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https://www.readbyqxmd.com/read/29803304/early-surgical-management-for-giant-omphalocele-results-and-prognostic-factors
#1
Nathalie Roux, Déborah Jakubowicz, Laurent Salomon, Gilles Grangé, Agnès Giuseppi, Véronique Rousseau, Naziha Khen-Dunlop, Sylvie Beaudoin
OBJECTIVES: Giant omphalocele often represents a major surgical challenge and is reported with high mortality and morbidity rates. The aim of this study was to assess the outcome of neonates with giant omphalocele managed with early operative surgical treatment, and subsequently to identify possible factors that could alter the prognosis. METHODS: We reviewed the medical records of 29 consecutive newborns with prenatally diagnosed giant omphalocele. In these cases one of two procedures had been performed: either staged closure after silo, or immediate closure with a synthetic patch...
May 23, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29754139/dichorionic-twins-discordant-for-body-stalk-anomaly-a-management-challenge
#2
Rita Caldas, Cátia Rodrigues, Carla Pina, Rosete Nogueira
Body-stalk anomaly is a sporadic and rare maldevelopment disorder characterised by large abdominal wall defect, spinal deformity and rudimentary umbilical cord. It is considered a lethal condition as there are only few reports of survival but there was at least one case of long-term survival after neonatal surgery.Differential diagnosis includes isolated omphalocele or gastroschisis, short umbilical cord, amniotic band, limb body-wall complex and other polymalformative syndromes.There are few reports about the expectant prenatal management of the body stalk anomaly as the majority of prenatal diagnosed cases undergo early elective termination...
May 12, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29735202/zorro-z-omphaloplasty-repair-for-omphalocele
#3
Michel Jean-Luc, Kassir Rani, Harper Luke, Gavage Laurent, Frade Fernanda, Clermidi Pauline, Sauvat Frederique, Ramful Duksha
PURPOSE: To suggest a novel technique for omphalocele closure which uses the circular base of the umbilical cord, thus allowing for a more physiological healing process and natural-looking scar. METHODS: Among 16 neonates operated for omphalocele between 2011 and 2016, 12 were closed with a one-stage procedure using a Z omphaloplasty (ZORRO). Median gestational age was 36.5 weeks; median birth weight was 3210 g. The umbilical arteries were divided and ligated outside the peritoneal cavity above the parietal musculocutaneous plane...
April 6, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29715697/epidemiology-and-outcome-of-major-congenital-malformations-in-a-large-german-county
#4
Boris Wittekindt, Rolf Schloesser, Nora Doberschuetz, Emilia Salzmann-Manrique, Jasmin Grossmann, Bjoern Misselwitz, Udo Rolle
INTRODUCTION:  Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA)...
May 1, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29709845/vitelline-fistula-associated-with-omphalocele-diagnostic-dilemma
#5
Edgar D Sy, Yen-Shen Shan, Chyi-Her Lin
INTRODUCTION: Combination vitelline fistula (VF) and omphalocele at birth is a rare congenital anomaly as a result disturbance in organogenesis with failure of normal return of intestines into the abdominal cavity and failed obliteration of the vitelline duct. CASE PRESENTATION: A newborn presented with omphalocele sac with visible intestine, stoma like lesion with prolapsing mucosa just lateral to the umbilical cord and passage of meconium stool. Operative surgery was confirmed an intact omphalocele sac and vitelline fistula...
April 16, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29676346/formal-saline-versus-honey-as-escharotic-in-the-conservative-management-of-major-omphaloceles
#6
Christopher O Bode, Adesoji O Ademuyiwa, Olumide A Elebute
Background: The use of honey as an escharotic agent in the conservative management of omphalocele major has not been widely explored in spite of its proven benefits in chronic wound management. We explored the use of local honey as an escharotic agent by comparing its use with 2.5% formal saline in the conservative management of major omphaloceles at the Lagos University Teaching Hospital, Lagos, Nigeria. Methods: From January 2006 to December 2009, 43 consecutive newborns with intact omphalocele major were alternately assigned into either Honey (H) or formal saline (FS) group...
January 2018: Nigerian Postgraduate Medical Journal
https://www.readbyqxmd.com/read/29660797/repair-of-a-giant-omphalocele-in-an-infant-with-a-pericardial-implant-crosslinked-with-oligourethane
#7
Juan Vargas-Mancilla, María A Torrero-Serrato, Aarón J Palacios-Rodríguez, Gloria B Rodríguez-de León, Metzeri I Montes-Rodríguez, Birzabith Mendoza-Novelo
The giant omphalocele (GO) represents a challenge for the pediatric surgeon in its management and wall abdominoplasty. Here, we report the outcome of a case in which a GO in a newborn patient was repaired with an implant derived from decellularized bovine pericardium crosslinked with oligourethane. The implantation time was extended for 6 months. This was then followed up by the retrieval of the implant and the subsequent reconstruction in a second surgical time by the closure of the abdominal wall fascia...
