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. Case of ascites and hypoxia
Manjurhusen G Aghariya
Jaslok Hospital & Research Centre
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. 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. 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. Short stenosis of RHV
Veno-Venous Collaterals
Cord like LHV
Veno-Venous collaterals
Colour doppler findings
8. Management
• Started on diuretics with anticoagulation
(LMWH overlap with warfarin- target INR 2-3)
• Ascites progressively increased, needing
paracentesis
@@
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. …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. 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. …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. …contd
• Macro Aggregated Albumin (MAA scan)
revealed Hepatopulmonary syndrome with
shunt fraction of 66%
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. 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. 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. 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. 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. 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. ‘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
