GERD in Children: an Update
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Abdominal Wall Defect
Abdominal wall defect Description An abdominal wall defect is an opening in the abdomen through which various abdominal organs can protrude. This opening varies in size and can usually be diagnosed early in fetal development, typically between the tenth and fourteenth weeks of pregnancy. There are two main types of abdominal wall defects: omphalocele and gastroschisis. Omphalocele is an opening in the center of the abdominal wall where the umbilical cord meets the abdomen. Organs (typically the intestines, stomach, and liver) protrude through the opening into the umbilical cord and are covered by the same protective membrane that covers the umbilical cord. Gastroschisis is a defect in the abdominal wall, usually to the right of the umbilical cord, through which the large and small intestines protrude (although other organs may sometimes bulge out). There is no membrane covering the exposed organs in gastroschisis. Fetuses with omphalocele may grow slowly before birth (intrauterine growth retardation) and they may be born prematurely. Individuals with omphalocele frequently have multiple birth defects, such as a congenital heart defect. Additionally, underdevelopment of the lungs is often associated with omphalocele because the abdominal organs normally provide a framework for chest wall growth. When those organs are misplaced, the chest wall does not form properly, providing a smaller than normal space for the lungs to develop. As a result, many infants with omphalocele have respiratory insufficiency and may need to be supported with a machine to help them breathe ( mechanical ventilation). Rarely, affected individuals who have breathing problems in infancy experience recurrent lung infections or asthma later in life. -
Gastro-Esophageal Reflux in Children
International Journal of Molecular Sciences Review Gastro-Esophageal Reflux in Children Anna Rybak 1 ID , Marcella Pesce 1,2, Nikhil Thapar 1,3 and Osvaldo Borrelli 1,* 1 Department of Gastroenterology, Division of Neurogastroenterology and Motility, Great Ormond Street Hospital, London WC1N 3JH, UK; [email protected] (A.R.); [email protected] (M.P.); [email protected] (N.T.) 2 Department of Clinical Medicine and Surgery, University of Naples Federico II, 80138 Napoli, Italy 3 Stem Cells and Regenerative Medicine, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK * Correspondence: [email protected]; Tel.: +44(0)20-7405-9200 (ext. 5971); Fax: +44(0)20-7813-8382 Received: 5 June 2017; Accepted: 14 July 2017; Published: 1 August 2017 Abstract: Gastro-esophageal reflux (GER) is common in infants and children and has a varied clinical presentation: from infants with innocent regurgitation to infants and children with severe esophageal and extra-esophageal complications that define pathological gastro-esophageal reflux disease (GERD). Although the pathophysiology is similar to that of adults, symptoms of GERD in infants and children are often distinct from classic ones such as heartburn. The passage of gastric contents into the esophagus is a normal phenomenon occurring many times a day both in adults and children, but, in infants, several factors contribute to exacerbate this phenomenon, including a liquid milk-based diet, recumbent position and both structural and functional immaturity of the gastro-esophageal junction. This article focuses on the presentation, diagnosis and treatment of GERD that occurs in infants and children, based on available and current guidelines. -
Omphalocele Handout
fetal treatment PROGRAM OF NEW ENGLAND Hasbro Children’s Hospital | Women & Infants Hospital | Brown University Omphalocele What is omphalocele? Omphalocele is a condition in which loops of intestines (and sometimes parts of the stomach, liver and other organs) protrude from the fetus’s body through a hole in the abdominal wall. The hole is located at the belly button and is covered by a membrane, which provides some protection for the exteriorized organs. The umbilical cord inserts at the top of this membrane rather than on the abdomen itself. Omphalocele is often confused with gastroschisis, a similar condition in which the hole in the abdominal