation of fluid in the peritoneal (abdominal) cavity giving rise to flank dullness (needs about 1500 cc to detect flank dullness). Caput medusa: In portal hypertension, the umbilical vein may open, to allow blood backing up the portal venous system to be shunted through the periumbilical veins into the umbilical vein& ultimately to the abdominal wall veins to the systemic

venous system. The increased prominence of the periumbilical veins manifest as caput medusa, so called because it resembles the head (caput) of Medusa. Cruveilhier-Baumgarten murmur: Venous hum heard in epigastric region by auscultation with a stethoscope. Similar to caput medusa, it results from collateral connections between the portal system& the remnant of the umbilical vein in portal

hypertension. The murmur is augmented by the Valsalva maneuver (increases intraabdominal pressure)& diminished by applying pressure on the skin above the umbilicus (flattening the blood vessels). [13]

Fetor hepaticus: A musty odor in the breath due to increased dimethyl sulfide in severe portal-systemic shunting from portal hypertension. [14]

Jaundice: Yellow discolouring of the skin, eye,& mucus membranes due to increased bilirubin (at least 2–3 mg/dL or 30 mmol/L). Urine may also appear darkened. Yellow discolouration of the skin may also result from excessive consumption of carotene (for example, in someone who eats lots of carrots); this can be distinguished from jaundice by absence of yellow discolouring of the sclera(white, external part of the eye) in carotenemia& presence of icteric sclera in jaundice.

Asterixis: Bilateral asynchronous flapping of outstretched, dorsiflexed hands seen in hepatic encephalopathy (from buildup of ammonia in liver failure that go to the brain). Asterixis is also seen in uremia& severe heart failure. Other:

– Pigment gallstone: May result from hemolysis[15]

– Enlarged parotid gland: May result from alcohol use leading to fatty infiltration, edema,& fibrosis;[16]

Rx for PHT

Diet, drugs, endoscopic therapy, surgery or radiology Rx are based on severity of the symptoms& liver functioning. First level of Rx

For variceal bleeding, endoscopic therapy or medications. Dietary& lifestyle changes are also important. Endoscopic Rx: either sclerotherapy or banding. Sclerotherapy: a solution is injected into the bleeding varices to stop or

control the risk of bleeding. Banding: a rubber bands to block the blood supply to each varix. Medications: beta blockers or nitrates may be prescribed alone or in combination with endoscopic therapy to reduce the

pressure in your varices& further reduce the risk of rebleeding. Medications such as propranolol& isosorbide may be prescribed to lower the pressure in the portal vein& reduce the risk of

rebleeding. The drug lactulose can help treat confusion& other mental changes associated with encephalopathy. Dietary& Lifestyle Changes

Maintaining good nutritional habits& keeping a healthy lifestyle will help your liver function properly. Some of the things you can do to improve the function of your liver include the following:

Do not use alcohol or street drugs. Do not take any over-the-counter or prescription drugs without first consulting with your physician or nurse. (Some

medications may make liver disease worse,& they may interfere with the positive effects of your other prescription medications).

Follow the dietary guidelines given to you by your physician or nurse. Follow a low-sodium (salt) diet. You will probably be required to consume no more than 2 grams of sodium per day. Reduced protein intake is required only if confusion is a symptom. Your dietitian will help you create a meal plan that helps you follow these dietary guidelines.

Second Level of TreatmentIf the first level of treatment does not successfully control your variceal bleeding, you may require one of the following decompression procedures to reduce the pressure in these veins.

Transjugular intrahepatic portosystemic shunt (TIPS), a radiological procedure in which a stent (a tubular device) is placed in the middle of the liver.

Distal splenorenal shunt (DSRS), a surgical procedure that connects the splenic vein to the left kidney vein in order to reduce pressure in your varices& control bleeding.

Treatment

The survival of the children with portal hypertension depends almost entirely on the etiology. Recent reports show that oesophageal varices in childhood are well controlled with either injection sclerotherapy or porto-systemic shunting& both methods have their advocates. Patients with portal vein obstruction& normal liver histology can be expected to live normal lives providing the oesophageal varices are under control.

A)Treatment of the acute bleed

Acute variceal bleeding, particularly in young infants, can pose problems in management. A delay in immediate management could prove fatal for a child. Medical measures include blood transfusion& the intravenous infusion of vasopressin (0.2 – 0.4 units/1.73 m / min) which may arrest the bleeding. Vasopressin or its precursor, glypressin may be used alone or in combination with nitrates to reduce the portal venous pressure. Unfortunately, these agents have side-effects related to systemic vaso-constriction like headache, nausea& abdominal cramps. Somatostatin reduces splanchnic blood flow& portal pressure with minimal side-effects, but it has a short half life of less than 3 minutes. Octreotide, a long acting analog of somatostatin, has a plasma half-life of more than 1 hour. Although the effectiveness of octreotide has been studied in a small number of children, its safety& side-effect profile have encouraged its use in cases of acute variceal bleeding.

