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Congenital hernia

Katarina Bojanić, Ruža Grizelj, Jurica Vuković, Lana Omerza, Marina Grubić, Tomislav Ćaleta, Toby N Weingarten, Darrell R Schroeder, Juraj Sprung
BACKGROUND: Patients with congenital diaphragmatic hernia (CDH) have a high residual morbidity rate. We compared self-reported health-related quality of life (HRQoL) between patients with CDH and healthy children. METHODS: Forty-five patients with CDH who were born from January 1, 1990, through February 15, 2015, were matched to healthy, age-matched control participants at a 1:2 ratio. The health records of the study participants were reviewed to determine comorbid conditions, and HRQoL was assessed by both the participants and their parents with the Pediatric Quality of Life Inventory (PedsQL)...
March 14, 2018: Health and Quality of Life Outcomes
Joanna L Kaplan, Catherine T Gunther-Harrington, Jessie S Sutton, Joshua A Stern
BACKGROUND: The teratogenic effects of immunomodulatory and certain antimicrobial therapies are described in small rodents and humans. While the described teratogenic effects in small rodents have been extrapolated to make conclusions about its use in the pregnant dam, teratogenic effects of prednisone and doxycycline have not yet been reported in the dog. Here we report and describe midline defects observed in a litter of golden retriever puppies exposed to mid-gestational immunosuppressive and antimicrobial therapy...
March 12, 2018: BMC Veterinary Research
Solina Tith, Garinder Bining, Laurent Bollag
Background : Opioid use during pregnancy is a growing concern in the United States. Buprenorphine has been recommended by "The American College of Obstetrics and Gynecology" as an alternative to methadone to decrease risks associated with the use of illicit opioids during pregnancy. The partial μ-opioid agonists' unique pharmacology, including its long half time and high affinity to the μ-opioid receptor, complicates patient management in a highly kinetic, and often urgent field like obstetric anesthesia...
2018: F1000Research
Gabriel Altit, Shazia Bhombal, Krisa Van Meurs, Theresa A Tacy
INTRODUCTION: Newborns with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia and pulmonary hypertension (PH), and there is limited evidence that cardiac dysfunction is present. We sought to study early neonatal biventricular function and performance in these patients by reviewing early post-natal echocardiography (ECHO) measurements and comparing them to normal term newborns. METHODS: Retrospective case-control study reviewing clinical and ECHO data on term newborns with CDH and normal controls born between 2009 and 2016...
March 9, 2018: Pediatric Cardiology
Serpil Sancar, Esra Özçakır, Mete Kaya
Objective: Persistent Müllerian duct syndrome (PMDS) is a rare congenital disease characterized by the presence of rudimentary Müllerian structures within an intra-abdominal or hernial sac in a virilized male, often presenting as undescended testes. In this study, we aim to present a series of the PMDS patients who were managed by orchiopexy without removal of Müllerian remnants (MR). Material and methods: Between May 2010 and June 2017, we treated six cases diagnosed as PMDS in our department...
March 2018: Turkish Journal of Urology
Karina Miura Costa, Amulya Kumar Saxena
BACKGROUND: Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates. METHODS: PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS). RESULTS: Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH) (n = 76, 71%), cardiac malformations (n = 25, 23...
March 5, 2018: World Journal of Pediatrics: WJP
Siyu Liu, Mugen Dai, Bin Ye, Zhigang Zhao, Yang Shi, Lirong Peng
RATIONALE: Diaphragmatic Hernia is rare as complication of Colonoscopy. Diaphragmatic hernia as a complication of colonoscopy has been reported only few cases. Additionally, it is often misdiagnosed as other disease by clinicians due to their lack of related knowledge, which delays diagnosis of Diaphragmatic hernia and thus exacerbates the prognosis. PATIENT CONCERNS: We report the case of a 66-year-old man with fecal occult blood. In the case, sudden epigastric pain after colonoscopy owing to diaphragmatic hernia in a left hemidiaphragm...
