Rapidly involuting congenital haemangioma RICH may present with thrombocytopenia, low fibrinogen and elevated fibrin degradation products and D-dimers. Such complications have rarely been reported. We wished to define the clinical characteristics of the thrombocytopenia and coagulopathy associated with RICH, to emphasize the transient nature of this haematological complication and to distinguish these abnormalities from true Kasabach-Merritt phenomenon KMP. We present a case series of seven patients with large RICH who presented with thrombocytopenia and coagulopathy during the first week of life.
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Rapidly involuting congenital haemangioma RICH may present with thrombocytopenia, low fibrinogen and elevated fibrin degradation products and D-dimers. Such complications have rarely been reported. We wished to define the clinical characteristics of the thrombocytopenia and coagulopathy associated with RICH, to emphasize the transient nature of this haematological complication and to distinguish these abnormalities from true Kasabach-Merritt phenomenon KMP.
We present a case series of seven patients with large RICH who presented with thrombocytopenia and coagulopathy during the first week of life. Clinical and haematological characteristics were recorded retrospectively.
Two of the patients were treated with embolization due to early signs of high-output cardiac failure; four patients received oral corticosteroids in the range of 2 mg kg -1 daily; one patient did not receive any treatment in the neonatal period, although the tumour was excised at 6 months of age. Two patients with platelet counts lower than 10 x 10 9 L -1 received a platelet transfusion.
There were no bleeding complications and only one patient presented with petechiae. However, in contrast to true KMP, these abnormal laboratory findings are self-limited and are usually not complicated by bleeding problems.
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Hemangiomas of Infancy: Epidemiology
While ulceration is a common, predominantly benign complication in infantile hemangioma, little is known about the prognosis of ulcerated CH. However, it has been observed that ulcerated CH may be complicated by life-threatening bleeding episodes. In both cases, the CHs were fed by high-flow vessels and the ensuing massive bleeding was due to superficial vessel wall erosion induced by the ulceration. Both patients were successfully treated with intravascular embolization; one patient underwent additional hemostatic surgery. Embolization is the treatment of choice in the case of severe bleeding, as the natural history of RICH is to spontaneously regress.
Hemangiomas and Vascular Malformations
Since the first edition of this book was published, much new knowledge has been gained on hemangiomas and vascular malformations. This revised and updated second edition, written by worldwide leaders in the field, fully reflects this progress. Important improvements in the treatment of hemangiomas are reviewed, including the use of beta-blockers and other medical therapies as well as advances in interventional treatments and the treatment of hemangiomas in specific locations. Vascular malformations are being investigated with increasing frequency all over the world and new challenges are emerging regarding both diagnosis and treatment.
We performed a retrospective case series of IHs with prolonged growth to further characterize these lesions and their treatment. Recognition of this subset of hemangiomas is important for clinicians, and further study of IHs may provide clues to their pathogenesis. Infantile hemangiomas are the most common tumor of infancy and have a well-described natural history of rapid growth during early infancy followed by gradual involution. Although there are other types of vascular tumors sometimes referred to under the umbrella term hemangiomas eg, lobular capillary hemangioma [pyogenic granuloma] , hereafter in this article the term hemangioma will refer to infantile hemangiomas specifically. The duration of the growth phase can be variable, but it has long been reported that most hemangiomas reach their maximal size within the first 6 to 10 months of life.
Hemangiomas and Vascular Malformations pp Cite as. Few reports about the epidemiology of infantile hemangiomas IH exist and prospective studies are missing; it is difficult to provide some data. The incidence in the general newborn population is between 1. The majority of hemangiomas occur sporadically; however, familial occurrence of IH has been reported. Although IH can be seen in all races, they are more common in Caucasian infants, and less common in those of African or Asian descent.