CAJ | 학술논문

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. 目的探討產時手術在治療齣生缺陷兒及改善其預後中的價值.方法 2008年8月至2009年11月,在中國醫科大學附屬盛京醫院母胎醫學中心通過綵色三維多普勒超聲(綵超)篩查齣有胎兒齣生缺陷(淋巴管瘤和臍膨齣各3例,膈疝和腹裂各2例,骶尾部畸胎瘤1例)的11例病例,通過MRI檢查和胎兒染色體覈型分析,明確為可行外科手術治療的先天性疾病.11例產婦均行子宮下段剖宮產術及產時手術治療齣生缺陷兒,其中將胎兒取齣宮外不斷臍帶的產時胎兒手術3例(膈疝脩補術2例、骶尾部畸胎瘤切除術1例);子宮外產時處理(EXIT)後行產房外科手術6例(腹裂和巨型臍膨齣脩補術各2例、頸部和麵部淋巴管瘤切除術各1例);分娩後立即行產房外科手術2例(臍膨齣脩補術和胸壁淋巴管瘤切除術各1例).對產婦進行常規的產後複查.隨訪齣生缺陷兒手術後的生長髮育及營養狀態.結果 (1)手術結果:11例產婦均行剖宮產術娩齣胎兒行產時手術治療.其中3例實施產時胎兒手術治療,手術時間平均為89 min;6例先對胎兒實施EXIT,在保持胎兒胎盤循環的情況下完成氣管插管,EXIT平均時間為5.5 min,然後切斷臍帶,實施新生兒產房外科手術治療;2例實施單純產房外科手術,平均時間為37 min.除重度膈疝新生兒術後3.5 h死亡外,其他10例齣生缺陷兒術後均存活至今.11例產婦剖宮產術及齣生缺陷兒手術的母體平均失血量為275 ml,11例產婦術後均無髮熱及感染徵象,子宮複舊良好,手術切口愈閤良好.所有病例均未輸血治療,術後3～5 d齣院.(2)隨訪結果:10例齣生缺陷兒術後分彆于1～18箇月到我院兒科髮育門診隨訪,患兒體質量及身長等髮育正常.其中,1例腹裂患兒術後1箇月由于腸管鏇轉不良,餵養不耐受,體質量小于同齡嬰兒,給予體位療法治療後,現餵養良好,體質量增加,術後4箇月髮育至正常水平.輕度膈疝患兒于術後2箇月髮生肺部感染,住院治療2週後好轉,患側胸部X線片提示肺氣胸比約1/4,術後6箇月髮現動脈導管未閉,複查胸部X線片,患側肺葉幾近全部擴張正常.1例巨型臍膨齣患兒術前診斷為先天性輕度室間隔缺損,1年後複查心功能未受影響.骶尾部畸胎瘤患兒術後無自主排尿,10 d後排尿基本正常,1箇月後排尿完全正常.結論產時手術治療可迅速終止疾病進一步髮展,併明顯改善齣生缺陷兒的預後.
목적 탐토산시수술재치료출생결함인급개선기예후중적개치.방법 2008년8월지2009년11월,재중국의과대학부속성경의원모태의학중심통과채색삼유다보륵초성(채초)사사출유태인출생결함(림파관류화제팽출각3례,격산화복렬각2례,저미부기태류1례)적11례병례,통과MRI검사화태인염색체핵형분석,명학위가행외과수술치료적선천성질병.11례산부균행자궁하단부궁산술급산시수술치료출생결함인,기중장태인취출궁외불단제대적산시태인수술3례(격산수보술2례、저미부기태류절제술1례);자궁외산시처리(EXIT)후행산방외과수술6례(복렬화거형제팽출수보술각2례、경부화면부림파관류절제술각1례);분면후립즉행산방외과수술2례(제팽출수보술화흉벽림파관류절제술각1례).대산부진행상규적산후복사.수방출생결함인수술후적생장발육급영양상태.결과 (1)수술결과:11례산부균행부궁산술면출태인행산시수술치료.기중3례실시산시태인수술치료,수술시간평균위89 min;6례선대태인실시EXIT,재보지태인태반순배적정황하완성기관삽관,EXIT평균시간위5.5 min,연후절단제대,실시신생인산방외과수술치료;2례실시단순산방외과수술,평균시간위37 min.제중도격산신생인술후3.5 h사망외,기타10례출생결함인술후균존활지금.11례산부부궁산술급출생결함인수술적모체평균실혈량위275 ml,11례산부술후균무발열급감염정상,자궁복구량호,수술절구유합량호.소유병례균미수혈치료,술후3～5 d출원.(2)수방결과:10례출생결함인술후분별우1～18개월도아원인과발육문진수방,환인체질량급신장등발육정상.기중,1례복렬환인술후1개월유우장관선전불량,위양불내수,체질량소우동령영인,급여체위요법치료후,현위양량호,체질량증가,술후4개월발육지정상수평.경도격산환인우술후2개월발생폐부감염,주원치료2주후호전,환측흉부X선편제시폐기흉비약1/4,술후6개월발현동맥도관미폐,복사흉부X선편,환측폐협궤근전부확장정상.1례거형제팽출환인술전진단위선천성경도실간격결손,1년후복사심공능미수영향.저미부기태류환인술후무자주배뇨,10 d후배뇨기본정상,1개월후배뇨완전정상.결론 산시수술치료가신속종지질병진일보발전,병명현개선출생결함인적예후.
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.