Gastroschisis

Este  es el resumen de la presentacion en video de la manera de abordar las gastrosquisis en los últimos  25 años  por el grupo de cirugía pediatrica  de alta complejidad y minima invasion  de la Org  Clinica General del Norte
Ver video:    http://youtu.be/86oMGrH8PY0

Omphalocele and Gastroschisis are two of the rarest ventral abdominal wall defects which occur due to congenital malformations in utero. These defects fail to fuse the abdominal wall properly, which expose the abdominal organs such as the intestines, stomach, and liver outside the abdominal cavity. Omphalocele is the protrusion of the abdominal contents covered with peritoneum sac through the base of the umbilical cord.[1] It consists of Wharton’s jelly in between both the amnion and peritoneum membrane. Meanwhile, Gastroschisis usually occurs to the right of the belly button and is the protrusion of the abdominal contents without the peritoneum sac covering.[1] Both of these birth defects can be detected prenatally by fetal ultrasonography as well as treated. Despite the technological advances in medicine, the explanation behind exactly why or how these defects occur are unclear. To examine the roles of different vitamins and their possible roles in the prevalence as well as manifestation of ventral

We report a baby boy with gastroschisis with left non-palpable undescended testis who had a defect on the left side of an intact and normal umbilical cord and had associated testicular atrophy and abnormalities of the ductus deferens. They were successfully managed by primary repair and had uneventful recovery. Subsequent inguinal exploration confirmed blind ending vas deferens and vanishing left undescended testis. Our case confirms vascular accidents at the narrow abdominal wall defect can lead to vanishing testis following attempts at closing gastroschisis making the defect narrow and compromising the blood supply to the testis.

Hypertrophic pyloric stenosis is a very common surgical problem in infants. It occurs most often in otherwise well babies with normal gestation and birth history. Rarely, pyloric stenosis has been described in babies with history of prior abdominal surgery. Below we discuss the management of hypertrophic pyloric stenosis in a child who remained hospitalized, recovering from repair of a congenital abdominal wall defect.

The birth prevalence of gastroschisis worldwide has increased over the past decades. We aim to determine the Irish national incidence of gastroschisis repairs (NIGR) over a 5 year period (2007- 2011) and clinical outcomes by a retrospective cohort review of cases admitted to all Irish paediatric surgical units. Seventy patients were identified. The NIGR per 10,000 live births was 1.96 (SD 0.51) per year. Fifty eight (82%) were antenatally detected. Twenty eight (40%) had primary repair day 1 with the remaining repaired in a median of 3(2-5.75) days. Thirty three (47%) experienced a central catheter related infection. Duration of stay was significantly correlated with decreasing gestational age (p=0.016), decreasing birthweight (p=0.005), increasing numbers of blood transfusions (p<0.001) and co-morbidity or complication (p<0.001). This study provides individual centres with patient outcomes and national data that can be provided to parents and clinical staff regarding the clin...

We present a neonate with gastroschisis and evidence of bile aspiration in utero, who developed severe respiratory distress that did not respond to postnatal intensive respiratory care. Although rare, a newborn with gastroschisis may develop severe respiratory distress due to bile aspiration in utero. Given the poor outcome in this case, we suggest a possible role for prenatal diagnosis and therapy.

Gastroschisis is a congenital abdominal wall defect, thought by many to represent a disruption in intrauterine blood flow, where there is herniation of abdominal organs. Dietary intake is an important environmental factor that has been implicated in the development of many diseases. Omega-6 polyunsaturated fatty acids (PUFAs) are nutrients that are substrates for eicosanoid and cytokine synthesis and prone to oxidation, and play a role in modulating inflammation, immune function, and vascular system development. This pilot case-control study explored the association of dietary intake of the omega-6 PUFA linoleic acid with risk of gastroschisis. Between 2008 and 2011, we recruited 13 pregnant women in mid-gestation who were referred to the UCSD Prenatal Center for evaluation of an abnormal alpha-fetoprotein (AFP) test and subsequently identified as carrying a baby with gastroschisis. Nine controls were selected from a false positive AFP or from the UCSD prenatal clinic. Maternal dietary intake was collected via repeated food record during the last 20 weeks of gestation. Logistic regression was used to test the association between dietary intake of linoleic acid and odds of gastroschisis. Dietary intake of linoleic acid was associated with increased odds of gastroschisis (OR = 1.72; 95% CI: 1.08, 2.74; P = 0.02). A higher maternal intake of omega-6 PUFAs may increase the risk of having a baby with gastroschisis. The mechanism by which this occurs may be via inflammatory processes and oxidative stress leading to a vascular disruption. More research is needed including studies investigating integrated markers of PUFA status or inflammatory markers. © 2012 Wiley Periodicals, Inc.

Abstract:  Long-term graft dysfunction and/or graft loss after intestinal transplantation (ITx) is a significant concern. Sclerosing peritonitis (SP) is a manifestation of chronic allograft failure and its presence may also include classic arterial obliterative arteriopathy (OA) as in chronic rejection. We describe the clinical presentation and management of SP occurring after ITx in children. Case records of 121 children undergoing ITx from 1990 to 2003 were reviewed. Three children (2.4%) presented with SP of the intestine allograft at a mean time of 6.6 yr following ITx as follows: age at Tx (yr) 8.2, and 3.7, with indication for ITx being gastroschisis in two and midgut volvulus in one patient. Type of ITx was isolated intestine in one and liver/intestine in two patients. Gross findings of SP included fibrosis/strictures; microscopically SP showed fibrosis/serositis, and fibrous adhesions; one patient had evidence of chronic allograft vasculopathy. All patients presented with clinical signs and symptoms of bowel obstruction and gastrointestinal contrast studies confirmed distal ileal obstruction (DIO). Operative findings confirmed SP and DIO in all patients; all patients were initially treated with distal segmental intestine allograft resection and lysis of the fibrous peel. All three patients recovered, although two required repeat laparotomy, there is only one long-term survival. SP after ITx may be a different manifestation of long-term intestine allograft degeneration. Surgical resection appears to offer palliation.

Antenatal detection of anterior abdominal wall defects (gastroschisis and exomphalos) enables detailed prenatal planning and counselling with appropriate intrauterine transfer, delivery in a tertiary referral centre with prompt access to paediatric surgery and early surgical intervention. The authors believed that there was a relatively low rate of antenatal detection in Ireland and that an improved antenatal screening program would allow increased detection thus avoiding emergency retrievals from peripheral obstetric centres. Our hypothesis was that there was a significant difference in management and outcome in children with anterior abdominal wall defects detected antenatally and those detected at birth. All anterior abdominal wall defects in The Republic of Ireland are assessed and closed in two surgical centres, Our Lady's Hospital for Sick Children, and the Children's University Hospital, Dublin. A retrospective review of all admissions in both centres revealed 96 neon...

Gastroschisis, a congenital anomaly of the abdomen, is associated with young maternal age and has increased in prevalence in many countries. Maternal illness and medication exposure are among environmental risk factors implicated in its aetiology. A population-based case-malformed control study was conducted using data from 18 European congenital anomaly registries, with information on first trimester medication use, covering 8 million births 1995-2012. 1577 gastroschisis cases (of which 4% stillbirths, 11% terminations of pregnancy) were compared to 153 357 non-chromosomal/monogenic controls. Literature review identified previous associations concerning maternal illness and medication exposure to be tested as signals. Logistic regression adjusted for maternal age group, registry, and time period was used to evaluate associations. Comparing gastroschisis to other congenital anomalies, the data supported signals concerning maternal depression (aOR 2.52, 95% CI 1.45, 4.39), antidepres...