Can we improve outcome of congenital diaphragmatic hernia?

This review gives an overview of the disease spectrum of congenital diaphragmatic hernia (CDH). Etiological factors, prenatal predictors of survival, new treatment strategies and long-term morbidity are described. Early recognition of problems and improvement of treatment strategies in CDH patients may increase survival and prevent secondary morbidity. Multidisciplinary healthcare is necessary to improve healthcare for CDH patients. Absence of international therapy guidelines, lack of evidence of many therapeutic modalities and the relative low number of CDH patients calls for cooperation between centers with an expertise in the treatment of CDH patients. The international CDH Euro-Consortium is an example of such a collaborative network, which enhances exchange of knowledge, future research and development of treatment protocols

Anorectal Physiology: Test and Clinical Application

The physiology of the anorectal region is very complex, and it is only recently that detailed investigations have given us a better understanding of its function. The methods that are used for the evaluation of anorectal physiology include anorectal manometry, defecography, continence tests, electromyography of the anal sphincter and the pelvic floor, and nerve stimulation tests. These techniques furnish a clearer picture of the mechanisms of anorectal disease and demonstrate pathophysiologic abnormalities in patients with disorders of the anorectal region. Therefore, therapeutic recommendations for anorectal disease can be made best when the anatomy and the physiology of the anorectal region are understood

Therapeutic approach to Gradenigo's syndrome: a case report

Abstract Introduction Traditional management of Gradenigo's syndrome requires aggressive and radical surgery without any attempt to preserve hearing. Recent reports, however, describe a successful outcome after conservative surgical intervention without labyrinthectomy. A similar outcome has also been reported in patients who were only prescribed with antibiotics and did not undergo myringotomy. Case presentation We report the case of a 24-year-old Caucasian Greek woman with Gradenigo's syndrome who was treated by draining her petrous apex via an infralabyrithine approach between her posterior semicircular canal and the jugular bulb. Her inner ear was not sacrificed during the procedure. She presented pre-operatively with ipsilateral conductive hearing loss, which recovered completely four weeks after the surgery. Conclusions Patients with Gradenigo's syndrome may be successfully treated with a combination of long-term permanent drainage and ventilation of the apical cells with corresponding hearing preservation. This can be achieved via a combination of transmastoid, infralabyrinthine and suprajugular approaches, if such would be allowed by the anatomy of the region or if there is enough space between the posterior semicircular canal and the jugular bulb.</p

2009 H1N1 influenza virus infection and necrotizing pneumonia treated with extracorporeal membrane oxygenation

A 3-year-old girl with acute respiratory distress syndrome due to a H1N1 2009 influenza virus infection was complicated by necrotizing pneumonia was successfully treated with extracorporeal membrane oxygenation (ECMO). This is the first reported case in which a pediatric patient was rescued with ECMO during the H1N1 influenza epidemic in Korea in 2009

Sinugrafia direta via seio sagital superior na criança. Contribuição para sua interpretação

