a case of ascites and hepatomegaly
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Case of Ascites and Hepatomegaly
By :DR. Jigisha Patel2nd Yr Resident
C.U.Shah Medical College,
Surendranagar
Moderator: Dr. Aabha NagralJaslok Hospital & Research Centre
HISTORY 1 yr old girl , FTND, B/O NCM with Birth Wt. 3kg presented first at age of 8 months with Diarrhea for 10 days, 10-12 episodes a day, watery, without blood or mucus associated with
reduced urination, lethargy, needed hospitalisation and IV fluids Rapidly progressive generalised
abdominal distension 3 days later
Negative history No h/o fever, vomiting or jaundice No h/o acidosis/ breathlessness/
hypoglycemia in neonatal period No history s/o chronic liver disease No h/o tuberculosis in family
Requiring repeated paracentesis every 15 days despite diuretics & was referred to us
EXAMINATION AT 1 YEAR Wt 7.5kg (below 5th centile); Length 71cm (3rd to 25th
centile) Vitals stable Hepatojugular Reflux absent Mild Pallor + No icterus / LNs /edema ft / clubbing / cyanosis/
stigmata of chronic liver disease
Per abdomen Hepatomegaly 3 cm, firm, non tender
with Liver span of 9.5 cm Moderate ascites (shifting dullness) Prominent veins around umbilicus and
flanks
CVS examination – Normal
SUMMARIZING…
1 year old child
Presentation at 8 months of age
H/o Diarrhea followed by progressive ascites
Refractory Ascites, needing repeated paracentesis
Hepatomegaly, prominent abdominal wall veins
HOW IS ASCITES IN CHRONIC LIVER DISEASE MANAGED?
Diet Diuretics Paracentesis
What Is Refractory Ascites? Management of refractory ascites?
DIFFERENTIAL DIAGNOSIS
Hepatic Vein Outflow Tract Obstruction (Budd-Chiari Syndrome)
Cirrhosis with portal hypertension
Constrictive Pericarditis
Peritoneal TB
HOW TO INVESTIGATE?????
INVESTIGATIONS AT 1 YEAR
Hemoglobin (g/dl) 10.6 Total Bil (mg/dl)
2
WBC/mm3 14220 Direct Bil (mg/dl)
1.2
Platelet /mm3 3 Lac AST (40 IU/L) 96
Creatinine (mg/dL) 0.7 ALT (40 IU/L) 85
Electrolytes (mEq/L) 138/4.3/110 ALP (99-150) 125
Albumin (g/dl) 2.9 GGT (6-19 IU/L)
46
Globulin (g/dl) 3.1 INR 1.2
USG ABDOMEN hepatomegaly(9.5cm) with nodularity caudate lobe enlargement moderate ascites hepatofugal flow in PV with PV diameter 8 mm Spleen size 9 cm
INVESTIGATIONS Viral Markers – HbsAg, Anti HCV
Negative Autoimmune Markers – ANA, Anti SMA
Negative
Ascitic fluid examination- WBC 108 cells/mm3, Lympho 90%, Neutr 10% Total protein 2.7 gm%, albumin 1.4 gm
% SAAG 1.5 (>1.1) Therefore ascites was secondary to portal
hypertension Whats next???
HEPATIC VENOUS DOPPLER
Short stenosis of RHV Veno-Venous Collaterals
Cord like LHV Veno-Venous collaterals
Thrombophilia profile negative
Protein CProtein SAnti Thrombin 3Homocysteine levelsLupus anticoagulant & Anti phospholipid Antibody
JAK2 gene mutationFactor 5 leidenprothrombin gene mutation
FINAL DIAGNOSIS
Budd Chiari Syndrome (all 3 hepatic veins)
Diarrhea Causing Dehydration Leads To Formation Of Thrombosis.
HOW TO MANAGE FURTHER ?
