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Gastroschisis

Laparoschisis

Gastroschisis is the herniation of the intestines through an opening in the abdominal wall. It is a congenital defect that can be detected prenatally using imaging technologies, mainly ultrasonography. In most cases, the surgical intervention is performed on the first day after birth. In complicated cases, the repair is done in stages using a pouch (a silo) to contain extruded bowels, and wall closure is achieved over one or two weeks.


Presentation

There are several types of congenital abdominal wall defects that result in the extrusion of bowels and other organs, with gastroschisis being the most frequent. Gastroschisis and omphalocele are both examples of an abnormal opening in the abdominal wall that results in the bowels protruding out of the abdomen into the amniotic sac, but they are different, well-defined conditions. Omphalocele is associated with chromosomal aberrations and malformations in several other organs; in this condition, a membrane surrounds the externalized intestines. Gastroschisis does not tend to be associated with chromosomal abnormalities or problems with other organs, and there is no membranous covering on the extruded bowels.

Gastroschisis is a relatively common congenital defect affecting about 1 in 2000 births [1]. There has been a recent increase in its prevalence. The opening in the abdominal wall is mainly to the right of the umbilical cord. The herniated organ is most often the small bowel, which is not rotated. The bowels are exposed to the amniotic fluid, which may lead to a decrease in its functional capacity. Exposure to the amniotic fluid also results in increased levels of maternal alpha-fetoprotein, the detection of which may lead to the discovery of gastroschisis. Other clues for gastroschisis are fetal growth retardation and polyhydramnios [2]. Gastroschisis may also be discovered by chance during a second-trimester sonography. Fetal abdominal circumference is low in gastroschisis, making the assessment of the age of the fetus difficult [2]. Very low abdominal circumference and an abnormal gastric bubble are among the indicators for increased perinatal death or serious bowel injuries such as necrotizing enterocolitis [3]. In one study, babies with gastroschisis delivered between 35 and 37 gestational weeks were found to have the fewest perinatal complications [4].

Vomiting
  • For any other child, vomiting and fever would normally mean a bug, for our children it could mean intussusception, volvulus, or obstruction. Things most doctors rarely see. Simply put, gastroschisis means there are no road maps.[nicuawareness.org]
  • After returning home, call your provider if your baby develops any of these symptoms: Decreased bowel movements Feeding problems Fever Green or yellowish green vomit Swollen belly area Vomiting (different than normal baby spit-up) Worrisome behavioral[keckmedicine.adam.com]
  • After returning home, call your health care provider if your baby develops any of these symptoms: Decreased bowel movements Feeding problems Fever Green or yellowish green vomit Swollen belly area Vomiting (different than normal baby spit-up) Worrisome[medlineplus.gov]
  • A tube is used to drain away bile from the stomach and reduce the risk of vomiting during surgery.[news-medical.net]
Abdominal Distension
  • We report here on a newborn infant who initially presented with a history of gastroschisis, abdominal distension, and jaundice. Further studies revealed that the child had findings consistent with extrahepatic biliary atresia (EHBA).[ncbi.nlm.nih.gov]
  • Neurodevelopment was not delayed. [ 25 ] One study found that the readmission rate of gastroschisis patients after initial discharge was 40%. 65% of readmissions occurred in the first year, the main indications being abdominal distension/pain and bowel[patient.info]
Nausea
  • Zofran, also known as ondansetron, is an anti-nausea medication approved for cancer patients and people undergoing surgery who experience the side effects of nausea and vomiting.[zofranlawsuitguide.com]
Recurrent Abdominal Pain
  • Prevalence of recurrent abdominal pain was 22.5% (9/40) among children with gastroschisis compared to 12% in a study on Danish school children, p 0.068.[ncbi.nlm.nih.gov]
Tachycardia
  • In rare circumstances, the catheter tip may advance into intracardiac chambers, precipitating supraventricular re-entrant tachycardia.[ncbi.nlm.nih.gov]
Brachydactyly
  • In this report we describe a girl with gastroschisis and pulmonary hypoplasia in addition to features of Moebius syndrome comprising hypoplasia of the tongue and mandible, brachydactyly of halluces, cranial nerve palsies with bilateral facial paralysis[ncbi.nlm.nih.gov]

Workup

The most important imaging tool for the diagnosis of gastroschisis is antenatal ultrasonography, which is the cornerstone of imaging in pregnancy because it is noninvasive and not associated with exposure to radiation. Radiography is only used in the postnatal period and, for the same reason, the application of computerized tomography (CT) is inappropriate antenatally.

