Weaning failure due to isolated residual diaphragmatic paralysis after cervical spinal cord ischemia following aortic surgery- a case report.

BACKGROUND: Bilateral diaphragmatic dysfunction can lead to dyspnea and recurrent respiratory failure. In rare cases, it may result from high cervical spinal cord ischemia (SCI) due to anterior spinal artery syndrome (ASAS). We present a case of a patient experiencing persistent isolated diaphragmatic paralysis after SCI at level C3/C4 following thoracic endovascular aortic repair (TEVAR) for Kommerell's diverticulum. This is, to our knowledge, the first documented instance of a patient fully recovering from tetraplegia due to SCI while still exhibiting ongoing bilateral diaphragmatic paralysis. CASE PRESENTATION: The patient, a 67-year-old male, presented to the Vascular Surgery Department for surgical treatment of symptomatic Kommerell's diverticulum in an aberrant right subclavian artery. After successful surgery in two stages, the patient presented with respiratory insufficiency and flaccid tetraparesis consistent with anterior spinal artery syndrome with maintained sensibility of all extremities. A computerized tomography scan (CT) revealed a high-grade origin stenosis of the left vertebral artery, which was treated by angioplasty and balloon-expandable stenting. Consecutively, the tetraparesis immediately resolved, but weaning remained unsuccessful requiring tracheostomy. Abdominal ultrasound revealed a residual bilateral diaphragmatic paralysis. A repeated magnetic resonance imaging (MRI) 14 days after vertebral artery angioplasty confirmed SCI at level C3/C4. The patient was transferred to a pulmonary clinic with weaning center for further recovery. CONCLUSIONS: This novel case highlights the need to consider diaphragmatic paralysis due to SCI as a cause of respiratory failure in patients following aortic surgery. Diaphragmatic paralysis may remain as an isolated residual in these patients.

Congenital diaphragmatic hernia: relationship between defect size and outcome. Experience in a reference centre.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) remains a therapeutic challenge. The surgical classification recommended by the Congenital Diaphragmatic Hernia study group (CDHSG), based on the size of the defect, is used for staging in reference centres. Larger defects are associated with poorer outcomes. Our aim was to describe and compare the morbidity at hospital discharge of newborns who underwent surgical correction of CDH at the Juan P. Garrahan, according to the surgical staging of the defect proposed by the CDHSG. MATERIAL AND METHODS: The study included patients with CDH admitted to the Juan P. Garrahan Hospital between 2012 and 2020, and we analysed the distribution, morbidity and mortality associated with the size of the defect. We carried out a descriptive analysis, calculating measures of central tendency and dispersion, and bivariate and multivariate analyses. RESULTS: A total of 230 patients with CDH were admitted and 158 underwent surgery. We found that defect sizes C and D sizes were associated with an increased risk of chronic pulmonary disease (CPD) (OR, 5.3; 95% CI, 2.2-13.4; P<.0000), need of extracorporeal membrane oxygenation (OR 3.9; 95% CI, 1.3-12.8; P<.005) and chylothorax (OR, 2.1; 95% CI, 0.8-6.4; P<.10]. The multivariate analysis revealed that a large defect size (C-D) was independently and significantly associated with CPD (OR 4.19; 95% CI, 1.76-9.95). CONCLUSION: Staging the defect according to de CDHSG classification during surgery allows the application of uniform management criteria and the prediction of patient outcomes and complications during the hospital stay.

Diaphragmatic and pericardial reconstruction using a Gore-Tex® patch in a patient with an invasive liver malignancy.

The Gore-Tex® polytetrafluoroethylene patch is one of the most used prostheses for diaphragm, vessel, and pericardial reconstruction. It is strong, flexible, and relatively inexpensive and can be fitted to match the size of the resected area. In addition, it can be used to reconstruct the pericardium and diaphragm following resection to treat diffuse malignant pleural mesothelioma or repair large hiatal hernias. However, the use of polytetrafluoroethylene for hepatocellular carcinoma with diaphragmatic and pericardial invasion has not yet been reported. We report the case of a 72-year-old man with hepatocellular carcinoma with diaphragmatic and pericardial invasion. Subsequently, laparotomic liver subsegmentectomy of segment 3 and resection of the diaphragm and pericardium were performed. The defects were successfully reconstructed using the polytetrafluoroethylene patch, without postoperative complications. This is the first report describing a case of invasive liver malignancy that required simultaneous diaphragmatic and pericardial reconstruction using a polytetrafluoroethylene patch, indicating that the polytetrafluoroethylene patch could effectively and directly treat invasive liver malignancies.

