先天性膈疝的宫内诊断和治疗

影响先天性膈疝(CDH)预后的主要因素是肺发育不良和肺动脉高压,目前各种出生后治疗手段均不能直接、立即改变已经存在的肺发育不良状态,故无法从根本上改善严重CDH的预后。宫内诊断并适时进行干预可能成为CDH有效的一种治疗手段。文章介绍了超声及MR I检查在CDH宫内诊断与肺发育程度评估方面的应用、以胎儿镜下胎儿气管球囊封堵术为重点的宫内干预及子宫外产时处理技术的应用。     

多因素联合预测先天性膈疝胎儿的生存

目的:探讨影响先天性膈疝（congenital diaphragmatic hernia,CDH）胎儿生存的因素,并建立CDH的预测模型。方法:对2010年1月1日至2018年12月31日在上海交通大学医学院附属新华医院收治的252例产前超声诊断为CDH的新生儿进行回顾性分析。采用 χ 2检验...     

胎儿先天性膈疝及其相关异常的产前诊断

目的 分析胎儿先天性膈疝与合并其他畸形及染色体异常的相关性,探讨可能影响膈疝预后的因素. 方法 总结2002年1月至2008年11月在我院产前超声诊断的胎儿膈疝病例,分析其类型、合并畸形种类、与染色体异常的关系及临床结局.采用Fisher精确概率法进行统计学分析. 结果 产前超声诊断胎儿膈疝71例,左侧62例(87.3%),右侧9例(12.7%).单纯型膈疝38例(53.5%),复合型膈疝33例(46.5%).复合型膈疝中合并的其他异常中常见的有心血管系统畸形18例(54.5%),神经系统畸形10例(30.3%),泌尿系统畸形9例(27.2%).71例膈疝病例中19例行胎儿染色体核型检查,其中12例单纯型膈疝胎儿的染色体均正常,7例复合型膈疝中4例(4/7)染色体核型异常:18-=体综合征2例,13、21-三体综合征各1例.复合型膈疝病例染色体异常的发生率高于单纯型膈疝(P=0.009).复合型膈疝病例均引产;单纯型膈疝病例中32例引产,6例继续妊娠,其中5例足月出生后接受膈疝修补术,均存活;1例出生后死亡. 结论 胎儿先天性膈疝以左侧多见,近半数合并其他异常且以心血管系统畸形为主.单纯型膈疝发生染色体异常的风险低,复合型膈疝发生染色体异常的风险高.膈疝手术对预后的影响尚不能确定.     

先天性膈疝的产前诊断及宫内治疗进展

先天性膈疝是膈肌的发育缺陷导致腹腔内容物疝人胸腔。通常是散发的，活产儿中发病率为0．02％-0．05％，若将死产也计人其中，则发病率可达0．04％。仅有不到2％的先天性膈疝为家族性发病，单纯的先天性膈疝在兄弟姐妹中的再发率为2％。84％的先天性膈疝发生于左侧，13％发生于右侧，仅2％发生于双侧。约44％的先天性膈疝胎儿合并染色体异常及心脏、肾脏、中枢神经系统、消化道畸形心或多种综合征，     

产前超声及MRI在胎儿先天性膈膨升的诊断及预后评估中的应用价值探讨

目的:探讨胎儿先天性膈膨升(EOD)的产前超声及磁共振成像(MRI)诊断情况,以明确两者在胎儿EOD诊断及预后评估中的应用价值。方法:回顾性分析产前超声初筛疑似EOD的21例患者的临床资料,比较胎儿产前超声及MRI的EOD诊断符合率,并结合出生后随访结果进行分析。结果:①经产后胸片或手术证实EOD 13例(左侧6例,右侧7例),其中单纯性EOD 3例,EOD合并患侧肺不张3例,EOD合并多发异常7例;胸骨后疝3例;疝囊型膈疝5例。产前超声诊断EOD 21例,误诊8例,超声诊断EOD准确率为61.9%(13/21);MRI诊断EOD 14例,误诊1例,MRI诊断EOD准确率为92.9%(13/14)。②EOD合并多发异常7例中6例选择引产,1例选择继续妊娠,新生儿出生后死亡。单纯性EOD 3例及EOD合并患侧肺不张3例超声检测健侧肺头比实测值与预测值的比值(O/E LHR)在60%～91%,出生后均存活。结论:产前超声诊断胎儿EOD有一定的准确性,是产前常规筛查EOD的首选,但胸骨后疝和疝囊型膈疝易误诊为EOD,应注意鉴别,结合MRI检查能提高其准确性。单纯性EOD及EOD合并患侧肺不张胎儿出生后预后较好。     

