胸腔镜下膈肌折叠术治疗婴幼儿右侧膈膨升

目的总结胸腔镜下膈肌折叠术治疗婴幼儿膈膨升的临床经验及疗效。方法自2009年2月至2011年12月作者收治儿童膈膨升15例。其中男12例,女3例,年龄3个月至3岁（平均年龄9个月）,回顾性分析手术方法、手术时间、住院时间、患儿康复及随访情况。结果15例均为右侧膈膨升,均在胸腔镜下顺利完成膈肌折叠术。手术时间1-3．5h（平均约2．5h）。术中出血少,无一例并发症,无中转开胸病例。患儿于术后3～7d出院,平均术后住院时间5．2d,2例术后出现肺部感染。术后随访2个月至2年,疗效满意,胸部正位片显示患儿膈肌分别较术前降低1～3个肋间隙,胸壁创伤小,外观满意。结论胸腔镜下膈肌折叠术适合于婴幼儿膈膨升,具有创伤小,恢复快,胸廓外观影响小的优势。     

腹腔镜下膈肌折叠术治疗小儿先心病术后膈膨升

目的介绍腹腔镜技术治疗小儿先心病术后膈膨升的手术方法并探讨其安全性和疗效。方法自2007年至2014年,我们收治20例先心病术后膈膨升患儿,其中左侧13例,右侧7例,平均年龄9.5个月。采用腹腔镜三孔法完成膈肌折叠术,必要时在左上腹放一3 mm Trocar辅助牵拉。探查膈肌后,用2-0不可吸收带针缝线自左腹壁穿入腹腔,尾线留在腹壁外,间断"手风琴"式膈肌折叠缝合。采用腹腔内绕线、腹腔外牵拉尾线辅助打结,效果不满意时缝合两层,使膈肌平直并下降至正常水平。结果 20例均在腹腔镜下完成手术,平均手术时间60 min,出血量5 mL。术后24~48 h进食。出院前复查立位胸片,患侧膈肌下降至正常水平,无术中术后并发症。术后平均随访14.5个月,1个月、3个月复查胸片膈肌位置无明显改变,呼吸道症状明显缓解,无复发病例。结论腹腔镜下膈肌折叠术治疗小儿先心病术后膈膨升操作方便,安全性好,疗效确切。     

横膈高度指数在儿童膈膨升中的临床意义

目的:检测中国儿童横膈高度指数（diaphragmatic height index,DHI）正常值范围,探讨其在膈膨升患儿中的临床意义。方法:收集2007年1月至2019年8月在上海交通大学医学院附属新华医院确诊为膈膨升的52例患儿相关资料。其中,男39例,女13例;平均年龄为15.7个月,范围在2.0~132.0个...     

先天性膈膨升伴动脉导管未闭1例

患儿 ,系第 2胎 ,第 2产 ,孕 41周 ,出生时有重度窒息 ,1分钟Ａｐｇａｒ评分 3分 ,经抢救后呼吸好转 ,但哭声仍不好 ,于生后 1小时从产科转入我科。入院查体 :体温 36 7℃ ,呼吸 6 0次 /分 ,体重 41 0 0ｇ发育良好 ,营养好 ,神志清 ,反应差 ,哭无回声 ,呼吸急促 ,面色及口唇发绀 ,颈无抵抗 ,并囟平软 ,双肺呼吸音清 ,未闻及干湿罗音。心率 1 40次 /分 ,未闻及杂音。腹平软 ,肝右肋下 2ｃｍ ,质软 ,脾未触及。拥抱反射、觅食反射减弱。入院诊断 :1 新生儿窒息 ;2 新生儿缺氧缺血性脑病 ;3 巨大儿 ;入院后予头罩吸氧、抗炎、减轻脑细胞水肿…     

