G.I. Anomalies in Newborn and Pediatric Care

 

 

 

Necrotizing Entero-Colitis –NEC

  • Definition
  • Cause
  • Genetics
  • Associated problems – Prematurity
  • Epidemiology
  • Immediate care
  • Surgical Repair
  • Complications
  • Prognosis mortality in Georgia 38.5%.  15% of all deaths after the first week of life for infants weighting 1500 g or less at birth.

 

 

Gastroschesis:

 

  • Definition- an abdominal wall defect to the side of the umbilical cord (umbilicus). The infant is born with intestines protruding through the defect and no protective sac is present.  MSAPF levels are greater in gastroschisis than in omphalocele
  • Cause- Abdominal wall defects occur as a result of failure of the mesoderm to replace the body stalk. Disruption of blood flow to the affected abdominal wall occurs. Seen more often in younger mothers or those who use cocaine, nicotine. Recurrance 3-5%
  • Antepartum- IUFD, growth retardation, preterm delivery & stillbirth.
  • Genetics- Gastroschisis is rarely associated with other birth defects. Gastroschisis is a life-threatening event requiring immediate intervention.
  • Associated Problems-  include; malrotation, atresia, volvulus, infarction
  • Epidemiology- 77 – 100% survive  Occurs in 1:10,000 births
  • Immediate Care- Delivery by C/S may be helpful. Immediately after delivery, the exposed organs are covered with warm, moist, sterile dressings. A tube is inserted into the stomach (nasogastric tube, also called NG tube) to keep the stomach empty and to prevent choking on or breathing in of stomach contents into the lungs.
  • Surgical Repair- Surgical repair of abdominal wall defects involves replacing the abdominal organs back into the abdomen through the abdominal wall defect, repairing the defect if possible, or creating a sterile pouch to protect the intestines while they are gradually pushed back into the abdomen over 1-2 weeks using gravity.
  • Complications- Necrotizing entero colitis which may be reduced by feeding with breast milk.
  • Prognosis – normalization within 3 months
  •  

Procedure

 

 

 

 

 Omphalocele
ProcedureCase 52: Image 2

  • Definition- An omphalocele is an abdominal wall defect at through an opening in the abdominal muscles in the area of the umbilical cord. the base of the umbilical cord (umbilicus); the infant is born with a translucent membrane protruding through the defect which contains small intestine, liver, and large intestine.  The defect occurs in-utero The omphalocele may be small, with only a portion of the intestine protruding outside the abdominal cavity, or large, with most of the abdominal organs (including intestine, liver, and spleen) present outside the abdominal cavity. Further, the abdominal cavity itself may be small due to underdevelopment during pregnancy.  Rupture may occur in utero (> 10%)

  • Cause- As a fetus is growing in the mother's uterus before birth, different organ systems are developing and maturing. Between the sixth and 10th weeks of pregnancy, the intestines actually project into the umbilical cord as they are growing. By the 11th week of development, the intestines should return to the abdomen. When the fetus is growing and developing during pregnancy, there is a small opening in the abdominal muscles that the umbilical cord can pass through, connecting the mother to the fetus. As the fetus matures, the abdominal muscles should meet in the middle and grow together, closing off this opening. An omphalocele occurs when the abdominal organs do not return to the abdominal cavity as they should.  It is not known what causes omphalocele.

 

  • Genetics- When an omphalocele is isolated (no other birth defects are present), the risk for it to happen in a future pregnancy is 1 percent, or one in 100. There are some families that have been reported to have an omphalocele inherited as an autosomal dominant or X-linked recessive trait. In these cases, the chance for reoccurrence would be higher.
      1. Thirty percent have a chromosomal (genetic) abnormality, most commonly trisomy 13, trisomy 18, trisomy 21 (Down syndrome), Turner syndrome, or triploidy.

 

 

  • Associated problems- Many babies born with an omphalocele also have other abnormalities. The chance for reoccurrence depends upon the underlying disorder: Associated with increased maternal age

 

    • Some infants with omphalocele have a syndrome known as Beckwith-Wiedemann syndrome.
    • More than two-thirds of babies with omphalocele have abnormalities of other organs or body parts, most commonly the spine, digestive system, heart, urinary system, and limbs.

 

  • Epidemiology-  
    • A small omphalocele (involving protrusion of a small portion of the intestine only) occurs in one out of every 5,000 live births.
    • A large omphalocele (involving protrusion of the intestines, liver, and other organs) occurs in one out of every 10,000 live births.
      Omphalocele occurs relatively equally in boys and girls.

 

  • Immediate care Because some or all of the abdominal organs are outside the body, infection is a concern, especially if the protective membrane around the organs breaks. Also, an organ may lose its blood supply if it becomes pinched or twisted. A loss of blood flow can damage the affected organ. Omphalocele can often be detected on fetal ultrasound in the second and third trimesters of pregnancy. A fetal echocardiogram (ultrasound of the heart) may also be done to check for heart abnormalities before the baby is born.  After birth, the omphalocele can be noted by the physician during the physical examination. X-rays (diagnostic tests that use invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film) may also be done after birth to evaluate abnormalities of other organs or body parts.

  • Surgical Repair- Specific treatment for an omphalocele will be determined by: the baby's gestational age, overall health, and medical history extent of the condition the baby's tolerance for specific medications, procedures, or therapies.
    • For a small omphalocele (only a portion of the intestine protruding outside the abdominal cavity), shortly after birth, an operation is done to return the organs to the abdomen and close the opening in the abdominal wall.
    • For a large omphalocele (most of the abdominal organs, including intestine, liver, and spleen, are present outside the abdominal cavity), the repair is done in stages and may include the following:
      1. Initially, sterile, protective sheeting is placed over the abdominal organs.
      2. Because the abdomen may be small and underdeveloped, it may not be able to hold all of the organs at once. Therefore, the exposed organs are gradually moved back into the abdomen over several days or weeks. The abdominal wall is closed surgically once the organs have been returned to the abdominal cavity.
      3. Because the abdominal cavity may be small and underdeveloped and the organs may be swollen, a baby with an omphalocele may have breathing difficulties as the organs are returned to the abdomen. The baby may need help from mechanical ventilation while the swelling is decreasing and the size of the abdominal cavity is increasing.
  • Post-Operative Care- The infant is cared for post-operatively in a neonatal intensive care unit. The baby is placed in an isolette (incubator) to keep warm and prevent infection. Oxygen is given and mechanical ventilation is often required. Intravenous fluids, antibiotics, and pain medications will be given. A nasogastric tube will be in place to keep the stomach emptied of gastric secretions. Feedings are started by nasogastric tube as soon as bowel function resumes. Feedings are started very slowly and often infants are reluctant to feed. These babies may need feeding therapy and lots of encouragement.

 

  • Complications- Problems in the future often depend on:
    • the size of the omphalocele.
    • if there was a loss of blood flow to part of the intestine or other organs.
    • the extent of other abnormalities

 

  • Prognosis.

o        Babies who have damage to the intestines or other abdominal organs may have long-term problems with digestion, elimination, and infection.



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. Calzolari E, Bianchi F, Dolk H, et al: Omphalocele and gastroschisis in Europe: A survey of 3 million births, 1980-1990. Am J Med Genet 58:187, 1995

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

17. Epidemiologic characteristics of necrotizing enterocolitis: a population-based study R Wilson, WP Kanto Jr, BJ McCarthy, T Burton, P Lewin, J Terry and RA Feldman

created November 16, 2005 by; Kim Hayes, Daniel Henriquez, Michael Jackson, Jody Kenneally, Doris Obando, Brooke Roebuck (NU Class of 2006)

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