Objective To discuss the value of intrapartum operation in management of birth defects and the prognosis. Methods From August 2008 to November 2009, 11 fetuses were identified with birth defects through 3D color Doppler ultrasound and confirmed by MRI and fetal karyotype in the Maternal Fetal Medicine Center, Affiliated Shengjing Hospital, China Medical University including three lymphangiomas,two congenital diaphragmatic hernias (CDH), one sacrococcygeal teratoma, three omphalocele and two gastroschisi. All the above identified birth defects were indications for surgery. All fetuses were born abdominally and received intrapartum operations, including three intrapartum fetal operations with placental infusion (two repairs of CDH, one sacrococcygeal teratoma resection), six ex-utero intrapartum treatment (EXIT; two repairs of omphalocele, two repairs of gastroschisi, two lymphangioma resection) and two surgeries in house (one omphalocele repair and one lymphangioma resection). Both the mothers and fetuses were regularly followed up. Results ( 1 ) Operations: the average operating time for the three intrapartum fetal operations was 89 minites, 5.5 minites for the six EXIT, during which EXIT was performed first,followed by blocking the umbilical circulation and neonatal surgery, and 37 minites for the two surgeries in house. All neonates survived except for one death from severe CDH at 3.5 hours after the operation. The average blood loss for cesarean section and fetal operation was 275 ml. All mothers recovered soon without fever or infection and were discharged three to five days after the operation. (2) Follow-ups: the ten survived neonates were followed up at 1 - 18 mouths at the pediatric clinics and all were growing and developing normally except for one baby with gastroschisi suffered from enteral torsion and feeding intolerance showed lower weight than babies at the same age, but catched up to normal at four months old after posture therapy. One baby with mild CDH developed pulmonary infection at two months after operation with 1/4 pneumothorax on chest X-ray, and were hospitalized for two weeks. At six months old, patent ductus arteriosus was diagnosed in the same baby and chest X-ray was normal. The baby with omphalocele was complicated with ventricular septal defect before operation and the cardiac function was normal during followups for one year. The baby with sacrococcygeal teratoma was reported to have no automatic micturition, but recovered to normal at one month of age. Conclusion Babies with certain birth defects can be managed through intrapartum operation with better outcomes.%目的 探讨产时手术在治疗出生缺陷儿及改善其预后中的价值.方法 2008年8月至2009年11月,在中国医科大学附属盛京医院母胎医学中心通过彩色三维多普勒超声(彩超)筛查出有胎儿出生缺陷(淋巴管瘤和脐膨出各3例,膈疝和腹裂各2例,骶尾部畸胎瘤1例)的11例病例,通过MRI检查和胎儿染色体核型分析,明确为可行外科手术治疗的先天性疾病.11例产妇均行子宫下段剖宫产术及产时手术治疗出生缺陷儿,其中将胎儿取出宫外不断脐带的产时胎儿手术3例(膈疝修补术2例、骶尾部畸胎瘤切除术1例);子宫外产时处理(EXIT)后行产房外科手术6例(腹裂和巨型脐膨出修补术各2例、颈部和面部淋巴管瘤切除术各1例);分娩后立即行产房外科手术2例(脐膨出修补术和胸壁淋巴管瘤切除术各1例).对产妇进行常规的产后复查.随访出生缺陷儿手术后的生长发育及营养状态.结果 (1)手术结果:11例产妇均行剖宫产术娩出胎儿行产时手术治疗.其中3例实施产时胎儿手术治疗,手术时间平均为89 min;6例先对胎儿实施EXIT,在保持胎儿胎盘循环的情况下完成气管插管,EXIT平均时间为5.5 min,然后切断脐带,实施新生儿产房外科手术治疗;2例实施单纯产房外科手术,平均时间为37 min.除重度膈疝新生儿术后3.5 h死亡外,其他10例出生缺陷儿术后均存活至今.11例产妇剖宫产术及出生缺陷儿手术的母体平均失血量为275 ml,11例产妇术后均无发热及感染征象,子宫复旧良好,手术切口愈合良好.所有病例均未输血治疗,术后3～5 d出院.(2)随访结果:10例出生缺陷儿术后分别于1～18个月到我院儿科发育门诊随访,患儿体质量及身长等发育正常.其中,1例腹裂患儿术后1个月由于肠管旋转不良,喂养不耐受,体质量小于同龄婴儿,给予体位疗法治疗后,现喂养良好,体质量增加,术后4个月发育至正常水平.轻度膈疝患儿于术后2个月发生肺部感染,住院治疗2周后好转,患侧胸部X线片提示肺气胸比约1/4,术后6个月发现动脉导管未闭,复查胸部X线片,患侧肺叶几近全部扩张正常.1例巨型脐膨出患儿术前诊断为先天性轻度室间隔缺损,1年后复查心功能未受影响.骶尾部畸胎瘤患儿术后无自主排尿,10 d后排尿基本正常,1个月后排尿完全正常.结论 产时手术治疗可迅速终止疾病进一步发展,并明显改善出生缺陷儿的预后.
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