Neonatal cholestasis : How to proceed?
Avinash Khade - LTMGH, Mumbai
S K Yachha – Pediatric Gastroenterologist
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
Neonatal cholestasis –
How to proceed?
Dr. Avinash Khade
Under guidance of
Dr. Alka Jadhav
L.T.T.M.C. SION, MUMBAI 22
1 month 20 days old girl child
1st by birth order
Product of a non consanguineous marriage
• Progressively increasing yellowish
discoloration of skin and eyes since day 5 of
• Intermittent Clay colored stools
• Excessive irritability
• No other significant history
• 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.
• No h/o fever, lethargy, irritability, refusal to
• No h/o convulsion
• No h/s/o defective vision, hearing
• No h/o recurrent respiratory infections.
• No h/o constipation.
• No h/o abdominal distension, bleeding from
any sites, altered sensorium
*Permission has been taken from parents to show eyes for icterus.
• 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.
• TORCH screening revealed
CMV IgM positivity (2.34).
• Ophthal for chorio-retinitis normal.
• USG brain : No Hydrocephalus
• BERA - awaited
• CMV -viral load awaited
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.
• 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.
• 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
• She was subsequently started on Gancyclovir for CMV
• 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
• 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
How to do it ?
3 wk baby, jaundice
Does the urine stain diapers: yes
● What is the stool color ?
Worried, fast track
A few but precise investigations
Consider biliary atresia
Ultrasonography: best bet for choledochal cyst
Liver biopsy: best for diagnosis
± HIDA scan: to rule out biliary atresia
Endoscopic retrograde cholangiography