先天性肺淋巴管扩张症伴胎儿胸腔积液1例报告并文献复习

目的探讨先天性肺淋巴管扩张的临床及预后。方法回顾性分析1例表现为胎儿双侧胸腔积液的先天性肺淋巴管扩张症患儿的临床资料。结果患儿于胎儿期就表现为双侧胸腔积液,生后呼吸困难,双侧大量气胸、乳糜胸,低蛋白血症,持续性肺动脉高压。给予肺泡表面活性物质、NO吸入、高频机械通气、反复白蛋白输注,治疗3个月后出院。出院后第10天又以"重症肺炎、慢性肺病"收治入院,经机械通气、抗感染治疗2个月余,仍不能离氧,存活半年死亡。结论先天性肺淋巴管扩张症罕见,产前诊断困难。对于生后呼吸困难严重、有明显间质性肺气肿表现的患儿,应适时给予肺组织活检或核素淋巴管显影扫描以明确诊断。     

膈疝

新生儿膈疝多为先天性,食道裂孔疝临床发现较晚,诊断和治疗均较困难。先天性膈疝形成的原因是膈肌融合不全,使胸腹腔相通所致。膈疝的发病率左侧多于右侧,这是因为右侧肝脏挡住腹腔内脏对膈肌的突出所致。先天性膈疝于出生后表现为呼吸困难、循环障碍和消化功能紊乱。新生儿时期膈疝,如全身情况允许,应立即经腹腔修补;年龄稍大与胸腔有粘连者,应经胸腔修补。手术时如发现腹腔太小,不能容纳自胸腔内还纳至腹腔内之脏器,可以先缝合     

先天性右侧膈膨升并膈疝1例

先天性膈肌膨升及膈疝同时发生在一侧胸腔，且在右侧胸腔者极为罕见。我们经治１例，报告如下：患儿：女，２个月。足月顺产。生后哭闹时常出现紫绀，近日因肺炎收入作者单位：７１００３２西安，中国人民解放军第四军医大学西京医院（张洪伟、张广忖、陆军、舒茂国）；洛...     

胎儿磁共振在先天性膈疝患儿预后评估中的应用研究CSCD

目的回顾性分析先天性膈疝患儿产前胎儿磁共振图像,探讨磁共振测量和评价参数与患儿预后的关系,初步探讨胎儿磁共振参数在先天性膈疝患儿预后评估中的应用价值。方法收集西安交通大学第二附属医院2011—2020年胎儿磁共振图像结果,收集诊断为先天性膈疝的50例胎儿磁共振图像,包括左侧膈疝37例,右侧膈疝13例,检测并分析观察-预期肺容积比、肺肝比、纵膈移位角、肺底基线的完整性、肝脏及胃泡位置等与患儿预后的关系。结果50例患儿中,存活35例(为存活组),死亡15例(为死亡组)。胎儿磁共振图像观察-预期肺容积比:存活组比死亡组为(35.09±10.87)比(24.29±6.86);肺肝比:存活组比死亡组为(2.68±1.01)比(1.93±0.93);纵膈移位角:存活组比死亡组为(30.88±7.07)比(39.05±4.21);差异均有统计学意义(P<0.05)。图像分析结果显示,胎儿磁共振肺底基线不完整、肝脏及胃泡位置疝入胸腔患儿的预后较差,差异具有统计学意义(P<0.05)。受试者操作特征曲线分析结果显示,联合应用观察-预期肺容积比、肺肝比、纵膈移位角、肺底基线的完整性、肝脏及胃泡位置等判断患儿预后,较单一参数判断更为准确,差异具有统计学意义(P<0.05)。结论胎儿磁共振观察-预期肺容积比、肺肝比、纵膈移位角、肺底基线完整性、肝脏及胃泡位置均可作为判断先天性膈疝患儿预后的评价指标,联合应用上述六项参数可以更加准确地判断患儿预后,以上述参数为基础构建先天性膈疝的评估体系,有助于先天性膈疝患儿的肺发育评估、体外膜肺氧合的应用判断以及手术方式的选择。     

