Pediatric Clinics of North America - CIPERJ

344 ADAMS & WENTZEL
Fig. 4. PHACES syndrome.
[19–21]. Some of these infants are at increased risk for stroke. Because other
more complicated hemangiomas also are identified in these patients, the
phenotypic spectrum of this syndrome currently is being followed prospectively
in an international clinical registry.
Hemangiomas should not be confused with the lesions known as KHE
and TA (Fig. 5). These latter lesions first were described in 1940 by Kasabach
and Merritt [22], who reported an infant who had thrombocytopenic
purpura resulting from what they believed was a giant capillary hemangioma.
Subsequently, the association of capillary hemangiomas with thrombocytopenia
was referred to as Kasabach-Merritt syndrome.
In 1997, two groups of investigators demonstrated that these lesions
were not true hemangiomas, rather, distinct vascular tumors diagnosed
Fig. 5. KHE: aggressive benign vascular tumor with high mortality/morbidity rate associated
with KMP.