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Hernia diafragmatica

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21 papers 0 to 25 followers
J Gien, J P Kinsella
In infants with congenital diaphragmatic hernia (CDH), a posterolateral diaphragmatic defect results in herniation of abdominal contents into the chest and compression of the intrathoracic structures. In the most severe cases, hypoplasia of the ipsilateral and contralateral lungs, severe pulmonary hypertension (PH) and left ventricular (LV) hypoplasia/dysfunction all contribute to increased mortality. The management of PH in CDH is complicated by structural and functional changes in the heart, pulmonary vasculature, airways and lung parenchyma; consequently, determining optimal management strategies is challenging...
June 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
Adesola C Akinkuotu, Stephanie M Cruz, Darrell L Cass, Christopher I Cassady, Amy R Mehollin-Ray, Jennifer L Williams, Timothy C Lee, Rodrigo Ruano, Stephen E Welty, Oluyinka O Olutoye
BACKGROUND: Studies comparing outcomes of right- and left-sided congenital diaphragmatic hernia (R-CDH and L-CDH) have yielded conflicting results. We hypothesized that R-CDH is associated with higher short-term pulmonary morbidity than L-CDH. METHODS: We reviewed all CDH patients at a tertiary children's hospital over 10 y. In prenatally diagnosed CDH, the observed-to-expected total fetal lung volume and percentage liver herniation (%LH) were calculated using fetal magnetic resonance imaging-based measurements...
October 2015: Journal of Surgical Research
Adesola C Akinkuotu, Fariha Sheikh, Darrell L Cass, Irving J Zamora, Timothy C Lee, Christopher I Cassady, Amy R Mehollin-Ray, Jennifer L Williams, Rodrigo Ruano, Stephen E Welty, Oluyinka O Olutoye
INTRODUCTION: Patients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology. METHODS: Records of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated...
January 2015: Journal of Pediatric Surgery
Enrico Danzer, Holly L Hedrick
Despite years of progress in perinatal care, severe congenital diaphragmatic hernia (CDH) remains a clinical challenge. Controversies include almost every facet of clinical care: the definition of severe CDH by prenatal and postnatal criteria, fetal surgical intervention, ventilator management, pulmonary hypertension management, use of extracorporeal membrane oxygenation, surgical considerations, and long-term follow-up. Breakthroughs are likely only possible by sharing of experience, collaboration between institutions and innovative therapies within well-designed multicenter clinical trials...
December 2014: Seminars in Fetal & Neonatal Medicine
Leslie A Lusk, Katherine C Wai, Anita J Moon-Grady, Martina A Steurer, Roberta L Keller
OBJECTIVES: To describe the natural history of pulmonary hypertension (PH) and the risk of death and pulmonary morbidity associated with the persistence of PH through the neonatal hospitalization for these infants. STUDY DESIGN: We performed a retrospective cohort study of infants with congenital diaphragmatic hernia (CDH) cared for at University of California San Francisco (2002-2012). Infants with other major anomalies or syndromes were excluded (n = 43). Clinical echocardiograms were performed weekly for up to 6 weeks or until PH resolved off respiratory support or until hospital discharge...
February 2015: Journal of Pediatrics
Mark R McGivern, Kate E Best, Judith Rankin, Diana Wellesley, Ruth Greenlees, Marie-Claude Addor, Larraitz Arriola, Hermien de Walle, Ingeborg Barisic, Judit Beres, Fabrizio Bianchi, Elisa Calzolari, Berenice Doray, Elizabeth S Draper, Ester Garne, Miriam Gatt, Martin Haeusler, Babak Khoshnood, Kari Klungsoyr, Anna Latos-Bielenska, Mary O'Mahony, Paula Braz, Bob McDonnell, Carmel Mullaney, Vera Nelen, Anette Queisser-Luft, Hanitra Randrianaivo, Anke Rissmann, Catherine Rounding, Antonin Sipek, Rosie Thompson, David Tucker, Wladimir Wertelecki, Carmen Martos
INTRODUCTION: Published prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of Congenital Anomalies (EUROCAT). METHODS: Cases of CDH delivered between 1980 and 2009 notified to 31 EUROCAT registers formed the population-based case series. Prevalence over time was estimated using multilevel Poisson regression, and heterogeneity between registers was evaluated from the random component of the intercept...
