TY  - CHAP
M1  - Book, Section
TI  - Chapter 43. Surgical Disorders
A1  - Lowry, Adam W.
A1  - Bhakta, Kushal Y.
A1  - Nag, Pratip K.
PY  - 2011
T2  - Texas Children's Hospital Handbook of Pediatrics and Neonatology
AB  - Table  Graphic  Jump  Location|Download  (.pdf)|PrintAbdominal  Wall  DefectsGastroschisisOmphalocelePathophysiologyRight-sided  defect  lateral  to  the  base  of  the  umbilical  stalk  through  which  a  portion  of  the  GI  tract  has  herniatedMay  be  secondary  to  vascular  accident  with  disruption  of  body  wall  formation  or  weakness  at  the  insertion  of  the  umbilical  cord  after  involution  of  the  right  umbilical  veinExposure  to  amniotic  fluid  and  partial  closure  of  the  defect  may  result  in  mesenteric  vascular  constriction  →  further  bowel  injuryBowel  may  be  edematous  and  matted  with  inflammatory  “peel”Small  bowel  with  villous  atrophy  and  blunting,  muscular  hypertrophy,  and  serosal  inflammationDevelopmental  arrest  of  somites  in  the  abdominal  wall  or  failure  of  midgut  to  return  to  the  abdominal  cavity  after  herniation  into  the  umbilical  stalkCentral  defect;  umbilical  ring  fails  to  contract  and  leaves  a  round  defectDefect  is  covered  by  a  translucent  mesenchymal  membrane  that  protects  the  underlying  abdominal  organs  from  exposure  to  amniotic  fluidThe  sac  usually  remains  intact  but  occasionally  is  ruptured  during  deliveryThe  liver  and  small  intestine  usually  occupy  part  of  the  sac  along  with  variable  other  abdominal  organsEpidemiologySporadic;  1.5  of  10,000  live  birthsRisk  factors:  Young  maternal  age;  low  SES;  exposure  to  vasoconstricting  decongestants,  NSAIDs,  cocaine,  cigarette  smoking,  pesticidesCan  be  associated  with  malrotation;  associated  with  intestinal  atresias  in  5%–25%Not  commonly  associated  with  anomalies  unrelated  to  the  GI  tractSporadic;  2–2.5  of  10,000  live  birthsSeen  with  mothers  at  extremes  of  reproductive  ageAssociated  with  maternal  obesity  and  SSRIsAssociated  with  other  structural  or  genetic  defects  in  50%–75%Associated  syndromes:  VACTERL  association;  Beckwith-Wiedemann  syndrome;  pentalogy  of  Cantrell;  CHD;  pulmonary  hypoplasia;  congenital  malrotation  of  the  colon;  EEC  syndrome;  OEIS  complex;  trisomies  13,  18,  and  21Presentation  and  DiagnosisUsually  diagnosed  antenatally  on  USMother  has  elevated  AFP  levelsMost  may  still  be  delivered  vaginally  without  further  injury  to  the  bowelMost  diagnosed  prenatally;  should  have  a  thorough  evaluation  for  any  other  anomalies  and  consider  chromosomal  analysisMother  may  or  may  not  have  elevated  AFP  levelsMay  be  delivered  vaginally,  but  consider  cesarean  section  for  giant  omphalocele  because  of  concern  for  ruptureTreatmentIn  delivery  room,  stabilize  airway;  wrap  intestine  with  warm,  sterile  saline  dressings;  place-large  bore  orogastric  tube  to  continuous  suction;  and  place  peripheral  IV  for  fluid  management  and  broad-spectrum  antibioticsPlace  the  infant  in  clear  plastic  bowel  bag  to  minimize  heat  and  water  lossInspect  bowel  for  evidence  of  ischemia,  torsion,  and  atresiaPlace  the  infant  in  lateral  decubitus  position  to  avoid  mesenteric  kinkingReduce  or  place  the  bowel  in  a  sterile  silo  as  soon  as  possible;  delay  may  lead  to  bowel  swelling  and  resultant  ischemiaIf  bowel  is  forced  into  abdominal  cavity,  may  cause  decreased  diaphragmatic  excursion,  requiring  increased  ventilator  support;  may  also  impair  mesenteric,  hepatic,  and  renal  perfusionDuring  staged  closure,  parenteral  nutrition,  gastric  decompression,  and  spontaneous  breathing  are  preferredWhen  reduction  of  silo  contents  is  complete,  final  closure  is  performedRepair  of  atresias  includes  primary  anastomosis,  or  if  peel  exists,  internalization  of  uncorrected  atresia  and  later  reexploration  and  repair  after  peel  resolvesAwait  bowel  sounds,  passage  of  meconium,  and  cessation  of  bilious  gastric  aspirates  before  starting  enteral  feedsConservative  advancement  of  enteral  feedsMay  have  return  of  bowel  function  in  a  few  days  to  weeksThose  who  fail  to  demonstrate  bowel  patency  within  3–4  wk  of  ...
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PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=7453388
ER  -