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Pediatrics omphalocele

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
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
Xiaoying Li, Yuqiang Lyu, Min Gao, Xiuli Yan, Chen Meng, Kaihui Zhang, Yi Liu, Zhongtao Gai
OBJECTIVE: To explore the genetic cause for two children with omphalocele. METHODS: The patients were examined, and the medical history of their families was collected. Methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) was performed to detect potential mutation in the patients. RESULTS: Loss of methylation of imprinting center 2 (IC2) at the 11p15.5 region of the maternal chromosome was detected in both children...
December 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Erin P Ward, Andrew Wang, Hari Thangarajah, David Lazar, Stephen Bickler, Timothy Fairbanks, Karen M Kling
INTRODUCTION: Patients with congenital diaphragmatic hernia (CDH), gastroschisis, and omphalocele are prone to abnormalities of intestinal rotation and thus future midgut volvulus. Controversy exists whether routine preemptive Ladd procedure in this subgroup of asymptomatic patients is of value to reduce future volvulus. METHODS: The Pediatric Health Information System (PHIS) database was queried for neonates with CDH, gastroschisis, and omphalocele between January 2009 and September 2015...
December 2017: Journal of Pediatric Surgery
Masanori Tsukamoto, Takashi Hitosugi, Takeshi Yokoyama
Airway obstruction in pediatric patients always poses a challenge for anesthesiologists. Beckwith-Wiedemann syndrome causes various abnormalities such as macroglossia and omphalocele. Patients with these abnormalities often need corrective surgeries. Management of difficult airway caused by conditions such as macroglossia in patients with this syndrome could be challenging. We encountered a case of difficult airway in an infant with Beckwith-Wiedemann syndrome. It was predicted that macroglossia might cause difficult ventilation, intubation, and extubation...
December 2016: Journal of Dental Anesthesia and Pain Medicine
James D Vargo, Michael T Larsen, Gregory D Pearson
BACKGROUND: Massive defects of the abdominal wall are commonly repaired with the component separation technique (CST) when insufficient tissue exists to close the defect primarily. Although the utility of CST has been documented in cases of large ventral hernias in adults, its application to congenital and acquired defects in pediatric patients has been largely unreported. This study is a retrospective case series discussing the success of CST at a large pediatric hospital. METHODS: Seven patients with massive abdominal wall defects, including ventral hernia and omphalocele, repaired with CST at a pediatric hospital were identified as candidates...
November 2016: Annals of Plastic Surgery
Jamie Golden, Wesley E Barry, Gene Jang, Nam Nguyen, David Bliss
INTRODUCTION: The Morgagni hernia (MH) accounts for 3-4% of congenital diaphragmatic hernias. There is a paucity of data regarding this rare defect. The purpose of this study is to describe the characteristics of children with MH, surgical approaches for repair, and patient outcomes. METHODS: Pediatric patients (ages 0-18) with a MH from 2002 to 2014 at a single, freestanding pediatric hospital were retrospectively reviewed. Patient presentation, demographics, operative methods and findings, and outcomes were evaluated...
July 2017: Pediatric Surgery International
Osnat Zmora, Shannon L Castle, Stephanie Papillon, James E Stein
BACKGROUND: Our aim was to explore the indications for and outcome of biological prostheses to repair high risk abdominal wall defects in children. METHODS: A retrospective chart review was performed of all cases of abdominal wall reconstruction in a single institution between 2007 and 2015. Demographic and clinical variables, technique and complications were described and compared between prosthesis types. RESULTS: A total of 23 patients underwent abdominal wall reconstruction using a biological prosthesis including 17 neonates...
September 2017: American Journal of Surgery
Carmen C Tong, Kelly A Duffy, David I Chu, Dana A Weiss, Arun K Srinivasan, Douglas A Canning, Jennifer M Kalish
Beckwith-Wiedemann Syndrome (BWS) is an overgrowth disorder with various congenital anomalies. Although the most classic constellation includes macrosomia, macroglossia, and omphalocele, nephrourological findings are commonly associated with BWS. Clinical presentation is highly variable because of its complex molecular heterogeneity, which involves changes in DNA methylation and disruption of growth regulatory genes. We report 3 pediatric patients, ages 13 months to 3 years old, who presented with clinical features consistent with BWS...
February 2017: Urology
V Lambropoulos, E Mylona, V Mouravas, C Tsakalidis, I Spyridakis, G Mitsiakos, P Karagianni
Introduction. Incisional hernias are a common complication appearing after abdominal wall defects reconstruction, with omphalocele and gastroschisis being the most common etiologies in children. Abdominal closure of these defects represents a real challenge for pediatric surgeons with many surgical techniques and various prosthetic materials being used for this purpose. Case Report. We present a case of repair of a postoperative ventral hernia occurring after congenital omphalocele reconstruction in a three-and-a-half-year-old child using an acellular, sterile, porcine dermal mesh...
2016: Case Reports in Medicine
Erin A Miller, Adam Goldin, Geoffrey N Tse, Raymond Tse
Abdominal wall reconstruction ideally involves maintenance of domain by restoration of competent fascia and innervated muscle. Component separation allows closure of ventral hernias, but the technique is limited for high abdominal defects in the epigastric region. We describe an extended component separation that facilitated mobilization of the rectus abdominis muscle along its costal insertion to close an upper midline defect in a child with giant omphalocele, who had already undergone previous traditional component separation...