March 2015: Archives of Disease in Childhood. Fetal and Neonatal Edition
Kazuki Yokota, Hiroo Uchida, Kenichiro Kaneko, Yasuyuki Ono, Naruhiko Murase, Satoshi Makita, Masahiro Hayakawa
PURPOSE: This study aimed to characterize the surgical complications, especially gastroesophageal reflux disease (GERD), intestinal adhesion obstruction (IAO), and diaphragmatic hernia recurrence, in patients with congenital diaphragmatic hernia (CDH). METHODS: Between January 1995 and December 2013, we determined the incidence of surgical complications and their predictors in CDH patients. We also examined whether the CDH repair and patch closure were associated with the incidence of IAO and the severity of adhesion...
September 2014: Pediatric Surgery International
Mary Elizabeth Brindle, Earl Francis Cook, Dick Tibboel, Pamela A Lally, Kevin P Lally
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm...
August 2014: Pediatrics
Kevin P Lally, Robert E Lasky, Pamela A Lally, Pietro Bagolan, Carl F Davis, Bjorn P Frenckner, Ronald M Hirschl, Max R Langham, Terry L Buchmiller, Noriaki Usui, Dick Tibboel, Jay M Wilson
BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH. METHODS: Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system...
December 2013: Journal of Pediatric Surgery
Ryan P Cauley, Alexander Stoffan, Kristina Potanos, Nora Fullington, Dionne A Graham, Jonathan A Finkelstein, Heung Bae Kim, Jay M Wilson
PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with significant in-hospital mortality, morbidity and length-of-stay (LOS). We hypothesized that the degree of pulmonary support on hospital day-30 may predict in-hospital mortality, LOS, and discharge oxygen needs and could be useful for risk prediction and counseling. METHODS: 862 patients in the CDH Study Group registry with a LOS ≥ 30 days were analyzed (2007-2010). Pulmonary support was defined as (1) room-air (n=320) (2) noninvasive supplementation (n=244) (3) mechanical ventilation (n=279) and (4) extracorporeal membrane oxygenation (ECMO, n=19)...
June 2013: Journal of Pediatric Surgery
John J Greer
This review outlines research that has advanced our understanding of the pathogenesis and etiology of congenital diaphragmatic hernia (CDH). The majority of CDH cases involve incomplete formation of the posterolateral portion of the diaphragm, clinically referred to as a Bochdalek hernia. The hole in the diaphragm allows the abdominal viscera to invade the thoracic cavity, thereby impeding normal lung development. As a result, newborns with CDH suffer from a combination of severe pulmonary hypoplasia and pulmonary hypertension...
November 1, 2013: Respiratory Physiology & Neurobiology
Masahiro Hayakawa, Miharu Ito, Tetsuo Hattori, Yutaka Kanamori, Hiroomi Okuyama, Noboru Inamura, Shigehiro Takahashi, Kouji Nagata, Tomoaki Taguchi, Noriaki Usui
BACKGROUND: During the last decade, new supportive modalities and new therapeutic strategies to treat congenital diaphragmatic hernia (CDH) have been introduced. In Japan, the large number of hospitals prevents centralizing infants with CDH in tertiary centers. The aim of this study was to evaluate the correlations between the number of CDH patients, survival rates, and the current strategies employed to treat CDH at the individual hospitals. METHODS: Eighty-three hospitals with 674 CDH cases were analyzed using questionnaires...
April 2013: Pediatrics International: Official Journal of the Japan Pediatric Society
Shaji C Menon, Lloyd Y Tani, Hsin Yi Weng, Pamela A Lally, Kevin P Lally, Bradley A Yoder
OBJECTIVE: To evaluate the impact of associated heart defects on outcomes to discharge, and identify factors affecting survival of all infants born with congenital diaphragmatic hernia (CDH) in last decade using Congenital Diaphragmatic Hernia Study Group data. STUDY DESIGN: This was a retrospective review of all infants with CDH enrolled in Congenital Diaphragmatic Hernia Study Group database from January 2000 to December 2010. The study cohort was divided into 3 groups (GRP): GRP 1, CDH with major heart defects; GRP 2, CDH with minor heart defects; and GRP 3, CDH with no reported heart defects...
