MedWorm: Abdominal Wall Repair

[Liver torsion leading to death in a 16-month-old infant treated neonatally for an omphalocele.]

Archives de Pediatrie — Thu, 02 Feb 2012 05:00:00 +0100

We report the case of an infant who underwent surgery on the first day of life for a giant omphalocele. At the age of 16 months, he presented an acute abdominal syndrome and died a few hours later. Autopsy revealed a twisted left liver lobe (LL) including a suprahepatic vein. To our knowledge, this is the first case of giant omphalocele complicated by twisted liver lobe and fatal outcome. PMID: 22305089 [PubMed - as supplied by publisher] (Source: Archives de Pediatrie)

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Maternal Asthma Medication Use and the Risk of Selected Birth Defects

PEDIATRICS — Wed, 01 Feb 2012 05:00:00 +0100

CONCLUSIONS: Positive associations were observed for anorectal atresia, esophageal atresia, and omphalocele and maternal periconceptional asthma medication use, but not for other defects studied. It is possible that observed associations may be chance findings or may be a result of maternal asthma severity and related hypoxia rather than medication use. (Source: PEDIATRICS)

First‐trimester detection of structural abnormalities and the role of aneuploidy markers

Ultrasound in Obstetrics and Gynecology — Fri, 27 Jan 2012 08:35:08 +0100

ConclusionAbout half of major structural abnormalities can be diagnosed in the first trimester. Increased nuchal translucency or abnormal ductus venosus blood flow appear to be associated with cardiac and skeletal defects and may facilitate early detection. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

The jury is still out: changes in gastroschisis management over the last decade are associated with both benefits and shortcomings

Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

Conclusion: Change in our management strategy has resulted in prolonged intensive care unit stay and time to full feeds but reduced postoperative hernias and wound infections. (Source: Journal of Pediatric Surgery)

Long-term neurodevelopmental outcomes in children born with gastroschisis: the tiebreaker

Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions: Children born with gastroschisis have similar 2-year neurodevelopmental outcomes as nonsurgical, nonsyndromic neonatal intensive care unit children of similar gestational age and birth weight. Both groups of children have a higher rate of enrollment in early intervention than their healthy peers. These data suggest that neurodevelopmental outcomes in gastroschisis children are delayed secondary to prematurity rather than the presence of the surgical disease. (Source: Journal of Pediatric Surgery)

Fetoscopic management of gastroschisis in a lamb model

Surgical Endoscopy — Sat, 17 Dec 2011 06:43:34 +0100

Conclusions Although technically demanding, we were able to produce and reassess six cases of gastroschisis by fetoscopy. As primary repositioning appears unfavorable, fetoscopic prosthetic bag placement may become an alternative. Content Type Journal ArticlePages 1-5DOI 10.1007/s00464-011-2048-8Authors Robert Bergholz, Department of Pediatric Surgery, UKE: University Hospital Eppendorf, Altona Children’s Hospital, Bleickenallee 38, 22767 Hamburg, GermanyThomas Krebs, Department of Pediatric Surgery, UKE: University Hospital Eppendorf, Altona Children’s Hospital, Bleickenallee 38, 22767 Hamburg, GermanyKatharina Wenke, Department of Pediatric Surgery, UKE: University Hospital Eppendorf, Altona Children’s Hospital, Bleickenallee 38, 22767 Hamburg, GermanyThomas ...

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Suspected opioid-induced hyperalgesia in an infant

British Journal of Anaesthesia — Mon, 12 Dec 2011 05:00:00 +0100

We report a case of OIH in an infant with gastroschisis, requiring multiple surgical interventions and prolonged sedation for ventilation. This is the first report of OIH in an infant. On day 41 of life after nine separate surgical interventions, the patient's pain scores increased and remained elevated, despite increasing opioid administration. The patient also developed hyperalgesia, allodynia, and photophobia and became extremely irritable upon handling. Other possible causes were excluded, including interruption to opioid delivery, sepsis, acid–base and electrolyte disturbance, and ongoing surgical pathology. An opioid rotation to hydromorphone was initiated and ketamine was commenced. Sedation for ventilation was achieved with dexmedetomidine and midazolam infusions. Over a peri...

Fetal Gastroschisis: A Comparison of Second vs. Third-Trimester Bowel Dilatation for Predicting Bowel Atresia and Neonatal Outcomes.

Ultraschall in der Medizin — Fri, 09 Dec 2011 05:00:00 +0100

CONCLUSION: Fetuses with isolated gastroschisis successfully underwent postnatal surgery in most cases (93.2 %), except for one termination, one intrauterine death and 3 cases of neonatal death. A fetal bowel dilatation > 10 mm before 30 + 0 weeks had the highest predictive value for postnatal bowel complications. PMID: 22161619 [PubMed - as supplied by publisher] (Source: Ultraschall in der Medizin)

Infectious complications in the management of gastroschisis

Pediatric Surgery International — Wed, 07 Dec 2011 12:54:42 +0100

Conclusion Infectious complications remain an important consideration in the management of gastroschisis. GPS correlates with the development of infectious complications. Prophylaxis for skin flora and early closure, when feasible, may reduce WI rates. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s00383-011-3038-6Authors Robert Baird, Division of Pediatric Surgery, McGill University Health Center, The Montreal Children’s Hospital, McGill University, 2300 Tupper Street, Room C812, Montreal, QC H3H 1P3, CanadaPramod Puligandla, Division of Pediatric Surgery, McGill University Health Center, The Montreal Children’s Hospital, McGill University, 2300 Tupper Street, Room C812, Montreal, QC H3H 1P3, CanadaErik Skarsgard, Division of Pediatri...

Persistent pupillary membrane, strabismus, and optic nerve hypoplasia in Donnai-Barrow syndrome

Journal of AAPOS — Thu, 01 Dec 2011 05:00:00 +0100

We report a case of Donnai-Barrow syndrome in a 2-year-old boy who presented with exotropia and prominent eyes since birth. Ocular findings included high myopia, persistent pupillary membrane, and optic nerve hypoplasia. Facial manifestations included broad nasal bridge, hypertelorism, and downward-slanting of palpebral fissures. The patient had associated omphalocele, sensorineural hearing loss, and developmental delay. Magnetic resonance imaging revealed agenesis of the corpus callosum. To our knowledge, this is the first reported association of persistent pupillary membrane, strabismus, and optic nerve hypoplasia in a patient with Donnai-Barrow syndrome. (Source: Journal of AAPOS)

Folate and vitamin B12-related genes and risk for omphalocele

Human Genetics — Thu, 24 Nov 2011 17:44:18 +0100

Abstract Both taking folic acid-containing vitamins around conception and consuming food fortified with folic acid have been reported to reduce omphalocele rates. Genetic factors are etiologically important in omphalocele as well; our pilot study showed a relationship with the folate metabolic enzyme gene methylenetetrahydrofolate reductase (MTHFR). We studied 169 non-aneuploid omphalocele cases and 761 unaffected, matched controls from all New York State births occurring between 1998 and 2005 to look for associations with single nucleotide polymorphisms (SNPs) known to be important in folate, vitamin B12, or choline metabolism. In the total study population, variants in the transcobalamin receptor gene (TCblR), rs2232775 (p.Q8R), and the MTHFR gene, rs1801131 (c.1298A>C)...

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Early prenatal sonographic diagnosis of gastroschisis

Journal of Clinical Ultrasound — Mon, 21 Nov 2011 05:00:00 +0100

We report a case of gastroschisis that was suspected at sonography at 11 weeks + 3 days of amenorrhea. Although early detection does not alter prenatal management of this condition nor does it affect neonatal outcome, it allows early counseling ofthe parents.©2011 Wiley Periodicals, Inc. J Clin Ultrasound, 2011 (Source: Journal of Clinical Ultrasound)

Iatrogenic arteriovenous fistula in an infant: Diagnostic and therapeutic considerations

Journal of Clinical Ultrasound — Mon, 21 Nov 2011 05:00:00 +0100

We report the case of a 3‐month‐old infant with complicated gastroschisis requiring multiple venipunctures who presented with signs and symptoms suggestive of traumatic arteriovenous fistula. Ultrasound imaging confirmed the presence of a wide‐necked AVF between the brachial artery and vein. The fistula was surgically repaired. This case report describes the clinical imaging and treatment options for infants with iatrogenic AVF. © 2011 Wiley Periodicals, Inc. J Clin Ultrasound, 2011 (Source: Journal of Clinical Ultrasound)

Preclosure Fluid Resuscitation Influences Outcome in Gastroschisis

American Journal of Perinatology — Thu, 17 Nov 2011 05:00:00 +0100

Amer J PerinatolDOI: 10.1055/s-0031-1295639Optimal preclosure fluid resuscitation in gastroschisis (GS) is unknown. The purpose of our study was to evaluate effects of preclosure intravenous fluid resuscitation on GS outcome. Cases were accrued from a national GS database. Risk variables analyzed included gestational age (GA), birth weight (BW), neonatal illness severity score, and bolus fluid administration within 6 hours of neonatal intensive care unit admission. Outcomes analyzed included closure success, days of ventilation/total parenteral nutrition (TPN), and bacteremic episodes. Linear and logistic regression analyses were performed. Four hundred seven live-born GS cases were identified (362 with complete resuscitative fluids data). Mean BW, GA, and Score for Neonatal Acute Physiolo...

Predictors of postnatal outcome in neonates with gastroschisis

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusion: Outcome in our patients was favorable as measured by survival, LOS, and days on PN. Primary predictors of poor outcome were factors related to short bowel syndrome and secondary closure, indicating a need to further improve treatment of short bowel syndrome. (Source: Journal of Pediatric Surgery)

Liver herniation in gastroschisis: incidence and prognosis

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions: Liver herniation was found in 6% of patients with gastroschisis and was associated with a high rate of mortality. Liver herniation appears to be a risk factor for poor outcome in gastroschisis. Documentation of liver herniation may be helpful in prenatal consultation for gastroschisis. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

The authors examined the patient records to evaluate the effect of immediate repair and other factors on the outcome of gastroschisis in the last 12 years. Data of 87 newborns were evaluated specifically to determine the effect of the place of repair (obstetric hospital delivery room) vs children's hospital time of repair less than 1 hour after delivery or more than 1 hour, and type of repair (primary fascial repair and skin closure [PR] vs staged repair [SR]). (Source: Journal of Pediatric Surgery)

Umbilical artery pulsatility index and fetal abdominal circumference in isolated gastroschisis

Ultrasound in Obstetrics and Gynecology — Thu, 27 Oct 2011 08:53:02 +0100

ConclusionsIn fetal gastroschisis, it is well known that AC tends to be smaller, though UA‐PI has not been reported to be abnormal in any consistent way. There is a clear relationship between the fetus's AC for gestation and UA‐PI, which is not the case for normally grown fetuses. The data suggest that the growth restriction seen in gastroschisis may be explained by hypoxia, and not simply by the classical explanation of extra‐abdominal displacement of the abdominal viscera. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Is complex gastroschisis predictable by prenatal ultrasound?

