/ /

  • linkedin
  • Increase Font
  • Sharebar

    Baby boy bleeds into neck hemangioma

    The Case

    A 4-month-old boy presents with sudden onset of a massive hematoma on the right side of his neck (Figure 1) and bruising on his legs. At age 2 weeks, he was noted to have developed 4 superficial infantile hemangiomas on his scalp (Figure 2) and mid-back, as well as a 1-cm subcutaneous mass on the right side of his neck. All the lesions continued to grow, and he was started on oral propranolol at age 3 months. Although the superficial red plaques on his scalp and back stopped growing, the lesion on his neck continued to grow slowly.

    Laboratory testing

    A complete blood count (CBC) showed a platelet count of less than 10,000; depressed fibrinogen of 113 mg/dL (normal, 150-400 mg/dL); and an elevated D-dimer of 22 mg/L (normal, <0.5 mg/L).


    Kasabach-Merritt syndrome is a rare condition characterized by an enlarging vascular tumor, consumptive coagulopathy, and thrombocytopenia.1 It occurs during the first few years of life and carries a significant mortality rate due to progression to disseminated intravascular coagulation (DIC).

    Although the cause of Kasabach-Merritt syndrome is unknown, it is believed to be secondary to sequestration of platelets within the tumor and subsequent development of DIC.2 It is most commonly associated with kaposiform hemangioendothelioma (KHE) and tufted angioma.1

    Kaposiform hemangioendotheliomas, which were confirmed by biopsy in this patient, typically present as solitary soft-tissue tumors on the head, neck, or limbs that may regress over time but that often persist.3 The condition accounts for the majority of cases of Kasabach-Merritt syndrome. Tufted angiomas present as firm, brown or red nodules or plaques that are often painful and do not spontaneously regress.4

    The differential diagnosis in this case includes a subcutaneous infantile hemangioma. Infantile hemangiomas usually appear at 1 to 3 weeks, grow for 2 to 4 months, and regress during early childhood. Morphologically, they present as superficial, deep, or mixed lesions and in different patterns, including focal (round to oval plaques), multifocal (multiple focal lesions), or segmental (generally large lesions with unusual embryologic patterns). Hemangiomas that compromise function can be treated with oral propranolol, the only therapy approved by the US Food and Drug Administration (FDA).


    If Kasabach-Merritt is suspected, a CBC with differential, fibrinogen, D-dimer, prothrombin time (PT), and partial thromboplastin time (PTT) should be ordered. In addition, magnetic resonance imaging (MRI) may be done to assess the extent of the lesion. Finally, a biopsy will confirm the diagnosis.

    Next: Newborn's rash is more than skin deep

    This syndrome is best treated by a multidisciplinary team, including pediatricians, dermatologists, oncologists, radiologists, and surgeons. Administration of blood products to correct the coagulopathy and the platelet count usually is the first step. Although there is no gold standard of treatment, sirolimus has proven effective in many cases because of its antiangiogenic activity.3 Embolization or surgical removal also may be considered.

    Patient outcome

    The boy in this case required weekly fresh-frozen plasma (FFP) in order to achieve normalization of platelets and resolution of DIC. Propranolol was discontinued and oral prednisone was started without improvement. He was subsequently started on oral sirolimus (1 mg/mL), resulting in a 75% reduction in the size of the neck tumor and continued normalization without weekly FFP. He is scheduled for excision of the residual tumor shortly.


    1. Arunachalam P, Kumar VR, Swathi D. Kasabach-Merritt syndrome with large cutaneous vascular tumors. J Indian Assoc Pediatr Surg. 2012;17(1):33-36.

    2. Yadav D, Maheshwari A, Aneja S, Seth A, Chandra J. Neonatal Kasabach-Merritt phenomenon. Indian J Med Paediatr Oncol. 2011;32(4):238-241.

    3. Margolin JF, Soni HM, Pimpalwar S. Medical therapy for pediatric vascular anomalies. Semin Plast Surg. 2014;28(2):79-86.

    4. Kim T, Roh MR, Cho S, Chung KY. Kasabach-Merritt syndrome arising from tufted angioma successfully treated with systemic corticosteroid. Ann Dermatol. 2010;22(4):426-430.

    5. Dasgupta R, Fishman SJ. ISSVA classification. Semin Pediatr Surg. 2014;23:158-161. 

    Casey Ross, BA, MS4
    Ms Ross is a fourth-year medical student at the Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania.


    You must be signed in to leave a comment. Registering is fast and free!

    All comments must follow the ModernMedicine Network community rules and terms of use, and will be moderated. ModernMedicine reserves the right to use the comments we receive, in whole or in part,in any medium. See also the Terms of Use, Privacy Policy and Community FAQ.

    • No comments available


    Latest Tweets Follow