January 2013: Journal of Pediatrics
Neil Patel
BACKGROUND: Pulmonary hypertension and secondary cardiac dysfunction are important contributors of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Milrinone, a phosphodiesterase-3 inhibitor, may be useful in this setting for its combined actions as a pulmonary vasodilator and to improve systolic and diastolic function. OBJECTIVES: This study aimed to assess the effects of milrinone on cardiac function and pulmonary artery pressure in infants with CDH...
2012: Neonatology
R Cruz-Martinez, M Castañon, O Moreno-Alvarez, R Acosta-Rojas, J M Martinez, E Gratacos
OBJECTIVE: To explore the potential value of intrapulmonary artery Doppler velocimetry in predicting neonatal morbidity in fetuses with left-sided congenital diaphragmatic hernia (CDH) treated with fetoscopic tracheal occlusion (FETO). METHODS: Observed/expected lung-to-head ratio (O/E-LHR), and intrapulmonary Doppler pulsatility index and peak early-diastolic reversed flow were evaluated within 24 h before FETO in a consecutive cohort of 51 fetuses with left-sided CDH at between 24 and 33 weeks' gestation...
January 2013: Ultrasound in Obstetrics & Gynecology
D C M Veenma, A de Klein, D Tibboel
Congenital diaphragmatic hernia (CDH) is a frequent occurring cause of neonatal respiratory distress and occurs 1 in every 3,000 liveborns. Ventilatory support and pharmaceutical treatment of the co-occurring lung hypoplasia and pulmonary hypertension are insufficient in, respectively, 20% of isolated cases and 60% of complex ones leading to early perinatal death. The exact cause of CDH remains to be identified in the majority of human CDH patients and prognostic factors predicting treatment refraction are largely unknown...
June 2012: Pediatric Pulmonology
Marjolein Spoel, Monique H M van der Cammen-van Zijp, Wim C J Hop, Dick Tibboel, Johan C de Jongste, Hanneke Ijsselstijn
BACKGROUND: Survival rates of patients with congenital diaphragmatic hernia (CDH) have improved to up to 80%. Little is known about long-term consequences of the disease and its treatment. We evaluated lung function and respiratory symptoms longitudinally in a previously studied cohort of CDH patients and age-matched non-CDH patients who underwent similar neonatal intensive care treatment. STUDY DESIGN: We tested 27 young adults [mean (SD) age: 26.8 years (2.9)] with CDH and 30 non-CDH patients...
February 2013: Pediatric Pulmonology
Christophe Delacourt, Alice Hadchouel, Jaan Toelen, Maissa Rayyan, Jacques de Blic, Jan Deprest
Intrathoracic congenital malformations may be associated with long-term pulmonary morbidity. This certainly is the case for congenital diaphragmatic hernia, esophageal atresia and cardiac and aortic arch abnormalities. These conditions have variable degrees of impaired development of both the airways and lung vasculature, with a postnatal impact on lung function and bronchial reactivity. Pulmonary complications are themselves frequently associated to non-pulmonary morbidities, including gastrointestinal and orthopaedic complications...
April 2012: Seminars in Fetal & Neonatal Medicine
Nikesh R Lath, Csaba Galambos, Alejandro Best Rocha, Marcus Malek, George K Gittes, Douglas A Potoka
Congenital diaphragmatic hernia (CDH) is associated with significant mortality due to lung hypoplasia and pulmonary hypertension. The role of embryonic pulmonary innervation in normal lung development and lung maldevelopment in CDH has not been defined. We hypothesize that developmental defects of intrapulmonary innervation, in particular autonomic innervation, occur in CDH. This abnormal embryonic pulmonary innervation may contribute to lung developmental defects and postnatal physiological derangement in CDH...
February 15, 2012: American Journal of Physiology. Lung Cellular and Molecular Physiology
S Kotecha, A Barbato, A Bush, F Claus, M Davenport, C Delacourt, J Deprest, E Eber, B Frenckner, A Greenough, A G Nicholson, J L Antón-Pacheco, F Midulla
Infants with congenital diaphragmatic hernia (CDH) have significant mortality and long-term morbidity. Only 60-70% survive and usually those in high-volume centres. The current Task Force, therefore, has convened experts to evaluate the current literature and make recommendations on both the antenatal and post-natal management of CDH. The incidence of CDH varies from 1.7 to 5.7 per 10,000 live-born infants depending on the study population. Antenatal ultrasound scanning is routine and increasingly complemented by the use of magnetic resonance imaging...
April 2012: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
2016-07-03 16:58:06
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