胎儿镜下选择性胎盘血管凝结术治疗双胎输血综合征的临床研究进展

双胎输血综合征是单绒毛膜双羊膜囊妊娠可能出现的严重并发症之一,致死率高。治疗方案包括期待治疗、羊水减量术、胎儿镜下选择性胎盘血管凝结术等。胎儿镜下选择性胎盘血管凝结术在提高出生成活率、降低远期并发症方面具有独特的优势,已成为治疗双胎输血综合征的首选方案。但由于其存在流产、早产、胎膜早破等风险,如何做到早诊断、早治疗,从而提高成活率、降低远期并发症等日益受到国内外专家的关注。     

胎儿镜下激光凝固胎盘吻合血管术治疗双胎输血综合征的研究进展

双胎输血综合征(TTTS)是双胎妊娠中的一种严重的并发症,其围产儿死亡率极高。胎儿镜下激光凝固胎盘吻合血管术(FLOC)的诞生给TTTS的治疗带来了光明的前景,其疗效远优于羊水减量法,总生存率已达70%-80%。对FLOC手术指征的严格把握,术前对疾病分期的准确评估,胎盘造影术的运用,是有效降低FLOC手术失败发生率的关键。宫颈环扎术的运用和FLOC技术的改进,使宫颈短和有前置胎盘的TTTS患者同样有机会进行FLOC治疗。FLOC术后的早期预测及再次手术干预,是改善术后远期预后的关键。     

胎儿右侧先天性膈疝合并肝肺融合3例并文献复习

目的探讨右侧先天性膈疝(right-sided congenital diaphragmatic hernia, RCDH)合并肝肺融合(hepatopulmonary fusion, HPF)的临床特点和管理。方法回顾性分析2022年6月至12月在广州市妇女儿童医疗中心收治的3例RCDH合并HPF患儿的临床特点, 并根据国际膈肌缺损分级标准进行缺损分级。在中国知网、万方数据库、中华医学期刊全文数据库及维普中文期刊以"先天性膈疝""肝肺融合"检索1997年1月至2023年4月的中文文献报道, 在PubMed、Embase及UpToDate数据库以"congenital diaphragmatic hernia""hepatopulmonary fusion"为关键词检索同期相关英文文献。分析RCDH合并HPF的临床特点、管理及预后等情况。结果 (1)本院病例资料:病例1和病例2产前超声发现胎儿右侧膈疝, 产前MRI均提示胎儿肝脏疝入右侧胸腔、右肺发育不良、左肺大小正常和无纵隔左移。病例1和病例3出生后的胸部X射线检查可见右肺致密影和纵隔右移。病例2为D型缺损, 出生后胸部X射线检查示纵...     

