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Papillary stenosis and sclerosing cholangitis in an immunodeficient child

D A Gremse1, J C Bucuvalas, G L Bongiovanni

  • 1Children's Hospital Research Foundation, University of Cincinnati College of Medicine, Ohio.

Gastroenterology
|June 1, 1989
PubMed

Insights

Sclerosing cholangitis and papillary stenosis, rare in children, can occur with immunodeficiency syndromes. Endoscopic sphincterotomy offers symptomatic relief for papillary stenosis in pediatric cases.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Immunology

Background:

  • Sclerosing cholangitis and papillary stenosis are inflammatory biliary conditions.
  • These conditions are infrequently observed in childhood.
  • In adults, they have been associated with acquired immunodeficiency syndrome.

Observation:

  • A 10-year-old child with a familial immunodeficiency syndrome (defective T-cell function, IgA and IgG deficiencies) presented with these conditions.
  • The child also had cryptosporidium enteritis.
  • Symptoms included fever, jaundice, right upper quadrant pain, and elevated liver enzymes.

Findings:

  • The patient experienced symptom resolution after endoscopic sphincterotomy.
  • Biochemical abnormalities, however, persisted post-procedure.
  • This case highlights that the combination of papillary stenosis and sclerosing cholangitis can manifest in children and be linked to various immunodeficiency syndromes.

Implications:

  • This pediatric case expands the known association of sclerosing cholangitis and papillary stenosis beyond acquired immunodeficiency syndrome.
  • Endoscopic sphincterotomy provides symptomatic benefit for pediatric papillary stenosis.
  • The long-term impact of sphincterotomy on sclerosing cholangitis progression in children remains undetermined.

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