产前诊断先天性膈疝79例治疗与结局分析

目的总结先天性膈疝的围手术期管理经验,探讨产前诊断先天性膈疝的手术治疗时机、手术方法与临床疗效。方法 2014年1月至2016年11月通过产前诊断确诊为先天性膈疝患儿79例。根据LHR、O/E LHR以及肝脏位置将患儿分为轻、中、重度三组,对比分析各组手术时机、手术方法以及胸腔引流管放置情况。依据手术时机分为急诊手术组和亚急诊手术组;依据手术方法分为非微创组(经腹或经胸膈肌修补术)和微创组(胸腔镜下膈肌修补术)。结果 79例患儿中,产前评估轻度46例,中度24例,重度9例。产前诊断评估与出生后评估结果 Kappa值为0.695,具有较好一致性。治愈55例,死亡24例。比较轻度CDH患儿急诊手术和亚急诊手术,两组存活率差异无统计学意义(x~2=0.06,P=0.914);分别比较中度、重度CDH患儿急诊手术和亚急诊手术,两组存活率差异有统计学意义(P=0.001);79例CDH患儿中,轻、中度患儿复发率差异无统计学意义(P0.05)。32例未留置胸腔引流管的轻度CDH患儿中,8例在后期因胸腔积液行胸腔闭式引流;13例手术治疗的中度CDH患儿,8例未留置胸腔引流管;中度CDH患儿6例在后期因胸腔积液行胸腔闭式引流,1例术后死亡;5例手术治疗的重度CDH患儿,3例未留置胸腔引流管,术后死亡。结论手术时机对于轻度CDH预后无影响;对于中重度CDH,呼吸循环功能稳定后延期手术可以提高存活率;微创手术是一种安全、可行的手术方法;中重度CDH术后需留置胸腔引流管。     

先天性乳糜性心包积液1例报告并文献复习

目的报告1例先天性乳糜性心包积液并胸腔积液新生儿的临床表现、辅助检查与治疗转归。方法2010 - 07 - 05山东大学附属省立医院小儿重症医学科收治1例先天性原发性乳糜性心包积液并胸腔积液新生儿,回顾分析该患儿临床资料及诊疗过程,并复习国内外相关文献。结果该患儿产前超声检查即发现胎儿大量心包积液并胸腔积液,生后无明显症状,胸部正位X线片示心影增大（心胸比值0.75）,心脏超声示大量心包积液,胸部CT显示大量心包积液并左侧胸腔积液,心包穿刺抽液乳糜定性及生化检查证实积液性质为乳糜性;连续3次心包穿刺术无效后行心包闭式引流,放置导管持续性引流2d后痊愈;术后1周、1个月心脏超声检查无复发。结论原发性特发性乳糜性心包积液是一少见病症,多数病例仅能通过心包穿刺术证实诊断,心包穿刺及心包置管引流是有效的保守治疗手段,对复发性患者需要行手术治疗。     

先天性膈疝的临床特点及外科治疗经验

为提高新生儿先天性膈疝（CDH）的早期诊断、治疗及预后水平，对28例先天性膈疝病例进行临床特征、手术过程回顾性分析。全部进行正位及胸部X-线检查，5例做上消化道造影，13例做血气分析。X-线证实胸腔内有含水的肠管，腹析显示部分婴儿严重的缺氧、呼吸性酸中毒；消化道造影显示结肠或小肠在胸腔内。结果：2例新生儿放弃治疗，26例患儿手术治疗，3例死亡，23例病人恢复良好。结果：对患有先天性膈疝的新生儿必须早期诊断和手术治疗。     

小儿膈疝合并膈肌上气管支气管囊肿一例

患儿,男,5个月.因生后间断呼吸困难5个月入院.入院检查:生命体征平稳,心界右移,左侧胸腔中下野可闻及肠鸣音.胸部X光片及胸部CT均表现左侧胸腔下野含气空腔影,上消化道造影提示:左侧膈疝.胃、肠疝入胸腔.入院完善检查后,在气管插管全麻下手术.术中见左侧膈肌后外侧缺损约5 cm×3 cm,有一巨大薄壁疝囊突入胸腔,左肺下叶受压不张,疝囊后膈肌处有一4cm×3 cm×1 cm暗红色肿物,暗红色肿物起源于膈肌,无异常滋养血管,考虑为膈肌肿瘤,完整切除暗红色肿物.打开疝囊见内容物为胃、脾脏、部分结肠、小肠,探查脾脏正常,还纳胃、脾、肠管至腹腔,切除疝囊.膈肌缺损处缝合修补.术后痊愈出院.病理诊断:气管支气管囊肿(图1).     

