Congenital Diaphragmatic hernia

先天性膈疝
  • 文章类型: Case Reports
    先天性膈疝(CDH)是一种发育障碍,其中膈肌,将胸部和腹部分开的肌肉,在产前发育期间不会关闭,让腹部器官突出进入胸腔.它主要发生在左侧(80%-85%的病例)。CDH通常在产前评估期间被识别。然而,婴儿期后出现CDH的情况非常罕见,导致频繁的误诊和延迟的治疗干预。我们介绍了一例10个月大的女性,其产前和围产期病史平安无事,出现呼吸窘迫和多次呕吐。她到达时的生命体征稳定,但她因活动不足被送进了PICU,减少口服摄入,和激动。紧急CT扫描后,发现了小肠和大肠环向右半胸部的突出,以及右半膈肌的缺陷。这导致右侧胸腔积液,左肺部分塌陷,纵隔向左移动。膈疝是通过第六根肋骨的侧方开胸手术用多根间断缝线纠正的,然后在碗顺利复位后,将胸管插入隔膜上方的胸膜腔中。这个病例突出了早期诊断的重要性,适当的临床调查,和治疗。通过及时发现和检查病情,可以预期良好的预后。
    Congenital diaphragmatic hernia (CDH) is a developmental disorder in which the diaphragm, the muscle that separates the chest from the abdomen, does not close during prenatal development, allowing abdominal organs to herniate into the chest cavity. It occurs mainly on the left side (80%-85% of cases). CDH is often identified during prenatal assessment. However, instances of late-presenting CDH beyond infancy are exceedingly uncommon, contributing to frequent misdiagnosis and delayed therapeutic intervention. We present a case of a 10-month-old female with an uneventful antenatal and perinatal history who presented with respiratory distress and multiple episodes of vomiting. Her vital signs were stable upon arrival, but she was sent to the PICU due to hypoactivity, reduced oral intake, and agitation. After an urgent CT scan, a herniation of the small and large bowel loops into the right hemithorax was discovered, along with a defect in the right hemidiaphragm. This resulted in a pleural effusion on the right side, a partially collapsed left lung, and a mediastinal shift to the left. The diaphragmatic hernia was corrected through a lateral thoracotomy at the sixth rib with multiple interrupted sutures, and a chest tube was then inserted into the pleural space above the diaphragm following a smooth reduction of the bowl. This case highlights the importance of early diagnosis, appropriate clinical investigation, and treatment. A good prognosis can be anticipated by promptly discovering and examining the condition.
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  • 文章类型: Case Reports
    与肝肺融合(HPF)相关的右侧先天性膈疝(CDH)是一种罕见的先天性异常,其中疝肝与肺实质融合。我们讨论了在索引手术中发现的右侧CDH和HPF婴儿的情况,以修复右侧CDH。由于与HPF相关的血管异常发生率很高,因此决定关闭患者并进行进一步的成像以表征HPF,然后返回手术室进行最终修复。我们描述了一种新颖而成功的手术方法,其中肝脏完全与肺部融合,而肝脏部分从胸部向下移动,并折叠到隔膜上,从而在顶腔和胸膜腔之间形成密封以修复CDH。
    Right sided congenital diaphragmatic hernia (CDH) associated with hepatopulmonary fusion (HPF) is a rare congenital anomaly in which the herniated liver is fused with lung parenchyma. We discuss the case of an infant with right-sided CDH and HPF found on index operation for repair of right-sided CDH. Due to the high incidence of vascular anomalies associated with HPF the decision was made to close the patient and get further imaging to characterize the HPF before returning to the operating room for definitive repair. We describe a novel and successful operative approach where the liver was left entirely fused to the lung and the liver was partially brought down from the chest and was plicated to the diaphragm form a seal between the parietal and pleural cavities to repair the CDH.
