Ascites and Hypoxia
Presenter:
Manjurhusen Aghariya - Jaslok Hospital, Mumbai

Moderator:

PSG Inst. of Medical Sciences, ...
Case of ascites and hypoxia
Manjurhusen G Aghariya
Jaslok Hospital & Research Centre
History
• 3 years old girl, FTNVD, B/O non consanguinous
marriage, birth wt 2.8kg
• At the age of 8 months, Diarrhea of 10...
Examination
• Vitals stable
• Pallor +
• No icterus/lymphadenopathy/edema
feet/clubbing/cyanosis
• PA- generalised distens...
Investigations
• Hb 10.6 gm%, WBC 14220 cells/mm3, PLT 3 lacs
• T BR 2mg%, D BR 1.2mg%, SGOT 96 IU, SGPT 85 IU,
ALP 125, G...
Short stenosis of RHV

Veno-Venous Collaterals

Cord like LHV

Veno-Venous collaterals

Colour doppler findings
…contd
➢Ascitic fluid exam➢WBC 282 cells/mm3, Lympho 90%,
➢Total protein 1.7 gm%, albumin 0.7 gm%
➢SAAG 2.2 (>1.1)
➢Thromb...
Management
• Started on diuretics with anticoagulation
(LMWH overlap with warfarin- target INR 2-3)
• Ascites progressivel...
Hepatic venography- left and middle hepatic veins could not be
cannulated, right hepatic vein showed ostial block which wa...
…contd
• Her ascites improved and her diuretics were
tapered
• After 8 months of stable course, she had
recurrence of asci...
Repeat Hepatic venography
• Repeat hepatic venography showed high
grade restenosis at RHV ostium with
significant gradient...
…contd

• She was clinically better for 6 months with
good control of ascites and no variceal
bleeding
• But she was found...
…contd
• Macro Aggregated Albumin (MAA scan)
revealed Hepatopulmonary syndrome with
shunt fraction of 66%
…contd
• Advised liver transplantation but parents were
reluctant
• Again had variceal hemorrhage 4 months
after HPS diagn...
Now
• She is 3 years old, has moderate ascites
• Weight 10kg, Height 83cm (between 3rd-25th
percentiles)
• Alb 2.6 gm%, BR...
Diagnosis
• Budd chiari syndrome involving all 3 hepatic
veins
• Post venoplasty and stenting status
(probable restenosis)...
Take home message!!!
• Consider BCS in children with isolated ascites
• The ascites is out of proportion to liver dysfunct...
Take home message!!!
• Consider HPS in pts with clubbing, cyanosis and
hypoxemia.
• HPS is triad of
Liver dysfunction/PHT,...
Take home message!!!
• PELD (Pediatric End stage Liver Disease) score
Log calculation to assess liver dysfunction
Predicts...
‘From inability to let well alone
From too much zeal for the new and contempt for what is old
From putting knowledge befor...
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Ascites and hypoxia