April 16, 2018: Artificial Organs
https://www.readbyqxmd.com/read/29602885/beckwith-weidemann-syndrome-with-ic2-kvdmr1-hypomethylation-defect-a-novel-mutation
#8
Aakash Pandita, Shikha Gupta, Girish Gupta, Astha Panghal
The Beckwith-Wiedemann syndrome (BWS) is a rare genetic syndrome. However, this is one of the most common overgrowth syndromes. This is a genetically and clinically heterogeneous syndrome. Here, we report a case of Beckwith-Weidemann syndrome without macrosomia, visceromegaly and hemihyperplasia but having macroglossia, omphalocele and anterior linear ear lobe creases. The diagnosis was confirmed by gene analysis suggestive of imprinting centre 2 (KvDMR1) hypomethylation defect.
March 30, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29563149/omphalocele-from-diagnosis-to-growth-and-development-at-2-years-of-age
#9
Annelieke Hijkoop, Nina C J Peters, Rosan L Lechner, Yolande van Bever, Annabel P J M van Gils-Frijters, Dick Tibboel, René M H Wijnen, Titia E Cohen-Overbeek, Hanneke IJsselstijn
OBJECTIVES: To compare the prenatal frame of reference of omphalocele (ie, survival of fetuses) with that after birth (ie, survival of liveborn neonates), and to assess physical growth and neurodevelopment in children with minor or giant omphalocele up to 2 years of age. DESIGN: We included fetuses and neonates diagnosed in 2000-2012. Physical growth (SD scores, SDS) and mental and motor development at 12 and 24 months were analysed using general linear models, and outcomes were compared with reference norms...
March 21, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29557630/-surgical-treatment-of-macroglossia-in-beckwith-wiedemann-syndrome-case-report
#10
Pablo Roa Rojas, Hernán Arango Fernández, Martha Rebolledo Cobos, Jonathan Harris Ricardo
Beckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic epilepsy, renal failure, hypoglycemia, tracheotomy and gastrostomy, due to airway collapse and dysphagia...
April 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29555972/tio-2-nanoparticles-induce-omphalocele-in-chicken-embryo-by-disrupting-wnt-signaling-pathway
#11
Shweta Patel, Sarmita Jana, Rajlakshmi Chetty, Sonal Thakore, Man Singh, Ranjitsinh Devkar
Titanium dioxide nanoparticles (TiO2 NPs) are among abundantly used metal oxide NPs but their interactions with biomolecules and subsequent embryonic toxicity in higher vertebrates is not extensively reported. Physicochemical interactions of TiO2 NPs with egg albumen reveals that lower doses of TiO2 NPs (10 and 25 µg/ml) accounted for higher friccohesity and activation energy but an increment in molecular radii was recorded at higher doses (50 and 100 µg/ml). FTIR analysis revealed conformational changes in secondary structure of egg albumen as a result of electrostratic interactions between egg albumen and TiO2 NPs...
March 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29551244/surgical-outcomes-of-patients-with-beckwith-wiedemann-syndrome
#12
Candace C Style, Stephanie M Cruz, Patricio E Lau, Timothy C Lee, David E Wesson, Oluyinka O Olutoye
PURPOSE: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital. METHODS: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes. RESULTS: Forty-seven children with a diagnosis of BWS were identified...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29550866/fetal-anterior-abdominal-wall-defects-prenatal-imaging-by-magnetic-resonance-imaging
#13
REVIEW
Teresa Victoria, Savvas Andronikou, Diana Bowen, Pablo Laje, Dana A Weiss, Ann M Johnson, William H Peranteau, Douglas A Canning, N Scott Adzick
Abdominal wall defects range from the mild umbilical cord hernia to the highly complex limb-body wall syndrome. The most common defects are gastroschisis and omphalocele, and the rarer ones include the exstrophy complex, pentalogy of Cantrell and limb-body wall syndrome. Although all have a common feature of viscera herniation through a defect in the anterior body wall, their imaging features and, more important, postnatal management, differ widely. Correct diagnosis of each entity is imperative in order to achieve appropriate and accurate prenatal counseling and postnatal management...
April 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29550350/is-omphalocele-a-non-specific-malformation-in-new-zealand-white-rabbits
#14
George P Daston, Manon Beekhuijzen
We evaluated the incidence of omphalocele, a malformation that occurs sporadically in many studies. We assembled data on external malformations using all treatment groups from every study published in three major journals over the past 35 years using New Zealand White rabbits. Fifty-eight papers were included: 4905 litters and 36,977 fetuses. Omphalocele was reported in 43% and was among the most common defects, occurring at a rate of 1.10% (litter) and 0.16% (fetus). The defect did not appear to be treatment-related, although it may have been in two studies, based on rate and dose-responsiveness...
