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Pediatric Congenital Diaphragmatic Hernia

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Early Left Ventricular Dysfunction and Severe Pulmonary Hypertension Predict Adverse Outcomes in "Low-Risk" Congenital Diaphragmatic Hernia.

Duy T Dao, Neil Patel, Matthew T Harting, Kevin P Lally, Pamela A Lally, Terry L Buchmiller,

OBJECTIVES:Given significant focus on improving survival for "high-risk" congenital diaphragmatic hernia, there is the potential to overlook the need to identify risk factors for suboptimal outcomes in "low-risk" congenital diaphragmatic hernia cases. We hypothesized that early cardiac dysfunction or severe pulmonary hypertension were predictors of adverse outcomes in this "low-risk" ... Read more >>

Pediatr Crit Care Med (Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies)
[2020, :]

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Thoracoscopic Repair of Congenital Diaphragmatic Hernia.

Richard Wagner, Steffi Mayer, Xiaoyan Feng, Jan-Hendrik Gosemann, Peter Zimmermann, Martin Lacher,

The surgical management of patients with congenital diaphragmatic hernia (CDH) is challenging and ever changing. It requires the highest expertise not only on the surgical level but also of neonatologists and anesthesiologists. In selected patients traditional open surgery is increasingly replaced by thoracoscopic CDH repair in many centers worldwide. Despite ... Read more >>

Eur J Pediatr Surg (European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie)
[2020, :]

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Predictors of long ECMO runs for congenital diaphragmatic hernia.

Patrick T Delaplain, Peter T Yu, Louis Ehwerhemuepha, Danh V Nguyen, Tim Jancelewicz, James Stein, Matthew T Harting, Yigit S Guner,

BACKGROUND:Although longer ECMO run times for patients with congenital diaphragmatic hernia (CDH) have been associated with worse outcomes, a large study has not been conducted to examine the risk factors for long ECMO runs. METHODS:The Extracorporeal Life Support Organization (ELSO) Registry from 2000 to 2015 was used to identify predictors ... Read more >>

J. Pediatr. Surg. (Journal of pediatric surgery)
[2020, :]

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When primary repair is not enough: a comparison of synthetic patch and muscle flap closure in congenital diaphragmatic hernia?

Emrah Aydın, Heather Nolan, Jose Luis Peiró, Patricia Burns, Beth Rymeski, Foong-Yen Lim,

PURPOSE:Primary closure is often inadequate for large congenital diaphragmatic hernia (CDH) and necessitates repair by prosthetic patch or autologous muscle flap. Our aim was to evaluate outcomes of open patch versus flap repair, specifically diaphragmatic reherniation. METHODS:A retrospective review (IRB #2017-6361) was performed on all CDH patients repaired from 2005 ... Read more >>

Pediatr. Surg. Int. (Pediatric Surgery International)
[2020, 36(4):485-491]

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Use of treprostinil in pediatric pulmonary hypertension: case reports and review of the literature.

Arthur Gavotto, Fanny Thomas, Oscar Werner, Johan Moreau, Pascal Amedro,

BACKGROUND:Add-on therapy with prostacyclin in pediatric refractory pulmonary hypertension poses a challenge, especially when considering continuous intravenous administration in younger children. A search for alternate routes of drug delivery has led to the clinical investigation of stable and long-acting prostacyclin analogues, such as subcutaneous treprostinil. We reported two pediatric cases ... Read more >>

J. Cardiovasc. Pharmacol. (Journal of cardiovascular pharmacology)
[2020, :]

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Drug Treatment of Pulmonary Hypertension in Children.

Catherine M Avitabile, Erika E Vorhies, David Dunbar Ivy,

Pulmonary arterial hypertension (PAH) is a rare disease in infants and children that is associated with significant morbidity and mortality. The disease is characterized by progressive pulmonary vascular functional and structural changes resulting in increased pulmonary vascular resistance and eventual right heart failure and death. In many pediatric patients, PAH ... Read more >>

Paediatr Drugs (Paediatric drugs)
[2020, 22(2):123-147]

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Morgagni-Larrey diaphragmatic hernia repair in adult patients: a retrospective single-center experience.