Ultrasonography allows for the detection of the location and features of the abdominal faults and differentiates amongst the different types of abdominal anomalies [5]. In the case of gastroschisis, the herniated bowels have no membranous covering; thus, the image shows free-floating bowel loops with irregular shapes. Although the small bowel is most frequently extruded, other organs may also be found floating in the amniotic fluid, such as the large intestines, stomach, urinary bladder, uterus, fallopian tubes, and testes. The extruded intestines may be inflamed and swollen.

Various studies have examined the value of fetal intestinal and other features detected by ultrasonography in predicting later complications. In addition to the signs mentioned above in connection with adverse outcomes, several other characteristics have been studied as predictors of complications. Intra-abdominal bowel dilatation and polyhydramnios were both associated with bowel atresia [6]. The predictive value of extra-abdominal dilatation is questionable: although one recent study found a significant association between extra-abdominal bowel dilatation and morbidity, a large percentage of patients with this type of anomaly were free from complications [7]. Magnetic resonance imaging (MRI) is used mainly in complicated cases or where ultrasound results are equivocal.

Abnormal Amniotic Fluid
  • By establishing the significance of features, including extra- and intra-abdominal bowel dilatation, stomach herniation, stomach dilatation, bowel matting, growth restriction, abnormal umbilical artery (UA) Doppler ultrasounds, and abnormal amniotic fluid[ncbi.nlm.nih.gov]
Liver Biopsy
  • The diagnosis of metastatic neuroblastoma was confirmed after birth through liver biopsy. At 2 days of life, the prothrombrin time was abnormal, and the patient needed vitamin K.[ncbi.nlm.nih.gov]

Treatment

  • Knowledge of the complexities with medically fragile children can assist the pediatric physical therapist develop an appropriate treatment-planning approach.[ncbi.nlm.nih.gov]
  • BACKGROUND: Core outcome sets (COS) facilitate clinical research by providing an agreed set of outcomes to be measured when evaluating treatment efficacy. Gastroschisis is increasing in frequency and evidence-based treatments are lacking.[ncbi.nlm.nih.gov]
  • She is now seven months of age, gaining weight and has had no additional fractures with the ongoing pamidronate treatment.[ncbi.nlm.nih.gov]
  • PURPOSE OF REVIEW: The diagnosis and treatment of gastroschisis spans the perinatal disciplines of maternal fetal medicine, neonatology, and pediatric surgery.[ncbi.nlm.nih.gov]
  • This study aimed to identify the factors associated with the successful early enteral feeding in gastroschisis and to develop further guidelines of treatment.[ncbi.nlm.nih.gov]

Prognosis

  • In newborns with isolated gastroschisis, the overall prognosis is very good. In rare cases, gastroschisis is associated with other congenital malformations, making the prognosis worse.[ncbi.nlm.nih.gov]
  • As a result of the complexity and rarity of this congenital abnormality, reports with a positive prognosis carry clinical relevance.[ncbi.nlm.nih.gov]
  • Overall prognosis is good with low long-term morbidity in the majority of cases, but approximately 15% of cases are very complex with complicated hospital course, extensive intestinal loss, and early childhood death.[ncbi.nlm.nih.gov]
  • The prognosis of these cases is poor, but detecting these problems during pregnancy (antenatal diagnosis) and having fetal intervention may improve the outcome in the future. [6] Last updated: 11/21/2016[web.archive.org]
  • This indicated good prognosis of the antenatally eviscerated bladder.[ncbi.nlm.nih.gov]

