Atresia

Atresia in children

Esophageal Atresia
Duodenal Atresia
Jejunoileal Atresia
Colonic Atresia
Extrahepatic Biliary Atresia

Esophageal atresia

 Esophageal atresia is a congenital condition

of esophageal discontinuity that results in
proximal esophageal obstruction.
Associated with Tracheo esophageal Fistula
The incidence of this anomaly is 1 in 1500 to
3000 live births, with a slight male
predominance.
Approximately one third of infants with
esophageal atresia have a low birth weight,
and 60% to 70% have associated anomalies.

EA with TEF

The diagnosis of esophageal atresia

is considered in an infant
with excessive salivation along with coughing or
choking during the first oral feeding.
A maternal history of polyhydramnios is often
present, more often in isolated proximal atresia
(86%).
In an infant with esophageal atresia and TEF, acute
gastric distention may occur as a result of air
entering the distal esophagus and stomach with
each inspired breath. Reflux of gastric contents into
the distal esophagus will traverse the TEF and spill
into the trachea, resulting in cough, tachypnea,
apnea, and/or cyanosis.

 The inability to pass a

nasogastric tube into the
stomach of the neonate is a
cardinal feature for the
diagnosis of esophageal
atresia.
If gas is present in the GI
tract below the diaphragm,
an associated TEF is
confirmed
Conversely,the inability to
pass a nasogastric tube in
an infant with absent
radiographic evidence of GI
gas is almost diagnostic of
an isolated esophageal
atresia.

Treatment
Initial treatment includes
decompression of the proximal
esophageal pouch with a sump tube
(e.g., Replogle tube) placed on
continuous suction.
Broad-spectrum IV antibiotic
coverage should be started
empirically.

Surgical management
The typical surgical approach for the
mostcommon esophageal atresia with TEF
involves an open thoracotomy,with an
extrapleural approach through the fourth
intercostal space.
Bronchoscopy is routinely performed to
determine the relative site of the fistula and
exclude the presence of a second fistula.
Alternatively, a cervical esophagostomy may be
constructed and a formal esophageal
replacement performed at a later date.

 In patients with pure esophageal atresia,

primary anastomosis in the newborn period
is not feasible because of a long gap
between the two esophageal ends.
Initially, a cervical esophagostomy for
drainage of oral secretions and gastrostomy
for enteral feeding access are performed.
An esophageal replacement using the
stomach, small intestine, or colon is then
carried out at approximately 1 year of age.

Post op complications
Postoperative complications unique
to esophageal atresia include
esophageal motility disorders,
GER (25% to 50%),
anastomotic
stricture (15% to 30%),
anastomotic leak (10% to 20%), and
tracheomalacia (8% to 15%).

Duodenal Atresia

 Duodenal atresia is thought to occur as

a result of failure of vacuolization of the
duodenum from its solid cord stage.
The range of anatomic variants includes
duodenal stenosis, mucosal web with
intact muscular wall (so-called windsock
deformity), two ends separated by a
fibrous cord, and complete separation,
with a gap within the duodenum.

 It is associated with several conditions, including

prematurity, Down syndrome, maternal
polyhydramnios, malrotation, annular pancreas,
and biliaryatresia (BA).
Other anomalies, such as cardiac, renal,
esophageal, and anorectal anomalies, are also
common.
In most cases, the duodenal obstruction is distal
to the ampulla of Vater (85%), and therefore
infants present with bilious emesis.
In patients with a mucosal web, the symptoms of
postprandial emesis may occur later in life.

Clinical presentation
Infants with duodenal obstruction are generally first
detected during a prenatal ultrasound evaluation.
Immediately after birth, a plain abdominal
radiograph shows a typical doublebubble sign, if
obtained before orogastric tube decompression of
swallowed gastric air
If distal air is present, an upper GI contrast study
should be obtained rapidly, not only to confirm the
diagnosis of duodenal stenosis or atresia but also to
exclude midgut volvulus, which would constitute a
surgical emergency.

