7,087 results match your criteria Diaphragmatic Hernias Congenital


Thoracoscopic repair of congenital diaphragmatic hernia: a new anatomical reconstructive concept for tension dispersal at primary closure.

Surg Endosc 2020 Jul 2. Epub 2020 Jul 2.

Tanta University Hospital, Tanta, Egypt.

Background: Several measures were implemented among authors striving to tail off recurrence rates of thoracoscopic congenital diaphragmatic hernia repair. In the presented study, we extended the use of rib-anchoring stitches to reorient the diaphragmatic muscle leaflets in the types B&C diaphragmatic hernias, to achieve tension dispersal at primary thoracoscopic repair.

Patients And Methods: Included in this study were early and late-onset lateral congenital diaphragmatic hernia patients, who had been operated upon in the years 2012 through 2018. Read More

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http://dx.doi.org/10.1007/s00464-020-07764-5DOI Listing

Prenatal regenerative fetoscopic interventions for congenital anomalies.

Authors:
Rodrigo Ruano

BMJ 2020 Jul 1;370:m1624. Epub 2020 Jul 1.

Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology and Center for Regenerative Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.

Fetal intervention has progressed in the past two decades from experimental proof-of-concept to practice-adopted, life saving interventions in human fetuses with congenital anomalies. This progress is informed by advances in innovative research, prenatal diagnosis, and fetal surgical techniques. Invasive open hysterotomy, associated with notable maternal-fetal risks, is steadily replaced by less invasive fetoscopic alternatives. Read More

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http://dx.doi.org/10.1136/bmj.m1624DOI Listing

Optimal gestational age at delivery for congenital diaphragmatic hernia.

Ultrasound Obstet Gynecol 2020 Jul 1. Epub 2020 Jul 1.

Centre Reference Maladie Rare, Hernie de Coupole Diaphragmatique, Clamart, France.

Objective: To evaluate the neonatal morbidity and mortality of babies with isolated congenital diaphragmatic hernia (CDH) according to gestational age at delivery.

Methods: We conducted a retrospective study in the University Hospitals of Antoine Béclère-Bicêtre and Leuven between January 1, 2010 and December 31, 2018. Isolated left-sided CDH cases were included. Read More

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http://dx.doi.org/10.1002/uog.22133DOI Listing

Thromboelastography-guided management of coagulopathy in neonates with congenital diaphragmatic hernia supported by extracorporeal membrane oxygenation.

Pediatr Surg Int 2020 Jun 30. Epub 2020 Jun 30.

Department of Surgery, Division of Pediatric Surgery, University of Colorado School of Medicine, Aurora, CO, USA.

Purpose: Congenital diaphragmatic hernia (CDH) can cause severe hemodynamic deterioration requiring support with extracorporeal membrane oxygenation (ECMO). ECMO is associated with hemorrhagic and thromboembolic complications. In 2015, we standardized anti-coagulation management on ECMO, incorporating thromboelastography (TEG) as an adjunct to manage hemostasis of CDH patients. Read More

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http://dx.doi.org/10.1007/s00383-020-04694-0DOI Listing

New device permitting non-invasive reversal of fetoscopic tracheal occlusion: ex-vivo and in-vivo study.

Ultrasound Obstet Gynecol 2020 Jun 30. Epub 2020 Jun 30.

My FetUZ Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU, Leuven, Belgium.

Objective: One of the drawbacks of fetoscopic endoluminal tracheal occlusion for congenital diaphragmatic hernia is the need for a second invasive intervention to reestablish airway patency. The "Smart-TO" (Strasbourg University-BSMTI, France) is a new balloon for endoluminal tracheal occlusion, which spontaneously deflates when placed in a strong magnetic field, therefore overcoming the need of a second procedure. The safety and efficacy of this device has not been demonstrated. Read More

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http://dx.doi.org/10.1002/uog.22132DOI Listing

Prenatal sonography of extracorporeal ductus venosus in association with large fetal gastroschisis.

J Clin Ultrasound 2020 Jun 29. Epub 2020 Jun 29.

The Division of Maternal Fetal Medicine, The Department of Obstetrics and Gynecology, State University of New York (SUNY), Downstate Heath Sciences University, Brooklyn, New York, USA.

Liver herniation commonly associated with omphalocele occurs in only approximately 2.3% to 16% of fetuses with gastroschisis. Liver herniation in such cases is associated with considerably decreased survival rates (43% vs 97% with or without liver herniation, respectively). Read More

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http://dx.doi.org/10.1002/jcu.22881DOI Listing

Application of Tissue Engineering and Regenerative Medicine in Maternal-Fetal Medicine.

