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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
Neonatal cholestasis –
How to proceed?
Dr. Avinash Khade
Registrar,
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
Maratha community
Exclusively breastfed
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
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.
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
*Permission has been taken from parents to show eyes for icterus.
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.
• 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
biliary cirrhosis
• She was subsequently started on Gancyclovir for CMV
hepatitis.
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
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
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
Triangular cord sign

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Neonatal cholestasis

  • 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. • • • • • 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