腹腔镜下膈肌折叠术治疗小儿先天性右侧膈膨升的疗效分析

目的探讨腹腔镜下膈肌折叠术治疗小儿先天性右侧膈膨升的可行性。方法选取2002年1月至2013年12月于本院诊断为右侧膈膨升,并行膈肌折叠术的患儿17例,其中7例采取开放性手术(均经腹完成手术),为开放手术组;10例采取腔镜手术,为腹腔镜手术组。结果17例手术均获成功,术后胸片(DR)复查膈肌反常运动消失,Kienbock征均为阴性。治愈16例中,经开放手术治愈6例,腹腔镜手术治愈10例。开放手术组平均手术时间145 min,术后住院时间(11.19±2.29)d,术后1例并发切口感染,经换药至切口愈合出院;腹腔镜手术组平均手术时间130 min,术后住院时间(8.86±2.31)d,无一例发生并发症。随访半年至2年,无一例复发。结论腹腔镜治疗膈膨升相对安全,创口小,恢复快,效果满意,且可实现对腹腔其他脏器的探查,是值得推广的微创手术方式。     

经胸腔镜手术治疗13例新生儿及小婴儿膈膨升

目的 介绍胸腔镜下手术矫治新生儿及小婴儿膈膨升的治疗经验.方法 回顾性分析我院2011年4月至2013年7月经胸腔镜手术治疗的13例新生儿及小婴儿膈膨升临床资料.13例中,右侧11例,左侧2例;男性10例,女性3例;年龄8～177 d,平均96 d;体重2.5～6.9 kg,平均4.8 kg;均采取三孔法,在胸腔镜下行膈肌折叠术.结果 12例顺利经胸腔镜完成手术,1例因合并胎粪性腹膜炎而中转开腹手术.术后用氧时间36～ 150 h,平均66 h;住院时间6～14d,平均9d,术后胸片膈面位置均基本正常,随访3个月至2年,所有患儿生长发育正常,无一例复发.结论 经胸腔镜手术治疗新生儿、小婴儿膈膨升安全有效,能达到微创效果,但术者需具备熟练的微创手术技术及掌握适当的手术技巧.     

胸腔镜手术治疗新生儿先天性膈疝

目的探讨胸腔镜手术治疗新生儿膈疝的手术经验及技术难点分析。方法对27例新生儿CDH患儿采用胸腔镜手术治疗。顺序还纳疝入脏器,脾脏还纳时切忌使用尖锐器械直接压迫还纳,应间接推挤或以胃结肠等空腔脏器辅助推挤还纳,避免脾脏及血管撕裂导致出血。后外侧膈肌缺损严重者可行膈肌缘-肋间肌-膈肌缘缝合,适度打紧线结完成膈肌闭合修复。膈肌缺损严重可先连续缝合尽可能对合关闭缺损,测量缺损薄弱处大小,将体外裁剪好的补片置入胸腔,贴紧固定补片至膈肌薄弱处并以4-0 prolene线间断缝合。结果27例膈疝中左侧25例,右侧2例。产前诊断23例,产前诊断胎龄为(28.2±5.1)周;出生孕周为(37.5±2.7)周,孕周最小为28.0周;出生体重为(2.90±0.70)kg,最小为1.36 kg。所有患儿均采用胸腔镜手术,23例顺利完成,4例中转。存活23例(85%),死亡4例(15%)。手术年龄为出生后(41.0±40.0)h,手术时间为(159.0±14.0)min,行补片修补3例,术后有创呼吸机使用时间为(5.1±1.2)d,术后住院时间为(18.0±4.0)d。术后1例复发,第二次经胸腔镜完成手术,预后良好,3例并发胸腔积液均经保守治疗痊愈。结论胸腔镜手术治疗新生儿CDH安全可行,术者有丰富的胸腔镜操作经验下,可优先考虑,补片修补可以在腔镜下顺利完成,术中心肺功能不稳定,二氧化碳分压(PaCO2)持续高于65~75 mmHg(1 mmHg=0.133 kPa)应及时中转开放手术。低体重早产儿对胸腔镜手术的耐受性并不低于足月儿。     

胸腔镜手术治疗先天性膈疝的经验及技术要点分析

目的 总结分析胸腔镜手术治疗先天性膈疝的技术要点. 方法 回顾性分析首都儿科研究所附属儿童医院2014年4月至2021年4月采用胸腔镜手术治疗的59例先天性膈疝患者临床资料,包括出生体重、出生孕周、产前诊断孕周、膈疝位置等,对其手术后治疗情况及手术操作经验进行总结.结果 59例新生儿先天性膈疝患者中,产前诊断54例(9...     