Etiology

  • CONCLUSIONS: These previously unreported combinations provide further support for vascular disruption in embryonic or fetal life as an etiological factor in these conditions. 2010 Wiley-Liss, Inc.[ncbi.nlm.nih.gov]
  • Because gastroschisis patients are known to have abdominal pain later in life, this case shows that the differential diagnosis should include etiologies beyond the gastrointestinal tract.[ncbi.nlm.nih.gov]
  • We cannot rule out the possibility that a clotting defect, commonly observed in disseminated malignancies such as a metastatic neuroblastoma may be associated with the etiology of the gastroschisis, as this defect may result from a thrombosis occurring[ncbi.nlm.nih.gov]
  • The etiology of gastroschisis is unknown. Ongoing research suggests multiple causal factors may be involved, including Chlamydia infection in mothers during pregnancy.[ncbi.nlm.nih.gov]
  • Although the etiology remains unknown, it may differ from that of right-sided gastroschisis.[ncbi.nlm.nih.gov]

Epidemiology

  • We analyzed 11 administrative regions of Poland with complete epidemiologic data.[ncbi.nlm.nih.gov]
  • Since gastroschisis is one of the commonest and costliest structural birth defects treated in neonatal ICUs, a comprehensive review of its epidemiology, prenatal diagnosis, postnatal treatment, and short and long-term outcomes is both timely and relevant[ncbi.nlm.nih.gov]
  • A review of gastroschisis, which emphasizes its epidemiology, multidisciplinary care strategies and contemporary outcomes is timely.[ncbi.nlm.nih.gov]
  • Author information 1 National Perinatal Epidemiology Unit, Oxford, OX37LF, UK. 2 Department of Paediatric Surgery, Oxford Children's Hospital, Oxford, OX39DU, UK. 3 University of Oxford Medical School Medical Sciences Divisional Office University of Oxford[ncbi.nlm.nih.gov]
  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.ro]
Sex distribution
Age distribution

Pathophysiology

  • Outcomes were extracted and assigned to the core areas, 'Pathophysiological Manifestations', 'Life Impact', 'Resource Use', 'Adverse Events' and 'Mortality'. RESULTS: A total of 50 outcomes were identified.[ncbi.nlm.nih.gov]
  • […] understood, and a better understanding of these forces would help to explain why gastroschisis occurs mostly to the right of the umbilicus, while other ventral body wall defects occur in the midline.At least six hypotheses have been proposed for the pathophysiology[en.wikipedia.org]

Prevention

  • Bird TM, Robbins JM, Druschel C, Cleves MA, Yang S, Hobbs CA, & the National Birth Defects Prevention Study. Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study.[web.archive.org]
  • It also prevents future herniation through the anatomical defect of the abdominal wall.[ncbi.nlm.nih.gov]
  • AF exchange and infusion may prevent intestinal damage and improve postnatal outcome in gastroschisis by diluting the AF, probably by lowering the concentrations of intestinal waste products.[ncbi.nlm.nih.gov]

References

Article

  1. Eggink BH, Richardson CJ, Malloy MH, Angel CA. Outcome of gastroschisis: a 20-year case review of infants with gastroschisis born in Galveston, Texas. J Pediatr Surg. 2006;41(6):1103-1108.
  2. Siemer J, Hilbert A, Hart N, et al. Specific weight formula for fetuses with abdominal wall defects. Ultrasound Obstet Gynecol. 2008;31(4):397-400.
  3. Sinkey RG, Habli MA, South AP, et al. Sonographic markers associated with adverse neonatal outcomes among fetuses with gastroschisis: an 11-year, single-center review. 2016;214(2):275.e1-275.e7.
  4. Mesas Burgos C, Svenningsson A, Vejde JH, Granholm T, Conner P. Outcomes in infants with prenatally diagnosed gastroschisis and planned preterm delivery. Pediatr Surg Int. 2015;31(11):1047-1053.
  5. Fogata ML, Collins HB 2nd, Wagner CW, Angtuaco TL. Prenatal diagnosis of complicated abdominal wall defects. Curr Probl Diagn Radiol. 1999;28(4):101-128.
  6. D'Antonio F, Virgone C, Rizzo G, et al. Prenatal Risk Factors and Outcomes in Gastroschisis: A Meta-Analysis. Pediatrics. 2015;136(1):e159-169.
  7. Robertson JA, Kimble RM, Stockton K, Sekar R. Antenatal ultrasound features in fetuses with gastroschisis and its prediction in neonatal outcome. Aust N Z J Obstet Gynaecol. 2017;57(1):52-56.

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Last updated: 2019-07-11 22:09