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Seminars in Pediatric Surgery

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https://www.readbyqxmd.com/read/28641757/long-term-follow-up-of-congenital-diaphragmatic-hernia
#1
Laura E Hollinger, Matthew T Harting, Kevin P Lally
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...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641756/regenerative-medicine-solutions-in-congenital-diaphragmatic-hernia
#2
Paolo De Coppi, Jan Deprest
Congenital diaphragmatic hernia (CDH) remains a major challenge and associated mortality is still significant. Patients have benefited from current therapeutic options, but most severe cases are still associated to poor outcome. Regenerative medicine is emerging as a valid option in many diseases and clinical trials are currently happening for various conditions in children and adults. We report here the advancement in the field which will help both in the understanding of further CDH development and in offering new treatment options for the difficult situations such as repair of large diaphragmatic defects and lung hypoplasia...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641755/ecmo-in-cdh-is-there-a-role
#3
David W Kays
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...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641754/ventilation-modalities-in-infants-with-congenital-diaphragmatic-hernia
#4
Francesco Morini, Irma Capolupo, Willem van Weteringen, Irwin Reiss
Neonates with congenital diaphragmatic hernia are among the more complex patients to support with mechanical ventilation. They have particular features that add to the difficulties already present in the neonatal patient. A ventilation strategy tailored to the patient's underlying physiology rather than mode of ventilation is a crucial issue for clinicians treating these delicate patients.
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641753/cardiac-dysfunction-in-congenital-diaphragmatic-hernia-pathophysiology-clinical-assessment-and-management
#5
Neil Patel, Florian Kipfmueller
Cardiac dysfunction is an important consequence of pulmonary hypertension in congenital diaphragmatic hernia and a determinant of disease severity. Increased afterload leads to right ventricular dilatation and diastolic dysfunction. Septal displacement and dysfunction impair left ventricular function, which may also be compromised by fetal hypoplasia. Biventricular failure contributes to systemic hypotension and hypoperfusion. Early and regular echocardiographic assessment of cardiac function and pulmonary artery pressure can guide therapeutic decision-making, including choice and timing of pulmonary vasodilators, cardiotropes, ECMO, and surgery...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641752/congenital-diaphragmatic-hernia-associated-pulmonary-hypertension
#6
Matthew T Harting
Congenital diaphragmatic hernia (CDH) is a complex entity wherein a diaphragmatic defect allows intrathoracic herniation of intra-abdominal contents and both pulmonary parenchymal and vascular development are stifled. Pulmonary pathology and pathophysiology, including pulmonary hypoplasia and pulmonary hypertension, are hallmarks of CDH and are associated with disease severity. Pulmonary hypertension (PH) is sustained, supranormal pulmonary arterial pressure, and among patients with CDH (CDH-PH), is driven by hypoplastic pulmonary vasculature, including alterations at the molecular, cellular, and tissue levels, along with pathophysiologic pulmonary vasoreactivity...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641751/fetal-imaging-and-therapy-for-cdh-current-status
#7
Titilayo Oluyomi-Obi, Tim Van Mieghem, Greg Ryan
In congenital diaphragmatic hernia (CDH), herniation of the abdominal organs into the fetal chest causes pulmonary hypoplasia and pulmonary hypertension, the main causes of neonatal mortality. As antenatal ultrasound screening improves, the risk of postnatal death can now be better predicted, allowing for the identification of fetuses that might most benefit from a prenatal intervention. Fetoscopic tracheal occlusion is being evaluated in a large international randomized controlled trial. We present the antenatal imaging approaches that can help identify fetuses that might benefit from antenatal therapy, and review the evolution of fetal surgery for CDH to date...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641750/predicting-outcomes-in-congenital-diaphragmatic-hernia
#8
Oluwatomilayo Daodu, Mary E Brindle
Identification of CDH infant populations at high risk for mortality postnatally may help to develop targeted care strategies, guide discussions surrounding palliation and contribute to standardizing reporting and benchmarking, so that care strategies at different centers can be compared. Clinical prediction rules are evidence-based tools that combine multiple predictors to estimate the probability that a particular outcome in an individual patient will occur. In CDH, a suitable clinical prediction rule can stratify high- and low-risk populations and provide the ability to tailor management strategies based on severity...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641749/congenital-diaphragmatic-hernia-the-role-of-multi-institutional-collaboration-and-patient-registries-in-supporting-best-practice
#9
Pamela A Lally, Erik D Skarsgard
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...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641748/abnormal-lung-development-in-congenital-diaphragmatic-hernia
#10
Dustin Ameis, Naghmeh Khoshgoo, Richard Keijzer
The outcomes of patients diagnosed with congenital diaphragmatic hernia (CDH) have recently improved. However, mortality and morbidity remain high, and this is primarily caused by the abnormal lung development resulting in pulmonary hypoplasia and persistent pulmonary hypertension. The pathogenesis of CDH is poorly understood, despite the identification of certain candidate genes disrupting normal diaphragm and lung morphogenesis in animal models of CDH. Defects within the lung mesenchyme and interstitium contribute to disturbed distal lung development...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641747/preface
#11
EDITORIAL
Kevin P Lally
No abstract text is available yet for this article.
