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  • Imaging Casebook
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Imaging Case Book

Congenital enormous high flow capillary hemangioma of forearm

Journal of Perinatology volume 28, pages 235–237 (2008)Cite this article

The forearm mass at MR angiography was characterized by arterial blood supply from a normal branching large left subclavian artery and subsequent rapid venous filling of large but normal venous structures. There was no simultaneous arterial and venous filling to suggest actual arteriovenous shunting. Intense, persistent homogeneous contrast enhancement of the mass occurred. The appearance of the osseous and muscular structures of the forearm were normal, and not hypertrophied. Therefore, the mass represents a juvenile capillary hemangioma with high flow. The low platelets and schistocytes suggested that a Kassabach Merritt syndrome was developing. However, the platelet levels slowly returned to normal. At 1 week of age the platelets were 263 000 mm−3. The hematocrit returned to normal, although schistocytes persisted. The patient had a slightly enlarged heart, but cardiac function remained normal and congestive heart failure did not develop. The patient was started on prednisolone and discharged from the hospital at 10 days of life with bandaging and dermal care of the large mass.

Juvenile hemangiomas are the most common tumor of infancy.1 These lesions are three times more common in females than males, are characteristically dermal in location and have a three- phase pattern of post-natal growth. They have a proliferative phase usually between 3 to 9 months of life, a variable phase of stability and an involution phase, which begins at about 18 months and is usually complete by 5 to 6 years, but may persist until 10 years of age.1, 2 It is known that hemangiomas that proliferate in utero and present as congenital masses, may involute much earlier in infancy.1 In fact, an uncommon variety of congenital hemangioma with rapid involution called rapidly involuting congenital hemangioma(RICH)3 presents as a large mass at birth, as in this patient. However, in RICH the arterial vascular supply is disorganized with small aneurysms and the contrast staining of the mass is not homogeneous.2 The angiographic appearance of RICH, may suggest a congenital fibrosarcoma. Despite the excellent prognosis of RICH, a biopsy may be needed initially because of the angiographic appearance.

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  1. Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Children's Hospital, Washington University School of Medicine, St Louis, MO, USA

    T E Herman & M J Siegel

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  1. T E Herman
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  2. M J Siegel
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Correspondence to T E Herman.

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Herman, T., Siegel, M. Congenital enormous high flow capillary hemangioma of forearm. J Perinatol 28, 235–237 (2008). https://doi.org/10.1038/sj.jp.7211901

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