Fetal intrahepatic Umbilical-Porto-Systemic venous shunts (IHUPSVS): In-utero anatomic classification

Area of Science:

• Medical Imaging
• Fetal Medicine
• Pediatric Surgery

Background:

• Congenital intrahepatic shunts disrupt fetal development by diverting oxygenated blood flow away from the liver.
• Accurate diagnosis of these shunts is crucial for managing pregnancy and ensuring proper fetal and postnatal development.
• Existing classifications for Intrahepatic Umbilical-Porto-Systemic Venous Shunts (IHUPSVS) lack standardization.

Purpose of the Study:

• To propose a novel in utero classification system for Intrahepatic Umbilical-Porto-Systemic Venous Shunts (IHUPSVS).
• To establish a standardized anatomic basis for IHUPSVS for improved clinical assessment and management.
• To facilitate future research and updates on this congenital anomaly.

Main Methods:

• A prospective study utilizing grayscale ultrasound with 2D and 3D high-definition Doppler modalities.
• IHUPSVS defined as abnormal communication between intrahepatic umbilical/portal veins and systemic circulation.
• Postnatal targeted sonography used for verification of all diagnosed shunts.

Main Results:

• Twenty-five fetuses diagnosed with IHUPSVS.
• Three primary anatomic types identified: Porto-hepatic (single/multiple), Umbilical-Porto-Hepatic (umbilical or combined), and Cavernous-aneurysmatic.
• Porto-hepatic shunts were most common (78%), followed by Umbilical-Porto-Hepatic (8%) and Cavernous-aneurysmatic (8%).

Conclusions:

• The proposed classification provides a foundational anatomic framework for IHUPSVS.
• This standardized nomenclature is essential for consistent diagnosis and reporting of IHUPSVS.
• The classification facilitates future advancements in understanding and managing this fetal anomaly.