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Presentation of congenital diaphragmatic hernia in adulthood is rare. Further, most cases are attributable to Bochdalek and Morgagni hernias with central tendon hernias being particularly uncommon. We report a case of central congenital diaphragmatic hernia in an adult presenting as large intestinal obstruction and respiratory failure. Open repair of the hernia was performed, which brought about the resolution of the patient's symptoms. This case report highlights the possibility for congenital diaphragmatic hernia to present in adulthood and the importance of early diagnosis and treatment in these situations., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Declaration of competing interest The authors have no conflicts of interest concerning this article.

Aim: Acquired post-transplant diaphragmatic hernia (PTDH) is a rare complication of liver transplantation (LT) in children. We aimed to present our experience in PTDH, and a possible causative background is discussed., Methods: Medical records of patients who had undergone diaphragmatic repair following LT between 2015 and 2023 were reviewed. Demographic information, details of primary diseases necessitating LT, transplantation techniques, and clinical findings associated with PTDH were evaluated., Results: There were seven patients with PTDH. Median age at transplantation was 69 (range: 9-200) months. Five patients received a left lateral sector, one patient had a right lobe, and one had a left lobe graft. Time between LT and PTDH was 9 (2-123) months. One patient who was diagnosed in the postoperative 10th year was asymptomatic. Respiratory distress and abdominal pain were the main symptoms among all. All patients underwent laparotomy, and primary repair was performed in six patients, and one patient required mesh repair because of a large defect. Small intestine herniated in most cases. There were two complicated cases with perforation of the stomach and colonic volvulus. There is no recurrence or long-term complications for the median 60 (20-119) month follow-up period., Conclusion: PTDH is a rare but serious complication. Majority of symptomatic cases present within the first postoperative year, whereas some late-presenting cases may not be symptomatic. Inadvertent injury to the inferior phrenic vasculatures due to excessive use of cauterization for control of hemostasis may be a plausible explanation in those cases., (© 2024 Wiley Periodicals LLC.)

Despite advances in caring for neonates with congenital diaphragmatic hernia (CDH), mortality and morbidity continues to be high. Additionally, the pathophysiology of cardiac dysfunction in this condition is poorly understood. Postnatal cardiac dysfunction in neonates with CDH may be multifactorial with origins in fetal life. Mechanical obstruction, competition from herniated abdominal organs into thoracic cavity combined with redirection of ductus venosus flow away from patent foramen ovale leading to smaller left-sided structures may be a contributing factor. This shunting decreases left atrial and left ventricular blood volume, which may result in altered micro- and macrovascular aberrations affecting cardiac development in the prenatal period. Direct mass effect from herniated intra-abdominal contents restricting cardiac growth and/or reduced left ventricular preload may contribute independently to left ventricular dysfunction in the absence of right ventricular dysfunction and or pulmonary hypertension. With variable clinical phenotypes of cardiac dysfunction, pulmonary hypertension, and respiratory failure in patients with CDH, there is increased need for individualized diagnosis and tailored therapy. Routine use of therapy such as inhaled nitric oxide and sildenafil that induces significant pulmonary vasodilation may be detrimental in left ventricle dysfunction, whereas in a patient with pure right ventricle dysfunction, they may be beneficial. Targeted functional echocardiography serves as a real-time tool for defining the pathophysiology and aids optimization of vasoactive therapy in affected neonates. KEY POINTS: · Cardiac dysfunction in neonates with CDH is multifactorial.. · Postnatal cardiac dysfunction in patients with CDH has its origins in fetal life.. · Right ventricular dysfunction contributes to systemic hypotension.. · Left ventricular dysfunction contributes to systemic hypotension.. · Supportive therapy should be tailored to clinical phenotype.., Competing Interests: None declared., (Thieme. All rights reserved.)

