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Thread: Omphalocele Pictures - Pediatric cases

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    Default Omphalocele Pictures - Pediatric cases

    Embryology
    Initially, the embryo is a flat disk surrounded by the umbilical ring. Gastrulation proceeds in a cephalocaudal direction and converts the original two-layered disk into three germ layers. The dorsal layer is the ectoderm, which becomes either the central nervous system (CNS) or the skin and sensory organs. The middle layer is the mesoderm, which forms the skeleton, connective tissue, and the cardiovascular and urogenital systems. The ventral layer is the endoderm, which develops into the intestines, liver, gallbladder, and pancreas.

    Proliferation of the neuroectoderm and the underlying mesoderm pushes the embryonic disk above the umbilical ring like a sprouting mushroom. The amnion bulges over the embryo and fuses with the yolk sac and body stalk. As the embryo elongates, longitudinal enfolding of its lateral walls creates the appearance of a ridged cylinder. Ventral enfolding separates the thoracic and abdominal cavities from the extra-embryonic space. With caudal enfolding, the embryo begins to resemble a fetus.

    The yolk sac is incorporated into the hindgut and the allantois is incorporated into the urogenital sinus creating the cloaca. The cloacal membrane separates the coelom from the amniotic cavity.

    The mesoderm invades the cloacal membrane and unites the genital tubercles to form the ventral wall of the urogenital sinus. As the hindgut elongates, condensation of mesoderm anteriorly forms the urorectal septum. The body folds (cephalic, caudal, and lateral) unite where the amnion invests the yolk sac and body stalk.

    Fusion is a complex process that also occurs in formation of the neural tube, palate, and lip. In these areas, a surface glycoprotein promotes adhesion; then, planned cellular death (apoptosis) and migration create continuity between the opposing surfaces.

    In an analogous fashion, the amnion and the lateral folds of the body wall coalesce about the attenuated yolk sac, constricting the umbilical ring. This process requires a "component separation and reorganization"” for the opposing sides to become a continuous layer. Development of the gut, suspended on its mesentery, occurs coincidentally with these events.
    By the sixth week of intrauterine life, rapid growth of the liver and intestines causes herniation of the midgut through the umbilical ring.
    By the tenth week, the abdominal cavity has enlarged sufficiently to accommodate the return of the midgut.
    Rotation and fixation of the duodenum and the proximal colon occur as the intestine returns to the abdominal cavity.

    History
    Infants with gastroschisis and omphalocele can be identified using prenatal ultrasonography. Defects in other organ systems may also be diagnosed, and chromosomal abnormalities may be discovered by amniocentesis. See Workup.

    Causes
    Folic acid deficiency, hypoxia, and salicylates cause rats to develop abdominal wall defects, but the clinical significance of these experiments is conjectural.

    Elevation of maternal serum alpha-fetoprotein (MSAFP) is associated with omphalocele and gastroschisis. An elevated MSAFP warrants ultrasonography to determine if structural abnormalities are present in the fetus. If the study is suspicious for an omphalocele, amniocentesis is indicated to determine any associated genetic abnormality.
    Omphalocele Pictures Pediatric cases attachment.php?s=d74946946107e2281ea7aa5f445e1bd2&attachmentid=1758&d=1441141822

    Polyhydramnios occurs in association with intestinal atresia, which may complicate gastroschisis. If polyhydramnios is identified by fetal ultrasonography, the mother should be referred to a tertiary care facility for optimal care of her newborn.

    Diagnostic Considerations
    Note that ventral body-wall defects are immediately obvious. However, although the diagnosis is simple, treatment of these defects is complex.

    Omphalomesenteric duct remnants, occasionally associated with intestinal atresia, occur in babies with umbilical cord hernias, in which the abdominal wall defect is less than 4 cm.

    The association of chromosomal abnormalities with omphalocele is 10-50%. This includes trisomy , and 21 and Beckwith-Wiedemann syndrome.

    Genetic abnormalities rarely occur in babies with gastroschisis, but the clinical course of these infants is frequently complicated by malabsorption and intestinal dysmotility, gastroesophageal reflux disease, and occasionally Hirschsprung disease.

    Special concerns

    With the increased availability of ultrasonography, prenatal diagnosis is made more frequently.

    When an omphalocele is diagnosed, further workup is indicated to determine whether an associated genetic abnormality is present.

    When gastroschisis is diagnosed, serial ultrasonography should be performed to detect signs of intestinal injury, including decreased peristalsis or bowel distension.

    Parents should be educated about the baby's anomaly before delivery. Optimal management requires that the obstetrician anticipates the needs of the baby and ensures that delivery occurs in a facility where neonatology, pediatric anesthesia, and pediatric surgery consultation is available.

    Laboratory Studies
    Elevation of maternal serum alpha-fetoprotein (MSAFP) is associated with abdominal wall defects.

    MSAFP levels are greater in gastroschisis than in omphalocele.

    MSAFP levels are also increased in spina bifida, which additionally increases the ratio of acetylcholinesterase and pseudocholinesterase.

    Imaging Studies
    In utero ultrasonography may demonstrate a structural defect that is associated with a karyotypic abnormality.

    The suspicion that the baby has a genetic abnormality should be confirmed with amniocentesis.

    Fetal echocardiography may identify a cardiac abnormality.

    If serial ultrasonography shows dilatation and thickening of the intestine in a baby with gastroschisis, delivery should occur as soon as amniocentesis demonstrates lung maturity.

    Appropriate tests to determine other congenital anomalies should precede surgical intervention in infants with omphaloceles.

    Intestinal inflammation
    Intestinal inflammation may occur with either gastroschisis or rupture of an omphalocele.

    The eviscerated intestine may appear entirely normal, as if a surgeon had made a fresh laparotomy incision. Alternatively, the intestines may be grossly abnormal, matted together, congealed, and covered with a thick inflammatory membrane or “peel”. Function follows appearance; in the first instance, babies may tolerate feedings immediately. In the second, they may require prolonged parenteral nutrition because of intestinal dysmotility and malabsorption. The extent of intestinal dysfunction depends on the magnitude of the inflammatory and ischemic injury caused by exposure to the amniotic fluid and compression of the herniated intestinal mesentery by the abdominal wall defect.

    Histology reveals atrophy of the myenteric ganglion cells.

    References:
    http://emedicine.medscape.com/article/975583-treatment











    Last edited by Medical Photos; 09-01-2015 at 09:10 PM.

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