wall is located to the side (usually the left) of the umbilical cord. Omphaloceles come in all sizes: they may only contain one or two small loops of intestine and resemble an umbilical hernia, or they may be much larger and contain most of the liver. These are called “giant” omphalocele and are more difficult to treat. How common is it? Omphalocele occurs in approximately one in 5,000 births and is associated with other conditions and chromosomal anomalies in 50 percent of cases. How is it diagnosed? Omphalocele can be detected through ultrasound from 14 weeks of gestation; however, it is easier to diagnose as the pregnancy progresses and organs can be seen outside the abdomen protruding into the amniotic cavity. Because of the high risk of associated conditions, a prenatal test called an amniocentesis may be performed to help detect chromosomal and heart anomalies. • Amniocentesis: Under ultrasound guidance, a fine needle is inserted through the abdomen into the uterus. -
Gastroschisis and Omphalocele
Gastroschisis and Omphalocele The two most common congenital abdominal wall At delivery, the ABC (airway, breathing, circulation) rule defects are gastroschisis and omphalocele. Both involve should be followed for babies with gastroschisis or incomplete closure of the abdominal wall during fetal omphalocele. Immediately afterward, protection of the development, and for both, their cause is unknown. A herniated contents and management of evaporative loss gastroschisis is usually an isolated congenital defect, should be accomplished. Abdominal contents should be whereas a baby with an omphalocele often has chromo- wrapped in warm, saline-soaked gauze and covered with some anomalies, cardiac conditions, and other major birth plastic wrap. Alternatively, the baby should be placed in defects. a sterile bowel bag up to the nipple line. Preventing evap- orative fluid loss is particularly important for the baby A gastroschisis is a herniation of abdominal contents with gastroschisis because of the lack of the protective through a defect in the abdominal wall, usually just to the membranous covering of the abdominal contents. Dili- right of the umbilicus. An omphalocele is a herniation of gent observation of the color and perfusion of the abdom- abdominal contents into the umbilical cord itself. The con- inal contents of a baby with gastroschisis is imperative. tents of a gastroschisis are directly exposed to amniotic The baby should be placed on his or her right side with fluid, whereas the contents of an omphalocele are usually abdominal contents supported with additional gauze or covered with a protective membranous sac. blankets to prevent kinking of the mesentery blood ves- sels. An echocardiogram also should be considered to rule out potential cardiac anomalies (Escobar & Caty, 2016). -
Abdominal Wall Defects—Current Treatments
children Review Abdominal Wall Defects—Current Treatments Isabella N. Bielicki 1, Stig Somme 2, Giovanni Frongia 3, Stefan G. Holland-Cunz 1 and Raphael N. Vuille-dit-Bille 1,* 1 Department of Pediatric Surgery, University Children’s Hospital of Basel (UKBB), 4056 Basel, Switzerland; [email protected] (I.N.B.); [email protected] (S.G.H.-C.) 2 Department of Pediatric Surgery, University Children’s Hospital of Colorado, Aurora, CO 80045, USA; [email protected] 3 Section of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, 69120 Heidelberg, Germany; [email protected] * Correspondence: [email protected]; Tel.: +41-61-704-27-98 Abstract: Gastroschisis and omphalocele reflect the two most common abdominal wall defects in newborns. First postnatal care consists of defect coverage, avoidance of fluid and heat loss, fluid administration and gastric decompression. Definitive treatment is achieved by defect reduction and abdominal wall closure. Different techniques and timings are used depending on type and size of defect, the abdominal domain and comorbidities of the child. The present review aims to provide an overview of current treatments. Keywords: abdominal wall defect; gastroschisis; omphalocele; treatment 1. Gastroschisis Citation: Bielicki, I.N.; Somme, S.; 1.1. Introduction Frongia, G.; Holland-Cunz, S.G.; Gastroschisis is one of the most common congenital abdominal wall defects in new- Vuille-dit-Bille, R.N. Abdominal Wall borns. Children born with gastroschisis have a full-thickness paraumbilical abdominal Defects—Current Treatments. wall defect, which is associated with evisceration of bowel and sometimes other organs Children 2021, 8, 170. -