Approaches to nutrition and feeding in congenital diaphragmatic hernia.

In patients with congenital diaphragmatic hernia1, nutrition can represent a challenge both in the short and long term. Its failure to resolve can have a significant impact on multiple aspects of the lives of patients with congenital diaphragmatic hernia (CDH), ranging from lung function to neurodevelopment. In this review, we will describe the causes of nutritional problems in patients with CDH, their consequences, and possible strategies to address them.

Parents-reported nutrition and feeding difficulties in infants with congenital diaphragmatic hernia after hospital discharge.

PURPOSE: Congenital diaphragmatic hernia (CDH) affects 1 in 3000-5000 newborns. In survivors, long-term complications include gastroesophageal reflux (GER), feeding difficulties, and failure to thrive. Data from the parents' perspective remain scarce. This study aims to report the prevalence and impact of feeding difficulties on CDH families after discharge. METHODS: National web-based survey amongst families with CDH infants in 2021. RESULTS: Caregivers of 112 CDH survivors participated. The baseline characteristics were representative with 54 % male, 83 % left-sided CDH, prenatal diagnosis in 83 %, and 34 % requiring extracorporeal membrane oxygenation. Most infants (81 %) were discharged within three months, with 62 % feeding by mouth, and 30 % requiring a feeding tube. Persisting feeding difficulties were experienced by 73 %, GER being the most common (66 %), followed by insufficient weight gain (64 %). After discharge, 41 % received medical support for failure to thrive. The primary-care pediatrician was consulted most frequently for information (61 %) and treatment of feeding difficulties (74 %). Therapeutic success was reported in 64 %. A cessation of symptoms was achieved in 89 % within three years. CONCLUSION: The majority of CDH infants had persistent feeding difficulties. This survey highlights the impact surrounding feeding problems on CDH families. Further studies and support systems are needed to raise the quality of life in CDH infants and their families.

Comprehensive meta-analysis of surgical procedure for congenital diaphragmatic hernia: thoracoscopic versus open repair.

PURPOSE: 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.

Bochdalek's hernia in adults: A unique case with gastric volvulus and intrathoracic kidney.

INTRODUCTION AND IMPORATNCE: 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. CASE OF PRESENTATION: 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. DISCUSSION: 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. CONCLUSION: 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.

Long-term follow-up in congenital diaphragmatic hernia.

With improvements in initial care for patients with congenital diaphragmatic hernia (CDH), the number of CDH patients with severe disease who are surviving to discharge has increased. This growing population of patients faces a unique set of long-term challenges, multisystem adverse outcomes, and post-intervention complications requiring specialized multidisciplinary follow-up. Early identification and intervention are essential to mitigate the potential morbidity associated with these challenges. This manuscript outlines a general framework for long-term follow-up for the CDH patient, including cardiopulmonary, gastrointestinal, neurodevelopmental, surgical, and quality of life outcomes.

Diaphragmatic Hernia With Secondary Pneumothorax Due to Intestinal Perforation After Thoracoabdominal Aortic Aneurysm Operation: A Case Report.

A male in his 70s with a history of artificial vessel replacement for a thoracoabdominal aneurysm had been treated non-operatively for adhesive bowel obstruction during the past two months. The initial symptom was nausea and the patient was transferred to our hospital because of diffuse abdominal pain. Computed tomography revealed pneumothorax, diaphragmatic hernia, and bowel perforation. A left thoracic drain was inserted and air and clear yellow fluid were drained. Secondary pneumothorax was presumably caused by intestinal perforation associated with diaphragmatic hernia. Although reported cases with secondary pneumothorax associated with diaphragmatic hernia and intestinal perforation are caused by trauma, this complication can occur postoperatively.

Diaphragmatic eventration associated with megacolon: A case report.

