1. OMPHALOCELE AND GASTROSCHISIS
MAN MOHAN HARJAI
MAN MOHAN HARJAI, M Ch
Associate Professor
Army Hospital (Research and Referral)
Delhi Cantt
INDIA

2. OVERVIEW
Description of lesion
Preoperative stabilization
Preanesthetic evaluation
Anesthetic management
Postoperative considerations

3. GUT DEVELOPMENT Primitive gut - Divided into 3 regions
Foregut- Pharynx, esophagus and stomach
Midgut- Small and large intestine
Hindgut- Colon and rectum
Abdominal wall- somatic and splanchnic layers of the cephalic
lateral and caudal folds
Failure in development of one of these folds can result in
anterior abdominal wall defects
primitive gut divided into three regions
foregut: giving rise to pharynx,esophagus,stomach
midgut:giving rise to small and large intestine
hindgut:giving rise to colon and rectum
abdominal wall is formed by the somatic and splanchnic layers
of the cephalic,lateral, and caudal folds
failure in the development of one of these folds will result in formation of an anterior wall defect

4. GUT DEVELOPMENT
Week five
Week ten
Week eleven

5. OMPHALOCELE Greek- omphalos-navel, cele- hernia
Absence abdominal wall fascia
Herniation abdominal contents
Eccentric displacement umbilical cord
Small underdeveloped abdominal cavity
Thin sac covering defect
greek omphalos=navel,cele=hernia
absence of abdominal wall fascia tela subcutanea
thin sac part amnion/part peritoneum

6. OMPHALOCELE divided into two groups
Incidence: 1 in 3 - 5,000
Divided into 2 groups
Small hernia umbilical cord (<4 cm)
Giant Omphalocele (>4 cm with herniated liver)
Associated congenital abnormalities (30-70%)
Gastrointestinal, Genitourinary, central nervous system, congenital heart defects
Cardiac defects- seen in 25% of patients (TEF most common)
divided into two groups
small hernia of umbilical cord<4cm
giant omphalocele >4cm with liver herniated

7. ASSOCIATED MALFORMATIONS
UPPER MIDLINE SYNDROME
Pentalogy of Cantrell, Sternal defect, Ectopia cordis, Pericardial and cardiac defects,
Diaphragmatic defect, Omphalocele
LOWER MIDLINE SYNDROME
Vesicointestinal fistula, Imperforate anus, Colonic agenesis, Bladder extrophy,
Omphalocele
BECKWITH-WIEDEMANN SYNDROME
Macroglossia, Visceromegaly, Omphalocele

8. OMPHALOCELE 30- 50% develop hypoglycemia
May last for first year of life
Associated mortality
Small defect (30%)
Giant defect (48%)

9. GASTROSCHISIS Greek: Gaster-stomach, schisis- cleft
Incidence 1 in 50,000
Infarction /atresia bowel common
Infrequent congenital malformations
High association prematurity
Herniated contents (rarely liver)
Umbilical cord left defect, Absence sac over herniation
Abdominal cavity more developed
greek gaster=stomach,SCHISIS=cleft

10. GASTROSCHISIS… ISOLATED OMPHALOCELE DEVELOPMENT SPECULATIVE
Failure of lateral folds to engulf the midgut and form the future
umbilical ring
DEVELOPMENT SPECULATIVE
Shaw (Early 1980’s) – Simple herniation of the cord that ruptures
after completion of the anterior abdominal wall but, before
completion of the umbilical ring.
isolated omphalocele represents failure of lateral folds to engulf the midgut and form the future umbilical ring
Shaw suggested in early 80's that gastroschisis is a simple herniation of the cord that ruptures after completion of the anterior abdominal wall but before completion of the umbilical ring

11. GASTROSCHISIS… GLICK (1984) OBSERVATION CESAREAN SECTION
Ultrasound for chronologic in utero development of Gastroschisis
OBSERVATION
27 - Moderate soft tissue mass adjacent to fetal anterior wall, contained in sac
31 - Mass with loops of bowel identified, contained in sac
35 - Free floating bowel in amniotic fluid
CESAREAN SECTION
4 cm wall defect to the right of the umbilical cord, no sac remnant visible
Glick in 1984 used ultrasound to observe the chronologic in utero development of gastroschisis
27 weeks moderate size soft tissue mass adjacent to fetal anterior wall contained in sac
31 weeks mass with loops of bowel identified contained in sac
35 weeks free floating bowel in amniotic fluid
at csx 4cm wall defect to right of the umbilical cord no sac remnant visible

12. PREOPERATIVE STABILIZATION
AIRWAY SUPPORT
Often intubated in delivery room
GASTRIC DECOMPRESSION
Prevent aspiration
Air progressing past pylorus where irretrievable and cause increased
difficulty in repair
TEMPERATURE REGULATION
Infant covered with plastic wrap to minimize heat loss
BOWEL CARE
Bowel covered by moist saline dressing, protect from dehydration
Care to be taken not to twist bowel – impair vascular integrity
often intubated in delivery room
premature remember risk ROP <36 week
Ph ,PaC ,PaO ,Sa %
gastric decompression to prevent aspiration
prevent air progressing past pylorus where irretrievable and cause increased difficulty in repair
bowel covered by moist saline dressing
protect from dehydration
care not to twist bowel impair vascular integrity
infant covered with plastic wrap to minimize heat loss

13. INITIAL RESUSCITATION
Consider hypoglycemia until proven otherwise
Dextrose solution at 5-7 mg / kg / min
D20 / D10 / Ringers lactate / 5% albumin
Brain & Heart depend on glucose as major energy substrate
Limited hepatic glycogen storage < 2.5 kg
consider hypoglycemia until proven otherwise
dextrose solution at 5-7 mg/kg/min
brain and heart depend on glucose as major energy substrate
limited hepatic glycogen storage <2.5 kg
third space loss open bowel

14. PREOPERATIVE EVALUATION
Inspect the protruding viscera, R/O torsion or angulation of bowel
Correct dehydration / hypovolemia / hypoglycemia
Evaluation respiratory system (Chest X-ray)
Cardiac evaluation (EKG, ECHO, especially in Omphalocele)
Temperature stabilization
Evaluation intravascular status
r/o torsion or angulation of bowel
correct dehydration/hypovolemia/hypoglycemia
ekg,echo especially in omphalocele
chest x-ray
blood gases pH< 7.2 with B.E. > -6 correct
electrolytes especially calcium
bowel exposure to acidic amniotic fluid
marked reduction in total serum protein,albumin

15. MANAGEMENT ANESTHETIC MANAGEMENT SURGICAL PROCEDURE Airway Maintenance
Monitors
SURGICAL PROCEDURE
Reduction herniated viscera
Closure of defect
Cardio/respiratory function

16. SURGICAL PROCEDURE PRIMARY CLOSURE STAGED CLOSURE
Reduced complications
Sepsis,sac dehiscence,prolonged ileus
Increased complication
Hypotension,bowel ischemia, anuria, respiratory failure
STAGED CLOSURE
Avoid abdominal viscera compression
Allow early extubation
POSTOPERATIVE MANAGEMENT