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)