MedWorm: Abdominal Wall Repair

Gastroschisis: preterm or term delivery?

Clinics — Fri, 12 Mar 2010 14:39:55 +0100

AIM: The main objective of this study was to evaluate the association between prematurity and the time to achieve full enteral feeding in newborns with gastroschisis. The second objective was to analyze the associations between length of hospital stay and time to achieve full enteral feeding with mode of delivery, birth weight and surgical procedure. METHODS: The medical records of newborns with gastroschisis treated between 1997 and 2007 were reviewed. Two groups were considered: those delivered before 37 weeks (group A) and those delivered after 37 weeks (group B). The variables of gestational age, mode of delivery, birth weight, time to achieve full enteral feeding, length of hospital stay and surgical approach were analyzed and compared between groups. RESULTS: Forty-one patients were ...

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Outcomes in Children After Intestinal Transplant

PEDIATRICS — Mon, 01 Mar 2010 16:02:28 +0100

CONCLUSIONS: Survival after intestinal transplant was associated with the underlying disease state. The explanation for these findings requires additional investigation into the differences in characteristics of the population of children with intestinal failure. (Source: PEDIATRICS)

Agricultural-related chemical exposures, season of conception, and risk of gastroschisis in Washington State

American Journal of Obstetrics and Gynecology — Mon, 01 Mar 2010 00:00:00 +0100

Objective: We sought to determine if periconceptional exposure to agrichemicals was associated with the development of gastroschisis.Study Design: We conducted a retrospective, case-controlled study using Washington State Birth Certificate and US Geological Survey databases. Cases included all live-born singleton infants with gastroschisis. Distance between a woman's residence and site of elevated exposure to agrichemicals was calculated. Multivariate regression was used to estimate the association between surface water concentrations of agrichemicals and the risk of gastroschisis.Results: Eight hundred five cases and 3616 control subjects were identified. Gastroschisis occurred more frequently among those who resided (Source: American Journal of Obstetrics and Gynecology)

Method to our madness: an 18-year retrospective analysis on gastroschisis closure

Journal of Pediatric Surgery — Mon, 01 Mar 2010 00:00:00 +0100

Conclusion: Although limited by retrospective biases, this study demonstrates that time to closure is the most significant variable related to LOS in gastroschisis. This relationship is intuitive since longer time to closure is probably determined by the severity of gastroschisis. The method of closure, by primary repair or silo, is of secondary importance. Conversely, unnecessarily increasing the time to closure may increase the LOS. The speed of reduction, whether through primary repair or by silo, should be guided by physiologic principles. (Source: Journal of Pediatric Surgery)

Serious birth defects linked to the agricultural chemical atrazine

NaturalNews.com — Mon, 22 Feb 2010 07:00:00 +0100

(NaturalNews) Gastroschisis is a birth defect in which the intestines, and sometimes other organs, develop outside the fetal abdomen and poke out through an opening in the abdominal wall. Long considered a rare occurrence, gastroschisis has mysteriously been on the rise over the last three decades. In fact, the incidence of the defect has soared, increasing two to four times in the last 30 years. But why?Researchers think they've found the answer. The culprit behind the suffering of babies born with this condition appears to be the agricultural chemical atrazine. That's the conclusion of a study just presented at the annual meeting of the Society for Maternal-Fetal Medicine (SMFM) held in Chicago. Researchers at the University of Washington in Seattle were alerted to a higher than normal n...

The Outcome of Closing Gastroschisis: Two Case Reporta and Literature Review

European Journal of Pediatric Surgery — Thu, 11 Feb 2010 03:10:44 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1249141© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

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Weed Killer Atrazine May Be Linked to Gastroschisis

Medscape Ob/Gyn & Women's Health Headlines — Tue, 09 Feb 2010 17:52:50 +0100

Living near farms that use the weed killer atrazine may up the risk of the rare birth defect gastroschisis, according to a study presented this past Friday at the annual meeting of the Society for Maternal-Fetal Medicine in Chicago. Reuters Health Information (Source: Medscape Ob/Gyn & Women's Health Headlines)

Link between birth defect gastroschisis and the agricultural chemical atrazine found

ScienceDaily Headlines — Sun, 07 Feb 2010 16:00:00 +0100

New findings demonstrate a link between the birth defect gastroschisis and the agricultural chemical atrazine. Gastroschisis is a type of inherited congenital abdominal wall defect in which the intestines, and sometimes other organs, develop outside the fetal abdomen through an opening in the abdominal wall. The incidence of gastroschisis is on the rise, increasing two to four times in the last 30 years. (Source: ScienceDaily Headlines)

Discovery Of Link Between Birth Defect Gastroschisis And The Agricultural Chemical Atrazine

Health News from Medical News Today — Fri, 05 Feb 2010 13:00:00 +0100

In a study to be presented at the Society for Maternal-Fetal Medicine's (SMFM) annual meeting, The Pregnancy Meeting™ in Chicago, researchers unveiled findings that demonstrate a link between the birth defect gastroschisis and the agricultural chemical atrazine. Gastroschisis is a type of inherited congenital abdominal wall defect in which the intestines, and sometimes other organs, develop outside the fetal abdomen through an opening in the abdominal wall. The incidence of gastroschisis is on the rise, increasing two to four times in the last 30 years... (Source: Health News from Medical News Today)

Discovery Of Link Between Birth Defect Gastroschisis And The Agricultural Chemical Atrazine

GastroIntestinal News From Medical News Today — Fri, 05 Feb 2010 13:00:00 +0100

Link between birth defect gastroschisis and the agricultural chemical atrazine found

EurekAlert! - Medicine and Health — Fri, 05 Feb 2010 05:00:00 +0100

(Society for Maternal-Fetal Medicine) In a study to be presented today at the Society for Maternal-Fetal Medicine's (SMFM) annual meeting, The Pregnancy Meeting , in Chicago, researchers will unveil findings that demonstrate a link between the birth defect gastroschisis and the agricultural chemical atrazine. (Source: EurekAlert! - Medicine and Health)

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Birth Defect Linked to Crop Chemical

Medical Headlines From Ivanhoe.com — Fri, 05 Feb 2010 05:00:00 +0100

Cases of gastroschisis, an abdominal wall birth defect, have risen two to four times in the last 30 years, and researchers have now linked it to the agricultural chemical atrazine. (Source: Medical Headlines From Ivanhoe.com)

A strategy for treatment of giant omphalocele.

World Journal of Pediatrics : WJP — Mon, 01 Feb 2010 00:00:00 +0100

CONCLUSION: The method we used can be performed at bedside and without the application of anesthesia, but should be tried on more patients to determine its effect. PMID: 20119875 [PubMed - as supplied by publisher] (Source: World Journal of Pediatrics : WJP)

[Integrating the environmental clinic history into prenatal counseling and health care in gastroschisis: 2 case reports.]

Anales de Pediatria — Sat, 30 Jan 2010 00:00:00 +0100

CONCLUSIONS: 1. The PECH should be obtained in all patients with gastroschisis. 2. A thorough PECH requires a proper review of the related RFs and basic training to characterise and quantify environmental exposures. 3. Following these steps, useful recommendations to improve patient care and family advice in future pregnancies are provided. PMID: 20122885 [PubMed - as supplied by publisher] (Source: Anales de Pediatria)

First-trimester sonographic findings in trisomy 18: a review of 53 cases

Prenatal Diagnosis — Thu, 28 Jan 2010 00:00:00 +0100

To report our experience with first-trimester sonographic findings in pregnancies complicated by trisomy 18.Proven cases of trisomy 18 undergoing sonographic examination between 11 + 0 and 13 + 6 weeks of gestation were retrospectively identified. Information on maternal demographics, prenatal sonographic findings, and chromosomal analysis results was obtained by reviewing the ultrasound reports and medical records.During the 12-year period from July 1997 to June 2009, 53 cases of full trisomy 18 had first-trimester sonographic examination performed at our institution. All but one fetus displayed one or more abnormal sonographic finding, most commonly increased nuchal translucency thickness (n = 48, 91%). Absent or hypoplastic nasal bone was documented in 53% of the fetuses in which this m...

Outcome Differences between Gastroschisis Repair Methods

Journal of Surgical Research — Mon, 25 Jan 2010 16:42:02 +0100

Introduction: Gastroschisis is a congenital abdominal wall defect that is repaired either with primary closure or staged closure. The outcome of these infants may differ because of difference in closure techniques. In addition to the usual markers of parenteral nutrition (PN) use and length of stay (LOS) as outcome measures, we also examined the duration of postoperative acidosis as a surrogate marker for physiologic stress associated with these two techniques. Methods: A retrospective review of newborns with gastroschisis at a free-standing children's hospital from 2002 to 2008 was conducted. The demographic data, gestational age, birth weight, Apgar scores, operative reports, days on PN, hospital length of stay (LOS) and the duration of postoperative acidosis (defined as blood gas pH (So...

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Inflammatory response in a rat model of gastroschisis is associated with an increase of NF-kappaB.

Braz J Med Biol Res — Fri, 22 Jan 2010 00:00:00 +0100

The objective of this study was to identify components of the inflammatory response in the intestine and liver in an experimental model of gastroschisis in rats. The model was surgically created at 18.5 days of gestation. The fetuses were exposed through a hysterotomy and an incision at the right of the umbilicus was made, exposing the fetal bowel. Then, the fetus was placed back into the uterus until term. The bowel in this model had macro- and microscopic characteristics similar to those observed in gastroschisis. The study was conducted on three groups of 20 fetuses each: gastroschisis, control, and sham fetuses. Fetal body, intestine and liver weights and intestine length were measured. IL-1beta, IL-6, IL-10, TNF-alpha, IFN-gamma and NF-kappaB levels were assessed by ELISA. Data were a...

Association of Paternal Age and Risk for Major Congenital Anomalies From the National Birth Defects Prevention Study, 1997 to 2004

Annals of Epidemiology — Thu, 07 Jan 2010 00:00:00 +0100

Conclusions: Our findings suggest that paternal age may be a risk factor for some multifactorial birth defects. (Source: Annals of Epidemiology)

Association of Paternal Age and Risk for Major Congenital Anomalies From the National Birth Defects Prevention Study, 1997 to 2004.

Annals of Epidemiology — Tue, 05 Jan 2010 00:00:00 +0100

CONCLUSIONS: Our findings suggest that paternal age may be a risk factor for some multifactorial birth defects. PMID: 20056435 [PubMed - as supplied by publisher] (Source: Annals of Epidemiology)

Aplasia cutis congenita associated with multiple congenital anomalies: case report.

