Omphalocele & Gastroschisis

Michelle D. Nguyen MD

Anatomy


Congenital defect in anterior abdominal wall closure Abdominal contents externally herniated

Miller s Anesthesia, 7th edition

Embryology
  

5th 10th week of fetal life: abdominal contents extruded into the extraembryonic coelom By 10th week, the gut returns to the abdominal cavity Amnion: membrane covering abdominal contents
Protection from infection Prevents loss of extracellular fluid

Omphalocele: abdominal contents fail to return to abdominal cavity

Gastroschisis vs. Omphalocele

Gastroschisis Pathophysiology Omphalomesenteric artery occlusion leading to ischemia/atrophy of layers of abdominal wall at base of umbilical cord (develops later than omphalocele) 1/15,000 births 10-15% 10Omphalocele Failiure of gut migration from the yolk sac into abdomen (During 5th to 10th week of fetal development)

Incidence Associated anomalies

1/6000 births 40-60% 40E.g. trisomy 21, diaphragmatic hernia, cardiac (VSD) & bladder malformations Have a hernia sac Within the umbilical cord, at the base Congenital heart disease-20%, diseasebladder exstrophy, BeckwithBeckwithWiedemann syndrome (macroglossia, gigantism, hypoglycemia, hyperviscosity), OEIS (omphalocele, exstrophy, imperforate anus, spinal defects syndrome)

Associated findings Location of defect Problems associated with defect

No hernia sac Lateral to the umbilicus, usually to the right Edema, inflammation of exposed abdominal contents, chemical peritonitis, premature delivery due to irritation of abdominal contents with uterine lining

Diagnosis


High maternal serum alpha-fetoprotein (AFP) alphaNormal protein in fetal tissues When abdominal wall and neural tube closes, large amts of AFP will be prevented from being released Gastroschisis levels usually higher than omphalocele

 

Antenatal ultrasound
Can also dx cardiac abnormalities

Elective c/s at 38 weeks

Prevent trauma to exposed bowel Surgical teams prepared in advance

Surgical repair

Associated Problems
Severe dehydration and fluid losses from exposure (especially with gastroschisis) and third space losses from bowel obstruction  Heat losses  Difficulties in surgical closure  Incidences of associated abnormalities


Anesthetic Considerations: Pre-Op Pre  

Optimize fluid and electrolyte balance Adequate IV access Correct hypoglycemia slowly with glucose infusion Staged closure
Primary closure can cause abdominal compartment syndrome Silastic silo gradual closure over period of 7-10 days, 7with reduction of silo occurring one to two times daily

Anesthetic Considerations
      

NGT placement: decompress stomach Intubation: awake/asleep +/- muscle relaxant +/Maintenance anesthetic with volatile and narcotics Routine monitors, arterial line, +/- CVP +/Avoid nitrous oxide: do not want bowel distention Need muscle relaxant when placing bowel inside abdominal cavity Watch for hypotension: can be due to tension on organs such as liver or IVC compression

Anesthetic Considerations
Replacement of third space fluid losses  Keep intubated, with ventilator weaning over period of 1-2 days 1

Give time for abdominal wall to stretch to accommodate visceral contents

Anesthetic Considerations: Post-Op Post

Increased abdominal pressure (>20-25 cm H20) (>20if tight closure

Compromise hepatic function Alter drug metabolism Abdominal compartment syndrome
 Increased abdominal pressure leading to organ dysfunction: decreased cardiac output, oliguria, restriction in pulmonary ventilation  Bowel is most sensitive organ  Must reopen and decompress

Hypertension: due to decreased circulation to kidneys

Causes renin release

References
Barash, P ed. Clinical Anesthesia , 6th edition.  Faust, R ed. Anesthesiology Review, 3rd Review, edition.  Miller, ed. Miller s Anesthesia, 7th edition. Anesthesia,  Morgan, ed. Clinical Anesthesiology. Anesthesiology.


Any Questions?