Congenital esophageal atresia

先天性食管闭锁
  • 文章类型: Journal Article
    背景:微创手术(MIS)作为修复先天性异常的一线方法正在获得关注。这项研究旨在评估接受开放式与MIS修复术治疗食管闭锁/气管食管瘘(EA/TEF)的新生儿的预后。
    方法:在这项回顾性研究中,从2013年至2020年接受EA/TEF修复的新生儿使用国家外科质量改善计划-儿科数据库进行鉴定.手术入路的比例(开放与开放MIS)随时间的变化进行了分析。使用术前特征进行倾向评分匹配分析,并比较结果,包括复合发病率和再干预率(总体,主要的[胸腔镜检查,开胸手术],和次要[胸部/喂食管放置,内窥镜检查])在手术方法之间。适当时使用Pearson的卡方检验或Fisher的精确检验。
    结果:我们确定了1738例接受EA/TEF修复的新生儿。MIS利用率随着时间的推移而增加。赛前,接受开放式修复的新生儿更有可能早产,较低的重量,依赖呼吸机,并有严重程度较高的心脏危险因素。赛后,两组相似,每组340例新生儿.MIS修复与较长的中位手术时间(209vs.174分钟,p<0.001),术后总体干预率增加(7.6%vs.2.9%,p=0.01)。复合发病率没有差异(24.4%与25.0%,p=0.86)在再干预之外。
    结论:对于EA/TEF新生儿的MIS方法似乎与更高的再干预率相关。需要进一步研究评估用于修复EA/TEF的MIS方法,以更好地定义短期和长期结果。
    方法:回顾性对比研究。
    方法:三级。
    BACKGROUND: Minimally invasive surgery (MIS) is gaining traction as a first-line approach to repair congenital anomalies. This study aims to evaluate outcomes for neonates undergoing open versus MIS repairs for esophageal atresia/tracheoesophageal fistula (EA/TEF).
    METHODS: In this retrospective study, neonates undergoing EA/TEF repair from 2013 to 2020 were identified using the National Surgical Quality Improvement Program-Pediatric database. Proportions of operative approach (open vs. MIS) over time were analyzed. A propensity score-matched analysis using preoperative characteristics was performed and outcomes were compared including composite morbidity and reintervention rates (overall, major [thoracoscopy, thoracotomy], and minor [chest/feeding tube placement, endoscopy]) between operative approaches. Pearson\'s chi-square or Fisher\'s exact tests were used as appropriate.
    RESULTS: We identified 1738 neonates who underwent EA/TEF repair. MIS utilization increased over time. Pre-match, neonates undergoing open repair were more likely to be premature, lower weight, ventilator dependent, and have cardiac risk factors with higher severity. Post-match, the groups were similar and included 340 neonates per group. MIS repair was associated with longer median operative time (209 vs. 174 min, p < 0.001) and increased overall post-operative intervention rates (7.6% vs. 2.9%, p = 0.01). There were no differences in composite morbidity (24.4% vs. 25.0%, p = 0.86) outside of reintervention.
    CONCLUSIONS: MIS approach for neonates with EA/TEF appears to be associated with a higher rate of reinterventions. Further studies evaluating MIS approaches for the repair of EA/TEF are needed to better define short- and long-term outcomes.
    METHODS: Retrospective comparative study.
    METHODS: Level III.
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  • 文章类型: Case Reports
    我们介绍了三例左心发育不良综合征(HLHS)并发先天性食管闭锁和气管食管瘘(EA/TEF)的病例。HLHS的标准治疗包括分阶段手术方法,最终达到Fontan完成。尚无HLHS和EA/TEF患者的报告,对于这种情况,没有既定的治疗策略。鉴于同时在HLHS和EA/TEF上运行的重大风险,我们选择对每种情况分别进行分阶段修复。最初,出生后不久,我们进行了胃造口术以确保EA/TEF的营养途径并稳定呼吸.随后,我们对HLHS进行了双侧肺动脉束带术(bil-PAB)和导管支架置入术,诺伍德手术在这些患者中带来了不可接受的高风险。其中两名患者能够过渡到家庭护理,而另一名患者因EA修复后并发症在住院期间死亡。bil-PAB与HLHS导管支架置入术和EA/TEF分期修复术的组合可以为两种情况的患者提供有效的治疗。
    左心发育不良综合征(HLHS)和先天性食管闭锁(EA)都是危及生命的疾病,需要在出生后进行早期干预。几乎没有关于这两种情况的患者的报告,并且没有建立治疗策略。尽管该程序具有很高的风险,我们成功地对HLHS进行了导管支架置入术和双侧肺动脉束带术,以及EA的分阶段修复程序。我们的方法可能是解决这些情况的可行策略。
    We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately. Initially, soon after birth, we performed gastrostomy to secure the nutritional pathway for EA/TEF and stabilize breathing. Subsequently, we conducted bilateral pulmonary artery banding (bil-PAB) and ductal stenting for HLHS, as the Norwood operation carried an unacceptably high risk in these patients. Two of these patients were able to transition to home care, while the other patient died during hospitalization due to complications after EA repair. A combination of bil-PAB with ductal stenting for HLHS and staged repair for EA/TEF may provide effective management for patients with both conditions.
