Congenital hemangiomas are benign pediatric vascular anomalies that can be divided into at least 4 categories: rapidly involuting (RICH), noninvoluting (NICH), partially involuting (PICH), and fetally involuting (FICH). Congenital hemangiomas arise in utero and are differentiated based upon their behavior. The RICH subtype begins regressing within the first few weeks of life, with complete regression by 6-14 months. The NICH subtype does not regress and may enlarge over time. The PICH subtype demonstrates a mix of rapid involution in some areas and noninvolution in others. This subtype exhibits the most rapid regression from birth to 12 months. The FICH subtype presents in utero and begins regression even before birth. FICH continues to regress after birth until it is fully involuted.
With all congenital hemangiomas, patients will present with a solitary lesion at birth. Some case reports show a slight male predominance. Patients with all subtypes of congenital hemangiomas are typically asymptomatic. However, there have been reports of pain in half of patients with the NICH subtype.
All subtypes are thought to be due to underlying activating mutation in the GNAQ gene. There have been no associations as of yet with any other conditions.
Congenital hemangioma in Infant/Neonate
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Synopsis
Codes
ICD10CM:
D18.00 – Hemangioma unspecified site
SNOMEDCT:
32361000119104 – Congenital hemangioma
D18.00 – Hemangioma unspecified site
SNOMEDCT:
32361000119104 – Congenital hemangioma
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Differential Diagnosis & Pitfalls
- Infantile hemangioma – Typically not present at birth. Infantile hemangiomas demonstrate rapid growth after birth, whereas congenital hemangiomas do not. Infantile hemangioma will demonstrate GLUT-1 positivity on histopathology. Congenital hemangioma will be negative.
- Tufted angioma – Can present at birth or during infancy. It will present as a firm, violaceous plaque or papule with overlying hair.
- Kaposiform hemangioendothelioma – Present at birth or during childhood as a firm, subcutaneous mass resembling a raised ecchymosis. This is frequently associated with the Kasabach-Merritt phenomenon whereby patients will exhibit severe thrombocytopenia and coagulopathy.
- Vascular malformation (venous or arteriovenous) – These can be present at birth and will be soft and compressible. They additionally stain negative for Wilms tumor protein-1 (WT-1), whereas congenital hemangiomas will stain positive.
- Pyogenic granuloma – Congenital forms have been reported; this may be multifocal.
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Last Reviewed:10/29/2018
Last Updated:10/11/2022
Last Updated:10/11/2022
Congenital hemangioma in Infant/Neonate