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OMPHALOCELE AND GASTROSCHISIS

OMPHALOCELE AND GASTROSCHISIS. MAN MOHAN HARJAI MAN MOHAN HARJAI, M Ch Associate Professor Army Hospital (Research and Referral) Delhi Cantt 110 010 INDIA. OVERVIEW. Description of lesion Preoperative stabilization Preanesthetic evaluation Anesthetic management

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OMPHALOCELE AND GASTROSCHISIS

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  1. OMPHALOCELE AND GASTROSCHISIS MAN MOHAN HARJAI MAN MOHAN HARJAI, M Ch Associate Professor Army Hospital (Research and Referral) Delhi Cantt 110 010 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

  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

  6. OMPHALOCELE • 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)

  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

  10. GASTROSCHISIS… ISOLATED OMPHALOCELE • 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.

  11. GASTROSCHISIS… GLICK (1984) • 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

  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

  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

  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

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

  16. SURGICAL PROCEDURE PRIMARY 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

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