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目的:尝试对肝血管瘤进行进一步分类，以探讨多发型和弥漫型肝血管瘤间是否存在连续性。方法:对2000年1月至2020年1月在复旦大学附属儿科医院确诊的43例多发型或弥漫型肝血管瘤患儿的临床资料进行回顾性分析。依据影像学特征将患儿分为多发型组(34例)及弥漫型组(9例)，再依据病灶数是否可数将多发型组分为可计数(multifocal-type with countable lesions，MC)组(6例)和难以计数(multifocal-type with uncountable lesions，MU)组(28例)，比较各组间临床特征差异。结果:MC组、MU组及弥漫型组皮肤血管瘤伴发率分别为66.7%(4/6)、50.0%(14/28)和44.4%(4/9)，肝肿大发生率分别为0、46.4%(13/28)和100%(9/9)，心力衰竭发生率分别为0、28.6%(8/28)和44.4%(4/9)，呼吸困难发生率分别为0、25.0%(7/28)和66.7%(6/9)，甲状腺功能减退发生率分别为0、21.4%(6/28)和77.8%(7/9)。其中，弥漫型组肝肿大、呼吸困难、甲状腺功能减退发生率较MC组和MU组均明显升高，组间比较，差异均有统计学意义(n P<0.001、＝0.017、0.006和n P＝0.004、0.032、0.004)。而MU组较MC组肝肿大发生率明显增高，组间比较，差异有统计学意义(n P＝0.040)。多发型组34例中，有16例(47.1%)仅观察随访，18例(52.9%)采用药物或药物联合介入治疗。其中，MC组6例(100%)均仅观察随访；MU组28例中，10例(35.7%)仅观察随访，18例(64.3%)患儿采用药物或药物联合介入治疗。弥漫型组9例(100%)均采用药物治疗，其中3例(33.3%)联合介入治疗。弥漫型组药物或药物联合介入治疗率较多发性组、MU组和MC组均显著增高，差异均具有统计学意义(n P＝0.027、0.038和<0.001)；MU组与MC组药物或药物联合介入治疗率比较，差异亦有统计学意义(n P＝0.006)。所有患儿随访6个月以上，其中多发性组患儿均存活且病情好转；弥漫型组患儿死亡2例(22.2%)，均采用药物联合肝动静脉瘘栓塞术治疗，均因并发严重心力衰竭死亡。MC组、MU组和弥漫型组分别有5例(83.3%)、14例(50.0%)和3例(33.3%)患儿病变完全消退，三组消退率逐渐降低。n 结论:多发型肝血管瘤中部分病例CT或MRI横断面上病灶数量过多以至于难以准确计数，其临床特征与弥漫型肝血管瘤相似，提示其可能是多发性病变和弥漫型病变的中间连续体，需加以区分以得到及时治疗。“,”Objective:To further classify liver hemangiomas to examine whether or not a continuum existed between multifocal versus diffuse liver hemangioma.Methods:Between January 2000 and January 2020, a total of 43 children with a diagnosis of multifocal or diffuse hepatic hemangioma at Children's Hospital of Fudan University were analyzed retrospectively. They were divided into multifocal-type (n=34) and diffuse-type (n=9) groups according to radiological features. And multifocal-type group was further divided into countable (MC)(n=6) or uncountable (MU)(n=28) subgroups according to whether lesions were countable or not. Clinical features between each group were compared.Results:For MC, MU and diffuse-type groups, the incidence of cutaneous hemangioma was 66.7%(4/6), 50.0%(14/28) and 44.4%(4/9); the incidence of hepatomegaly 0, 46.4%(13/28) and 100%(9/9); the incidence of heart failure 0, 28.6%(8/28) and 44.4%(4/9); the incidence of dyspnea 0, 25.0%(7/28) and 66.7%(6/9); the incidence of hypothyroidism 0, 21.4%(6/28) and 77.8%(7/9). The incidence of hepatomegaly, dyspnea and hypothyroidism was higher in diffuse-type group with statistical significance than that in MC group (n P<0.001;n P=0.017; n P=0.006) and that in MU group (n P=0.004; n P=0.032; n P=0.004). And the incidence of hepatomegaly was higher in MU group than that in MC group (n P=0.040). Among 34 cases in multifocal-type group, 16 cases (47.1%) were observed and 18 cases (52.9%) received medical treatment or medical treatment plus interventional measures. Particularly, 6 cases (100%) in MC group were observed; 10/28 cases (35.7%) in MU group were observed and 18 cases (64.3%) received medical treatment or medical treatment plus interventional measures. Nine cases (100%) in diffuse-type group received medical treatment and 3 cases (33.3%) received medical treatment plus interventional measures. The proportion of children receiving medical treatment or medical treatment plus interventional measures in diffuse-type group was higher than those in MC/MU/multifocal-type group with statistical significance (n P=0.027; n P=0.038; n P<0.001). And the proportion of those receiving medical treatment or medical treatment plus interventional measures was also higher in MU group than that in MC group with statistical significance (n P=0.006). During a follow-up period of over 6 months, all children in multifocal-type group finally survived with stable conditions. However, 2 cases (22.2%) in diffuse-type group died of severe heart failure after medical treatment plus hepatic arteriovenous fistula embolization. In contrast, the rate of complete remission was 83.3%(5/6) in MC group, 50.0%(14/28) in MU group and 33.3%(3/9) in diffuse-type group. It decreased gradually.n Conclusions:MU group is a subgroup of multifocal hepatic hemangioma with too-numerous-to-count lesions on transverse sections of CT/MRI. With similar clinical features to diffuse hepatic hemangioma, there may be a continuum between multifocal and diffuse hepatic hemangiomas. Timely treatment is needed.