Objective: The aim of this study was to evaluate the risk factors associated with xerostomia and hyposalivation in a group of hypertensive patients., Subjects and Methods: A cross-sectional study was conducted. Hypertensive patients belonged to two healthcare centers were included. Xerostomia was assessed by asking a question and using the Xerostomia Inventory. Unstimulated salivary flow was collected. Different epidemiological variables were analyzed such as age, sex, habits, diseases, drugs, and blood pressure., Results: 221 individuals were included. Xerostomia was reported in 51.13% of patients. Patients with xerostomia suffered more from osteoarthritis and diaphragmatic hernia. These patients took more anticoagulants (acenocoumarol), antiarrhythmics (amiodarone), analgesics (paracetamol) and epilepsy drugs (pregabalin) and less platelet aggregation inhibitors and angiotensin II receptor blockers (losartan). Unstimulated flow was reduced in 37.56% of patients. Patients suffering hyposalivation presented more diseases such as anxiety, infectious or parasitic diseases, hepatitis C, diaphragmatic hernia, and osteoarthritis. These patients took more repaglinide, thiazides, anti-inflammatories, anti-rheumatics, glucosamine, diazepam, and selective beta-2-adrenoreceptor agonists and less combinations of candesartan and diuretics., Conclusions: Xerostomia and hyposalivation are frequent in hypertensive patients. It is advisable to take into consideration the comorbidities and the drugs they receive, since they can increase the risk of these salivary disorders., (© 2021 Wiley Periodicals LLC.)

Competing Interests: Conflict of interest: None declared.

A diaphragmatic hernia is the protrusion of abdominal tissues into the thoracic cavity secondary to a defect in the diaphragm. Reviewing the literature, we found only 44 references to diaphragmatic hernia secondary to percutaneous radiofrequency treatment. The vast majority of these cases were secondary to the treatment of hepatocellular carcinoma in segments V and VIII. Nevertheless, to date, this is the first reported case of diaphragmatic hernia after radiofrequency ablation of a liver metastasis from colorectal cancer. Complications secondary to diaphragmatic hernias are very diverse. The principal risk factor for complications is the contents of the hernia; when small bowel or colon segments protrude in the thoracic cavity, they can become incarcerated. Asymptomatic cases have also been reported in which the diaphragmatic hernia was discovered during follow-up. The pathophysiological mechanism is not totally clear, but it is thought that these diaphragmatic hernias might be caused by locoregional thermal damage. Given that most communications correspond to asymptomatic and/or treated cases, it is likely that the incidence is underestimated. However, due to the advent of percutaneous treatments, this complication might be reported more often in the future. Most cases are treated with primary herniorrhaphy, done with a laparoscopic or open approach at the surgeon's discretion; no evidence supports the use of one approach over the other. Nevertheless, it seems clear that surgery is the only definitive treatment, as well as the treatment of choice if complications develop. However, in asymptomatic patients in whom a diaphragmatic hernia is discovered in follow-up imaging studies, management should probably be guided by the patient's overall condition, taking into account the potential risks of complications (contents, diameter of the opening into the thoracic cavity …)., (Copyright © 2021 SERAM. Published by Elsevier España, S.L.U. All rights reserved.)

