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Congenital diaphragmatic hernia

Jessica H Rankin, Marsha Elkhunovich, Dina Seif, Mikaela Chilstrom
Neonatal respiratory distress is an emergent condition with a wide differential diagnosis. A 12-day-old newborn presented to the emergency department in respiratory distress. Point-of-care ultrasound allowed clinicians to rapidly exclude cardiac disease and pneumothorax as possible causes of the patient's respiratory distress, and expedited the identification of congenital diaphragmatic hernia. The ultrasound findings and technique, epidemiology, pathophysiology, and radiological diagnosis of congenital diaphragmatic hernia are reviewed...
October 2016: Pediatric Emergency Care
Nazan Ülgen-Tekerek, Başak Akyıldız, Alper Özcan, Ayşe Betül Ergül, Cüneyt Turan, Serdal Özcan
Late presentation of congenital diaphragmatic hernia is 5-30% of all congenital diaphragma hernia cases. It can present as Morgagni, Bochdalek and paraesophageal hernia. Misdiagnosis can result in significant morbiditiy. A 17-month-old girl presented with vomiting and abdominal pain. On physical examination, circulatory disturbance, cyanosis, abdominal distantion were present. Her O2 saturation was 60% and she was tachycardic (180 bpm) and tachypneic (58 bpm) with hypotension (60/35 mmhg). Patient's heart and mediastinum were shifted into the right hemithorax on the chest X-ray...
November 2015: Turkish Journal of Pediatrics
Wan-Ting Hung, Shu-Chien Huang, Dania El Mazloum, Wen-Hsi Lin, Hui-Hsin Yang, Hung-Chieh Chou, En-Ting Wu, Chien-Yi Chen, Po-Nien Tsao, Wu-Shiun Hsieh, Wen-Ming Hsu, Yih-Sharng Chen, Hong-Shiee Lai
BACKGROUND/PURPOSE: Extracorporeal membrane oxygenation (ECMO) is a treatment option for stabilizing neonates with congenital diaphragmatic hernia (CDH) in a critical condition when standard therapy fails. However, the use of this approach in Taiwan has not been previously reported. METHODS: The charts of all neonates with CDH treated in our institute during the period 2007-2014 were reviewed. After 2010, patients who could not be stabilized with conventional treatment were candidates for ECMO...
October 7, 2016: Journal of the Formosan Medical Association, Taiwan Yi Zhi
Luke R Putnam, Kuojen Tsao, Francesco Morini, Pamela A Lally, Charles C Miller, Kevin P Lally, Matthew T Harting
Importance: Inhaled nitric oxide (iNO) is an expensive, commonly used therapy among patients with congenital diaphragmatic hernia (CDH); however, data to support its ongoing use in this patient population are lacking. Objective: To describe the spectrum of iNO use among patients with CDH and its association with pulmonary hypertension (pHTN) and mortality. Design, Setting, and Participants: A review was conducted of prospectively collected patient data in the Congenital Diaphragmatic Hernia Study Group registry between January 1, 2007, and December 31, 2014, from 70 participating centers in 13 countries...
October 10, 2016: JAMA Pediatrics
Catherine Fox, Michael Stewart, Sebastian K King, Neil Patel
BACKGROUND: Congenital diaphragmatic hernia (CDH) affects 1 in 3000 live births. Modern management strategies include delayed repair of the diaphragm to permit pre-operative optimization of cardiorespiratory status. We describe a cohort of neonates in whom early emergency operative intervention was required for potentially fatal intestinal compromise. METHODS: A retrospective review was performed of all neonatal CDH patients managed at a tertiary center in an 8-year period (2005-2012)...
September 15, 2016: Journal of Pediatric Surgery
R Sahni, X Ameer, K Ohira-Kist, J-T Wung
OBJECTIVES: Inhaled nitric oxide (iNO) is effective in conjunction with tracheal intubation (TI) and mechanical ventilation (MV) for treating arterial pulmonary hypertension and hypoxemic respiratory failure (HRF) in near-term and term newborns. Non-invasive respiratory support with nasal continuous positive airway pressure (CPAP) is increasingly used to avoid morbidity associated with TI and MV, yet the effectiveness of iNO delivery via nasal CPAP remains unknown. To evaluate the effectiveness of iNO delivered via the bubble nasal CPAP system in term and preterm newborns with HRF...
