非免疫性胎儿水肿患儿的临床特点

Clinical features of non-immune hydrops fetalis in neonates

目的 总结非免疫性胎儿水肿患儿的临床特征.方法 2012年1月至2014年6月,复旦大学附属妇产科医院共收治10例非免疫性胎儿水肿患儿,回顾性分析胎儿水肿的产前特点、新生儿期表现、预后及病因特点.采用描述性统计分析.结果 （1）一般情况：1例新生儿（孕母未规律产前检查）生后发现皮肤水肿、颈蹼和腹部膨隆,诊断胎儿水肿;另外9例均于产前得到诊断.10例非免疫性胎儿水肿新生儿中男6例,女4例;2例足月儿,8例早产儿;平均出生胎龄（33.6±2.4）周,平均出生体重（2 680±478）g.（2）孕母情况：10例胎儿水肿患儿孕母年龄平均（30.3±4.6）岁;2例合并妊娠期糖尿病,1例合并妊娠期糖尿病及甲状腺功能亢进;10例孕母血型均为非O、Rh阳性,均未行微小病毒B19检测;5例行羊膜腔穿刺胎儿染色体核型分析,均正常.（3）胎儿表现：9例产前诊断者超声检查首次发现胎儿水肿的平均孕周为（31.3±2.4）周（25.1～37.0周）.产前超声提示水肿部位：胸腔积液7例次,腹腔积液6例次,羊水过多5例次和皮肤水肿4例次.（4）分娩及生后情况：产前胎心监护2例正常,8例异常（出生时均重度窒息,均行气管插管复苏）.皮肤水肿10例次,胸腔积液7例次,腹腔积液6例次,心包积液1例次.合并多发畸形1例,合并染色体异常1例.4例新生儿行胸腔穿刺,3例行腹腔穿刺.（5）预后：存活1例,死亡9例,其中6例因复苏效果不佳放弃抢救产房内死亡,2例放弃治疗后1d死亡,1例生后半年救治无效死亡.（6）病因：贫血2例,先天性膈肌血管瘤、频发房性早搏、21-三体综合征、先大性肺淋巴管扩张症、阵发性室上性心动过速、胎盘绒毛膜血管瘤、可疑遗传综合征及病因不明各1例.结论 非免疫性胎儿水肿原因各异,病因不同,预后也不同.水肿胎儿出生时窒息发生率高,复苏困难,病死率高.

Objective To summarize the clinical characteristics of neonates with non-immune hydrops fetalis.Methods The clinical data of ten neonates with non-immune hydrops fetalis, who were admitted to the Department of Neonatology, Gynecology and Obstetrics Hospital of Fudan University between January 2012 and June 2014, were retrospectively studied.Prenatal characteristics, causes, clinical features and prognosis were explored and analyzed with descriptive statistical methods.Results （1） One of the ten neonates was diagnosed after birth presented with hydroderma and abdominal distension, and the rest ones were diagnosed antenatally.There were six males and four females, and eight premature and two term neonates with the average gestational age of （33.6±2.4） weeks and birth weight of（2 680±478） g.（2） The mean maternal age was （30.3 ±4.6） years.Two of the mothers had gestational diabetes mellitus and one had gestational diabetes mellitus complicating with hyperthyroidism.The blood types of all mothers were O and Rh（＋）.None of the mothers were tested for parvovirus B19 and no fetus was reported with abnormal chromosome karyotype in the five cases received fetal karyotype analysis.（3） The average gestational age at initial identification of hydrops fetalis by fetal ultrasound was （31.3 ± 2.4） weeks （25.1～37.0 weeks） among nine cases diagnosed prenatally.By ultrasound screening, there were seven cases with hydrothorax, six with seroperitoneum, five with polyhydramnios and four with hydroderm.（4） Two neonates had normal non-stress test results during labor, and eight were abnormal and suffered from severe neonatal asphyxia at birth and resuscitated by endotracheal intubation.Ten neonates had hydroderm, seven had hydrothorax, six had seroperitoneum and one had hydropericardium.One complicated with multiple malformations and one had chromosome abnormality.Four cases received thoracentesis and three had abdominal paracentesis after parturition.（5） Prognosis： One neonate survived and nine died among which six due to resuscitation failure in delivery room, two died one day after giving up treatment after one day, and one died due to treatment failure six months after birth.（6） The causes of hydrops fetalis were anemia （two cases） and congenital diaphragmatic hemangioma, recurrent atrial premature beat, Down＇s syndrome, congenital pulmonary lymphangiectasia, paroxysmal supraventricular tachycardia, placental chorioangioma, suspicious genetic syndrome and idiopathic （one for each）.Conclusions The prognosis varies because of different etiology of non-immune hydrops fetalis.Neonates with hydrops fetalis might have a higher rate of asphyxia and mortality rate, and difficulties in resuscitation at birth.