1. Tracheoesophageal fistula occurs due to deviation or altered cellular growth in the septum that separates the respiratory and esophageal primordia during development. It has an incidence of 1 in 4,000 live births. 2. Clinical features include excessive drooling, choking, and cyanosis during feeding as well as aspiration pneumonia from overflow of secretions into the lungs. 3. Diagnosis is made by passing a stiff catheter into the esophagus and obtaining an x-ray, which will show an air bubble in the stomach if there is a communication between the esophagus and trachea.

1. G.ERIC MD4

2. Upper part of esophagus is developed from
retropharyngeal
segment and the lower part from pregastric
segment of the first part of the primitive gut. At four
weeks
of gestation, the laryngotracheal groove is formed. Later,
two longitudinal furrows develop to separate the
respiratory primordium from the esophagus. Deviation
or altered cellular growth in this septum results in
formation of tracheoesophageal fistulae. Incidence is 1 in
4000 live births. In the most common variety (over 80%
of
cases), the upper part of the esophagus ends blindly and
the lower part is connected to the trachea by a fistula

3. Clinical Features
The presence of maternal polyhydramnois and single
umbilical artery should alert the health provider to look
for atresia of the upper digestive tract. Association of
congenital anomalies of vertebrae, anorectal region,
heart,
kidneys or limbs should also arouse suspicion. The
newborn baby has excessive drooling soon after birth
with
frothing. There is choking and cyanosis on feeding.
Overflow of milk and saliva from esophagus and
regurgitation of secretion through the fistulous tract
(when
present) into the lungs results in aspiration pneumonia.

4. Diagnosis
A stiff red rubber catheter cannot be passed into stomach
as it gets arrested at a distance of 7-10 cm from the
mouthA skiagram may be obtained after instilling
1-2 ml of air through the catheter. It is not advisable to
use barium as a contrast material since it may be aspirated
in lungs.
On X-ray, an air bubble is seen in the stomach if there is
communication between the lower part of the esopagus
and trachea, which occurs in the commonest variety of
tracheoesophageal fistula. In other variety, wherein there
is no communication of esophagus and trachea, there will
be no gas in stomach

5. Management
The baby should be nursed supine or in an
upright
position and esophageal pouch should be
gently suckedevery five minutes, or
continuously using a slow suction
device. Intravenous fluids should be
administered and
infection, if any should be treated. Surgical
repair should
be undertaken as early as possible.

7. A variety of anorectal anomalies have been described.
These may be anatomically classified as high,
intermediate
or low. The position is determined by the relation of
terminal part of bowel to the puborectalis sling. High or
intermediate lesions are more common in males. Anal
stenosis or covered anus, anocutaneous fistulae are
common in both sexes. Anovestibularfistula (low
lesion)
in females and anorectal agenesis with rectoprostatic
urethral fistula (high lesion) are common in male
infants.

8. Among the males with high or intermediate lesions,
80% are rectovesical fistulae, but among the females with
high defect, 80% have a rectovaginal fistula. There are
significant chances of associated anomalies in case of
higher anorectal anomalies. An X-ray film of the abdomen is
obtained 12-24 hr after
birth, with the baby being kept in an inverted position. A
lateral picture of the pelvis should be obtained to define
whether the rectal pouch is above or below a line drawn
from the pubis to the coccyx.
Treatment is surgical. Prognosis is better with low
defects. About 80 to 90% of patients become continents
after surgery for low defects. More than two-thirds of
patients are incontinent after surgery of high defects.

9. Anencephaly. Anencephaly is due to a defect in
the
development of neural axis and is not
compatible with life.
Encephalocele. In encephalocele, the brain
and/ or its
coverings herniate through a defect in the
skull.

10. Congenital hydrocephalus. Congenital hydropcephalus
results from impaired CSP circulation or absorption in
basal cisterns. This usually follows intrauterine infections
such as toxoplasmosis, rubella, cytomegalovirus and
syphilis, but may also be the result of a congenital
malformation of the aqueduct, Dandy-Walker syndrome
(posterior fossa cyst and a defect of cerebellar verrnis),
Arnold-Chiari malformation (displacement of brainstem
and cerebellum in the spinal canal) or multiple congenital
malformations of the nervous system. Diagnosis should be suspected if
the head is too large
or sutures and fontanels are wide open or if the head
circumference increases rapidly (more than 1 cm in a
fortnight during the first three months). CT or MRI scan
should be done to confirm the diagnosis. The type of
dilatation of ventricles indicates the site of obstruction.
Isolated aqueductal stenosis has better prognosis.
Treatment should be directed at the specific cause if
amenable to therapy and surgical intervention such as
ventriculocaval or ventriculoperioneal shunt.

11. Myelomeningocele. It presents as membranous protrusion
at the lumbosacral region and contains meninges,
cerebrospinal fluid, nerve roots and a dysplastic spinal
cord. The defect is open and not covered by skin. In
contrast, meningocele is covered with skin. There may be
no associated neurological deficit, but Arnold-Chiari
malformation and congenital hydrocephalus are often
associated. Severe motor and sensory deficit are common
and urinary and fecal incontinence are usually present.
Meningomyelocele is operated only if there is no paralysis
of lower limbs and if there is no bladder /bowel involvement.
Folic acid 4 mg per day should be prescribed to the
women in periconceptional period to prevent recurrence.

12. Cleft lip is recognized readily, but a careful
inspection of the oral cavity is necessary to identify
cleftpalate. A cleft of the soft palate can be easily missed
unless
the baby is examined carefully. Ventricular septal defect
is a common associated anomaly with cleft palate.
In Pierre-Robin syndrome, cleft palate is associated with
retracted jaw (micrognathia) and large tongue, with a
tendency for glossoptosis. Feeding is difficult in cases of
cleft palate. For the first few days, gavage feeding or
spoon-feeding may be done. Bottle feeding may be tried
with a soft nipple with rubber flange, which close the cleft
and help the baby in sucking. If this is not successful,
palatal prosthesis may be used.

14. Management. Management of cleft palate
requires a team
effort involving a p ediatrician, a plastic
surgeon,
orthodontist, ENT specialist and speech
therapist. Cleft
lip is repaired in the neonatal period. Operation
for cleft
palate is generally deferred until the second
year.

15. Diaphragmatic Hernia
Diaphragmatic hernia occurs because of failure of
closure
of the pleuroperitoneal membrane. This allows intestinal
loops to ascend to the thorax that compress the
developing
lung and can result in pulmonary hypoplasia (Fig. 8.56).
These babies can present at any time after birth. At
birth,
a baby may be suspected to have diaphragmatic hernia
if
there is respiratory distress and a scaphoid abdomen.
Bag
and mask ventilation should be avoided in these babies.
Surgical repair after stabilization is the treatment of
choice.