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    Neonatal Cholestasis

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    Mateen Shukri
    1. Neonatal cholestasis requires prompt evaluation and management as some causes can be fatal if left untreated, while others may cause long-term liver damage. It is defined as prolonged elevation of conjugated bilirubin beyond 14 days of life. 2. The causes of neonatal cholestasis include obstructive causes like biliary atresia, infections, metabolic disorders, genetic syndromes, and storage diseases. Biliary atresia is a particularly urgent cause, as the prognosis depends on factors like age at diagnosis and surgery. 3. Investigations help determine the cause and include liver function tests, stool color assessment, imaging like ultrasound and HIDA scan, and specific tests for conditions

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    Neonatal Cholestasis

    Uploaded by

    Mateen Shukri
    97 views5 pages
    1. Neonatal cholestasis requires prompt evaluation and management as some causes can be fatal if left untreated, while others may cause long-term liver damage. It is defined as prolonged elevation of conjugated bilirubin beyond 14 days of life. 2. The causes of neonatal cholestasis include obstructive causes like biliary atresia, infections, metabolic disorders, genetic syndromes, and storage diseases. Biliary atresia is a particularly urgent cause, as the prognosis depends on factors like age at diagnosis and surgery. 3. Investigations help determine the cause and include liver function tests, stool color assessment, imaging like ultrasound and HIDA scan, and specific tests for conditions

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    1. Neonatal cholestasis requires prompt evaluation and management as some causes can be fatal if left untreated, while others may cause long-term liver damage. It is defined as prolonged elevation of conjugated bilirubin beyond 14 days of life. 2. The causes of neonatal cholestasis include obstructive causes like biliary atresia, infections, metabolic disorders, genetic syndromes, and storage diseases. Biliary atresia is a particularly urgent cause, as the prognosis depends on factors like age at diagnosis and surgery. 3. Investigations help determine the cause and include liver function tests, stool color assessment, imaging like ultrasound and HIDA scan, and specific tests for conditions

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    © All Rights Reserved
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    97 views5 pages

    Neonatal Cholestasis

    Uploaded by

    Mateen Shukri
    1. Neonatal cholestasis requires prompt evaluation and management as some causes can be fatal if left untreated, while others may cause long-term liver damage. It is defined as prolonged elevation of conjugated bilirubin beyond 14 days of life. 2. The causes of neonatal cholestasis include obstructive causes like biliary atresia, infections, metabolic disorders, genetic syndromes, and storage diseases. Biliary atresia is a particularly urgent cause, as the prognosis depends on factors like age at diagnosis and surgery. 3. Investigations help determine the cause and include liver function tests, stool color assessment, imaging like ultrasound and HIDA scan, and specific tests for conditions

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    © All Rights Reserved
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    of 5

    Approach to a case of Neonatal Cholestasis

    Ira Shah (Co-Incharge, Consultant Pediatrician, Pediatric Liver Clinic)


    Gunjan Narkhede (Resident in Pediatric Liver Clinic)
    Pediatric Hepatobiliary Clinic, B.J.Wadia Hospital For Children, Mumbai.
    Email: [email protected]
    Introduction:
    Neonatal cholestasis accounts for almost 30% of pediatric hepatobiliary diseases. It requires prompt
    evaluation and management as some of the causes are fatal in early life if untreated whereas others
    cause substantial morbidity by causing chronic liver disease. It is defined as prolonged elevation of
    conjugated bilirubin beyond 14 days of life (i.e. the conjugated fraction >20% of total bilirubin.).
    Conjugated Jaundice that appears within 3 months of life is called as neonatal cholestasis. History of
    jaundice with high coloured urine (often noted as yellow staining of diapers), clay coloured /light stools
    with or without hepatomegaly is suggestive of cholestasis. Thus any jaundice that persists beyond 2
    weeks of life, progresses after this time or does not regress by this time get a serum bilirubin done
    with a conjugated fraction.

    Etiology:
    The cause of neonatal cholestasis can be genetic, metabolic, infectious or undefined causing mechanical obstruction of bile flow e.g. Biliary atresia or bile acid secretion or functional impairment of
    hepatic excretory e.g. Congenital infections.
    The common causes of Neonatal cholestasis include:
    1.
    2.
    3.
    4.

    Obstructive causes: Biliary Atresia, Choledochal cysts


    Infections TORCH infections, Reo virus and Group C rotavirus infections
    Metabolic Tyrosinemia, Fructosemia, galactosemia, Hypothyroidism, Panhypopituitarism
    Genetic Alagille syndrome, Progressive familial intrahepatic cholestasis (PFIC), bile acid
    defects, Cystic fibrosis
    5. Storage Niemann Pick Disease C.
    6. Idiopathic

    Why is neonatal cholestasis an emergency?


    Neonatal cholestasis is an emergency as timely intervention can prevent liver cirrhosis and death. Biliary
    atresia is an emergency as prognosis depends on age of presentation, age of operation, expertise of the
    surgeon and the bile duct size at the time of porta. At our center, incidence of Biliary Atresia amongst
    neonatal cholestasis syndrome is 36.4%. We see about 45-50 new patients of neonatal cholestasis
    syndrome per year in our Pediatric Liver Clinic. The mean age of presentation of patients with biliary
    atresia is 89 days + 55.8days. (almost 3 months). Those with bile duct sizes >200microns had better
    prognosis than those with sizes 100-200 microns (Odds ratio=1.8) and <100microns (Odds ratio=3).
    Operation before 3 months had a better prognosis as compared to operation after 3 months of age.
    (Odds ratio = 2). (1)
    Other conditions such as Galactosemia and metabolic disorders are also urgent as timely intervention
    can prevent irreversible liver damage. At our center, common causes of neonatal cholestasis have
    included Biliary atresia, CMV infection, galactosemia, alagille syndrome, PFIC and idiopathic causes. Thus
    urgent diagnosis becomes essential in these conditions.