Adv Exp Med Biol 2020 ;1249:161-171

Department of obstetrics and gynecology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

A rapid development of ultrasonography has enabled physicians to make earlier prenatal diagnosis of various fetal congenital diseases, in maternal-fetal medicine. Due to the significant mortality and irreversible damage to fetal vital organs during pregnancy, fetal surgeries have been tried in some congenital disease including congenital diaphragmatic hernia, twin-to-twin transfusion syndrome (TTTS), myelomeningocele (MMC), and lower urinary tract obstruction. However, open fetal surgery requires laparotomy followed by hysterotomy, which can cause preterm premature rupture of membrane (pPROM), oligohydramnios, preterm delivery, dehiscence of uterine wall, and other maternal complications during pregnancy. Read More

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http://dx.doi.org/10.1007/978-981-15-3258-0_11DOI Listing
January 2020

Laparoscopic approach for diaphragmatic hernia repair in adult: our experience of four cases.

J Surg Case Rep 2020 Jun 19;2020(6):rjaa178. Epub 2020 Jun 19.

Department of Surgery, BPKIHS, Dharan, Nepal.

Diaphragmatic hernia can be congenital or acquired. Usual clinical presentation may range from asymptomatic cases to serious respiratory/gastrointestinal symptoms and occasionally atypical presentation as lumbar hernia also. The diagnosis of diaphragmatic hernia is based on clinical investigation and is confirmed by plain X-ray film and computed tomography scan. Read More

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http://dx.doi.org/10.1093/jscr/rjaa178DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7303022PMC

Rectal Omeprazole in Infants With Gastroesophageal Reflux Disease: A Randomized Pilot Trial.

Eur J Drug Metab Pharmacokinet 2020 Jun 27. Epub 2020 Jun 27.

Intensive Care and Department of Pediatric Surgery, Erasmus MC Sophia, Rotterdam, The Netherlands.

Background And Objective: Omeprazole is a proton pump inhibitor that is used in acid suppression therapy in infants. Infants cannot swallow the oral tablets or capsules. Since, infants require a non-standard dose of omeprazole, the granules or tablets are often crushed or suspended in water or sodium bicarbonate, which may destroy the enteric coating. Read More

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http://dx.doi.org/10.1007/s13318-020-00630-8DOI Listing

Transgenic animal models of congenital diaphragmatic hernia: a comprehensive overview of candidate genes and signaling pathways.

Pediatr Surg Int 2020 Jun 26. Epub 2020 Jun 26.

National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland.

Congenital diaphragmatic hernia (CDH) is a relatively common and life-threatening birth defect, characterized by incomplete formation of the diaphragm. Because CDH herniation occurs at the same time as preacinar airway branching, normal lung development becomes severely disrupted, resulting almost invariably in pulmonary hypoplasia. Despite various research efforts over the past decades, the pathogenesis of CDH and associated lung hypoplasia remains poorly understood. Read More

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http://dx.doi.org/10.1007/s00383-020-04705-0DOI Listing

A proteome signature of umbilical cord serum associated with congenital diaphragmatic hernia.

Nagoya J Med Sci 2020 May;82(2):345-354

Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Congenital diaphragmatic hernia (CDH) is a congenital anomaly characterized by a defect in the diaphragm. Despite the recent improvements in its treatment, CDH is associated with a high rate of neonatal mortality, which is often related to pulmonary hypoplasia (PH) as well as pulmonary hypertension. A better understanding of the underlying pathological mechanisms of PH in CDH could help establish a new treatment to improve its prognosis. Read More

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http://dx.doi.org/10.18999/nagjms.82.2.345DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7276398PMC

Laparoscopic repair of Morgagni's hernia in an elderly female patient.

J Surg Case Rep 2020 Jun 15;2020(6):rjaa182. Epub 2020 Jun 15.

General Surgery, Centro Hospitalar Tondela-Viseu, Viseu, Portugal.

Morgagni's hernia is a very uncommon congenital diaphragmatic hernia. A few patients may remain asymptomatic until adulthood. Clinical presentation may include bowel obstruction, chest pain or dyspnoea. Read More

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http://dx.doi.org/10.1093/jscr/rjaa182DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7295565PMC

Extracellular vesicles influence the pulmonary arterial extracellular matrix in congenital diaphragmatic hernia.

