Portal Hypertension in Children.. Dr.Malathi Sathiyasekaran

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Portal Hypertension in Children by Dr.Malathi Sathiyasekaran, Pediatric Gastroenterologist, Chennai.

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Portal Hypertension in Children.. Dr.Malathi Sathiyasekaran

  1. 1. Portal hypertension in children Malathi Sathiyasekaran Pediatric Gastroenterologist. Chennai
  2. 2. S.G L.G Portal venous System
  3. 3. Portal Hypertension <ul><li>Normal range of portal venous pressure is 5 to 10 mm of Hg above the pressure present in the IVC. </li></ul><ul><li>Portal hypertension is defined as elevation of this pressure gradient to values above 10 to 12 mm Hg. </li></ul>
  4. 4. Portal Hypertension <ul><li>Definition :PHT is a pathologic increase in portal pressure in which the pressure gradient between the portal vein and the IVC (Portal pressure gradient or PPG)is increased above the upper limit of 5 mm of Hg. </li></ul><ul><li>PPG > 10 mmHg(varices) </li></ul><ul><li>PPG > 12mmHg(variceal bleed,ascites) </li></ul><ul><li>PPG > 6 to 10 mmHg(subclinical PHT) </li></ul>
  5. 5. Classification of PHT <ul><li>Pre Sinusoidal:Extrahepatic(1) </li></ul><ul><li>Intrahepatic(2) </li></ul><ul><li>Sinusoidal(3) </li></ul><ul><li>Post sinusoidal:intrahepatic(4) </li></ul><ul><li>extrahepatic(5) </li></ul>1 2 3 4 5
  6. 6. Causes of PHT VOD,Classical BCS IVC obstruction <ul><li>Postsinusoidal </li></ul><ul><li>Intrahepatic. </li></ul><ul><li>Extrahepatic </li></ul>cirrhosis <ul><li>Sinusoidal </li></ul>Portal,splenic vein thrombosis,cong.malformations CHF,NCPF <ul><li>Presinusoidal: </li></ul><ul><li>Extrahepatic </li></ul><ul><li>Intrahepatic </li></ul>
  7. 7. Clinical Evaluation • Splenomegaly • Abdominal veins • Ascites <ul><li>Varices </li></ul>
  8. 8. <ul><li>Splenomegaly </li></ul><ul><li>Ascites </li></ul><ul><li>PS Collaterals – abd. veins & varices </li></ul><ul><li>Hyper dynamic circulation </li></ul><ul><li>Porto Systemic Encephalopathy </li></ul>I Evaluation of PHT: type and consequences of PHT
  9. 9. II Evaluate: Physical signs of chronic liver disease
  10. 10. III Evaluate: Presence of PSE <ul><li>Neuropsychological tests - NCT </li></ul><ul><li>Asterixis </li></ul><ul><li>Foetor Hepaticus </li></ul>
  11. 11. <ul><li>An enlarged spleen is the single most important diagnostic sign of PHT </li></ul><ul><li>Does not correlate with height of portal pressure, size of varices or age of pt. </li></ul><ul><li>Correlates with type of PHT ( * NCPF 12cm, * * EHPVO 6cm) </li></ul><ul><li>Spleen may not be palpable soon after a bleed </li></ul>Splenomegaly
  12. 12. Dilated Abdominal Veins <ul><li>Presence supports the diagnosis of PHT (Cirrhosis, BCS) </li></ul><ul><li>Absence does not exclude PHT (EHPVO) </li></ul><ul><li>Periumbilical veins indicate intrahep PHT, (murmur – Cruvellier Baumgarten) </li></ul><ul><li>Back veins – indicates HVOO (Classical BCS/IVC) </li></ul>
  13. 13. Dilated Veins
  14. 14. Ascites <ul><li>Presence of ascites supports diag of PHT </li></ul><ul><li>Present in sinusoidal/post-sinusoidal </li></ul><ul><li>Sudden accumulation of ascites – HVOO </li></ul><ul><li>“ Frog belly” – IVC obstruction </li></ul><ul><li>Ascites in EHPVO (0-36%), NCPF (5-10%) transient </li></ul>
  15. 15. <ul><li>Consistency more significant than size </li></ul><ul><li>Size correlates poorly with height of pp. </li></ul><ul><li>Normal, soft or small liver EHPVO </li></ul><ul><li>Firm, nodular ,  vertical span or enlarged,L .lobe palpable- cirrhosis </li></ul><ul><li>Left lobe liver enlarged - CHF </li></ul><ul><li>Firm liver – NCPF (10-15% nodular) </li></ul>Liver Size & Consistency
  16. 16. Age & PHT India Children Adults West - Children Anand A C et al. Yachha et al Goncalves M E
  17. 17. Age & PHT ICH & HC Chennai, 1999 - 2001 Total Number 165