射频消融选择性减胎术和胎儿镜下选择性胎盘血管交通支凝结术治疗Ⅲ~Ⅳ期双胎输血综合征临床结局对比研究北大核心CSCD

目的探讨射频消融(radiofrequency ablation,RFA)选择性减胎术和胎儿镜下选择性胎盘血管交通支凝结术(selective laser coagulation of placental vessels,SLCPV)两种不同方式治疗严重双胎输血综合征(twin-twin transfusion syndrome,TTTS)患者的临床结局。方法收集中国医科大学附属盛京医院2011年7月至2015年5月行RFA治疗(5例)、SLCPV治疗(15例)的Quintero分期Ⅲ~Ⅳ期TTTS患者的临床资料进行回顾性分析对比。结果 (1)RFA组手术孕周为22~26+1周,平均24.4周;平均手术时间15.6 min;SLCPV组手术孕周为17+5~27+4周,平均23.1周;平均手术时间30 min。RFA组无一例发生未足月胎膜早破(premature rupture of the membrane,PPROM),SLCPV组5例(P>0.05)。(2)SLCPV组患者有1例发生肠梗阻并最终发生流产;术后两组20例患者的胎儿染色体检测结果均未见异常。(3)RAF组患者分娩孕周为28^(+1)~36^(+1)周,平均32.2周,SLCPV分娩孕周为26^(+3)~37^(+1)周,平均30.3周。对新生儿定期随访发现:SLCPV组中1例胼胝体发育不良,而其同胞胎儿表现正常。至少一胎存活率全部20例患者为16/20,RAF组为5/5,SLCPV组为11/15(P>0.05)。结论 RAF选择性减胎术和胎儿镜SLCPV治疗严重双胎输血综合征均具有良好效果;射频消融手术是治疗严重双胎输血综合征一种可考虑的选择。     

不同孕周及方法测量的观察-预期肺头比对先天性左侧膈疝胎儿生后符合体外膜肺氧合指征的预测价值

目的:探讨不同孕周及超声测量方法得到的观察-预期肺头比（observed to expected lung area to head circumference ratio,o/e LHR）对左侧先天性膈疝（congenital diaphragmatic hernia,CDH）胎儿生后病情符合体外膜肺氧合（extra...     

Congenital diaphragmatic hernia: Treatment and outcomes

Congenital diaphragmatic hernia (CDH) is a congenital defect in the diaphragm that allows herniation of abdominal contents into the fetal chest and leads to varying degrees of pulmonary hypoplasia and pulmonary hypertension. Advances in prenatal diagnosis and the institution of standardized delivery and postnatal care protocols have led to improved survival. Fetal endoscopic tracheal occlusion shows early promise for patients with the most severe CDH, but prospective randomized data is still required. CDH survivors have a variety of associated morbidities that require long-term follow-up and early intervention strategies for optimal care.     

Complications Related to the Initial Trocar Insertion of 3 Different Techniques: A Systematic Review and Meta-analysis

This systematic review aimed to investigate complications related to initial trocar insertion among 3 different laparoscopic techniques: Veress needle (VN) entry, direct trocar entry (DTE), and open entry (OE). A literature search was completed, and complications were assessed. Major vessel injury, gastrointestinal injury, and solid organ injury were defined as major complications. Minor complications were defined as subcutaneous emphysema, extraperitoneal insufflation, omental emphysema, trocar site bleeding, and trocar site infection. Arm-based network meta-analyses were performed to identify the differences in complications among the 3 techniques. Seventeen studies were included in the quantitative analysis. DTE resulted in fewer major complications when compared with VN entry although the difference was not significant (p?=?.23) as well as significantly fewer minor complications (p < .001). There were no significant differences in minor complications when comparing OE and DTE (p?=?.74). Fewer major complications were observed with OE compared with VN entry although the difference was not significant (p?=?.31). There were significantly fewer minor complications for patients who underwent OE (p?=?.01). DTE patients experienced the least number of minor complications followed by VN entry and OE. In conclusion, major complications are extremely rare, and all 3 insertion methods can be performed without mortality.     

Reproductive Outcomes after Fertility-Sparing Surgery for Focal and Diffuse Adenomyosis: A Systematic Review