    UNASSIGNED: Hypoplastic left heart syndrome (HLHS) and congenital esophageal atresia (EA) are both life-threatening conditions that require early intervention after birth. There are few reports of patients with both conditions, and no treatment strategy is established. Although the procedure carries a high risk, we successfully performed ductal stenting with bilateral pulmonary artery banding for HLHS, as well as staged repair procedures for EA. Our approach may be a viable strategy for these conditions.
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  • 文章类型: Case Reports
    背景:我们报道了一例低出生体重(1.8kg)新生儿III型先天性食管闭锁(CEA)合并有症状动脉导管未闭(PDA)的患儿。经过综合评价,在排除手术禁忌症后的出生后第11天进行食管吻合术,同时进行动脉导管结扎。文献中尚未明确报道CEA联合PDA的同期手术。
    方法:我们报告了一名6天大的女性儿童,患有III型CEA和PDA。患者出生后口腔出现泡沫,进食后咳嗽和呼吸急促。在另一家医院,她被认为是新生儿肺炎,新生儿黄疸和先天性心脏病转院。食道碘油造影和超声心动图检查后,我们确认了CEA和PDA的诊断。PDA的直径为8毫米,有明显的左向右分流。我们进行了右后胸膜外口瘘结扎术和食管吻合术,住院5d后经左腋下直切口结扎PDA。手术成功了,切口12d后愈合,病人出院了.我们在手术后1个月重新检查了患者。她吃米粉时没有呕吐。食管血管造影显示吻合口无狭窄。患者体重3.2kg。
    结论:对于具有多种危险因素的CEA患者,全面,及时准确的诊断和评估,早期治疗可改善预后。
    BACKGROUND: We report a low-birth-weight child (1.8 kg) with neonatal type III congenital esophageal atresia (CEA) combined with symptomatic patent ductus arteriosus (PDA). After comprehensive evaluation, esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications, and arterial catheter ligation was performed at the same time. Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature.
    METHODS: We report a 6-day-old female child with type III CEA and PDA. The patient presented with foam at the mouth after birth, cough and shortness of breath after feeding. At another hospital, she was considered to have neonatal pneumonia, neonatal jaundice and congenital heart disease and transferred to our hospital. After iodine oil radiography of the esophagus and echocardiography we confirmed diagnosis of CEA and PDA. The diameter of the PDA was 8 mm, with obvious left to right shunting. We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis, and ligation of PDA via left axilla straight incision after 5 d of hospitalization. The operations were successful, and the incision healed after 12 d, and the patient was discharged. We re-examined the patient 1 mo after surgery. She did not vomit when she ate rice flour. Esophageal angiography showed no stricture of the anastomotic stoma. The patient weighed 3.2 kg.
    CONCLUSIONS: For CEA patients with multiple risk factors, comprehensive, timely and accurate diagnosis and evaluation, and early treatment may improve prognosis.