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  • 文章类型: Case Reports
    先天性肺隔离症是一种罕见的肺异常,可分为叶内肺隔离症或叶外肺隔离症(ELS)。膈肌性肺隔离症是一种罕见的ELS。此外,胸内肾(ITK)是一种罕见的疾病,在0.25%的病例中可能与先天性膈疝(CHD)相关。我们报告了第一例与儿童CDH相关的the肌隔离症和ITK。
    病人,男性,6个月大,2个月前因呼吸急促到我们医院就诊.基于胸部超声和增强计算机断层扫描(CT)检查,膈肌隔离症和ITK被认为与CDH相关.患者入院治疗。入院后,他的血压是85/61mmHg,没有血尿或蛋白尿,肌酐为14μmol/L,尿素氮为2.96mmol/L,所有这些都没有异常。在手术治疗之前进行了完整的术前检查。胸腔镜检查显示,右肾在隔膜的后外侧突出到胸腔中。右肾回到腹腔,疝囊被打开了,观察到明亮的红色病变组织,边界清晰,血管供应异常。切断异常血管后,LigaSureTM用于切除病变组织,可见肾脂肪囊和肾组织。对疝环进行间歇性缝合以密封膈疝。术后病理检查提示膈肌隔离症。患者术后恢复顺利,2个月时胸部CT扫描显示右肾已回到腹腔,右膈肌位置正常。
    与CDH相关的膈肌隔离症和ITK极为罕见。可以使用增强的CT来制定诊断和适当的手术计划。对于位于CHD疝囊顶部的膈肌隔离症,胸腔镜下切除膈肌隔离症和修补术治疗膈肌疝是可行和有效的。
    UNASSIGNED: Congenital pulmonary sequestration is a rare lung anomaly that can be classified as intralobar pulmonary sequestration or extralobar lung sequestration (ELS). Infradiaphragmatic pulmonary sequestration is a rare type of ELS. Furthermore, intrathoracic kidney (ITK) is a rare disease that can be associated with a congenital diaphragmatic hernia (CHD) in 0.25% of cases. We report the first case of infradiaphragmatic pulmonary sequestration and ITK associated with CDH in a child.
    UNASSIGNED: The patient, male, aged 6 months, visited our hospital 2 months prior due to shortness of breath. Based on chest ultrasonography and enhanced computed tomography (CT) examination, infradiaphragmatic pulmonary sequestration and ITK were considered to be associated with CDH. The patient was admitted to our hospital for treatment. After admission, his blood pressure was 85/61 mmHg, there was no hematuria or proteinuria, creatinine was 14 µmol/L, and urea nitrogen was 2.96 mmol/L, all of which showed no abnormalities. A complete preoperative examination was performed prior to surgical treatment. Thoracoscopy revealed that the right kidney had herniated into the chest cavity on the posterolateral side of the diaphragm. The right kidney was returned to the abdominal cavity, the hernia sac was opened, and a bright red lesion tissue with clear boundaries and an abnormal blood vessel supply was observed. After cutting off the abnormal blood vessels, LigaSure TM was used to remove the diseased tissue, and the renal fat sacs and renal tissue were visible. Intermittent suturing of the hernia ring was performed to seal the diaphragmatic hernia. Postoperative pathological examination revealed infradiaphragmatic pulmonary sequestration. The postoperative recovery of the patient was smooth, and a chest CT scan at 2 months showed that the right kidney had returned to the abdominal cavity and the right diaphragm was in the normal position.
    UNASSIGNED: Infradiaphragmatic pulmonary sequestration and ITK associated with CDH is extremely rare. A diagnosis and appropriate surgical planning can be developed using enhanced CT. For infradiaphragmatic pulmonary sequestration located at the top of the hernia sac in CHD, thoracoscopic resection of the infradiaphragmatic pulmonary sequestration and repair of the diaphragmatic hernia is feasible and effective.