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Ascites and hypoxia

  1. 1. Ascites and Hypoxia Presenter: Manjurhusen Aghariya - Jaslok Hospital, Mumbai Moderator: PSG Inst. of Medical Sciences, Coimbatore John Matthai - Pediatric Gastroenterologist, Panelists: Shaji Marar – Interventional Radiologist, Jaslok Hospital, Mumbai Winita Hardikar - Pediatric Hepatologist, Royal Children's Hospital, Australia S K Yachha – Pediatric Gastroenterologist , SGPGI, Lucknow
  2. 2. Case of ascites and hypoxia Manjurhusen G Aghariya Jaslok Hospital & Research Centre
  3. 3. History • 3 years old girl, FTNVD, B/O non consanguinous marriage, birth wt 2.8kg • At the age of 8 months, Diarrhea of 10 days duration,10-12 episodes a day, watery, without blood or mucus associated with reduced urination, lethargy, needed hospitalisation and IV fluids • 3 days later, generalised abdominal distension • No fever, vomiting or jaundice • Weight 7kg (between 5-10th percentile)
  4. 4. Examination • Vitals stable • Pallor + • No icterus/lymphadenopathy/edema feet/clubbing/cyanosis • PA- generalised distension, hepatomegaly 3cm, moderate ascites, few prominent veins around umbilicus • No dilated veins in flank or at back • Other systems-unremarkable @@
  5. 5. Investigations • Hb 10.6 gm%, WBC 14220 cells/mm3, PLT 3 lacs • T BR 2mg%, D BR 1.2mg%, SGOT 96 IU, SGPT 85 IU, ALP 125, GGT 46, Alb 2.9 gm%, Glob 3.1 gm%, INR 1.2, • Creat, Electrolytes- normal • USG abdomen showed hepatomegaly with moderate ascites @@
  6. 6. Short stenosis of RHV Veno-Venous Collaterals Cord like LHV Veno-Venous collaterals Colour doppler findings
  7. 7. …contd ➢Ascitic fluid exam➢WBC 282 cells/mm3, Lympho 90%, ➢Total protein 1.7 gm%, albumin 0.7 gm% ➢SAAG 2.2 (>1.1) ➢Thrombophilia profile negative (protein C/S/AT3/homocysteine levels, JAK2/factor5 leiden/prothrombin gene mutation) @@
  8. 8. Management • Started on diuretics with anticoagulation (LMWH overlap with warfarin- target INR 2-3) • Ascites progressively increased, needing paracentesis @@
  9. 9. Hepatic venography- left and middle hepatic veins could not be cannulated, right hepatic vein showed ostial block which was dilated (venoplasty) and multiple veno-venous collaterals RHV balloon venoplasty Veno-Venous collaterals
  10. 10. …contd • Her ascites improved and her diuretics were tapered • After 8 months of stable course, she had recurrence of ascites with variceal hemorrhage, which was treated with endoscopic sclerotherapy • Her INR was in therapeutic range with good compliance @@
  11. 11. Repeat Hepatic venography • Repeat hepatic venography showed high grade restenosis at RHV ostium with significant gradient and narrowed LHV • RHV stenting across ostium with LHV venoplasty done RHV stent
  12. 12. …contd • She was clinically better for 6 months with good control of ascites and no variceal bleeding • But she was found to have clubbing and hypoxemia (SpO2 83%, PO2 56 mmhg) on routine follow up, chest exam and chest xray were normal
  13. 13. …contd • Macro Aggregated Albumin (MAA scan) revealed Hepatopulmonary syndrome with shunt fraction of 66%
  14. 14. …contd • Advised liver transplantation but parents were reluctant • Again had variceal hemorrhage 4 months after HPS diagnosis, which was managed endoscopically with sclerotherapy. @@
  15. 15. Now • She is 3 years old, has moderate ascites • Weight 10kg, Height 83cm (between 3rd-25th percentiles) • Alb 2.6 gm%, BR 3.3 mg%, INR 1.9 • SGPT 40 IU, SGOT 65 IU • PELD 17 • Being considered for LDLT with mother as a potential donor
  16. 16. Diagnosis • Budd chiari syndrome involving all 3 hepatic veins • Post venoplasty and stenting status (probable restenosis) • Recurrent variceal hemorrhage • Severe hepatopulmonary syndrome • Considered for LDLT (PELD 17)
  17. 17. Take home message!!! • Consider BCS in children with isolated ascites • The ascites is out of proportion to liver dysfunction • Pedal edema implies IVC obstrn or hypoalbuminemia • SAAG >1.1 means portal hypertension • GI bleed after shunt indicates varices, not warfarin
  18. 18. Take home message!!! • Consider HPS in pts with clubbing, cyanosis and hypoxemia. • HPS is triad of Liver dysfunction/PHT, Gas exchange abnormalities (raised A-a gradient +/- hypoxemia) and Intrapulmonary vasodilatation • Contrast Echo and MAA scan are diagnostic • Treatment is supportive and liver transplant cures
  19. 19. Take home message!!! • PELD (Pediatric End stage Liver Disease) score Log calculation to assess liver dysfunction Predicts 3 months mortality Used to allocate livers by UNOS Comprises BR, INR, Albumin, Age, Growth failure • Liver transplant is curative in end stage disease
  20. 20. ‘From inability to let well alone From too much zeal for the new and contempt for what is old From putting knowledge before wisdom, science before art, and Cleverness before common sense; From treating patients as cases; And from making the cure of the disease more grievous than the Endurance of the same, Good Lord, deliver us.’ Sir Robert Hutchison

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