Pancreatic Kaposiform haemangioendothelioma complicated by Kasabach-Merritt phenomenon and recurrent cholangitis in an infant: A case report

Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular tumour which primarily affects children. Pancreatic involvement is exceptionally uncommon, and its association with Kasabach-Merritt Phenomenon (KMP), a life-threatening consumptive coagulopathy, poses significant diagnostic and therapeutic challenges. A 6-month-old female presented with a five-day history of bilious vomiting, lethargy, and poor oral intake. Initial investigations revealed severe anaemia (Hb 57 g/L), coagulopathy (INR 9.3) and thrombocytopenia (platelets 57 × 10 9 /L). Imaging identified bilateral subdural haemorrhages and a retroperitoneal pancreatic mass encasing vital structures causing obstructive hepatopathy, biliary dilation, and ascites. Biopsy confirmed KHE, with spindle cell proliferation and immunohistochemical features consistent with the diagnosis. The clinical course was complicated by KMP, recurrent cholangitis with Pseudomonas bacteraemia, and a pancreatic leak. Management included sirolimus, vincristine, corticosteroids, biliary drainage, and a feeding jejunostomy. Despite significant complications, including failure to thrive and prolonged jejunostomy site issues, the patient achieved resolution of coagulopathy, stabilisation of hepatic function, and stable disease on maintenance sirolimus therapy. Chemotherapy was discontinued after six months, with ongoing outpatient monitoring. A combination of sirolimus, vincristine and supportive therapy including percutaneous biliary drainage seems to be a safe and effective management option for children with pancreatic KHE with KMP and cholestasis.