Turkish Neurosurgery — Fri, 01 Jan 2010 00:00:00 +0100

DISCUSSION: Multiple factors have been described as etiological factors for ACC but the etiology is not clear yet. ACC may also be associated with some syndromes. Management strategies are based on the size, presence of an underlying skull defect and the child's physical condition. PMID: 20066625 [PubMed - in process] (Source: Turkish Neurosurgery)

Long-Term Outcomes of Short Bowel Syndrome Requiring Long-Term/Home Intravenous Nutrition Compared in Children With Gastroschisis and Those With Volvulus

Transplantation Proceedings — Fri, 01 Jan 2010 00:00:00 +0100

Conclusions: Infants and young children with short gut and >10 cm small intestine gain intestinal autonomy. In contrast, those with gastroschisis have poor outcomes and should be referred for intestinal transplantation assessment more readily than those with after intestinal resection for volvulus short gut. (Source: Transplantation Proceedings)

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Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:38 +0100

The prenatal or postnatal factors that predict complex gastroschisis in patients (atresia, volvulus, necrotic bowel and bowel perforation) remain controversial. The authors from Mott Children's Hospital in Ann Arbor evaluated the prognostic value of prenatal ultrasonographic parameters and early postnatal factors in predicting clinical outcomes. (Source: Journal of Pediatric Surgery)

Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:26 +0100

Conclusions: In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population. (Source: Journal of Pediatric Surgery)

In utero repair of gastroschisis in the sheep (Ovis aries) model

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:25 +0100

Conclusion: In utero anatomic repair of gastroschisis is possible in mid-gestation in the fetal lamb model, and it appears to ameliorate the inflammatory process. (Source: Journal of Pediatric Surgery)

Diagnosis and management of intestinal motility disorders

Seminars in Pediatric Surgery — Thu, 31 Dec 2009 14:54:02 +0100

Gastrointestinal motility disorders in their most severe forms may directly lead to intestinal failure. Abnormal motor function may also contribute to the overall gut dysfunction of children who have other underlying gastrointestinal diseases, such as Hirschsprung disease or gastroschisis. Understanding the extent and the severity of the dysmotile segments has direct therapeutic and prognostic implications. Our ability to study gastrointestinal motility has greatly improved in the past few years, with the development of less-invasive diagnostic tests. Optimal treatment of children with intestinal motility disorders relies on a multidisciplinary approach, which focuses on optimizing nutrition, improving gastrointestinal motility, and reducing psychosocial disability. Patient education is im...

Acetaminophen use in pregnancy and risk of birth defects: findings from the national birth defects prevention study.

Obstetrics and Gynecology — Thu, 24 Dec 2009 14:45:32 +0100

CONCLUSION:: Single-ingredient-acetaminophen use during the first trimester does not appear to increase the risk of major birth defects. It may decrease the risk of selected malformations when used for a febrile illness. LEVEL OF EVIDENCE:: II. PMID: 20027042 [PubMed - in process] (Source: Obstetrics and Gynecology)

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Anal-transitional and bronchial metaplasia within the ileostomy of a small bowel allograft: A case report

Pediatric Transplantation — Tue, 08 Dec 2009 00:00:00 +0100

We present a case of anal-transitional and bronchial epithelial metaplasia that occurred near the ileostomy of a small bowel allograft in a seven-month-old male transplanted for short bowel syndrome related to gastroschisis. The anal-transitional metaplastic mucosa had an immunophenotype (CK7- and p63-positive) similar to that of normal mucosa at the anal transition zone and a DNA STR profile consistent with the donor. To our knowledge, this represents the first report of this type of metaplasia occurring in ileostomies in the English literature. It is important for clinicians and pathologists to recognize these benign mucosal alterations to avoid diagnostic confusion. (Source: Pediatric Transplantation)

Gastroschisis prevalence in Texas 1999-2003

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 01 Dec 2009 00:00:00 +0100

The worldwide prevalence of gastroschisis is increasing. Maternal age, race/ethnicity, and place of residence have been associated with increased risk.We obtained descriptive characteristics of mothers of infants with gastroschisis and mothers of all live births from the Texas Birth Defects Registry and Texas vital records for 1999-2003. We calculated prevalence, crude prevalence ratios, and prevalence ratios adjusted for maternal age, parity, education, race/ethnicity, and geographic entity (Mexican border proximity, urban/rural residence, health service region, and county).We observed 764 cases of gastroschisis among 1,827,317 live births, for a prevalence of 4.18 per 10,000 births (95% confidence interval 3.88-4.48). Prevalence increased during 1999-2003 (p for trend (Source: Birth Defe...

Mechanical compression with secondary ischemia as a possible cause of atresias associated with omphalocele

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

We report 2 cases of omphalocele associated with intestinal atresia. In the context of this unusual sequence, we discuss the etiopathogenesis of intestinal atresias in association with tight abdominal wall defects. (Source: Journal of Pediatric Surgery)

Surgical management of gastroschisis in North Queensland from 1988 to 2007

Journal of Paediatrics and Child Health — Mon, 23 Nov 2009 00:00:00 +0100

Conclusion: No significant differences were found between PNOR and other techniques for uncomplicated cases. PNOR avoids the use of theatre and staff, reducing delay and cost, and the need to transport a sick baby. (Source: Journal of Paediatrics and Child Health)

Hypothesis: Could Epstein-Barr virus play a role in the development of gastroschisis?

Birth Defects Research Part A: Clinical and Molecular Teratology — Fri, 20 Nov 2009 00:00:00 +0100

The strong inverse association between maternal age and risk of gastroschisis in offspring has spurred many investigators to hypothesize that behaviors among younger females are the cause. Examples include cigarette smoking, illicit drugs, genitourinary infections, and sexually transmitted diseases, each of which has been reported to be associated with gastroschisis. Although these exposures are more common in young women, recent studies have shown that cigarette smoking, genitourinary infections, and sexually transmitted diseases are most strongly associated with gastroschisis in older women. There is both anecdotal and published evidence showing that gastroschisis sometimes (but not always) occurs in clusters, raising the possibility that an infectious agent might be involved in its path...

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Perioperative management of a neonate with Cantrell syndrome

Journal of Anesthesia — Tue, 17 Nov 2009 23:58:50 +0100

Abstract Cantrell syndrome is a congenital malformation with a pentalogy characterized by defects involving the abdominal wall, lower sternum, anterior diaphragm, and diaphragmatic pericardium, as well as congenital cardiac anomalies. We recently managed anesthesia in a patient with this syndrome and herein report our experience. The patient was a 14-day-old male neonate, who had been diagnosed with Cantrell syndrome, including ventricular septal defect, left ventricular diverticulum, abdominal wall defect, omphalocele, and sternal hypoplasia. Surgical interventions to close the ventricular septal defect, resect the left ventricular diverticulum, and close the omphalocele were scheduled. After cardiac surgery, the hernial contents were returned to their original compartmen...

Light Shed On Gastroschisis Birth Defect By Local Health Investigation

Health News from Medical News Today — Mon, 09 Nov 2009 08:00:00 +0100

Light Shed On Gastroschisis Birth Defect By Local Health Investigation

GastroIntestinal News From Medical News Today — Mon, 09 Nov 2009 08:00:00 +0100

Results of an investigation conducted by University of Nevada, Reno researchers, public health officials and area physicians published in the Archives of Pediatrics & Adolescent Medicine, indicate that Washoe County experienced a cluster of a particular birth defect, gastroschisis, during the period April 2007 - April 2008. Subsequent review of medical records since the study's conclusion indicates that while the rate is still elevated, the cluster appears to have subsided. (Source: GastroIntestinal News From Medical News Today)

Local health investigation sheds light on gastroschisis birth defect

EurekAlert! - Social and Behavioral Science — Fri, 06 Nov 2009 05:00:00 +0100

(University of Nevada, Reno) Results of an investigation conducted by University of Nevada, Reno researchers, public health officials and area physicians published this week in the Archives of Pediatrics & Adolescent Medicine, indicate that Washoe County experienced a cluster of a particular birth defect, gastroschisis, during the period April 2007-April 2008. Subsequent review of medical records since the study's conclusion indicates that while the rate is still elevated, the cluster appears to have subsided. (Source: EurekAlert! - Social and Behavioral Science)

ARTICLE: Case-control Study of a Gastroschisis Cluster in Nevada

Archives of Pediatrics — Mon, 02 Nov 2009 21:01:14 +0100

Conclusions These findings add strength to the hypothesis that use of methamphetamine and related drugs is a risk factor for gastroschisis and raise questions about the risks associated with infections. (Source: Archives of Pediatrics)

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Case-control Study of a Gastroschisis Cluster in Nevada [Article]

Archives of Pediatrics — Mon, 02 Nov 2009 21:01:14 +0100

Staged reduction of gastroschisis using preformed silos: practicalities and problems

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

Conclusions: Staged reduction of gastroschisis with PFS is simple, convenient, and safe. The low rates of associated complications and mortality appear favorable when compared to infants managed with more traditional techniques. We recommend that PFS should be used for the routine management of gastroschisis. (Source: Journal of Pediatric Surgery)

The Exstrophy-epispadias complex

BioMed Central — Fri, 30 Oct 2009 00:00:00 +0100

Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate an...

Outcomes in Neonates with Gastroschisis in U.S. Children's Hospitals

American Journal of Perinatology — Thu, 29 Oct 2009 15:58:42 +0100

We examined descriptive characteristics and conducted multivariate regression models examining risk factors for mortality, during the birth hospitalization, and sepsis. Analysis of 2490 neonates with gastroschisis found 90 deaths (3.6%) and sepsis in 766 (31%). Critical comorbidities and procedures are cardiovascular defects (15%), pulmonary conditions (5%), intestinal atresia (11%), intestinal resection (12.5%), and ostomy formation (8.3%). Factors associated with mortality were large bowel resection (odds ratio [OR] 8.26, 95% confidence interval [CI] 1.17 to 58.17), congenital circulatory (OR 5.62, 95% CI 2.11 to 14.91), and pulmonary (OR 8.22, 95% CI 2.75 to 24.58) disease, and sepsis (OR 3.87, 95% CI 1.51 to 9.91). Factors associated with sepsis include intestinal ostomy (OR 2.94, 95% ...

Disruption of calreticulin-mediated cellular adhesion signaling in the cadmium-induced omphalocele in the chick model

Pediatric Surgery International — Thu, 29 Oct 2009 06:56:53 +0100

Conclusion Downregulation of CRT, E-cadherin and β-catenin genes may cause omphalocele in the Cd chick model by disrupting CRT-mediated Ca2+ signaling and AJs. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-009-2505-9Authors Takashi Doi, Our Lady’s Children’s Hospital The Children’s Research Centre Dublin 12 IrelandPrem Puri, Our Lady’s Children’s Hospital The Children’s Research Centre Dublin 12 IrelandJohn Bannigan, University College Dublin School of Medicine and Medical Science, Conway Institute of Biomolecular and Biomedical Research Dublin IrelandJennifer Thompson, University College Dublin School of Medicine and Medical Science, Conway Institute of Biomolecular and Biomedical Research Dublin Ireland Journal Pediatric ...

