A newborn infant presents with this right knee lesion at birth. What is the best next step in his management?

 

 

[size=20px]a Observation

 

b Propranolol

 

c Sirolimus

 

d Radiation therapy

 

e Wide excision[/size]

A

A

A

A

A

A

Correct answer

A Observation

 

The lesion pictured is an example of a rapidly involuting congenital hemangioma (RICH). Congenital hemangiomas are present at birth. They may be recognized in utero and may be safely observed. There are three subtypes, rapidly involuting congenital hemangioma (RICH), which regress rapidly in the first year of life. Noninvoluting congenital hemangioma (NICH) which does not regress, and partially involuting congenital hemangioma (PICH), which shares features of both types. Large rapidly involuting congenital hemangioma may ulcerate and bleed. Thrombocytopenia and transient coagulopathy may be observed in the first few weeks of life with large RICH, but these issues are usually self-limited. MRI/MRA may help clarify the diagnosis, where these lesions demonstrate large flow voids, heterogenous enhancement and hyperintensity of T2-weighted sequences. If there is diagnostic uncertainty, a biopsy may be performed but should be done at the periphery of the tumor where the histologic architecture is preserved. Immunohistochemistry reveals endothelial cells that are GLUT-1 protein negative in comparison to infantile hemangiomas that are GLUT-1 positive.

 

The management of congenital hemangiomas is expectant, so radiation therapy and wide excision are not required. Propranolol is utilized as first line treatment for infantile hemangiomas that require therapy. Sirolimus is an mTOR inhibitor that is used to treat lymphatic malformations.