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  • 文章类型: Case Reports
    背景:食管闭锁(EA)是新生儿中危及生命的先天性畸形,传统的修复方法带来了技术挑战,并且极具侵入性。因此,外科医生一直在积极研究新的微创技术来解决这个问题。在一些研究中已经报道了磁压缩吻合在修复EA中的潜力。在本文中,报告了胸腔镜下磁压吻合术对EA的初次修复。
    方法:一名体重为3500g的足月男性被诊断为EAgrossC型。该程序中使用的磁性装置由两个磁性环和几个导管组成。进行气管食管瘘结扎术和两个荷包串。然后通过胸腔镜完成磁压缩吻合。初次修复后,没有进行额外的手术。术后第15天观察到吻合通畅,磁铁在第23天被移除。经口喂养开始时不存在渗漏。
    结论:胸腔镜下磁压吻合术可能是修复EA的一种有前途的微创方法。
    BACKGROUND: Esophageal atresia (EA) is a life-threatening congenital malformation in newborns, and the traditional repair approaches pose technical challenges and are extremely invasive. Therefore, surgeons have been actively investigating new minimally invasive techniques to address this issue. Magnetic compression anastomosis has been reported in several studies for its potential in repairing EA. In this paper, the primary repair of EA with magnetic compression anastomosis under thoracoscopy was reported.
    METHODS: A full-term male weighing 3500 g was diagnosed with EA gross type C. The magnetic devices used in this procedure consisted of two magnetic rings and several catheters. Tracheoesophageal fistula ligation and two purse strings were performed. The magnetic compression anastomosis was then completed thoracoscopically. After the primary repair, no additional operation was conducted. A patent anastomosis was observed on the 15th day postoperatively, and the magnets were removed on the 23rd day. No leakage existed when the transoral feeding started.
    CONCLUSIONS: Thoracoscopic magnetic compression anastomosis may be a promising minimally invasive approach for repairing EA.
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  • 文章类型: Journal Article
    患有某些罕见疾病的儿科患者患严重呼吸道合胞病毒(RSV)感染的风险增加。然而,日本目前没有在这些患者中预防性使用抗RSV抗体(帕利珠单抗).
    这是世界上第一个多中心,不受控制,开放标签,2019年7月28日至2021年9月24日在日本7家医疗机构进行了II期临床试验,以调查疗效,安全,帕利珠单抗在新生儿招募的23名受试者中的药代动力学,婴儿,或24个月或更小的儿童患有以下任何一种疾病:肺发育不全,气道狭窄,先天性食管闭锁,遗传性代谢病,或者神经肌肉疾病。以15mg/kg以30天的间隔肌内施用至少四个连续剂量的帕利珠单抗。
    23名受试者完成了研究。没有受试者需要因RSV住院治疗。不良事件(AE)与最新访谈表格中描述的事件术语没有显着差异。5例严重不良事件需要计划外住院,但没有RSV感染就解决了。在整个研究期间维持帕利珠单抗的治疗有效浓度。
    帕利珠单抗可能具有良好的耐受性,可有效预防严重呼吸道感染引起的严重呼吸道症状和住院,表明本研究中包括的儿科患者的预防性使用。
    日本医学研究与发展机构(AMED),赠款编号19lk0201097h0001(至MM),20lk0201097h0002(至MM),21lk0201097h0003(至MM),和22lk0201097h0004(至MM)。AMED在这项研究的执行中没有任何作用,分析和解释数据,或提交结果的决定。
    UNASSIGNED: Pediatric patients with certain rare diseases are at increased risk of severe respiratory syncytial virus (RSV) infection. However, the prophylactic use of anti-RSV antibody (palivizumab) in these patients is not indicated at present in Japan.
    UNASSIGNED: This first-in-the-world multicenter, uncontrolled, open-label, phase II clinical trial was carried out between 28 July 2019 and 24 September 2021 at seven medical institutions in Japan to investigate the efficacy, safety, and pharmacokinetics of palivizumab in 23 subjects recruited from among neonates, infants, or children aged 24 months or younger who had any of the following conditions: pulmonary hypoplasia, airway stenosis, congenital esophageal atresia, inherited metabolic disease, or neuromuscular disease. At least four continuous doses of palivizumab were administered intramuscularly at 15 mg/kg at intervals of 30 days.
    UNASSIGNED: Twenty-three enrolled subjects completed the study. No subject required hospitalization for RSV. Adverse events (AE) did not notably differ from the event terms described in the latest interview form. Five severe AEs required unplanned hospitalization, but resolved without RSV infection. Therapeutically effective concentrations of palivizumab were maintained throughout the study period.
    UNASSIGNED: Palivizumab might be well tolerated and effective in preventing serious respiratory symptoms and hospitalization due to severe RSV infection, indicating the prophylactic use in the pediatric patients included in this study.
    UNASSIGNED: Japan Agency for Medical Research and Development (AMED), grant numbers 19lk0201097h0001 (to MM), 20lk0201097h0002 (to MM), 21lk0201097h0003 (to MM), and 22lk0201097h0004 (to MM). AMED did not have any role in the execution of this study, analysis and interpretation of the data, or the decision to submit the results.