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  • 文章类型: Case Reports
    隔膜的先天性缺陷,被称为Bochdalek疝气(BH),是一种允许腹部内脏突出进入胸部的疾病。BH是最常见的先天性膈疝(CDH)类型,通常在左侧检测到。异位肾脏是一种罕见的疾病。胸内异位肾脏是一种极其罕见的疾病。在成年患者中,胸内肾BH的存在极为罕见,通常是无意发现的。一名51岁的男性患者被送往肺科门诊。他表示自己一直有咳嗽等症状,喘息,呼吸困难一年。胸部X射线检查显示左下区域有明确的不透射线的病变。胸部计算机断层扫描(CT)扫描显示左半膈后外侧区域有缺陷,以及左肾突出和左半胸腹膜后脂肪。发现胸内异位肾大小正常,表现为正常衰减和增强,在CT尿路造影期间,造影剂迅速排泄到骨盆系统中。由于疝气体积小,CT尿路造影缺乏异常,患者被建议保守治疗.每年对患者进行随访检查。在整个随访期间,没有一例肾脏相关问题.为了避免不必要的图像引导活检和外科手术,对于出现胸部肿块或半膈肌抬高的患者,必须寻找胸内肾脏。
    A congenital defect in the diaphragm, known as a Bochdalek hernia (BH), is a condition that allows herniation of the abdominal viscera into the thorax. BH is the most common type of congenital diaphragmatic hernia (CDH) and is typically detected on the left side. An ectopic kidney is a rare condition. An intra-thoracic ectopic kidney is an extremely uncommon condition. In adult patients, the presence of BH with an intra-thoracic kidney is extremely uncommon and is often a finding discovered unintentionally. A 51-year-old male patient presented to the outpatient unit of the pulmonology department. He stated that he had been suffering symptoms such as coughing, wheezing, and breathing difficulties for one year. A chest X-ray showed a well-defined radio-opaque lesion in the lower left zone. A computed tomography (CT) scan of the chest demonstrated a defect in the posterolateral region of the left hemidiaphragm, as well as herniation of the left kidney and retroperitoneal fat in the left hemithorax. The intra-thoracic ectopic kidney was found to be normal in size and showed normal attenuation and enhancement, with the contrast being promptly excreted into the pelvicalyceal system during CT urography. Due to the hernia\'s small size and lack of abnormalities on CT urography, the patient was recommended a conservative treatment. A follow-up examination was performed on the patient annually. Throughout the follow-up period, there was not a single episode of kidney-related issues. To avoid unwanted image-guided biopsies and surgical procedures, it is imperative to look for intra-thoracic kidneys in patients presenting with a thoracic mass or an elevated hemi-diaphragm.
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  • 文章类型: Journal Article
    目的:血管加压素具有全身血管收缩和肺血管舒张作用,使其成为先天性膈疝(CDH)相关肺动脉高压婴儿低血压管理的理想药物。加压素在这个人群中的副作用,比如低钠血症,研究不足。这项研究旨在表征加压素对患有和不患有CDH的婴儿钠浓度的影响。
    方法:本研究是对接受血管加压素治疗的患者进行的回顾性研究。主要结果是血管加压素治疗期间低钠血症(血钠<135mmol/L)的发生率。次要结果包括低钠血症时间,加压素的剂量和持续时间,严重低钠血症的发生率(血钠<125mmol/L),和高渗盐水的使用。血清和血气样品钠浓度均用于比较CDH与非CDH患者。
    结果:对于所有样本,CDH和非CDH患者,基线和最低血钠之间的平均差异均显着(p<0.001)。主要结局没有显着差异,低钠血症时间或血管加压素输注持续时间的次要结局也是如此。CDH组的加压素平均剂量高于非CDH组(p=0.018)。对于收集了血清钠样本的患者,CDH组的严重低钠血症和高渗盐水使用的发生率高于非CDH组(分别为p=0.049和p=0.033)。
    结论:这项研究表明,CDH患者与非CDH患者相比,严重低钠血症的发生率更高。在CDH患者中管理总钠时,必须格外小心。
    OBJECTIVE: Vasopressin has systemic vasoconstrictive yet pulmonary vasodilatory effects, making it an ideal agent for hypotension management in infants with congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension. The side effects of vasopressin in this population, such as hyponatremia, are understudied. This study aims to characterize the effect of vasopressin on sodium concentrations in infants with and without CDH.