HEPATIC VENOGRAPHY LEFT AND MIDDLE HEPATIC VEINS COULD NOT BE CANNULATED,
RIGHT HEPATIC VEIN SHOWED OSTIAL BLOCK - DILATED (VENOPLASTY) AND MULTIPLE VENO-VENOUS COLLATERALS
PRE DILATION GRADIENT (20 MM HG) & POST DILATION (3 MM HG)
RHVballoon venoplasty Veno-venous collaterals
PERI AND POST VENOPLASTY PROGRESS Anticoagulation peri and post procedure
(LMWH overlap with warfarin- target INR 2-3) Monitor USG doppler 3 monthly CBC, LFTs 3 monthly
Resolution of ascites within 10 days At 6 months post procedure - catch up height
and weight above 50th percentile Milestones – improved – walking without
support, talking monosyllables
Regular in follow up but noncompliant with INR monitoring (1.5-1.8)
AT 9 MONTHS POST VENOPLASTY
Presented With An Episode Of Massive Hematemesis
O/E She Had • 2 Cm Enlarged Liver• 2 Cm Enlarged Spleen• No Ascites• Vitals- Pulse-130/Min Blood Pressure – 86/58 mmHg Done endoscopy found variceal haemorrhage Treated with sclerotherapy(Ethoxysclerol)
INVESTIGATIONSBaseline (at venoplasty)
3 Months
6 Months 9 Months(variceal bleed)
Hemoglobin 10.6 g/dl 11.2 12.5 9WBC 14220/mm3 13400 11270 12890Platelet 3 Lac 3.5 3.7 3.1Creatinine (g/dl) 0.7 0.7 0.6 0.9Electrolytes 138/4.3/110 N N NAlbumin (g/dl) 2.9 3.3 3.5 3.0Globulin (g/dl) 3.1 3.2 3.3 3.2TotalBil (mg/dl) 2 1 0.9 1.4Direct (mg/dl) 1.2 0.6 0.5 1.0AST (40 IU/L) 96 55 50 88ALT (40 IU/L) 85 60 53 96ALP (99-150 IU/L)
125 131 133 139
GGT (6-19 IU/L) 46 51 42 50INR 1.2 1.9 1.7 1.8
REPEAT HEPATIC VENOGRAPHY(9 MONTHS POST VENOPLASTY)
High grade restenosis at RHV ostium with gradient of 22 mm Hg.
RHV stenting across ostium done and gradient dropped to 2 mm Hg
RHV stent
DIAGNOSIS & EVENTS Budd Chiari Syndrome (All 3 hepatic veins
involved) Post venoplasty and Post stenting status One episode of Variceal bleed due to
restenosis of hepatic vein
PRESENTLY... She is 2 years old, (3 months post stenting) –
has no ascites, no further bleeds Normal milestones for age Better compliance with INR monitoring Weight 12kg, Height 88cm (>50th
percentile) Liver profile near normal - Alb 3.4 gm%,
Bilirubin 1.1 mg%, ALT 30 IU, AST 25 IU Long term issues
Management of Budd Chari syndrome
Correct underlying cause Anticoagulation – usually life long Angioplasty/stenting of hepatic veins/IVC TIPSS if above fail/not possible surgical shunt when TIPSS not
available/feasible Liver transplantation if TIPSS fails or fulminant
(anti-coagulation needs to be continued) Supportive care of liver disease Long term monitoring for Hepatocellular
carcinoma
TAKE HOME MESSAGES... Budd Chiari syndrome is a treatable cause for ascites and
should not be missed (ask for Doppler in child with ascites) Consider Budd Chiari syndrome when isolated ascites
especially if out of proportion to liver dysfunction, diuretic refractory
SAAG >1.1 means portal hypertension GI bleed post stenting indicates varices, not warfarin Nodularity and ascites with variceal bleed in Budd Chiari
does not always mean cirrhosis Long term role for Radiological Intervention
(Nagral et al- JPGN 2010 Radiological intervation in budd chiari syndrome in children)
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