A retrospective study (A Alhammad et al. JPGN 2016; 62: 226-32) of 55 children from 1999-2014 examined the frequency of a solitary hepatic nodule adjacent to the right portal vein as a potential diagnostic finding for Alagille syndrome. Only 39 had appropriate imaging to examine.
- In 12 (of 39) focal hepatic lesions were evident (11 were solitary).
- The median diameter was 8.1 cm.
- In those with pathology review, the cases were suggestive of a regenerative nodule.
- In all but one case, the alpha-fetoprotein levels were normal.
- 10 of these lesions were adjacent to the right portal vein.
One other point from the discussion:
- ~21% of patients with Alagille require liver transplantation, primarily for unremitting cholestasis and pruritus
My take: Recognition of this entity will help avoid mistaking this lesion for hepatocellular carcinoma.
Related blog posts:
- Severe Pruritus with Alagille Syndrome | gutsandgrowth
- AASLD/NASPGHAN 2014 Guidelines for Evaluation … – gutsandgrowth
- Learning a lot from ChiLDREN (part 1) | gutsandgrowth
- Alagille and Liver Transplantation