1. Neonatal cholestasis : How to proceed?
Presenter:
Avinash Khade - LTMGH, Mumbai
Moderator:
S K Yachha – Pediatric Gastroenterologist
SGPGI, Lucknow
Panelists:
Malathi Sathiyasekaran – Pediatric Gastroenterologist,
Kanchi K C Trust Hospital, Chennai
Girish Gupte - Pediatric Hepatologist,
Birmingham Children's Hospital, UK
Ashley D'cruz -Pediatric Hepatobiliary Surgeon,
Narayana Hrudayalaya, Bangalore

2. Neonatal cholestasis –
How to proceed?
Dr. Avinash Khade
Registrar,
Under guidance of
Dr. Alka Jadhav
L.T.T.M.C. SION, MUMBAI 22

3. •
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1 month 20 days old girl child
1st by birth order
Product of a non consanguineous marriage
Maratha community
Exclusively breastfed

4. Chief complaints
• Progressively increasing yellowish
discoloration of skin and eyes since day 5 of
life.
• Intermittent Clay colored stools
• Excessive irritability
• No other significant history

5. On examination
• She was deeply icteric with some pallor.
• Anthropometry wise well grown with weight
around median and length between -1SD &
median. Head circumference normal.
• Hepatomegaly with liver span of 8 cms , firm
consistency and smooth surface
• Splenomegaly of 4 cm , firm consistency.

6. History
• No h/o fever, lethargy, irritability, refusal to
feed
• No h/o convulsion
• No h/s/o defective vision, hearing
impairment.
• No h/o recurrent respiratory infections.
• No h/o constipation.
• No h/o abdominal distension, bleeding from
any sites, altered sensorium

7. *Permission has been taken from parents to show eyes for icterus.

8. Investigations
• Hemoglobin of 7.7g/dl, Total WBC count
12,700/cmm and 3.2 lakh platelets.
• LFT showed Total serum bilirubin of 7.9 mg% with
direct of 4.6 mg%.
• SGOT - 654 and SGPT- 236.
• ALP - 445
• GGT - 262.
• INR - 1.06.
• Thyroid function tests were normal.
• Urine reducing substances was negative.

9. • TORCH screening revealed
CMV IgM positivity (2.34).
• Ophthal for chorio-retinitis normal.
• USG brain : No Hydrocephalus
• BERA - awaited
• CMV -viral load awaited

10. Ultrasonography showed mild hepatomegaly
with no alteration of echo texture and normal
CBD without any malformation of the biliary
system. The gall bladder could be visualized.

11. • HIDA scan showed good uptake but poor
excretion of the same.
• MRCP was suggestive of hypoplastic gall
bladder with no visualization of LHD, RHD and
CHD and CBD, findings s/o biliary atresia.

12. • Child was operated with Kasai`s procedure.
• Liver biopsy : nodules separated by thick fibrous bands
composed of ballon hepatocytes. Intracanalicular and
ductular cholestasis. Fibrous septa shows bile duct
proliferation with chronic infiltrate s/o secondary
biliary cirrhosis
• She was subsequently started on Gancyclovir for CMV
hepatitis.

13. Messages
• We wish to present this case so as to highlight the
importance of aggressively screening for
correctable causes like biliary atresia.
• In this case, attributing the cholestatic jaundice to
CMV alone could have caused us to miss the biliary
atresia.
• It also highlights that CMV infection is very
common be it congenital or acquired , so other
possibilities should be ruled out before labeling a
diagnosis of CMV hepatitis

14. How to do it ?
3 wk baby, jaundice
Does the urine stain diapers: yes
● What is the stool color ?
●
Yellow
Confirm
Confirm
Confirm
Pale
Not sure
Worried, fast track
+
Nuclear scan
LFT
Ultrasound
Liver biopsy

15. A few but precise investigations
Pale stools
Consider biliary atresia
Ultrasonography: best bet for choledochal cyst
Liver biopsy: best for diagnosis
± HIDA scan: to rule out biliary atresia
Endoscopic retrograde cholangiography

16. Triangular cord sign