Este trabalho, sugerido pela freqüência dos quadros clínicos atribuíveis a oclusão dêste ou daquele seio da dura-mater, tem por escopo o estudo radiológico dos seios durais mediante injeção de substância radiopaca diretamente no seio sagital superior. Dado o grande número de variações anatômicas dêsses canais venosos, a parte central dêste trabalho é representada pelo estudo de 50 casos de crianças cujos quadros clínicos nada apresentavam atribuível a qualquer afecção sinusal. Cinco casos patológicos incluídos também no material desta tese são utilizados apenas para maior objetivação do conceito de normalidade. Esta modalidade de exame não permite o estudo de todo o sistema sinusal, o que limita, até certo ponto, as indicações da sinugrafia direta. As conclusões a que chegamos pela análise de nosso material são as seguintes: 1. A sinugrafia direta, pela introdução de contraste no seio sagital superior, tem indicação nos casos em que se pretende o exame dos seios confluenciais, excetuado o seio reto. 2. A técnica do exame é fácil, permitindo, se necessário, sua repetição imediata. 3. O exame foi bem tolerado e inócuo em todos os 50 casos utilizados para a elaboração dêste trabalho. 4. Das variações do confluente dos seios descritas pelos anatomistas, apenas três são passíveis de identificação radiológica: tipo reservatório comum, tipo plexiforme (com seio sagital superior bifurcado) e tipo occipital. As variações tipo ipsilateral (com circulação cruzada) e plexiforme (com seio reto bifurcado), não são passíveis de identificação radiológica por este método. 5. Radiològicamente, são mais freqüentes as variações tipo reservatório comum e plexiforme (com seio sagital superior bifurcado). O tipo occipital é menos encontrado. A maior freqüência da confluência sinusal de tipo reservatório comum, em contraste com os achados anatômicos, decorre do fato de estarem incluídos nessa variação os tipos ipsilateral (com circulação cruzada) e plexiforme (com seio reto bifurcado). 6. São raros os casos de derivações unilaterais do fluxo venoso intracraniano, através do confluente dos seios. 7. Nossos achados radiológicos não assinalam predominância da drenagem venosa intracraniana por um dos seios transversos. 8. A sinugrafia direta via seio sagital superior, pela direção do fluxo venoso, permite o diagnóstico de oclusões orgânicas situadas: a) no seio sagital superior, a jusante do local da puncão; b) na origem de um dos seios transversos ao nível do confluente, sòmente quando existir seio sagital superior bifurcado ou canal de circulação cruzada; c) nos seios transversos. 9. Êste método não permite o diagnóstico: a) das oclusões incompletas dos canais venosos durais; b) da oclusão de um dos seios transversos em sua origem, na ausência de um seio sagital superior bifurcado ou de um canal de circulação cruzada, pois não seria possível diferençá-los das variações do tipo unilateral ou ipsilateral sem circulação cruzada. 10. A manobra de oclusão digital da veia jugular interna tem valor relevante quando não fôr visibilizado um dos seios transversos; sòmente essa manobra permite decidir entre um bloqueio orgânico e um bloqueio aparente, devido a circunstâncias ocasionais

Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe:The CDH EURO Consortium Consensus - 2015 Update

In 2010, the congenital diaphragmatic hernia (CDH) EURO Consortium published a standardized neonatal treatment protocol. Five years later, the number of participating centers has been raised from 13 to 22. In this article the relevant literature is updated, and consensus has been reached between the members of the CDH EURO Consortium. Key updated recommendations are: (1) planned delivery after a gestational age of 39 weeks in a high-volume tertiary center; (2) neuromuscular blocking agents to be avoided during initial treatment in the delivery room; (3) adapt treatment to reach a preductal saturation of between 80 and 95% and postductal saturation &gt;70%; (4) target PaCO2 to be between 50 and 70 mm Hg; (5) conventional mechanical ventilation to be the optimal initial ventilation strategy, and (6) intravenous sildenafil to be considered in CDH patients with severe pulmonary hypertension. This article represents the current opinion of all consortium members in Europe for the optimal neonatal treatment of CDH.</p

The efficacy of hypotonic and near-isotonic saline for parenteral fluid therapy given at low maintenance rate in preventing significant change in plasma sodium in post-operative pediatric patients: protocol for a prospective randomized non-blinded study