Acquired diaphragmatic hernia (ADH) is a rare complication after liver surgery in adult and pediatric patients. In the literature, a few low case series have been reported. Its incidence is estimated to be between 0.74% and 2.9%. Main descriptions of ADH concern liver resection for tumors, living donor hepatic donation in adult patients, and partial liver graft transplant in children [1,2]. We encountered a rare case of ADH in the postoperative time of a liver transplant with thrombosis of hepatic artery due to median arcuate ligament syndrome (MALS). The patient was a 65-year-old woman diagnosed with symptomatic hepatorenal polycystic disease who underwent a liver transplant with an isogroup graft from a cardiac-dead donor. During the first postoperative day, the rutinary color Doppler ultrasonography showed absent artery hepatic flow, and angiography suggested thrombosis of the hepatic artery (HA). With these findings, exploratory laparotomy was done. We performed thrombectomy and liberation of the celiac artery from the median arcuate ligament by dividing its fibers. At discharge, the liver function was normal, and Doppler showed good blood flow in the HA. At fourth postoperative month, she presented in the urgency unit with upper abdominal pain and vomiting. Radiologic and endoscopic evaluation revealed an incarcerated diaphragmatic hernia and signs of gastric ischemia. After emergency laparotomy and evaluation of the left hemithorax, we performed hernial sac reduction with recovery of gastric hypoperfusion. The diaphragmatic hernia was repaired. Diaphragmatic hernia is a rarely reported complication of liver transplant and should be considered a potential late complication [1]., Competing Interests: DISCLOSURES The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this article., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: Radiofrequency ablation is an effective management modality for irresectable primary and secondary liver tumors. Some serious complications have been reported including diaphragmatic hernia. Diaphragmatic hernia is the protrusion of abdominal viscera into the thoracic cavity through a diaphragmatic defect and usually classified into congenital and acquired. After RFA, diaphragmatic hernia is a rarely-reported complication., Case Report: A 62-year-old male patient, known to have liver cirrhosis on top of hepatitis C virus, presented to the emergency department with generalized abdominal pain and vomiting four months after having a RFA procedure for a liver tumor in segment VIII. Computed tomography showed diaphragmatic hernia with strangulated terminal ileum in the chest. Emergency laparotomy was performed with resection of an ileal segment and creation of double barrel ileostomy. The patient was discharged in a good condition after tolerating oral intake., Conclusion: Radiofrequency ablation is an effective modality for management of the primary and secondary liver tumors. Despite its safety, some complication may happen owing to its thermal effect and the associated patients general condition. Many techniques have been described to decrease its thermal injury. Diaphragmatic hernia is a rare complication after RFA. Its clinical presentation may be confusing and it may occur as early as one month after RFA. Its diagnosis depends mainly on computed tomography. Emergency surgical management is the standard approach.

Background: Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor., Methods: This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT., Results: During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation., Conclusion: Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes., (© 2021 Wiley Periodicals LLC.)

Introduction: In 15% of patients with iron deficiency anemia, large diaphragmatic hernias are found as the cause of chronic iron loss. Conversely, iron deficiency anemia is present in 10-40% of diaphragmatic hernia patients. However, it is unclear why some patients with large diaphragmatic hernias develop anemia and others do not., Methods: We retrospectively analyzed 116 patients with diaphragmatic hernias larger than 5 cm for the presence of anemia and the effect of surgery on this anemia, dividing these patients into 4 groups (group A: 21 patients with anemia/surgery, group B: 27 patients without anemia but with surgery, group C: 34 patients with anemia but without surgery, and group D: 34 patients without anemia/surgery)., Results: Women significantly predominated in the patient population (76%). Patients with iron deficiency anemia tended to be significantly older than patients without iron deficiency anemia (74.7 ± 12.2 vs. 69.6 ± 14.8 years, p = 0.08). The proportion of patients taking ASA was significantly higher in the anemia collective (41.8% vs. 9.8%, p < 0.001). Regression analysis further confirmed that higher age and ASA intake correlated significantly with lower hemoglobin in anemic patients. Performing hernia repair significantly decreased anemia rates and PPI use in the anemia patients, while both remained almost the same in the non-operated anemia patients., Conclusion: ASA use and advanced age are risk factors for the presence of iron deficiency anemia in patients with large diaphragmatic hernias. Surgical hernia repair is suitable to reduce anemia., (© 2023 S. Karger AG, Basel.)

The diagnosis of diaphragmatic hernia (DH) in adults is rare and may be due to missed congenital DH or acquired DH from trauma or as a postoperative complication of certain thoracic and abdominal surgeries. We present a case of a patient with well-controlled chronic obstructive pulmonary disease who presented to the hospital with progressive dyspnoea, 6 months after laparoscopic nephrectomy. The patient was initially misdiagnosed and treated for empyema after plain radiographic images were reported as consolidation with gas locules. Multislice CT imaging undertaken before diagnostic thoracocentesis confirmed the presence of a right-sided DH, which was subsequently surgically repaired in the outpatient setting, given her haemodynamic stability. As patients with DH usually present in the emergency setting, requiring urgent inpatient surgical repair, there are currently no guidelines on the method and urgency of management of asymptomatic or mildly symptomatic, stable patients. Furthermore, while plain radiography is the usual first-line imaging modality used, misdiagnosis of DH as pleural effusion or empyema can lead to unnecessary and potentially harmful procedures such as diagnostic thoracocentesis. These risks can potentially be minimised with early utilisation of multislice CT imaging in patients with high clinical suspicion., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