Pediatr Pulmonol 2020 Jun 22. Epub 2020 Jun 22.

Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center and Children's Memorial Hermann Hospital, Houston, TX, USA.

Objective: Abnormal pulmonary vasculature directly affects the development and progression of congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension (PH). Though overarching structural and cellular changes in CDH-affected pulmonary arteries have been documented, the precise role of the extracellular matrix (ECM) in pulmonary artery (PA) pathophysiology remains undefined. Here, we quantify the structural, compositional, and mechanical CDH-induced changes in the main and distal PA ECM and investigate the efficacy of mesenchymal stem cell-derived extracellular vesicles (MSC-EVs) as a therapy to ameliorate pathological vascular ECM changes. Read More

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http://dx.doi.org/10.1002/ppul.24914DOI Listing

Quality of life was similar in children with congenital diaphragmatic hernia and oesophageal atresia and related to respiratory morbidity.

Acta Paediatr 2020 Jun 21. Epub 2020 Jun 21.

Univ. Lille, CHU Lille, Paediatric Pulmonology and Allergy Unit, Hôpital Jeanne de Flandre, F-59000, Lille, France.

Aim: To assess quality of life (QoL) in children with congenital diaphragmatic hernia (CDH) and to compare it with oesophageal atresia (OA).

Methods: A cross-sectional study in CDH children (≥ 7 years) was conducted in Lille University Hospital, France, from January 2013 to April 2014. History, lung function (rest, exercise), Pediatric Quality of Life Inventory questionnaires (PedsQoL 4. Read More

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http://dx.doi.org/10.1111/apa.15426DOI Listing

The Influence of Various Cerebral and Extracerebral Pathologies on Apparent Diffusion Coefficient Values in the Fetal Brain.

J Neuroimaging 2020 Jun 17. Epub 2020 Jun 17.

Institute of Diagnostic and Interventional Neuroradiology, Bern University Hospital, Bern, Switzerland.

Background And Purpose: The changing MRI signal accompanying brain maturation in fetal brains can be quantified on apparent diffusion coefficient (ADC) maps. Deviations from the natural course of ADC values may reflect structural pathology. The purpose of this study was to determine the influence of fetal pathologies on the ADC values in different regions of the fetal brain and their evolution with increasing gestational age. Read More

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http://dx.doi.org/10.1111/jon.12727DOI Listing

Late presenting congenital diaphragmatic hernia misdiagnosed as a pleural effusion: A case report.

Medicine (Baltimore) 2020 Jun;99(24):e20684

Department of Surgery, Jeonbuk National University Medical School.

Rationale: Late presenting congenital diaphragmatic hernia (CDH) that develops after the neonatal period has various clinical manifestations and can often be misdiagnosed as pleural effusion, pneumonia, or pneumothorax. We report an adolescent case who was transferred to our hospital after iatrogenic gastric perforation during chest tube thoracotomy caused by misdiagnosis of pleural effusion.

Patient Concerns: A 13-year-old boy with no medical history of conditions relevant to CDH and traumatic events visited a community hospital complaining of left upper quadrant abdominal pain and vomiting over the previous 3 days. Read More

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http://dx.doi.org/10.1097/MD.0000000000020684DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7302620PMC

Acute Budd-Chiari syndrome caused by inferior vena cava compression from a congenital diaphragmatic hernia.

Ann R Coll Surg Engl 2020 Jun 15:e1-e3. Epub 2020 Jun 15.

Royal Surrey NHS Foundation Trust, UK.

Acute Budd-Chiari syndrome is a rare condition characterised by obstruction of hepatic venous outflow. We describe the case of a 52-year-old man, with a congenital Morgagni diaphragmatic hernia, who presented with acute onset abdominal pain, shortness of breath, lactic acidosis, hyperbilirubinaemia and transaminasaemia. Computed tomography revealed strangulation of the diaphragmatic hernia and extrinsic compression of the inferior vena cava from the herniated viscera. Read More

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http://dx.doi.org/10.1308/rcsann.2020.0106DOI Listing

Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience.

Eur J Pediatr Surg 2020 Jun 11. Epub 2020 Jun 11.

Division of Child Health, Institute of Child Health, Alder Hey Children' Hospital, University of Liverpool, Liverpool, United Kingdom.