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Hydrogel protection: a novel approach to reduce bowel inflammation in experimental gastroschisis

European Journal of Obstetrics, Gynecology, and Reproductive Biology — Wed, 28 Oct 2009 00:00:00 +0100

Abstract: Objective: In gastroschisis there is herniation of the fetal bowel into the amniotic cavity that results in severe intestinal dysfunction. In order to reduce bowel exposure to amniotic fluid we used a hydrogel of N-isopropylacrylamide copolymerized with acrylic acid (P(NIPAAm-co-AAc)) to coat the herniated bowel through the use of a fibrin adhesive (Beriplast®).Study design: Gastroschisis was created in fetuses of 31 pregnant Sprague–Dawley rats by evisceration of the bowel through a right paramedian incision in the abdominal wall on day 18.5 of pregnancy. The fetuses were separated in four groups of 12 fetuses: control (C), gastroschisis (G), gastroschisis+fibrin adhesive (GA) and gastroschisis+fibrin adhesive+dry hydrogel (GAH). Animals were harvested at day 21.5 of pregnanc...

Modified silo technique—An easy and effective method to improve the survival rate of neonates with gastroschisis in Shanghai

European Journal of Obstetrics, Gynecology, and Reproductive Biology — Mon, 05 Oct 2009 00:00:00 +0100

Conclusions: Placing a spring-loaded silo bag as a bedside procedure without anesthesia on newborns with severe gastroschisis whose viscera cannot be reduced primarily has increased the survival rate (94.1%, 16/17, 2004–2008) of infants with severe gastroschisis in comparison to our previous experience (60.9%, 14/23, 1996–2003, p=0.026, Chi-square test) in Shanghai, China. This modified procedure deviates from the classic silo procedure, which is carried out under anesthesia.. (Source: European Journal of Obstetrics, Gynecology, and Reproductive Biology)

Concomitant Craniorachischisis and Omphalocele in a Male Fetus: Prenatal Magnetic Resonance Imaging Findings and Literature Review

Taiwanese Journal of Obstetrics and Gynecology — Thu, 01 Oct 2009 10:44:40 +0100

Objective: To present the prenatal magnetic resonance imaging (MRI) findings of concomitant craniorachischisis and omphalocele, review the literature, and discuss the pathogenesis. Case Report: A 20-year-old, gravida 2, para 0, woman was re ...more (Source: Taiwanese Journal of Obstetrics and Gynecology)

Outcomes of sutureless gastroschisis closure

Journal of Pediatric Surgery — Wed, 30 Sep 2009 23:00:00 +0100

Conclusion: Sutureless closure of uncomplicated gastroschisis is a safe technique that reduces length of intubation and does not significantly alter the time required to reach full enteral feeds or hospital discharge. (Source: Journal of Pediatric Surgery)

Gastroschisis Associated with an Intra-Abdominal Umbilical Vein Varix: A Report of 2 Cases

Karger Publishers — Tue, 29 Sep 2009 23:00:00 +0100

Fetal Diagn Ther 2009;25:404-406 (DOI:10.1159/000236155) (Source: Karger Publishers)

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Has the liver and other visceral organs migrated to its normal position in children with giant omphalocele? A follow-up study with ultrasonography

European Journal of Pediatrics — Tue, 29 Sep 2009 04:05:10 +0100

Conclusion In all former patients with a giant omphalocele, an abnormal position of the liver and in the majority of them, an incisional hernia was also found. The liver and sometimes also the spleen and the kidneys do not migrate to their normal position. Exact documentation and good information are important for both the patient and their caretakers in order to avoid liver trauma. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00431-009-1068-zAuthors Floortje Clemens van Eijck, Radboud University Nijmegen Medical Centre Department of Surgery, Division of Paediatric Surgery Nijmegen The NetherlandsWillemijn M. Klein, Radboud University Nijmegen Medical Centre Department of Radiology Nijmegen The NetherlandsCarla Boetes, Academic Hospital Maastricht D...

Clinical management of motility disorders in children

Seminars in Pediatric Surgery — Mon, 28 Sep 2009 17:21:49 +0100

We review the current clinical evaluation and management of the most common esophageal and gastrointestinal motility disorders in children based on the literature and our experience in a pediatric motility center in the United States. The disorders discussed include esophageal achalasia, pre- and post-fundoplication motility disorders, gastroparesis, motility disorders occurring after repair of congenital atresias, motility disorders associated with gastroschisis, chronic intestinal pseudo-obstruction, motility after intestinal transplantation, motility disorders after colonic resection for Hirschsprung's disease, chronic functional constipation, and motility disorders associated with imperforate anus. (Source: Seminars in Pediatric Surgery)

Teratogens inducing congenital abdominal wall defects in animal models

Pediatric Surgery International — Wed, 16 Sep 2009 18:48:06 +0100

Abstract Congenital abdominal wall defects are common anomalies which include gastroschisis, omphalocele and umbilical cord hernia. Recent reports indicate that gastroschisis is increasing in prevalence, whereas omphalocele has remained steady, suggesting that environmental factors may play a part in their pathogenesis. The aim of this study is to review animal teratogen studies resulting in abdominal wall defects to investigate their possible causes. Each report was examined not only for the teratogens causing the defects, but also to carefully identify the defect occurring and its correlation with the known clinical anomalies. We found many discrepancies between the nomenclature used by animal teratology investigators and that used by clinicians. We were able to confirm ...

Vitamin A deficiency in an infant with PAGOD syndrome

American Journal of Medical Genetics Part A — Tue, 15 Sep 2009 23:00:00 +0100

We describe an infant with PAGOD syndrome phenotype. The patient had a normal male karyotype and no copy number changes were seen on chromosome genomic hybridization (CGH) microarray. Endocrine evaluation was consistent with primary hypogonadism. The testes and Müllerian structures were absent by imaging studies, raising the possibility of arrest of early gonadogenesis. The plasma free vitamin A was low, consistent with moderate to severe vitamin A deficiency; the maternal plasma vitamin A level was normal. During pregnancy maternal vitamin A is taken up by retinol binding protein 4 (RBP4) which is expressed in the embryonic visceral endoderm from pregastrulational stages. This transport is mediated via the specific membrane receptor for RBP, stimulated by retinoic acid 6 (STRA6). STRA6 i...

Descriptive epidemiology of nonsyndromic omphalocele in Texas, 1999-2004

American Journal of Medical Genetics Part A — Tue, 15 Sep 2009 23:00:00 +0100

Omphalocele is a congenital malformation that involves protrusion of abdominal contents into the umbilicus. Though omphalocele may present as a manifestation of several chromosomal and Mendelian syndromes, the etiology for nonsyndromic omphalocele is unknown. The present study sought to estimate the birth prevalence of nonsyndromic omphalocele in offspring of women residing in Texas from 1999 to 2004, and to describe prevalence as a function of parental and infant demographic characteristics. Data on 325 cases with nonsyndromic omphalocele and 2,208,758 live births delivered during 1999-2004 were obtained from the Texas Birth Defects Registry. These data were used to estimate omphalocele birth prevalence and obtain both crude and adjusted prevalence ratios for the association of nonsyndrom...

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Dichorionic Diamniotic Twin Pregnancy Discordant for Bladder Exstrophy

Advances in Urology — Sun, 13 Sep 2009 17:24:43 +0100

A 38 year-old woman presented with a dichorionic diamniotic twin pregnancy at gestational age of 32 weeks concerning for an abdominal wall mass in one of the twins. Initial ultrasound evaluation was suspicious for an omphalocele, but the affected twin was found to have bladder exstrophy at birth. This illustrates the difficulties of accurate prenatal diagnosis of bladder exstrophy in a twin pregnancy at a late gestation. (Source: Advances in Urology)

[PAUL procedure : A new biocompatible concept for the therapy of congenital abdominal wall defects.]

Der Chirurg — Fri, 11 Sep 2009 23:00:00 +0100

Authors: Meyer T, Seifert A, Meyer B, Ulrichs K, Germer CT Treatment of small congenital abdominal wall defects (e.g. omphalocele and gastroschisis) can be performed by direct closure. In large defects non-resorbable artificial materials (e.g. Gore-Tex(R)) are necessary to close the fascia. The aim of this study was to find out whether a new procedure, the PAUL pocedure, might be suitable for the treatment of large abdominal wall defects. A full thickness abdominal wall defect was created in young Wistar Rats. These defects were then closed by implantation of a 1x2 cm sized piece of PTFE (Dual-Mesh(R)), a polypropylene mesh (Prolene(R)) or by using the PAUL procedure. Over a period of 6 weeks no wound infections or hernias were monitored. In contrast to PTFE the PAUL procedure showed o...

Early high C-reactive protein in infants with open abdominal wall defects does not predict sepsis or adverse outcome

Acta Paediatrica — Mon, 07 Sep 2009 23:00:00 +0100

Conclusion: Infants with gastroschisis exhibit high early CRP, which may not indicate sepsis or adverse outcome. This increase can complicate the assessment of these infants. Clinicians should be aware of this finding as it could inform management decisions in this group. (Source: Acta Paediatrica)

Predicting adverse neonatal outcomes in fetuses with abdominal wall defects using prenatal risk factors

American Journal of Obstetrics and Gynecology — Sun, 30 Aug 2009 23:00:00 +0100

Conclusion: Intrauterine growth restriction and exteriorization of the liver are significant predictors of adverse neonatal outcome with gastroschisis and omphalocele. (Source: American Journal of Obstetrics and Gynecology)

Maternal obesity and morbid obesity: The risk for birth defects in the offspring

Birth Defects Research Part A: Clinical and Molecular Teratology — Wed, 26 Aug 2009 23:00:00 +0100

CONCLUSION: The risk for a morbidly obese pregnant woman to have an infant with a congenital birth defect is small, but for society the association is important in the light of the ongoing obesity epidemic. Birth Defects Research (Part A), 2009. © 2009 Wiley-Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

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Spontaneous sutureless closure of the abdominal wall defect in gastroschisis using a commercial wound retractor system

Pediatric Surgery International — Mon, 24 Aug 2009 16:51:49 +0100

Conclusion The cosmetic appearance of the abdomen is improved using our technique compared with primary closure involving suturing and a conventional silo. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-009-2450-7Authors Yuki Ogasawara, Juntendo University School of Medicine Department of Pediatric General and Urogenital Surgery 2-1-1, Hongo, Bunkyo-ku Tokyo 113-8421 JapanTadaharu Okazaki, Juntendo University School of Medicine Department of Pediatric General and Urogenital Surgery 2-1-1, Hongo, Bunkyo-ku Tokyo 113-8421 JapanYoshifumi Kato, Juntendo University School of Medicine Department of Pediatric General and Urogenital Surgery 2-1-1, Hongo, Bunkyo-ku Tokyo 113-8421 JapanGeoffrey J. Lane, Juntendo University School of Medicine Department o...