P U Oppelt, I Askevold, F Bender, J Liese, W Padberg, A Hecker, M Reichert,

PURPOSE:Morgagni-Larrey congenital diaphragmatic hernia (MLH) is rare in adult patients and surgery is performed infrequently. The evidence regarding the most beneficial treatment modality is low. Nevertheless, with increasing experience in minimally-invasive surgery, the literature proves the laparoscopic approach as being safely feasible. However, knowledge on the disease as well as ... Read more >>

Hernia (Hernia : the journal of hernias and abdominal wall surgery)
[2020, :]

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Single-Center Outcome of Fetoscopic Tracheal Balloon Occlusion for Severe Congenital Diaphragmatic Hernia.

Ahmet A Baschat, Mara Rosner, Sarah E Millard, Jamie D Murphy, Karin J Blakemore, Amaris M Keiser, Jennifer Kearney, Janine Bullard, Lawrence M Nogee, Melania Bembea, Eric B Jelin, Jena L Miller,

OBJECTIVE:To assess feasibility and maternal and infant outcome after fetoscopic tracheal balloon occlusion in patients with severe congenital diaphragmatic hernia. METHODS:We conducted a prospective cohort study of fetuses with congenital diaphragmatic hernia and observed/expected lung/head ratio less than 30%. Eligible women had planned fetoscopic tracheal balloon occlusion at 26 0/7-29 ... Read more >>

Obstet Gynecol (Obstetrics and gynecology)
[2020, 135(3):511-521]

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Can circular RNAs be used as prenatal biomarkers for congenital diaphragmatic hernia?

Richard Wagner, Aruni Jha, Lojine Ayoub, Shana Kahnamoui, Daywin Patel, Thomas H Mahood, Andrew J Halayko, Martin Lacher, Christopher D Pascoe, Richard Keijzer,

Eur. Respir. J. (The European respiratory journal)
[2020, 55(2):]

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Validation of a Prediction Rule for Mortality in Congenital Diaphragmatic Hernia.

Suzan C M Cochius-den Otter, Özge Erdem, Joost van Rosmalen, Thomas Schaible, Nina C J Peters, Titia E Cohen-Overbeek, Irma Capolupo, Carolin J Falk, Arno F J van Heijst, Regina Schäffelder, Mary E Brindle, Dick Tibboel,

BACKGROUND:Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly with a mortality of ∼27%. The Congenital Diaphragmatic Hernia Study Group (CDHSG) developed a simple postnatal clinical prediction rule to predict mortality in newborns with CDH. Our aim for this study is to externally validate the CDHSG rule in the European ... Read more >>

Pediatrics (Pediatrics)
[2020, :]

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Congenital diaphragmatic hernia: current management strategies from antenatal diagnosis to long-term follow-up.

Eimear Kirby, Richard Keijzer,

Congenital diaphragmatic hernia (CDH) is a developmental birth defect consisting of a diaphragmatic defect and abnormal lung development. CDH complicates 2.3-2.8 per 10,000 live births. Despite efforts to standardize clinical practice, management of CDH remains challenging. Frequent re-evaluation of clinical practices in CDH reveals that management of CDH is evolving ... Read more >>

Pediatr. Surg. Int. (Pediatric surgery international)
[2020, 36(4):415-429]

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Standardizing congenital diaphragmatic hernia care in Canada: Implementing national clinical practice guidelines.

Kathryn LaRusso, Robert Baird, Richard Keijzer, Erik Skarsgard, Pramod Puligandla,

PURPOSE:We sought to identify implementation barriers and opportunities to increase utilization of the Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative's clinical practice guideline. METHODS:A validated readiness assessment was sent via SurveyMonkey™ to CAPSNet site coordinators and local CDH stakeholders. The survey was open from 11/2018 to 02/2019. Data and responses were ... Read more >>

J. Pediatr. Surg. (Journal of pediatric surgery)
[2020, :]

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Long-term follow-up in surgical newborns: A single-institution experience.