    METHODS: This was a retrospective review of patients who received vasopressin while admitted to a level IV neonatal intensive care unit. The primary outcome was the incidence of hyponatremia (blood sodium <135 mmol/L) during vasopressin therapy. Secondary outcomes included time to hyponatremia, dose and duration of vasopressin, incidence of severe hyponatremia (blood sodium <125 mmol/L), and hypertonic saline use. Both blood serum and blood gas sample sodium concentrations were used to compare CDH vs non-CDH patients.
    RESULTS: The average difference between baseline and lowest blood sodium was significant for both CDH and non-CDH patients for all samples (p < 0.001). There was no significant difference in the primary outcome, nor in the secondary outcomes of time to hyponatremia or duration of vasopressin infusion. The average dose of vasopressin was higher in the CDH vs non-CDH group (p = 0.018). The incidences of severe hyponatremia and hypertonic saline use were greater in the CDH vs non-CDH group for patients who had blood serum sodium samples collected (p = 0.049 and p = 0.033, respectively).
    CONCLUSIONS: This study showed that severe hyponatremia occurred more frequently in CDH vs non-CDH patients. Extreme caution is necessary when managing total body sodium in patients with CDH.
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  • 文章类型: Case Reports
    膈疝是一种先天性畸形,经常在新生儿期发现,它在成人中的发生非常罕见。这个病人,在62岁之前完全无症状,患有肠梗阻,并在外耳道癌手术后出现呼吸衰竭。他随后被诊断出患有迟发性先天性膈疝(CDH),因此需要手术治疗。当围手术期观察到原因不明的膈肌抬高时,麻醉师和重症监护医师应牢记CDH和膈肌松弛的可能性。
    Diaphragmatic hernia is a congenital malformation, often discovered in the neonatal period, and its occurrence in adults is very rare. This patient, who was completely asymptomatic until the age of 62, had developed an intestinal obstruction and went into respiratory failure after surgery for an external auditory canal carcinoma. He was subsequently diagnosed with a late-presenting congenital diaphragmatic hernia (CDH), thus requiring surgical treatment. Anesthesiologists and critical care physicians should keep in mind the possibility of CDH as well as diaphragmatic relaxation when an unexplained elevation of the diaphragm is observed perioperatively.
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  • 文章类型: Case Reports
    Morgagni疝(MH),也称为胸骨后或胸骨旁疝,是一种罕见的先天性膈疝(CDH),其特征是前膈缺损。晚期诊断为MH的患者通常表现为模糊的胃肠道或呼吸道症状。在某些情况下,由于其他原因,通过胸部X光检查偶然发现了MH,如异物摄入,正如我们提出的案例所示。我们介绍了一个有异物摄入和严重腹痛史的两岁男孩的Morgagni型先天性膈疝的病例。诊断成像,包括胸部X光片和计算机断层扫描(CT)扫描,证实了膈肌缺损.手术修复,腹腔镜检查,导致简单的术后过程和良好的长期结果。
    Morgagni hernia (MH), also known as a retrosternal or parasternal hernia, is a rare type of congenital diaphragmatic hernia (CDH) characterized by a defect in the anterior diaphragm. Patients with late-diagnosed MH typically present with vague gastrointestinal or respiratory symptoms. In some instances, MH is incidentally identified through chest X-rays performed for other reasons, such as foreign body ingestion, as illustrated in our presented case. We present a case of a delayed congenital diaphragmatic hernia of the Morgagni type in a two-year-old boy with a history of foreign body ingestion and severe abdominal pain. Diagnostic imaging, including chest radiograph and computed tomography (CT) scan, confirmed the diaphragmatic defect. Surgical repair, performed laparoscopically, resulted in an uncomplicated postoperative course and a favorable long-term outcome.