October 6, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
Maria Esther J R Ceccon, Renata A Vieira, Werther Brunow de Carvalho
No abstract text is available yet for this article.
October 2016: Pediatric Critical Care Medicine
Nicholas E Bruns, Ian C Glenn, Neil L McNinch, Kelly Arps, Todd A Ponsky, Avraham Schlager
PURPOSE: Although recurrence remains one of the most feared complications following congenital diaphragmatic hernia (CDH) repair, there are minimal data on the optimal surgical approach to these complex situations. The purpose of this study was to survey the international pediatric surgery community to ascertain practice patterns for both minimally invasive (MIS) and open approaches for recurrent CDH. MATERIALS AND METHODS: A survey was e-mailed to members of an online community of pediatric surgeons...
October 5, 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
María Paz Bidondo, Boris Groisman, Agostina Tardivo, Fabián Tomasoni, Verónica Tejeiro, Inés Camacho, Mariana Vilas, Rosa Liascovich, Pablo Barbero
BACKGROUND: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. METHODS: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). RESULTS: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12-29)...
October 5, 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
Noemi Cantone, Caterina Gulia, Vittorio Miele, Margherita Trinci, Vito Briganti
Wandering spleen and gastric volvulus are two rare entities that have been described in association with congenital diaphragmatic hernia. The diagnosis is difficult and any delay can result in ischemia and necrosis of both organs. We present a case of a 13-year-old girl, previously operated on for anterior diaphragmatic hernia and intrathoracic gastric volvulus, that presented to our service for a subdiaphragmatic gastric volvulus recurrence associated with a wandering spleen. In this report we reviewed the literature, analyzing the clinical presentation, diagnostic assessment, and treatment options of both conditions, in particular in the case associated with diaphragmatic hernia...
2016: Case Reports in Surgery
Kartik Chandra Mandal, Pankaj Halder, Shibsankar Barman, Rajarshi Kumar, Biswanath Mukhopadhyay, Ram Mohan Shukla
AIMS: To assess the role of measuring of intragastric pressure (IGP) during closure of congenital diaphragmatic hernia. MATERIALS AND METHODS: Thirty-two cases were included in the study (August 2008 to February 2014), and IGP has been measured intraoperatively during closure. RESULT: Patients were categorized in group A (15 patients), group B (14 patients) and group C (3 patients). In group C, IGP was more than 30 cm of water pressure and all of tehm required creation of ventral hernia and ventilator support...
October 2016: Journal of Indian Association of Pediatric Surgeons
Marta Unolt, Lauren DiCairano, Kathryn Schlechtweg, Jessica Barry, Lori Howell, Stefanie Kasperski, Michael Nance, N Scott Adzick, Elaine H Zackai, Donna M McDonald-McGinn
We report the important association of congenital diaphragmatic hernia (CDH) and 22q11.2 deletion syndrome (22q11.2DS). The prevalence of CDH in our cohort of patients with 22q11.2DS is 0.8% (10/1246), which is greater than in the general population (0.025%). This observation suggests that 22q11.2DS should be considered when a child or fetus presents with CDH, in particular when other clinical findings associated with the 22q11.2DS are present, such as congenital cardiac defects. Furthermore, this finding may lead to the identification of an additional locus for diaphragmatic hernia in the general population...
September 28, 2016: American Journal of Medical Genetics. Part A
Toshiaki Takahashi, Julia Zimmer, Florian Friedmacher, Prem Puri
BACKGROUND/PURPOSE: Pleuroperitoneal folds (PPFs) are the source of the primordial diaphragm's muscle connective tissue (MCT), and developmental mutations have been shown to result in congenital diaphragmatic hernia (CDH). The protein paired-related homeobox 1 (Prx1) labels migrating PPF cells and stimulates expression of transcription factor 4 (Tcf4), a novel MCT marker that controls morphogenesis of the fetal diaphragm. We hypothesized that diaphragmatic Prx1 and Tcf4 expression is decreased in the nitrofen-induced CDH model...