BJOG: An International Journal of Obstetrics and Gynaecology — Fri, 21 Oct 2011 04:00:00 +0100

Conclusions Intra‐abdominal bowel dilatation is the only ultrasound marker predictive of complex GS but it is a strong marker. (Source: BJOG: An International Journal of Obstetrics and Gynaecology)

ATXN1 Protein Family and CIC Regulate Extracellular Matrix Remodeling and Lung Alveolarization.

Developmental Cell — Tue, 18 Oct 2011 04:00:00 +0100

Authors: Lee Y, Fryer JD, Kang H, Crespo-Barreto J, Bowman AB, Gao Y, Kahle JJ, Hong JS, Kheradmand F, Orr HT, Finegold MJ, Zoghbi HY Abstract Although expansion of CAG repeats in ATAXIN1 (ATXN1) causes Spinocerebellar ataxia type 1, the functions of ATXN1 and ATAXIN1-Like (ATXN1L) remain poorly understood. To investigate the function of these proteins, we generated and characterized Atxn1L(-/-) and Atxn1(-/-); Atxn1L(-/-) mice. Atxn1L(-/-) mice have hydrocephalus, omphalocele, and lung alveolarization defects. These phenotypes are more penetrant and severe in Atxn1(-/-); Atxn1L(-/-) mice, suggesting that ATXN1 and ATXN1L are functionally redundant. Upon pursuing the molecular mechanism, we discovered that several Matrix metalloproteinase (Mmp) genes are overexpressed and that the...

Growth restriction in gastroschisis: quantification of its severity and exploration of a placental cause

BMC Pediatrics - Latest articles — Mon, 17 Oct 2011 04:00:00 +0100

Background: Gastroschisis patients are commonly small for gestational age (SGA, birth weight [BW] < 10th centile). However, the extent, symmetry and causes of that growth restriction remain controversial. Methods: We compared BW, crown-heel length (LT), occipitofrontal circumference (OFC) and ponderal index (PI) in 179 gastroschisis cases and 895 matched controls by univariate and multiple regression. Fetal ultrasounds (N= 80) were reviewed to determine onset of growth restriction. Placental histology was examined in 31 gastroschisis patients whose placental tissue was available and in 29 controls. Results: Gastroschisis cases weighed less than controls (BW = 2400 + 502 g vs. 2750 + 532 g, p (Source: BMC Pediatrics - Latest articles)

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Cloacal exstrophy: An epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research

American Journal of Medical Genetics Part C: Seminars in Medical Genetics — Fri, 14 Oct 2011 04:00:00 +0100

AbstractCloacal exstrophy presents as a complex abdominal wall defect thought to result from a mesodermal abnormality. Anatomically, its main components are Omphalocele, bladder Exstrophy and Imperforate anus. Other associated malformations include renal malformations and Spine defects (OEIS complex). Historically, the prevalence ranges from 1 in 200,000 to 400,000 births, with higher rates in females. Cloacal exstrophy is likely etiologically heterogeneous as suggested by its recurrence in families and occurrence in monozygotic twins. The defect has been described in infants with limb‐body wall, with trisomy 18, and in one pregnancy exposed to Dilantin and diazepam. Due to its rarity, the use of a nonspecific diagnostic code for case identification, and lack of validation of the clinica...

Gastroschisis with Ceco‐Appendicular Agenesis: A novel presentation

Congenital Anomalies — Thu, 13 Oct 2011 04:00:00 +0100

We report such a case of gastroschisis associated with ceco – appendicular agenesis. The possible embryological explanation for such a presentation has been discussed. (Source: Congenital Anomalies)

Prenatal findings of omphalocele‐extrophy of the bladder‐imperforate anus‐spinal defects (OEIS) complex

Congenital Anomalies — Thu, 13 Oct 2011 04:00:00 +0100

We describe two cases of OEIS complex diagnosed prenatally by ultrasounds and MRI findings. In both cases, OEIS complex was suspected by conventional sonography in 2nd trimester, and fetal magnetic resonance imaging (MRI) was performed at 27 and 32 weeks each. The conventional sonography revealed low abdominal wall mass, spina bifida, absent bladder and ambiguous genitalia, but those findings were inconclusive. By fetal MRI, we were able to detect the omphalocele, imfraumbilical mass connected to gut tract, absent bladder, ambiguous external genitalia and spinal defect. Our findings suggest that fetal MRI is a useful tool for the prenatal diagnosis of OEIS complex. It helps prenatal counseling and planning of postnatal early treatment strategy. (Source: Congenital Anomalies)

Presenilin genes are downregulated during somitogenesis in the cadmium-induced omphalocele chick model

Pediatric Surgery International — Tue, 11 Oct 2011 05:49:12 +0100

Conclusion We provide evidence, for the first time, that gene expression of presenilins is downregulated during the narrow window of very early embryogenesis in the Cd chick model. Decreased expression of presenilins may contribute to omphalocele phenotype in Cd chick model, by disrupting somite development. Content Type Journal ArticleCategory Original ArticlePages 1-5DOI 10.1007/s00383-011-2994-1Authors Takashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandNaho Fujiwara, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJohn Bannigan, UCD School of Medicine and Medical...

The Incidence of Symptomatic Malrotation Post Gastroschisis Repair

European Journal of Pediatric Surgery — Wed, 05 Oct 2011 04:00:00 +0100

This study examined the incidence of symptomatic malrotation and volvulus following gastroschisis repair.Patients who had undergone gastroschisis repair between 1999 and 2009 in any of 2 tertiary centers were identified using the Hospital Inpatient Enquiry system. The medical records were reviewed to obtain demographic data and postoperative outcomes. Patients were contacted for follow-up.128 patients were identified with a median postoperative follow-up of 4 years (range: 6 weeks to 12 years). Upper gastrointestinal (GI) contrast studies were performed in 30 patients (23.4%), 21 (16.4%) of whom showed evidence of malrotation. Malrotation was documented during the primary repair in 12 patients (9.4%); however Ladd’s procedure was performed primarily in only 3 patients. 7 patients underwe...

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Evaluation of Nitric Oxide (NO) and Nitric Oxide Synthases (NOS) in the Amniotic Fluid in an Experimental Gastroschisis Rat Model

European Journal of Pediatric Surgery — Thu, 29 Sep 2011 04:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1285872Intestinal damage due to gastroschisis (G), an anomaly found with increasing incidence by pedriatic surgeons, is intimately associated with endogenous nitric oxide (NO) production and NO synthase (NOS) expression.Aim of the study was to evaluate NO production and NOS isoforms in the intestine and amniotic fluid (AF) using a rat model of gastroschisis.A gastroschisis rat model was surgically created at 18.5 days of gestation (term=22 days). 3 groups of 12 fetuses each were studied: control (C), sham (S) and (G). Morphometric data of body weight (BW), intestinal weight (IW) and the IW/BW ratio were evaluated and compared. Indirect quantification of NO (nitrite and nitrate – NOx) was analyzed by chemiluminescence, and the expression of the 3 isof...

Birth defects in newborns and stillborns: an example of the Brazilian reality

BMC Research Notes — Fri, 09 Sep 2011 04:00:00 +0100

Conclusions: The current incidence of birth defects among newborns and stillbirths of in our population is similar to those obtained by other studies performed in Brazil and in other underdeveloped countries. Birth defects are one of the major causes leading to lost years of potential life. The study of birth defects in underdeveloped countries should continue. The identification of incidence, risk factors and consequences are essential for planning preventive measures and effective treatments. (Source: BMC Research Notes)

Management of cryptorchidism and gastroschisis

Journal of Pediatric Surgery — Thu, 01 Sep 2011 04:00:00 +0100

Conclusion: The watch-and-wait approach for cryptorchidism in gastroschisis is safe and appropriate, with a high rate of spontaneous migration during the first year of life and greater than 90% testes viable at follow-up. Laparoscopy is a safe and feasible option for management of undescended testes that remain intraabdominal at follow-up. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Thu, 01 Sep 2011 04:00:00 +0100

The ongoing epidemic of gastroschisis has created multiple challenges and continues to raise questions concerning the optimal management of these high-risk patients. Although the overall survival rate has increased over the past 3 decades, morbidity and mortality remain significant. The purpose of this study was to analyze the main factors associated with mortality in neonates admitted to an intensive care unit for the management of this abdominal wall defect. (Source: Journal of Pediatric Surgery)

S175 administration of ropivacaine with low dose ketamine reduces local cytokine expression in operative incision after experimental model gastroschisis

European Journal of Pain Supplements — Thu, 01 Sep 2011 04:00:00 +0100

(Source: European Journal of Pain Supplements)

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Primary Suture-less Closure of Gastroschisis Using Negative Pressure Dressing (Wound Vacuum)

European Journal of Pediatric Surgery — Thu, 25 Aug 2011 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1286254[...]Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Is gastroschisis truly a sporadic defect? Familial cases of gastroschisis in Utah, 1997 to 2008

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 23 Aug 2011 23:00:00 +0100

CONCLUSIONS: We found a statistically significant excess risk for gastroschisis because of familial factors. Similar to many other birth defects, gastroschisis may fit a multifactorial model of inheritance. The UBDN‐UPDB linkage provides a robust approach to investigating genetic factors. Genetic susceptibility should be further investigated because it may have a greater role in the etiology of gastroschisis than currently appreciated. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Time trends in the prevalence of birth defects in Texas 1999–2007: Real or artifactual?

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 23 Aug 2011 23:00:00 +0100

CONCLUSIONS: In our opinion, the weight of evidence in our study suggests that the observed increase over time in total birth defects and in many specific birth defects is artifactual. This likely reflects increased awareness, referral, detection, or documentation in health care facilities visited by TBDR staff, resulting in more complete ascertainment by the registry, rather than a true change over time in the occurrence of most birth defects. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Maternal smoking in pregnancy and birth defects: a systematic review based on 173 687 malformed cases and 11.7 million controls

Human Reproduction Update — Mon, 15 Aug 2011 23:00:00 +0100

CONCLUSIONS Birth defects that are positively associated with maternal smoking should now be included in public health educational materials to encourage more women to quit before or during pregnancy. (Source: Human Reproduction Update)

First trimester detection of structural abnormalities and the role of aneuploidy markers

Ultrasound in Obstetrics and Gynecology — Sun, 14 Aug 2011 23:00:00 +0100

Conclusions:About half of major structural abnormalities can be diagnosed in the first trimester. Increased nuchal translucency or abnormal ductus venosus blood flow appear to be associated with cardiac and skeletal defects and may facilitate an early detection. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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First trimester detection of structural abnormalities and the role of aneuploidy markers.

The Ultrasound Review of Obstetrics and Gynecology — Sun, 14 Aug 2011 23:00:00 +0100

CONCLUSIONS: About half of major structural abnormalities can be diagnosed in the first trimester. Increased nuchal translucency or abnormal ductus venosus blood flow appear to be associated with cardiac and skeletal defects and may facilitate an early detection. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 21845742 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Limb–body wall defect. Is there a defensible hypothesis and can it explain all the associated anomalies?