January 2018: Medicine (Baltimore)
Matthew Wong, Janette Reyes, Eveline Lapidus-Krol, Monping Chiang, Tilman Humpl, Malikah Al-Faraj, Greg Ryan, Priscilla P L Chiu
Prenatal observed/expected lung-to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients. METHODS: A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5years of age...
February 7, 2018: Journal of Pediatric Surgery
Q Jiang, W Y Liu, W Feng, H H Jia, F Hou
Objective: To investigate the effect of prenatal administration of tetrandrine on the expression of Kruppel-like factors 5 (KLF5) and Survivin in the lung of congenital diaphragmatic hernia (CDH) rat model. Methods: This experiment took simple random group method, 11 pregnant SD rats were randomly divided into three groups: normal control group (group NC, 3), model control group (group MC, 4), model+ tetrandrine group (group MT, 4). The rats in group MC and MT were given gavage administration with 125 mg nitrofen to induce CDH and the rats in group MT were administered with 30 mg/kg tetrandrine for three days (gestational age of 18...
February 13, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Bashar Aldeiri, Urmas Roostalu, Alessandra Albertini, Julia Behnsen, Jason Wong, Antonino Morabito, Giulio Cossu
Pentalogy of Cantrell (PC) is a rare multi-organ congenital anomaly that impedes ventral body wall closure and results in diaphragmatic hernia, intra- and pericardial defects. The underlying cellular and molecular changes that lead to these severe developmental defects have remained unknown largely due to the lack of representative animal models. Here we provide in depth characterization of a mouse model with conditional ablation of TGFβRII in Transgelin (Tagln) expressing cells. We show that Tagln is transiently expressed in a variety of cells that participate in the embryonic development and patterning of ventral structures...
February 26, 2018: Scientific Reports
Ahmad Uzair Qureshi, Maliha Fansur, Sidra Shoaib Qureshi
Congenital trans-mesenteric hernias (CTMHs) are caused by rent in the mesentry of bowel. The lesions commonly present in pediatric age group. Adult CTMHs usually present with complication of the problem. The diagnosis is often late and is associated with morbidity as well as mortality due to bowel ischemia. There is no specific sign associated with this condition. A case is reported of a patient diagnosed with CTMH on high suspicion by the radiologist based on her finding of abnormal swirling of superior mesenteric artery on CT angiogram...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Lennart van der Veeken, Francesca M Russo, Johannes van der Merwe, David Basurto, Dyuti Sharma, Tram Nguyen, Marie P Eastwood, Namesh Khoshgoo, Jaan Toelen, Karel Allegaert, Philip Dekoninck, Stuart B Hooper, Richard Keijzer, Paolo DE Coppi, Jan Deprest
Congenital diaphragmatic hernia is rare birth defect, which can be easily corrected after birth. The main problem is that herniation of viscera during fetal development impairs lung development, leading to a 30 % mortality and significant morbidity. In isolated cases the outcome can be accurately predicted prenatally by medical imaging. Cases with a poor prognosis can be treated before birth; clinically this is by fetoscopic endoluminal tracheal occlusion. Obstruction of the airways triggers lung growth. This procedure and is currently being evaluated in a global clinical trial for left sided cases; right sided cases with poor prognosis are offered the procedure clinically...
February 23, 2018: Minerva Pediatrica
Anu Bashamboo, Caroline Eozenou, Anne Jorgensen, Joelle Bignon-Topalovic, Jean-Pierre Siffroi, Capucine Hyon, Attila Tar, Péter Nagy, Janos Sólyom, Zita Halász, Annnabel Paye-Jaouen, Sophie Lambert, David Rodriguez-Buritica, Rita Bertalan, Laetitia Martinerie, Ewa Rajpert-De Meyts, John C Achermann, Ken McElreavey
Emerging evidence from murine studies suggests that mammalian sex determination is the outcome of an imbalance between mutually antagonistic male and female regulatory networks that canalize development down one pathway while actively repressing the other. However, in contrast to testis formation, the gene regulatory pathways governing mammalian ovary development have remained elusive. We performed exome or Sanger sequencing on 79 46,XX SRY-negative individuals with either unexplained virilization or with testicular/ovotesticular disorders/differences of sex development (TDSD/OTDSD)...