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  • 文章类型: Journal Article
    暂无摘要。
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  • 文章类型: Journal Article
    本研究旨在评估原发性后气管固定术(PPT)在食管闭锁(EA)修复后降低呼吸机依赖性的有效性,以及需要在一年内再入院的呼吸道感染(RTI)的风险。
    这项回顾性队列研究招募了在2020年6月至2021年12月期间入院的EA患者。
    在PPT组(n=17)中,12例患者术后拔管时间为86.7h,1例患者(8.3%)需要重复术后插管;16例患者中有6例(37.5%)经历过至少一次RTI,需要在一年内住院。在非PPT组(n=17)中,14例患者拔管时间为127.0h,14例患者中有6例(42.9%)需要反复插管;17例患者中有12例(70.6%)在一年内经历了至少一次需要住院治疗的RTI。
    尽管由于参与者数量有限,差异没有达到统计学意义,在EA修复期间接受PPT的患者重复插管的机率较低,需要在一年内接受RTI的风险降低.
    UNASSIGNED: This study aimed to evaluate the effectiveness of primary posterior tracheopexy (PPT) in reducing ventilator dependence after repair of esophageal atresia (EA), and the risk of respiratory tract infections (RTI) requiring readmissions within one year.
    UNASSIGNED: This retrospective cohort study recruited patients with EA admitted to our hospital between June 2020 and December 2021.
    UNASSIGNED: In the PPT group (n = 17), the time to extubation after surgery was 86.7 h for 12 patients, with one patient (8.3%) requiring repeated postoperation intubation; six-in-sixteen patients (37.5%) experience at least one RTI requiring hospitalization in one year. In the non-PPT group (n = 17), the time to extubation was 127.0 h for 14 patients, with six-in-fourteen patients (42.9%) requiring repeated intubation; twelve-in-seventeen patients (70.6%) experienced at least one RTI requiring hospitalization in one year.
    UNASSIGNED: Although the differences did not reach statistical significance due to limited number of participants, patients underwent PPT during EA repair had lower chance of repeated intubation and decreased risk of RTI requiring admissions within one year.
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  • 文章类型: Journal Article
    背景:我们对1例先天性食管闭锁(CEA)术后吻合口狭窄患者进行了首次自体口腔粘膜来源的上皮细胞片移植治疗,并证实了其安全性。在这项研究中,新增加CEA和先天性食管狭窄患者作为研究对象,以进一步评估细胞片移植治疗的安全性和有效性.
    方法:从受试者的口腔粘膜制备上皮细胞片,并移植到内窥镜球囊扩张术(EBD)产生的食管泪液中。细胞片的安全性通过质量控制测试得到证实,48周的随访检查证实了移植治疗的安全性.
    结果:受试者1切除狭窄,因为第二次移植后EBD的频率没有降低。切除的狭窄的组织病理学检查显示粘膜下层明显增厚。受试者2和3在移植后48周内不需要EBD,在此期间,他们能够通过口腔维持正常的饮食。
    结论:移植后,受试者2和3长时间没有EBD,证实细胞片移植疗法在某些情况下明显有效。在未来,有必要研究更多的病例;开发新的技术,例如评估细胞片移植疗法疗效的客观指标和实现更准确移植的装置;确定当前疗法有效的病例;找到最佳的移植时机;并阐明当前疗法改善狭窄的机制。
    背景:UMIN,UMIN000034566,2018年10月19日注册,https://upload。乌明。AC.jp/cgi-open-bin/ctr_e/ctr_view。cgi?recptno=R000039393。
    We performed the first autologous oral mucosa-derived epithelial cell sheet transplantation therapy in a patient with refractory postoperative anastomotic stricture in congenital esophageal atresia (CEA) and confirmed its safety. In this study, patients with CEA and congenital esophageal stenosis were newly added as subjects to further evaluate the safety and efficacy of cell sheet transplantation therapy.
    Epithelial cell sheets were prepared from the oral mucosa of the subjects and transplanted into esophageal tears created by endoscopic balloon dilatation (EBD). The safety of the cell sheets was confirmed by quality control testing, and the safety of the transplantation treatment was confirmed by 48-week follow-up examinations.
