Pediatr Gastroenterol Hepatol Nutr.  2016 Mar;19(1):1-11. 10.5223/pghn.2016.19.1.1.

Cholestasis beyond the Neonatal and Infancy Periods

• Khalaf R
• Phen C
• Karjoo S
• Wilsey M

• KMID: 2160470
• DOI: http://doi.org/10.5223/pghn.2016.19.1.1

Abstract

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.

Keyword

Conjugated hyperbilirubinemia; Cholestasis; Jaundice; Hepatitis; Biliary cysts; Cholelithiasis; Hepatolenticular degeneration; Alagille syndrome; Pruritus

MeSH Terms

Alagille Syndrome
Bile
Bile Canaliculi
Cholelithiasis
Cholestasis*
Diagnosis
Diagnosis, Differential
Hepatitis
Hepatolenticular Degeneration
Humans
Jaundice
Pruritus

Figure

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  Fig. 1 Expanded differential diagnosis of cholestatic disease in childhood (image courtesy of Sara Karjoo MD). PFIC: progressive familial hepatocellular cholestasis.

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  Fig. 2 Endoscopic view of impacted distal common bile duct stone protruding from the ampulla of Vater (image courtesy of David Troendle MD.

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  Fig. 3 Fluoroscopic cholangiogram showing multiple choledocholithiasis (arrows) in a dilated common bile duct (>11 mm) (image courtesy of David Troendle MD).

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  Fig. 4 Step-wise treatment protocol for cholestatic pruritus.

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