Rare disorder characterized by development of a
gray-brown discoloration that may persist for months in neonates undergoing phototherapy for neonatal
Combination of hepatocellular dysfunction and
increased bilirubin products from photodestruction during phototherapy.
The exact source of the pigment is unknown; it may be a photoisomer of natural
bilirubin. Phototherapy with bilirubin acts as a catalyst, forming
photoproducts from copper-bound porphyrins, causing skin discoloration.
Age and clinical course (appearance of the discoloration
with the use of phototherapy), liver biopsy (hepatocellular dysfunction with
decreased excretion of bile constituents and photooxidation products at the
level of bile canaliculi). Causative factor for the hyperbilirubinemia
should be determined by appropriate tests.
Discoloration usually improves over time. Outcome
usually is benign in cases where the hyperbilirubinemia is caused by icterus
neonatorum, although the prognosis depends on the causative liver disease.
Risk of bilirubin encephalopathy may be increased.
Exclude other causes of
discoloration (e.g., cyanosis, gray baby syndrome from chloramphenicol overdose).
Evaluate severity of hepatic dysfunction and determine the underlying cause
of impairment. Kernicterus should be excluded and appropriate treatment
instituted (exchange transfusion, phenobarbital). Preoperative
investigations should include a complete blood count, liver function tests,
coagulation studies, arterial blood gas analysis, urea, and creatinine.
Anesthetic issues related to neonatal
period should be considered (immaturity of various systems, differences in
pharmacokinetics and pharmacodynamics). Adequate hydration should be
maintained in patients with significant hyperbilirubinemia to prevent
development of renal failure. Administer vitamin K. If prothrombin time is
abnormal, clotting factors should be available. Hypotension should be avoided to
minimize the reduction in hepatic perfusion. Anesthetic management of the
neonate must take into account the causative factor for the development of
hyperbilirubinemia. The perioperative risk is significantly increased in the
presence of kernicterus. Factors known to precipitate seizures in neonates should be
avoided (e.g., hypoxia, hypercarbia, hyponatremia, hypoglycemia).
Hepatocellular dysfunction prolongs
the elimination half-life of drugs excreted via the biliary system (e.g.,
pancuronium, vecuronium). Atracurium or cisatracurium is the drug of choice
for paralysis. Halothane should be avoided in view of a theoretical risk of
hepatitis, especially since sevoflurane is available for inhalational
induction. Anesthetic agents that are proconvulsants should be avoided.
Ashley JR, Littler CM, Burgdorf WH, et al: Bronze baby
syndrome. J Am Acad Dermatol
Bertini G, Dani C, Fonda C, et al. Bronze baby syndrome and the risk of kernicterus.
Rubaltelli FF, Da Riol R, D'Amore ES, Jori G: The bronze baby syndrome: evidence of increased
tissue concentration of copper porphyrins. Acta Paediatr