Intrauterine Growth Patterns in Fetal Gastroschisis

American Journal of Perinatology — Tue, 18 Aug 2009 06:19:12 +0100

The objective of this study was to evaluate patterns of intrauterine growth in fetal gastroschisis. This was a retrospective review of prenatally diagnosed cases of fetal gastroschisis delivered at the University of North Carolina Hospital from January 2000 to January 2007. Fetal growth (biparietal diameter, head circumference, abdominal circumference, femur length, and estimated fetal weight) and amniotic fluid volume were evaluated by gestational age. Gastroschisis was diagnosed in 83 pregnancies; outcomes were available in 71 fetuses. The mean gestational age at diagnosis was 17 weeks and 1 day. The mean gestational age at delivery was 35 weeks and 4 days. Mean birth weight was 2306 g. As early as the second trimester, all morphometric measures demonstrated impaired in utero growth, wit...

Prenatal diagnosis and postnatal outcome of fetal spinal defects without Arnold-Chiari II malformation

Prenatal Diagnosis — Sun, 16 Aug 2009 23:00:00 +0100

To determine the prenatal evolution/natural history and postnatal outcome of fetuses diagnosed with a neural tube defect (NTD) lacking the Arnold-Chiari-II malformation (ACM II).This retrospective study reviewed 16 fetuses evaluated with ultrasound (US) and MRI at a single referral center from 1/2000 to 8/2007. Follow-up studies and available postnatal outcomes were reviewed.Postpartum diagnosis was terminal myelocystoceles 7/16 (44%); myelomeningoceles (MMCs) 3/16 (19%); lipomyelomeningoceles 2/16(13%); and thoracic myelocystocele 1/16 (6%). Three patients (19%) were lost to follow-up or termination of pregnancy. Two prenatally diagnosed 'closed' NTD were postnatally found to be MMCs. Three of the myelocystoceles had additional omphalocele, bladder extrophy, imperforate anus and spinal de...

Maternal nativity as a risk factor for gastroschisis: A population-based study

Birth Defects Research Part A: Clinical and Molecular Teratology — Wed, 29 Jul 2009 23:00:00 +0100

CONCLUSIONS: Although young maternal age remains a strong significant risk factor for gastroschisis in Florida, other factors such as maternal race/ethnicity and nativity could be important in explaining the increasing prevalence of gastroschisis. Birth Defects Research (Part A), 2009. © 2009 Wiley-Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Catheter enterostomy and patch repair of the abdominal wall for gastroschisis with intestinal atresia: Report of a case

Surgery Today — Tue, 28 Jul 2009 18:46:50 +0100

Abstract A male infant, weighing 2177 g, was born with the entire intestine protruding through a defect on the right side of the navel. Intestinal atresia, approximately 70 cm from the Treitz ligament, was also confirmed. Primary anastomosis and abdominal wall repair were impossible because of the intestinal dilation and thick peel, as well as the small abdominal cavity. Thus, we initially performed catheter enterostomy with a 14-F balloon catheter and patch repair of the abdominal wall, to enable the baby to be fed. Secondary anastomosis and abdominal wall repair was safely performed when the baby was 106 days old. The combination of catheter enterostomy and patch repair of the abdominal wall does not require dissection of the intestine and it can be safely performed in l...

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Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study

Journal of Pediatric Surgery — Tue, 28 Jul 2009 11:48:03 +0100

Conclusions: Our results suggest a moderately increased risk of gastroschisis among women who used tobacco, alcohol, and ibuprofen during early pregnancy. A modestly elevated risk was observed for omphaloceles among women who used alcohol during the first trimester and among women who were heavy smokers. (Source: Journal of Pediatric Surgery)

46, XY female with cloacal exstrophy and masculinization at puberty

Journal of Pediatric Urology — Thu, 09 Jul 2009 23:00:00 +0100

We report the case of a 17-year-old girl with a history of 46, XY cloacal exstrophy and gender reassignment presenting with an ectopic testis of unclear location. We then review controversial literature surrounding gender assignment in these patients. (Source: Journal of Pediatric Urology)

Re: "maternal asthma medication use and the risk of gastroschisis"

American Journal of Epidemiology — Wed, 08 Jul 2009 23:00:00 +0100

(Source: American Journal of Epidemiology)

Completely Separated Scrotum and Vesicointestinal Fistula Without Exstrophy as a Novel Manifestation of Aphallia: A Case Report

Urology — Wed, 08 Jul 2009 00:00:00 +0100

A male infant born at 32 weeks of gestation and weighing 1.44 kg had penile agenesis, bifid scrotums, an anorectal anomaly, and suspected omphalocele. An operation revealed complete urethral agenesis, duplicated and blind bowels, a vesicointestinal fistula without exstrophy, and a urachal fistula; however, no omphaloceles were detected. To our knowledge, this is the first report on aphallia accompanied with completely separated scrotum and a vesicointestinal fistula without exstrophy; this condition is associated with urorectal septum malformation sequence (URSMS) and covered cloacal exstrophy. The possible embryogenesis is discussed in this study. (Source: Urology)

Delayed, life-threatening lower gastrointestinal hemorrhage in an infant after serial transverse enteroplasty: treatment with transcatheter n-butyl-2-cyanoacrylate embolization

Pediatric Radiology — Tue, 07 Jul 2009 15:22:47 +0100

We report a case of n-butyl-2-cyanoacrylate glue embolization of life-threatening lower gastrointestinal (LGI) hemorrhage in a 10-month-old boy. The child had a history of gastroschisis and short-bowel syndrome. Six months prior to the LGI bleed, he had undergone a serial transverse enteroplasty (STEP) to lengthen his intestine. To the best of our knowledge this is both the first report of successful glue embolization for LGI bleeding in a child and also the first report of severe hemorrhage after the STEP procedure. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00247-009-1328-0Authors Conor O. Bogue, The Hospital for Sick Children Division of Image Guided Therapy, Department of Diagnostic Imaging 555 University Ave. Toronto M5G 1X8 CanadaAmin I. Alzahrani, The Hospi...

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Minor and giant omphalocele: long-term outcomes and quality of life

Journal of Pediatric Surgery — Tue, 30 Jun 2009 23:00:00 +0100

Conclusions: Our study indicates that after a high level of medical intervention perinatally, quality of life is good to very good in both groups and comparable to healthy young adults. (Source: Journal of Pediatric Surgery)

Delivery of gastroschisis patients before 37 weeks of gestation is associated with increased morbidities

Journal of Pediatric Surgery — Tue, 30 Jun 2009 23:00:00 +0100

Conclusions: Preterm delivery should be avoided because there is no clear benefit to the gut in avoiding derivative injuries. Meticulous attention should be given to the nutritional needs of patients with gastroschisis. (Source: Journal of Pediatric Surgery)

Omphalocele: how big does it have to be a giant one?

Journal of Pediatric Surgery — Tue, 30 Jun 2009 23:00:00 +0100

We read with great interest the recent article by Kumar et al showing the significant differences in the frequency of associated anomalies between small and large omphaloceles. Children were divided in 2 groups, depending on the size of the abdominal wall opening: small (≤4 cm) or large omphaloceles (>4 cm). As the authors emphasized, determination of the omphalocele's size was based on a nonstandardized method of measurement made by initial physical examination and/or at operation. (Source: Journal of Pediatric Surgery)

RE: Response to Letter to the Editor by Campos et al

Journal of Pediatric Surgery — Tue, 30 Jun 2009 23:00:00 +0100

I would like to thank the authors for their comments regarding our recent article “Impact of omphalocele size on associated conditions” . As they very eloquently point out and defend with the published literature, our profession has yet to offer scientifically based definitions to either designation of small or large omphaloceles. With this lack of standardization in the segregation of children with this anomaly, the corresponding published literature does not lend itself to straightforward conclusions that can be generalized to treatment or expected outcomes. In the end, the clinical designation may very well be based on the suitability of the defect for primary closure or not, yet the successful use of prosthetics in closure may confound this naive simplification. (Source: Journal of...

Maternal age-specific risk of non-chromosomal anomalies.

BJOG : An International Journal of Obstetrics and Gynaecology — Sat, 27 Jun 2009 20:01:41 +0100

CONCLUSIONS: Clinical and public health interventions are needed to reduce environmental risk factors for NCA, giving special attention to young mothers among whom some risk factors are more prevalent. Reassurance can be given to older mothers that their age in itself does not confer extra risk for NCA. PMID: 19485989 [PubMed - in process] (Source: BJOG : An International Journal of Obstetrics and Gynaecology)

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The Outcome of Closing Gastroschisis: Two Case Reporta and Literature Review

European Journal of Pediatric Surgery — Thu, 25 Jun 2009 08:25:32 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0029-1216380© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

Left-sided Gastroschisis: Is it the Same Pathology as on the Right-Side?