    Subject 1 had a stenosis resected because the frequency of EBD did not decrease after the second transplantation. Histopathological examination of the resected stenosis revealed marked thickening of the submucosal layer. Subjects 2 and 3 did not require EBD for 48 weeks after transplantation, during which time they were able to maintain a normal diet by mouth.
    Subjects 2 and 3 were free of EBD for a long period of time after transplantation, confirming that cell sheet transplantation therapy is clearly effective in some cases. In the future, it is necessary to study more cases; develop new technologies such as an objective index to evaluate the efficacy of cell sheet transplantation therapy and a device to achieve more accurate transplantation; identify cases in which the current therapy is effective; and find the optimal timing of transplantation; and clarify the mechanism by which the current therapy improves stenosis.
    UMIN, UMIN000034566, registered 19 October 2018, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000039393 .
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  • 文章类型: Journal Article
    Several substitutes, including gastric transposition, colonic interposition, reverse gastric tube (RGT), etc., have been described for esophageal replacement in children and adolescents. However, the search for the ideal esophageal substitute continues due to adverse events associated with these procedures. This report presents our technique for creating an isoperistaltic gastric tube (IGT). We believe that the IGT is a versatile option for esophageal replacement as it is never length-limiting, and additional length can be gained by making minor adjustments.
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  • 文章类型: Journal Article
    先天性食管闭锁术后吻合口狭窄发生在30-50%的病例中。严重吞咽困难的患者接受内窥镜球囊扩张术(EBD)和/或局部注射类固醇治疗,但是许多患者仍然经常出现狭窄。在这项研究中,我们研究了自体口腔粘膜来源的细胞片(上皮细胞片)移植作为先天性食管闭锁术后吻合口狭窄的预防性治疗。
    从患者的口腔上皮组织制作上皮细胞片,它们的安全性得到了质量控制测试的证实。在受控条件下将上皮细胞片从制造设施运输到移植设施,并使用新开发的儿科患者移植设备成功移植到由EBD引起的撕裂上。通过48周的随访检查证实了移植的安全性。
    记录上皮细胞片移植前后一年进行EBD的日期,和间隔(天)进行评估。移植后约6个月,EBD之间的间隔时间比移植前一年长.患者还意识到移植后吞咽困难的减少。
    这些结果表明,细胞片移植可能是预防先天性食管闭锁术后吻合口狭窄的有效方法。但在这种情况下,效果是暂时的和有限的。尽管我们选择了一个非常严重的病例进行首次人体临床研究,如果在疾病变得如此严重之前进行移植,可能会获得更明确的效果。需要进一步的研究来确定细胞片移植最有效的情况,并确定移植的适当时间表。
    UMIN,UMIN000034566,2018年10月19日注册,https://upload。乌明。AC.jp/cgi-open-bin/ctr_e/ctr_view。cgi?recptno=R000039393。
    Congenital esophageal atresia postoperative anastomotic stricture occurs in 30-50% of cases. Patients with severe dysphagia are treated with endoscopic balloon dilatation (EBD) and/or local injection of steroids, but many patients continue to experience frequent stricture. In this study, we investigated the transplantation of autologous oral mucosa-derived cell sheets (epithelial cell sheets) as a prophylactic treatment for congenital esophageal atresia postoperative anastomotic stricture.
    Epithelial cell sheets were fabricated from a patient\'s oral epithelial tissue, and their safety was confirmed by quality control tests. The epithelial cell sheets were transported under controlled conditions from the fabrication facility to the transplantation facility and successfully transplanted onto the lacerations caused by EBD using a newly developed transplantation device for pediatric patients. The safety of the transplantation was confirmed by follow-up examinations over 48 weeks.
    The dates that EBD was performed were recorded for one year before and after epithelial cell sheet transplantation, and the intervals (in days) were evaluated. For about 6 months after transplantation, the intervals between EBDs were longer than in the year before transplantation. The patients were also aware of a reduction in dysphagia after transplantation.
    These results suggest that cell sheet transplantation may be effective in preventing anastomotic stricture after surgery for congenital esophageal atresia, but the effect was temporary and limited in this case. Although we chose a very severe case for the first human clinical study, it may be possible to obtain a more definitive effect if the transplantation is performed before the disease becomes so severe. Future studies are needed to identify cases in which cell sheet transplantation is most effective and to determine the appropriate timeframes for transplantation.
    UMIN, UMIN000034566, registered 19 October 2018, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000039393 .
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