Massive Liver Haemangioma Causing Kasabach-Merritt Syndrome in an Adult

M Alimoradi , H Sabra , E El-Helou , A Chahal , R Wakim

PMID: 32538112DOI: 10.1308/rcsann.2020.0146


Liver haemangiomas are common, but their size very rarely exceeds 40cm. Most people with liver haemangiomas are asymptomatic, and diagnosis is usually made incidentally during imaging for other complaints. When a liver haemangioma is symptomatic or produces complications, surgical intervention may be warranted. Kasabach-Merritt syndrome is an uncommon complication reported in certain rare vascular tumours in children, with only a few cases reported in adults. The syndrome describes a consumptive coagulopathy initiated within a vascular tumour, mainly tufted angiomas and kaposiform haemangioendotheliomas and, less commonly, giant haemangiomas. The process can extend beyond the tumour and become disseminated in certain cases due to trauma or surgery. The definitive treatment for giant liver haemangiomas can include arterial embolisation, surgical excision, hepatectomy or even liver transplantation. We report the case of a 32-year-old woman with a 42 × 32 × 27cm (18,870ml) liver haemangioma associated with Kasabach-Merritt syndrome. The diagnosis was challenging, even with proper imaging, owing to the rarity of the condition. It was achieved with an exploratory laparotomy with biopsy.

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