Introduction:  A developing body of literature suggests that the presence of a hernia sac in fetuses with congenital diaphragmatic hernia (CDH) may indicate improved prognosis. By examining a large cohort of CDH newborns admitted to a single United Kingdom specialist center, we aimed to establish if presence of hernia sac is a robust predictor of improved survival.

Materials And Methods:  All CDH patients admitted to a single center were recruited. Read More

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http://dx.doi.org/10.1055/s-0040-1713131DOI Listing

Extracellular Vesicles Attenuate Nitrofen-Mediated Human Pulmonary Artery Endothelial Dysfunction: Implications for Congenital Diaphragmatic Hernia.

Stem Cells Dev 2020 Jun 27. Epub 2020 Jun 27.

Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center (UTHealth) and Children's Memorial Hermann Hospital, Houston, Texas, USA.

Congenital diaphragmatic hernia (CDH) leads to pathophysiologic pulmonary vasoreactivity. Previous studies show that mesenchymal stromal cell-derived extracellular vesicles (MSCEv) inhibit lung inflammation and vascular remodeling. We characterize MSCEv and human pulmonary artery endothelial cell (HPAEC) interaction, as well as the pulmonary artery (PA) response to MSCEv treatment. Read More

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http://dx.doi.org/10.1089/scd.2020.0063DOI Listing
June 2020
3.727 Impact Factor

Risk factors associated with mortality in newborn infants with congenital diaphragmatic hernia.

Arch Argent Pediatr 2020 Jun;118(3):180-186

Departamento de Pediatría, Servicio de Neonatología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina.

Introduction: Morbidity and mortality are high in congenital diaphragmatic hernia. Some tools help to predict survival, both prenatally (observed/expected lung-to-head ratio [OELHR], presence of the liver in the chest) and postnatally (Congenital Diaphragmatic Hernia Study Group [CDHSG] score). Our objective was to identify the risk factors associated with mortality and estimate the risk-adjusted mortality in the prenatal period in the subgroup of patients with isolated left-sided hernia. Read More

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http://dx.doi.org/10.5546/aap.2020.eng.180DOI Listing

Congenital diaphragmatic hernia: postnatal predictors of mortality.

Arch Argent Pediatr 2020 Jun;118(3):173-179

Área de Terapia Intensiva Neonatal, Hospital de Pediatría SAMIC "Prof. Dr. Juan. P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.

Introduction: Congenital diaphragmatic hernia (CDH) prevalence is low while its associated morbidity and mortality rates are high. Postnatal prognostic factors on the first day of life are useful for predicting the outcome.

Objectives: To determine the mortality predictive ability of postnatal echocardiographic, clinical, and biochemical factors among newborn infants with CDH in their first day of life. Read More

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http://dx.doi.org/10.5546/aap.2020.eng.173DOI Listing

Acute massive gastric dilatation: a rare, forgotten complication of fundoplication.

BMJ Case Rep 2020 May 27;13(5). Epub 2020 May 27.

Department of Paediatric Surgery, Nottingham University Hospital NHS Trust, Nottingham, UK.

An 8-year-old boy with a history of multiple neonatal laparotomies, including congenital diaphragmatic hernia repair and an open fundoplication, presented acutely with severe abdominal pain, distension, vomiting and shock. A large abnormal opacity in the left upper quadrant was visible on a plain abdominal radiograph. The patient was taken to the theatre for emergency laparotomy and was found to have a massively distended stomach, the fundus and body of which were necrotic. Read More

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http://dx.doi.org/10.1136/bcr-2019-232479DOI Listing

Prenatal stomach position predicts gastrointestinal morbidity at 2 years in fetuses with congenital diaphragmatic hernia.

Ultrasound Obstet Gynecol 2020 May 27. Epub 2020 May 27.

Antoine Béclère Hospital, Gynecology and Obstetrics Department, Clamart, Paris Sud University, France.

Objective: The long-term morbidity of isolated left-sided congenital diaphragmatic hernia (L-CDH) has already been described. However, antenatal criteria impacting gastrointestinal morbidity (GIM) are not yet defined. The objective of this study was to evaluate the effect of fetal stomach position on GIM at 2 years. Read More

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http://dx.doi.org/10.1002/uog.22086DOI Listing

Growth patterns and associated risk factors of congenital malformations in twins.

Ital J Pediatr 2020 May 24;46(1):73. Epub 2020 May 24.

Department of Health Promotion Sciences, Maternal and Infant Care, Internal Medicine and Medical Specialties "G. D'Alessandro", University of Palermo, Piazza delle Cliniche, 2, 90127, Palermo, Italy.