American Journal of Medical Genetics Part A — Tue, 02 Aug 2011 23:00:00 +0100

We present four patients that illustrate the spectrum of ventral body wall defects, and from there critique the current hypotheses of pathogenesis. We conclude that this association of malformations originates as early as the embryonic disc stage, and that some of the observed associated anomalies are secondary complications of the primary disturbance in embryogenesis. We propose a new explanation for the atypical facial clefts and cranial malformations that are often observed. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Medical Genetics Part A)

Medical management of motility disorders in patients with intestinal failure: a focus on necrotizing enterocolitis, gastroschisis, and intestinal atresia

Journal of Pediatric Surgery — Sun, 31 Jul 2011 23:00:00 +0100

Conclusion: Despite relatively poor level of evidence regarding the application of promotility and antidiarrheal medications in patients with SBS and IF, these agents continue to be used. Herein, we provide a review of the physiology and pathophysiology of intestinal motility/dysmotility and available strategies for the use of promotility and antidiarrheal agents in patients with IF/SBS. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Sun, 31 Jul 2011 23:00:00 +0100

In the article “Effects of multidisciplinary prenatal care and delivery mode on gastroschisis outcomes” (J Pediatr Surg. 2011 Jan;46:86-9), there was an error in the byline. The correct byline is below. (Source: Journal of Pediatric Surgery)

Omphalocele Management using Goal-oriented Classification in African Centre with Limited Resources

Journal of Tropical Pediatrics — Wed, 27 Jul 2011 23:00:00 +0100

In 2000–09, 96 children comprising 57 males and 39 females who were presented between 2 h and 1 week of birth with omphalocele were prospectively managed using goal-oriented classification at the University of Benin Teaching Hospital, Nigeria. All were born through spontaneous vaginal delivery, out of which 9 (9.4%) were preterm. Eighty-two (85.4%) mothers in villages with no supervised antenatal care/delivery and/or prenatal diagnosis presented their babies late. Thirty-three (34.4%) babies in group A, with defect size ≤4.5 cm and intact sac, were managed conservatively and had fascial closure after neonatal period, resulting in 32 (97%) survivors. Forty-two (43.8%) babies in group B, with defect size >4.5 cm and intact sac, were managed conservatively and had fascial closures...

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Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997–2005

Paediatric and Perinatal Epidemiology — Wed, 20 Jul 2011 23:00:00 +0100

SummaryTinker SC, Reefhuis J, Dellinger AM, Jamieson DJ, the National Birth Defects Prevention Study. Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997–2005. Paediatric and Perinatal Epidemiology 2011; ••:••–••.Maternal injuries during pregnancy are common (∼7% prevalence). However, few studies have examined the association between maternal injuries and birth defects. The National Birth Defects Prevention Study is a population‐based case–control study of birth defects in 10 US states. Cases were ascertained through surveillance; controls were randomly selected from infants delivered without major birth defects in the study regions. Mothers completed a telephone interview on exposures befor...

Primary Suture-less Closure of Gastroschisis Using Negative Pressure Dressing (Wound Vacuum)

European Journal of Pediatric Surgery — Wed, 20 Jul 2011 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1280823Abstract[...]Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Birth defects linked for first time to smoking in pregnancy

Guardian Unlimited Science — Mon, 11 Jul 2011 23:01:03 +0100

Clubfoot and missing limbs among malformations in newborns proven to be associated with expectant mothers who smokeDoctors are urging mothers-to-be to give up cigarettes after new research linked smoking in pregnancy to babies suffering birth defects such as clubfoot, missing limbs and deformed limbs.Those who smoke while expecting a baby increase the risk of their child being born with a serious malformation by as much as 50%, the study found. The disclosure led to calls for new measures to reduce what the authors called "staggeringly high" levels of smoking among pregnant women.Although smoking by pregnant women has already been linked to a higher risk of a woman having a miscarriage or her baby being born prematurely or having a low birth weight, 45% of women under 20 do so while one in...

Maternal Treatment with Opioid Analgesics and Risk for Birth Defects: Broussard CS, Rasmussen SA, Reefhuis J, et al. Am J Obstet Gynecol 2011;204:314.e1–11.

The Journal of Emergency Medicine — Thu, 30 Jun 2011 23:00:00 +0100

This study used data from the National Birth Defects Prevention Study for infants born between October 1, 1997 and December 31, 2005. This database includes information on infants born with birth defects of unknown etiology as well as an annual random sample of control infants without birth defects. Interviews are conducted to assess for maternal exposures. Opioid exposure was defined as maternal report of use of codeine, hydrocodone, meperidine, oxycodone, hydrocodone, propoxyphene, morphine, tramadol, methadone, hydromorphone, fentanyl, or pentazocine for the therapeutic treatment of pain between 1 month before conception and 3 months after conception. Multivariable logistic regression analysis was used to calculate adjusted odds ratios (OR). Adjustments were made for maternal age, race/...

Surgical treatment of cor triatriatum, ventricular septal defect in an infant with a giant omphalocele

Journal of Pediatric Surgery — Thu, 30 Jun 2011 23:00:00 +0100

Abstract: Cor triatriatum is a rare cardiac anomaly characterized by a membrane in the left atrium that separates the atrium into the proximal and distal chambers. Herein, we describe an unusual case of cor triatriatum combined with a ventricular septal defect in a newborn infant with a giant omphalocele and underwent a successful cardiac surgical repair. In this abnormality, there is a high incidence of associated congenital abnormalities; but to the best of our knowledge, this is the first time that a combination of cor triatriatum and omphalocele has been reported in the literature. (Source: Journal of Pediatric Surgery)

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Outcomes of plastic closure in gastroschisis

Surgery — Wed, 29 Jun 2011 23:00:00 +0100

Conclusion: Plastic closure of abdominal wall defects in gastroschisis is effective both as a primary procedure and after silo placement. A multivariate analysis shows plastic closure to be associated with fewer days of mechanical ventilation and less likelihood of developing infection or sepsis. (Source: Surgery)

Congenital Omphalocele and Polyhydramnios: A Study of 52 Cases

Fetal Diagnosis and Therapy — Fri, 24 Jun 2011 23:00:00 +0100

Fetal Diagn Ther (DOI:10.1159/000327542) (Source: Fetal Diagnosis and Therapy)

Fetal Gastroschisis: Epidemiological Characteristics and Pregnancy Outcomes in Mississippi

American Journal of Perinatology — Tue, 21 Jun 2011 23:00:00 +0100

We describe the epidemiological characteristics and identify maternal–fetal outcomes in pregnancies complicated by gastroschisis. We retrospectively reviewed 115 cases of gastroschisis at the University of Mississippi Medical Center. The incidence of gastroschisis trended upward between 2000 and 2008. Significant proportions of mothers were nonobese, nulliparous, teenagers, smokers, and nonconsumers of alcohol. Infants delivered at >36 weeks or without sepsis had shorter hospital stay (HS) and interval to full enteral feeding (FEF). The rates of low birth weight (LBW), fetal growth restriction, and spontaneous preterm birth (PTB) were 63%, 45%, and 24%, respectively. Bowel atresia was noted in 9%. Rates of primary closure (25%), neonatal sepsis (29%), fetal death (2%), and infant ...

Fetal omphalocele ratios predict outcomes in prenatally diagnosed omphalocele

American Journal of Obstetrics and Gynecology — Tue, 21 Jun 2011 23:00:00 +0100

Conclusion: The O/HC of 0.21 or greater best predicted staged or delayed omphalocele closure. Giant omphalocele designation, regardless of definition, poorly predicted outcome. (Source: American Journal of Obstetrics and Gynecology)

Umbilical Cord as Temporary Coverage in Gastroschisis

European Journal of Pediatric Surgery — Tue, 14 Jun 2011 23:00:00 +0100

Conclusion: Our experience confirms the advantage of a staged reduction in giant gastroschisis. The use of autogenic material such as the umbilical cord has advantages such as low infection rates and easy availability.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

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Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register‐based study

Ultrasound in Obstetrics and Gynecology — Mon, 06 Jun 2011 04:00:00 +0100

ConclusionsSchizencephaly occurs more frequently in the fetuses of younger mothers. It is often associated with septo‐optic dysplasia, suggesting that the two conditions may share a common origin, arising as a result of destructive processes that cause changes in the brain which only become apparent on ultrasound in the second half of pregnancy. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Does staged closure have a worse prognosis in gastroschisis?

Clinics — Sun, 05 Jun 2011 23:23:16 +0100

CONCLUSION: No significant difference was observed in the outcome of newborns who underwent primary closure or staged closure of gastroschisis when using an IVP below 20 cm H2O as the criterion for primary closure. (Source: Clinics)

Maternal use of hot tub and major structural birth defects

Birth Defects Research Part A: Clinical and Molecular Teratology — Sun, 05 Jun 2011 23:00:00 +0100

CONCLUSIONSThese results suggest that women who use hot tubs more than once during early pregnancy and for long periods of time have an increased risk of certain birth defect phenotypes, particularly anencephaly and gastroschisis. Because of multiple statistical tests and small sample sizes, we cannot exclude the possibility that some of these elevated associations may be due to chance. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Schizencephaly prevalence, prenatal diagnosis and clues to aetiology; a register based study.

The Ultrasound Review of Obstetrics and Gynecology — Sun, 05 Jun 2011 23:00:00 +0100

CONCLUSIONS: Schizencephaly occurs more frequently in the fetuses of younger mothers. It is often associated with septo-optic dysplasia suggesting that the two conditions may share a common origin, arising as a result of destructive processes that cause changes which only become apparent on ultrasound in the second half of pregnancy. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 21647999 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Schizencephaly prevalence, prenatal diagnosis and clues to aetiology; a register based study

Ultrasound in Obstetrics and Gynecology — Tue, 31 May 2011 23:00:00 +0100

Conclusions:Schizencephaly occurs more frequently in the fetuses of younger mothers. It is often associated with septo‐optic dysplasia suggesting that the two conditions may share a common origin, arising as a result of destructive processes that cause changes which only become apparent on ultrasound in the second half of pregnancy. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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Antenatal diagnosis of bowel dilatation in gastroschisis is predictive of poor postnatal outcome

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

Conclusions: We found that sonographically detected bowel dilatation more than 20 mm in utero in fetuses with gastroschisis may have value in predicting clinically significant adverse postnatal outcomes. (Source: Journal of Pediatric Surgery)

Gastroschisis and extreme prematurity: a report of two survivors

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

We report 2 such cases. (Source: Journal of Pediatric Surgery)

Associations between periconceptional alcohol consumption and craniosynostosis, omphalocele, and gastroschisis

Birth Defects Research Part A: Clinical and Molecular Teratology — Mon, 30 May 2011 23:00:00 +0100

CONCLUSIONS: Results suggest that maternal periconceptional alcohol consumption is associated with omphalocele and gastroschisis, and second and third trimester alcohol consumption are inversely associated with craniosynostosis. Birth Defects Research (Part A) 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Manitoba-oculo-tricho-anal (MOTA) syndrome is caused by mutations in FREM1

Journal of Medical Genetics — Tue, 24 May 2011 23:00:00 +0100

Conclusions The milder phenotypes associated with FREM1 deficiency in humans (MOTA syndrome and BNAR syndrome) compared to that resulting from FRAS1 and FREM2 loss of function (Fraser syndrome) are also consistent with the less severe phenotypes resulting from Frem1 loss of function in mice. Together, Fraser, BNAR and MOTA syndromes constitute a clinically overlapping group of FRAS–FREM complex diseases. (Source: Journal of Medical Genetics)

Early severe hypoalbuminemia is an independent risk factor for intestinal failure in gastroschisis

Pediatric Surgery International — Fri, 20 May 2011 05:57:17 +0100

Conclusions Early severe hypoalbuminemia appears to be an independent risk factor for long-term intestinal compromise rather than merely an indicator of overall illness. Further interventional studies are needed to determine whether clinical protocols utilizing judicious fluid administration, exogenous albumin, and early enteral feeding can improve clinical outcomes in gastroschisis. Content Type Journal ArticlePages 1-4DOI 10.1007/s00383-011-2921-5Authors Christopher W. Snyder, Division of Pediatric Surgery, Department of Surgery, University of Alabama, ACC 300, 1600 7th Avenue South, Birmingham, AL 35294-0011, USAJoseph R. Biggio, Division of Maternal and Fetal Medicine, Department of Obstetrics and Gynecology, University of Alabama, Birmingham, USADonna T. Bartle...

