小儿局灶性结节性肝脏增生临床诊治经验探讨

Clinical diagnosis and treatment of focal nodular hyperplasia of liver in children

目的探讨小儿局灶性结节性肝脏增生的临床特点、诊断方法及治疗经验,提高其诊治水平。方法回顾性分析2006年1月至2018年1月由首都医科大学附属北京儿童医院经手术切除及病理检查证实为局灶性结节性肝脏增生的患儿临床资料,其中男童9例,女童13例,发病年龄7个月至11岁1个月,中位年龄4岁6个月,所有病灶为单发,均经不规则性肝切除术治疗。结果临床表现:22例局灶性结节性肝脏增生(focal nodular hyperplasia,FNH)患儿中腹痛7例,腹部膨隆或包块4例,体检发现11例;实验室检验:肝功异常8例,AFP升高3例,均于术后恢复正常;影像学检查:所有22例均行超声检查,10例拟诊断为FNH,拟诊断正确率为45.5%;误诊为肝脏血管瘤5例,其中肝母细胞瘤1例,间叶错构瘤1例。20例行CT(含增强)检查,11例拟诊断为FNH,拟诊断正确率为55%。3例术前行MRI检查,1例拟诊断为FNH,2例未予明确诊断。术后肿瘤最长径线5~15 cm,中位长度8 cm,其中≥10 cm者5例,最大肿物体积为15 cm×10 cm×8 cm。所有病例为单发病灶,均行手术切除,病理提示肿物中央灰白色放射样瘢痕特征11例(50%)。术后随访0.5~10.8年,未见复发或严重并发症发生。结论小儿局灶性结节性肝脏增生在临床及影像学上有一定特征,联合应用CT等影像学检查和AFP水平及肝功能实验室检查指标能提高其诊断水平,最终确诊需术后病理检查,手术切除治疗能够有效解除病灶,远期预后良好。

Objective To review the clinical features and management experiences of children with focal nodular hyperplasia(FNH)of liver.Methods A review of medical records was conducted for 22 children pathologically diagnosed as FNH between January 2006 and January 2018.There were 9 boys and 13 girls with a median age of 54(7~133)months.All lesions were single and surgical resections performed.Results Clinical manifestations included abdominal pain(n=7),abdominal bulge or mass(n=4)and detection by physical examination(n=11).And laboratory findings of abnormal liver function(n=8)and elevated alpha fetoprotein(AFP,n=3)were both restored postoperatively.Ultrasonography confirmed the diagnosis of FNH(n=10)with a correct rate of 45.5%.The misdiagnoses were hepatic hemangioma(n=5),hepatoblastoma(n=1)and mesenchymal hamartoma(n=1).Plain and enhanced computed tomography(CT)revealed 11/20 cases of FNH with a correct rate of proposed diagnosis at 55%.Three cases received preoperative magnetic resonance imaging(MRI).One case was diagnosed definitely as FNH while another two were diagnosed non-definitely.The longest diameters of tumor were 5 to 15 cm with a median length of 8 cm.The maximal diameter of tumor was >10 cm(n=5)and the volume of the largest mass 15 cm×10 cm×8 cm.Central fibrovascular scar was found(n=11,50%).After a follow-up period of 0.5 to 10.8 years,all cases survived without recurrence or serious complications.Conclusion The clinical and imaging features of FNH in children have some characteristics.The combined application of imaging methods,AFP and liver function test may improve the diagnostic accuracy.A definite diagnosis is dependent upon postoperative pathology examination and surgical resection can effectively remove the lesion.