September 2015: Plastic and Reconstructive Surgery. Global Open
M Yagi, M Kohno, K Asagiri, T Ikeda, T Okada, S Kanada, S Kawashima, Y Goto, S Takano, M Yasufuku, M Wada
AIM: To discuss the chronological changes observed in a national survey of neonatal surgery in Japan performed every 5 years by the Committee in the Japanese Society of Pediatric Surgeons. METHODS: We analyzed the data obtained for 20 years from 1993 to 2013 and herein report the chronological changes. RESULTS: The number of summarized cases was least in 1993, with 2806 cases, and subsequently increased to 3753 cases in 2013. The mortality rate among the patients with maternal transport linearly decreased (p = 0...
October 2015: Pediatric Surgery International
John G Pappas
Beckwith-Wiedemann syndrome (BWS) is the most common genetic overgrowth syndrome, and it is frequently clinically recognizable because of characteristic features. These features include macrosomia, hemihypertrophy, macroglossia, facial nevus flammeus, earlobe creases and pits, omphalocele, and organomegaly. The most common molecular cause is hypomethylation of the maternal imprinting control region 2 (ICR2) in 11p15. Other molecular causes include hypermethylation of the maternal ICR1 in 11p15, mutations in CDKN1C, mosaic uniparental disomy 11p15, and chromosomal abnormalities involving 11p15...
April 2015: Current Problems in Pediatric and Adolescent Health Care
Tomoaki Taguchi, Kouji Nagata, Yoshiaki Kinoshita, Genshiro Esumi
The Japanese Society of Pediatric Surgeons has performed a nationwide survey of neonatal surgery every 5 years during the 50 years since its foundation. The number of neonatal surgical cases has increased 5-fold during those 50 years, while the mortality rate has decreased from 60% to 15% for the main life-threatening diseases (such as esophageal atresia, diaphragmatic hernia, omphalocele, and gastroschisis). Currently, most neonatal patients who undergo surgery for severe cardiac or chromosomal anomalies survive...
November 2014: Nihon Geka Gakkai Zasshi
Piergiorgio Gamba, Paola Midrio
Omphalocele and gastroschisis represent the most frequent congenital abdominal wall defects a pediatric surgeon is called to treat. There has been an increased reported incidence in the past 10 years mainly due to the diffuse use of prenatal ultrasound. The early detection of these malformations, and related associated anomalies, allows a multidisciplinary counseling and planning of delivery in a center equipped with high-risk pregnancy assistance, pediatric surgery, and neonatology. At present times, closure of defects, even in multiple stages, is always possible as well as management of most of cardiac-, urinary-, and gastrointestinal-associated malformations...
October 2014: Seminars in Pediatric Surgery
E Tarcă, Sg Aprodu
BACKGROUND: Omphalocele is a congenital abnormality whose prognosis has improved significantly over the last few decades, reaching a survival rate of 80-90% in developed countries. Currently, in Romania no comprehensive study on the incidence, treatment, and survival of patients with this defect of the anterior abdominal wall has been carried out. METHODS: This retrospective analytical study was conducted over a period of 23 years and included 105 children with omphalocele...
July 2014: Chirurgia
Catalina Correa, Christina Mallarino, Rafael Peña, Luis Carlos Rincón, Gloria Gracia, Ignacio Zarante
BACKGROUND: Congenital anomalies (CAs) cause nearly one third of infant deaths worldwide. Various surveillance systems have been established, such as the Bogota Congenital Malformations Surveillance Program (BCMSP). Some CAs are of special interest to pediatric surgeons: omphalocele, gastroschisis, intestinal and esophageal atresia, anorectal malformations, vascular anomalies, diaphragmatic hernias, hypospadias and cryptorchidism. The aim of this study is to determine the prevalence of such CAs, and identify possible risk factors...
July 2014: Journal of Pediatric Surgery
S Ionescu, M Mocanu, B Andrei, B Bunea, C Carstoveanu, A Gurita, R Tabacaru, E Licsandru, D Stanescu, M Selleh
A newborn with abdominal wall defect is one of the most dramatic cases in neonatology, but also a challenge for pediatric surgeons. This article describes the fundamental concepts of two major abdominal wall defects - gastroschisis and omphalocele - including options and principles of prenatal and postnatal care. Although these birth defects of the abdominal wall are always grouped together, they are two separate and distinct entities, with many differences in terms of pathology and associated anomalies; this explains the different therapeutic approach and results...
January 2014: Chirurgia
Shauna Levy, KuoJen Tsao, Charles S Cox, Uma R Phatak, Kevin P Lally, Richard J Andrassy
PURPOSE: Operative repair of large abdominal wall defects in infants and children can be challenging. Component separation technique (CST) is utilized in adults to repair large abdominal wall defects but rarely used in children. The purpose of this report is to describe our experience with the CST in pediatric patients including the first description of CST use in newborns. METHODS: After IRB approval, we reviewed all patients who underwent CST between June 1, 2010 and December 31, 2012 at a large children's hospital...
December 2013: Journal of Pediatric Surgery
A Clausner, A Lukowitz, K Rump, S Berger, A Würfel
The outcome data of 132 patients treated at the Department of Pediatric Surgery in Mainz during the last 25 years were reviewed. Prenatal diagnosis of abdominal wall defects (AWDs) and associated malformations led to increasing selection of the patient population. The aim of primary closure of the abdominal wall can be achieved more frequently in gastroschisis (GS) than omphalocele (OC), while the postoperative course is more complicated and of longer duration in GS. Delayed or secondary closure extended the hospitalization period but had no negative effects on the outcome...
March 1996: Pediatric Surgery International
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