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The cholinergic response is increased in isolated ileum from gastroschisis rat model

Pediatric Surgery International — Wed, 18 May 2011 06:12:49 +0100

Conclusion The frequency and the amplitude of rhythmic contractions were reduced along with an increase in the contraction induced by mucarinic agonist and by EFS in G. These results suggest the occurrence of an adaptative supersensitivity to cholinergic response via increases in the protein expression for M3 receptor. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-011-2923-3Authors Aline Cristina Régis, Department of Surgery, School of Medical Sciences, State University of Campinas-UNICAMP, Campinas, SP, BrazilJulio Alejandro Rojas-Moscoso, Department of Pharmacology, State University of Campinas-UNICAMP, Campinas, SP, BrazilFrances Lilian Lanhellas Gonçalves, Department of Surgery, School of Medical Sciences, State University of Campinas-UNICAMP, Campin...

Craniorachischisis and omphalocele in a stillborn cynomolgus monkey (Macaca fascicularis)

American Journal of Medical Genetics Part A — Wed, 11 May 2011 23:00:00 +0100

AbstractNonhuman primates have been a common animal model to evaluate experimentally induced malformations. Reports on spontaneous malformations are important in determining the background incidence of congenital anomalies in specific species and in evaluating experimental results. Here we report on a stillborn cynomolgus monkey (Macaca fascicularis) with multiple congenital anomalies from the colony maintained at the Southwest National Primate Research Center at the Texas Biomedical Research Institute, San Antonio, Texas. Physical findings included low birth weight, craniorachischisis, facial abnormalities, omphalocele, malrotation of the gut with areas of atresia and intussusception, a Meckel diverticulum, arthrogryposis, patent ductus arteriosus, and patent foramen ovale. The macaque ha...

Perinatal Outcome in the Live-Born Infant with Prenatally Diagnosed Omphalocele

American Journal of Perinatology — Mon, 02 May 2011 23:00:00 +0100

Amer J PerinatolDOI: 10.1055/s-0031-1276737ABSTRACTWe compared perinatal outcomes between live-born nonisolated and isolated omphaloceles diagnosed during a prenatal ultrasound. Fetuses (n = 86) with omphalocele were identified between 1995 and 2007 at a single institution. Inclusion criteria were an omphalocele > 14 weeks' gestation, available fetal and/or neonatal karyotype, and a live-born infant (n = 46). Perinatal outcomes were compared in nonisolated (n = 23) and isolated omphaloceles (n = 23). For all omphaloceles, the majority delivered after 34 weeks by cesarean. Mean birth weight (2782 versus 2704 g), median length of stay (27 versus 25 days), and mortality (two deaths in each group) were not different between the nonisolat...

Monitoring the prenatal detection of structural fetal congenital anomalies in England and Wales: register-based study

Journal of Medical Screening — Sun, 01 May 2011 23:00:00 +0100

Conclusions BINOCAR registers can, uniquely, provide contemporary data on PND and birth prevalence rates to enable monitoring of the ultrasound component of FASP at a national and regional level, allowing comparisons between populations to be made, planning of resources facilitated and assistance for parents making informed decisions on whether to enter the screening programme. (Source: Journal of Medical Screening)

Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

Conclusions: The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis. (Source: Journal of Pediatric Surgery)

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Maternal dietary glycaemic intake during pregnancy and the risk of birth defects

Paediatric and Perinatal Epidemiology — Sat, 23 Apr 2011 23:00:00 +0100

SummaryYazdy MM, Mitchell AA, Liu S, Werler MM. Maternal dietary glycaemic intake during pregnancy and the risk of birth defects. Paediatric and Perinatal Epidemiology 2011.High sugar intake has been linked to fetal anomalies in the presence and absence of insulin resistance. Using dietary data collected in the Boston University Slone Epidemiology Birth Defects Study, we examined whether high dietary glycaemic index (dGI) or load (dGL) increased the risk of birth defects. Non‐diabetic mothers of 1921 cases and 704 controls were interviewed within 6 months of delivery (1988–98) about pregnancy events and exposures, including a 99‐item food frequency questionnaire. Case groups included amniotic bands, craniosynostosis, gastroschisis, hypospadias, small intestinal defects, anorectal def...

Unusual content of omphalocele: a congenital mature cystic teratoma of falciform ligament of the liver

Pediatric Surgery International — Sat, 16 Apr 2011 15:56:33 +0100

We report unusual content of omphalocele that is congenital mature cystic teratoma of falciform ligament of liver which was confirmed by histopathology. This is a very uncommon entity and not described in world literature. Content Type Journal ArticlePages 1-2DOI 10.1007/s00383-011-2898-0Authors Punit Kumar Srivastava, Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, IndiaA. N. Gangopadhyay, Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, IndiaD. K. Gupta, Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, IndiaS. P. Sharma, Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, IndiaVijayendra Kumar, Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, India Journal Pediatric Surgery InternationalOnline ISSN 1437-9...

Co‐occurrence of severe Goltz–Gorlin syndrome and pentalogy of Cantrell – Case report and review of the literature

American Journal of Medical Genetics Part A — Sun, 10 Apr 2011 23:00:00 +0100

We report on an infant with findings fitting both Goltz–Gorlin syndrome (sparse hair; anophthalmia; clefting; bifid nose; irregular vermillion of both lips; asymmetrical limb malformations; caudal appendage; linear aplastic skin defects; unilateral hearing loss) and the pentalogy of Cantrell (absent lower sternum; anterior diaphragmatic hernia; ectopia cordis; omphalocele). The clinical diagnosis Goltz–Gorlin syndrome was confirmed molecularly by a point mutation in PORCN (c.727C>T). The presence of molecularly confirmed Goltz–Gorlin syndrome and pentalogy of Cantrell in a single patient has been reported twice before. The present patient confirms that the pentalogy of Cantrell can be caused in some patients by a PORCN mutation. It remains at present uncertain whether this can be ...

Prenatal diagnosis of OEIS (omphalocele, bladder exstrophy, imperforate anus, clubfeet) variant associated with increased nuchal translucency and OEIS complex with ambiguous genitalia associated with corrected transposition of the great arteries: case series and review of the literature

Archives of Gynecology and Obstetrics — Thu, 07 Apr 2011 17:09:13 +0100

Conclusion Differential diagnosis with exstrophy-epispadias complex and/or cloacalexstrophy complex may be difficult antenatally by means of ultrasound. However, color Doppler has been proved to aid the diagnosis of bladder exstrophy by depicting the urine flow in direct communication with the abdominal cavity and has been useful in showing the course of the perivesical umbilical arteries. Prenatal 3D ultrasound with tomographic ultrasound imaging (TUI) and antenatal MR imaging might be useful adjuncts to conventional 2D scan in aiding the prenatal diagnosis of such malformation. Content Type Journal ArticlePages 1-9DOI 10.1007/s00404-011-1900-3Authors G. Tonni, Prenatal Diagnostic Service, AUSL Reggio Emilia, Via Amendola, 1, 42100 Reggio Emilia, ItalyG. Grisolia...

Neonatal abdominal wall defects

Seminars in Fetal and Neonatal Medicine — Wed, 06 Apr 2011 23:00:00 +0100

Summary: Gastroschisis and omphalocele are the two most common congenital abdominal wall defects. Both are frequently detected prenatally due to routine maternal serum screening and fetal ultrasound. Prenatal diagnosis may influence timing, mode and location of delivery. Prognosis for gastroschisis is primarily determined by the degree of bowel injury, whereas prognosis for omphalocele is related to the number and severity of associated anomalies. The surgical management of both conditions consists of closure of the abdominal wall defect, while minimizing the risk of injury to the abdominal viscera either through direct trauma or due to increased intra-abdominal pressure. Options include primary closure or a variety of staged approaches. Long-term outcome is favorable in most cases; howeve...

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Anaesthesia for specialist surgery in infancy

Anaesthesia and intensive care medicine — Thu, 31 Mar 2011 23:00:00 +0100

Abstract: Common indications for neonatal surgery include inguinal hernias and hypertrophic pyloric stenosis. Less common conditions, that have major implications for anaesthesia, include tracheo-oesophageal fistula, congenital diaphragmatic hernia, exomphalos, gastroschisis and congenital lobar emphysema. The anaesthetic management of these conditions is outlined in this article. (Source: Anaesthesia and intensive care medicine)

Erratum

Journal of Pediatric Surgery — Thu, 31 Mar 2011 23:00:00 +0100

In the January issue of the Journal of Pediatric Surgery, the article “Conditional mutation of fibroblast growth factor receptors 1 and 2 results in an omphalocele in mice associated with disruptions in ventral body wall muscle formation” on pages 90 to 96 contained several errors. The grant funding listed for this article was incomplete, and was incorrect. In addition, it should be noted that authors Nichol PF and Saijoh Y contributed equally. The complete grant funding information is as follows: (Source: Journal of Pediatric Surgery)

Macroscopic and histopathologic findings in a laparoschisis model in fetal sheep: comparisons with gastroschisis in human fetuses and implications for prenatal interventions

Archives of Gynecology and Obstetrics — Wed, 30 Mar 2011 23:55:26 +0100

Conclusions Macroscopic and histopathological findings in a new minimally invasive laparoschisis model in sheep resemble those found in human fetuses with gastroschisis. The new model seems therefore suitable for assessing the potential of prenatal minimally invasive fetoscopic interventions in this condition. Content Type Journal ArticlePages 1-5DOI 10.1007/s00404-011-1890-1Authors Philip Kahl, Department of Pathology, University of Bonn, Sigmund Freud Strasse 25, 53127 Bonn, GermanyReinhard Buettner, Department of Pathology, University of Bonn, Sigmund Freud Strasse 25, 53127 Bonn, GermanyKristina Tchatcheva, Department of Obstetrics and Prenatal Medicine, University of Bonn, Sigmund Freud Strasse 25, 53127 Bonn, GermanyRüdiger Stressig, Department of Obstetrics ...