September 15, 2016: Journal of Pediatric Surgery
J Zimmer, T Takahashi, A D Hofmann, Prem Puri
PURPOSE: High mortality and morbidity in infants born with congenital diaphragmatic hernia (CDH) are attributed to pulmonary hypoplasia and pulmonary hypertension (PH). Forkhead box (Fox) transcription factors are known to be crucial for cell proliferation and homeostasis. FoxF1 is essential for lung morphogenesis, vascular development, and endothelial proliferation. Mutations in FoxF1 and also the Fox family member FoxC2 have been identified in neonates with PH. In human and experimental models of arterial PH, the Fox protein FoxO1 was found to be downregulated...
September 23, 2016: Pediatric Surgery International
Tara A Morgan, Amaya Basta, Roy A Filly
PURPOSE: The aim of this study was to identify sonographic (US) findings that can assist in prenatal diagnosis of stomach-down left congenital diaphragmatic hernia (CDH), specifically related to positioning of the abdominal contents including the stomach, bladder, and gallbladder. METHODS: All US examinations with a postnatally confirmed diagnosis of stomach-down left CDH over a 13-year period were retrospectively reviewed for abnormal position of the abdominal contents, including whether the fetal stomach was in contact with the urinary bladder...
September 24, 2016: Journal of Clinical Ultrasound: JCU
Masaya Yamoto, Noboru Inamura, Keita Terui, Kouji Nagata, Yutaka Kanamori, Masahiro Hayakawa, Yuko Tazuke, Akiko Yokoi, Hajime Takayasu, Hiroomi Okuyama, Koji Fukumoto, Naoto Urushihara, Tomoaki Taguchi, Noriaki Usui
BACKGROUND/PURPOSE: The purpose of this study was to investigate echocardiographic parameters in relation to the outcomes of isolated left-sided congenital diaphragmatic hernia (CDH). METHODS: This multicenter, retrospective, observational study was conducted among patients with CDH born between 2006 and 2010. Patients in this study did not have severe cardiac malformations or chromosomal aberrations. Patients with incomplete echocardiographic examinations were excluded...
September 15, 2016: Journal of Pediatric Surgery
Toshiaki Takahashi, Florian Friedmacher, Julia Zimmer, Prem Puri
PURPOSE: Congenital diaphragmatic hernia (CDH) is presumed to originate from defects in the primordial diaphragmatic mesenchyme, mainly comprising of muscle connective tissue (MCT). Thus, normal diaphragmatic morphogenesis depends on the structural integrity of the underlying MCT. Developmental mutations that inhibit normal formation of diaphragmatic MCT have been shown to result in CDH. Desmin (DES) is a major filament protein in the MCT, which is essential for the tensile strength of the developing diaphragm muscle...
September 20, 2016: Pediatric Surgery International
Edward Araujo Júnior, Gabriele Tonni, Wellington P Martins, Rodrigo Ruano
No abstract text is available yet for this article.
September 20, 2016: European Journal of Pediatric Surgery
Ian S Reynolds, Peter Coyle, Michael Murray, Ronan Waldron, Kevin Barry
Diaphragmatic hernias are typically congenital or caused by trauma. Delayed presentation is not uncommon. Repair can be undertaken through an open or minimally invasive abdominal or thoracic approach. Small defects can be repaired with sutures, while larger defects require a mesh repair. This patient's comorbidities precluded him from surgery.
September 2016: Clinical Case Reports
Rita Carmona, Ana Cañete, Elena Cano, Laura Ariza, Anabel Rojas, Ramon Muñoz-Chápuli
Congenital diaphragmatic hernia (CDH) is a severe birth defect. Wt1-null mouse embryos develop CDH but the mechanisms regulated by WT1 are unknown. We have generated a murine model with conditional deletion of WT1 in the lateral plate mesoderm, using the G2 enhancer of the Gata4 gene as a driver. 80% of G2-Gata4(Cre);Wt1(fl/fl) embryos developed typical Bochdalek-type CDH. We show that the posthepatic mesenchymal plate coelomic epithelium gives rise to a mesenchyme that populates the pleuroperitoneal folds isolating the pleural cavities before the migration of the somitic myoblasts...
2016: ELife
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