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  • 文章类型: Journal Article
    背景:一些先天性膈疝的诊断时间超过1个月。晚期表现的先天性膈疝表现出多种临床表现,术前临床过程是可变的。我们在此报告了一例诊断为急腹症的迟发性先天性膈疝的儿科病例。
    方法:一名5岁男孩因肠疝进入左胸腔而被带到我们医院,这是在腹部疼痛的X线摄影中观察到的。增强计算机断层扫描显示小肠和结肠突出进入左胸腔。根据左膈疝的诊断进行急诊腹腔镜手术。整个小肠和部分结肠从膈后外侧缺损突出。我们能够将突出的肠缩回到腹部,但证实通过腹腔镜检查,膈肌缺损和缺损的闭合似乎在技术上具有挑战性;因此,我们将手术改为开腹手术.膈肌缺损直接用断续缝线闭合,胸腔被脱气。术后,发现左肺扩张不良,但在这种情况下,肺发育不全并不明显。
    结论:我们在此报告一例诊断为腹痛的迟发性先天性膈疝的儿科病例。迟发性先天性膈疝表现出各种各样的症状;因此,对于病因不明的急性或慢性呼吸道或胃肠道症状的儿童,重要的是要注意这些情况并检查胸片。
    BACKGROUND: Some congenital diaphragmatic hernias are diagnosed beyond 1 month. A late-presenting congenital diaphragmatic hernia shows a variety of clinical manifestations, and the preoperative clinical course is variable. We herein report a pediatric case of late-presenting congenital diaphragmatic hernia diagnosed as acute abdomen.
    METHODS: A 5-year-old boy was brought to our hospital because of herniation of the intestine into the left thoracic cavity, which was observed on radiography performed for abdominal pain. Enhanced computed tomography showed herniation of the small intestine and colon into the left thoracic cavity. Emergency laparoscopic surgery was performed based on the diagnosis of left diaphragmatic hernia. The entire small intestine and part of the colon herniated from the posterolateral defect of the diaphragm. We were able to retract the herniated intestine back into the abdomen but confirmed that the diaphragmatic defect and closure of the defect seemed to be technically challenging via laparoscopy; therefore, we converted the procedure to open laparotomy. The diaphragmatic defect was directly closed with interrupted sutures, and the thoracic cavity was degassed. Postoperatively, the left lung was found to be poorly expanded, but pulmonary hypoplasia was not evident in this case.
    CONCLUSIONS: We herein report a pediatric case of late-presenting congenital diaphragmatic hernia diagnosed as abdominal pain. Late-presenting congenital diaphragmatic hernias present with a wide variety of symptoms; therefore, it is important to be reminded of these conditions and check chest radiographs in children presenting with acute or chronic respiratory or gastrointestinal symptoms of unknown etiology.
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  • 文章类型: Journal Article
    目的:先前的研究表明,与开放修补术(OR)相比,胸腔镜(TR)先天性膈疝(CDH)的复发率更高,手术时间更长。进行了更新的荟萃分析以重新评估TR的手术结果。
    方法:根据PRISMA声明(PROSPERO:CRD42020166588),对新生儿的TR和OR进行了全面的文献检索。
    结果:选择了14项研究进行定量分析,包括总共709例患者(TR:308例,或:401例)。复发率较高[赔率:4.03,95%CI(2.21,7.36),p<0.001]和手术时间(分钟)更长[平均差(MD):43.96,95%CI(24.70,63.22),与OR相比,TR的p<0.001。与OR(14.8%)相比,TR(5.0%)观察到术后肠梗阻的发生率显着降低[赔率:0.42,95%CI(0.20,0.89),p=0.02]。
    结论:TR仍然与较高的复发率和较长的手术时间相关。然而,术后肠梗阻风险降低提示潜在的长期益处.这项研究强调了精心选择TR患者以减轻对严重疾病患者的不利影响的重要性。
    OBJECTIVE: Previous studies have shown a higher recurrence rate and longer operative times for thoracoscopic repair (TR) of congenital diaphragmatic hernia (CDH) compared to open repair (OR). An updated meta-analysis was conducted to re-evaluate the surgical outcomes of TR.