<p>Abstract</p> <p>Background</p> <p>Hyponatremia is the most frequent electrolyte abnormality observed in post-operative pediatric patients receiving intravenous maintenance fluid therapy. If plasma sodium concentration (p-Na⁺) declines to levels below 125 mmol/L in < 48 h, transient or permanent brain damage may occur. There is an intense debate as to whether the administered volume (full rate <it>vs. </it>restricted rate of infusion) and the composition of solutions used for parenteral maintenance fluid therapy (hypotonic <it>vs. </it>isotonic solutions) contribute to the development of hyponatremia. So far, there is no definitive pediatric data to support a particular choice of parenteral fluid for maintenance therapy in post-surgical patients.</p> <p>Methods/Design</p> <p>Our prospective randomized non-blinded study will be conducted in healthy children and adolescents aged 1 to 14 years who have been operated for acute appendicitis. Patients will be randomized either to intravenous hypotonic (0.23% or 0.40% sodium chloride in glucose, respectively) or near-isotonic (0.81% sodium chloride in glucose) solution given at approximately three-fourths of the average maintenance rate. The main outcome of interest from this study is to evaluate 24 h post-operatively whether differences in p-Na⁺between treatment groups are large enough to be of clinical relevance. In addition, water and electrolyte balance as well as regulatory hormones will be measured.</p> <p>Discussion</p> <p>This study will provide valuable information on the efficacy of hypotonic and near-isotonic fluid therapy in preventing a significant decrease in p-Na⁺. Finally, by means of careful electrolyte and water balance and by measuring regulatory hormones our results will also contribute to a better understanding of the physiopathology of post-operative changes in p-Na⁺in a population at risk for hyponatremia.</p> <p>Trial registration</p> <p>The protocol for this study is registered with the current controlled trials registry; registry number: <a href="http://www.controlled-trials.com/ISRCTN43896775">ISRCTN43896775</a>.</p

The CoDiNOS trial protocol: an international randomised controlled trial of intravenous sildenafil versus inhaled nitric oxide for the treatment of pulmonary hypertension in neonates with congenital diaphragmatic hernia

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that impairs normal lung development, causing pulmonary hypertension (PH). PH in CDH newborns is the main determinant for morbidity and mortality. Different therapies are still mainly based on 'trial and error'. Inhaled nitric oxide (iNO) is often the drug of first choice. However, iNO does not seem to improve mortality. Intravenous sildenafil has reduced mortality in newborns with PH without CDH, but prospective data in CDH patients are lacking. METHODS AND ANALYSIS: In an open label, multicentre, international randomised controlled trial in Europe, Canada and Australia, 330 newborns with CDH and PH are recruited over a 4-year period (2018-2022). Patients are randomised for intravenous sildenafil or iNO. Sildenafil is given in a loading dose of 0.4 mg/kg in 3 hours; followed by continuous infusion of 1.6 mg/kg/day, iNO is dosed at 20 ppm. Primary outcome is absence of PH on day 14 without pulmonary vasodilator therapy and/or absence of death within the first 28 days of life. Secondary outcome measures include clinical and echocardiographic markers of PH in the first year of life. We hypothesise that sildenafil gives a 25% reduction in the primary outcome from 68% to 48% on day 14, for which a sample size of 330 patients is needed. An intention-to-treat analysis will be performed. A p-value (two-sided) <0.05 is considered significant in all analyses. ETHICS AND DISSEMINATION: Ethics approval has been granted by the ethics committee in Rotterdam (MEC-2017-324) and the central Committee on Research Involving Human Subjects (NL60229.078.17) in the Netherlands. The principles of the Declaration of Helsinki, the Medical Research Involving Human Subjects Act and the national rules and regulations on personal data protection will be used. Parental informed consent will be obtained. TRIAL REGISTRATION NUMBER: NTR6982; Pre-results

Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

In 2010, the congenital diaphragmatic hernia (CDH) EURO Consortium published a standardized neonatal treatment protocol. Five years later, the number of participating centers has been raised from 13 to 22. In this article the relevant literature is updated, and consensus has been reached between the members of the CDH EURO Consortium. Key updated recommendations are: (1) planned delivery after a gestational age of 39 weeks in a high-volume tertiary center; (2) neuromuscular blocking agents to be avoided during initial treatment in the delivery room; (3) adapt treatment to reach a preductal saturation of between 80 and 95% and postductal saturation >70%; (4) target PaCO2 to be between 50 and 70 mm Hg; (5) conventional mechanical ventilation to be the optimal initial ventilation strategy, and (6) intravenous sildenafil to be considered in CDH patients with severe pulmonary hypertension. This article represents the current opinion of all consortium members in Europe for the optimal neonatal treatment of CDH

Mortality factors in infants with congenital diaphragmatic hernia : A systematic review

Acknowledgements: We thank Alex Inskip, senior library assistant at Newcastle University, for assistance in utilising the databases and composing the search strategy. Funding Source: RV is funded by Public Health England (PHE).Peer reviewedPostprintPostprin