Infantile hepatic hemangioendothelioma with heart failure as the main manifestation: a report of 4 cases and literature review / 中华实用儿科临床杂志

ABSTRACT

Objective To summarize the clinical features,treatment and prognosis of infantile hepatic hemangioendothelioma(IHHE) combined with congestive heart failure(CHF) in children.Methods A retrospective analysis was made with the clinical data from 4 children with IHHE combined with CHF in Department of Pediatric Cardiology,Affiliated Guangzhou Women and Children's Medical Center of Guangzhou Medical University from May 2013 to December 2015.Results Among 4 infants,there were 3 male and 1 female,mean aged of 109 days (21-219 days),the average weight of 4 350 g (2 750-6 500 g),and 1 case was admitted because of abdominal swelling and respiratory distress,1 case of abdominal swelling,jaundice,shortness of breath admission,1 case of fever and shortness of breath admission,and 1 case of cyanosis and shortness of breath admission.Two patients were associated with hemangioma and Kasabach-Merritt syndrome.Three cases had multiple liver hemangioma scattered in the left lobe and right lobe of the liver,and 1 case had solitary liver hemangioma scattered in the right lobe.Tumor size diameters were ranged from 2-10 mm.Four children had been used Prednisone [1-2 mg/(kg · d)] and Digoxin,Dopamine,Furosemide,Spironolactone.Two cases responsed well to the therapy and were followed up for 2 years or 1 year and 6 months respectively,whose heart size,heart function and pulmonary hypertension became normal.One case with Kasabach-Merritt syndrome responsed poorly to the therapy.Two cases experienced transcatheter hepatic arteriovenous fistula embolization by using coil occlusion of hepatic artery-venous fistula.The postoperative hepatic tumor volume was significantly decreased,and the heart function was improved.One patient was followed up for 1 year and 6 months,and the heart size,pulmonary hypertension and heart function returned to normal.One case was still under follow-up.One case with Kasabach-Merritt syndrome died from heart failure and severe infection before surgery.The mortality rate was 25％ (1/4 cases).Conclusions IHHE is a rare cause of CHF in children.If heart failure is found unexplained in children,abdominal ultrasound or CT examination should be done immediatey in order to confirm the presence or absence of IHHE.The mortality rate in IHHE associated with CHF is very high.Early identification,early intervention,especially Prednisone combined with transcatheter hepatic arteriovenous fistula embolization can significantly improve heart function,reduce the mortality and improve the prognosis of IHHE associated with CHF in children.