Primary Sclerosing Cholangitis in Children: A Histologic Follow-up Study

Abstract

Primary sclerosing cholangitis (PSC) is rare in the pediatric population. Little information exists on the progression of the disease in children. This study evaluated the experience with PSC at the Children's Hospital of Philadelphia over the past 20 years, with an emphasis on the histologic features at presentation, during disease progression, and after liver transplantation. We retrospectively reviewed the medical records of patients diagnosed with PSC between 1981 and 2001. Nineteen patients met the inclusion criteria with classic radiographic evidence of PSC. One additional patient with normal biliary imaging was subsequently diagnosed at the time of liver transplantation. The 20 patients with PSC (14 males) ranged in age from 1 month to 15 years at time of presentation. Inflammatory bowel disease was evident in 50% of patients. Initial histology revealed advanced disease (bridging fibrosis or cirrhosis) in 13 patients and an earlier histologic stage in 7 patients. Of the latter, 3 remain stable, 2 required transplantation, and 2 were lost to follow-up. Seven of the 13 patients with advanced disease required orthotopic liver transplantation, and 3 of these subsequently showed recurrence of primary disease in the allograft. Thus, most subjects presented insidiously at an advanced stage that required transplantation. Subjects who presented at an earlier stage progressed or remained stable, but the histologic findings at diagnosis were generally not predictive of disease progression. Recurrence of PSC after transplantation is a significant complication in children.

Primary sclerosing cholangitis - What is the difference between east and west?

“…PSC is more commonly a disease of adults, with patients typically presenting during the 4th and 5th decades [1][2][3] , although it has been reported to occur in the pediatric age group [1] . Patients typically present with pruritus and fatigue at the early stages of the disease, although patients with incidental elevated liver enzymes may be diagnosed earlier.…”

Pediatric hepatobiliary disease

“…Batres et al [4] presented the 20-year experience of patients with PSC at the Children's Hospital of Philadelphia. They retrospectively reviewed the medical records of 64 patients with clinical findings suggestive of PSC cared for from 1981 to 2001.…”

Pathology of Pediatric Gastrointestinal and Liver Disease

“…As in adults, there is a close association with chronic infl ammatory bowel disease, with predominance of ulcerative colitis (Batres et al 2005 ). It is probably fair to state that primary sclerosing cholangitis is underestimated and, in many instances, unrecognized in children.…”

“…10.14 a ) (Batres et al 2005 ). The earliest changes are frequently characterized by a "cholangitic" picture, with bile duct and ductular proliferation and a portal infl ammatory infi ltrate including lymphocytes, plasma cells, and neutrophils ( Fig.…”

“…Liver transplantation is a successful treatment modality for advance liver disease. 10.17 ) (Batres et al 2005 ;Feldstein et al 2003 ). 10.14 Primary sclerosing cholangitis.…”