INTRODUCTION: Diaphragmatic eventration (DE) associated with gastrointestinal anomalies is rarely reported, and its treatment is not well defined. CASE PRESENTATION: We present a 24-years-old male admitted for cough, dyspnea, and chest pain, all of gradual onset. Chest computerized tomography (CT) and chest x-ray diagnosed diaphragmatic eventration and colonic distension. He underwent surgery via a posterolateral thoracotomy. We found colonic distension which pushed the left hemidiaphragm under intense pressure further into the chest, making immediate diaphragmatic plication via the chest difficult. We managed this by colonic needle decompression followed by resection and plication of the diaphragm. The patient had a satisfactory postoperative recovery with a good respiratory profile and no constipation. At a follow up visit, barium enema showed megacolon with no apparent etiology. He is to have a long-term outpatient follow up. DISCUSSION: Diaphragmatic eventration coexisting with megacolon is rare. Data available suggests supplementary abdominal workup is necessary to better plan the surgery. Surgical correction via an abdominal route tends to be more appropriate in these scenarios. CONCLUSION: In cases of gastrointestinal anomaly associated with diaphragmatic eventration, more careful abdominal consideration is needed to better plan the surgery.

Robotic-assisted repair of an anterior diaphragmatic hernia secondary to Xiphoidectomy.

Anterior diaphragmatic hernias manifest when a diaphragmatic defect permits abdominal contents to enter the thoracic cavity. They may be congenital or acquired; when acquired, the typical etiology is traumatic injury. Without treatment, they risk incarceration or strangulation. A 55-year-old male with a history of xiphoidectomy during sternotomy for cardiac disease was incidentally found to have an anterior diaphragmatic hernia on a screening chest CT (computed tomography) scan. He developed gastric obstruction shortly after an outpatient surgical consultation. He was admitted to the hospital, and further workup revealed a right-sided type-4 diaphragmatic hernia with an incarcerated colon, antrum, and pylorus. Nasogastric decompression was performed, followed by robotic-assisted transabdominal preperitoneal (r-TAPP) repair with mesh. He recovered without complications. There are currently no reports in the literature of an anterior diaphragmatic hernia secondary to a xiphoidectomy. This case demonstrates the successful use of r-TAPP for this rare presentation of an anterior diaphragmatic hernia.

Exercise Attenuate Diaphragm Atrophy in COPD Mice via Inhibiting the RhoA/ROCK Signaling.

Background: Exercise is an indispensable component of pulmonary rehabilitation with strong anti-inflammatory effects. However, the mechanisms by which exercise prevents diaphragmatic atrophy in COPD (chronic obstructive pulmonary disease) remain unclear. Methods: Forty male C57BL/6 mice were assigned to the control (n=16) and smoke (n=24) groups. Mice in the smoke group were exposed to the cigarette smoke (CS) for six months. They were then divided into model and exercise training groups for 2 months. Histological changes were observed in lung and diaphragms. Subsequently, agonist U46639 and antagonist Y27632 of RhoA/ROCK were subjected to mechanical stretching in LPS-treated C2C12 myoblasts. The expression levels of Atrogin-1, MuRF-1, MyoD, Myf5, IL-1ß, TNF-α, and RhoA/ROCK were determined by Western blotting. Results: Diaphragmatic atrophy and increased RhoA/ROCK expression were observed in COPD mice. Exercise training attenuated diaphragmatic atrophy, decreased the expression of MuRF-1, and increased MyoD expression in COPD diaphragms. Exercise also affects the upregulation of RhoA/ROCK and inflammation-related proteins. In in vitro experiments with C2C12 myoblasts, LPS remarkably increased the level of inflammation and protein degradation, whereas Y27632 or combined with mechanical stretching prevented this phenomenon considerably. Conclusion: RhoA/ROCK plays an important role in the prevention of diaphragmatic atrophy in COPD.