先天性膈疝术后食管功能及胃食管反流的临床观察

目的 了解先天性膈疝修补术后预后及术后食管功能和胃食管反流情况。方法 对46例先天性膈疝修补术后进行随访，13例进行了24h食管双极pH及24h动态压力测定，了解胃食管功能。结果 13例进行了24h食管pH及压力测定的膈疝修补术后患儿中，7例有胃食管反流，其中3例术中应用补片，4例术前显示胃疝入胸腔。结论 先天性膈疝修补术后可出现胃食管反流，24h测压及pH测定表现为食管蠕动功能明显减弱，这种食管下端蠕动功能减弱与膈疝修补术后出现胃食管连接解剖异常及膈肌发育不良可能相关。     

新生儿先天性右侧乳糜胸1例

患儿，男，10天，以“生后气促10天”为主诉入院。患儿系G1P1，孕37周在我院出生，足月顺产，出生体重2510g，Apgar评分9’-10’-10’，羊水清，无早破水。生后不久发现气促，无呻吟、发绀、口吐泡沫，但哭声洪亮，未引起家长重视。生后第7天院外行胸片检查发现右侧胸腔积液，给予头孢他啶抗感染治疗无效，遂转入我院。追述病史，其孕母在孕32周做产前B超时即发现胎儿右侧胸腔积液。     

Yellow nail syndrome in infancy

Abstract: This report describes an infant with clinical features consistent with the yellow nail syndrome (YNS), a rare autosomal dominant disorder. He presented at birth with congenital lymphoedema and was referred at 6 months of age for investigation of recurrent cough and wheeze. He had clinical and radiological evidence of bilateral pleural effusions and a pericardial effusion. Following a lung biopsy and pericardial window these were shown to be manifestations of his lymphatic abnormality. He also had persisting middle ear effusions causing conductive deafness requiring hearing aids and secondary immunodeficiency requiring regular immunoglobulin infusions.     

Right-sided diaphragmatic hernia masquerading as staphylococcal pneumonia

A 9-month-old infant who was diagnosed to have right-sided diaphragmatic hernia with no other associated anomalies, is being reported here. He had presented with cough for one month and respiratory difficulty for one-week duration and history of bilious vomiting two days prior to admission. His chest X-ray showed multiple lucent shadows in the right lower zone in A-P and lateral views, with not very well defined diaphragm on the same side. A diagnosis of staphylococcal pneumonia was considered but with clinical and radiological picture, a right-sided diaphragmatic hernia was strongly suspected which was confirmed by doing fluoroscopy and CT-scan of the chest. He underwent a successful correction of the defect. So radiological finding of pleural effusion with displacement of mediastinum to the left and the presence of bowel gas high in the right upper quadrant should alert the possibility of a right-sided Bochdalek hernia.     

Pleural effusions in the neonate

Introduction: Pleural effusions are rare in the neonate and may be associated to several clinical conditions. Only a few series of pleural effusions in the fetus and newborn are described in the literature.

Aim: This study was undertaken to determine more accurately the causes and prognostic significance of pleural effusions in a population of high-risk neonates.

Materials and methods: A retrospective chart review of 62 neonates admitted to the neonatal intensive care unit of six medical centers in the north of Portugal, between 1997 and 2004, that presented the diagnosis of pleural effusion.

Results: 33M/29F newborns; preterms 47 (76%); GA 33 (25-40) wk; BW 1830 (660-4270) g; C-section 39 (63%). Pleural effusions were congenital in 20 (32%) newborns and acquired in 42 (68%). Congenital pleural effusions occurred as fetal hydrops in 11 (18%) patients and as chylothorax in 9 (15%). In four cases of hydrops, the cause was a congenital chylothorax. Congenital chylothorax (n=13) was the most common (65%) congenital pleural effusion in this study. The incidence of congenital chylothorax was 1:8.600 deliveries and male:female ratio was 2:1. Mortality occurred in five newborns due to pulmonary hypoplasia. Traumatic (iatrogenic) were the most frequent (n=31) acquired pleural effusions. These included 8 (13%) cases of hemothorax and 8 (13%) cases of total parenteral nutrition leakage. Pleural effusions after intra-thoracic surgery were mainly (79%) chylothoraces. There were 11 (26%) non-iatrogenic acquired pleural effusions. No mortality was associated with acquired pleural effusions.

