Gastroschisis
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An opening beside the umbilical cord that allows bowel to protrude is called gastroschisis. Gastroschisis  is thought to arise from disruption of blood flow  to the affected abdominal wall [1]. Although gastroschisis is not associated with an increased rate of chromosomal abnormalities intestinal malrotation, atresia, volvulus,  and infarction may complicate the condition. [2] The prognosis is generally favorable with survival rates between 77% and 100% [3, 4]

Gastroschisis occurs with an incidence of 1:3,000 births in California, and for unclear reasons is seen more frequently in younger mothers [5]. The defect is also seen more frequently in mothers who use vasoactive substances such as nicotine and cocaine [7,8]. The recurrence risk of 3% to 5% suggests a complex mode of inheritance. Although, cases of a familial pattern of inheritance have been reported [6].

Antepartum

Gastroschisis is associated with intrauterine fetal growth restriction , preterm delivery, and stillbirth [9,10] .Serial songrams to evaluate growth and antenatal testing are therefore recommended. However, sonographic evaluation of bowel dilation  for the purpose of  preventing bowel injury by early delivery is not generally helpful [11].
 

Delivery

Delivery by cesarean section has not been shown to consistently improve the outcome of infants with gastroschisis. [12,13 ].
 

Treatment

At delivery the bowel is protected with moist sterile dressings, and a naso-gastric tube is placed to prevent  distention of the bowel. [14].  If the abdomen is too small to accomodate the bowel, a plastic pouch (a Silo)  is placed over the intestines, and the bowel is allowed to return to the abdominal cavity with the aid of gravity over the course of one to two weeks. The baby is usually on a breathing machine during this time. Once the bowel has returned to the abdomen, the abdomen is closed surgically.

After the procedure, the baby is fed via a feeding tube until normal bowel function occurs (usually within 3 months).

A relatively common complication is necrotizing enterocolitis (NEC), a condition where segments of the bowel die [15]. Some research suggests that feeding the infant breast milk may help to reduce the chances of developing NEC [16].
 
 



REFERENCES

1. Hoyme HE, Higginbottom MC, Jones KL.  The vascular pathogenesis of gastroschisis: intrauterine interruption of the omphalomesenteric artery.  J Pediatr. 98:228, 1981

2. Paidas MJ, Crombleholme TM, Robertson FM: Prenatal diagnosis and management of the fetus with an abdominal wall defect. Semin Perinatol 18:196, 1994

3.Morrow RJ, Whittle MJ, McNay MB, Raine PA, Gibson AM, Crossley J. Prenatal diagnosis and management of anterior abdominal wall defects in the west of Scotland. Prenat Diagn 1993;13:111-5.

4. Sipes SL, Weiner CP, Sipes DR, Grant SS, Williamson RA. Gastroschisis and omphalocele: does either antenatal diagnosis or route of delivery make a difference in perinatal outcome? Obstet Gynecol 1990;76:195-9.

5.The California Birth Defects Monitoring Program http://www.cbdmp.org/bd_gastroschisis.htm
Accessed 11/1/2008

6. Torfs CP, Curry CJ: Familial cases of gastroschisis in a population-based registry. Am J Med Genet 45:465, 1993

7.Haddow JE, Palomaki GE, Holman MS: Young maternal age and smoking during pregnancy as risk factors for gastroschisis. Teratology 47:225, 1993

8. Hume RF, Gingras JL, Martin LS, et al: Ultrasound diagnosis of fetal anomalies associated with in utero cocaine exposure: Further support for cocaine-induced vascular disruption teratogenesis. Fetal Diagn Ther 9:239, 1994

9. Tan KH, Kilby MD, Whittle MJ, et al: Congenital anterior abdominal wall defects in England and Wales 1987-1993: Retrospective analysis of OPCS data. BMJ 313:903, 1996

10. Crawford RA, Ryan G, Wright VM, et al: The importance of serial biophysical assessment of fetal wellbeing in gastroschisis. Br J Obstet Gynaecol 99:899, 1992

11.Alsulyman OM, Monteiro H, Ouzounian JG, et al: Clinical significance of prenatal ultrasonographic intestinal dilatation in fetuses with gastroschisis. Am J Obstet Gynecol 175:982, 1996

12. How HY, Harris BJ, Pietrantoni M, Evans JC, Dutton S, Khoury J, Siddiqi TA. Is vaginal delivery preferable to elective cesarean delivery in fetuses with a known ventral wall defect? Am J Obstet Gynecol 182:1527,2000

13. Quirk JG Jr, Fortney J, Collins HB 2nd, West J, Hassad SJ, Wagner C. Outcomes of newborns with gastroschisis: The effects of mode of delivery, site of delivery, and interval from birth to surgery American Am J Obstet Gynecol 174:1134 ,1996

14. Karamanoukian HL, O'Toole SJ, Glick PL: Antenatal diagnosis and perinatal care of anterior abdominal wall defects. Fetal Mat Med Rev 7:109, 1995

15. Oldham KT, Coran AG, Drongowski RA, Baker PJ, Wesley JR, Polley TZ Jr. The development of necrotizing enterocolitis following repair of gastroschisis: a surprisingly high   incidence. J Pediatr Surg. 10:945, 1988

16. Jayanthi S, Seymour P, Puntis JW, Stringer MD. Necrotizing enterocolitis after gastroschisis repair: a preventable complication? J Pediatr Surg. 33:705,1988 MEDLINE

 

 


 

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