Gastrointestinal reconstruction and outcomes for patients with the OEIS complex

Seminars in Pediatric Surgery — Wed, 30 Mar 2011 21:27:53 +0100

The OEIS complex (ie, omphalocele-exstrophy-imperforate anus-spinal defects) is a rare and complex anomaly requiring collaboration among multiple pediatric surgical subspecialties throughout the early childhood period. Initial gastrointestinal management involves identification of all intestinal components with reconstruction of the entire length of intestines in-line leading to an end colostomy. Candidacy for an abdominoperineal intestinal pull-through procedure is dependent upon the patient's ability to form solid stools, degree of pelvic neuromuscular development, and ability to comply with a bowel management program. (Source: Seminars in Pediatric Surgery)

Evolution of critically ill patients with gastroschisis from three tertiary centers

Clinics — Tue, 22 Mar 2011 18:43:46 +0100

CONCLUSION: In newborns with gastroschisis, more aggressive attention to hyponatremia and hypoalbuminemia would improve the outcome. (Source: Clinics)

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Maternal nutrition and gastroschisis: findings from the National Birth Defects Prevention Study

American Journal of Obstetrics and Gynecology — Mon, 14 Mar 2011 00:00:00 +0100

Conclusion: This study does not support an increased risk for gastroschisis with decreasing tertiles of individual nutrients. (Source: American Journal of Obstetrics and Gynecology)

Paper 4: EUROCAT statistical monitoring: Identification and investigation of ten year trends of congenital anomalies in Europe

Birth Defects Research Part A: Clinical and Molecular Teratology — Fri, 04 Mar 2011 00:00:00 +0100

CONCLUSIONSSignificant increasing trends were detected for abdominal wall anomalies, gastroschisis, hypospadias, Trisomy 18 and renal dysplasia in the Pan‐Europe analysis while 68 increasing trends were identified in individual registries. A decreasing trend was detected in over one‐third of anomaly subgroups in the Pan‐Europe analysis, and 16.9% of individual registry tests. Registry preliminary investigations indicated that many trends are due to changes in data quality, ascertainment, screening, or diagnostic methods. Some trends are inevitably chance phenomena related to multiple testing, while others seem to represent real and continuing change needing further investigation and response by regional/national public health authorities. Birth Defects Research (Part A), 2011.© 2011...

Past and current surgical treatment of giant omphalocele: outcome of a questionnaire sent to authors

Journal of Pediatric Surgery — Tue, 01 Mar 2011 00:00:00 +0100

Conclusions: The results of the questionnaire did not show a generally accepted method of treatment after more than 30 years of innovations in managing patients with a giant OC. There are generally 2 main treatment modalities: staged closure and delayed closure. Because of the lack of large patient numbers and late follow-up, long-term results of the published techniques are needed, and randomized multicenter trials based on these outcomes are recommended. Until then, we remain dependent on expert opinions. (Source: Journal of Pediatric Surgery)

Letter to the Editor

Journal of Pediatric Surgery — Tue, 01 Mar 2011 00:00:00 +0100

I was intrigued by the letter of Dr Sheth and reply of Dr Luks , in the June 2010 issue of the journal, regarding the use of pneumoperitoneum to expand the peritoneal cavity to accommodate the external viscera encountered with giant omphalocele. Both correspondents pled ignorance of any previous reports of the use of pneumoperitoneum in giant omphalocele. (Source: Journal of Pediatric Surgery)

Gastroschisis with extracorporeal liver and stomach associated with sacrococcygeal teratoma and limb abnormalities

Congenital Anomalies — Tue, 01 Mar 2011 00:00:00 +0100

ABSTRACTGastroschisis is the herniation of abdominal viscera through paramedian abdominal wall fusion defect without involvement of umbilical cord. Evisceration usually contains intestinal loops and has no surrounding membrane. Rarely, herniation of other major viscera like stomach and liver occurs which makes the prognosis worst. In common, gastroschisis usually is not associated with sacrococcygeal teratomas. In the present case report, a very rare incidence of gastroschisis associated with sacrococcygeal teratoma was described. The gastroschisis showed a complete evisceration of stomach, bowel and extracorporeal liver. The large sacrococcygeal mass was seen on the posteroinferior part of the trunk and gluteal region and it was lying completely external. The fetus also showed malrotated ...

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Maternal treatment with opioid analgesics and risk for birth defects

American Journal of Obstetrics and Gynecology — Thu, 24 Feb 2011 00:00:00 +0100

Conclusion: Consistent with some previous investigations, our study shows an association between early pregnancy maternal opioid analgesic treatment and certain birth defects. This information should be considered by women and their physicians who are making treatment decisions during pregnancy. (Source: American Journal of Obstetrics and Gynecology)

Induction of Fetal Diuresis with Intraamniotic Furosemide Injection Reduces Intestinal Damage in a Rat Model of Gastroschisis

European Journal of Pediatric Surgery — Mon, 21 Feb 2011 00:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1271708AbstractBackground/Purpose: Contact with amniotic fluid causes intestinal damage (ID) in fetuses with gastroschisis. Intraamniotic meconium has been shown to be responsible for ID, and ID has been shown to correlate with intraamniotic meconium concentrations. ID can be prevented by lowering the intraamniotic meconium concentration. A new method to lower intraamniotic meconium concentration might consist in the induction of fetal diuresis with intraamniotic diuretic injection. This hypothesis was tested in a rat model.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Negative pressure wound therapy in the management of neonates with complex gastroschisis

Pediatric Surgery International — Fri, 18 Feb 2011 16:56:06 +0100

We describe the application of the RENASYS™ system (Smith and Nephew, UK) in delivering NPWT to four neonates with complex gastroschisis, all of whom achieved successful outcomes. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-011-2868-6Authors Wilson W. Choi, School of Medicine, University of Queensland, 288 Herston Road, Herston, QLD 4006, AustraliaCraig A. McBride, Department of Paediatrics and Child Health, Queensland Children’s Medical Research Institute, Royal Children’s Hospital, University of Queensland, Herston, QLD 4029, AustraliaRoy M. Kimble, Department of Paediatrics and Child Health, Queensland Children’s Medical Research Institute, Royal Children’s Hospital, University of Queensland, Herston, QLD 4029, Australia Journal Pediatric Surgery In...

Management of isolated and associated colonic atresia

Pediatric Surgery International — Thu, 17 Feb 2011 08:13:52 +0100

Conclusions Isolated colonic atresia is amenable with a short hospital stay and an excellent outcome. In contrast, infants with colonic atresia associated with gastroschisis present a serious clinical challenge. Content Type Journal ArticlePages 1-6DOI 10.1007/s00383-010-2802-3Authors Emir Q. Haxhija, Department of Pediatric and Adolescent Surgery, Medical University Graz, Auenbruggerplatz 34, 8036 Graz, AustriaJohannes Schalamon, Department of Pediatric and Adolescent Surgery, Medical University Graz, Auenbruggerplatz 34, 8036 Graz, AustriaMichael E. Höllwarth, Department of Pediatric and Adolescent Surgery, Medical University Graz, Auenbruggerplatz 34, 8036 Graz, Austria Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Sourc...

Factors Associated with Mortality in Neonates with Gastroschisis

European Journal of Pediatric Surgery — Wed, 16 Feb 2011 00:00:00 +0100

Eur J Pediatr Surg 2011; 21: 21-24DOI: 10.1055/s-0030-1262791AbstractPurpose: The ongoing epidemic of gastroschisis has created multiple challenges and continues to raise questions concerning the optimal management of these high-risk patients. Although the overall survival rate has increased over the past 3 decades, morbidity and mortality remain significant. The purpose of this study was to analyze the main factors associated with mortality in neonates admitted to an intensive care unit for the management of this abdominal wall defect.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

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A familial syndromal form of omphalocele.

European Journal of Medical Genetics — Wed, 16 Feb 2011 00:00:00 +0100

We report a family with facial dysmorphism somewhat reminiscent of Robinow syndrome (flat face, very short, upturned nose, very long and unusually wide philtrum, and flattened maxillary arch), observed in 3 generations. Four sibs in the second generations had large omphaloceles. One child had ectrodactyly. Genomic rearrangements, and WNT5A or ROR2 mutations were excluded in this family. At this point, we feel reasonable to consider this family as expressing a "new" syndrome related but different from Robinow syndrome, associating facial dysmorphism and abdominal wall defect, and compatible with dominant inheritance with variable expressivity, although recessively inherited omphalocele occurring in a family showing independently some dominant craniofacial peculiarities cannot be ruled out. ...

Characteristics and Outcome and the Omphalocele Circumference/Abdominal Circumference Ratio in Prenatally Diagnosed Fetal Omphalocele

Fetal Diagnosis and Therapy — Tue, 15 Feb 2011 10:39:35 +0100

Fetal Diagn Ther (DOI:10.1159/000323326) (Source: Fetal Diagnosis and Therapy)

Laparoscopic Single-stage Fowler-Stephens Orchidopexy in a Case of Orchidogastric Fusion

Urology — Mon, 14 Feb 2011 00:00:00 +0100

This report describes laparoscopic Fowler-Stephens orchidopexy for cryptorchidism resulting from orchidogastric fusion in an infant born with gastroschisis. At 11 months of age, the left testicle remained impalpable, and diagnostic laparoscopy was performed. Intraoperatively, a normal-appearing testicle was found attached to the stomach. The testicle was dissected, mobilized down to the left inguinal ring, exteriorized through a transscrotal trocar, and subsequently fixated in the lower left scrotum. On follow-up 5 months later, both testicles were normal in size and location. Single-stage laparoscopic Fowler-Stephens orchidopexy is easily accomplished in cases of orchidogastric fusion resulting from a long vas deferens. (Source: Urology)

Minimally invasive mitral valve replacement in a patient with previous omphalocele repair and severe scoliosis

General Thoracic and Cardiovascular Surgery — Thu, 10 Feb 2011 07:00:24 +0100

Abstract Mitral valve surgery in a patient with severe chest deformity and poor respiratory function can be associated with a high risk due to difficult access and postoperative respiratory failure. A 45-year-old man with scoliosis and respiratory dysfunction who had undergone previous omphalocele repairs presented with severe mitral regurgitation. Mitral valve replacement via right mini-thoracotomy was successfully performed. The minimally invasive approach was considered useful in this patient with anatomical difficulty and respiratory dysfunction. Content Type Journal ArticlePages 117-119DOI 10.1007/s11748-010-0642-7Authors Tadashi Kitamura, Cardiothoracic Surgical Unit, Royal Adelaide Hospital, Adelaide, AustraliaJames Edwards, Cardiothoracic Surgical Unit, Royal ...

Hospital readmission among infants with gastroschisis

Journal of Perinatology — Thu, 10 Feb 2011 00:00:00 +0100

Authors: A P South, J J Wessel, A Sberna, M Patel & A L Morrow (Source: Journal of Perinatology)

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Does gastric dilation predict adverse perinatal or surgical outcome in fetuses with gastroschisis?