    METHODS: A comprehensive literature search comparing TR and OR in neonates was performed in accordance with the PRISMA statement (PROSPERO: CRD42020166588).
    RESULTS: Fourteen studies were selected for quantitative analysis, including a total of 709 patients (TR: 308 cases, OR: 401 cases). The recurrence rate was higher [Odds ratio: 4.03, 95% CI (2.21, 7.36), p < 0.001] and operative times (minutes) were longer [Mean Difference (MD): 43.96, 95% CI (24.70, 63.22), p < 0.001] for TR compared to OR. A significant reduction in the occurrence of postoperative bowel obstruction was observed in TR (5.0%) compared to OR (14.8%) [Odds ratio: 0.42, 95% CI (0.20, 0.89), p = 0.02].
    CONCLUSIONS: TR remains associated with higher recurrence rates and longer operative times. However, the reduced risk of postoperative bowel obstruction suggests potential long-term benefits. This study emphasizes the importance of meticulous patient selection for TR to mitigate detrimental effects on patients with severe disease.
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  • 文章类型: Case Reports
    简介:Bochdalek\'s疝(BH)是一种先天性膈疝,主要在儿科人群中诊断,但很少在成人中发现。本文介绍了一名成年患者的独特病例,该患者患有左侧BH并伴有胃扭转和胸内肾。
    一名21岁男性出现腹痛和呕吐。MDCT扫描显示胃扭曲,脾,脾肾脏因左膈膨出而突出进入胸部.手术包括解开胃,重新安置器官,切除疝囊.
    结论:Bochdalek疝(BHs)是罕见的疾病,其中腹部器官由于隔膜缺损而进入胸部。BH通常发生在左侧,可以由怀孕等因素触发,肥胖,或者外伤.症状可能从腹痛到胸部不适,诊断可能具有挑战性。诸如CT扫描之类的成像测试对于准确诊断至关重要。在成年人中,BH可以包含各种器官,如脾脏和肾脏。很少,BH可与位于胸腔内的异位肾相关联。在BH的某些情况下,有并发症的风险,如胃扭转,胃会自己扭曲,导致严重的症状,如严重的腹痛和呕吐。
    结论:该案例强调了成人BH的严重风险,例如胃扭曲和阻塞,需要紧急手术。及时诊断和手术干预对于预防危及生命的结果至关重要。需要更多的研究来改善这种罕见疾病的管理。
    UNASSIGNED: Introduction: Bochdalek\'s hernia (BH) is a congenital diaphragmatic hernia predominantly diagnosed in the pediatric population but infrequently found in adults. This paper presents a unique case of an adult patient with a left-sided BH accompanied by gastric volvulus and an intrathoracic kidney.
    UNASSIGNED: A 21-year-old male presented with abdominal pain and vomiting. An MDCT scan revealed a twisted stomach, spleen, and kidney herniated into the chest due to left diaphragmatic eventration. Surgery involved untwisting the stomach, relocating the organs, and removing the hernia sac.
    CONCLUSIONS: Bochdalek hernias (BHs) are rare conditions in which abdominal organs move into the chest due to defects in the diaphragm. BH usually occurs on the left side and can be triggered by factors such as pregnancy, obesity, or trauma. Symptoms can vary from abdominal pain to chest discomfort, and diagnosis can be challenging. Imaging tests such as CT scans are essential for accurate diagnosis. In adults, the BH can contain various organs, such as the spleen and kidney. Rarely, BH can be associated with an ectopic kidney located inside the chest cavity. In some cases of BH, there is a risk of complications such as gastric volvulus, where the stomach twists on itself, leading to potentially serious symptoms such as severe abdominal pain and vomiting.
    CONCLUSIONS: This case underscores the severe risks of BH in adults, such as gastric twisting and blockage, necessitating urgent surgery. Timely diagnosis and surgical intervention are crucial for preventing life-threatening outcomes. More research is needed to improve the management of this rare condition.
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