Hajime Takayasu, Kouji Masumoto, Takato Sasaki, Fumiko Chiba, Kentaro Ono, Chikashi Gotoh, Yasuhisa Urita, Toko Shinkai,

BACKGROUND:To assess the actual experiences of long-term follow-up and discuss ways to improve care during the period from childhood to adulthood in newborns who have undergone surgery. METHODS:A total of 306 patients with congenital anomalies requiring newborn surgery who survived to discharge from 1994 to 2013 were eligible for inclusion. ... Read more >>

Asian J Surg (Asian journal of surgery)
[2020, :]

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Therapy at 30 days of life predicts lung function at 6 to 12 months in infants with congenital diaphragmatic hernia.

Etze Chotzoglou, Holly L Hedrick, Lisa M Herkert, Matthew A Goldshore, Natalie E Rintoul, Howard B Panitch,

INTRODUCTION:Congenital diaphragmatic hernia (CDH) is associated with variable degrees of lung hypoplasia. Pulmonary support at 30 days postnatal age was found to be the strongest predictor of inpatient mortality and morbidity among CDH infants and was also associated with higher pulmonary morbidity at 1 and 5 years. It is not ... Read more >>

Pediatr. Pulmonol. (Pediatric pulmonology)
[2020, :]

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Impact of consolidation of cases on post-operative outcomes for index pediatric surgery cases.

Liese C C Pruitt, David E Skarda, Douglas C Barnhart, Brian T Bucher,

BACKGROUND:The effect of the consolidation of neonatal pediatric surgical cases to limited surgeons within a hospital is unknown. We elected to model the distribution of complex neonatal procedures using an economic measure of market concentration, the Herfindahl-Hirschmann Index (HHI), and study its effect on outcomes of index pediatric surgical operations. ... Read more >>

J. Pediatr. Surg. (Journal of pediatric surgery)
[2020, :]

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Congenital hypothyroidism, cardiac defects, and pancreatic agenesis in an infant with GATA6 mutation.

Nikhil Raghuram, Ashish Marwaha, Mary-Louise C Greer, Estelle Gauda, David Chitayat,

GATA6 pathogenic variants primarily manifest a phenotype with pancreatic agenesis and cardiac malformations. However, additional congenital malformations affecting the biliary system, congenital diaphragmatic hernia and developmental delay have been reported. We report a newborn, prenatally diagnosed with truncus arteriosus and intrauterine growth restriction, who was postnatally found to have pancreatic ... Read more >>

Am. J. Med. Genet. A (American journal of medical genetics. Part A)
[2020, :]

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Heterogeneous Response in Rabbit Fetal Diaphragmatic Hernia Lungs After Tracheal Occlusion.

Evgenia Dobrinskikh, Saif I Al-Juboori, Marc Oria, Julie A Reisz, Connie Zheng, Jose L Peiro, Ahmed I Marwan,

BACKGROUND:Fetal tracheal occlusion (TO) is an experimental therapeutic approach to stimulate lung growth in the most severe congenital diaphragmatic hernia (CDH) cases. We have previously demonstrated a heterogeneous response of normal fetal rabbit lungs after TO with the appearance of at least two distinct zones. The aim of this study ... Read more >>

J. Surg. Res. (The Journal of surgical research)
[2020, 250:23-38]

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Prostaglandin E1 in infants with congenital diaphragmatic hernia (CDH) and life-threatening pulmonary hypertension.

Kévin Le Duc, Sébastien Mur, Dyuti Sharma, Estelle Aubry, Morgan Recher, Thameur Rakza, Laurent Storme, ,

BACKGROUND:Suprasystemic pulmonary hypertension (PH) is highly predictive of pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH). OBJECTIVES:To report the effects and tolerability of Prostaglandin E1 (PGE1) in newborns with severe CDH and life-threatening PH. METHODS:Newborn infants with isolated CDH and life-threatening PH defined by an acute worsening ... Read more >>

J. Pediatr. Surg. (Journal of pediatric surgery)
[2020, :]

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Left-sided congenital diaphragmatic hernia: can we improve survival while decreasing ECMO?

