1. Congenital Anomalies
Ralph Vogel, RN, PhD, CPNP

2. Gastroschisis
There is no sac and the intestine is herniated through the abdominal wall lateral to the umbilicus.
The eviscerated mass is dark with a bloody or green appearance and covered in a gelatinous substance.
The cause is not known

3. Clinical Manifestations Gastroschisis
Polyhydramnios is noted in utero.
Sixty percent are born prematurely
Fifteen percent have associated jejunoileal stenoses or atresias.
Other than the appearance of the mass there are few clinical signs.
GASTROSCHISIS IS A SURGICAL EMERGENCY. WRAP IN TOWEL AND GET TO OR. MAY START ANTIBIOTICS.

4. Tracheoesophageal Fistula
Congenially from abnormal anastomosis of inferior and superior esophogus. The lower section develops from the primitive foregut.
85% of these abnormalies are TEF.
Associated anomolies include PDA, cardiac vascular ring, coarctation of the aorta, inperforate anus, malrotation and duodenal abnormalities

5. VACTERL Syndrome associated with TEF: Vertebral Anal Cardiac, Tracheal
Esophageal
Renal and
Limb anomalies

6. Clinical Manifestations TEF
Excessive oral secretions
Inability to feed with gagging and respiratory distress
Polyhydramnios may be seen on ultrasound in utero
May or may not have gas in abdomen on flat plate depending on type.

7. Treatment TEF Place infant in 60 degree head-up prone position
Minimize disturbance of infant
May place Repogle tube to suction swallowed oral secretions
Surgery is usually necessary but may be delayed if the ends are not close enough for anastomosis.

8. Duodenal Atresia May be complete or partial with stenosis
Duodeum does not open up during 8th to 10th week of gestation. More common in prematurity and Trisomy 21.
Seventy percent are associated with other malformations such as cardiac, annular pancreas, malrotation of intestines, and imporforate anus.

9. Clinical Manifestation DA
In utero polyhydraminios with complete obstruction
After birth has bilious emesis within hours of first feed
Gastric distention on radiograph proximal to site known as “double bubble” sign
Treatment is surgical correction.

10. Congenital Diaphragmatic Hernia
Defect in diaphragm, usually left side, that allows abdominal content to enter thorax and compromise pulmonary development
Also called Bochdalek hernia
Combination of pulmonary compromise and pulmonary hypertension often make this lethal.

11. Con. Hernia Clinical Man.
Respiratory distress from birth.
Decreased breath sounds on affected side and heart sound shifted to other side.
Scaphoid abdomen (boat shape)
Diagnosed by ultrasound as fetus or radiograph after birth

12. Con. Hernia Treatment
Because of pulmonary compromise and hypertension the child needs to be ventilated and may need high frequency ventilation or ECMO.
Once pulmonary problems are stable the treatment of choice is surgery.

13. Omphalocele
Results when the abdominal viscera herniate through the umbilical and suprumbilical portions of the abdominal wall and create a sac. Incidence 1:6000
Large defects may contain gastrointestinal tract, liver and spleen.
Sac covering is thin and may rupture in utero or during delivery

14. Omphalocele Manifestations
Polyhydramnios is noted in utero and 10% are born premature.
Often diagnosed by prenatal ultrasound
35% with GI defects, 20% cardiac and 10% with Beckwith-Wiedeman syndrome.
S/S BWS include: exophthalmos, macroglossia, gigantism, hyperinsulinemia and hypoglycemia.

15. Omphalocele Treatment
Cesarean section to prevent rupture
Cover sac with petroleum gauze
Nasogastric tube to minimize gastrointestinal distention
Wrap infant in dry sterile cloth to decrease heat loss and Kling gauze to support viscera.
Do not reduce, get surgical consult

16. Omphalocele Use broad spectrum antibiotics
Definitive surgery should be delayed until infant is stable.
May be delayed as long as sac is intact, if ruptures use same measures and get emergency surgery intervention.