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My baby still appears
jaundice at 1 month old
Chris Cheung
MBBS V O&G Rotation 5
Causes of neonatal jaundice
<24 hours of age 24 hours to 2 weeks of age > 2 weeks of age (persistent
neonatal jaundice)
Congenital infection
Hemolytic disorders
• Rhesus incompatibility
• ABO incompatibility
• G6PD deficiency
• Spherocytosis
• Pyruvate kinase deficiency
Physiological jaundice
Breast milk jaundice
Dehydration
Infections (esp. UTI)
Rarer causes: Crigler-Najjar
syndrome
Unconjugated:
- Breast milk jaundice
- Infection (esp. UTI)
- Hypothyroidism
- Hemolytic anemia
- High GI obstruction (e.g.
pyloric stenosis)
Conjugated:
- Bile duct obstruction (biliary
atresia, choledochal cyst)
- Neonatal hepatitis
Clinical assessment of persistent
neonatal jaundice
 History
 Onset of jaundice
 Dietary and bowel habits
 Urine and stool color
 Any gestation problems
 Any other symptoms of the baby
 Fever
 Respiratory distress
 Family history
Clinical assessment of persistent
neonatal jaundice
 Physical examination
 Vitals: respiratory rate, temperature
 Petechiae?
 Hepatosplenomegaly
 Assess severity of jaundice:
 Press skin to assess jaundice
 Progresses from head to limbs
 Assess urine
 Dark color?
 Purulent?
Laboratory investigations for persistent
neonatal jaundice
 Hematology:
 ABO + Rh testing
 CBC: look for anemia and infections
 Direct Coombs Test
 LFT
 Clotting profile
 Bilirubin measurements:
 Ways to measure:
 Mild: transcutaneous bilirubinometry
 Moderate to severe: Serum bilirubin
 Look for: bilirubin fractions and levels
Imaging in persistent neonatal jaundice
 USG: liver and bile ducts
 Radionuclide scanning (HIDA):
 Indicated if extrahepatic biliary atresia is suspected
Neonatal liver disease
 URGENCY to diagnose – early diagnosis and management improves prognosis
 Persistent neonatal jaundice
 Raised conjugated bilirubin (>20 umol/L)
 Common clinical features:
 Pale stool
 Dark urine
 Bleeding tendency
 Failure to thrive
Biliary atresia
 Normal birth-weight but fails to thrive as disease progresses
 Clinical:
 Picture of obstructive jaundice
 Hepatosplenomegaly
 Ultrasound features:
 Triangular cord sign
 Large hepatic arterial caliber
 Gall bladder ghost triad:
 Atretic gallbladder, length less than 19 mm
 Irregular or lobular contour
 Lack of smooth/complete echogenic mucosal lining with an indistinct wall
Kasai classification
 Type I: obliteration of common bile duct
(patent cystic and common hepatic duct)
 Type II
 IIa: obliteration of common hepatic duct
(patent cystic and common bile duct),
sometimes with a cyst at hilum hence
termed cystic biliary atresia
 IIb: obliteration of common hepatic duct,
cystic and common bile duct
 Type III: obliteration of left and right
main hepatic ducts at the level of porta
hepatis (most common, 90%)
Kasai procedure
 Surgical bypass of the fibrotic ducts +
hepatoportoenterostomy
 Success rate ~80% if done within 60 days
after birth
 Success rate declines with increasing age
 Liver transplant if failed procedure
 Main post-operative complications:
 Malabsorption of fats and fat-soluble
vitamins
 Cholangitis
Take-home message
 Conjugated and unconjugated causes of persistent neonatal jaundice
 Identify causes through multi-system clinical features and laboratory tests
 Counsel parents of prognosis
References
 Illustrated textbook of Paediatrics 2012 - P.168-171, P.353-355
 Humphrey TM, Stringer MD. Biliary atresia: US diagnosis. Radiology. 2007;244 (3): 845-51.
doi:10.1148/radiol.2443061051
 Lee MS, Kim MJ, Lee MJ et-al. Biliary atresia: color doppler US findings in neonates and
infants. Radiology. 2009;252 (1): 282-9. doi:10.1148/radiol.2522080923
 Kanegawa K, Akasaka Y, Kitamura E et-al. Sonographic diagnosis of biliary atresia in pediatric
patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J
Roentgenol. 2003;181 (5): 1387-90. AJR Am J Roentgenol
 Tan Kendrick AP, Phua KB, Ooi BC et-al. Biliary atresia: making the diagnosis by the
gallbladder ghost triad. Pediatr Radiol. 2003;33 (5): 311-5. doi:10.1007/s00247-003-0867-z
 Lee HJ, Lee SM, Park WH et-al. Objective criteria of triangular cord sign in biliary atresia on
US scans. Radiology. 2003;229 (2): 395-400. doi:10.1148/radiol.292020472
 Sinha CK, Davenport M. Biliary atresia. J Indian Assoc Pediatr Surg. 2008;13 (2): 49-56.
doi:10.4103/0971-9261.43015

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My baby still appears jaundice at one month

