Abstract
IntroductionHaemangiomas are vascular lesions resulting from abnormal proliferation of blood vessels. They are the most common pediatric neoplasm. Kasabach-Merritt syndrome is a rare type of vascular lesion with peculiar characteristics. The diagnosis is based upon three basic findings; enlarging haemangioma, thrombocytopenia and consumption coagulopathy.Case presentationA 5 month old boy was admitted to the Pediatrics department for the management of an abdominal wall mass. He was the first child of consanguineous parents, born in a private hospital following uncomplicated pregnancy and delivery. At birth a bluish birth mark 5 cm × 5 cm was noted below the umbilicus. Over the next five months, this birth mark increased in size and evolved into a swelling. As a result, the patient was admitted to Maternal and Child Health (MCH) unit for the management of this swelling.The clinical findings and imagining studies followed by laboratory investigations strongly suggested the diagnosis of Kasabach-Merritt syndrome.Vincristine was initiated after a trial of corticosteroids when the platelet count was 6000/cmm. One week after the start of vincristine the size of the lesion started to decrease. At the end of 6th week the lesion size decreased to half and the platelet count increased to 49,000/cmm. Vincristine was continued for another 2 weeks, no further improvement in lesion size or platelet count was observed. Vincristine was discontinued and the patient was shifted to the paediatric surgery department. A fresh platelet transfusion was given and the haemangioma was excised completely.The histopathological examination of the excised mass revealed a caverno-capillary haemangioma with infiltration into skeletal muscles.ConclusionSix weeks treatment with vincristine in a dose of 0.5 mg/kg/week followed by surgical excision may be the best management in selected cases of Kasabach-Merritt syndrome.
Highlights
Six weeks treatment with vincristine in a dose of 0.5 mg/kg/week followed by surgical excision may be the best management in selected cases of Kasabach-Merritt syndrome
Haemangiomas are vascular lesions resulting from abnormal proliferation of blood vessels
Kasabach-Merritt syndrome is a rare type of vascular lesion with peculiar characteristics
Summary
Haemangiomas are vascular lesions resulting from abnormal proliferation of blood vessels They are the most common pediatric neoplasm. Some haemangiomas are very small and hardly visible while others are large producing significant disfigurement [1] The management of these lesions depends upon their size and site and on several other distinct features as for Kasabach-Merritt syndrome, Klippel-Trenaunay-Weber syndrome, Sturge-Weber syndrome, Rendu-Osler-Weber syndrome and von HippelLindau disease[1]. Platelet transfusion and fresh frozen plasma were given and prednisolone was started at a dose of 2 mg/kg/day in 3 divided doses This therapy was continued for four weeks and tapered off in another four weeks period without any response. At the end of 6th week the lesion size decreased to half and platelet count increased to 49,000/cmm. Four and six month follow-up after surgery shows complete recovery with total correction of haemotological parameters. (Table 1)
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