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

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Presentation on theme: "OMPHALOCELE AND GASTROSCHISIS MAN MOHAN HARJAI M Ch MAN MOHAN HARJAI, M Ch Associate Professor Army Hospital (Research and Referral) Delhi Cantt 110 010."— Presentation transcript:

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

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

3 GUT DEVELOPMENT Primitive gut - Divided into 3 regions Primitive gut - Divided into 3 regions Foregut- Pharynx, esophagus and stomach Foregut- Pharynx, esophagus and stomach Midgut- Small and large intestine Midgut- Small and large intestine Hindgut- Colon and rectum Hindgut- Colon and rectum Abdominal wall- somatic and splanchnic layers of the cephalic Abdominal wall- somatic and splanchnic layers of the cephalic lateral and caudal folds lateral and caudal folds Failure in development of one of these folds can result in Failure in development of one of these folds can result in anterior abdominal wall defects anterior abdominal wall defects

4 GUT DEVELOPMENT Week fiveWeek five Week tenWeek ten Week elevenWeek eleven

5 OMPHALOCELE Greek- omphalos-navel, cele- hernia Greek- omphalos-navel, cele- hernia Absence abdominal wall fascia Absence abdominal wall fascia Herniation abdominal contents Herniation abdominal contents Eccentric displacement umbilical cord Eccentric displacement umbilical cord Small underdeveloped abdominal cavity Small underdeveloped abdominal cavity Thin sac covering defect Thin sac covering defect

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

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

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

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

10 GASTROSCHISIS… ISOLATED OMPHALOCELE Failure of lateral folds to engulf the midgut and form the future Failure of lateral folds to engulf the midgut and form the future umbilical ring umbilical ring DEVELOPMENT SPECULATIVE Shaw (Early 1980s) – Simple herniation of the cord that ruptures Shaw (Early 1980s) – Simple herniation of the cord that ruptures after completion of the anterior abdominal wall but, before after completion of the anterior abdominal wall but, before completion of the umbilical ring. completion of the umbilical ring.

11 GASTROSCHISIS… GLICK (1984) Ultrasound for chronologic in utero development of Gastroschisis Ultrasound for chronologic in utero development of GastroschisisOBSERVATION 27 - Moderate soft tissue mass adjacent to fetal anterior wall, contained in sac 27 - Moderate soft tissue mass adjacent to fetal anterior wall, contained in sac 31 - Mass with loops of bowel identified, contained in sac 31 - Mass with loops of bowel identified, contained in sac 35 - Free floating bowel in amniotic fluid 35 - Free floating bowel in amniotic fluid CESAREAN SECTION 4 cm wall defect to the right of the umbilical cord, no sac remnant visible 4 cm wall defect to the right of the umbilical cord, no sac remnant visible

12 PREOPERATIVE STABILIZATION AIRWAY SUPPORTAIRWAY SUPPORT Often intubated in delivery room Often intubated in delivery room GASTRIC DECOMPRESSIONGASTRIC DECOMPRESSION Prevent aspiration Prevent aspiration Air progressing past pylorus where irretrievable and cause increased Air progressing past pylorus where irretrievable and cause increased difficulty in repair difficulty in repair TEMPERATURE REGULATIONTEMPERATURE REGULATION Infant covered with plastic wrap to minimize heat loss Infant covered with plastic wrap to minimize heat loss BOWEL CAREBOWEL CARE Bowel covered by moist saline dressing, protect from dehydration Bowel covered by moist saline dressing, protect from dehydration Care to be taken not to twist bowel – impair vascular integrity Care to be taken not to twist bowel – impair vascular integrity

13 INITIAL RESUSCITATION Consider hypoglycemia until proven otherwise Consider hypoglycemia until proven otherwise Dextrose solution at 5-7 mg / kg / min Dextrose solution at 5-7 mg / kg / min D20 / D10 / Ringers lactate / 5% albumin D20 / D10 / Ringers lactate / 5% albumin Brain & Heart depend on glucose as major energy substrate Brain & Heart depend on glucose as major energy substrate Limited hepatic glycogen storage < 2.5 kg Limited hepatic glycogen storage < 2.5 kg

14 PREOPERATIVE EVALUATION Inspect the protruding viscera, R/O torsion or angulation of bowel Inspect the protruding viscera, R/O torsion or angulation of bowel Correct dehydration / hypovolemia / hypoglycemia Correct dehydration / hypovolemia / hypoglycemia Evaluation respiratory system (Chest X-ray) Evaluation respiratory system (Chest X-ray) Cardiac evaluation (EKG, ECHO, especially in Omphalocele) Cardiac evaluation (EKG, ECHO, especially in Omphalocele) Temperature stabilization Temperature stabilization Evaluation intravascular status Evaluation intravascular status

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

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


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