March 14, 2018: Reproductive Toxicology
https://www.readbyqxmd.com/read/29519568/dressed-for-success-silver-impregnated-nanocrystalline-dressing-for-initial-treatment-of-giant-omphalocele
#15
Dean B Percy, Candace Haddock, Vivian Ma, Amie Nowak, Julia Panczuk, Sonia Butterworth
OBJECTIVE: The purpose of this study was to describe outcomes and resource utilization in patients treated with twice-weekly silver impregnated (SI) nanocrystalline dressings for initial non-operative management of giant omphalocele (GO). METHODS: A retrospective review of patients with GO treated with SI dressings was undertaken. Clinical parameters, cost, and complications were recorded. RESULTS: Five patients with GO were treated with SI dressings between 2014 and 2016...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29519207/adhd-medication-use-during-pregnancy-and-risk-for-selected-birth-defects-national-birth-defects-prevention-study-1998-2011
#16
Kayla N Anderson, Annelise C Dutton, Cheryl S Broussard, Sherry L Farr, Jennifer N Lind, Susanna N Visser, Elizabeth C Ailes, Stuart K Shapira, Jennita Reefhuis, Sarah C Tinker
OBJECTIVE: The objective of this study was to examine the prevalence of, and maternal characteristics associated with, ADHD medication use before and during pregnancy, and associations between early pregnancy ADHD medication use and risk for 12 selected birth defects. METHOD: We used data from the National Birth Defects Prevention Study (1998-2011), a U.S. population-based case-control study examining risk factors for major structural birth defects. RESULTS: There was an increase in ADHD medication use from 1998-1999 (0...
March 1, 2018: Journal of Attention Disorders
https://www.readbyqxmd.com/read/29515966/erratum-to-delayed-closure-of-giant-omphaloceles-in-west-africa-report-of-five-cases
#17
Oumama El Ezzi, Raymond Bossou, Olivier Reinberg, Sabine Vasseur Maurer, Anthony de Buys Roessingh
[This corrects the article DOI: 10.1055/s-0037-1599796.].
January 2018: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/29508360/ex-utero-intrapartum-treatment-for-giant-congenital-omphalocele
#18
Xu-Yong Chen, Ji-Xin Yang, Hong-Yi Zhang, Xiao-Feng Xiong, Khalid Mohamoud Abdullahi, Xiao-Juan Wu, Jie-Xiong Feng
BACKGROUND: To determine whether ex utero intrapartum treatment (EXIT) is an appropriate approach for managing fetuses antenatally diagnosed with giant congenital omphaloceles. METHODS: We retrospectively reviewed patients with omphaloceles who underwent either an EXIT procedure or a traditional repair surgery. Basic and clinical parameters including gender, gestational age, birth weight, maternal blood loss, operative times and operative complications were analyzed...
March 5, 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29498037/prenatal-diagnosis-of-persistent-right-umbilical-vein-incidence-and-clinical-impact-a-prospective-study
#19
Arkadiusz Krzyżanowski, Dariusz Swatowski, Tomasz Gęca, Maciej Kwiatek, Aleksandra Stupak, Sławomir Woźniak, Anna Kwaśniewska
BACKGROUND: Persistent right umbilical vein (PRUV) is usually an isolated finding but it may be accompanied by other fetal malformations. AIMS: We aimed to determine the incidence of prenatally diagnosed PRUV in a referral population, assess the neonatal outcome and discuss the findings together with those from previous publications. MATERIALS AND METHODS: A total of 2360 women with low-risk singleton pregnancies were examined in the second and third trimesters...
March 2, 2018: Australian & New Zealand Journal of Obstetrics & Gynaecology
https://www.readbyqxmd.com/read/29450693/the-effect-of-assisted-reproductive-technology-on-the-incidence-of-birth-defects-among-livebirths
#20
Gil Shechter-Maor, Nicholas Czuzoj-Shulman, Andrea R Spence, Haim Arie Abenhaim
PURPOSE: Our study objective is to examine the association between births conceived with assisted reproductive technology (ART) and birth defects using a large database from the United States. METHODS: Using the Centers for Disease Control and Prevention's Period-linked birth-infant death data files and fetal death database for 2011-2013, we conducted a retrospective cohort study comprised of live births that occurred in the USA during that time. Multivariate logistic regression was used to estimate the association between ART and birth defects, both overall and by specific defects...
February 15, 2018: Archives of Gynecology and Obstetrics
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