A 78-year-old man with no surgical history or recent trauma presented to the emergency department with sudden onset right-sided chest pain and dyspnoea. He was admitted under the physicians for investigations and was subsequently diagnosed with empyema of the right thorax. After no improvement with intravenous antibiotics, a chest drain was inserted; no pus was drained. He worsened clinically; a repeated CT scan demonstrated an incarcerated loop of small bowel within the right thoracic cavity secondary to a diaphragmatic hernia (DH). The patient had emergency surgery to remove necrotic small bowel and to lavage the thorax. Strangulated DH should be considered as a differential diagnosis where presentation is unusual and empyema does not improve after initial management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Abnormal localization of the appendix in hernial sac occurs in 2-4% of cases. Appendix is often found in inguinal and femoral hernias. Localization of the appendix in diaphragmatic hernia is described as a casuistic case. Case report of successful surgical treatment of a patient with perforated appendicitis in the left diaphragmatic hernia is presented.

Background: Jarcho-Levin syndrome (JLS) is a rare congenital disorder characterized by different clinical and radiologic findings. The disease was first reported by Jarcho and Levin in 1938, and it was described as the presence of various malformations or abnormal fusion in the thoracic vertebrae and ribs, short trunk, and respiratory distress., Case Description: In our case, fusion at the thoracic and cervical vertebrae, butterfly vertebrae, and a crablike-shaped thorax was present. The patient had a short trunk, short stature, and long extremities. Moreover, he had a syndromic face and restrictive-type respiratory distress. There was a glomus tumor in the carotid space. In our literature review, we found that neural tube defects are frequently present in this syndrome. However, we could not identify any cases with affected neural crest cells., Conclusions: JLS may affect cells derived from the neural crest located between the neural tube and surface ectoderm. Therefore patients with JLS should be screened for other tumors located in this area., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Diaphragmatic herniations of the ureter are a rare occurrence overall, with fewer than 10 cases reported. Obstructing nephrolithiasis in the setting of diaphragmatic ureteral herniation has sparsely been documented. Management options to consider include ureteroscopy and laser lithotripsy to alleviate obstruction in herniated ureters. The abnormal path of the ureter and surrounding structures pose unique risks that require careful attention and a high level of operator skill. We present imaging of an 84-year-old woman incidentally found to have right ureteral herniation through the diaphragm with an obstructing 8 mm stone in the right proximal ureter with moderate hydronephrosis., (Copyright © 2018. Published by Elsevier Inc.)

Acquired diaphragmatic hernia is a rare complication of pediatric intervention or surgery. In this study, we report an infant with iatrogenic diaphragmatic hernia following neonatal complex congenital cardiac surgery, and then we review the associated literature.

Background: RIPPLY2-associated spondylocostal dysostosis is a rare disorder that leads to segmentation defects of the vertebrae. These vertebral defects can result in severe instability of the cervical spine, leading to cardiac arrest after only minor whiplash injury., Case Report: We present the case of a healthy 7-year-old child who experienced an out-of-hospital cardiac arrest. He was reported to have profound respiratory distress and collapsed after going down a slide, without trauma. He was resuscitated in the field, and presented to the emergency department, where return of spontaneous circulation was achieved. Imaging of his cervical spine revealed multiple abnormalities. It was determined that a whiplash injury led to hypoxia and bradycardia due to the anatomic abnormalities of his cervical spine, resulting in cardiovascular collapse. He recovered fully and was later diagnosed with SCDO6, an autosomal recessive inherited disorder caused by a mutation in the RIPPLY2 gene. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Unfamiliarity of providers with this mechanism of cardiac arrest, and the rarity of the syndrome itself, make early recognition very difficult. Late diagnosis and lack of preventative measures, including immediate cervical spine stabilization, can lead to catastrophic outcomes. In patients with cardiac arrest of unclear etiology, early consideration of cervical spine immobilization and evaluation can be lifesaving., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: ADH is a rare and potentially fatal complication following LT. In this study, a systematic review was completed to identify risk factors which may contribute to ADH., Methods: Transplant databases at three LT programs were reviewed. Four pediatric and zero adult cases were identified. Next, a systematic review was completed. Fourteen studies describing 41 patients with ADH were identified. Patient demographics, transplant characteristics, and features of ADH diagnosis were examined., Results: The majority (90.2%) of ADH were in children. In pediatric LT, 95.1% received a segmental allograft. ADH occurred in the right P diaphragm 92.7% of the time, and 87.8% were repaired primarily. Patient demographics, post-transplant complications, and immunosuppression regimens were broad and failed to predict ADH. Most patients presented with either respiratory or gastrointestinal symptoms. There were two pediatric deaths related to undiagnosed ADH. The combined worldwide incidence of ADH in pediatric LT is 1.5% (34/2319 patients)., Conclusion: ADH is a rare complication post-LT that primarily occurs in pediatric recipients. When diagnosed early, ADH can be repaired primarily with good outcomes., (© 2018 Wiley Periodicals, Inc.)