  18. 18. Presentation:GI Bleed <ul><li>GI Bleed usually is the first presentation in EHPVO/NCPF. </li></ul><ul><li>Bleeds well tolerated in presinusoidal PHT. </li></ul><ul><li>Bleeds occur night / morning (Peaks at 10.44P.M, 9.12A.M). </li></ul><ul><li>Mortality following variceal bleed in cirrhosis 20% to 30%. </li></ul>
  19. 19. Porto Systemic Hepatic Encephalopathy <ul><li>Minimal Encephalopathy (>50%) </li></ul><ul><li>Recurrent </li></ul><ul><li>Persistent </li></ul><ul><li>Acute </li></ul><ul><li>All 4 forms seen in cirrhosis. In NCPF / EHPVO, this may follow GI bleed but majority recover </li></ul>
  20. 20. <ul><li>Hypersplenism </li></ul><ul><ul><li>Thrombocytopenia - NCPF > EHPVO > Cirrhosis </li></ul></ul><ul><ul><li>Anemia </li></ul></ul><ul><li>Anemia could also be secondary to GI Bleed </li></ul>Hematological changes
  21. 21. Clinical Features <ul><li>Growth Retardation – Resistance to the action of growth hormone ( EHPVO ) </li></ul><ul><li>Portopulmonary hypertension – non-embolic pulmonary vasoconstriction in the presence of PHT. ( 4% of cirrhosis, 9% of NCPF)) </li></ul><ul><li>Binay K De IJ Gastro 1997. </li></ul><ul><li>Hepatorenal syndrome – renal insufficiency in patients with severe liver failure in the absence of any other cause of renal pathology (cirrhosis). </li></ul>
  22. 22. Clinical Features <ul><li>Hepato pulmonary syndrome – triad of PHT, intrapulmonary vascular dilatation and arterial hypoxemia (PaO2 < 70mm of Hg) In the absence of primary cardio pulmonary disease. (17.5% cirrhotics, 13.3% NCPF, 10% EHPVO) Anand A C IJ Gastro 2001. </li></ul><ul><li>Foetor Hepaticus – results from porto systemic shunting of blood, allows mercaptans to pass directly to the lungs. </li></ul><ul><li>Portal Biliopathy </li></ul>
  23. 23. Evaluation of various forms of portal hypertension Enlarged / firm / nodular Decreased vol / firm / nodular Firm Normal or Small volume Liver + + + ++ + + Spleen + / - + - - Encephalopathy +++ ++ - - Pedal oedema + + + + + 5% - 10% 5% - 10% Ascites + / - + ++ Well tolerated ++ Well tolerated GI Bleed All ages All ages 18-25 Children & occ. adults Mean age (years) HVOO Cirrhosis NCPF EHPVO Parameter
  24. 24. Features EHPVO NCPF CIRRHOSIS HVOO Centrilobular necrosis, fibrosis Reversed lobulation Necrosis, nodules fibrosis Normal / Peri Portal fibrosis Normal Liver biopsy Liver enlarged Hepatic vein thrombosis or IVC obstruction Liver coarse echoes Collaterals dilated PV ascites Splenomegaly Patent dilated PV splenomegaly collaterals PV thrombosis Cavernoma Collaterals Splenomegaly US T P decreased Glob increased (Chronic) T.P decreased Glob increased Normal Normal T. Protein A/G ratio + + + Back vein ++ + / - - / few veins on lumbar region Anterior Abdominal Veins
  25. 25. EHPVO <ul><li>Most common cause of PHT in children in India. </li></ul><ul><li>Usual presentation is UGIB(>80%) </li></ul><ul><li>Asymptomatic splenomegaly(<10%) </li></ul><ul><li>Pain LHC. </li></ul><ul><li>Age of presentation :4-7 yrs. </li></ul><ul><li>Triggered by respiratory infection </li></ul>
  26. 26. Causes of EHPVO <ul><li>Portal thrombosis :Infections </li></ul><ul><li>Umbilical sepsis(10-22%) </li></ul><ul><li>Neonatal Sepsis </li></ul><ul><li>Intra abd infections </li></ul><ul><li>NEC </li></ul><ul><li>Acute appendicitis </li></ul><ul><li>Peritonitis </li></ul><ul><li>Recurrent gut infection. </li></ul>
  27. 27. Causes of EHPVO <ul><li>Hypercoagulable states </li></ul><ul><li>Protein C def </li></ul><ul><li>Protein S def </li></ul><ul><li>Anti thrombin III def </li></ul><ul><li>Congenital </li></ul><ul><li>Trauma </li></ul><ul><li>Invasion by tumours </li></ul><ul><li>Idiopathic </li></ul>
  28. 28. Natural history of EHPVO <ul><li>Recurrent well tolerated major GI bleeds. </li></ul><ul><li>Occasionally minor bleeds presenting as occult blood loss. </li></ul><ul><li>Frequency of bleed decreases as child grows older especially after puberty. </li></ul>
  29. 29. Complications of EHPVO <ul><li>Growth retardation. </li></ul><ul><li>Delay in sexual development. </li></ul><ul><li>Ano rectal varices. </li></ul><ul><li>Hypersplenism. </li></ul><ul><li>Portal Biliopathy </li></ul><ul><li>?chronic liver disease </li></ul>