Management of Omphalocele with Intestinal Perforation: a Case Report
Journal of Pediatrics and Neonatal Care Management of Omphalocele with Intestinal Perforation: A Case Report Abstract Case Report Volume 2 Issue 6 - 2015 Congenital abdominal wall defects present a huge challenge for pediatric surgeons in the care of neonates. The risks of infection and restriction of blood supply to Hector Quintero, Ortiz-Justiniano V*, Zasha abdominal organs challenge the surgeons’ capacity to restore the stability of the Vazquez and Ada Rivera patient. Omphalocele is a defect of the abdominal wall where the organs protrude University of Puerto Rico Medical Sciences Campus, Puerto enclosed within a membranous sac. Carrying a mortality rate of 34%, an incidence Rico of 1 in 4,000 live births and being more common in males than in females [1,2] makes it more difficult for the surgeon to manage complications. There are few *Corresponding author: Ortiz-Justiniano V, Puerto Rico reports of intestinal perforation in patients with omphalocele [3]. We report the case of a 2-days-old boy who presented with omphalocele that required surgical Childrens Hospital Ofic. 302 Carr Num. 2, Km 11.7 Edif. excision of the membranous sac for management of intestinal perforation as a life Medical Plaza Bayamon, PR 00960, Puerto Rico, Tel: 787- Keywords:saving procedure. 474-5423; Fax: 787-523-2768; Email: Congenital abdominal wall defect; Omphalocele; Intestinal Received: June 11, 2015 | Published: perforation; Neonate; Spring-loaded silo September 8, 2015 Abbreviations: WGA: Weeks of Gestational Age; NICU: common technique used nowadays is daily application of silver Neonatal Intensive Care Unit; NGT: French Nasogastric Tube; sulfadiazine covered by cotton free gauze and elastic bandage. -
Redalyc.Overview of Short Bowel Syndrome and Intestinal
Colombia Médica ISSN: 0120-8322 [email protected] u.co Universidad del Valle Colombia Duro, Debora; Kamin, Daniel Overview of short bowel syndrome and intestinal transplantation Colombia Médica, vol. 38, núm. 1, enero-marzo, 2007, pp. 71-74 Universidad del Valle Cali, Colombia Available in: http://www.redalyc.org/articulo.oa?id=28309911 How to cite Complete issue Scientific Information System More information about this article Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Journal's homepage in redalyc.org Non-profit academic project, developed under the open access initiative Colombia Médica Vol. 38 Nº 1 (Supl 1), 2007 (Enero-Marzo) Overview of short bowel syndrome and intestinal transplantation DEBORA D URO , M.D., M.S.*, D ANIEL K AMIN , M.D. * SUMMARY Short bowel syndrome is at once a surgical, medical, and a disorder, with potential for life-threatening complications as well as eventual independence from artificial nutrition. Navigating through the diagnostic and therapeutic decisions is ideally accomplished by a multidisciplinary team comprised of nutrition, pharmacy, social work, medicine, and surgery. Early identification of patients at risk for long-term PN-dependency is the first step towards avoiding severe complications. Close monitoring of nutritional status, steady and early introduction of enteral nutrition, and aggressive prevention, diagnosis and treatment of infections such as line sepsis, and bacterial overgrowth can significantly improve prognosis. Intestinal transplantation is an emerging treatment that may be considered when intestinal failure is irreversible and children are suffering from serious complications rela ted to TPN administration. Keywords: Short bowel syndrome; Intestinal transplantation; Children. -
Prenatal Counseling: Omphalocele