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550877/caustic-ingestion-in-children-a-review
#12
Marion Arnold, Alp Numanoglu
Various domestic or industrial chemicals may cause significant upper aerodigestive tract burns. Preventive measures should be up-scaled, especially in the developing world, to reduce the epidemic of accidental victims, largely unsupervised preschool children. External signs do not predict degree of injury. Non-invasive diagnostic screening includes radio-nuclear imaging, but early oesophago-gastroduodenoscopy remains the standard to predict stricture formation from circumferential submucosal scarring. Serial dilation is the mainstay of oesophageal stricture therapy, with oesophageal replacement reserved for severe refractory strictures...
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550876/neonatal-and-pediatric-esophageal-perforation
#13
Rebecca M Rentea, Shawn D St Peter
Esophageal perforation (EP) is a rare complication that is often iatrogenic in origin. In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has become a common therapeutic approach for EP in neonates and children. Principles of management pediatric EP includes rapid diagnosis, appropriate hemodynamic monitoring and support, antibiotic therapy, total parenteral nutrition, control of extraluminal contamination, and restoration of luminal integrity either through time or operative approaches...
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550875/esophageal-duplication-and-congenital-esophageal-stenosis
#14
A Francois Trappey, Shinjiro Hirose
Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment.
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550874/long-gap-esophageal-atresia
#15
Hester F Shieh, Russell W Jennings
The management of long-gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise. Given the complexity of this patient population with significant morbidity, treatment and long-term follow-up are best done in multidisciplinary esophageal and airway treatment centers...
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550873/esophageal-atresia-and-tracheo-esophageal-fistula
#16
David C van der Zee, Stefaan H A Tytgat, Maud Y A van Herwaarden
Management of esophageal atresia has merged from correction of the anomaly to the complete spectrum of management of esophageal atresia and all its sequelae. It is the purpose of this article to give an overview of all aspects involved in taking care of patients with esophageal atresia between January 2011 and June 2016, as well as the patients who were referred from other centers. Esophageal atresia is a complex anomaly that has many aspects that have to be dealt with and complications to be solved. By centralizing these patients in centers of expertise it is believed that the best care can be given...
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550872/hiatal-and-paraesophageal-hernia-repair-in-pediatric-patients
#17
Erin M Garvey, Daniel J Ostlie
Hiatal and paraesophageal hernia (HH/PEH) can be congenital, resulting from embryologic abnormalities/genetic predisposition, or acquired, most commonly after gastroesophageal surgery such as fundoplication. Minimizing circumferential esophageal dissection at the time of Nissen fundoplication has been shown to decrease the risk of acquired HH/PEH from 36.5% to 12.2%. Gastrointestinal, respiratory, and constitutional symptoms, including anemia and failure to thrive, are common with high rates of associated gastroesophageal reflux...
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550871/gastroesophageal-reflux
#18
Bethany J Slater, Steven S Rothenberg
Gastroesophageal reflux disease (GERD) is a very common condition and affects approximately 7-20% of the pediatric population. Symptoms from pathological GERD include regurgitation, irritability when feeding, respiratory problems, and substernal pain. Treatment typically starts with dietary modifications and postural changes. Antireflux medications may then be added. Indications for operative management in the pediatric population include failure of medical therapy with poor weight gain or failure to thrive, continued respiratory symptoms, and complications such as esophagitis...
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550870/preface-esophageal-disorders
#19
EDITORIAL
Mark Wulcan
No abstract text is available yet for this article.
April 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28550869/achalasia
#20
Saleem Islam
Achalasia is a rare neurogenic motility disorder of the esophagus, occurring in approximately 0.11 cases per 100,000 children. The combination of problems (aperistalsis, hypertensive lower esophageal sphincter (LES), and lack of receptive LES relaxation) results in patients having symptoms of progressive dysphagia, weight loss, and regurgitation. Treatment modalities have evolved over the past few decades from balloon dilation and botulinum toxin injection to laparoscopic Heller myotomy and endoscopic myotomy...
April 2017: Seminars in Pediatric Surgery
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