OMPHALOCELE
 
PEDBASE.org - The Pediatric Database - Detailed information of OMPHALOCELE

 

OMPHALOCELE

 

DEFINITION:

A congenital defect of the anterior abdominal wall characterized by the protrusion of the intestine covered by peritoneum and the amniotic membrane.

EPIDEMIOLOGY:

  • incidence: 1/4000 - 1/6000

PATHOGENESIS:

1. Background

  • an abnormal development of the gut prior to the 3rd week of embryonic life which somehow prevents the later return of the midgut into the abdominal cavity

2. Types

  • Epigastric - defect in the cephalic fold
  • Classic - defect in the lateral fold
  • Hypogastric - defect in the caudal fold

    • CLINICAL FEATURES:

    1. Classic Omphalocele

    1. Umbilical Cord

    • inserted into the apex of the sac

    2. Small Intestine

    • contained within the sac plus liver and spleen

    3. Bowel Loops

    • normal

    4. Abdominal Cavity

    • less developed than in a gastroschisis

    5. Covering Sac

    • avascular sac composed of fused layers of amnion and peritoneum

    6. Associated Anomalies

    1. Congenital Heart Defects

    • 33% - ToF
    • 19% - ASD (secundum)
    • rare - VSD, PS, coarctation, PDA, A-V canal

    2. Genetic Syndromes

    • Trisomies D, E, 21
    • Beckwith-Wiedemann Syndrome

    2. Other Types of Omphaloceles

    1. Epigastric Omphalocele (foregut)

    1. Cantrell's Pentology

    • lower thoracic wall malformations - cleft sternum
    • cardiac defects - pericardial
      • structural (ectopia cordis)
    • anterior midline diaphragmatic defects
    • epigastric omphalocele

    2. Hypogastric Omphalocele (hindgut)

    • colonic agenesis
    • exstrophy of the bladder
    • imperforate anus
    • vesicointestinal fistula

    3. Complications

    • IUGR (20%)
    • Premature (10-50%)
    • Mortality (47-60%)
    • SGA (35%)
    • Associated Anomalies (30-70%)
    • Cardiac Anomalies (20-41%)

    INVESTIGATIONS:

    1. Serum

    • routine blood work

    2. Imaging Studies

    • Abdominal X-Ray,
    • Abdominal Ultrasound
    • 2D-Echo
    • (work-up for other anomalies)

    MANAGEMENT:

    1. Supportive

    1. ABC's

    • intubate
    • ventilate and oxygenate
    • fluid resuscitation

    2. Temperature Control

    • wrap infant in Saran wrap or a plastic drape
    • warm saline bathing

    3. Infection Control

    • antibiotics (Ampicillin and Gentamicin)

    2. Gastroschisis or Omphalocele

    • carefully wrap in saline-soaked pads
    • support without tension
    • NG tube for intestinal decompression
    • abdominal ultrasound for nature of herniated viscera

    3. Search for other Congenital Anomalies

    • Cantrell's Pentology
    • congenital heart defects
    • Trisomies
    • Beckwith-Wiedemann Syndrome

    4. Surgical Management

    1. Primary Closure

    • Hey (1803)
    • indicated for infants a with small to moderate isolated intestinal evistration
    • primary closure without removal of imflammatory peel
    • limits: increase in intraabdominal pressures comprimising lungs, circulation, +/- gastrointestinal contents

    2. Staged Reduction

    • Schuster (1967)
    • indicated for large defects and in infants who can't tolerate a primary closure
    • prosthetic silo of mesh-reinforced silastic material with progressive daily reductions (1 week)
    • limits: mesenteric vascular comprimise and infections after 7-10 days along suture lines

    3. Skin-Flap Closure

    • Gross (1948)
    • indicated in infants with multiple complex congenital anomalies
    • skin flaps mobilized laterally and inferiorly but not superiorly to allow for throacic growth repair of ventral hernia at a later date
    • limits: no stimulus within the abdomen to increase size of abdominal cavity

    4. Non-Surgical

    • Grob (1957)
    • indicated in those with uncorrectable congenital anomalies
    • mecurochrome, alcohol (65%), silver nitrate
    • limits: 8-10 weeks for epithelialization, mercury poisoning,
    • 33% mortality

     

     

    Pediatric Database - OMPHALOCELE

     

    Pediatric Organization - Pedbase [at] Gmail.com

     
    1994 -2007 Pedbase.org 

    Powered by Database of Pediatrics- OMPHALOCELE