Purpose: Jarcho-Levin syndrome patients commonly suffer from repeated respiratory infections and become ventilator dependent due to an insufficient thoracic volume. Multiple congenital vertebral anomalies are associated with this genetic disorder and results in stunted spine growth. The purpose of this case report is to present the results of a hybrid vertical expandable prosthetic titanium rib (VEPTR) and magnetically controlled growing rod (MCGR) construct for the management of a patient with Jarcho-Levin syndrome., Methods: A boy with Jarcho-Levin syndrome undergoes a hybrid VEPTR-MCGR construct to treat his thoracic insufficiency syndrome and spinal deformity., Results: The patient could wean off ventilator and had reduced chest complications with the construct. He was also able to achieve some spine length gain with the distraction device. However, there were limitations in prolonged treatment as no spine height was gained once he reached 12 years old. Once no further growth can be achieved, the MCGR led to progressive kyphosis. Nevertheless, at latest follow-up after the removal of all implants, the patient could maintain satisfactory correction of both thoracic and spine deformities., Conclusions: A hybrid construct is necessary for managing Jarcho-Levin syndrome as the VEPTR deals with the thoracic cage deformity while the MCGR deals with the spine deformity.

Morgagni hernia (MH) is a very rare congenital defect found in the anterior aspect of the diaphragm between the costal and sternal portions of the muscle. The most common defect is congenital diaphragmatic hernia, 90% of which are Bochdalek type. MHs account for approximately 3% of all diaphragmatic hernias. Most MHs are found and repaired in children, but 5% are found in adults. Here, we present the case of an incarcerated and strangulated MH with synchronous prolapse of the liver and transverse colon in a 77-year-old man who was admitted to our hospital for abdominal pain and symptoms of intestinal obstruction.

A 71-yr-old male with a medical history significant for chronic obstructive pulmonary disease presented to emergency department for worsening cough, right-sided pleuritic chest pain, and dyspnea. This was the patient's third visit to the emergency department in 4 d. The patient was initially treated for chronic obstructive pulmonary disease exacerbation and subsequently evaluated for congestive heart failure. He was ultimately diagnosed with a spontaneous herniation of the lung parenchyma through the chest wall. The patient was transferred to an outside host nation facility for definitive treatment and repair of the chest wall defect. Refractory cough and dyspnea failing to respond to typical treatments should warrant an expanded differential to include the rare etiology of atraumatic spontaneous lung herniation.

Translocation of abdominal organs into the thoracic cavity may cause dyspnea, heart disorders, and gastric symptoms. Diaphragmatic hernias can cause diagnostic difficulties, since both clinical and radiological symptoms might imitate different disorders. In these cases computed tomography of the chest is the method of choice. The aim of this study was to assess clinical manifestations, risk factors, and prognosis in patients with huge diaphragmatic hernias with displacement of abdominal organs into the thorax, depending on the action taken. We carried out a retrospective study using data of patients hospitalized in the years 2012-2016. Ten patients were qualified for the study (8 women and 2 men). The mean age of the subjects was 86.5 ± 10.5 years. Thirty percent of the hernias were post-traumatic. All of the patients reported cardiovascular or respiratory symptoms. Upper gastrointestinal symptoms occurred in half of the patients. Twenty percent of patients underwent surgery with a positive outcome, while 30% of patients, who were not qualified for surgery due to numerous co-morbidities, died. The main risk factors predisposing to the occurrence of large diaphragmatic hernias were the following: old age, female gender, and thoracic cage deformities.