Background: The rate of twinning continues to increase due to the combined effect of a rise in parental age and increased use of assisted reproductive technology. The risk of congenital anomalies in twins is higher than in singletons, but it is less well reported in relation to growth patterns. We focused to the auxological outcome of twin pregnancies when one or both of twins are affected by one or more malformations. Read More

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http://dx.doi.org/10.1186/s13052-020-00838-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7247245PMC

A Case of Split Notochord Syndrome with Left Congenital Diaphragmatic Hernia: A Rare Association.

Authors:
Pradyumna Pan

J Pediatr Neurosci 2020 Jan-Mar;15(1):42-44. Epub 2020 Mar 18.

Pediatric Surgery Unit, Ashish Hospital and Research Centre, Jabalpur, Madhya Pradesh, India.

Split notochord syndrome is a rare neural tube malformation involving the brain, spinal cord, and vertebral column. In nearly half of the cases, the malformation also involves the gastrointestinal tract in the form of a dorsal neurenteric fistula and imperforate anus. In the literature, less than 50 cases have been reported. Read More

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http://dx.doi.org/10.4103/JPN.JPN_85_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7227745PMC

Risk of malformation after ondansetron in pregnancy: An updated systematic review and meta-analysis.

Birth Defects Res 2020 May 18. Epub 2020 May 18.

Service Hospitalo-Universitaire de Pharmaco-Toxicologie, Hospices Civils de Lyon, Lyon, France.

Ondansetron is increasingly used off label to treat nausea and vomiting during pregnancy. The main objective of this study was to evaluate the risk of major congenital malformations (MCM), cardiac defects and orofacial clefts associated with first trimester exposure to ondansetron using a meta-analytic approach. MEDLINE, ClinicalTrials. Read More

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http://dx.doi.org/10.1002/bdr2.1705DOI Listing

Treating Center Volume and Congenital Diaphragmatic Hernia Outcomes in California.

J Pediatr 2020 Jul 14;222:146-153.e1. Epub 2020 May 14.

Department of Surgery, Stanford University School of Medicine, Palo Alto, CA; Center for Health Policy/Center for Primary Care and Outcomes Research, Stanford University School of Medicine, Stanford, CA; Center for Fetal and Maternal Health, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, CA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA.

Objective: To examined outcomes for infants born with congenital diaphragmatic hernias (CDH), according to specific treatment center volume indicators.

Study Design: A population-based retrospective cohort study was conducted involving neonatal intensive care units in California. Multivariable analysis was used to examine the outcomes of infants with CDH including mortality, total days on ventilation, and respiratory support at discharge. Read More

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http://dx.doi.org/10.1016/j.jpeds.2020.03.028DOI Listing

Acute intrathoracic gastric volvulus: A rare delayed presentation of congenital diaphragmatic hernia: A case report.

Int J Surg Case Rep 2020 11;70:123-125. Epub 2020 May 11.

Department of Pediatric Surgery, Faculty of Medicine, University of Alexandria, Egypt. Electronic address:

Introduction: Acute intrathoracic gastric volvulus occurs when the stomach undergoes organoaxial rotation in the chest due presence of a diaphragmatic defect. Gastric volvulus associated with congenital diaphragmatic hernia is extremely rare and can be explained as 2 of the 4 ligaments supporting the stomach (gastrophrenic and gastrosplenic) which are connected to the left diaphragm may become elongated or absent. According to the current literature, only 27 pediatric cases have been reported so far. Read More

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http://dx.doi.org/10.1016/j.ijscr.2020.04.066DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229233PMC

Rare Course of Bilateral Congenital Diaphragmatic Hernia Treated Thoracoscopically-Case Report.

Front Pediatr 2020 29;8:209. Epub 2020 Apr 29.

Department of Pediatric Surgery and Urology, Wrocław Medical University, Wrocław, Poland.

We present a rare case of metachronous bilateral congenital diaphragmatic hernia (CDH) in a newborn with additional malformations: macroglossia, pectus excavatum, and confirmed Simpson-Golabi-Behmel syndrome. We performed a successful thoracoscopic subsequent repair with a patch of the bilateral type C CDH. Despite using insufflation that should cause bilateral pneumothorax at first procedure there was no clinical signs of such a one. Read More

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http://dx.doi.org/10.3389/fped.2020.00209DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200980PMC

Early postnatal changes of circulating N-terminal-pro-B-type natriuretic peptide in neonates with congenital diaphragmatic hernia.

