摘要
目的探讨巨大胎儿肝血管瘤(giant fetal hepatic hemangioma, GFHH)的临床特征及结局。方法回顾性分析于2012年8月1日至2020年3月31日在广州市妇女儿童医疗中心确诊, 生后接受保守观察或治疗并至少随访1年的GFHH患儿。收集这些患儿出生前后的资料, 分析GFHH的特征(大小、位置和病变类型)、患儿特征、临床表现、治疗和预后。结果最终纳入22例患儿。(1)22例患儿中, 男性17例(77.3%), 女性5例(22.7%);首次B超诊断GFHH的胎龄为(35.0±2.8)周, 范围为30~40周+1;出生胎龄为(38.9±1.5)周, 范围为33周+4~40周+3;早产1例(4.5%)。15例(68.2%)经阴道娩出, 7例(31.8%)因"胎儿窘迫"剖宫产娩出。22例患儿中, 21例(95.5%)为局灶型, 1例(4.5%)为多发型。22例胎儿期接受2.5次(2~4次)B超检查, 发现血管瘤直径呈现随胎龄增加而增大的趋势, 至出生时达到最大[56 mm(42~99 mm)]。9例(40.9%)患儿经超声诊断为GFHH, 12例经MRI诊断为GFHH。其中6例超声和MRI均分别诊断为GFHH。其余病例的超声或MRI均表现为占位性病变。(2)10例患儿仅接受随访观察(保守组), 12例患儿接受药物治疗(治疗组)。治疗组患儿血小板减少的比例明显低于保守组(7/12与0/10, Fisher精确概率法, P=0.014)。保守组患儿无合并心功能异常或肺动脉高压;治疗组2例合并肺动脉高压。2组患儿的结局差异无统计学意义。(3)随访3.3年(1.2~7.0年), 所有患儿均存活。保守组均未转行药物治疗, 该组1例于2岁时病灶完全消退, 其余9例病灶部分消退。治疗组12例均口服普萘洛尔治疗, 其中3例治疗早期联合使用地塞米松, 2例患儿同时使用雷帕霉素, 2例患儿因早期病灶逐渐增大, 接受了介入治疗。结果该组2例患儿分别于1.7及5.5岁时病灶完全消退, 其余10例病灶部分消退。结论 GFHH随着胎龄的增加而增长。部分患儿的影像学表现可能不典型。对于生后无症状的患儿可考虑仅随访观察。而对于出现呼吸困难、腹部膨隆(呕吐)等症状、伴或不伴异常检验结果的患儿可考虑药物治疗;如仍无效, 可行介入治疗。GFHH患儿多可存活, 长期疗效较满意。
Objective:To investigate the clinical features and outcomes of giant fetal hepatic hemangioma(GFHH).Methods:In this retrospective study,patients with GFHH from Guangzhou Women and Children's Medical Centre who received conservative management or medical intervention with a minimum of one year's follow-up after birth from August 2012 until March 2020 were enrolled.Prenatal and postnatal data were collected to analyze the pathological(size,location,and lesion type)and clinical manifestations,treatment,and prognosis of GFHH using independent sample t-test or Chi-square(or Fisher's exact)test.Results:(1)A total of 22 subjects were enrolled,including one preterm infant(4.5%),with an average gestational age of(38.9±1.5)weeks at birth,ranging from 33+4 to 40+3 weeks.Among them,17 were male(77.3%),and five were female(22.7%).The gestational age at the first diagnosis was(35.0±2.8)weeks,ranging from 30 to 40+1 weeks.Fifteen babies(68.2%)were born vaginally and seven(31.8%)by cesarean section due to fetal distress.Focal type accounted for 21 of the 22(95.5%)patients,and multifocal type in one patient(4.5%).All the patients received B-ultrasonography on an average of 2.5 times(2-4 times)prenatally,which showed that the diameter of hepatic hemangioma increased with gestational age and reached its maximum at birth[56 mm(42-99 mm)].Nine fetuses(40.9%)were diagnosed with GFHH with prenatal ultrasonography,and 12 with MRI.Among them,six fetuses were diagnosed with both ultrasonography and MRI.The other cases showed space-occupying lesions.(2)Ten babies received conservative management(conservative group),and 12 received drug therapy(treatment group).There was no significant difference between the two groups in gestational age at diagnosis or birth,birth weight,the maximum diameter of the tumor before birth,and location and classification of the lesions(all P>0.05).The incidence of thrombocytopenia in the treatment group was significantly lower than that in the conservative group(7/12 vs 0/10,Fisher's exact test,P=0.014).No abnormality of heart function or pulmonary hypertension was found in the conservative group,while two patients were complicated with pulmonary hypertension in the treatment group.The outcomes between the two groups were similar(P>0.05).(3)During a median followed-up of 3.3 years(1.2-7.0 years),all patients survived,and none of the children in the conservative group received drug therapy.One of the ten patients in the conservative group had a complete tumor involution at two years old without recurrence,and the other nine had the tumor regressed in size.Besides oral propranolol,three of 12 cases in the treatment group also had dexamethasone at the early stage,two had rapamycin,and two received invasive therapy due to progressive enlargement of lesions at the early stage,with the lesions completely involuted in two cases at 1.7 and 5.5 years of age and regressed in the other ten cases.Conclusions:The diameter of GFHH increases with gestational age.Conservative management conservative is recommended for asymptomatic GFHH cases.For those affected with dyspnea and abdominal distention resulting in repeated vomiting,drugs therapy can be considered regardless of the test results,and invasive therapy is an option when drugs are ineffective.Some fetuses may have unsignificant imaging manifestations.Most children prenatally detected with giant hepatic hemangiomas can survive,and the tumor begins to regress after birth with a good long-term prognosis.
作者
林土连
谢杰忠
何秋明
余家康
钟微
Tulian Lin;Jiezhong Xie;Qiuming He;Jiakang Yu;Wei Zhong(Department of Surgical Neonatal Intensive Care Unit,Guangzhou Women and Children's Medical Center,Guangzhou 510623,China)
出处
《中华围产医学杂志》
CAS
CSCD
北大核心
2022年第4期278-283,共6页
Chinese Journal of Perinatal Medicine
关键词
肝肿瘤
血管瘤
超声检查
产前
婴儿
新生
Liver neoplasms
Hemangioma
Ultrasonography,prenatal
Infant,newborn
