DIFFERENTIAL DIAGNOSIS Disease Omphalocele Rule In Physical Examination -Visceral protrusion Rule Out Physical Examination -No overlying sac

over bowel and solid viscera -Abdominal defect at the junction of umbilicus and normal skin -Size of defect less than 4cm Physical Examination - Abdominal defect at the junction of umbilicus and normal skin Physical Examination - Abdominal defect at the junction of umbilicus and normal skin

Cloacal Exstrophy

Physical Examination -Visceral protrusion

Bladder Exstrophy or hypogastric omphalocele

Physical Examination -Visceral protrusion

*Vin, galisud ko rule out and rule in kay wla ko kbalo sa imo PE findings and workup results. Ng.search nlng ko closest differentials. Provide nlng pud ko relevant info ma-edit nmo. Naa sa separate document ang info about sa differentials Vin.;) CASE DISCUSSION Gastroschisis A. Definition Gastroschisis is a paraumbilical defect involving all the layers of the abdominal wall, with evisceration of abdominal organs, usually the small bowel, on occasion large bowel and stomach. B. Epidemiology The combined incidence of omphalocele and gastroschisis is 1 case per 2000 births. The male-tofemale ratio is 1.5:1. The incidence of gastroschisis is increasing and is associated with young maternal age and low gravidity. Prematurity and low birth weights, secondary to in utero growth retardation, are commonly seen in babies with gastroschisis. The prognosis of babies with gastroschisis and omphalocele continues to improve; survival has increased from 60% during the 1960s to more than 90% currently. These babies are especially prone to hypothermia, dehydration, sepsis, and hypoglycemia, problems also commonly observed in LBW infants. Anesthetic management and surgical technique have also improved, as has the ability to provide parenteral nutrition for patients with GI dysfunction. Long-term morbidity from gastroschisis is related to intestinal dysfunction (including gastroesophageal reflux) and difficulties obtaining wound closure. C. Causes

Unlike the omphalocele. F. In 50% of cases. hypoxia. and covered with exudate. which is composed of amnion. Embryonic dysplasia. however. implying a more long-standing process. In babies with omphalocele. D. The umbilical vessels radiate onto the wall of the sac. and growth of skin between the resultant opening and the umbilical cord has been chronicled on prenatal ultrasonography. Rupture of a small omphalocele. Even though the thickness of the peel on the surface of the bowel indicates that a shorter gestational time would be less injurious. Wharton jelly. Pathophysiology Abdominal wall defects result from failure of the mesoderm to replace the body stalk. edematous. Associated chromosomal abnormalities are rare. there is no overlying sac and the size of the defect is much smaller (<4 cm). E. and is almost always to the right of the umbilicus. and. More commonly. The umbilicus becomes partly detached. Gastroschisis may also be sporadic abnormality. but the clinical significance of these experiments is conjectural. these are mainly gut atresias. creating a persistent communication between the intra-embryonic body cavity and the extra-embryonic coelom. and ovaries or testes accompany the extruded midgut. This defect can readily be diagnosed on prenatal ultrasound. failure of central fusion at the umbilical ring due to defective mesodermal growth causes incomplete closure of the abdominal wall and persistent herniation of the midgut. and peritoneum. Gastroschisis may result from one of the following: y y y Defective mesenchymal development at the junction of the body stalk and abdominal wall. the intestine is thick.Folic acid deficiency. and salicylates cause rats to develop abdominal wall defects. absorption of the sac. Abnormal involution of the right umbilical vein or a vascular accident involving the omphalomesenteric artery may cause localized weakness and subsequent rupture. the liver. allowing free communication with the abdominal cavity. suggesting that the rupture occurred relatively late during the pregnancy. There is no advantage to performing a cesarean section over a vaginal delivery. The intestine may be normal in appearance. there is no benefit to early versus late delivery. resulting in increased abdominal pressure that may cause the dysplastic abdominal wall to rupture. spleen. The abdominal wall defect is located at the junction of the umbilicus and normal skin. decreased apoptotic cell death. The abdominal viscera are contained in a translucent sac. discolored. Rather than investing the yolk sac and body stalk centrally at the umbilicus. associated anomalies seen with gastroschisis consist mostly of intestinal atresia. Workup . probably due to gut strangulation and infarction in utero. although other malformations are found in 10 30% of the cases. and inadequate mesodermal development result in enlargement of the diameter of the umbilical ring. Clinical Presentation Gastroschisis represents a congenital defect characterized by a defect in the anterior abdominal wall through which the intestinal contents freely protrude. the amnion remains attached to the margins of the body wall. Unlike infants born with omphalocele. The appearance of the bowel provides some information with respect to the in utero timing of the defect.