European Journal of Pediatric Surgery — Thu, 25 Jun 2009 08:25:32 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0029-1216341© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

[Original articles] Prognosis in isolated gastroschisis with bowel dilatation: a systematic review

Archives of Disease in Childhood - Fetal and Neonatal Edition — Thu, 18 Jun 2009 23:00:00 +0100

Conclusion: Current available evidence suggests that fetuses with isolated gastroschisis and bowel dilatation are not at increased risk of adverse perinatal outcome compared to those without bowel dilatation. However, there is a paucity of studies, and a randomised controlled trial is urgently needed. (Source: Archives of Disease in Childhood - Fetal and Neonatal Edition)

ORIGINAL ARTICLE: The Use of a Spring-Loaded Silo for Gastroschisis: Impact on Practice Patterns and Outcomes

Archives of Surgery — Sun, 14 Jun 2009 23:00:00 +0100

Conclusions The use of an SLS placed at the bedside has resulted in lower immediate fascial closure rates for infants with gastroschisis without significant detrimental clinical outcome. The main benefit of using the bedside-placed SLS is the avoidance of urgent surgical intervention. For patients undergoing delayed fascial closure, use of the bedside SLS resulted in shorter times to definitive fascial closure. (Source: Archives of Surgery)

The impact of iatrogenic gastroschisis on pulmonary maturation in the fetal rabbit models of congenital diaphragmatic hernia

Pediatric Surgery International — Sat, 13 Jun 2009 08:38:14 +0100

Conclusions In the fetal rabbit models of CDH, pulmonary hypoplasia is the most significant pathological feature. Iatrogenic gastroschisis does not improve pulmonary maturation due to the active growth of the liver that herniates into the thoracic cavity. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-009-2384-0Authors Gong Chen, FUDAN University Shanghai ChinaShan Zheng, FUDAN University Shanghai ChinaXian Min Xiao, FUDAN University Shanghai ChinaYi Luo, FUDAN University Shanghai China Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

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Omphalocele minor associated with complete absence of the large bowel

Pediatric Surgery International — Thu, 11 Jun 2009 13:07:44 +0100

We present an infant born with such combination of congenital anomalies. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00383-009-2397-8Authors Atakelet Ferede, Our Lady’s Children’s Hospital Crumlin IrelandFarhan Tareen, Our Lady’s Children’s Hospital Crumlin IrelandJohn Gillick, Our Lady’s Children’s Hospital Crumlin Ireland Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

Management of colonic atresia with primary resection and anastomosis

Pediatric Surgery International — Thu, 11 Jun 2009 13:07:43 +0100

Conclusions Uncomplicated colonic atresia can be managed by primary repair with little morbidity. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-009-2401-3Authors Melvin Dassinger, Arkansas Children’s Hospital Little Rock USARichard Jackson, Arkansas Children’s Hospital Little Rock USASamuel Smith, Arkansas Children’s Hospital Little Rock USA Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

Prevalence of gastroschisis and associated hospital time continue to rise in neonates who are admitted for intensive care

Journal of Pediatric Surgery — Sun, 31 May 2009 23:00:00 +0100

Conclusion: The prevalence and the number of hospital days needed to care for neonates with gastroschisis have continued to increase since 2001. (Source: Journal of Pediatric Surgery)

Conflicts in wound classification of neonatal operations

Journal of Pediatric Surgery — Sun, 31 May 2009 23:00:00 +0100

Conclusions: There is poor consensus on how neonatal operations are classified based on contamination. An improved classification system will provide more accurate risk assessment for development of surgical site infections and identify neonates who would benefit from antibiotic prophylaxis. (Source: Journal of Pediatric Surgery)

Letter to the Editor

Journal of Pediatric Surgery — Sun, 31 May 2009 23:00:00 +0100

We read with interest the article regarding closing gastroschisis by Estrada et al . We would agree with their conclusion that the prolapsed midgut should be preserved wherever possible and would like to present a similar case we have recently managed to highlight this. (Source: Journal of Pediatric Surgery)

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Reply to Letter to the Editor by Evans

Journal of Pediatric Surgery — Sun, 31 May 2009 23:00:00 +0100

We thank you for the opportunity to reflect on the comments made by Dr Stephen Evans re: closing gastroschisis. In his letter, he describes a management scheme very similar to ours in caring for a neonate with a closing gastroschisis. Our first experience with preservation of the amorphous mass occurred 8 years ago. At that time, we were not aware of any other similar successful cases of intestinal preservation using this technique. In that baby, the internalized mass was later found to represent more than 40 cm of viable intestine, enabling the baby to become total parenteral nutrition–independent within the first year of life. (Source: Journal of Pediatric Surgery)

Ward reduction of gastroschisis in a single stage without general anaesthesia may increase the risk of short-term morbidities: Results of a retrospective audit

Journal of Paediatrics and Child Health — Thu, 28 May 2009 04:00:00 +0100

Conclusions: These results raise concerns over the role of ward reduction of gastroschisis in a single sitting without the use of GA. Randomised trials with appropriate design and sample size are needed before embracing this method as a standard practice. (Source: Journal of Paediatrics and Child Health)

Current perspectives on pediatric intestinal transplantation

Current Gastroenterology Reports — Thu, 21 May 2009 06:02:42 +0100

Abstract Irreversible intestinal failure in children is predominantly caused by surgical conditions such as volvulus, necrotizing enterocolitis, and gastroschisis. Functional intestinal failure from motility disorders such as intestinal pseudo-obstruction or enterocyte dysfunction with microvillus inclusion disease also may require intestine replacement. Approved indications for intestinal transplantation include liver dysfunction, loss of major venous access, frequent central line-related sepsis, and recurrent episodes of severe dehydration despite intravenous fluid management. Surgical options include transplantation of the isolated intestine, combined liver-intestine transplantation, or multivisceral transplantation of the stomach, duodenum, pancreas, and small bowel (w...

Neonatal Outcome of Gastroschisis Is Mainly Influenced by Nutritional Management.

Journal of Pediatric Gastroenterology and Nutrition — Sun, 03 May 2009 20:47:51 +0100

Page: 612DOI: 10.1097/MPG.0b013e31818c5281Authors: Walter-Nicolet, Elizabeth *; Rousseau, Veronique +; Kieffer, Francois ++; Fusaro, Fabio +; Bourdaud, Nathalie +; Oucherif, Salima [S]; Benachi, Alexandra [S]; Sarnacki, Sabine +; Mitanchez, Delphine [P] (Source: Journal of Pediatric Gastroenterology and Nutrition)

Is timing everything? The influence of gestational age, birth weight, route, and intent of delivery on outcome in gastroschisis

Journal of Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

Conclusions: Gestational age, BW, and conformity to an antenatal birth plan are predictors of outcome in gastroschisis, whereas actual route of delivery is not. (Source: Journal of Pediatric Surgery)

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Predicting the outcome of newborns with gastroschisis

Journal of Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

Conclusion: Prematurity, nonelective silo, GI complications, and non-GI anomalies predict the short-term outcome of newborns with gastroschisis. Prenatal ultrasound serves primarily to predict the absence of GI complications. (Source: Journal of Pediatric Surgery)

Relationship between serum citrulline levels and progression to parenteral nutrition independence in children with short bowel syndrome

Journal of Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

Conclusion: Plasma CIT levels are strong predictors of PN independence in children with SBS and correlate well with a patient's recorded bowel length. A cutoff CIT level of 15 μmol/L may serve as a prognostic measure in counseling patients regarding the likelihood of future PN independence. (Source: Journal of Pediatric Surgery)

Journal of Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

There are some evidence to suggest that careful antenatal monitoring, scheduled preterm delivery, and immediate abdominal wall closure may reduce gastroschisis morbidity. We hypothesised that the advantages of a scheduled preterm delivery balance possible complications related to prematurity. A retrospective study was performed including all cases of gastroschisis born between 1990 and 2004 (n = 69). Cases were categorised in two groups. Group 1 contained gastroschisis cases born between 1990 and 1997. Group 2 contained cases occurring since 1997, when a new management pathway for gastroschisis was established: weekly evaluation of the foetal gut by ultrasound (>28 weeks), corticosteroids, and delivery by scheduled caesarean section at 35 weeks (before if evidence of bowel compromise was p...

Journal of Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

Non-operative management of gastroschisis also known as plastic closure (PC) has been described as an alternative to conventional primary operative closure (POC) or staged silo closure (SSC). The aim of this study was to compare these techniques in neonates with gastroschisis. A retrospective review of neonates with gastroschisis who underwent PC was undertaken. The minimum follow-up was 1 year. Premature neonates ( (Source: Journal of Pediatric Surgery)

Intestinal venous congestion as a complication of elective silo placement for gastroschisis

Seminars in Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

Use of a spring-loaded silastic silo has been advocated as a means of gentle reduction of the herniated bowel, while avoiding the possible complications of primary closure of gastroschisis. We recently encountered intestinal venous congestion during elective silo reduction of gastroschisis. Two babies with gastroschisis were treated postdelivery with a spring-loaded silo placed under the fascial defect and the eviscerated bowel suspended within the silo. Patient #1 had no bowel matting. On day of life 2, the bowel within the silo was noted to be dusky. The silo was removed, and the bowel was indeed congested, but viable. Complete reduction with a modified Bianchi closure was performed at the bedside. Patient #2 had severe matting of the bowel and did not require intubation for silo placeme...

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Sour grapes: Ultrasound abnormalities spurred delivery and neonatal surgery

American Journal of Obstetrics and Gynecology — Sun, 26 Apr 2009 23:00:00 +0100

An 18-year-old woman, gravida 1, para 0, underwent sonographic evaluation at 22 weeks' gestation, because of a maternal serum alpha-fetoprotein (AFP) level of 9.76 MoM. Imaging revealed an abdominal wall defect to the right of the umbilical cord insertion, a finding consistent with fetal gastroschisis. No other anomalies were identified. (Source: American Journal of Obstetrics and Gynecology)

Intestinal loop lengthening: early treatment of vanishing bowel

Pediatric Surgery International — Sun, 26 Apr 2009 05:50:17 +0100

Abstract Bianchi’s procedure experience for short bowel syndrome in children is positive. This technique is generally performed after the first year of life. Here the authors propose a case of gastroschisis with prenatal spontaneous closure of abdominal defect and vanishing gut presenting as intestinal atresia, absence of ileo-cecal valve, and residual short intestinal dilatation, treated by early gut lengthening (ILP). Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00383-009-2359-1Authors A. Buluggiu, Hôpital Timone Enfant Department of Pediatric Surgery 264, Rue Saint-Pierre 13385 Marseille Cedex 05 FranceM. Haddad, Hôpital Timone Enfant Department of Pediatric Surgery 264, Rue Saint-Pierre 13385 Marseille Cedex 05 FranceM. Coste, Hôpital Timone Enfa...

Meckel's diverticulum complicating neonatal umbilical cord clamping

International Journal of Gynaecology and Obstetrics — Tue, 21 Apr 2009 20:23:25 +0100

A male neonate weighing 3838 g was born at 39 weeks of gestation by elective cesarean delivery due to breech presentation. The umbilical cord was clamped by the surgeon approximately 20 cm from the umbilical base and the neonate was transferred to the midwife for final clamping. The cord was clamped again at approximately 3 cm from the umbilical base. The residual umbilical cord was approximately 4 cm in diameter. Immediately after final clamping, meconium appeared in the umbilical stump. This clinical finding led to the presumptive diagnosis of omphalocele. Ultrasound examinations during pregnancy had not demonstrated any sign of abnormality around the umbilicus. The neonate was transferred in a stable condition to the pediatric surgical unit for immediate operative exploration of th...