  30. 30. Diagnosis of PHT <ul><li>Clinical </li></ul><ul><li>Upper GI Endoscopy </li></ul><ul><li>Ultrasound/Doppler </li></ul>
  31. 31. ENDOSCOPY <ul><li>Site,Grade </li></ul><ul><li>Predictors of bleed </li></ul><ul><li>Portal hypertensive </li></ul><ul><li>gastropathy </li></ul>
  32. 32. <ul><li>Liver size and echogenicity. </li></ul><ul><li>Portal vein-visualization,size </li></ul><ul><li>cavernomatous malformation,phasic </li></ul><ul><li>variations with respiration. </li></ul><ul><li>Ascites </li></ul><ul><li>Collaterals </li></ul><ul><li>Splenomegaly </li></ul>Ultrasound&Doppler
  33. 33. Ultrasound and Doppler <ul><li>Liver echotexture: </li></ul><ul><li>Cirrhosis: coarse </li></ul><ul><li>EHPVO:Normal </li></ul><ul><li>Portal vein: </li></ul><ul><li>Cirrhosis:Portal Vein dilated>10mm </li></ul><ul><li>EHPVO:portal vein not visualised,replaced by a fibrous cord,cavernomatous malformation. </li></ul>
  34. 34. Other features on US <ul><li>Variation of splenic and SMV diameter with respiration:Normally increases but not in PHT </li></ul><ul><li>Thickness of lesser omentum:Ratio of omental thickness to diameter of aorta >1.7 in PHT </li></ul><ul><li>Direction of portal flow:Normal is hepato petal in severe cirrhosis it may be hepato fugal. </li></ul><ul><li>Presence of collaterals:Gastric,lieno renal.(left renal vein may appear wider) </li></ul>
  35. 35. Measurement of Variceal Pressure <ul><li>Direct puncture of varix </li></ul><ul><li>Indirect – pressure gauge or manometric capsule mounted on endoscope </li></ul><ul><li>High Variceal Pressure–increased risk of Hghe </li></ul>
  36. 36. Hepatic Venous Pressure Gradient <ul><li>HVPG </li></ul><ul><li>WHVP – FHVP = HVPG </li></ul><ul><li>HVPG 5-7mmHg </li></ul><ul><li>WHVP – Pressure distal to inflated balloon in hepatic venous radicle </li></ul><ul><li>FHVP – Pressure after deflation </li></ul>
  37. 37. Management of variceal bleed <ul><li>Pharmacotherapy </li></ul><ul><li>Endosopy-EST,EVL. </li></ul><ul><li>Surgery </li></ul>
  38. 38. Management of EHPVO <ul><li>Without bleed </li></ul><ul><li>With Bleed </li></ul><ul><li>Acute massive </li></ul><ul><li>Following bleed . </li></ul>
  39. 39. EHPVO :without bleed . <ul><li>Medical :Primary prophylaxis:Non selective B blockers 1 mg/kg/day. </li></ul><ul><li>Endoscopic :EVL if there are predictors of bleed(Grade II or IV varices,daughter varices,CR spots,hemocystic spots) No EST </li></ul><ul><li>Surgical :No role unless child presents with massive splenomegaly and hyper splenism </li></ul>
  40. 40. EHPVO with bleed <ul><li>Medical :Resuscitation,vasoactives,vasodilators. </li></ul><ul><li>Octreotide and somatostatin recommended. </li></ul><ul><li>Endoscopic :Endoscopic sclerotherapy or Endoscopic variceal ligation. </li></ul><ul><li>Surgical :Devas procedure.Suguira, </li></ul><ul><li>Sadasivam,ModifiedTanner </li></ul>
  41. 41. EHPVO following bleed . <ul><li>Medical :Continue b blockers. </li></ul><ul><li>Endoscopic :regular EST till varices are sclerosed </li></ul><ul><li>Surgery :Shunt procedures </li></ul>
  42. 42. Indications for surgery in EHPVO <ul><li>Devascularisation and decompression surgeries </li></ul><ul><li>Failure of EST. </li></ul><ul><li>Hypersplenism. </li></ul><ul><li>Child living in remote areas or with rare blood groups. </li></ul><ul><li>?Growth retardation. </li></ul>
  43. 43. Reasons for not advocating early surgery in EHPVO <ul><li>Natural history of the disease. </li></ul><ul><li>Veins may be too thin for good anastomosis. </li></ul><ul><li>Chance of thrombosis at operated site. </li></ul>
  44. 44. TIPSS <ul><li>Trans jugular Intra hepatic Porto Systemic Shunt </li></ul><ul><li>Helps in resistant ascites ,massive bleeds. </li></ul><ul><li>Bridge before liver Tx. </li></ul><ul><li>HVPG,Liver Bx can also be done at the same time. </li></ul><ul><li>Cannot be done in EHPVO. </li></ul>
  45. 45. Thank You Thank you

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