A 70-year-old female patient presented with acute severe respiratory distress at a district general hospital. Medical history included type 2 diabetes, recurrent pulmonary embolisms and pre-existing diaphragmatic hernia containing part of the liver. Despite initial treatment with steroid inhalers, her clinical picture rapidly deteriorated requiring emergency intubation and positive pressure ventilation. Imaging investigations revealed tension enterothorax and hepatothorax with tracheal deviation. The patient was transferred and underwent an emergency laparotomy at the Regional Oesophagogastric Unit. A large diaphragmatic hernia (central tendon defect) which contained the duodenum, porta hepatis, right lobe of liver, gallbladder and right colon was reduced and successfully repaired. Her postoperative course was uneventful with no signs of recurrence at 2 months follow-up.This case describes an extremely rare and life-threatening condition of tension enterothorax and hepatothorax, which should be considered in the differential diagnosis of acute respiratory distress with tracheal deviation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Ureteral herniations are a rare occurrence, generally found incidentally on cross sectional imaging or during surgical intervention for unrelated processes. Several locations of ureteral herniations can occur including the inguinal, femoral, sciatic, obturator, and thoracic regions. While few reports of ureteral hernias are reported in the literature overall, the vast majority of those reported are inguinoscrotal herniations found during evaluation and treatment of inguinal hernias. Pelvic outlet ureteral herniations intrinsically are more common secondary to their dependent locations. Intrathoracic ureteral herniations through diaphragmatic defects are an exceptionally rare subset of ureteral herniations and have only been described sparingly. Fewer than ten case reports of diaphramatic ureteral herniations have been reported and none have described both cystoscopic management and open reconstruction., Case Presentation: We report the case of a 81 year old female with flank pain who was found to have idiopathic diaphragmatic hernia with incarcerated proximal ureter. She had no prior injury or surgery that explained her clinical presentation. She was initially observed and then managed conservatively with ureteral stent exchanges. Ultimately she underwent open surgical repair of her diaphragmatic hernia, reduction, resection and anastomosis of redundant proximal incarcerated ureteral segment, and nephropexy for a hypermobile right renal unit. This case report illustrates the pre- and post-operative imaging studies of a very rare intrathoracic ureteral herniation as well as surgical approach to repair., Conclusion: A herniated ureter is a potential source of serious renal and ureteral complications. The thoracic herniation of ureter is the rarest of the ureteral herniations. When discovered, they should be managed to preserve renal function and prevent strangulation of the affected segment of ureter. This case report documents the treatment of a thoracic ureteral herniation with observation, conservative endoscopic management, and finally open surgical reconstruction.

Transdiaphragmatic peritoneopericardial hernia is a rare complication after peritoneopericardial window formation, coronary artery bypass grafting using the gastroepiploic artery, or subxiphoid epicardial pacemaker insertion. We describe two different clinical presentations of transdiaphragmatic peritoneopericardial hernia in patients who had undergone recent heart transplantation. One was an exceptional case of cardiac tamponade caused by small bowel strangulation through a diaphragmatic defect.

Jarcho-Levin syndrome is a rare disorder characterised by defects in vertebral and costal segmentation of varying severity. Respiratory complications are the main cause of death or severe comorbidity due to a restrictive rib cage. A 3 months old infant with Spondylocostal dysostosis and associated bronchomalacia experiencing severe asynchrony during the weaning process is reported. The Neurally Adjusted Ventilatory Assist (NAVA) mode was used to improve adaptation to mechanical ventilation after Vertical Expandable Prosthetic Titanium Ribs (VEPTRs) were implanted. The synchrony achieved with the NAVA mode allowed a decrease of the sedoanalgesia he received. A follow-up CT scan showed a reduction in the volume of the posterobasal atelectasis. The evolution of this patient suggests that the combined use of VEPTR for thoracic expansion and ventilation using NAVA can favour the global improvement. This mode could be an option to consider in selected patients with difficult weaning from mechanical ventilation in paediatric intensive care units., (2017 BMJ Publishing Group Ltd.)