  • 1. My baby still appears jaundice at 1 month old Chris Cheung MBBS V O&G Rotation 5
  • 2. Causes of neonatal jaundice <24 hours of age 24 hours to 2 weeks of age > 2 weeks of age (persistent neonatal jaundice) Congenital infection Hemolytic disorders • Rhesus incompatibility • ABO incompatibility • G6PD deficiency • Spherocytosis • Pyruvate kinase deficiency Physiological jaundice Breast milk jaundice Dehydration Infections (esp. UTI) Rarer causes: Crigler-Najjar syndrome Unconjugated: - Breast milk jaundice - Infection (esp. UTI) - Hypothyroidism - Hemolytic anemia - High GI obstruction (e.g. pyloric stenosis) Conjugated: - Bile duct obstruction (biliary atresia, choledochal cyst) - Neonatal hepatitis
  • 3. Clinical assessment of persistent neonatal jaundice  History  Onset of jaundice  Dietary and bowel habits  Urine and stool color  Any gestation problems  Any other symptoms of the baby  Fever  Respiratory distress  Family history
  • 4. Clinical assessment of persistent neonatal jaundice  Physical examination  Vitals: respiratory rate, temperature  Petechiae?  Hepatosplenomegaly  Assess severity of jaundice:  Press skin to assess jaundice  Progresses from head to limbs  Assess urine  Dark color?  Purulent?
  • 5. Laboratory investigations for persistent neonatal jaundice  Hematology:  ABO + Rh testing  CBC: look for anemia and infections  Direct Coombs Test  LFT  Clotting profile  Bilirubin measurements:  Ways to measure:  Mild: transcutaneous bilirubinometry  Moderate to severe: Serum bilirubin  Look for: bilirubin fractions and levels
  • 6. Imaging in persistent neonatal jaundice  USG: liver and bile ducts  Radionuclide scanning (HIDA):  Indicated if extrahepatic biliary atresia is suspected
  • 7. Neonatal liver disease  URGENCY to diagnose – early diagnosis and management improves prognosis  Persistent neonatal jaundice  Raised conjugated bilirubin (>20 umol/L)  Common clinical features:  Pale stool  Dark urine  Bleeding tendency  Failure to thrive
  • 8. Biliary atresia  Normal birth-weight but fails to thrive as disease progresses  Clinical:  Picture of obstructive jaundice  Hepatosplenomegaly  Ultrasound features:  Triangular cord sign  Large hepatic arterial caliber  Gall bladder ghost triad:  Atretic gallbladder, length less than 19 mm  Irregular or lobular contour  Lack of smooth/complete echogenic mucosal lining with an indistinct wall
  • 9. Kasai classification  Type I: obliteration of common bile duct (patent cystic and common hepatic duct)  Type II  IIa: obliteration of common hepatic duct (patent cystic and common bile duct), sometimes with a cyst at hilum hence termed cystic biliary atresia  IIb: obliteration of common hepatic duct, cystic and common bile duct  Type III: obliteration of left and right main hepatic ducts at the level of porta hepatis (most common, 90%)
  • 10. Kasai procedure  Surgical bypass of the fibrotic ducts + hepatoportoenterostomy  Success rate ~80% if done within 60 days after birth  Success rate declines with increasing age  Liver transplant if failed procedure  Main post-operative complications:  Malabsorption of fats and fat-soluble vitamins  Cholangitis
  • 11. Take-home message  Conjugated and unconjugated causes of persistent neonatal jaundice  Identify causes through multi-system clinical features and laboratory tests  Counsel parents of prognosis
  • 12. References  Illustrated textbook of Paediatrics 2012 - P.168-171, P.353-355  Humphrey TM, Stringer MD. Biliary atresia: US diagnosis. Radiology. 2007;244 (3): 845-51. doi:10.1148/radiol.2443061051  Lee MS, Kim MJ, Lee MJ et-al. Biliary atresia: color doppler US findings in neonates and infants. Radiology. 2009;252 (1): 282-9. doi:10.1148/radiol.2522080923  Kanegawa K, Akasaka Y, Kitamura E et-al. Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J Roentgenol. 2003;181 (5): 1387-90. AJR Am J Roentgenol  Tan Kendrick AP, Phua KB, Ooi BC et-al. Biliary atresia: making the diagnosis by the gallbladder ghost triad. Pediatr Radiol. 2003;33 (5): 311-5. doi:10.1007/s00247-003-0867-z  Lee HJ, Lee SM, Park WH et-al. Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology. 2003;229 (2): 395-400. doi:10.1148/radiol.292020472  Sinha CK, Davenport M. Biliary atresia. J Indian Assoc Pediatr Surg. 2008;13 (2): 49-56. doi:10.4103/0971-9261.43015

Editor's Notes

  1. Congenital infection: bilirubin is conjugated and the infants have other abnormal clinical signs, such as growth restriction, hepatosplenomegaly Rhesus hemolytic disease – usually identified antenatally and monitored and treated if necessary ABO incompatibility – nowadays more common, most ABO antibodies are IgM and they cannot go across the placenta, but some group O women have an IgG anti-A-hemolysin in the blood which can cross the placenta and hemolyse the red cells of group A infant. However, it is less severe than Rhesus disease Neonatal hepatitis syndrome: can be caused by inborn errors of metabolism, such as a1-antitrypsin deficiency, cystic fibrosis, congenital infections
  2. Gestation: lower treatment threshold if baby is pre-term
  3. Gestation: lower treatment threshold if baby is pre-term Usually, a total serum bilirubin level test is the only one required in an infant with moderate jaundice who presents on the typical second or third day of life without a history and physical findings suggestive of a pathologic process. Measurement of bilirubin fractions (conjugated vs unconjugated) in serum is not usually required in infants who present as described above. However, in infants who have hepatosplenomegaly, petechiae, thrombocytopenia, or other findings suggestive of hepatobiliary disease, metabolic disorder, or congenital infection, early measurement of bilirubin fractions is suggested. The same may apply to infants who remain jaundiced beyond the first 7-10 days of life, and to infants whose total serum bilirubin levels repeatedly rebound following treatment.