If abnormality present C/S usually perfomed but doesn't necessarily improve outcome

Umbilical Herniation
    physiologic rotation starting at 8th wk & back by 12wks
    Midgut grows faster than abd wall
        Rotates clockwise 90deg on the way out, sm bowel returns 1st
        Colon rotates additional 180deg coming back, for a total of 270deg

Gastroschisis
    Right paraumbilical defect involving all layers of wall
    Mostly in young populations (teens to early 20's nearly exclusively, if seen in older population think of another diagnosis ie ruptured omphalocele)
    Small bowel herniates and is by definition nonrotated without fixation
        kinked vessels may cause ischemia with assoc jejunal atresia
        no mem covers bowel which is matted & fibrous resulting in high alpha-fetoprotien
        rarely includes bladder, uterus, & liver
        No ascites because it cannot accumulate
        Usually isolated finding, poss due to vascular insult to wall. Amniocentesis not indicated
        7% w VACTERL defects
        Early identification allows near 100% survival with postnatal Sx repair
    U/S -  free floating loops of bowel with cord inserted to the left

Omphalocele
    midline herniation of abdominal contents (liver, stomach, bowel)  into umbilical cord base
    has  parietal peritoneum cover and Wharton's jelly over it
    commonly has ascites which shows the peritoneal cover
    33% have polyhdramnios
    70% association with chromosomal (trisomy 18, 13) or organ malformations (cardiac, CNS, GU, GI)
    elevated alpha fetoprotein, and amniocentesis is performed  
    50% still born, but good prognosis if isolated
    U/S - smooth mem covered mass with cord inserting into it and umbilical vien can be seen passing through it
    Pentalogy of Cantrell: omphalocele and anterior thorax wall defect with ectopia cordis (ectopic heart) due to defect in lateral mesoderm folds of day 28-32
          has pericardial, anterior diaphragm and inferior sternal defects
          associated internal cardiac defects, vertebral defects.
          associated with trisomies
          if detected in utero is uniformly fatal. Small defects postnatally can be repaired
    Beckwith Wiedemen Syndrome: Omphalocele-Macroglossia-Gigantism (OMG)
            A group of disorders with these 3 findings in common
            75% have O (4% of all incidence), 97% have M, 32% G
            also get hemihypertrophy, hepatosplenomegaly & cardiac anomalies
            due to placental endocrine dysfunction where insulinlike growth factors are secreted causing visceromegaly.
             Has associated risk of Wilm's tumor and renal cysts
             asphyxia is a common birth complication
             outcome if survives birth is of normal intelligence
        U/S  Omphalocele, macroglossia seen in coronal with tongue abnormally projecting beyond gums

Cloacal Exstrophy
        failure of fusion of urogenital sinus and rectum with absence of urorectal septum and infraumbilical anterior abdominal wall and genital tubercles cannot develop resulting in:
            bladder extrophy with the bladder open in half with imperforate anus and rectum communicating with the bladder
           the clitoris/penis may be split in half, and 2 vaginas may be present
           always has omphalocele and frequently 2 vessel cord +/- meningocele
           clubfoot, multicystic kidneys, hypoplastic chest, diaphragmatic hernia
        U/S genitals & bladder split in midline & divided with bladder emptying into amnion.
            Do not see a bladder filling to discriminate
             DDx - amniotic band, sacrococcygeal teratoma, body stalk anomaly        
   
Short Umbilical Cord Syndrome (=limb-body wall complex=body stalk anomaly)
 abnormalities involving failure of closure of ventral wall maybe related to vascular insult at 4-6weeks
        adhesion to amnion & persistence of extraembryonic coelom
        short or absent umbilical cord, spine, limbs, cranium, etc
        gross evisceration of chest & abdominal contents
        karyotype is normal however 100% die
    U/S mass of organs & membranes with very little fluid  


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