Bladder augmentation in a young adult female exstrophy patient with associated omphalocele: An extremely unusual case

Journal of Pediatric Urology — Sun, 19 Apr 2009 23:00:00 +0100

We present the case of a 20-year-old woman with uncorrected bladder exstrophy and omphalocele treated with ileocystoplasty and continent urinary stoma. To our knowledge this is the first reported case of a young adult patient presenting with both congenital anomalies. The treatment result suggests that bladder preservation is a safe and feasible therapeutic option in bladder exstrophy. (Source: Journal of Pediatric Urology)

Supernumerary Kidney in a Child With OEIS Complex

Urology — Tue, 14 Apr 2009 23:00:00 +0100

We present an exceedingly rare case of a supernumerary third kidney discovered by antegrade nephrostography of a 6-month-old child with OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). (Source: Urology)

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Outcome of fetuses with a prenatal ultrasound diagnosis of isolated omphalocele

Prenatal Diagnosis — Tue, 14 Apr 2009 04:00:00 +0100

To assess the outcome following an ultrasound diagnosis of isolated fetal omphalocele (normal karyotype).Ultrasound database (1988-2002) was searched for omphalocele. Categories assigned as (1) isolated - no additional structural/karyotype anomalies, (2) associated minor abnormality, and (3) major structural/karyotype abnormalities.Ultrasound identified 65 cases, 13 with isolated omphalocele and 6 with associated minor anomalies. These 19 cases represented 29.2% of the population (19/65); isolated omphalocele was confirmed in 14 neonates (14/19, 73.7%). In five neonates, unrecognized by prenatal ultrasound were cardiac anomalies (2 cases), tracheoesophageal atresia with cardiac anomaly (1 case), and Beckwith-Wiedemann syndrome (BWS) (2 cases). A minor ultrasound finding did not predict fur...

Intestinal venous congestion as a complication of elective silo placement for gastroschisis.

Seminars in Pediatric Surgery — Thu, 09 Apr 2009 13:45:18 +0100

Authors: Ryckman J, Aspirot A, Laberge JM, Shaw K Use of a spring-loaded silastic silo has been advocated as a means of gentle reduction of the herniated bowel, while avoiding the possible complications of primary closure of gastroschisis. We recently encountered intestinal venous congestion during elective silo reduction of gastroschisis. Two babies with gastroschisis were treated postdelivery with a spring-loaded silo placed under the fascial defect and the eviscerated bowel suspended within the silo. Patient #1 had no bowel matting. On day of life 2, the bowel within the silo was noted to be dusky. The silo was removed, and the bowel was indeed congested, but viable. Complete reduction with a modified Bianchi closure was performed at the bedside. Patient #2 had severe matting of the...

Gastroschisis and absence of the septum pellucidum: an accidental association?

Prenatal Diagnosis — Tue, 07 Apr 2009 04:00:00 +0100

No Abstract. (Source: Prenatal Diagnosis)

Escape of the yolk sac: a hypothesis to explain the embryogenesis of gastroschisis.

Clinical Genetics — Tue, 31 Mar 2009 23:00:00 +0100

Authors: Stevenson RE, Rogers RC, Chandler JC, Gauderer MW, Hunter AG Gastroschisis is a significant birth defect that in many countries has shown an increased prevalence in recent decades, and the change has affected primarily younger mothers. Despite numerous epidemiological studies no other consistent associated risk factor has been identified. In this paper we review the five main theories related to the pathogenesis of this malformation and outline the reasons why we think none fully explains the embryogenesis of gastroschisis. We briefly present some clinical observations we have made that we consider germane to the pathogenesis and outline a hypothesis that we think can account for the origins of this malformation. Our proposal is that the determining defect in gastroschisis is ...

Gastroschisis: etiology and developmental pathogenesis.

Clinical Genetics — Tue, 31 Mar 2009 23:00:00 +0100

Authors: Jones KL, Benirschke K, Chambers CD PMID: 19419414 [PubMed - in process] (Source: Clinical Genetics)

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Gastroschisis with jejunal and colonic atresia, and isolated colonic atresia in dichorionic, diamniotic twins

Pediatric Surgery International — Tue, 24 Mar 2009 07:08:05 +0100

We present the first case of dichorionic, diamniotic male twins in which there was gastroschisis with jejunal and colonic atresia in Twin A and isolated colonic atresia in Twin B. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00383-009-2353-7Authors Matthew A. Saxonhouse, University of Florida College of Medicine Division of Neonatology, Department of Pediatrics P.O. Box 100296 Gainesville FL 32610-0296 USADavid W. Kays, University of Florida College of Medicine Division of Pediatric Surgery, Department of Surgery Gainesville FL USADavid J. Burchfield, University of Florida College of Medicine Division of Neonatology, Department of Pediatrics P.O. Box 100296 Gainesville FL 32610-0296 USARoger Hoover, University of Florida College of Medicine Division of Biomedical Med...

Exomphalos and gastroschisis

Continuing Education in Anaesthesia, Critical Care — Mon, 16 Mar 2009 04:00:00 +0100

(Source: Continuing Education in Anaesthesia, Critical Care)

Gastroschisis: a third world perspective

Pediatric Surgery International — Sat, 14 Mar 2009 13:15:41 +0100

Conclusion The prevalence of gastroschisis amongst neonatal surgical admissions appears to be increasing. Most babies were “outborn” resulting in delays in diagnosis and referral for surgical management. Despite the availability of NICU and total parenteral nutrition the mortality remains high. Reduction in mortality will depend upon improvements in antenatal diagnosis, primary care and transportation, as well as a reduction in postsurgical sepsis. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-009-2348-4Authors J. Sekabira, University of KwaZulu-Natal Department of Paediatric Surgery, Nelson R Mandela School of Medicine Durban South AfricaG. P. Hadley, University of KwaZulu-Natal Department of Paediatric Surgery, Nelson R Mandela School ...

Risk stratification in gastroschisis: can prenatal evaluation or early postnatal factors predict outcome?

Pediatric Surgery International — Wed, 11 Mar 2009 15:11:53 +0100

Conclusions Prenatal bowel wall dilation and/or thickness did not predict complex patients or adverse outcome. Complex gastroschisis patients can be identified postnatally and have substantial morbidity. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-009-2342-xAuthors Ryan P. Davis, University of Michigan Hospitals, Mott Children’s Hospital F3970 Section of Pediatric Surgery, Department of Surgery Box 0245 Ann Arbor MI 48109 USAMarjorie C. Treadwell, The University of Michigan Medical School, The C. S. Mott Children’s Hospital Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology Ann Arbor MI USARobert A. Drongowski, University of Michigan Hospitals, Mott Children’s Hospital F3970 Section of Pediatric Surgery, Depar...

Recurrent Septo-Optic Dysplasia Accompanied by Omphalocele: A Case Report

Karger Publishers — Fri, 06 Mar 2009 13:36:42 +0100

Fetal Diagn Ther 2009;25:119-122 (DOI:10.1159/000207450) (Source: Karger Publishers)

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Rising incidence of gastroschisis and exomphalos in New Zealand

Journal of Pediatric Surgery — Sun, 01 Mar 2009 05:00:00 +0100

Conclusion: The incidence of gastroschisis and exomphalos is increasing in New Zealand, which is consistent with worldwide trends in showing the increasing incidence of anterior abdominal wall defects. (Source: Journal of Pediatric Surgery)

Gastroschisis: effect of mode of delivery

Journal of Pediatric Surgery — Sun, 01 Mar 2009 05:00:00 +0100

Recent issues of the Journal of Pediatric Surgery have presented 2 articles examining the effect of mode of delivery on the outcomes of infants with gastroschisis, each with diametrically different conclusions. Before commenting, I believe that basic principles regarding any outcome comparisons should be outlined. (Source: Journal of Pediatric Surgery)

Reply

Journal of Pediatric Surgery — Sun, 01 Mar 2009 05:00:00 +0100

We thank Dr White for the interest and comments about our recent work regarding the mode of delivery and neonatal survival of infants with gastroschisis in Australia and New Zealand . Dr White proposed reanalysis of cases according to elective prelabor and include simple gastroschisis alone. The main aim of our article was to provide evidence to determine if mode of delivery was associated with neonatal mortality and hospital morbidities. In our population cohort of 631 infants with gastroschisis, the elective cesarean deliveries were indeed prelabor. Dr White may have missed this important distinction in our report. The median (interquartile) gestation at delivery for this elective prelabor cesarean delivery group was 36 (35-37) weeks. This is comparable to the group studied by their stud...

Reply to Letter to the Editor

Journal of Pediatric Surgery — Sun, 01 Mar 2009 05:00:00 +0100

I would like to thank Dr John White for his constructive comments regarding our article on early cesarean delivery in gastroschisis recently published in the Journal of Pediatric Surgery (J Pediatr Surg 2008;43:1342-1346.) (Source: Journal of Pediatric Surgery)

Association of gastroschisis and choledochal cyst

Journal of Pediatric Surgery — Sun, 01 Mar 2009 05:00:00 +0100

Abstract: Two infants are described who presented in the neonatal period with a direct hyperbilirubinemia. This was initially presumed to be because of the diagnosis of gastroschisis and the prolonged use of parenteral nutrition. However, both infants were eventually found to have an associated choledochal cyst. The cases are a novel association not previously reported and should heighten the awareness that anatomical causes of a direct hyperbilirubinemia need to be ruled out in all neonates. (Source: Journal of Pediatric Surgery)

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Sutureless Delayed Primary Gastroschisis Repair with Negative Pressure Dressing

European Journal of Pediatric Surgery — Thu, 19 Feb 2009 03:02:38 +0100

Eur J Pediatr SurgDOI: 10.1055/s-2008-1038643© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

Left-sided gastroschisis with meromelia of the limbs: A rare association

Congenital Anomalies — Wed, 18 Feb 2009 05:00:00 +0100

Gastroschisis is a malformation of the anterior abdominal wall that presents as herniation of abdominal contents through a paramedian abdominal defect, usually to the right side of the midline. The defect involves all the layers of the abdominal wall and there is no membranous covering. In newborns with isolated gastroschisis, the overall prognosis is very good. In rare cases, gastroschisis is associated with other congenital malformations, making the prognosis worse. We herein report a rare case of left-sided gastroschisis associated with meromelia of the limbs. (Source: Congenital Anomalies)

REVIEW: Maternal Overweight and Obesity and the Risk of Congenital Anomalies: A Systematic Review and Meta-analysis

JAMA — Tue, 10 Feb 2009 05:00:00 +0100

Conclusions Maternal obesity is associated with an increased risk of a range of structural anomalies, although the absolute increase is likely to be small. Further studies are needed to confirm whether maternal overweight is also implicated. (Source: JAMA)

Obesity During Pregnancy Associated With Increased Risk of Birth Defects

Diabetes News from dLife.com — Tue, 10 Feb 2009 05:00:00 +0100

February 10, 2009 (Newswire) - For women who are obese during pregnancy there is an associated increased risk of certain birth defects, such as spina bifida and neural tube defects, although the absolute increase in risk is likely to be small, according to an analysis of previous studies, reported in the February 11 issue of JAMA. Obesity is a major public health and economic concern. In the United States, a third of women age 15 years and older were obese (body mass index [BMI] greater than 30) in 2004. There are significant health implications of prepregnancy maternal obesity for both mother and child. There is evidence that suggests that maternal obesity may be associated with the development of some congenital anomalies (abnormality present at birth). Congenital anomalies are a lea...

