Infants born with kaposiform hemangioendothelioma, or KHE, often require treatment to minimize the impact of this rare congenital vascular tumor. Although these tumors are benign, they can have complications and put the child at higher risk for Kasabach-Merritt phenomenon (KMP), which can lead to a bleeding or clotting blood disorder. At the Vascular Birthmark Institute, our expertise in treating vascular abnormalities can help children with KHE overcome this condition and lead healthy, happy lives.
KHE only occurs in about one in 100,000 infants and is usually present at birth but may appear within a few days after delivery. The tumor can be superficial, with a bluish or purple coloring that looks like a birthmark, with tightened skin over the area. Most KHEs are in the abdomen area, but they can appear on the head, neck or limbs. Diagnosis of this condition is needed, using physical examination, blood tests, MRI and possibly a biopsy.
Risks of KHEs and Options for Treatment
KHEs can put a child at higher risk for liver and other types of organ damage, as well as platelet destruction if KMP is present. The location of the tumor and the impact on nearby organs can influence the risk of health complications. Treatment options can include:
- Vincristine – This is a popular option for treating KHEs. This is a chemotherapeutic agent that is administered through a central line on a weekly basis to diminish the tumor.
- Steroids – Limited treatment with steroids can limit growth of a KHE and even shrink the tumor. However, our team is careful with the use of steroids for treatment in children due to the possible side effects.
- Surgery – In aggressive cases of KHEs, surgery may be recommended.
At the Vascular Birthmark Institute, we understand that every case is unique and needs a customized approach for treatment. With kaposiform hemangioendothelioma patients, we carefully observe the tumor and determine the best course of treatment based on our extensive expertise and training in this specialized medical field. As with all our patients, we work closely with our patients and their families to find the best treatment option that will have the least risk and highest benefits. Contact us today to schedule a consultation with our doctors.