The Ultrasound Review of Obstetrics and Gynecology — Tue, 01 Feb 2011 00:00:00 +0100

Authors: Alfaraj MA, Ryan G, Langer JC, Windrim R, Seaward PG, Kingdom J To compare perinatal and infant surgical outcomes in fetuses with gastroschisis with and without gastric dilation in a single-center cohort. PMID: 21264982 [PubMed - in process] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Use of tissue expanders in the repair of complex abdominal wall defects

Journal of Pediatric Surgery — Tue, 01 Feb 2011 00:00:00 +0100

Conclusions: Tissue expanders possess a broad range of applications for abdominal wall reconstruction and can be used in patients of all ages. (Source: Journal of Pediatric Surgery)

Effective surveillance of gastroschisis and omphalocele: separate International Classification of Diseases, Ninth Revision codes!

Journal of Pediatric Surgery — Tue, 01 Feb 2011 00:00:00 +0100

Many birth defects surveillance programs use the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) coding system for identification of cases and data collection. It is critical to effectively code birth defects for accurate surveillance, monitoring, and prevention strategies. A major challenge in interpreting data on gastroschisis and omphalocele results from inaccurate coding. Until just recently, both gastroschisis and omphalocele have been coded by a common ICD-9 code 756.79, even though they are completely different abdominal wall defects . However, in the 2010 version of the Ingenix ICD-9-CM expert for physicians , both gastroschisis and omphalocele have separate specific ICD-9 codes (): 756.72 for omphalocele and 756.73 for gastroschisis. It i...

Donor transmission of pineoblastoma in a two‐yr‐old male recipient of a multivisceral transplant: A case report

Pediatric Transplantation — Mon, 31 Jan 2011 00:00:00 +0100

We report a case of donor‐transmitted pineoblastoma, a PNET, in a two‐yr‐old male recipient, who presented with a rapidly growing mass in the right mandible, four months after multiple visceral organ transplantation. The recipient had liver, pancreas, and small bowel transplants because of end‐stage liver failure and short gut syndrome, which was secondary to large bowel resection for management of gastroschisis complicated by intestinal volvulus. The donor autopsy results became available seven wk after transplantation, which found a pineoblastoma with meningeal spread. Evaluation of eyes, adrenal glands, bone marrow, and other organs did not identify metastasis outside the CNS. A biopsy of the recipient’s right mandibular mass revealed a malignant small round blue cell tumo...

Umbilical artery PI and fetal abdominal circumference in isolated gastroschisis

Ultrasound in Obstetrics and Gynecology — Mon, 24 Jan 2011 00:00:00 +0100

ConclusionsIn fetal gastroschisis, it is well known that AC tends to be smaller, though UAPI has not been reported to be abnormal in any consistent way. There is a clear relationship between the degree of smallness of the baby's abdominal circumference for gestation and the umbilical PI. This is not the case for normally grown babies. The data suggest that the growth restriction seen in gastroschisis may be explained by hypoxia, and not simply because of the classical explanation of extra‐abdominal displacement of the abdominal viscera. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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Altered PITX2 and LEF1 gene expression in the cadmium-induced omphalocele in the chick model

Pediatric Surgery International — Sat, 22 Jan 2011 20:11:58 +0100

Conclusions Downregulation of PITX2 and LEF1 genes may interfere with ventral body wall formation in Cd chick model causing omphalocele by disrupting somite myogenesis. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-010-2829-5Authors Takashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJohn Bannigan, School of Medicine and Medical Science, University College Dublin, Dublin, IrelandJennifer Thompson, School of Medicine and Medical Science, University College Dublin, Dublin, Ireland Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery Internatio...

BMPR1A-mediated BMP1 signalling is disrupted in the cadmium-induced omphalocele in the chick model

Pediatric Surgery International — Sat, 22 Jan 2011 08:00:05 +0100

Conclusion Disruption of BMPR1A-mediated BMP1 signalling during the narrow window of early embryogenesis may interfere with normal VBW formation, causing omphalocele phenotype in the Cd chick model. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-010-2842-8Authors Takashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJohn Bannigan, School of Medicine and Medical Science, Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin, IrelandJennifer Thompson, School of Medicine and Medical Science, Conway Institute of Biomolecular and Biomedical Research, University College Dub...

Use of clomiphene citrate and birth defects, National Birth Defects Prevention Study, 1997-2005

Human Reproduction — Fri, 21 Jan 2011 00:00:00 +0100

CONCLUSIONS Several associations between CC use and birth defects were observed. However, because of the small number of cases, inconsistency of some findings with previous reports, and the fact that we cannot assess the CC effect separately from that of the subfertility, these associations should be interpreted cautiously. (Source: Human Reproduction)

Rising Prevalence of Gastroschisis in Washington State

Journal of Toxicology and Environmental Health Part A — Thu, 20 Jan 2011 22:37:19 +0100

(Source: Journal of Toxicology and Environmental Health Part A)

Outcomes of Abdominal Wall Closure in Babies With Gastroschisis: Does Plastic Closure Measure Up?

Journal of Surgical Research — Wed, 19 Jan 2011 20:26:50 +0100

Introduction: Gastroschisis is a congenital abdominal wall defect in which the viscera develop outside the abdominal cavity. Exposure to amniotic fluid, intestinal edema and obstruction result in long-term morbidities including dysmotility and atresias. After reduction of abdominal contents, closure involves either a traditional suture closure (SC) of the fascia or a “sutureless” plastic closure (PC). The advantages of plastic closure are rapid coverage, limited utilization of resources, and avoidance of abdominal compartment syndrome. We compared outcomes of SC and PC in gastroschisis babies, with and without Silo. Methods: With IRB approval, we completed a retrospective case control study of 80 gastroschisis babies treated from 2000-2009. There were 31 silos placed; 52 babies underwe...

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Necrotizing Enterocolitis After Gastroschisis Repair: Modifiable Risk Factors

Journal of Surgical Research — Wed, 19 Jan 2011 20:25:33 +0100

(Source: Journal of Surgical Research)

Non-occlusive Mesenteric Hypertension: Elucidating The Pathophysiology Of Gastroschisis-Related Intestinal Dysfunction

Journal of Surgical Research — Wed, 19 Jan 2011 20:25:32 +0100

(Source: Journal of Surgical Research)

The association of gastroschisis with other congenital anomalies: how important is it?

Prenatal Diagnosis — Thu, 13 Jan 2011 00:00:00 +0100

ConclusionThis study highlights the importance of identifying other anomalies when evaluating fetuses with gastroschisis to permit counselling concerning the postnatal outcomes. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Prenatal Diagnosis)

Challenges in the diagnosis of fetal non‐chromosomal abnormalities at 11–13 weeks

Prenatal Diagnosis — Sat, 01 Jan 2011 00:00:00 +0100

ConclusionAt 11–13 weeks some abnormalities are always detectable, some can never be and others are potentially detectable depending on their association with increased NT, the phenotypic expression of the abnormality with gestation and the objectives set for such a scan. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Prenatal Diagnosis)

Effects of multidisciplinary prenatal care and delivery mode on gastroschisis outcomes

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Conclusions: Gestational age and intestinal complications are the major determinants of outcome in gastroschisis. Multidisciplinary prenatal care may facilitate term delivery. (Source: Journal of Pediatric Surgery)

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Conditional mutation of fibroblast growth factor receptors 1 and 2 results in an omphalocele in mice associated with disruptions in ventral body wall muscle formation

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Conclusions: Mutant embryos with omphalocele arising from mutation in Fgfr1 and Fgfr2 exhibit disruptions in the development of the secondary abdominal wall structures. These findings are consistent with a model of ventral abdominal wall development in which organization of the muscles and connective tissue (secondary abdominal wall structures) is influenced by positional information emanating from the primary abdominal wall. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

The authors' report a rare case of gastroschisis (GS) in which the defect occurred to the left of the umbilicus. The parents were first cousins, and the GS was diagnosed on routine ultrasound at 21 weeks of gestation. The infant was delivered by emergency cesarian birth at 34 weeks with the defect repaired primarily. Slow feeding prompted a contrast enema at 3 weeks of life that revealed small left colon syndrome. Full feeds were commenced at 4 weeks of life, and the child remains well with no other identified anomalies at 5 years of age. (Source: Journal of Pediatric Surgery)

Maternal caffeine intake and risk of selected birth defects in the national birth defects prevention study

Birth Defects Research Part A: Clinical and Molecular Teratology — Sat, 01 Jan 2011 00:00:00 +0100

CONCLUSIONSWe did not find convincing evidence of an association between maternal caffeine intake and the birth defects included in this study. The increasing popularity of caffeine‐containing energy drinks and other caffeinated products may result in higher caffeine intake among women of childbearing age. Future studies should consider more detailed evaluation of such products. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Assessing the trend of gastroschisis prevalence in China from 1996 to 2007 using two analytical methods

Birth Defects Research Part A: Clinical and Molecular Teratology — Sat, 01 Jan 2011 00:00:00 +0100

CONCLUSIONThe overall prevalence of gastroschisis in China did not change remarkably during 1996 to 2007; but the prevalence of gastroschisis significantly increased among infants whose mothers were 20 to 24 years old and decreased among infants whose mothers were 30 to 34 years old. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Review of the evidence on the closure of abdominal wall defects

Pediatric Surgery International — Wed, 15 Dec 2010 19:11:30 +0100

Abstract Infants with congenital abdominal wall defects pose an interesting and challenging management issue for surgeons. We attempt to review the literature to define the current treatment modalities and their application in practice. In gastroschisis, the overall strategies for repair include immediate closure or delayed operative repair. The best level of data for gastroschisis is grade C and appears to support that there is no major difference in survival between immediate closure or delayed repair. In patients with omphalocele, the management techniques are more varied consisting of immediate closure, staged closure or delayed closure after epithelialization. The literature is less clear on when to use one technique over the other, consisting of mostly grade D and E ...

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Umbilical cord inverting technique: a simple method to utilize the umbilical cord as a biologic dressing for sutureless gastroschisis closure

Pediatric Surgery International — Wed, 01 Dec 2010 20:18:45 +0100

Abstract A sutureless gastroschisis closure provides a cosmetically appealing outcome. The umbilical cord is usually used as a covering material in a sutureless closure because it is a native tissue. However, during the staged closure with a silo placement, special attention is required to keep the umbilical cord moist. The authors report a simple technique to preserve the feasibility of the umbilical cord as a biologic dressing during the silo placement in staged gastroschisis closures. Content Type Journal ArticleDOI 10.1007/s00383-010-2713-3Authors Mizuho Machida, Department of Surgery, Nagano Children’s Hospital, 3100 Toyoshina, Azumino, Nagano 399-8288, JapanShigeru Takamizawa, Department of Surgery, Nagano Children’s Hospital, 3100 Toyoshina, Azumino, Nagano ...

