Morgagni hernia with respiratory failure aggravated by noninvasive positive pressure ventilation: A case report and overview of the literature

An elderly woman diagnosed with multiple myeloma (MM) in 2007 had improved with chemotherapy. She had severe kyphosis and a diaphragmatic hernia (DH), but no respiratory symptoms. In 2011, because of thoracic deformity and emaciation, we advised her to continue the previously prescribed domiciliary noninvasive positive pressure ventilation (NPPV) therapy for chronic type II respiratory failure. However, she refused to continue NPPV. She was later admitted for deterioration in respiratory status and carbon dioxide (CO₂) narcosis. We believed her low adherence to domiciliary NPPV caused CO₂ narcosis; hence, we advised her to continue domiciliary NPPV and she complied. In May 2012, the now 79-year-old patient was admitted for acute exacerbation of chronic respiratory failure and CO₂ narcosis. Chest imaging suggested that DH had caused a deterioration of her status. She underwent laparoscopic diaphragmatic hernia repair. Operative findings revealed a retrosternal hernia sac, and she was diagnosed as having a Morgagni hernia (MH). Her respiratory status subsequently improved. We hypothesize that NPPV increased intra-abdominal pressure, thereby worsening the MH and exacerbating respiratory failure. We believe that clinicians should be cautious when prescribing NPPV for MH patients.     

Variable role of patent ductus arteriosus

Although patent ductus arteriosus is essential in fetal life, interventions to close or minimize the adverse hemodynamic effects associated with the left-to-right shunt are often needed after birth, especially in extremely premature infants. However, there are clinical conditions where maintaining patency of the ductus is essential for survival. In this article we discuss use of prostaglandin E₁ in the management of congenital heart defects, pulmonary hypertension and left ventricular failure in early neonatal period.     

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.     

Long-term follow-up of congenital diaphragmatic hernia

Increased survival of patients with congenital diaphragmatic hernia has created a unique cohort of children, adolescent, and adult survivors with complex medical and surgical needs. Disease-specific morbidities offer the opportunity for multiple disciplines to unite together to provide long-term comprehensive follow-up, as well as an opportunity for research regarding late outcomes. These children can exhibit impaired pulmonary function, altered neurodevelopmental outcomes, nutritional insufficiency, musculoskeletal changes, and specialized surgical needs that benefit from regular monitoring and intervention, particularly in patients with increased disease severity. Below we aim to characterize the specific challenges that these survivors face as well as present an algorithm for a multidisciplinary long-term follow-up program.     

Congenital diaphragmatic hernia: The role of multi-institutional collaboration and patient registries in supporting best practice

Among congenital malformations, congenital diaphragmatic hernia (CDH) is distinguished by its relatively low occurrence rate, need for resource intensive, integrated multidisciplinary care, and widespread variation in practice and outcome. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence, they are poorly suited to the study of a condition like CDH due to challenges in illness severity adjustment, unpredictability in clinical course and the impact limitations of studying a single intervention at a time. An alternative to RCTs for comparative effectiveness research for CDH is the patient registry, which aggregates multi-institutional condition-specific patient level data into a large CDH-specific database for the dual purposes of collaborative research and quality improvement across participating sites. This article discusses patient registries from the perspective of structure, data collection and management, and privacy protection that guide the use of registry data to support collaborative, multidisciplinary research. Two CDH-specific registries are described as illustrative examples of the “value proposition” of registries in improving the evidence basis for best practices for CDH.     

ECMO in CDH: Is there a role?

Despite wide use and decades of experience, survival of congenital diaphragmatic hernia (CDH) patients treated with extra-corporeal membrane oxygenation (ECMO), as reported by the extra-corporeal life support organization (ELSO), remains unchanged at 50%. High-survival rates both with and without utilizing ECMO have been reported, fueling questions about the utility of ECMO support in this difficult population. This review looks at data from the Congenital Diaphragmatic Hernia Study Group and individual center reports, to evaluate the role of ECMO in CDH, focusing on defining the patients most likely to benefit, and discussing how those benefits can best be achieved. These data show that ECMO improves survival in those CDH patients who are most severely affected, but potential complications of ECMO delivery outweigh benefit in patients with less severely affected. Improved results can be expected by minimizing ECMO complications, and by improving rates of CDH repair in patients that require ECMO.     

