Choanal Atresia

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INTRODUCTION

Choana is the posterior nasal hole where air passes from nasal cavity into

the nasopharynx Choanal atresia is a condition that obstructs nasal

airways leading to breathing difficulties. Choanal atresia manifests when

the back of nose does not open or is not able to communicate with the rest

of airway system. It is usually a congenital disorder. It results from failed

resorption of the buccopharyngeal membrane either unilaterally or

bilaterally or even partial with severe stenosis.


DEFINITION

• Choanal Atresia is a congenital anomaly of the anterior skull base

characterized by closure of one or both posterior nasal cavities (the back

of the nasal passage called choana is blocked by abnormal bony or soft

tissue formed during fetal development).

• In choanal atresia, nasal aperture is occluded from birth, due to failed

recanalization of the nasal fossae during fetal development.


INCIDENCE

• Most common nasal abnormality in newborn infants

• Affecting about 1 in 5,000 and in 9000 live births.

• Females get this condition about twice as often as males.

• More than half of affected infants also have other congenital

problems. It is more often unilateral than bilateral (60% vs 40%)


CAUSES

• Choanal atresia is a developmental abnormality.

• The cause of choanal atresia is unknown.

• It is thought to occur when the thin tissue separating the nose

and mouth area during fetal development remains after birth.


• EMBRYOGENESIS THEORY: buccopharyngeal membrane

from foregut ruptures between 5-6 week of gestation which

forms choanae. Failure of rupture of this membrane can cause

choanal atresia
Embryogenesis
The nasal cavities extend posteriorly during development under the
influence of the posteriorly directed fusion of the palatal processes.
Thinning of the membrane occurs, which separates the nasal cavities
from the oral cavity. By the 38th day of development, the 2-layer
membrane consisting of nasal and oral epithelia ruptures and forms the
choanae (posterior nares). Failure of this rupture results in choanal
atresia.
PATHOPHYSIOLOGY
Multifactor involvement (embryo genetic theory)

Failure of rupture of buccopharyngeal membrane

Abnormal mesodermal adhesion forming in the choanal area

Choanal atresia

Respiratory arrest
Aspiration
Death
PRESENTATION

It can be

• Unilateral

• Bilateral

i.e. Choanal atresia may affect one or both sides of the nasal
airway. More commonly right sided.
Unilateral Choanal Atresia: More than half of infants have a blockage on
only one side, unilateral atresia may present simply with unilateral
discharge and feeding difficulties, which causes less severe problems
and is not detected until much later in life because the baby manages to
get along with only one nostril available for breathing. The inability to
pass 5 or 6 French catheter through the nose may suggest the
diagnosis. In unilateral cases repair can be delayed up to 2 years,
drilling of atretic plate, balloon and use of laser have been described in
repair.
Bilateral Choanal Atresia is a very serious life-threatening
condition because the baby will then be unable to breathe directly
after birth as babies are obligate nasal breathers (they must use
their noses to breathe).
ASSOCIATED CONDITIONS
• Sometimes babies born with choanal atresia also have other
abnormalities

• Approximately 50-70% of affected infants have other congenital


abnormalities. The CHARGE syndrome is one of the most common
anomalies associated with choanal atresia.
DIAGNOSIS

• Physical examination

• Endoscopy/rhinoscopy

• Computed tomography (CT) scan


TREATMENT

Emergency intervention is necessary at birth to ensure the

infant's survival. Surgery within the first month is frequently

necessary to perforate the blocked nasal passages. After these

interventions, the infant requires continued care within the home


EMERGENCY INTERVENTION
Emergent management of choanal atresia is focused on ensuring that
the oropharyngeal airway is patent. Infants with bilateral choanal atresia
may need resuscitation at delivery. The immediate concern is to
resuscitate the baby if necessary.

Temporary alleviation can be achieved by inserting an ORAL AIRWAY


into the mouth. In some cases, intubation or tracheostomy may be
needed.
Treatment for mild cases (unilateral)

Sometimes, a child with unilateral choanal atresia has no respiratory

distress or significant feeding issues. These patients may initially be

managed with close observation and occasionally supplemental oxygen

Nasal saline can help to keep the nasal linings healthy and free of

discharge. Children diagnosed with unilateral choanal atresia may wait

until the child grows older before deciding to repair the condition.
Bilateral choanal atresia requires urgent management by inserting a finger

in the baby’s mouth and depressing the tongue down and forward away

from the back of the throat. This should be replaced with a plastic

oropharyngeal airway or a McGovern open-tip nipple. Failure of these

measures may necessitate intubation or tracheostomy.


SURGERY
(is the only definitive treatment) to remove the obstruction cures the
problem. Surgery may be delayed if the infant can tolerate mouth breathing.
The surgery may be done through the nose (transnasal) or through the
mouth (transpalatal). Surgery corrects the defect by perforating the atresia to
create a nasopharyngeal airway. If a membranous obstruction is present, it
can be pierced by the use of nasoscope. If the blockage is caused by bone,
this is drilled through and stent inserted. A stent may be inserted to keep the
newly formed airway patent or repeated dilatation may be performed.
Treatment for severe cases (bilateral)
If child is diagnosed with bilateral choanal atresia the condition must be

treated as soon as possible. Surgery should be performed as soon as the

patient is stable and has been evaluated for other anomalies. The major

goal of the surgical repair is to open the nasal airway sufficiently, allowing

the infant to breathe on his own. There are several approaches available

to accomplish this goal, all of which are performed in the operating room
Transnasal endoscopic approach
Currently prefer to treat children with choanal atresia using the transnasal
endoscopic approach. It can be done safely on infants just a few days old.
The posterior nasal airway is opened using a combination of dilators,
sinus instruments, ear curettes and drills. In some cases, a piece of
endotracheal tube is placed inside the nasal passage as a stent to
maintain the opening while the area heals. The stent will be removed a
few weeks after the surgery at the bedside. Sometimes stenting is not
required.
Transpalatal approach

In rare circumstances where the skull base is abnormally

developed (other craniofacial anomalies are present), may need

to use a transpalatal approach to reach the area of choanal

atresia through the roof of the mouth.


Aero-digestive evaluation
If infant has choanal atresia, he should be evaluated for both airway and
feeding issues. Management of the airway often requires a combination of
supportive, medical and surgical care. Feeding and swallowing issues are
very common in children with these conditions and often need to be
addressed by speech pathologists and Gl specialists. Children born with
syndromes often need other subspecialty evaluations (cardiology,
ophthalmology, etc.) and benefit greatly from the coordinated care
provided in the multidisciplinary setting of the Center for Pediatric Airway
Disorders.
NURSING MANAGEMENT
• Keep the nostrils clean

• Prevent upper respiratory infections

• Gavage feeding for infants with bilateral choanal atresia until defect is
corrected

• Follow precautions and techniques of feeding for unilateral atresia as


there arises trouble with breathing and sucking at the same time and
from the danger of aspiration: feed the infant slowly with frequent
pauses to permit breathing.
• Watch for any problems with difficulty in swallowing after sucking, if so

remove the nipple at once.

• Ensure the size of the nipple hole should be carefully chosen to

provide the rate of flow that the infant finds easier to handle.

• Keep the infants in an upright position when they are fed and that the

bottle is supported in proper angle.


PROGNOSIS

Full recovery is expected.

COMPLICATIONS

Possible complications include:

•Aspiration while feeding and attempting to breathe through the mouth

•Respiratory arrest

•Re narrowing of the area after surgery

•Brain damage and death due to hypoxia


PREVENTION

• There is no known prevention

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