Background: Nitric oxide (NO) is a major endogenous regulator of vascular tone. Inhaled nitric oxide (iNO) gas has been investigated as treatment for persistent pulmonary hypertension of the newborn., Objectives: To determine whether treatment of hypoxaemic term and near-term newborn infants with iNO improves oxygenation and reduces rate of death and use of extracorporeal membrane oxygenation (ECMO), or affects long-term neurodevelopmental outcomes., Search Methods: We used the standard search strategy of the Cochrane Neonatal Review Group to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1), MEDLINE via PubMed (1966 to January 2016), Embase (1980 to January 2016) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1982 to January 2016). We searched clinical trials databases, conference proceedings and reference lists of retrieved articles for randomised controlled trials and quasi-randomised trials. We contacted the principal investigators of studies published as abstracts to ascertain the necessary information., Selection Criteria: Randomised studies of iNO in term and near-term infants with hypoxic respiratory failure, with clinically relevant outcomes, including death, use of ECMO and oxygenation., Data Collection and Analysis: We analysed trial reports to assess methodological quality using the criteria of the Cochrane Neonatal Review Group. We tabulated mortality, oxygenation, short-term clinical outcomes (particularly use of ECMO) and long-term developmental outcomes., Statistics: For categorical outcomes, we calculated typical estimates for risk ratios and risk differences. For continuous variables, we calculated typical estimates for weighted mean differences. We used 95% confidence intervals and assumed a fixed-effect model for meta-analysis., Main Results: We found 17 eligible randomised controlled studies that included term and near-term infants with hypoxia.Ten trials compared iNO versus control (placebo or standard care without iNO) in infants with moderate or severe severity of illness scores (Ninos 1996; Roberts 1996; Wessel 1996; Davidson 1997; Ninos 1997; Mercier 1998; Christou 2000; Clark 2000; INNOVO 2007; Liu 2008). Mercier 1998 compared iNO versus control but allowed back-up treatment with iNO for infants who continued to satisfy the same criteria for severity of illness after two hours. This trial enrolled both preterm and term infants but reported most results separately for the two groups. Ninos 1997 studied only infants with congenital diaphragmatic hernia.One trial compared iNO versus high-frequency ventilation (Kinsella 1997).Six trials enrolled infants with moderate severity of illness scores (oxygenation index (OI) or alveolar-arterial oxygen difference (A-aDO2)) and randomised them to immediate iNO treatment or iNO treatment only after deterioration to more severe criteria (Barefield 1996; Day 1996; Sadiq 1998; Cornfield 1999; Konduri 2004; Gonzalez 2010).Inhaled nitric oxide appears to have improved outcomes in hypoxaemic term and near-term infants by reducing the incidence of the combined endpoint of death or use of ECMO (high-quality evidence). This reduction was due to a reduction in use of ECMO (with number needed to treat for an additional beneficial outcome (NNTB) of 5.3); mortality was not affected. Oxygenation was improved in approximately 50% of infants receiving iNO. The OI was decreased by a (weighted) mean of 15.1 within 30 to 60 minutes after the start of therapy, and partial pressure of arterial oxygen (PaO2) was increased by a mean of 53 mmHg. Whether infants had clear echocardiographic evidence of persistent pulmonary hypertension of the newborn (PPHN) did not appear to affect response to iNO. Outcomes of infants with diaphragmatic hernia were not improved; outcomes were slightly, but not significantly, worse with iNO (moderate-quality evidence).Infants who received iNO at less severe criteria did not have better clinical outcomes than those who were enrolled but received treatment only if their condition deteriorated. Fewer of the babies who received iNO early satisfied late treatment criteria, showing that earlier iNO reduced progression of the disease but did not further decrease mortality nor the need for ECMO (moderate-quality evidence). Incidence of disability, incidence of deafness and infant development scores were all similar between tested survivors who received iNO and those who did not., Authors' Conclusions: Inhaled nitric oxide is effective at an initial concentration of 20 ppm for term and near-term infants with hypoxic respiratory failure who do not have a diaphragmatic hernia.