Early Hum Dev 2020 Jul 25;146:105049. Epub 2020 Apr 25.

Department of Neonatology and Pediatric Intensive Care Medicine, University of Bonn, Germany. Electronic address:

Background: Severity of lung hypoplasia, pulmonary hypertension (PH) and cardiac dysfunction are major contributors to mortality in congenital diaphragmatic hernia (CDH). Therefore, early assessment and management is important to improve outcome. NT-proBNP is an established cardiac biomarker with only limited data for early postnatal risk assessment in CDH newborns. Read More

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http://dx.doi.org/10.1016/j.earlhumdev.2020.105049DOI Listing
July 2020
1.931 Impact Factor

The Use of Inhaled Nitric Oxide in Congenital Diaphragmatic Hernia.

Authors:
Ashley M Novotny

Adv Neonatal Care 2020 May 6. Epub 2020 May 6.

Neonatal Intensive Care Unit, Children's Hospital Colorado-Colorado Springs, and Regis University in Denver, Colorado.

Background: Congenital diaphragmatic hernia (CDH) is a complex congenital defect of the diaphragm that allows abdominal contents to herniate into the chest cavity, altering pulmonary development, and leading to pulmonary hypoplasia and hypertension. Patient presentation is variable in severity, making management difficult. Many common management strategies have improved the survival rate of infants with CDH, including gentle ventilation and permissive hypercapnia. Read More

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http://dx.doi.org/10.1097/ANC.0000000000000753DOI Listing

Congenital diaphragmatic hernia presenting with symptoms within the first day of life; outcomes from a non-ECMO centre in Denmark.

BMC Pediatr 2020 May 7;20(1):196. Epub 2020 May 7.

Research Unit for Department of Anaesthesiology & Intensive Care, Odense University Hospital, Odense, Denmark; University of Southern Denmark, Odense, Denmark.

Background: Between 1998 and 2015, we report on the survival of congenital diaphragmatic hernia (CDH)-infants presenting with symptoms within the first 24 h of life, treated at Odense University Hospital (OUH), a tertiary referral non-extracorporeal membrane oxygenation (ECMO) hospital for paediatric surgery.

Methods: We performed a retrospective cohort study of prospectively identified CDH-infants at our centre. Data from medical records and critical information systems were obtained. Read More

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http://dx.doi.org/10.1186/s12887-020-02072-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204041PMC

Perinatal Outcomes in Fetuses Prenatally Diagnosed with Congenital Diaphragmatic Hernia and Concomitant Lung Lesions: A 10-Year Review.

Fetal Diagn Ther 2020 May 7:1-6. Epub 2020 May 7.

Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Aim: To describe perinatal outcomes of fetuses with a prenatal diagnosis of a concomitant lung lesion in the setting of congenital diaphragmatic hernia (CDH) and to compare outcomes with an isolated CDH control group without a lung lesion, matched by ultrasound-based prognostic markers including presence of liver herniation and lung measurements.

Material And Methods: This was a retrospective case-control study, wherein all pregnancies diagnosed with CDH and concomitant lung lesions were identified between July 1, 2008, and December 31, 2018. For each case, 2 controls with isolated CDH from the same study period were selected after matching for the presence of liver herniation into the thoracic cavity and ultrasound-based lung measurements either observed over expected lung-to-head ratio (LHR) or absolute LHR with their corresponding gestational age. Read More

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http://dx.doi.org/10.1159/000507481DOI Listing

Relationship between volume and outcome for surgery on congenital diaphragmatic hernia: A systematic review.

J Pediatr Surg 2020 Apr 1. Epub 2020 Apr 1.

Institute for Research in Operative Medicine, Faculty of Health, School of Medicine, Witten/Herdecke University, Ostmerheimer Str. 200, Building 38, 51109 Cologne, Germany.

Background: Congenital diaphragmatic hernia (CDH) is a rare and life-threatening anomaly that needs surgical therapy after clinical stabilization of the neonate. Given an existing volume-outcome relationship for other high-risk, low volume procedures, we aimed at examining the relationship between hospital or surgeon volume and outcomes for surgery on CDH.

Methods: We conducted a systematic search in multiple databases in September 2019 and searched for additional literature. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2020.03.025DOI Listing

Maldevelopment of intrapulmonary bronchial cartilage in congenital diaphragmatic hernia.

Pediatr Pulmonol 2020 Jul 5;55(7):1771-1780. Epub 2020 May 5.