肺癌骨转移所致腰腿麻痛的误诊原因(附1例报告并文献复习)

目的分析肺癌骨转移引起腰腿痛症状导致误诊的原因及教训。方法对1例误诊为腰椎间盘突出症、腰臀部慢性软组织损害的肺癌骨转移致腰腿痛患者的诊疗过程结合文献进行综合分析。结果肺癌骨转移所致腰腿麻痛症状是因受累椎体发生溶骨性破坏或楔形变,对应椎管位置狭窄,肿瘤侵犯骨皮质或软组织肿块压迫刺激脊髓和神经根而引起,本病误诊的主要原因是病史询问不详,查体简单、粗疏,未做针对性强的影像学检查。结论仔细询问病史,认真查体,与早期行针对性强的影像学检查相结合,可有效降低误诊率。     

腹腔镜手术治疗老年腹股沟疝2771例分析

目的 分析老年腹股沟疝病人的临床特点,探讨腹腔镜治疗的合理性、安全性和有效性。方法 回顾性分析2001年1月至2016年12月在上海交通大学医学院附属瑞金医院接受腹腔镜腹股沟疝修补术（LIHR）的5050例病人（6047侧）的临床资料。其中＜65岁组2279例（2622侧）,65～80岁组2332例（2883侧）,＞80岁组439例（542侧）。结果 老年病人（65~80岁组和>80岁组,下同）的男/女比、手术史、双侧疝、直疝、复合疝、Ⅲ型疝、Ⅳ型疝（复发疝）比例高于中青年病人（<65岁组,下同）（P=0.000）。＞80岁组的经腹腔腹膜前疝修补术（TAPP）比例最高,65～80岁组的TEP比例最高。重量型补片（HWM）的使用和机械性固定的比例在＞80岁组最高,＜65岁组最低,三组差异有统计学意义（P=0.000）。随访时间18～60个月（中位时间36个月）。三组在单侧手术时间、术后第1天疼痛视觉模拟评分（VAS）评分、术后2周和4周恢复非限制性活动病例数等方面差异无统计学意义（P>0.05）。老年病人双侧手术时间长于中青年病人（P=0.001）,三组病人术后住院时间比较,＞80岁组最长,＜65岁组最短,差异有统计学意义（P=0.000）。共有13例复发（0.021%）病例,老年病人的复发率高于中青年病人（P=0.006）。并发症依次为血清肿、尿潴留、暂时性神经感觉异常和肠麻痹,其中,病人的尿潴留发生率＞80岁组最高,＜65岁组最低,三组差异有统计学意义（P=0.000）。结论 对于术前评估合格的老年腹股沟疝病人,LIHR是安全、有效、合理的术式。     

疝环充填式无张力疝修补术治疗

目的总结疝环充填式无张力疝修补术的手术经验和临床效果. 方法采用美国巴德公司的疝环充填物及网状补片施行无张力修补术52例 ,观察手术时间、切口疼痛、术后患者自主能力的恢复、并发症、复发率及术后住院时间.结果平均手术时间35min,切口疼痛轻,术后5～8h就能下床活动, 并发尿潴留4例,阴囊水肿2例,切口积液1例,术后平均住院时间4d,随访2～12个月无1例复发.结论疝环充填式无张力疝修补术具有操作方便,术后恢复快,并发症少,复发率低的优点,是一种符合人体解剖结构的理想的疝修补术.     

感染因素对无张力疝修补术疗效影响的研究

目的 探讨无张力疝修补术是否适用于嵌顿性（伴有全腹膜炎）腹股沟斜疝.方法 根据疝嵌顿后腹膜炎体征情况,统一手术切口、术式及抗生素使用剂量应用天数,对75例腹股沟嵌顿性斜疝患者与同期81例择期的腹股沟疝患者分为两组对比.采用巴徳mesh prefix plug定型产品做无张力疝修补术.比较两组患者手术时间、术后并发症、切口感染率、复发率.结果 与对照组（n=81）相比,研究组（n=75）手术时间明显延长差异有统计学意义（t检验P=4.96＜0.01 ）.术后并发症、切口感染率、复发率差异无统计学意义（χ^20.55,P=0.46;χ^22.73,P=0.09;χ^20.02,P=0.888）.结论 无张力疝修补术同样适用于伴有全腹膜炎的嵌顿性腹股沟斜疝.