Among the variety of treatment options to improve reproductive outcomes for infertile women with adenomyosis (AD), uterine-conserving surgery has shown varying success. Hence, we conducted a systematic review around the topic of fertility-sparing surgery across 18 studies and 1396 infertile women with focal and diffuse AD. Patients with focal AD showed mean pregnancy and miscarriage rates of 52.7% (range,14.3%–77.5%) and 21.1% (range, 0%–44.4%), respectively, whereas patients with diffuse AD had mean pregnancy and miscarriage rates of 34.1% (range, 9.4%–100%) and 21.7% (range, 12.5%–33.3%), respectively. Uterine rupture and preterm birth were observed in 6.8% (3/44) and 4.5% (2/44) of pregnant patients with diffuse AD versus 0% (0/35) and 10.9% (12/110) of patients with focal AD, respectively. No significant differences were observed between natural conception versus assisted reproductive technology (ART) with or without gonadotropin-releasing hormone agonist pretreatment. Overall, patients with focal AD appeared to have higher pregnancy rates after conservative surgery compared with diffuse AD, whereas a higher incidence of uterine rupture was reported after surgery for diffuse AD. However, significant heterogeneity precludes any direct comparison, and prospective controlled trials are required to further elucidate the benefits of fertility-preserving surgery over medical or expectant management for AD-related infertility. In view of the debatable benefits of conservative surgery and the possible increase in adverse pregnancy outcomes, particularly in cases of diffuse AD, clinicians should consider surgery on a case-by-case basis because it may be appropriate for women with concurrent AD-associated pelvic pain or menorrhagia, younger infertile women who have failed medical management or older women with infertility despite ART, and those with a history of recurrent pregnancy loss or implantation failure.     

Strangulated Congenital Diaphragmatic Hernia of Bochdalek Diagnosed in Late Pregnancy: A Case Report and Review of the Literature

BackgroundCongenital diaphragmatic hernia is an unusual condition in the adult population because it is mostly a neonatal diagnosis. This entity may be triggered by pregnancy and threaten the life of the mother and her fetus.CaseThis report presents the case of a maternal diaphragmatic hernia diagnosed at 35 weeks of pregnancy with epigastric pain, nausea, and vomiting. Patient developed respiratory distress, and a radiograph revealed left lung collapse. A chest tube was inserted for a presumed tension pneumothorax. The patient's condition deteriorated, and a diaphragmatic hernia containing the stomach, transverse colon, and small bowel was diagnosed. The patient underwent laparotomy with Caesarean section, hernia reduction, and diaphragmatic repair.ConclusionA high degree of suspicion is required to avoid misdiagnosis and management delay. Surgical treatment must be individualized according to gestational age and clinical setting.     

Female Genital Hair-Thread Tourniquet Syndrome: A Case Report and Literature Systematic Review

“Hair-thread tourniquet syndrome” (HTTS) describes the condition in which fibers of hair or thread wrap around an appendage (ie, toes, fingers, genital structures, tongue, uvula, and neck), eventually causing ischemia and tissue necrosis. To date, few cases of female genitalia HTTS have been described. We report a case of female genitalia HTTS in a 5-year-old girl and report the state of the art by systematically reviewing all existing evidence about female genital HTTS. A total of 29 studies, describing a total of 34 patients, were identified. The presence of a hair-thread wrapping genitalia should be suspected in prepubertal girls complaining of genital pain associated with vulvar/vaginal swelling, wide-based gait, and voiding symptoms. Genital examination disclosing an extremely tender, swollen, and erythematous lesion on the clitoris or labia minora encircled by a hair confirms the diagnosis. The aim of the management is to remove the hair-thread in the shortest time possible, with the use of forceps, scissors, or scalpels, and this is often performed under sedation/anesthesia because of the patient's pain reaction. When the hair-thread is difficult to find or when the lesion is necrotic, excision of the lesion itself can be the only option. Complications include partial or total amputation because of tissue necrosis and recurrence.     

口服米索前列醇用于早期妊娠人工流产术前扩张宫颈的有效性和安全性评价

目的:系统评价口服米索前列醇与安慰剂比较用于孕早期人工流产术前扩张宫颈的有效性和安全性。方法:检索相关文献并行Meta分析。据异质性检验结果采用固定效应模型、敏感性分析或随机效应模型等处理。结果:纳入文献5篇688例,国外文献质量高于国内。口服米索前列醇较之安慰剂宫颈扩张度好的比例较高,可降低术中出血量及疼痛发生率;在人工流产综合征(PAAS)、胃肠道副反应和用药后腹痛发生率及手术时间上两者差异无统计学意义。结论:国内原始研究质量有待提高,结局指标需更加规范。口服米索前列醇对于扩张宫颈有较好临床效果,而在其他有效性指标方面效果不明显,其安全性有待更多证据加以评价。