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

OMPHALOCELE AND GASTROSCHISISOMPHALOCELE AND GASTROSCHISIS

MAN MOHAN HARJAIMAN MOHAN HARJAI, M ChM Ch

Associate Professor

Army Hospital (Research and Referral)

Delhi Cantt 110 010

INDIA

Description of lesionDescription of lesion Preoperative stabilizationPreoperative stabilization Preanesthetic evaluationPreanesthetic evaluation Anesthetic managementAnesthetic management Postoperative Postoperative considerationsconsiderations

OVERVIEWOVERVIEW

GUT DEVELOPMENTGUT DEVELOPMENT

Primitive gut - Divided into 3 regionsPrimitive gut - Divided into 3 regions

Foregut- Pharynx, esophagus and stomachForegut- Pharynx, esophagus and stomach

Midgut- Small and large intestineMidgut- Small and large intestine

Hindgut- Colon and rectum Hindgut- Colon and rectum

Abdominal wall- somatic and splanchnic layers of the cephalicAbdominal 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 defectsanterior abdominal wall defects

GUT DEVELOPMENTGUT DEVELOPMENT

•Week fiveWeek five•Week tenWeek ten•Week elevenWeek eleven

OMPHALOCELEOMPHALOCELE

Greek- Greek- omphalosomphalos-navel, -navel, celecele- hernia - hernia

Absence abdominal wall fasciaAbsence abdominal wall fascia

Herniation abdominal contentsHerniation abdominal contents

Eccentric displacement umbilical cordEccentric displacement umbilical cord

Small underdeveloped abdominal cavitySmall underdeveloped abdominal cavity

Thin sac covering defectThin sac covering defect

OMPHALOCELEOMPHALOCELE

Incidence: 1 in 3 - 5,000Incidence: 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 defectsGastrointestinal, 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)

ASSOCIATED MALFORMATIONSASSOCIATED MALFORMATIONS

UPPER MIDLINE SYNDROMEUPPER 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, OmphaloceleDiaphragmatic defect, Omphalocele

LOWER MIDLINE SYNDROMELOWER MIDLINE SYNDROME

Vesicointestinal fistula, Imperforate anus, Colonic agenesis, Bladder extrophy,Vesicointestinal fistula, Imperforate anus, Colonic agenesis, Bladder extrophy,

OmphaloceleOmphalocele

BECKWITH-WIEDEMANN SYNDROMEBECKWITH-WIEDEMANN SYNDROME

Macroglossia, Visceromegaly, OmphaloceleMacroglossia, Visceromegaly, Omphalocele

OMPHALOCELEOMPHALOCELE

30- 50% develop hypoglycemia30- 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%)

GASTROSCHISISGASTROSCHISIS

Greek: Greek: GasterGaster-stomach, -stomach, schisisschisis- cleft- cleft

Incidence 1 in 50,000Incidence 1 in 50,000

Infarction /atresia bowel commonInfarction /atresia bowel common

Infrequent congenital malformationsInfrequent congenital malformations

High association prematurityHigh association prematurity

Herniated contents (rarely liver)Herniated contents (rarely liver)

Umbilical cord left defect, Absence sac over herniationUmbilical cord left defect, Absence sac over herniation

Abdominal cavity more developedAbdominal cavity more developed

GASTROSCHISIS…GASTROSCHISIS…

ISOLATED OMPHALOCELEISOLATED 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 ringumbilical ring

DEVELOPMENT SPECULATIVEDEVELOPMENT SPECULATIVE

Shaw (Early 1980’s) – Simple herniation of the cord that ruptures Shaw (Early 1980’s) – 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.

GASTROSCHISIS…GASTROSCHISIS…

GLICK (1984) GLICK (1984)

Ultrasound for chronologic in utero development of GastroschisisUltrasound for chronologic in utero development of Gastroschisis

OBSERVATIONOBSERVATION

27 - Moderate soft tissue mass adjacent to fetal anterior wall, contained in sac27 - Moderate soft tissue mass adjacent to fetal anterior wall, contained in sac

31 - Mass with loops of bowel identified, contained in sac31 - Mass with loops of bowel identified, contained in sac

3535 -- Free floating bowel in amniotic fluid Free floating bowel in amniotic fluid

CESAREAN SECTION CESAREAN SECTION

4 cm wall defect to the right of the umbilical cord, no sac remnant visible4 cm wall defect to the right of the umbilical cord, no sac remnant visible

PREOPERATIVE STABILIZATIONPREOPERATIVE STABILIZATION

•AIRWAY SUPPORTAIRWAY SUPPORT

• Often intubated in delivery room Often intubated in delivery room

•GASTRIC DECOMPRESSIONGASTRIC DECOMPRESSION

• Prevent aspirationPrevent aspiration

• Air progressing past pylorus where irretrievable and cause increasedAir 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 lossInfant 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

INITIAL RESUSCITATIONINITIAL RESUSCITATION

Consider hypoglycemia until proven otherwiseConsider 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% albuminD20 / 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

PREOPERATIVE EVALUATIONPREOPERATIVE 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 stabilizationTemperature stabilization

Evaluation intravascular statusEvaluation intravascular status

MANAGEMENTMANAGEMENT

ANESTHETIC MANAGEMENTANESTHETIC MANAGEMENT AirwayAirway MaintenanceMaintenance MonitorsMonitors

SURGICAL PROCEDURESURGICAL PROCEDURE Reduction herniated visceraReduction herniated viscera Closure of defectClosure of defect Cardio/respiratory functionCardio/respiratory function

SURGICAL PROCEDURESURGICAL PROCEDURE

PRIMARY CLOSURE PRIMARY CLOSURE Reduced complicationsReduced complications

Sepsis,sac dehiscence,prolonged ileusSepsis,sac dehiscence,prolonged ileus

Increased complicationIncreased complication Hypotension,bowel ischemia, anuria, respiratory failureHypotension,bowel ischemia, anuria, respiratory failure

STAGED CLOSURESTAGED CLOSUREAvoid abdominal viscera compressionAvoid abdominal viscera compressionAllow early extubationAllow early extubation

POSTOPERATIVE MANAGEMENTPOSTOPERATIVE MANAGEMENT