胸腔镜手术治疗新生儿膈疝的临床研究

目的比较新生儿膈疝胸腔镜手术与开腹手术的临床疗效。方法2010年5月至2013年5月我们采取手术治疗23例新生儿膈疝病例。回顾性分析患儿术前准备、手术经过及术后恢复情况,比较开腹手术与胸腔镜手术相关并发症及预后。结果本组膈疝患儿总治愈率86．96％（20／23）,其中9例采取胸腔镜手术,1例中转开腹手术;1例实施胸腔镜手术后自动出院,治愈率87．50％（7／8）;15例采取开腹手术,其中2例死亡,治愈13例,治愈率86．67％（13／15）,两组比较差异无统计学意义。比较胸腔镜手术和开腹手术两组手术时间、术后抗生素使用时间以及住院时间,差异均有统计学意义（P值均〈0．05）;比较术后24hPCO：、胃肠喂养时间、术后机械通气时间、胸腔积液率及术后胸腔残余气腔率,胸腔镜手术组虽有优势,但差异无统计学意义。20例患儿随访I～37个月,均无复发。结论新生儿胸腔镜膈疝修补术不增加手术相关并发症,且治愈率同开腹手术,提示其手术安全可行。胸腔镜手术时间虽相对延长,但术后抗生素使用时间缩短,住院时间缩短,切口美观。     

Indications for thoracoscopic repair of congenital diaphragmatic hernia in neonates

Purpose  

We reviewed 26 consecutive cases of congenital diaphragmatic hernia (CDH) prospectively to establish selection criteria for successful thoracoscopic CDH repair (TR).     

Congenital diaphragmatic eventration and bilateral uretero-hydronephrosis in a patient with neonatal Marfan syndrome caused by a mutation in exon 25 of the FBN1 gene and review of the literature

Neonatal Marfan syndrome, the most severe presentation of Marfan syndrome phenotypes (MIM 154700), is characterised mainly by joint contractures, arachnodactyly, loose skin, crumpled ears, severe atrioventricular valve dysfunction and pulmonary emphysema. Death usually occurs within the first 2 years of life from congestive heart failure. We describe here a newborn male with many typical characteristics of neonatal Marfan syndrome associated with a diaphragmatic eventration and a bilateral uretero-hydronephrosis with bladder dilatation. He died from cardiac failure due to severe tricuspid and mitral regurgitation at 62 h of age. Conclusion:molecular analysis showed a heterozygous missense mutation at nucleotide 3165 (3165T>G) in exon 25 of the FBN1 gene, resulting in the substitution of cysteine for tryptophan (C1055W).Abbreviations cbEGF calcium binding EGF motif - CCA congenital contractural arachnodactyly - EGF epidermal growth factor - FBN1 fibrillin-1 gene - FBN2 fibrillin-2 gene - MFS Marfan syndrome - nMFS neonatal Marfan syndrome     

胸腔镜食管吻合治疗小儿先天性气管软骨食管异位症

目的 探讨小儿先天性气管软骨食管异位症的临床表现、诊断特点及胸腔镜食管端端吻合术治疗气管软骨食管异位症的方法、疗效.方法 收集2008年3月至2010年5月收治的5例先天性气管软骨食管异位症患儿,术前进行影像学检查及胃镜检查,术中经胸腔镜行食管狭窄段切除,食管端端吻合术,男3例,女2例,年龄1.1～4岁,无中转开胸.结...     

Pulmonary artery size as an indication for thoracoscopic repair of congenital diaphragmatic hernia in neonates

Purpose

We reviewed 24 consecutive cases of prenatally or immediately postnatally diagnosed left-sided congenital diaphragmatic hernia (CDH) to evaluate pulmonary artery (PA) size as an indication for thoracoscopic repair (TR).

Methods

CDH repair is planned once echocardiography confirms improvement in pulmonary hypertension. TR is chosen if cardiopulmonary status is stable more than 10?min in the decubitus position in the neonatal intensive care unit (NICU) under conventional mechanical or high frequency oscillatory ventilation (HFOV) with/without nitric oxide (NO) and the patient appears likely to tolerate manual ventilation during transfer to the operating room. Otherwise open repair (OR) is performed in NICU. Proximal right PA (RPA) and left PA (LPA) diameters measured at birth were assessed with respect to the type of repair.