Indications for induction of labour: a best-evidence review

BJOG: An International Journal of Obstetrics and Gynaecology — Wed, 04 Feb 2009 05:00:00 +0100

Background Rates of labour induction are increasing.Objectives To review the evidence supporting indications for induction.Search strategy We listed indications for labour induction and then reviewed the evidence. We searched MEDLINE and the Cochrane Library between 1980 and April 2008 using several terms and combinations, including induction of labour, premature rupture of membranes, post-term pregnancy, preterm prelabour rupture of membranes (PROM), multiple gestation, suspected macrosomia, diabetes, gestational diabetes mellitus, cardiac disease, fetal anomalies, systemic lupus erythematosis, oligohydramnios, alloimmunization, rhesus disease, intrahepatic cholestasis of pregnancy (IHCP), and intrauterine growth restriction (IUGR). We performed a review of the literature supporting each ...

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Indications for induction of labour: a best-evidence review.

BJOG : An International Journal of Obstetrics and Gynaecology — Wed, 04 Feb 2009 05:00:00 +0100

Authors: Mozurkewich E, Chilimigras J, Koepke E, Keeton K, King V Background Rates of labour induction are increasing. Objectives To review the evidence supporting indications for induction. Search strategy We listed indications for labour induction and then reviewed the evidence. We searched MEDLINE and the Cochrane Library between 1980 and April 2008 using several terms and combinations, including induction of labour, premature rupture of membranes, post-term pregnancy, preterm prelabour rupture of membranes (PROM), multiple gestation, suspected macrosomia, diabetes, gestational diabetes mellitus, cardiac disease, fetal anomalies, systemic lupus erythematosis, oligohydramnios, alloimmunization, rhesus disease, intrahepatic cholestasis of pregnancy (IHCP), and intrauterine growth rest...

Closing gastroschisis: diagnosis, management, and outcomes

Journal of Pediatric Surgery — Sun, 01 Feb 2009 05:00:00 +0100

Conclusions: Of infants with GS, 6% present with closing abdominal ring. Close antenatal monitoring may prevent severe bowel loss in some cases. After multiple surgical interventions, most have a favorable outcome. (Source: Journal of Pediatric Surgery)

Esophageal obstruction and intracardiac “mass” in association with omphalocele closure

Journal of Pediatric Surgery — Sun, 01 Feb 2009 05:00:00 +0100

We report 2 such complications in an infant after repair of a giant omphalocele that contained both liver and bowel. Esophageal obstruction resulted from sharp, anterior angulation of the distal esophagus that hindered placement of a feeding tube in the neonatal period. At 1 year of age, routine cardiac evaluation led to the discovery of a “mass” within the heart caused by invagination of a tongue of liver tissue into the right atrium. Knowledge of these less well-recognized anatomical variations is important to avoid unnecessary interventions. (Source: Journal of Pediatric Surgery)

Is the prevalence of gastroschisis increasing in selected US states?

Journal of Pediatric Surgery — Sun, 01 Feb 2009 05:00:00 +0100

The prevalence of gastroschisis is reported to be increasing in the United Kingdom and worldwide as reported by Keys et al who call the phenomenon an epidemic. In the United States, there have also been reports of rising gastroschisis rates from various individual states ; however, there is little information regarding the national trend of gastroschisis prevalence over time. Recent US national prevalence gastroschisis estimates for 1999 to 2001, using population-based data obtained by active surveillance, is 3.73 per 10,000 live births (LB) . The etiology of this rising prevalence remains unknown with as yet no definitive implication of a genetic or environmental factor. Although there have been reports of rising gastroschisis, the increasing trend is not considered a universal phenomenon...

Preformed silastic silos in the management of necrotizing enterocolitis.

The American Surgeon — Sun, 01 Feb 2009 05:00:00 +0100

Authors: Moore AB, Nakayama DK Necrotizing enterocolitis (NEC) is sometimes complicated by abdominal compartment syndrome, a clinical syndrome characterized by multiple organ dysfunction that arises as a consequence of increased intra-abdominal pressure. The evolving clinical picture of NEC sometimes requires "second-look" operations done after initial abdominal exploration to more accurately gauge the optimal extent of surgery. Placing intestines in a preformed, spring-loaded, transparent Silastic silo, traditionally used in the staged treatment of gastroschisis, addresses both situations: decompression of the abdomen and allowing periodic inspection of the intestines. Standard silos were used in three infants with advanced (Bell Class 3) NEC without perforation before definitive surg...

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Sex reversal and hypoplastic left heart syndrome

The Journal of Thoracic and Cardiovascular Surgery — Tue, 27 Jan 2009 00:00:00 +0100

We report the case of a patient with sex reversal and hypoplastic left heart syndrome without congenital diaphragmatic hernia who has survived to Fontan completion and is doing well. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Torsion of an accessory lobe of the liver in a child: Report of a case

Surgery Today — Fri, 09 Jan 2009 11:41:26 +0100

We report a case of torsion of an accessory lobe of the liver (ALL) in a 14-year-old girl. The patient was admitted for acute abdominal pain and laparotomy revealed an ALL, the pedicle of which was elongated with 180° torsion. The diagnosis was not made preoperatively because of the rarity of this condition. However, a close relationship between omphalocele repair and the development of ALL has been reported; thus, a history of omphalocele repair should alert the doctor to the possibility of this condition, which could be suggested by imaging findings preoperatively. Torsion of an ALL should be included in the differential diagnosis of a patient with a history of omphalocele, who presents with acute abdominal symptoms. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s0...

The use of intraabdominal tissue expanders as a primary strategy for closure of giant omphaloceles

Journal of Pediatric Surgery — Thu, 01 Jan 2009 05:00:00 +0100

Conclusions: We believe that early use of intraabdominal tissue expanders provides a more expedient method of obtaining closure of the defect in giant omphaloceles. (Source: Journal of Pediatric Surgery)

The association of cyclic parenteral nutrition and decreased incidence of cholestatic liver disease in patients with gastroschisis

Journal of Pediatric Surgery — Thu, 01 Jan 2009 05:00:00 +0100

Conclusions: Prophylactic cyclic PN is associated with a decreased incidence and prolonged time to onset of hyperbilirubinemia. Other factors, however, significantly affect this relationship. Prospective randomized investigation is warranted to investigate for a possible causal relationship. (Source: Journal of Pediatric Surgery)

Ruptured fetal omphalocele complicated by midgut volvulus with strangulation

Journal of Pediatric Surgery — Thu, 01 Jan 2009 05:00:00 +0100

We describe here the case of a ruptured omphalocele in utero complicated by volvulus, which eventually led to short bowel syndrome. (Source: Journal of Pediatric Surgery)

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MR Imaging of Fetal Omphalocele: A Case Report.

Magnetic Resonance in Medical Sciences : MRMS — Sun, 28 Dec 2008 14:14:42 +0100

We report a case of a fetal omphalocele in which the internal structure of the hernia sac was imaged in detail by magnetic resonance (MR) imaging. The hernia sac consisted of amnion, Wharton jelly, and externally, peritoneum. The umbilical vascular channels (2 arteries, one vein) were surrounded by Wharton jelly. Use of MR imaging facilitates a more accurate evaluation of fetal omphalocele with regard to hernial sac, intestinal structure, and differential diagnosis. PMID: 19110516 [PubMed - as supplied by publisher] (Source: Magnetic Resonance in Medical Sciences : MRMS)

Isolated fetal omphalocele, Beckwith-Wiedemann syndrome, and assisted reproductive technologies

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 23 Dec 2008 05:00:00 +0100

CONCLUSIONS: Beckwith-Wiedemann syndrome is present in a noteworthy portion of fetuses with isolated omphalocele on ultrasound, and prenatal molecular studies are warranted. Even among this small cohort, a recurring theme of conception by assisted reproduction exists. Birth Defects Research (Part A), 2009. © 2008 Wiley-Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Gastroschisis.

Paediatric Anaesthesia — Thu, 18 Dec 2008 14:29:24 +0100

Authors: Mayhew JF, Mychaskiw G PMID: 19076507 [PubMed - in process] (Source: Paediatric Anaesthesia)

[Intestinal obstruction in children due to isolated intestinal malrotation. Report of 11 cases.]

Archives de Pediatrie — Wed, 17 Dec 2008 05:00:00 +0100

CONCLUSION: These findings provide support for performing early diagnosis of intestinal malrotation to prevent the rare but potentially devastating complications of this anomaly. PMID: 19097872 [PubMed - as supplied by publisher] (Source: Archives de Pediatrie)

A review of Donnai-Barrow and facio-oculo-acoustico-renal (DB/FOAR) syndrome: Clinical features and differential diagnosis

Birth Defects Research Part A: Clinical and Molecular Teratology — Wed, 17 Dec 2008 03:48:53 +0100

Mutations in the gene LRP2 have recently been identified as the cause of Donnai-Barrow and Facio-oculo-acoustico-renal (DB/FOAR) syndrome. More than two dozen cases, the first reported more than 30 years ago by Holmes, have been published. Summarizing available information, we highlight the cardinal features of the disorder found in [ge]90% of published cases. These features include: agenesis of the corpus callosum, developmental delay, enlarged anterior fontanelle, high myopia, hypertelorism, proteinuria, and sensorineural hearing loss. Congenital diaphragmatic hernia and omphalocele are reported in only half of the patients. There is no evidence for genotype-phenotype correlation, though the sample size is too small to preclude this with certainty. Although several conditions to consider...

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Successful vaginal delivery following laparoscopic abdominal wall reconstruction in an adult survivor of an omphalocele without prior surgical repair: report of a case

Hernia — Tue, 16 Dec 2008 07:57:46 +0100

We report the case of a successful vaginal delivery following laparoscopic abdominal wall reconstruction in an adult survivor of an omphalocele without prior surgical repair. Untreated omphaloceles are rare in adulthood. A 30-year-old female patient presented with a large anterior abdominal wall defect due to an untreated omphalocele, who expressed a desire to have a baby in the near future. A laparoscopic herniorrhaphy was performed with a double-layered expanded polytetrafluoroethylene (ePTFE, Gore-Tex) mesh. The patient delivered a full-term healthy baby vaginally 2 years after surgical repair of the omphalocele. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10029-008-0456-zAuthors W. B. Kim, Korea University Department of Surgery, Korea University Medical C...

