自发性双胎贫血-红细胞增多序列征六例临床分析

Clinical analysis of six cases of spontaneous twin anemia-polycythemia sequence

目的探讨双胎贫血-红细胞增多序列征(twin anemia-polycythemia sequence,TAPS)新生儿围产期临床特点及结局,提高对TAPS的认识及管理。方法收集2016年1月至2020年6月首都医科大学附属北京妇产医院新生儿科收治的单绒毛膜双羊膜囊(monochorionic double amniotic,MCDA)双胎贫血-红细胞增多序列征6对新生儿及母亲的临床资料,对其临床特点进行归纳总结。结果自发性TAPS占MCDA妊娠的3.7%,6对均为早产儿,男女比例为1∶2,平均出生体质量1672.5 g,适于胎龄儿9例,小于胎龄儿3例,Hb差值(△Hb)为90~208 g/L。TAPS分期Ⅰ期2对,Ⅱ期1对,Ⅳ期1对,Ⅴ期1对。除1例胎儿宫内死亡外,其余11例均活产。11例存活新生儿均需呼吸支持,8例出现喂养不耐受,4例窒息,6例输血治疗,2例部分换血治疗,2例心脏发育异常,1例肾衰竭。1例因急性肾衰竭家长放弃治疗患儿死亡。随访患儿中,1例语言发育落后,1例大运动发育落后;其余存活儿生长发育未见异常。结论TAPS是MCDA双胎的严重并发症,易出现窒息、严重贫血、肾功能异常等严重围产期并发症,且远期发育异常概率高,临床应加强对其认识,积极优化产前管理,以期改善其预后。

Objective To investigate the clinical characteristics and perinatal outcome of twins with anemia-polycythemia sequence sign(TAPS),aiming at improving the understanding and management of TAPS.Methods The clinical data of six pairs of twin neonates with monochorionic double amniotic(MCDA)and their mothers were retrospectively reviewed in the department of neonatology in Beijing Obstetrics and Gynecology Hospital,Capital Medical University from January 2016 to Jun 2020.Results Spontaneous TAPS accounted for 3.7%of MCDA pregnancy,six pairs were premature,the ratio of male to female was 1∶2,the average birth weight was 1672.5 g.Nine newborn was at gestational age while three was less than gestational age.The hemoglobin values was 90-208 g/L,two pairs were in TAPS stageⅠ,while one pair in stageⅡ,Ⅳ,Ⅴ,respectively.Eleven neonates survived while one died before birth.Respiratory support was required in all 11 survived neonates,while blood transfusion therapy was carried in six cases,and partial blood exchange therapy was carried in two cases.Feeding intolerance was found in eight,asphyxia in four,cardiac dysplasia in two,and renal failure in one.One child died of acute renal failure and withdrawing treatment claimed by neonate’s parents.Among the children followed up,one case had language development lag and one case had macromotor development lag.No other abnormalities were observed in the growth and development of remaining children.Conclusions TAPS is a serious complication of twin fetuses with MCDA,asphyxia,severe anemia,renal dysfunction are common complicated,TAPS has a high probability of long-term developmental delay.It is important to highlight clinical understanding of TAPS,and actively optimize prenatal management,in order to improve its prognosis.