Michelle J Yang, Stephen Fenton, Katie Russell, Christian Con Yost, Bradley A Yoder,

BACKGROUND:Mortality and ECMO rates for congenital diaphragmatic hernia (CDH) remain ~30%. In 2016, we changed our CDH guidelines to minimize stimulation while relying on preductal oxygen saturation, lower mean airway pressures, stricter criteria for nitric oxide (iNO), and inotrope use. We compared rates of ECMO, survival, and survival without ECMO ... Read more >>

J Perinatol (Journal of perinatology : official journal of the California Perinatal Association)
[2020, :]

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Survival of infants with congenital diaphragmatic hernia in California: impact of hospital, clinical, and sociodemographic factors.

Suzan L Carmichael, Chen Ma, Henry C Lee, Gary M Shaw, Karl G Sylvester, Susan R Hintz,

OBJECTIVE:To understand factors associated with care and survival among babies with congenital diaphragmatic hernia (CDH). STUDY DESIGN:We used data on California births (2006-2011) to examine birth hospital level of care, hospital transfer before repair, and survival. RESULT:Among 577 infants, 25% were born at lower-level hospitals, 62% were transferred, and 31% ... Read more >>

J Perinatol (Journal of perinatology : official journal of the California Perinatal Association)
[2020, :]

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Sex differences in surgically correctable congenital anomalies: A systematic review.

Anna J Black, Daphne Yd Lu, Leeor S Yefet, Robert Baird,

PURPOSE:This study aims to compare the prevalence and outcomes of surgically correctable congenital anomalies between sexes. METHODS:Upon registration on PROSPERO (CRD42019120165), a librarian aided in conducting a systematic review using PRISMA guidelines. The five largest relevant studies were included for each anomaly. Cumulative prevalence differences and confidence intervals were calculated, ... Read more >>

J. Pediatr. Surg. (Journal of pediatric surgery)
[2020, :]

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Implications of Atrial-Level Shunting by Echocardiography in Newborns with Congenital Diaphragmatic Hernia.

Melissa Wehrmann, Sonali S Patel, Caitlin Haxel, Courtney Cassidy, Lisa Howley, Bettina Cuneo, Jason Gien, John P Kinsella,

OBJECTIVE:To determine whether postnatal echocardiographic indices of left ventricular (LV) size and function are associated with atrial-level shunt direction and investigate whether a relationship exists between LV function and LV size in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN:This was a single-center retrospective study of 51 neonates with CDH ... Read more >>

J. Pediatr. (The Journal of pediatrics)
[2020, 219:43-47]

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Prenatal Diagnosis and Management of a Rare Central Tendon Defect Type of Congenital Diaphragmatic Hernia with a Massive Pericardial Effusion.

Inas Babic, Haifa Al-Jobair, Osama Al Towaijri, Huda Al-Shammary, Merna Atiyah, Jamal Al Hudhaif, Amer Ammari,

The central tendon defect type of congenital diaphragmatic hernia (CDH) is extremely rare and usually associated with a significant pericardial effusion. Prenatal diagnostic ultrasound features of this quite rare entity remain often overlooked or misdiagnosed. There is a dearth of literature about the role of prenatal intervention, often through an ... Read more >>

Case Rep Obstet Gynecol (Case reports in obstetrics and gynecology)
[2020, 2020:6798253]

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Incidence of congenital thoracic malformations detected by prenatal ultrasound.

Mordechai Pollak, Michal Gur, Moshe Bronshtein, Ido Solt, Kamal Masarweh, Lea Bentur,

BACKGROUND:The increased and earlier use of prenatal ultrasound has facilitated the detection of congenital thoracic malformations (CTMs). Our Pediatric Pulmonology Institute follows an increasing number of patients with CTMs. Thus, we aimed to evaluate and describe prenatal sonographic findings of CTM, and to estimate changes in detection rates of CTMs ... Read more >>

Pediatr Int (Pediatrics international : official journal of the Japan Pediatric Society)
[2020, 62(1):89-93]

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Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study.

Kim Heiwegen, Arno Fj van Heijst, Horst Daniels-Scharbatke, Michelle Cp van Peperstraten, Ivo de Blaauw, Sanne Mbi Botden,

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and ... Read more >>

Eur. J. Pediatr. (European journal of pediatrics)
[2020, :]

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