Continued bleeding may be controlled with injection sclerotherapy but the small size of the paediatric endoscope channels can limit the clearance of blood from within the oesophagus. In addition to the above difficulties, there is an added risk of needing general anesthesia in a child with a compromised consciousness. The Sangstaken-Blackmore (S-B tube) compression balloon may be life saving when there is a failure of visualization of the varices due to overwhelming haemorrhage. However, the dangers of this instrument cannot be overemphasized. Correct placement of the gastric balloon must be checked with X-ray control in order to avoid the inflation within the lumen of the oesophagus. This accidental inflation with the oesophagus may result in oesophageal rupture or suffocation from airway obstruction. Inflation of the gastric balloon& moderate prolonged traction achieved by securing the S-B tube to the side of the face with an adhesive tape is usually sufficient to stop the bleeding. It is rarely necessary to inflate the oesophageal balloon present on the standard instrument. Balloon deflation is performed 18 to 24 hours later& this is followed immediately with endoscopic variceal injection

B) Inj. sclerotherapy for long-term Rx. Injection sclerotherapy was suggested for the treatment of oesophageal varices in children because of failures& complications of primary surgery. Portosystemic shunt thrombosis& rebleeding, the hazards of splenectomy in children& long term risks of encephalopathy all encouraged an alternative therapy. Controlled trials in adult patients confirmed that early endoscopic sclerotherapy after the onset of bleeding significantly reduced the risk of rebleeding& may prolong survival in the cirrhotic. Injections are performed through a flexible upper GI endoscope under general anaesthesia with an endotracheal tube in place. Intravenous sedation has been used occasionally in older children. A variety of sclerosants are available including ethanolamine oleate, sodium tetradecyl sulphate, sodium morrhuate, phenol in almond oil& polidocanol. The injections are given either intra or para-variceal& are mostly given into the cardia& lower 3 cm of the oesophagus. A maximum of 3 ml is injected into each varix to a maximum of 5 to 20 ml per session depending on the age& the size of the patient. A naso-gastric tube is inserted in small infants to control the degree of gastric distension. The initial 3 injections are given at weekly intervals& subsequent treatments on a monthly basis until the varices are obliterated.

Mild symptoms of retrosternal discomfort& a transient fever are common after endoscopic sclerotherapy. The variceal haemorrhage may recur, particularly between the first 2 or 3 treatments& oesophageal ulceration may be followed by stricture formation& dysphagia. Rare serious complications have included broncho-oesophageal fistula, chylothorax& pericarditis. One case of paraplegia has been reported from injection of segmental spinal vessels.

An analysis of seven reports published since 1984 of the results of sclerotherapy in 248 children shows a mortality rate of 3 percent& a rebleed rate of 12 percent. The rebleed rate in a series of 7 reports of surgery for portal hypertension (1980 – 86) was 14 percent.

C) Variceal banding.  This technique, which involves application of an elastic band to a variceal column, is done through flexible upper gastro-intestinal endoscopes. The strangulated varix, subsequently, thromboses& sloughs. Usually upto three bands are applied at each session. Multi-band devices allow the application of several bands without the need for reloading. Treatment is performed initially at 1 to 2 weekly intervals, extending to monthly intervals once the larger varices are treated. The incidence of oesophageal stricture& systemic side-effects is lower with this treatment modality. At present, equipment limitations make this technique difficult to use in small children less than 2 years of age.

D) Transjugular intrahepatic port-systemic shunt (TIPS)

The indications of TIPS in children include uncontrolled variceal bleeding especially the ones who are awaiting liver transplantation. Some patients of Budd-Chiari syndrome or intractable ascitis may also benefit by this procedure. Portal vein thrombosis, bacterial sepsis& coagulopathy are contraindications to TIPS.

This intervention involves insertion of an expandable metallic stent from the hepatic to the portal vein through the percutaneous tranjugular route under radiological guidance. Under fluoroscopic control, a guidewire is passed into a hepatic vein. A needle is then advanced over a guidewire into the hepatic vein& thence to the portal vein. A balloon catheter is subsequently used to dilate the intrahepatic tract& the stent is deployed