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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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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

slide2

OVERVIEW

  • Description of lesion
  • Preoperative stabilization
  • Preanesthetic evaluation
  • Anesthetic management
  • Postoperative considerations
gut development
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

slide4

GUT DEVELOPMENT

  • Week five
  • Week ten
  • Week eleven
slide5

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
slide6

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)
slide7

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
slide8

OMPHALOCELE

  • 30- 50% develop hypoglycemia
  • May last for first year of life
  • Associated mortality
        • Small defect (30%)
        • Giant defect (48%)
slide9

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
slide10

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.

slide11

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
slide12

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
slide13

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
slide14

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
slide15

MANAGEMENT

ANESTHETIC MANAGEMENT

  • Airway
  • Maintenance
  • Monitors

SURGICAL PROCEDURE

  • Reduction herniated viscera
  • Closure of defect
  • Cardio/respiratory function
slide16

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