Prenatal Presentation of Inguinoscrotal Hernia

Journal of Diagnostic Medical Sonography — Wed, 01 Dec 2010 00:00:00 +0100

Prenatal diagnosis of inguinal hernia is rare and easily confused with other entities, including masses of the scrotum, abdominal wall, and perineum. Pathologic processes of the scrotum rarely result in enlargement beyond 7 cm. Peristalsis can help to indentify bowel within the mass, suggestive of hernia or omphalocele rather than hydroceles or tumors that contain no bowel. Torsions have a double-ring appearance with no testicular blood flow, whereas hydroceles have visible testicles within a fluid-filled scrotum. Doppler evaluation may be helpful, as hernias typically have minimal vascularity, unlike tumors and teratomas, which are vascular with a widely variable appearance. The authors present the sonographic features of a late-presenting fetal mass that was clinically indistinguishable ...

Perinatal demography of gastroschisis in North Queensland

Journal of Paediatrics and Child Health — Wed, 01 Dec 2010 00:00:00 +0100

Conclusion: Gastroschisis is increasing in North Queensland, especially in mothers <20. Overall, the rate in Indigenous mothers is two to three times higher. (Source: Journal of Paediatrics and Child Health)

Closed gastroschisis

Pediatric Surgery International — Fri, 26 Nov 2010 18:04:58 +0100

Content Type Journal ArticleDOI 10.1007/s00383-010-2800-5Authors Aly Shalaby, NHS Foundation Trust, King’s College Hospital, Denmark Hill, London, SE5 9RS UKMark Davenport, NHS Foundation Trust, King’s College Hospital, Denmark Hill, London, SE5 9RS UK Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

Omphalocele: comparison of outcome following prenatal or postnatal diagnosis

Ultrasound in Obstetrics and Gynecology — Wed, 24 Nov 2010 10:34:22 +0100

ConclusionWhen counseling patients with a prenatal diagnosis of isolated omphalocele, it is important to remember that over one third could turn out to have associated anomalies. Liveborn infants with an isolated omphalocele detected prenatally have worse short‐term morbidity than do cases detected at birth. Those with non‐isolated omphaloceles detected prenatally have an increased need for ventilation and parenteral nutrition compared with those detected at birth. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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Development of gastroschisis as seen by MRI

Ultrasound in Obstetrics and Gynecology — Wed, 24 Nov 2010 10:33:33 +0100

Conclusions:Fetal development of gastroschisis is a dynamic process lasting until birth. The typical morphology of gastroschisis changes from the second to the third trimester, since intraabdominal bowel becomes eventrated by the end of the second trimester. This process of eventration is stopped in case of intestinal stenosis/atresia caused by narrowing of the abdominal wall defect, resulting in different lengths of intraabdominal bowel. The time when this occurs may correlate with the amount of viable bowel in case of intestinal atresia. The presented concept can explain the findings in closing gastroschisis. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Development of gastroschisis as seen by MRI.

The Ultrasound Review of Obstetrics and Gynecology — Tue, 23 Nov 2010 00:00:00 +0100

CONCLUSIONS:: Fetal development of gastroschisis is a dynamic process lasting until birth. The typical morphology of gastroschisis changes from the second to the third trimester, since intraabdominal bowel becomes eventrated by the end of the second trimester. This process of eventration is stopped in case of intestinal stenosis/atresia caused by narrowing of the abdominal wall defect, resulting in different lengths of intraabdominal bowel. The time when this occurs may correlate with the amount of viable bowel in case of intestinal atresia. The presented concept can explain the findings in closing gastroschisis. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 21105024 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecolog...

Development of gastroschisis as seen by magnetic resonance imaging

Ultrasound in Obstetrics and Gynecology — Tue, 23 Nov 2010 00:00:00 +0100

ConclusionsFetal development of gastroschisis is a dynamic process lasting until birth. The typical morphology of gastroschisis changes from the second to the third trimester, since intra‐abdominal bowel becomes eventrated by the end of the second trimester. This process of eventration is stopped in cases of intestinal stenosis/atresia caused by narrowing of the abdominal wall defect, resulting in different lengths of intra‐abdominal bowel. The time when this occurs may correlate with the amount of viable bowel in cases of intestinal atresia. The concept presented here explains the findings in closing gastroschisis. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Factors Affecting the Outcome in Patients with Gastroschisis: How Important Is Immediate Repair?

European Journal of Pediatric Surgery — Mon, 22 Nov 2010 00:00:00 +0100

We examined 12 recent years of patient records to evaluate the effect of immediate repair and other factors on the outcome of gastroschisis.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Early developmental assessment of children with major non‐cardiac congenital anomalies predicts development at the age of 5 years

Developmental Medicine and Child Neurology — Tue, 16 Nov 2010 00:00:41 +0100

Aim The aim of this study was to evaluate cognitive and motor development in children with major congenital anomalies and the predictability of development at age 5 years.Method A prospective, longitudinal follow‐up study was undertaken. The Dutch version of the Bayley Scales of Infant Development – Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) – were administered at the ages of 6, 12, and 24 months. The Revised Amsterdam Children’s Intelligence Test – IQ and the Movement Assessment Battery for Children – Total impairment score (TIS) were used at age 5 years. A total of 117 children participated in the study. After excluding 12 children who had a major chromosomal or syndromal abnormality, the analysis was limited to 105 children (50 fem...

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Pre-treatment with N-acetylcysteine upregulates superoxide dismutase 2 and catalase genes in cadmium-induced oxidative stress in the chick omphalocele model

Pediatric Surgery International — Wed, 10 Nov 2010 17:23:22 +0100

Conclusion Our results suggest that SOD2 and CAT may play an important role in preventing Cd-induced teratogenesis. Prenatal treatment with drugs which can upregulate SOD2 and CAT transcripts may have a therapeutic potential in preventing omphalocele phenotype. Content Type Journal ArticleDOI 10.1007/s00383-010-2794-zAuthors Takashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJohn Bannigan, School of Medicine and Medical Science, Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin, IrelandJennifer Thompson, School of Medicine and Medical Science, Conway Institute of Biomol...

Disruption of GLI3–ZIC3 interaction in the cadmium-induced omphalocele chick model

Pediatric Surgery International — Wed, 10 Nov 2010 17:23:11 +0100

Conclusion We provide evidence, for the first time, that Gli3 and Zic3 gene expression is downregulated during the narrow window of very early embryogenesis in Cd chick model. Disruption of Gli3–Zic3 interaction in the critical period for ventral body wall formation may contribute to omphalocele phenotype in Cd chick model. Content Type Journal ArticleDOI 10.1007/s00383-010-2779-yAuthors Takashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJohn Bannigan, School of Medicine and Medical Science and Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin, IrelandJennifer Thomps...

Anaesthesia for urgent and emergency surgery

Early Human Development — Sat, 30 Oct 2010 00:59:47 +0100

Abstract: The general principles that guide a pediatric anesthesiologist in the care of a newborn needing urgent and emergent neonatal surgery are reviewed. These include careful evaluation and assessment of the newborn including a detailed prenatal history. A brief review of neonatal physiology including the development of the autonomic nervous system and the development of nociceptive pathways is discussed and how important it is to suppress the stress response to surgical intervention in order to improve surgical outcomes. Most of the current general anesthetics have been associated with anesthetic neurotoxicity in juvenile mammals and several epidemiologic studies in human infants and toddlers have linked surgery occurring in the first 3 years of life with neurocognitive delays in scho...

Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant

Journal of Pediatric Surgery — Fri, 29 Oct 2010 22:28:29 +0100

We present an original case sharing characteristics common to PBS, MMIH, and OEIS complex, without a clear inclination toward any particular one. This case hints toward a common pathway in the creation of the 3 syndromes. We hypothesize that they are a spectrum of malformations based on the time frame when the mesoderm fails to create a normal interaction between infraumbilical mesoderm, urorectal septum, lumbosacral somites in the formation of the abdominal wall and the genitourinary and lower gastrointestinal tracts. (Source: Journal of Pediatric Surgery)

Postnatal intestinal ischemia in a patient with gastroschisis: a sinister problem of home delivery and improper transportation

Journal of Pediatric Surgery — Fri, 29 Oct 2010 22:28:24 +0100

Gastroschisis is a congenital malformation of anterior abdominal wall characterized by a defect usually to the right of the umbilical cord, through which intestine and rarely other organs eviscerate in utero . (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Fri, 29 Oct 2010 22:28:22 +0100

Foetal bowel dilatation in gastroschisis is traditionally taken to be an indicator of poor prognosis, and parents are counselled accordingly. Increased bowel distension is often a factor in the decision for early delivery. The aim of this study was to establish whether a correlation exists between antenatally detected bowel dilatation and the postnatal outcome for babies with gastroschisis. Antenatal ultrasound scans and subsequent postnatal notes were reviewed for cases of isolated gastroschisis from 2004 to 2008. Bowel dilatation was defined as >10 mm diameter. The type (intra- and/or extra-abdominal dilatation), maximum bowel diameter and gestation at which bowel dilatation was first seen was recorded. Outcome measures included number of days of TPN, type of closure (primary or delayed)...

What's new in obstetrics and gynecology from UpToDate

Clinical Cases and Images — Thu, 28 Oct 2010 18:28:00 +0100

35% of UpToDate topics are updated every four months. The editors select a small number of the most important updates and share them via "What's new" page. I selected the brief excerpts below from What's new in obstetrics and gynecology:ObstetricsInfluenza vaccination with inactivated vaccine is recommended for pregnant women, regardless of the stage of pregnancy. The 2010-2011 influenza vaccine is trivalent and includes antigens from both the 2009 pandemic H1N1 influenza virus and seasonal influenza viruses.Use of acetaminophen during pregnancy was associated with a reduction in neural tube defects, as well as cleft lip/palate and gastroschisis. These data support the safety of acetaminophen for relief of fever and pain.GynecologyLike CA 125, human epididymal secretory protein E4 (HE4) is...

Does gastric dilation predict adverse perinatal or surgical outcome in fetuses with gastroschisis?

Ultrasound in Obstetrics and Gynecology — Thu, 28 Oct 2010 00:00:00 +0100

ConclusionsIn gastroschisis, fetal gastric dilation is associated with meconium‐stained amniotic fluid at delivery, but is not predictive of any serious perinatal or postnatal complications. Fetal growth and well‐being should be serially evaluated on ultrasound using biophysical and Doppler assessment to decide on the optimal timing and mode of delivery. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Does gastric dilation predict adverse perinatal or surgical outcomes in fetuses with gastroschisis?