Racial Inequalities in the Initial Management of Congenital Diaphragmatic Hernia in the Houston Area.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is readily prenatally diagnosed and associated with significant perinatal morbidity and mortality. Delivery at facilities with adequate resources for neonatal resuscitation, such as Children's Surgery Verification (CSV) centers, is recommended; however, disparities have been clinically noted. We aimed to characterize locoregional care of CDH and the impact of race and ethnicity. METHODS: We conducted a population-based study using the Texas Inpatient Public Use Data File to identify infants <1 y-old with CDH based on international classification of diseases 9/10 codes (2013-2021). Only initial birth admissions in the Houston region were included. Data was analyzed using descriptive statistics and chi-squared analysis. RESULTS: We identified 257 newborns with CDH. While births were noted across 29 facilities, the majority were at the 2 CSV centers in Houston. There was no significant difference in illness severity, prematurity or insurance status by race. Black and 'other' patients were less likely to deliver at CSV facilities (Black 32% versus 'Other' 48% versus White 70% versus Asian 81%; P < 0.01), receive ECMO (Black 6% versus 'Other' 5% versus White 19% versus Asian 29%; P < 0.01) or undergo a CDH repair (Black 26% versus 'Other' 33% versus White 51% versus Asian 71%; P < 0.01) on their index admission and had lower average daily costs (Black $10,292 [$3219-25,021] versus 'Other' $9106 [$3617-15,672] versus White $12,906 [$9038-18,550] versus Asian $12,896 [$7469-23,817]; P < 0.05). Additionally, black and 'other' patients were more likely to be transferred (Black 23% versus 'Other' 28% versus White 12% versus Asian 14%; P < 0.05). None of the patients born at CSV centers transferred. CONCLUSIONS: Most Houston-born patients deliver at high-resource centers; however, Black and 'other' patients are less likely to deliver at CSV centers and more likely to require transfer during the critical neonatal period. This suggests a vulnerable population which may benefit from targeted intervention to improve prenatal care and delivery planning.

Ventilator strategies in congenital diaphragmatic hernia.

This review focuses on contemporary mechanical ventilator practices used in the initial management of neonates born with congenital diaphragmatic hernia (CDH). Both conventional and non-conventional ventilation modes in CDH are reviewed. Special emphasis is placed on the rationale for gentle ventilation and the current evidence-based clinical practice guidelines that are recommended for supporting these fragile infants. The interplay between CDH lung hypoplasia and other key cardiopulmonary elements of the disease, namely a reduced pulmonary vascular bed, abnormal pulmonary vascular remodeling, and left ventricular hypoplasia, are discussed. Finally, we provide insights into future avenues for mechanical ventilator research in CDH.

Surgical approaches to congenital diaphragmatic hernia.

Surgical repair of the diaphragm is essential for survival in congenital diaphragmatic hernia (CDH). There are many considerations surrounding the operation - why the operation matters, optimal timing of repair and its relation to extracorporeal life support (ECLS) use, minimally invasive versus open approaches, and strategies for reconstruction. Surgery is both affected by, and affects, the physiology of these infants and is an important factor in determining long-term outcomes. Here we discuss the evidence and provide insight surrounding this complex decision making, technical pearls, and outcomes in repair of CDH.

Exploring ultrasonographic diaphragmatic function in perioperative anesthesia setting: A comprehensive narrative review.

The ultrasound study of diaphragm function represents a valid method that has been extensively studied in recent decades in various fields, especially in intensive care, emergency, and pulmonology settings. Diaphragmatic function is pivotal in these contexts due to its crucial role in respiratory mechanics, ventilation support strategies, and overall patient respiratory outcomes. Dysfunction or weakness of the diaphragm can lead to respiratory failure, ventilatory insufficiency, and prolonged mechanical ventilation, making its assessment essential for patient management and prognosis in critical care and emergency medicine. While several studies have focused on diaphragmatic functionality in the context of intensive care, there has been limited attention within the field of anesthesia. The ultrasound aids in assessing diaphragmatic dysfunction (DD) by measuring muscle mass and contractility and their potential variations over time. Recent advancements in ultrasound imaging allow clinicians to evaluate diaphragm function and monitor it during mechanical ventilation more easily. In the context of anesthesia, early studies have shed light on the patho-physiological mechanisms of diaphragm function during general anesthesia. In contrast, more recent research has centered on evaluating diaphragmatic functionality at various phases of general anesthesia and by comparing diverse types of procedures or anatomical position during surgery. The objectives of this current review are to highlight the use of diaphragm ultrasound for the evaluation of diaphragmatic function during perioperative anesthesia and surgery. Specifically, we aim to examine the effects of anesthetic agents, surgical techniques, and anatomical positioning on diaphragmatic function. We explore how ultrasound aids in assessing DD by measuring muscle mass and contractility, as well as their potential variations over time. Additionally, we will discuss recent advancements in ultrasound imaging that allow clinicians to evaluate diaphragm function and monitor it during mechanical ventilation more easily.

Cellular origins and translational approaches to congenital diaphragmatic hernia.