Results

10/24 had TR and 14/24 had OR. TR cases had significantly larger RPA/LPA diameters (3.52?±?0.23 vs. 3.10?±?0.56?mm, p?p?p?p?n?=?3) and cardiopulmonary instability (n?=?1).

Conclusions

TR can be considered when RPA/LPA diameters are larger than 3.0/2.5?mm, respectively, and cardiopulmonary status is stable without NO.     

胸腔镜手术治疗婴儿先天性肺囊性畸形的可行性及手术时机探讨

目的探讨经胸腔镜手术治疗婴幼儿先天性肺囊性畸形的可行性、安全性及手术时机。方法回顾性分析本院2010年1月至2015年5月因先天性肺囊性畸形而采取胸腔镜手术治疗的10例患儿临床资料。分析其产前检查、产后情况、手术过程及手术后恢复情况。结果 10例中,男性4例,女性6例;手术年龄10天至6个月(平均4.6个月)。其中先天性肺囊腺瘤样畸形3例,肺隔离症6例,先天性肺囊腺瘤样畸形合并肺隔离症1例。10例均为产前检查疑肺囊性畸形,产后经胸部CT确诊。7例术前有肺部感染症状,经抗感染治疗后于生后6个月手术治疗,其中2例于生后1个月内手术,2例无症状,于生后3~6个月手术,患儿均治愈,元手术相关并发症,术后恢复顺利。患儿均在胸腔镜下完成手术。其中1例术后反复感染,2例术后并发肺炎,1例术后出现少许胸腔积液,均经治疗后痊愈。其余患儿恢复良好。平均手术时间175 min,术中平均出血约2 mL,术后拔除引流管时间平均为4.5 d,术后住院时间平均为8 d。全部患儿均门诊或电话随访,随访时间3个月至2年,平均1年4个月。均存活,无肺部相关症状,生长发育、活动量均与正常同龄儿无异。结论所有产前诊断怀疑肺囊性畸形的患儿产后均应随访,并定期行CT检查。对于产前诊断的肺囊性畸形患者,在产后3~6个月行胸腔镜手术治疗可取得满意效果。对于有症状的患儿1个月内行胸腔镜手术是安全的。经胸腔镜手术治疗婴幼儿先天性肺囊性畸形患儿,术后恢复快,手术创伤小,临床疗效满意,是安全和可行的。但手术者需具备熟练的微创手术技术及掌握适当的手术技巧。     

Efficacy of protocolized management for congenital diaphragmatic hernia. A review of 100 cases

A review of 100 consecutive cases of congenital diaphragmatic hernia (CDH) treated at our institute focusing on the efficacy of protocolized management (PM) was conducted. Of the 100 cases, 14 who became symptomatic more than 24 h after birth, and seven with fatal anomalies (four cardiac and three chromosomal) were excluded, leaving 79 subjects for this study. Of these, 41 were diagnosed prenatally (PD). Subjects were divided into four groups. Group I: No PD, no PM (n = 34), Group II: No PD, PM (n = 4), Group III: PD, no PM (n = 21), and Group IV: PD, PM (n = 20). PM includes criteria for planned delivery, use of high frequency oxygenation, nitric oxide, echocardiography (EC), and a medication schedule. Overall survival rates for Groups I, II, III, and IV were 73.5% (25/34), 75% (3/4), 38.1% (8/21), and 70.0% (14/20), respectively. Survival rates were higher when PM was used: 70.8% (Groups II, IV) versus 60.0% (Groups I, III). Survival rates were significantly lower if diagnosed prenatally (PD+): 53.7% (Groups III, IV) versus 73.7% (Groups I, II) (P < 0.01). However, in PD+ groups, survival was significantly higher if PM was used (P < 0.05). PM significantly reduced length of hospital stay (35.5 vs. 52.0 days: P < 0.05). EC was found to be a predictor for survival while post-ductal AaDO₂ was not. In 17 cases with cardiac anomalies, PM did not affect survival. Our study suggests that use of PM for prenatally diagnosed CDH cases is associated with improved outcome, although the components of PM need to be tested in prospective trials to determine their true value.     

