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 Postoperative considerations

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

GUT DEVELOPMENT Week five Week ten Week eleven

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

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

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

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

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

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

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

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 7.35-.40,PaC02 30-40,PaO2 50-70,Sa02 86-92% 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

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

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

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

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