From the Guidelines
A congenital hemangioma is the type of vascular malformation present in a newly born preterm infant when the lesion is present at birth. Unlike infantile hemangiomas which typically appear after birth and undergo a proliferative phase, congenital hemangiomas are fully formed at birth. These are further classified into three subtypes: Rapidly Involuting Congenital Hemangioma (RICH), which regresses completely within the first year of life; Non-Involuting Congenital Hemangioma (NICH), which persists without regression; and Partially Involuting Congenital Hemangioma (PICH), which shows partial regression. Congenital hemangiomas have distinct histological features, including negative GLUT-1 staining, which differentiates them from infantile hemangiomas 1.
Key Characteristics of Congenital Hemangiomas
- Fully formed at birth
- Negative GLUT-1 staining
- Classified into RICH, NICH, and PICH subtypes
- Management depends on subtype, location, and associated complications
Management depends on the subtype, location, and associated complications, ranging from observation for RICH lesions to potential surgical intervention for NICH lesions that cause functional impairment or cosmetic concerns. Close monitoring is essential in preterm infants as these vascular malformations can sometimes be associated with high-output cardiac failure or platelet trapping (Kasabach-Merritt phenomenon) in rare cases. The most recent guidelines for the management of infantile hemangiomas, which may also be relevant for congenital hemangiomas, recommend propranolol as the first-line treatment for problematic lesions 1.
Considerations for Preterm Infants
- Close monitoring for potential complications
- Management guided by subtype and location of the hemangioma
- Potential for high-output cardiac failure or Kasabach-Merritt phenomenon in rare cases
It's worth noting that while the provided evidence focuses on infantile hemangiomas, the key distinction between infantile and congenital hemangiomas lies in their timing of appearance and growth patterns. Congenital hemangiomas are present at birth and do not undergo the same proliferative phase as infantile hemangiomas. The classification and management strategies may overlap, but the approach should be tailored to the specific characteristics of congenital hemangiomas, as highlighted in studies from 1 and 1.
From the Research
Types of Hemangiomas in Newly Born Preterm Infants
The type of hemangioma present in a newly born preterm infant with a vascular malformation dating since birth can be either rapidly involuting congenital hemangioma (RICH) or non-involuting congenital hemangioma (NICH) 2, 3, 4.
Characteristics of RICH and NICH
- RICH is characterized by its rapid involution, often beginning in the first weeks of life 2, 3.
- NICH, on the other hand, does not involute and persists over time 2, 3, 4.
- Both types of congenital hemangiomas are benign vascular neoplasms that proliferate in utero and have completed development by birth 2, 3, 4.
Imaging Characteristics
Imaging characteristics of RICH and NICH can be distinguished from common infantile hemangiomas using ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI), and angiography 5.
- US findings of visible vessels and calcifications are statistically significant in cases of congenital hemangiomas 5.
- CT and/or MRI findings, such as well-defined limits and fat stranding, can also be used to distinguish between RICH, NICH, and common infantile hemangiomas 5.
Clinical Presentation
The clinical presentation of congenital hemangiomas can vary, with some lesions being fully developed at birth and others undergoing involution during fetal life 6.
- A new variant of congenital hemangioma, referred to as rapidly involuting congenital hemangioma with fetal involution, has been described, which arises prenatally and is nearly regressed at birth 6.