Neonatal cholestasis

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Neonatal cholestasis

  1. 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. 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. 3. • • • • • 1 month 20 days old girl child 1st by birth order Product of a non consanguineous marriage Maratha community Exclusively breastfed
  4. 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. 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. 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. 7. *Permission has been taken from parents to show eyes for icterus.
  8. 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. 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. 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. 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. 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. 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. 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. 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. 16. Triangular cord sign

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