[MIM 141 000]

(Kaposiform hemangio-endothelioma, Hemangioma-thrombocytopenia syndrome)

Rare. Association of a vascular tumor, major thrombocytopenia and a coagulopathy. Onset usually in the first 6 months of life. This tumor is not an infantile hemangioma but a true vascular tumor the histologic picture of which is either an tufted angioma  (before the active phase or after its regression) or a kaposiform hemangio-endothelioma in association with lymphatic-like vessels (in its active phase with thrombocytopenia). Brutal growth with thrombocytopenia following the sequestration of platelets in the tumor and the consumption of fibrinogen.

On the contrary to the classic hemangiomas, the vascular tumor is negative for GLUT-1 (glucose transporter 1) and Lewis Y antigen (LeY) .

Treatments:

-        surgical: if it is possible to completely remove the tumor (often infiltrating with  hemorrhagic risk)

-        medical: vincristine, corticosteroids, interferon α are used with varying success; sirolimus seems to have dramatic effects.

Anesthetic implications:

risk of bleeding with DIC and heart failure. Do not transfuse platelets which will be immediately consumed in the tumor and make  it grow further.

References : 

-         Enjolras O, Wassef M, Mazoyer E et al. 
Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. 
J Pediatr 1997; 130: 631-40.

-         Wassef M, Vanwijck R, Clapuyt P et al. 
Vascular tumours and malformations, classification, pathology and imaging. 
Ann Chir Plast Esth 2006; 51: 263-81.

-         Enjolras O, Picard A, Soupre V. 
Congenital haemangiomas and other rare infantile vascular tumours. 
Ann Chir Plast Esth 2006; 51: 339-46.

-        Kelly M. 
Kasabach-Merritt phenomenon. 
Pediatr Clin North Am 2010; 57:1085-9.

-        Rodriguez V, Lee A, Witman PM, Anderson PA. 
Kasabach-Merritt phenomenon: case series and retrospective review of the Mayo Clinic experience.
J Pediatr Hematol Oncol 2009; 31:522-6.

-        Hara K, Yoshida T, Kajiume T, Ohno N, Kawaguchi H, Kobayashi M. 
Successful treatment of Kasabach-Merritt syndrome with vincristine and diagnosis of the hemangioma using three-dimensional imaging. 
Pediatr Hematol Oncol 2009; 26:375-80.

-        Mizushima A, Kugimiya T, Horimoto Y. 
Anesthetic problems in a very low birth weight infant with Kasabach-Merritt syndrome. 
Masui 1996; 45:1009-14.

-        Kumar S, Taneja B, Saxena KN, Kalra N. 
Anaesthetic management of a neonate with Kasabach-Merritt syndrome. 
Indian J Anaesth 2013; 57: 292-4. 

-        Tribolet S, Hoyoux C, Boon LM, Cheruy C et al.
A not so harmless mass : Kaposiform hemangioendothelioma complicated by a Kassabach-Merritt phenomenon.
Arch Pédiatr 2019, 26 :365-9

-        Chandrakantan A, Bryant BE, Hensch L, Adler AC.
Anesthetic considerations for patients with Kasabach-Merritt phenomenon.
Pediatr Anesth 2020; 30 :1155-6.

Updated: October 2020