[Seasonality of Omphalocele in Northern Germany.]

Ultraschall in der Medizin — Fri, 12 Dec 2008 05:00:00 +0100

CONCLUSION: Since we were unable to demonstrate a relationship between the time of conception and the incidence of fetal Omphalocele in our cohort of pregnant women from a predominantly agricultural background, other factors should be investigated for possible associations with the onset of Omphalocele. PMID: 19085743 [PubMed - as supplied by publisher] (Source: Ultraschall in der Medizin)

Gastroschisis: Multicenter Trial of a SILASTIC Silo

AAP Grand Rounds — Tue, 02 Dec 2008 05:00:00 +0100

(Source: AAP Grand Rounds)

Impact of omphalocele size on associated conditions

Journal of Pediatric Surgery — Thu, 27 Nov 2008 19:45:38 +0100

Conclusion: Small omphalocele size correlates with an increased prevalence of associated gastrointestinal anomalies, a lower prevalence of cardiac anomalies, and a higher predominance of male sex. (Source: Journal of Pediatric Surgery)

Preservation of extracorporeal tissue in closing gastroschisis augments intestinal length

Journal of Pediatric Surgery — Thu, 27 Nov 2008 19:45:37 +0100

Conclusion: In this series, we show that this amorphous tissue, when preserved, may exhibit normal intestinal architecture and absorptive function. Therefore, such remnant tissue should be preserved as it may significantly increase bowel length and minimize parenteral nutrition requirement. (Source: Journal of Pediatric Surgery)

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Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity

Journal of Pediatric Surgery — Thu, 27 Nov 2008 19:45:36 +0100

Conclusions: More than one third of patients with GS/IA appear to have significant intestinal dysmotility without true SBS nor obstruction. Successful treatment of these infants may be achieved with the use of tapering enteroplasty and/or temporary diverting stomas. (Source: Journal of Pediatric Surgery)

Experience with a hybrid, minimally invasive gastrostomy for children with abnormal epigastric anatomy

Journal of Pediatric Surgery — Thu, 27 Nov 2008 19:45:20 +0100

Conclusion: Using a very small incision, this hybrid method permits safe and precise gastric and abdominal wall site selection and gastrostomy catheter placement. Gastrotomy as well as purse-string and peritoneal fixation sutures are not needed, and the danger of accidental catheter dislodgement is minimized. (Source: Journal of Pediatric Surgery)

Prevalence, prenatal diagnosis and survival of gastroschisis

Prenatal Diagnosis — Thu, 27 Nov 2008 05:00:00 +0100

To describe the prevalence, associated anomalies, prenatal diagnosis and survival of cases of gastroschisis.Data were extracted from the Northern Congenital Abnormality Survey (NorCAS) for cases delivered during 1997 to 2006.A total of 143 cases of gastroschisis were notified from 310 410 registered births. Ten (7%) gastroschisis cases were associated with other structural anomalies. The male to female ratio among isolated gastroschisis cases was 1:1.1 and 4:1 among non-isolated cases. The total prevalence for isolated cases was 4.28 per 10 000 births (95% CI 3.56-5.01), and the overall live birth prevalence was 4.21 per 10 000 live births (95% CI 3.49-4.93). There was an increase in total prevalence during the ten years, although this increase did not reach statistical significance ([chi]...

Changes in Intestinal Waste Products during the Antenatal Management of Gastroschisis by Serial Amniotic Fluid Exchange and Infusion

Fetal Diagnosis and Therapy — Tue, 25 Nov 2008 15:18:51 +0100

Fetal Diagn Ther 2008;24:448-451 (DOI:10.1159/000176298) (Source: Fetal Diagnosis and Therapy)

The joint effects of maternal prepregnancy body mass index and age on the risk of gastroschisis

Paediatric and Perinatal Epidemiology — Tue, 04 Nov 2008 05:00:00 +0100

Siega-Riz AM, Herring AH, Olshan AF, Smith J, Moore C, The National Birth Defects Prevention Study. The joint effects of maternal prepregnancy body mass index and age on the risk of gastroschisis. Paediatric and Perinatal Epidemiology 2008. Young maternal age has been associated with an increased risk of gastroschisis, while high maternal weight status has been associated with a decreased risk. We were interested in investigating the joint effect of these two risk factors to identify thresholds in risk associated with body mass index (BMI) for a given age. Data from the National Birth Defects Prevention Study included 464 case infants with gastroschisis and 4842 healthy controls. A generalised additive model with a bivariate spline for continuous maternal age and prepregnancy BMI was used ...

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Birth defects cluster study: A national approach to birth defects cluster investigations

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 04 Nov 2008 05:00:00 +0100

CONCLUSIONS: The Birth Defects Cluster Study offers a unique and effective approach to cluster investigations that improves the likelihood of identifying genetic and environmental causes of birth defects and provides a model for cluster investigations of other noninfectious health outcomes. Birth Defects Research (Part A), 2008. Published 2008 Wiley-Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

A case control population-based study investigating smoking as a risk factor for gastroschisis in Utah, 1997-2005

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 04 Nov 2008 05:00:00 +0100

CONCLUSIONS: Though first trimester cigarette smoking was reported on birth certificates by more mothers of gastroschisis cases than controls, adjustment for confounders (maternal age and preconception BMI) and smoking misclassification suggests the association is weak. Despite a decrease in smoking prevalence among all women of childbearing years in Utah between 1997 and 2005, the prevalence of gastroschisis has not followed a similar trend. Birth Defects Research (Part A), 2008. © 2008 Wiley-Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Parental age as a risk factor for isolated congenital malformations in a Polish population

Paediatric and Perinatal Epidemiology — Mon, 03 Nov 2008 05:00:00 +0100

Materna-Kiryluk A, Wiśniewska K, Badura-Stronka M, Mejnartowicz J, Wi[ecedil]ckowska B, Balcar-Boroń A, Czerwionka-Szaflarska M, Gajewska E, Godula-Stuglik U, Krawczyński M, Limon J, Rusin J, Sawulicka-Oleszczuk H, Szwalkiewicz-Warowicka E, Walczak M, Latos-Bieleńska A. Parental age as a risk factor for isolated congenital malformations in a Polish population. Paediatric and Perinatal Epidemiology 2008. Currently available data on the relationship between the prevalence of isolated congenital malformations and parental age are inconsistent and frequently divergent. We utilised the data from the Polish Registry of Congenital Malformations (PRCM) to accurately assess the interplay between maternal and paternal age in the risk of isolated non-syndromic congenital m...

Downregulation of ROCK-I and ROCK-II gene expression in the cadmium-induced ventral body wall defect chick model

Pediatric Surgery International — Tue, 28 Oct 2008 06:13:44 +0100

Conclusions Our results provide evidence, for the first time, that the gene expression levels of ROCK-I and ROCK-II are significantly downregulated at 4 h after treatment of Cd in the VBW defect model of chick embryo. We speculate that the downregulation of ROCK-I and ROCK-II gene expressions during this narrow window of embryogenesis may cause VBW defect by disrupting Wnt non-canonical pathway. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-008-2270-1Authors Takashi Doi, Our Lady’s Children’s Hospital The Children’s Research Centre Dublin 12 IrelandPrem Puri, Our Lady’s Children’s Hospital The Children’s Research Centre Dublin 12 IrelandJohn Bannigan, University College Dublin School of Medicine and Medical Science Dublin Ir...

Intestinal Edema: Effect of Enteral Feeding on Motility and Gene Expression

Journal of Surgical Research — Mon, 27 Oct 2008 00:00:00 +0100

Conclusions: Intestinal edema results in impaired small intestinal transit and globally increased gene expression. Early enteral nutrition improves edema-induced impaired transit and minimizes gene transcriptional activity. (Source: Journal of Surgical Research)

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Possible association of Down syndrome and exstrophy–epispadias complex: report of two new cases and review of the literature

European Journal of Pediatrics — Thu, 16 Oct 2008 09:57:24 +0100

Abstract In the past, several midline defects have been associated with Down syndrome (DS) on a regular basis, e.g. heart defects, cleft lip and palate, neural tube defects, omphalocele and anal atresia. The exstrophy–epispadias complex (EEC) represents a rare midline defect, rarely described in association with DS. Here, we report on the co-occurrence of DS and EEC in two, so far, unreported cases and present a review of the literature. We suggest that EEC represents a rare but inherent part in the spectrum of DS-associated midline defects. Content Type Journal ArticleCategory Short ReportDOI 10.1007/s00431-008-0852-5Authors Heiko Reutter, University of Bonn Institute of Human Genetics Wilhelmstr. 31 53111 Bonn GermanyArend Bökenkamp, Vrije Universiteit Medical Cent...

Challenges of giant ventral hernia repair in children in an African tertiary care center with limited resources

Hernia — Tue, 14 Oct 2008 11:04:20 +0100

Conclusion Despite the challenges, better results were achieved with repairs in infants/children compared to repairs performed in neonates. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s10029-008-0439-0Authors O. D. Osifo, University of Benin Teaching Hospital (UBTH) Pediatric Surgery Unit, Department of Surgery Benin City NigeriaA. C. Efobi, University of Benin Teaching Hospital (UBTH) Pediatric Surgery Unit, Department of Surgery Benin City Nigeria Journal HerniaOnline ISSN 1248-9204Print ISSN 1265-4906 (Source: Hernia)

Routine use of a SILASTIC spring-loaded silo for infants with gastroschisis: a multicenter randomized controlled trial

Journal of Pediatric Surgery — Wed, 01 Oct 2008 04:00:00 +0100

Conclusion: Routine use of a preformed silo was associated with similar outcomes to primary closure for infants with gastroschisis but with a strong trend toward fewer days on the ventilator. Use of a preformed silo has the advantage of permitting definitive abdominal wall closure in a more elective setting. (Source: Journal of Pediatric Surgery)

What Other Abnormalities are Associated with Prune Belly Syndrome?

PediatricEducation.org — Tue, 23 Sep 2008 12:45:19 +0100

Discussion Prune Belly syndrome is characterized by various anatomical urinary tract anomalies, cryptorchism and deficiency of abdominal wall musculature. It has a characteristic distended abdomen with wrinkled skin which gives rise to the name. This syndrome was first described in 1839 by Frohlich. In 1950 Eagle and Barrett described 9 cases and later other physicians suggested Eagle-Barrett syndrome as an alternative eponym. The incidence is approximately 1:40,000 live births. Females can have an incomplete form of the syndrome but obviously cannot have cryptorchism. They may have other genital abnormalities such as vaginal agenesis, hydrocolpos, and bicornuate uterus. The severity varies from lethality in utero, to children with abnormal musculature and undescended testes but with nor...