Liver Transplantation for Giant Hemangioma Complicated by Kasabach-Merritt Syndrome: A Case Report and Literature Review.

Abstract

Patient: Female, 37-year-old Final Diagnosis: Kasabach-Merritt syndrome Symptoms: Abdomen distension • pain Medication: — Clinical Procedure: — Specialty: Gastroenterology and Hepatology Objective: Rare disease Background:Liver hemangiomas are the most common benign liver tumor. Giant hepatic hemangiomas are hemangiomas that are greater than 4 cm in diameter. While asymptomatic giant hepatic hemangioma patients can be monitored without intervention, patients that experience complications can be managed by trans-arterial embolization, radiofrequency ablation, surgical resection, or enucleation. Although there is no consensus on definite medical treatment or optimal timing of surgery, liver transplantation is rarely indicated. Among giant hepatic hemangioma patients who received liver transplantation, Kasabach-Merritt syndrome (KMS), a consumptive coagulopathy associated with hemangiomas, is one of the most common indications. We present a case of giant hepatic hemangioma complicated by Kasabach-Merritt syndrome, which was successfully treated by orthotopic liver transplantation.Case Report:The patient was a 39-year-old woman with a known history of multiple giant hepatic hemangiomas who presented with abdominal pain and distension. She had life-threatening intra-abdominal hemorrhages caused by benign endometriomas due to hepatic hemangiomas complicated by Kasabach-Merritt syndrome. Despite interventional radiology embolization of a bleeding uterine artery and aggressive resuscitation with fluid and blood products, the patient’s status continued to decline. Emergent orthotopic liver transplantation was applied with subsequent resolution of the consumptive coagulopathy. She remained well at 2-month follow-up, with normal liver enzyme levels and intact liver allograft function.Conclusions:Liver transplantation is indicated for selected patients with giant hepatic hemangioma complicated by KMS; despite the high surgical risk, outcomes seem favorable.