SYMPTOMATIC CHOLELITHIASIS AS THE PRESENTATION OF PEDIATRIC PRIMARY SCLEROSING CHOLANGITIS - CASE SERIES AND...

Area of Science:

• Pediatric Hepatology
• Gastroenterology
• Rare Diseases

Background:

• Primary sclerosing cholangitis (PSC) is a rare pediatric hepatobiliary disorder with an unclear etiology.
• Data on pediatric PSC characteristics and natural history were limited prior to recent multicenter studies.
• Symptomatic cholelithiasis has not been previously documented as an initial presentation of PSC.

Observation:

• This study investigated three pediatric patients diagnosed with PSC presenting with symptomatic cholelithiasis between 2014-2020.
• Patient ages ranged from 10 to 12 years, with two experiencing subacute symptom onset preceding cholelithiasis.
• Histological findings from liver biopsies confirmed PSC in all three cases.

Findings:

• Two patients underwent cholecystectomy without complications; one was managed conservatively.
• No associated inflammatory bowel disease (IBD) was observed in these pediatric PSC patients.
• Mean follow-up duration was 3.4 years, with no reported surgical complications.

Implications:

• Highlights symptomatic cholelithiasis as a potential, previously unreported, presentation of PSC in children.
• Emphasizes the need to consider underlying hepatobiliary disorders in pediatric patients with unexplained or atypical cholelithiasis.
• Underscores the importance of early diagnosis and management of pediatric PSC, even with unusual initial symptoms.