Conclusions: Congenital pleural effusions usually occur as hydrops or congenital chylothorax. Traumatic (iatrogenic) are the most frequent acquired pleural effusions in a tertiary NICU. Pleural effusions after intra-thoracic surgery are mainly chylothoraces. Non-iatrogenic acquired pleural effusions are associated to several clinical conditions, and mortality is usually associated to the underlying condition.     

Congenital diaphragmatic hernia with a late disclosure

Two further cases of congenital diaphragmatic hernia with delayed presentation are reported: a 6-month-old male presented a posterolateral diaphragmatic hernia with small bowel in left hemithorax masquerading as pleural effusion; an 11-year-old boy with Down's syndrome presented a retrocostoxyphoid hernia revealed by vague faintness. The authors emphasize the deceptive clinical aspect, the different means for diagnosis, the risk of wrong diagnosis and pleural drain, the usual good outcome of these late-onset diaphragmatic herniation.     

Successful Treatment of Eosinophilic Pleural Effusions Following Congenital Heart Surgery

We report two children, age 7 months and 5 years, who underwent surgery for congenital heart disease and developed persistent pleural effusions with elevated eosinophil counts. Given the elevation of eosinophil counts in both blood and pleural fluid of these patients, it was considered that an allergic response might have caused the persistent effusion. In both cases, the effusion resolved within 48 hours after treatment with corticosteroids was begun. It is possible that postoperative eosinophilic pleural effusion may represent a subgroup of effusions that are more likely to respond to treatment with corticosteroids.     

Late onset of right Bochdalek's hernia with strangulation of the omentum

We report a case of bloody pleural effusion and infarction of the greater omentum caused by a non-traumatic diaphragmatic hernia with a late presentation. A 15-y-old boy with Down's syndrome developed abdominal pain and vomiting, as well as an elevated serum level of C-reactive protein. Chest roentgenograms showed a right-sided pleural effusion and computed tomography revealed a right diaphragmatic hernia. Barium enema confirmed the diagnosis. An operation revealed a right Bochdalek's hernia with strangulation of the greater omentum in the right pleural cavity. Conclusion: Diaphragmatic hernia should be considered in patients with pleural effusion, abdominal pain and vomiting.     

Interferon alpha 2b treatment in an eleven-year-old boy with disseminated lymphangiomatosis

Disseminated lymphangiomatosis is a rare disease mostly observed in children and young adults. If no surgical removal can be achieved, the prognosis is poor, especially for patients with thoracic localization and pleural effusions. Next to pleural drainage, therapeutic options include radiotherapy, local, and systemic pharmacotherapy. An 11-year-old boy presented with disseminated lymphangiomatosis involving thorax with massive pleural effusions, retroperitoneum, and bones. In immunohistochemical analysis, the tissue biopsy stained positive for vascular endothelial growth factor-receptor 3 (VEGFR-3). The patient has been treated with interferon alpha 2b for 2 years, and achieved a good clinical and radiological response.     

Late-appearing diaphragmatic hernia following resolution of right pleural effusion

A case of late-appearing congenital diaphragmatic hernia in a premature infant with previously normal chest X-ray is reported. Pleural effusion accumulation and resolution preceded herniation of the liver to the right hemithorax and development of respiratory symptoms. Chest X-ray, ultrasound and computed tomography of the chest were useful in establishing the correct diagnosis. Pleural effusion without obvious cause should alert the paediatrician to the possibility of this rare condition.     

Neonatal cardiac tamponade and pleural effusion resolved with chest tube placement

Pericardial effusion and cardiac tamponade secondary to umbilical venous catheterization are rare complications but potentially fatal. This article reports a case of cardiac tamponade and right pleural effusion secondary to transudation of hyperosmolar fluid from an appropriately placed umbilical venous catheter. The infant survived as a result of early diagnosis by echocardiography and urgent chest tube placement that drained both pleural and pericardial effusions. Cardiac tamponade should be highly suspected in any neonate with a central venous catheter who develops sudden, unexplained clinical deterioration in cardiopulmonary status even when the line is properly placed, and urgent echocardiography or pericardiocentesis should be considered early in management of such patients. Umbilical venous catheterization should be considered only for a select group of sick neonates due to risks involved with these lines. When an umbilical venous catheter is placed, special precautions should be taken and maintenance guidelines followed.