Department of Pathology and Applied Neurobiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine (KPUM), Kyoto, Japan.

Background: Pulmonary hypoplasia is an important cause of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). This study aimed to verify our hypothesis that the abnormal development of bronchial cartilage as well as alveolar immaturity, might play a central role in hypoplasia of the lung in human CDH.

Method: We retrospectively analyzed autopsied lungs from 10 CDH cases and compared with nine age-matched controls to assess the bronchial cartilage and alveolar maturity using morphological techniques. Read More

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http://dx.doi.org/10.1002/ppul.24799DOI Listing

Risk factors for pneumothorax associated with isolated congenital diaphragmatic hernia: results of a Japanese multicenter study.

Pediatr Surg Int 2020 Jun 28;36(6):669-677. Epub 2020 Apr 28.

Department of Pediatric Surgery, Kyushu University, Fukuoka, Japan.

Purpose: This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax.

Methods: A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study. Read More

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http://dx.doi.org/10.1007/s00383-020-04659-3DOI Listing

Fetal anesthesia: intrauterine therapies and immediate postnatal anesthesia for noncardiac surgical interventions.

Curr Opin Anaesthesiol 2020 Jun;33(3):368-373

Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia.

Purpose Of Review: This review describes maternal and fetal anesthetic management for noncardiac fetal surgical procedures, including the management of lower urinary tract obstruction, congenital diaphragmatic hernia (CDH), myelomeningocele, sacrococcygeal teratoma, prenatally anticipated difficult airway and congenital lung lesions.

Recent Findings: Fetal interventions range from minimally invasive fetoscopic procedures to mid-gestation open surgery, to ex-utero intrapartum treatment procedure. Anesthetic management depends on the fetal intervention and patient characteristics. Read More

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http://dx.doi.org/10.1097/ACO.0000000000000862DOI Listing

Bochdalek hernia in an adult female with intrathoracic left kidney and splenic flexure of the colon: a rare case report with literature review.

Pol Przegl Chir 2019 Jun;92(2):60-63

Department of Surgery, All India Institute of Medical Sciences, Rishikesh, India.

Congenital diaphragmatic hernia (CDH) is extremely rare in adults. Bochdalek hernia (BH) is the most common one among all congenital diaphragmatic hernias. This is due to incomplete fusion of pleuroperitoneal folds during early fetal development. Read More

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http://dx.doi.org/10.5604/01.3001.0013.2439DOI Listing

Adult congenital diaphragmatic hernia with absent pericardium and liver heterotopia.

Eur J Cardiothorac Surg 2020 Apr 17. Epub 2020 Apr 17.

Department of Thoracic Surgery, Changhai Hospital, Second Military Medical University, Shanghai, China.

Adult congenital diaphragmatic hernia (CDH) with an absent pericardium and liver heterotopia is extremely rare, and had only been described in 1 patient. Here, we present another case of left-sided central CDH in a 40-year-old Chinese woman. Read More

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http://dx.doi.org/10.1093/ejcts/ezaa111DOI Listing

Retinoic Acid Signaling and Development of the Respiratory System.

Subcell Biochem 2020 ;95:151-174

Pulmonary Center, Boston University School of Medicine, 72 East Concord Street, Boston, MA, R-30402118, USA.

Retinoic acid (RA), the bioactive metabolite of vitamin A (VA), has long been recognized as a critical regulator of the development of the respiratory system. During embryogenesis, RA signaling is involved in the development of the trachea, airways, lung, and diaphragm. During postnatal life, RA continues to impact respiratory health. Read More

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http://dx.doi.org/10.1007/978-3-030-42282-0_6DOI Listing

Is laterality of congenital diaphragmatic hernia a reliable prognostic factor? French national cohort study.

Prenat Diagn 2020 Apr 12. Epub 2020 Apr 12.

Department of Obstetrics and Gynecology, Hôpitaux universitaires de Strasbourg, Strasbourg, France.

Objectives: The objective of this study was to assess whether the laterality of congenital diaphragmatic hernia (CDH) was a prognostic factor for neonatal survival.

Methods: This was a cohort study using the French national database of the Reference Center for Diaphragmatic Hernias. The principal endpoint was survival after hospitalization in intensive care. Read More

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http://dx.doi.org/10.1002/pd.5706DOI Listing

A single-center observational study on congenital diaphragmatic hernia: Outcome, predictors of mortality and experience from a tertiary perinatal center in Singapore.