The Ultrasound Review of Obstetrics and Gynecology — Wed, 27 Oct 2010 23:00:00 +0100

CONCLUSIONS: In gastroschisis, fetal gastric dilation (FGD) was associated with meconium-stained amniotic fluid at delivery, but was not predictive of any serious perinatal or postnatal complications. Fetal growth and well-being should be serially evaluated on ultrasound using biophysical and Doppler assessment to decide on the optimal timing and mode of delivery. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 21031347 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

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Progression of a Giant Omphalocele in utero: Ultrasound and Fetal Magnetic Resonance Imaging Findings

Fetal Diagnosis and Therapy — Mon, 18 Oct 2010 15:14:49 +0100

Fetal Diagn Ther (DOI:10.1159/000320619) (Source: Fetal Diagnosis and Therapy)

Pathogenic implications of remnant vitelline structures in gastroschisis

Journal of Pediatric Surgery — Thu, 30 Sep 2010 23:00:00 +0100

Conclusions: Our findings may support the recently proposed hypothesis that the developmental failure of the yolk sac and related vitelline structures to merge with or to be incorporated into the umbilical stalk might be associated with the pathogenesis of the abdominal wall defect in gastroschisis. Paraumbilical bands derived from vitelline structures may possibly cause intestinal ischemia prenatally or postnatally. (Source: Journal of Pediatric Surgery)

Ultrafast MRI of the fetus: an increasingly important tool in prenatal diagnosis of congenital anomalies

Magnetic Resonance Imaging — Fri, 17 Sep 2010 00:00:00 +0100

Conclusion: Ultrasound is the screening method of choice for evaluation of the fetus. Ultrafast MRI is a complementary adjunctive modality with excellent tissue contrast that can image the fetus in multiple planes and add information in sonographically detected or equivocal congenital anomalies that may be significant to establish definitive accurate diagnosis and hence adequate management and counseling. (Source: Magnetic Resonance Imaging)

Comparative outcomes in intestinal atresia: a clinical outcome and pathophysiology analysis

Pediatric Surgery International — Tue, 07 Sep 2010 06:56:11 +0100

Conclusion Distal atresias are difficult to diagnose antenatally. Proximal atresias have a significantly lower birth weight than distal atresias. Associated anomaly screening is important in all atresias. Content Type Journal ArticleDOI 10.1007/s00383-010-2729-8Authors Sathyaprasad Burjonrappa, Division of Pediatric Surgery, Department of Surgery, New York Medical College, 321 Munger Pavilion, Valhalla, New York, NY 10595, USAElise Crete, Hospital Ste Justine, University of Montreal, Montreal, QC H3T 1C5, CanadaSarah Bouchard, Hospital Ste Justine, University of Montreal, Montreal, QC H3T 1C5, Canada Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature

Pediatric Surgery International — Thu, 02 Sep 2010 06:23:51 +0100

Abstract Gastroschisis and omphalocele are usually considered together since they are both congenital abdominal wall defects, and yet their anatomy, embryogenesis, and clinical presentation and problems are quite different. In addition, it appears that the risk factors for their occurrence differ. Etiologic factors contributing to the development of these defects are unknown. To investigate this we have reviewed reports of risk factors for each anomaly and report them here. We conducted a literature search using PubMed (http://www.ncbi.nlm.nih.gov/pubmed/) for risk factors implicated in the development of gastroschisis and omphalocele. The data reviewed here from clinical studies in the literature, closely parallels the data in animal studies which we reported earlier. Ther...

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Gastroschisis: a national cohort study to describe contemporary surgical strategies and outcomes

Journal of Pediatric Surgery — Tue, 31 Aug 2010 23:00:00 +0100

Conclusions: This study provides a comprehensive picture of current UK practice in the surgical management of gastroschisis. Further follow-up data will help to elucidate additional prognostic factors and guide future research. (Source: Journal of Pediatric Surgery)

Familial recurrence of gastroschisis: Literature review and data from the population-based birth registry “Mainz Model”

Journal of Pediatric Surgery — Tue, 31 Aug 2010 23:00:00 +0100

Conclusion: The existing data support the hypothesis that familial recurrence of gastroschisis is much more likely than previously thought. (Source: Journal of Pediatric Surgery)

The meaning of intraabdominal bowel dilation in fetuses with gastroschisis

American Journal of Obstetrics and Gynecology — Fri, 27 Aug 2010 00:00:00 +0100

We read with great interest the article by Huh et al, who reported their experience in the treatment of patients with a gastroschisis that was diagnosed antenatally. Their report concluded that prenatal intraabdominal bowel dilation (IBD) is associated with postnatal complication. Prediction of outcome in fetuses with gastroschisis remains an important issue in which prenatal IBD might prove useful. However, we do not believe that the data presented by Huh et al are supportive of such a conclusion at this stage. (Source: American Journal of Obstetrics and Gynecology)

The role of ultrasound and antenatal single-shot fast spin-echo MRI in the evaluation of herniated bowel in case of first trimester ultrasound diagnosis of fetal gastroschisis

Archives of Gynecology and Obstetrics — Thu, 19 Aug 2010 16:38:48 +0100

Conclusions The choice of integrating both the diagnostic procedures has shown to be clinically useful in planning the timing of delivery (Cesarean section) and in turn has been associated with an easy surgical repair and to a favorable postnatal outcome. The result of amniocentesis was crucial for the parent’s decision-making process whether to continuing with the pregnancy. Moreover, amniotic fluid α-fetoprotein levels may be used as an index of small bowel damage when loops of small bowel lied uncovered within the amniotic cavity. Content Type Journal ArticleDOI 10.1007/s00404-010-1642-7Authors Gabriele Tonni, Division of Obstetrics and Gynecology, Guastalla Civil Hospital, AUSL Reggio Emilia, Via Donatori Sangue, 2, 42016 Guastalla, RE ItalyPierpaolo Pattac...

Ultrasound prediction of birthweight and growth restriction in fetal gastroschisis

American Journal of Obstetrics and Gynecology — Wed, 18 Aug 2010 23:00:00 +0100

Conclusion: The formulas of Shepard et al and Siemer et al best estimate birthweight, and their use has the potential to reduce rates of overdiagnosis of FGR. (Source: American Journal of Obstetrics and Gynecology)

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Gastroschisis: an update

Pediatric Surgery International — Wed, 04 Aug 2010 20:29:58 +0100

Abstract Gastroschisis (GS) continues to increase in frequency, with several studies now reported an incidence of between 4 and 5 per 10,000 live births. The main risk factor would seem to be young maternal age, and it is in this group that the greatest increase has occurred. Whilst various geographical regions confer a higher risk, the impact of several other putative risk factors, including smoking and illicit drug use, may be less important than when first identified in early epidemiological studies. Over 90% of cases of GS will now be diagnosed on antenatal ultrasound, but its value in determining the need for early delivery remains unclear. There would appear no clear evidence for either routine early delivery or elective caesarean section for infants with antenatally...

A prenatally recognizable malformation syndrome associated with a recurrent post-zygotic chromosome rearrangement der(Y)t(Y;1)(q12:q21)

American Journal of Medical Genetics Part A — Mon, 02 Aug 2010 23:00:00 +0100

Several cases of mos 46,X,der(Y)t(Y;1)(q12;q21)/46,XY with multiple anomalies have been reported. I report on an additional case of a male fetus with a mosaic male karyotype mos 46,X,der(Y)t(Y;1)(q12;q21)[31]/46,XY[21] and multiple anomalies that included "teardrop"-shaped head with a triangular face, a short-nasal bridge with upturned nose, microretrognathia, microtia, kyphoscoliosis, oligodactyly, syndactyly, joint contractures, CNS malformation, omphalocele, diaphragmatic hernia, cardiac anomaly, and urogenital malformation. The findings together suggest a recurrent and recognizable syndrome and argue for using tissues such as skin or cartilage or amniotic fluid, instead of cord blood, for postmortem karyotyping in order to avoid missing mosaicism as a potential cause of multiple congen...

Correction: March 2010 (vol. 202, no. 3, page 241)

American Journal of Obstetrics and Gynecology — Sat, 31 Jul 2010 23:00:00 +0100

The authors of a research paper describing associations between atrazine exposure and fetal gastroschisis (Waller SA, Paul K, Peterson SE, Hitti J. Agricultural-related chemical exposures, season of conception, and risk of gastroschisis in Washington State. Am J Obstet Gynecol 2010;202:241.e1-6) wish to correct 2 errors. (Source: American Journal of Obstetrics and Gynecology)

Disruption of noncanonical Wnt/CA2+ pathway in the cadmium-induced omphalocele in the chick model

Journal of Pediatric Surgery — Sat, 31 Jul 2010 23:00:00 +0100

Conclusion: Downregulation of Wnt11, PKCα, and CaMKII gene expression during the narrow window of early embryogenesis may cause VBWD, interfering with cell movement and adhesion, disrupting Wnt/Ca2+ pathway. (Source: Journal of Pediatric Surgery)

Gastroschisis with Hirschsprung's disease: a therapeutic dilemma

Journal of Pediatric Surgery — Sat, 31 Jul 2010 23:00:00 +0100

Abstract: The authors report a case of a neonate with gastroschisis, which, after repair, was further complicated by the diagnosis of Hirschsprung's disease. The authors discuss the diagnostic and management dilemmas posed by the coexistence of these two conditions for the clinician. (Source: Journal of Pediatric Surgery)

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Neonatal care in patients with giant ompholocele: arduous management but favorable outcomes

Journal of Pediatric Surgery — Sat, 31 Jul 2010 23:00:00 +0100

Conclusion: Mortality rate of giant omphalocele without chromosomal anomaly or major malformations is low when treated by gradual reduction of the contents. Parents should be informed of the long hospitalization in the intensive care unit at birth, the potential nonthreatening associated malformations to be diagnosed after birth, and the high risk of sepsis. (Source: Journal of Pediatric Surgery)

Congenital Gastroschisis - Prenatal Diagnosis and Perinatal Management.

Zeitschrift fur Geburtshilfe und Neonatologie — Sat, 31 Jul 2010 23:00:00 +0100

Authors: Weichert J, Kahl FO, SchrÃ¶er A, Bohlmann MK, Diedrich K, Hartge DR The birth prevalence of gastroschisis is increasing world-wide. This situation applies particularly to young, slim women who smoke. At a first glance this is a paradox in light of the ever-increasing age of pregnant women among whom there are fewer and fewer smokers. In numerous studies it has been clearly demonstrated that not only (nutritional) teratogenic substances and environmental factors but also epidemiological causes can be held responsible for this phenomenon. Nowadays gastroschisis is detected prenatally in up to 90% of all foetuses. Advantages of a prenatal diagnosis include the identification of associated disorders and the determination of a high-risk constellation (IUGR, intraabdominal bowel d...

Intestinal atresia in association with gastroschisis: a 26-year review

Pediatric Surgery International — Fri, 30 Jul 2010 16:54:49 +0100

Conclusion Differences in outcome between the management strategies are likely to reflect an inherent variability in patient condition, site of atresia, and bowel suitability for anastomosis at first surgery, rather than the mode of surgical management. Individual management plans should be tailored to the clinical condition of each patient. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-010-2676-4Authors Rania Kronfli, Royal Hospital for Sick Children Glasgow UKTimothy J. Bradnock, Royal Hospital for Sick Children Glasgow UKAtul Sabharwal, Royal Hospital for Sick Children Glasgow UK Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

Midgut Atresia and Spontaneously Closed Gastroschisis: Support for a Mechanical Explanation

European Journal of Pediatric Surgery — Thu, 29 Jul 2010 05:19:20 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1261884© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Full text (Source: European Journal of Pediatric Surgery)