February 16, 2018: American Journal of Human Genetics
Arvind Sehgal, Kenneth Tan, Peter Ferguson
The objectives of this study were to characterize peri-operative echocardiographic (ECHO) assessments of right ventricular (RV) function and pulmonary hypertension (PH) and ascertain correlation with clinical outcomes in infants with left Bochdalek's hernia (LBH). This retrospective study in a quaternary neonatal intensive care unit involved electronic database search for infants with LBH during January 2009 to July 2017. Demographics, outcomes, and ECHO parameters were accessed from archived databases. Thirty-one infants with mean gestational age and birthweight (BW) 38...
February 23, 2018: Pediatric Cardiology
José Ricardo N DE Souza Neto, Érika Branco, Elane G Giese, Ana Rita DE Lima
The wall of the diaphragm can be affected by congenital or acquired alterations which allow the passage of viscera between the abdominal and chest cavities, allowing the formation of a diaphragmatic hernia. We characterized morphology and performed biometrics of the diaphragm in the common squirrel monkey Saimiri sciureus. After fixation, muscle fragments were collected and processed for optical microscopy. In this species the diaphragm muscle is attached to the lung by phrenopericardial ligament. It is also connected to the liver via the coronary and falciform ligaments...
February 19, 2018: Anais da Academia Brasileira de Ciências
Catarina Barroso, Jorge Correia-Pinto
Although improvements have been made, the management of congenital diaphragmatic hernia (CDH) remains a major challenge for perinatologists and neonatal surgeons. Many aspects of the disease remain unknown and, being a rare entity, evidence-based data are hard to find. Surgical morbidity is considerable and affects long-term quality of life. Perioperative complications have been reviewed focusing on thoracoscopic repair. Intraoperative acidosis was more severe during thoracoscopy when compared with open surgery (OS), though it is possible that later neurodevelopment was not affected...
February 19, 2018: European Journal of Pediatric Surgery
Emma J Cordes, Melanie LaPlant, Daniel A Saltzman, Robert D Acton, Bradley J Segura, Donavon J Hess
BACKGROUND: Umbilical hernia is a common congenital anomaly, and can result in the appearance of a protuberant umbilicus. In select cases, inversion of the umbilical skin can be impaired by the presence of thickened dermis or fascial remnants of the umbilical stalk. DESCRIPTION OF OPERATIVE TECHNIQUE: After umbilical herniorrhaphy, the skin is everted over the left index finger and radial partial thickness incisions in the fascia and dermis of the undersurface of the umbilicus...
February 14, 2018: Journal of Pediatric Surgery
Elin Öst, Björn Frenckner, Margret Nisell, Carmen Mesas Burgos, Maria Öjmyr-Joelsson
PURPOSE: The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH). METHODS: Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children's hospital in Stockholm. In 2012, long-term survivors (n = 77) were asked to participate in the present study, which resulted in a 46% (n = 35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed...
February 16, 2018: Pediatric Surgery International
Hai-Xiang Yu, Chun-Shan Han, Jin-Ru Xue, Zhi-Feng Han, Hua Xin
Esophageal hiatal hernia involves abnormal abdominal entry into thoracic cavity. It is classified based on orientation between esophageal junction and diaphragm. Sliding hiatal hernia (Type-I) comprises the most frequent category, emanating from right crus of diaphragm. Type-II esophageal hernia engages both left and right muscular crura. Type-III and IV additionally include the left crus. Age and increased body mass index are key risk factors, and congenital skeletal aberrations trigger pathogenesis through intestinal malrotations...
April 2018: Expert Review of Gastroenterology & Hepatology
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival of neonates with CDH and HLHS is only 1-5%. We review our experience with CDH and HLHS and compare our outcomes to published literature. METHODS: Retrospective review of all neonates with CDH and HLHS at our institution over a 10 year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies and outcomes were reviewed and an algorithmic approach is proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
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