Pediatr Neonatol 2020 Mar 13. Epub 2020 Mar 13.

Department of Neonatology, KK Women's and Children's Hospital, Singapore; Duke-NUS Medical School, Singapore.

Background: Congenital diaphragmatic hernia (CDH) is a common birth defect associated with significant mortality and morbidity. There is limited outcome data on CDH in the Southeast Asian region. Rapid accessibility to our CDH Perinatal Center, as a consequence of the small geographic size of our country and efficient land transportation system, has largely eliminated deaths of live outborn babies prior arrival at our center. Read More

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http://dx.doi.org/10.1016/j.pedneo.2020.03.003DOI Listing

Early behavioral and developmental interventions in ADNP-syndrome: A case report of SWI/SNF-related neurodevelopmental syndrome.

Mol Genet Genomic Med 2020 Jun 10;8(6):e1230. Epub 2020 Apr 10.

Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Background: Autism spectrum disorder (ASD) affects approximately one in 59 children. Variants in the activity-dependent neuroprotector homeobox ADNP (OMIM #611386) gene may be one of the most common single-gene causes of syndromic ASD. Most patients diagnosed with ADNP syndrome have ASD as a comorbidity, and all patients have mild-to-severe intellectual disability. Read More

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http://dx.doi.org/10.1002/mgg3.1230DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7284036PMC

An intriguing case of post-operative respiratory failure from an occult diaphragmatic hernia- be aware of the masquerader.

Ann Card Anaesth 2020 Apr-Jun;23(2):237-240

Department of Cardiac Anaesthesia, Artemis Hospital, Sector 51, Gurugram, Haryana, India.

Though respiratory complications after cardiac surgery for congenital heart disease are common, and malformations of the diaphragm can be expected in these patients, the presence of an occult diaphragmatic defect unrecognisible preoperatively and complicating the post operative course is very rare and need a high index of suspicion for diagnosis in the setting of post operative respiratory failure. We present here a case of post operative respiratory failure from a delayed presenting diaphragmatic hernia in a 2-month-old boy who underwent corrective surgery for Taussig bing anomaly and hypoplastic aortic arch. Surgical repair of the diaphragmatic defect and reduction of the bowel loops to the abdomen resulted in rapid weaning from ventilation and recovery with subsequent discharge from hospital. Read More

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http://dx.doi.org/10.4103/aca.ACA_227_18DOI Listing

Risk factors affecting postoperative pulmonary function in congenital diaphragmatic hernia.

Ann Surg Treat Res 2020 Apr 31;98(4):206-213. Epub 2020 Mar 31.

Department of Pediatric Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Purpose: It is well known that congenital diaphragmatic hernia (CDH) in infants impacts pulmonary function rehabilitation after surgery. However, the risk factors of postoperative pulmonary function are still unclear. In this research, we analyzed the potential risk factors of postoperative pulmonary function in CDH patients in order to improve the clinical management of CDH patients. Read More

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http://dx.doi.org/10.4174/astr.2020.98.4.206DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7118320PMC

Postoperative Clinical Course and Opioid Consumption Following Repair of Congenital Diaphragmatic Hernia: Open Versus Thoracoscopic Techniques.

J Laparoendosc Adv Surg Tech A 2020 May 8;30(5):590-595. Epub 2020 Apr 8.

Department of Anesthesiology and Pain Medicine, Nationwide Children's Hospital, Columbus, Ohio, USA.

Minimally invasive surgical (MIS) approaches for thoracic procedures in adults result in an improved postoperative course with less pain, but there are limited data on similar procedures in neonates. We aimed to evaluate postoperative opioid consumption and pain management practices in neonates and infants following MIS versus open repair of congenital diaphragmatic hernia (CDH). This was an IRB approved, retrospective study from 2012 to 2016. Read More

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http://dx.doi.org/10.1089/lap.2019.0510DOI Listing

All for One and One Delivery Room Approach for All?

Pediatrics 2020 May 2;145(5). Epub 2020 Apr 2.

Bioethics Center, Children's Mercy Hospital, Kansas City, Missouri.

Multiple births are increasing in frequency related to advanced maternal age and fertility treatments, and they have an increased risk for congenital anomalies compared to singleton births. However, twins have the same congenital anomalies <15% of the time. Thus, having multiple births with discordant anomalies is a growing challenge for neonatologists. Read More

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http://dx.doi.org/10.1542/peds.2019-2704DOI Listing