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Pediatrics omphalocele

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...
September 7, 2016: 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
Katherine B Kelly, Todd A Ponsky
This article reviews the incidence, presentation, anatomy, and surgical management of abdominal wall defects found in the pediatric population. Defects such as inguinal hernia and umbilical hernia are common and are encountered frequently by the pediatric surgeon. Recently developed techniques for repairing these hernias are aimed at improving cosmesis and decreasing pain while maintaining acceptably low recurrence rates. Less common conditions such as femoral hernia, Spigelian hernia, epigastric hernia, lumbar hernia, gastroschisis, and omphalocele are also discussed...
October 2013: Surgical Clinics of North America
Hussein Naji, Jane Foley, Henrik Ehren
AIM: Abdominal wall defects in children can present a challenge to the pediatric surgeon. Despite the development of new materials and modifications of surgical technique, no single approach has been established. The purpose of this study was to evaluate the authors experience using porcine small intestine submucosa for abdominal closure. PATIENTS AND METHODS: A retrospective review of all patients with abdominal wall defects who underwent reconstruction with Surgisis at the authors' institution from 2004 to 2011 was performed...
February 2014: European Journal of Pediatric Surgery
F Horn, E Kubikova, D Dubravova, P Babal, H El Falougy, P Selmeciova, L Hajnovic
BACKGROUND: Congenital anomalies of the abdominal wall are classified as anomalies with the abdominal wall defect (omphalocele, gastroschisis) and without the defect (umbilical hernia, persistent ductus omphaloentericus or urachus). Clinical presentations of these conditions are different, and so is the timing of surgical intervention and approach with or without the exploration of the peritoneal cavity. CASE: The presented case report refers to a rare finding of ectopic liver forming mesodermal cyst within the umbilical region...
2013: Bratislavské Lekárske Listy
Andrea Bischoff, Maria A Calvo-Garcia, Naira Baregamian, Marc A Levitt, Foong-Yen Lim, Jennifer Hall, Alberto Peña
INTRODUCTION: With the advance of prenatal imaging, more often pediatric surgeons are called for prenatal counseling in suspected cases of cloaca or cloacal exstrophy. This presents new challenges for pediatric surgeons since no specific guidelines have been established so far. The purpose of this review is to analyze our experience in prenatally diagnosed cloaca or cloacal exstrophy and to provide some guidelines for prenatal counseling of these complex congenital anomalies. METHODS: A retrospective review of the medical charts of patients with prenatally diagnosed cloaca and cloacal exstrophy who received postnatal care in our institution between July 2005 and March 2012 was performed...
August 2012: Pediatric Surgery International
Saleem Islam
Abdominal wall defects (AWDs) are a common congenital surgical problem in fetuses and neonates. The incidence of these defects has steadily increased over the past few decades due to rising numbers of gastroschisis. Most of these anomalies are diagnosed prenatally and then managed at a center with available pediatric surgical, neonatology, and high-risk obstetric support. Omphaloceles and gastroschisis are distinct anomalies that have different management and outcomes. There have been a number of recent advances in the care of patients with AWDs, both in the fetus and the newborn, which will be discussed in this article...
June 2012: Clinics in Perinatology
Daniel J Ledbetter
The embryology, epidemiology, associated anomalies, prenatal course and the neonatal and surgical care of newborns with gastroschisis and omphalocele are reviewed. For gastroschisis temporary intestinal coverage is often done before a more definitive operative closure that may be immediate or delayed. Outcomes in gastroschisis are determined by associated bowel injury. For omphalocele small defects are closed primarily while large defects are treated topically to allow initial skin coverage before a later definitive closure...
June 2012: Surgical Clinics of North America
Alana Beres, Emily R Christison-Lagay, Rodrigo L P Romao, Jacob C Langer
BACKGROUND: Abdominal wall defects in children are not always amenable to primary repair and may require a patch. The ideal material has yet to be established. We sought to evaluate our experience using the bioabsorbable material Surgisis (Cook Surgical, Bloomington, IN) for abdominal closure. METHODS: A retrospective chart review of abdominal wall defects repaired with Surgisis in our institution from 2000 to 2010 was performed. Data extracted included cause of defect, age at operation, possibility of skin coverage, recurrence, length of follow-up, and rate of wound infection...
May 2012: Journal of Pediatric Surgery
Ottavio Adorisio, Massimiliano Silveri, Massimo Rivosecchi, Alberto Eugenio Tozzi, Federico Scottoni, Paola Sabrina Buonuomo
INTRODUCTION: The quality medical information on Internet is highly variable. The aim of this study is to determine if Web pages addressing four common pediatric surgical topics (CT) and four uncommon pediatric surgical topics (UT) differ significantly in terms of quality and/or characteristics. MATERIALS AND METHODS: We performed an Internet search regarding four CT, addressing more frequent clinical conditions with an incidence≤1:1.500 children (inguinal hernia, varicocele, umbilical hernia, and phimosis) and four UT addressing less frequent clinical conditions with an incidence≥1:1...
June 2012: European Journal of Pediatric Surgery
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