Non-Involuting Congenital Hepatic Hemangioma: Lessons from a Case Series

Area of Science:

• Pediatric Surgery
• Hepatology
• Medical Genetics

Background:

• Congenital hepatic hemangiomas (CHHs) are usually rapidly involuting.
• Non-involuting congenital hepatic hemangiomas (NICHHs) are poorly understood.
• This study focuses on the evolution and management of NICHHs.

Purpose of the Study:

• To investigate the long-term evolutionary patterns of NICHHs.
• To evaluate management strategies for NICHHs.
• To identify potential treatments for NICHHs.

Main Methods:

• Retrospective review of clinical, imaging, histological, and genetic data.
• Study included children diagnosed with NICHH between 1991 and 2022.
• NICHH defined as no involution for at least 18 months.

Main Results:

• Seven patients (5 female, 2 male) with NICHH were identified.
• Median age at diagnosis was 42 days; lesions were mostly asymptomatic and in the right lobe.
• Sirolimus treatment in 3 giant NICHH cases showed partial response or stabilization; untreated cases had varied evolution.

Conclusions:

• NICHH lesions exhibit distinct long-term evolutionary behavior.
• Accurate diagnosis and regular monitoring are vital for NICHH management.
• Sirolimus shows promise as a non-surgical option for select NICHH patients, especially those with giant lesions.