Successful Surgical Excision of a Complex Vascular Lesion in an Infant with Kasabach–Merritt Syndrome

Abstract

Kasabach-Merritt syndrome, the association of a vascular lesion and consumptive coagulopathy, can represent a diagnostic and therapeutic challenge to clinicians. We describe an infant with a large complex vascular lesion of the left forearm that was successfully surgically excised. We propose surgical excision as an appropriate therapeutic option for some cases of Kasabach-Merritt syndrome.

Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon

“…27 Resection is possible in the rare instance of a small tumor. 15,28 In addition, arterial embolization may be effective in initiating regression. 29 Zukerberg et al 4 suggested that the prevalence of KHE is higher than indicated in published accounts because vascular tumors not manifested by coagulopathy and systemic involvement are less likely to be reported and examined histologically.…”

Successful surgical management of congenital Kasabach–Merritt syndrome

“…The surgical approach, however, remains a good option for small or medium‐sized lesions. Early one‐stage surgical resection (when possible) is a fast and effective treatment . It was particularly appropriate in the present case due to the pseudo‐pedicle aspect of the lesion.…”

Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult!

“…Complete surgical excision is the most effective treatment of small KHE lesions (Drolet et al 2001; George et al 2002). However, tumor site or localization may prevent this treatment option.…”