Congenital Diaphragmatic Hernia (CDH) is a complex developmental abnormality characterized by abnormal lung development, a diaphragmatic defect and cardiac dysfunction. Despite significant advances in management of CDH, mortality and morbidity continue to be driven by pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. The etiology of CDH remains unknown, but CDH is presumed to be caused by a combination of genetic susceptibility and external/environmental factors. Current research employs multi-omics technologies to investigate the molecular profile and pathways inherent to CDH. The aim is to discover the underlying pathogenesis, new biomarkers and ultimately novel therapeutic targets. Stem cells and their cargo, non-coding RNAs and agents targeting inflammation and vascular remodeling have produced promising results in preclinical studies using animal models of CDH. Shortcomings in current therapies combined with an improved understanding of the pathogenesis in CDH have given rise to novel promising experimental treatments that are currently being evaluated in clinical trials. This review provides insight into current developments in translational research, ranging from the cellular origins of abnormal cardiopulmonary development in CDH and the identification of novel treatment targets in preclinical CDH models at the bench and their translation to clinical trials at the bedside.

Extracorporeal Membrane Oxygenation Before 34 Wks' Gestation: A Single-Center Experience.

INTRODUCTION: Traditionally, gestational age <34 wk and weight <2 kg are considered relative contraindications to extracorporeal membrane oxygenation (ECMO). There is a paucity of information that explains the outcomes in this unique population of premature neonates. The purpose of this study is to examine outcomes of patients who undergo ECMO at <34 wk at a single institution. METHODS: A single-center retrospective review was performed for neonates managed with ECMO in the neonatal intensive care unit from January 2012 to April 2022. Characteristics and outcome data were collected. The primary outcome studied was survival at discharge. Secondary outcomes were intraventricular hemorrhage, ischemic brain injury, and thrombosis. Data were analyzed with descriptive statistics. RESULTS: Following exclusion, 107 patients were included with eight having initiating ECMO at <34 wk. Three (38%) patients, who received ECMO at <34 wk, incurred intraventricular hemorrhages compared to 14 (14%) in the ≥34-wk cohort. Two (25%), who underwent ECMO at <34 wk, exhibited signs of brain ischemia on imaging compared to 9 (9%) in those ≥34 wk, and 3 (38%) patients <34 wk experienced thrombosis compared to 31 (31%) in the ≥34-wk cohort. Five (63%) of those in the <34-wk cohort survived to discharge, similar to 61 (61%) in the ≥34 wk cohort. CONCLUSIONS: Our data suggest that EGA <34 wk may not be a contraindication for ECMO, with appropriate counseling of potential risks.

Prevalence of Gastroesophageal Reflux Disease in Congenital Diaphragmatic Hernia Survivors From Infancy to Adulthood.

BACKGROUND: Gastroesophageal reflux disease (GERD) is a common comorbidity associated with congenital diaphragmatic hernia (CDH), with reported cases of Barrett's esophagus (BE) and esophageal adenocarcinoma before the age of 25. The prevalence and natural course of GERD in CDH survivors remain uncertain due to variations in diagnostic methods. We aimed to analyse the GERD prevalence from infancy through young adulthood. METHODS: We retrospectively analyzed pH-impedance measurements and endoscopic findings in 96 CDH survivors evaluated as routine care using well established clinical protocols. GERD was defined as an abnormal acid exposure time for pH-MII measurements and as presence of reflux esophagitis or BE at upper endoscopy. Clinical data including symptoms at time of follow-up and use of antireflux medication were collected. RESULTS: GERD prevalence remained consistently low (≤10%) across all age groups, yet many patients experienced GER symptoms. Histological abnormalities were observed in 80% of adolescents and young adults, including microscopic esophagitis in 50%. BE was diagnosed in 7% before the age of 18, all had GER symptoms. CDH severity, anatomy at the time of CDH correction, alcohol usage, and smoking did not emerge as significant risk factors for GERD. CONCLUSIONS: Given the low GERD prevalence in CDH survivors, a symptom-driven approach to diagnosis and follow-up is warranted. We advise long-term follow-up for all adult patients due to the early onset of BE and the limited evidence available. The longitudinal course and impact of GERD on other long-term CDH-related comorbidities should be explored in larger cohorts. LEVEL OF EVIDENCE: Not applicable.