    Investigations
    Lets take a short overview of the investigations helpful in diagnosis. Investigations of preference depend
    on the clinical presentation of the patient. Usual investigations done are :
    Test

    Implication

    Serum bilirubin fractionation

    Confirms cholestasis

    Liver function tests including GGTP High GGTP with high alkaline phosphatase suggests
    obstruction
    and alkaline phosphatase
    Low GGTP with high alkaline phosphatase suggests PFIC
    Normal GGTP and normal alkaline phosphatase may suggest
    non-obstructive causes
    Indicates bile flow into intestine. Clay coloured stools
    Assessment of stool colour
    suggests obstruction
    Urine/serum bile acid measurement

    Confirms cholestasis

    USG

    Suggests/excludes choledochal cyst, may show triangular


    cord sign suggestive of biliary atresia.

    HIDA scan

    Confirms flow of bile into the intestine though absence of


    same does not confirm atresia- can occur in severe hepatitis.
    Depends on the liver function and low uptake of the tracer by
    liver due to poor function can lead to poor excretion and
    false sense of obstructions

    Specific investigations:
    Thyroxine and TSH

    Hypothyroidism, panhypopituitarism

    Sweat chloride

    Cystic fibrosis

    Urine/plasma amino
    reducing substance

    acids,urine Metabolic liver disease

    TORCH titres

    For congenital infections

    2 D Echo

    For Alagille syndrome and congenital infections

    Hearing
    and
    examination

    Ophthalmology For hearing loss and chorioretinitis respectively for congenital


    infections

    Liver biopsy

    Intra operative cholangiogram

    Most valuableBiliary atresia- bile ductular proliferation,portal/perilobular


    fibrosis, BUT BAISC ARCHITECTURE INTACT;
    Neonatal hepatitis- severediffuse hepatocellular
    disease,DISTORTION OF LOBULAR ARCHITECTURE,marked
    infiltration with inflammatory cells,focal hepatocellular
    necrosis.
    A low threshold should be kept for IOC in patients with clay
    stools without waiting for HIDA as HIDA is very often

    inconclusive.

    How to differentiate biliary atresia from non-obstructive causes?


    This simple table will help to differentiate between obstructive and non-obstructive causes. However it
    may not be the same all the time and thus an individual based approach will be essential.
    Obstructive causes

    Non-obstructive causes

    Full term

    Preterm/Full term

    Normal birth weight

    AGA/LBW

    No ANC/PNC complications

    ANC- fever, rash, lymphnodes

    CLAY COLOURED STOOLS

    Yellow stools

    Growing well

    Failure to thrive

    Usually isolated hepatomegaly

    Hepatosplenomegaly

    Approach to Neonatal Cholestasis:


    Consensus Report on Neonatal Cholestasis Syndrome by Pediatric Gastroenterology Subspecialty
    Chapter of Indian Academy of Pediatrics (Indian Pediatrics 2000;37: 845-851) has suggested the
    following algorithm for approach to Neonatal cholestasis. (2)

    * Babies with NCS due to infections of Herpes, Toxoplasmosis and rarely CMV may be sick, look for their
    extra hepatic manifestations. Stop milk feeds till galactosemia is ruled out. In febrile babies, look for
    malarial parasite, sepsis and UTI.
    ** This is to look for galactose in urine while on milk feeds. If reducing substances are positive, check
    urine samples with glucose stick. If negative, most likely reducing substances in urine are due to
    galactose. Treat as galactosemia. GALI-PUT should be done to confirm the diagnosis.
    However there may be instances where liver biopsy may be inconclusive due to improper reporting or
    due to evolving disease and in such instances either serial liver biopsies or urgent cholangiogram may be
    needed to find out the cause. (3). Thus based on our clinical experience of over 200 patients, where the
    only clinical marker for biliary atresia was clay stools (4) we have modified our approach to neonatal
    cholestasis as follows:

    Approach to Neonatal Cholestasis (Wadia Protocol)

    * Liver biopsy and Intraoperative cholangiogram are done depending on the reports of the tests in
    patients with yellow stools.

    Conclusion: Neonatal cholestasis syndrome is a hepatobiliary emergency and timely referral is essential
    to avoid irreversible liver damage.
    References:
    1. Sanghai Saket, Shah Ira, Bhatnagar Sushmita. Incidence and Prognostic factors associated with
    Biliary Atresia in Western India. Annals Hepatol. 2009; 8: 120-122.
    2. Pediatric Gastroenterology Subspecialty Chapter of Indian Academy of Pediatrics. Consensus
    Report on Neonatal Cholestasis Syndrome. Indian Pediatrics 2000;37: 845-851
    3. Sweta Mohanty, Ira Shah, Sushmita Bhatnagar. Evolving Biliary Atresia With Cytomegalovirus.
    Accepted For Publication In Indian Pediatrics. 2010
    4. Ira Shah, Sushmita Bhatnagar. Clinical and Laboratory Markers predictive of Biliary Atresia.
    Mahapedicon 2008, Mahabaleshwar, 14-15th November 2008.

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