In partnership with Primary Children’s Hospital Prenatal Counseling: Omphalocele What is an omphalocele? An omphalocele [ohm-FAL-oh-seel] occurs when a baby’s abdominal (belly) organs push out of the body and into the umbilical cord during early fetal development. The omphalocele is almost always covered by a membrane (thin covering), which keeps Umbilical cord the organs from floating freely in the amniotic fluid (liquid protecting the baby in the womb). Omphalocele Omphaloceles can vary in size from small to large. A “giant” omphalocele contains most of the organs from the belly, including the liver. This may tell the doctor that there is a space between the belly muscles larger than 5 centimeters (about two inches). About 1 in 5,000 babies are born with an omphalocele each year, but only 1 in 10,000 babies have a giant An omphalocele occurs when the belly organs omphalocele. We do not know what causes an push outside the body into the umbilical cord. omphalocele, but nothing the mother does or has done during pregnancy causes this problem. • A fetal echocardiogram [ek-oh-CAR-dee-oh-gram] or The Utah Fetal Center team will help you make ECG. This ultrasound, performed by a pediatric the best possible decisions about your baby’s cardiologist, looks at the structure and function of omphalocele. The team includes maternal-fetal your baby’s heart. Babies with omphaloceles have medicine specialists for the pregnant mother, an increased risk of heart problems. In fact, the neonatologists who are specially trained to care for smaller the omphalocele, the higher the risk of a newborns, and pediatric specialists to help with congenital heart defect. -
NICU Omphalocele Clinical Guideline
Omphalocele Clinical Guideline Inclusion Criteria: Available Resources: o Any neonate born with an Omphalocele regardless of size o Omphalocele PFE or gestation o Omphalocele wrap video Prenatal Recommendations: Antepartum Care: o Elevated maternal serum alpha-fetoprotein level o Ultrasound suspicious for Omphalocele : refer to Maternal Fetal Medicine for detailed ultrasound exam o MFM ultrasound to include evaluation for other abnormalities, description of organ involvement, and preliminary counseling/consultation o Referral to Pediatric Cardiology for fetal echocardiogram @ approximately 22 weeks. o Consider need for fetal MRI for further evaluation of anatomy and lung volumes o Genetics consultation with discussion of amniocentesis o Referral to Pediatric Surgery o Multidisciplinary care meeting to involve OB, MFM, Neonatology, Genetics and Pediatric Surgery Delivery: o Recommended delivery at a Level IV medical center o Vaginal delivery may be possible in small omphaloceles. Cesarean deliveries warranted for giant omphaloceles to prevent omphalocele rupture and trauma to enclosed organs, specifically liver o Encouragement of full term delivery but delivery may be warranted earlier for fetal and/ or maternal indications Delivery Room Anticipation and Resuscitation: Pre -briefing: o Team huddle with discussion of plan of care and clearly defined team member roles o Advanced preparation of supplies including equipment for intubation, 8 fr (preterm) and 10 fr (term) salem sump, bowel (lahey) bag, and potential normal saline fluid boluses and resuscitative medications. Delivery/ Resuscitation: Placement of 8 fr (preterm) and 10 fr (term) salem sump orogastric or nasogastric tube to low intermittent o suction o Assess respiratory status. Small omphaloceles may not require additional support, whereas large omphaloceles may require CPAP or intubation. -
PROGRAF (Tacrolimus)
HIGHLIGHTS OF PRESCRIBING INFORMATION These highlights do not include all the information needed to use PEDIATRIC PROGRAF® safely and effectively. See full prescribing information for PROGRAF. Initial Oral Dosage Whole Blood Trough Patient Population (formulation) Concentration Range PROGRAF (tacrolimus) capsules, for oral use PROGRAF (tacrolimus) injection, for intravenous use Kidney Transplant PROGRAF Granules (tacrolimus for oral suspension) Initial U.S. Approval: 1994 0.3 mg/kg/day capsules or Month 1-12: 5-20 ng/mL oral suspension, divided in two doses, every 12 hours WARNING: MALIGNANCIES and SERIOUS INFECTIONS Liver Transplant See full prescribing information for complete boxed warning. 