Thus. and allows real-time fetal examination. Plain radiographs and bowel contrast studies may be indicated in the postnatal postoperative period to assess bowel complications. echocardiogram and plain films of the sacrum may be useful to exclude associated abnormalities and rule out other causes of abdominal wall deformities. Ultrasonography This is the mainstay of diagnosis. although endotracheal intubation may still be needed. Magnetic resonance imaging This may be helpful as an adjunct to ultrasonography. Maternal serum alpha-fetoprotein (MSAFP) levels Because the herniated bowel is bathed by amniotic fluid. 2. b. Other Imaging modalities Renal ultrasound. This allows fetal intervention. planned delivery in a specialized unit. both maternal serum and amniotic fluid alpha-fetoprotein (AFP) levels are elevated. with detection rates of 70-72%. The exposed intestines may be covered with a moist laparotomy pad. Imaging a. particularly in identifying abnormalities in the position of the liver. these infants require vigorous fluid resuscitation on the order of 160 to 190 mL/kg per day to replace significant evaporative fluid losses. This will also help compensate for postoperative third space loses. Antenatal sonography is the key imaging examination available. b. gastroschisis is found incidentally or because of an elevated maternal AFP level. the diagnosis of a surgically treatable malformation is made before birth in an increasing number of fetuses. 4. Antibiotics Broad-spectrum antibiotics are administered to prevent contamination of the peritoneal cavity. Treatment 1. 3. 2. and antenatal counseling of the parents regarding the likely prognosis and outcome. Surgical treatment . Intubation Respiratory distress in a neonate with gastroschisis may respond to gastric decompression. 5. Differentiation of exomphalos from gastroschisis and other causes of abdominal wall mass can be made from the size and site of the defect and any associated features. Karyotyping The strong link between exomphalos and chromosomal abnormalities makes karyotyping an essential part of the investigation of this condition. Laboratory Tests a. in utero transfer. and the eviscerated intestine should be situated on top of the baby's abdomen and wrapped with Kerlix in a way that avoids applying traction upon the bowel mesentery. Radiant Heater and Moist Laparotomy pads The baby should be placed under a radiant heater.1. G. more so than in omphalocele. c. Fluid resuscitation Of equal importance to urgent surgical management. is rapid. Prenatal sonography is the primary imaging modality in pregnancy because it is noninvasive. a finding in 77-100% of cases. With the use of antenatal sonography.

rectal irrigation d. Central venous line is placed intraoperatively to provide parenteral nutrition Intestinal function does not typically return for several weeks in patients with gastroschisis.al... et.. In many instances. it may be impossible to reduce the bowel into the peritoneal cavity in the immediate postnatal period. GoreTex or Surgisis) may be required to bring the edges of the fascia together. The silo covers the bowel and allows for graduated reduction on a daily basis as the edema in the bowel wall decreases Surgical closure can usually be accomplished within approximately 1 week. respiratory dysfunction. evacuation of all meconium Care must be taken to prevent increased abdominal pressure during the reduction. As a result. Kummar. which would lead to compression of the inferior vena cava. 8th ed. and result in abdominal compartment syndrome. A prosthetic piece of material (e. Brunicardi.C.com/article/975583-overview . 2. then to return after several weeks once the edema has resolved to correct the atresia. the intestine can be returned to the abdominal cavity. a plastic spring-loaded silo can be placed onto the bowel and secured beneath the fascia. and a primary surgical closure of the abdominal wall is performed. In infants whose intestine has become thickened and edematous. it is helpful to monitor the bladder or airway pressure during reduction.medscape. 6. F. Schwartz s Principle of Surgery.al. If an atresia is noted at the time of closure.. 3. Techniques that facilitate primary closure a. it is prudent to reduce the bowel at the first operation.g. Robbin s and Cotran: Pathologic Basis of Disease. http://emedicine. mechanical stretching of the abdominal wall b. 9th Edition.All infants born with gastroschisis require urgent surgical treatment. thorough orogastric suctioning with foregut decompression c. et. Under such circumstances. To avoid this complication. This is especially true if the bowel is thickened and edematous. these patients will require central line placement and institution of total parenteral nutrition in order to grow and minimize catabolic protein loss during the period of GI dysfunction REFERENCES 1.