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
- 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
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- 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.
- 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:
- Initially, sterile, protective sheeting is
placed over the abdominal organs.
- 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.
- 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.
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