0.15-0.2 mg/kg/day capsules Month 1-12: 5-20 ng/mL Increased risk for developing serious infections and malignancies or 0.2 mg/kg/day oral with PROGRAF or other immunosuppressants that may lead to suspension, divided in two hospitalization or death. (5.1, 5.2) doses, every 12 hours -------------------------- RECENT MAJOR CHANGES -------------------------- Heart Transplant Indications and Usage (1.1) 7/2021 2 Dosage and Administration (2.2, 2.3) 7/2021 0.3 mg/kg/day capsules or Month 1-12: 5-20 ng/mL Warnings and Precautions (5.11) 12/2020 oral suspension, divided in two doses, every 12 hours --------------------------- INDICATIONS AND USAGE -------------------------- PROGRAF is a calcineurin-inhibitor immunosuppressant indicated for the Lung Transplant prophylaxis of organ rejection in adult and pediatric patients receiving 2 allogeneic liver, kidney, heart, or lung transplants, in combination with other 0.3 mg/kg/day1, capsules or Weeks 1-2: 10-20 ng/mL immunosuppressants. (1.1) oral suspension, divided in Week 2 to Month 12: 10- two doses, every 12 hours 15 ng/mL ---------------------- DOSAGE AND ADMINISTRATION ---------------------- MMF= Mycophenolate mofetil ADULT 1. -
Short Bowel Syndrome Definition of Short Bowel Syndrome
Short Bowel Syndrome Definition of Short Bowel Syndrome Short bowel compromises of a sequelae of; Nutrient Fluid Weight loss which occurs subsequent to a significant amount of small bowel resection with greatly reduced functional surface area. Average length of the small bowel is; Neonatal 250cm-300cm Adult 600-800cm Short bowel syndrome is defined by resection of >50% or more of the small bowel if no colon present and resection of 70-75% if some or all of colon present. Infants have more favorable long term prognosis outcomes, over adult prognosis outcomes Aetiology Can be congenital or acquired Congenital Intestinal atresia Gastroschisis Omphalocele Hirschsprung’s disease Acquired Necrotizing Enterocolitis (NEC) Mid-Gut volvulus Ischemic injury Crohn’s disease Radiation enteritis Manifestation SBS includes a spectrum of metabolic and physiologic disturbances which include Fluid & Electrolyte Imbalance Malabsorption of macro and Gastric acid hypersecretion micronutrients Cholelithiasis Steatorrhea Liver Disease Weight Loss & Malnutrition Bone Disease Mineral deficiencies : Ca, Mg, PN dependency initially Iron, Zinc PN related complications Fat soluble vitamins (Line Infections/Sepsis) Metabolic acidosis Types of Small Bowel Resections Duodenal Resection Jejunal Resection Ileal Resection Loss of the ileocecal valve Colon Duodenal Resection Resection of the Duodenum results in the following deficits: Protein, carbohydrates, fat malabsorption Calcium, Magnesium, Iron, Folate malabsorption Fat soluble vitamin -
Conservative Treatment Ofgiant Omphalocele*
Arch Dis Child: first published as 10.1136/adc.38.198.130 on 1 April 1963. Downloaded from Arch. Dis. Childh., 1963, 38, 130. CONSERVATIVE TREATMENT OF GIANT OMPHALOCELE* BY F. SOAVE From the Department ofPaediatric Surgery, Istituto G. Gaslini, Genoa, Italy Omphalocele is a congenital malformation charac- cases. Many, such as harelip, palatoschisis, extro- terized by the failure of the abdominal walls to join phia of the bladder, meningocele, inguinal hernia together on the middle line as a consequence of a and talipes, do not require immediate treatment. developmental defect. On the other hand, it is of the utmost importance The diameter of the resulting opening may range that the presence of more severe anomalies such as from a few centimetres to the almost total absence atresia of the intestine, duplication, caecal intussus- of the abdominal wall. ception, malrotation, patent omphalomesenteric The diameter of the defect usually ranges from ductus, or trans-diaphragmatic hernia is investi- 3 to 8 cm. Naturally, the wider the opening, the gated. more completely do the intestine and liver, covered These malformations may be associated with only by the thin amniotic sac and peritoneum, omphaloceles of any size. Whatever the size of protrude from the abdomen. the hernia, there is always a disproportion between The sac is so transparent that the abdominal the volume of the organs contained in it and the organs contained in it can easily be recognized. capacity of the abdominal cavity into which this mass must be replaced, thus giving rise to a difficult Incidence surgical problem. Omphalocele is a rare condition, with an incidence Size of Omphalocele and Indications for Treatment copyright.