Treatment
The management is by surgical bypass of the
duodenal obstruction as a side-to-side or proximal
transverse to distal longitudinal (diamond-shaped)
duodenoduodenostomy
At the time of anastomosis, a concomitant distal
intestinal atresia should be ruled out by injecting
saline into a distal limb using a soft red rubber
catheter. When the proximal duodenum is markedly
dilated, a tapering duodenoplasty with staples or
sutures should be considered to reduce the
duodenal caliber, which may improve postoperative
gastric emptying.

Jejunoileal Atresia
Jejunoileal atresia is the most
common GI atresia; it occurs in
approximately 1 in 2000 live births. It
is thought to occur as a result of an
intrauterine mesenteric vascular
occlusion.
Atresias occur slightly more
frequently in the jejunum than in the
ileum.

C/F
Infants present with bilious emesis, abdominal distention, and
failure to pass meconium.
Typically, the overall clinical presentation is dependent on the
level of obstruction.
In proximal atresia, abdominal distention is less, but significant
bilious emesis is present. Plain abdominal radiographs show airfluid levels with absent distal gas.
With distal atresias, abdominal distention is present more
frequently. A barium enema demonstrates a small unused colon
and may also be useful to exclude multiple atresias, which may be
present in 10% to 15% of cases.
Jejunoileal atresia is typically not associated with other anomalies
except cystic fibrosis (CF) in approximately 10% of patients.

Treatment
Infants are managed in a manner similar to that for other
conditions of neonatal bowel obstruction. An orogastric
tube is placed and appropriate IV fluid resuscitation is
implemented.
At operation, the main goal is to reestablish intestinal
continuity while preserving as much intestinal length as
possible.
In multiple atresias, multiple anastomoses over an
endoluminal stent may be necessary.
If the proximal intestine is significantly dilated, prolonged
dysmotility may persist and, therefore, a tapering
enteroplasty of the dilated bowel should be considered.
However,in cases of adequate bowel length, resection of
the dilated bowel segment can result in faster recovery.
The overall survival for infants with jejunoileal atresia is
more than 90%.

Colonic Atresia
Colonic atresia is uncommon, with an
estimated incidence of 1 in 20,000 live births.
It is the least common intestinal atresia,
accounting for only 5% to 10% of cases.
Infants with colonic atresia usually present
with failure to pass meconium, abdominal
distention, and bilious vomiting.
Plain radiographs may demonstrate distal
bowel obstruction pattern, but the diagnosis is
confirmed by barium enema.

Treatment
The classification of colonic atresias is
the same as that for the small intestine.
At operation, depending on the location
of the atretic point, primary
anastomosis after partial resection or
tapering of the proximal colon may be
considered; however, a diverting end
colostomy with delayed reversal is
preferred.

Extrahepatic Biliary
Atresia
BA is a rare disease of neonates characterized
by the inflammatory obliteration of
intrahepatic and extrahepatic bile ducts.
The incidence is estimated to be 1 in 5,000 to
12,000 infants.
It may be associated with other congenital
malformations, particularly splenic
abnormalities (e.g., asplenia, double spleen),
absence of the inferior vena cava (IVC), and
intestinal malformation.

Classify
The disease is classified according to the
level of the most proximal biliary obstruction.
Type 1 BA has patency to the level of the
common bile duct.
Type 2 has patency to the level of the
common hepatic duct and
Type 3, which accounts for more than 90% of
cases, occurs when the left and right hepatic
ducts at the level of the porta hepatis are
involved.

Presentation
Infants present shortly after birth with
jaundice, pale stools, and dark urine.
Older infants may have failure to thrive
and present with hepatomegaly and
ascites suggestive of cirrhosis.
If, in the postnatal period, the jaundice
persists after 14 days in a term infant,
a workup for liver disease should be
initiated.

Treatment
A Kasai hepatoportoenterostomy is the surgical
procedure of choice.
In this procedure, the extrahepatic biliary tree is
dissected proximally to the level of the liver
capsule, where the porta hepatis (portal plate) is
transected.
The reconstruction is performed via a Roux-en-Y
hepaticojejunostomy.
Unfortunately, if the Kasai procedure is unable to
reestablish bile flow, and liver failure and/or
cirrhosis ensue, liver transplantation is indicated.

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