Long-term morbidity of congenital diaphragmatic hernia: A plea for standardization

Congenital diaphragmatic hernia (CDH) survivors present long-term morbidities in several systems, including the neurodevelopmental, gastrointestinal, pulmonary, and musculoskeletal ones, and CDH long-term sequelae are increasingly being recognized. Due to high co-morbidity, health related quality of life in a significant proportion of CDH patients might be compromised. As a consequence of consciousness on the long-term sequelae of CDH survivors, and their consequences for life, several follow-up programs were brought to life worldwide. In this review, we will summarize the long-term sequelae of CDH survivors, the impact of new treatments, and analyze the consistency of follow-up programs.     

Thoracoscopic repair of diaphragmatic hernia in neonates and children: a new simplified technique

Needlescopic techniques have been used recently in different pediatric procedures, which made this type of surgery more feasible and less invasive with decreased hospital stay and improved cosmetic results. The technique is being developed further. New techniques with minor modifications are evolving every day. The objective of this study was to describe and assess the results that can be achieved by using a new simplified technique [Reverdin needle (RN)] in thoracoscopic repair of diaphragmatic hernia in neonates and children. Eighteen patients with symptomatic congenital diaphragmatic hernia (CDH), from Al-Azhar University Hospitals, Cairo, Egypt were assigned to elective thoracoscopic repair using RN to insert mattress sutures between the edges of diaphragmatic defects. The technique will be described in detail. A total of 18 diaphragmatic defects were repaired successfully; there were 12 males and 6 females with a mean age of 1.58 ± 21 months (range, 5 days–9 months). Left-sided CDH was present in 12 cases (67%) and right-sided CDH in 6 cases (33%). The mean operative time was 30.7 ± 1.18 min (range, 25–60 min) for each CDH repair. There were no intra or postoperative complications. There was one case of conversion and minimal blood loss. The mean postoperative hospital stay was 5.6 days (range, 2–10 days). There was only one case of mortality on the 10th postoperative day. There was no single case of recurrence. The new technique had all the advantages of thoracoscopy in children (less invasive, less pain, shorter hospital stay) combined with the advantages of reduced operating time, simplicity and feasibility. It may be preferable to intracorporeal suturing and knot tying.     

Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone

Aim  Prostaglandin-E1 (PGE₁) is used at most centers for treating pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH) because it has been regarded as effective. The aim of this study was to investigate the role of PGE1 for treating PH in CDH. Methods  We reviewed 49 CDH cases with echocardiography-proven PH. PH was treated with PGE₁ and nitric oxide (NO) and high frequency oscillatory ventilation (HFOV) from 1997 to 2001 (PG + NO; n = 19) and with NO and HFOV from 2002 to 2007 (NO; n = 30). Results  Subject demographics, severity of PH, and presence of other anomalies were not significantly different between the two groups. In the PG + NO group, 12/19 (63.2%) survived (PG + NO-s) and 7/19 (36.8%) died (PG + NO-d). In the NO group, 21/30 (70.0%) survived (NO-s) and 9/30 (30.0%) died (NO-d). Survival rates were not significantly different. In the NO-s group, spontaneous closure of the ductus arteriosus (DA) was significantly earlier compared with the PG + NO-s group (P < 0.01; 4.0 ± 0.9 vs. 9.5 ± 2.2 days after birth). DA diameters were significantly larger in groups that died compared with groups that survived (P < 0.01), and PH persisted in groups that died. In the NO-s group, surgery was possible significantly earlier compared with the PG + NO-s group (P < 0.01; 3.75 ± 0.67 vs. 6.12 ± 0.78 days after birth). No NO-s case developed a PH crisis even though PGE₁ was not used. Hospital stay was significantly shorter in the NO-s group compared with the PG + NO-s group (P < 0.05; 39.9 ± 19 vs. 53.2 ± 23 days). Conclusion  Nitric oxide alone would appear to simplify the management of CDH with PH and provide better outcome.