| Objective:1. To analyse the clinical characteristics and outcome of IHH.2. To explore the treatment strategy of IHH.3. To explore the factors that may influence the prognosis of children with IHH.Methods:Clinical materials of 43 patients with IHH diagnosed by image study and/or histopathology analysis in our hospital from Jan.2000 to Jan.2014 were retrospectively analyzed. Logistic multivariate analysis of the factors related to the management and prognosis was performed in the patients with IHH.Results:All diagnoses were based on clinical feature, and were confirmed in six patients by histopathology analysis.A total of 43 patients were included, there were 22 female and 21 male(ratio 1:0.95), with age of onset ranged from 0 day to 31 months(average age 3.54±7.41 months). Of 43 patients with IHH,71.4% were focal (n= 30,17 male,13 female), multifocal (n= 11,3male,8 female), and diffuse (n= 2, 1 male,l female).Gender did not differ significantly among the three types (P=0.095). Hepatomegaly, anemia , Cardiac insufficiency were the major symptoms.9 patients (20.9%) had cutaneous hemangiomas.Cutaneous hemangiomas accompanied 54.5% (6/11) of multifocal hepatic hemangioma,100% (2/2) of diffuse hepatic hemangioma,and 3.3%(1/30) of focal hepatic hemangioma.There was significant difference among the three types(p<0.01).37 patients have been followed up,at a median follow-up time of 42±35 months (range,4 to 120 months).20 (40.5%) patients were observed without receiving any treatment,whereas 16 patients received corticosteroids and/or propranolol.4 patients lost to follow up.Spontaneous resolution occurred in 7 of 16 asymptomatic patients without specific therapy (44%), and median time to complete resolution was approximately 18.9±17.3 months (range,1 to 53 months).56.3%(9/16) patients were in regression.7 of 16 patients were successfully treated with corticosteroids and/or propranolol with complete tumor regression(43.8%), and median time to complete resolution by medical treatment was approximately 23.9±10.8 months (range,11.5 to 40.5 months),31.3%(5/16) patients were in regression. Clinical course and treatment outcome did not differ significantly between medically treated and non-treated groups.Multivariate stepwise logistic regression analysis of 43 patients indicated that cardiac insufficiency was the only independent predictor of treatment (p= 0.001) (95% CI,1.434-39.092).The presence of a shunt was the only independent factor associated with the inability of medical therapy (steroids with or without propranolol) to adequately control a patient’s symptoms (P=0.002).Thus embolization or surgery was required.1 patient died, overall survival of IHH reached 97.3%.In general, the prognosis of IHH was favourable.Conclusions:1. In our study, focal hepatic hemangioma is found to be the most common type of IHH,followed by multifocal hepatic hemangioma.2. IHH have no specific clinical manifestaion, Infants are frequently seen with a triad of hepatomegaly, anemia, and cardiac insufficiency.3. IHH have a positive natural course,with the tendency to regress spontaneously.The motality and mobidity of IHH is low.4. Cardiac insufficiency is the only independent predictor of treatment.5. The presence of a shunt is the only independent factor associated with the choice of treatment for the patient. |
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