SEMINAR

ON
CHOANAL ATRESIA

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INDEX

Sl No Topic Page no

1 Introduction 4

2 Definition 5

3 Incidence 5

4 Causes 5

5 Pathophysiology 6

6 Types 7

7 Associated Conditions 7-8

8 Signs and symptoms 8-9

9 Diagnosis 9

10 Treatment 9-12

11 Nursing management 12

12 Prognosis 12

13 Complication 12

14 Prevention 13

15 Research evidence 13-15

16 Conclusion 15

17 Reference 15-16

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SPECIFIC OBJECTIVES

At the end of the seminar students will be able to

 define Choanal Atresia

 explain the incidence of choanal atresia

 enlist the causes of choanal atresia

 explain pathophysiology of choanal atresia

 list down the types of choanal atresia

 enlist the signs and symptoms of choanal atresia

 describe diagnostic findings choanal atresia

 explain treatment of choanal atresia

 enumerate the complication of choanal atresia

 describe prevention of 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.

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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

The condition is the 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%) and occurs more frequently in females than in males (2:1)

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

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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 or bilateral. i.e. Choanal atresia may affect one or both sides of the nasal

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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.

C = Coloboma (eye abnormality in which there are missing pieces of tissues in structure that

form the eye)

H = Heart disease

A = Atresia of the choanae

R = Retarded growth and development

G = Genito urinary defects

E = Ear anomalies or deafness or both

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Also any condition that causes significant depression of the nasal bridge or midface retraction

can be associated with choanal atresia. Examples include the craniosynostosis syndromes

such as Crouzon syndrome, Pfeiffer syndrome, and Antley-Bixler syndrome.

SIGNS AND SYMPTOMS

Symptoms

Unilateral choanal atresia: chronic nasal discharge, nasal obstruction, thick nasal secretions

on examination.

Babies with choanal atresia have difficulty breathing unless they are crying.

Choanal atresia blocking both sides (bilateral) of the nose causes acute breathing problems

with cyanosis and breathing failure.

Bilateral Choanal Atresia may present as cyanosis while the baby is feeding, because the

oral air passages are blocked by the tongue, further restricting the airway. The cyanosis may

improve when the baby cries, as the oral airway is used at this time.

Symptoms include:

 Chest retracts unless the child is breathing through mouth or crying

 Difficulty breathing following birth, which may result in cyanosis (bluish

discoloration), unless infant in crying.

 Inability to pass a catheter through each side of the nose into the throat

 Persistent one-sided nasal blockage or discharge

 Noisy breathing

Signs

Choanal atresia can be presented as

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  impossible to insert a nasal catheter.

 if there is a continuous stream of mucous draining from one or both nostrils

DIAGNOSIS

 Physical examination in which mainly insertion of catheter through one or both nares and

any inability to pass the catheter is noted as a sign of blockage in airways.

 Endoscopy/rhinoscopy

The scope is inserted either through mouth or nose which help to view the airways for any

abnormalities.

 Computed tomography (CT) scan: This is a form of an imaging test that uses X-rays to

produce comprehensive pictures of sinuses. It demonstrates the atretic plate thickness and

differentiates between bony and membranous atresia.

 Sinus x-ray

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 focussed 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.

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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

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to accomplish this goal, all of which are performed in the operating room under general

anesthesia.

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.

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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.

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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.

RESEARCH EVIDENCE

1. Prognostic Factors and Management of Patients with Choanal Atresia

Objective: To analyze prognostic factors in the management of patients with choanal atresia.

Study design: This is a review of 114 patients operated on for choanal atresia in a tertiary

care center between November 1986 and November 2016, including clinical characteristics,

surgical management, and postoperative course with final nasal patency. The data were

collected in a database that was updated over time.

Results: Among the 114 patients, 78 were female, 77 presented with unilateral choanal

atresia, and 37 presented with bilateral unilateral choanal atresia, corresponding to 151 nasal

fossae. Forty-seven patients had associated abnormalities (41.2%), including CHARGE,

diagnosed in 20 children (17.5%). At the end of follow-up, 91 patients (79.8%) had normal

choanal patency. The identified risk factors of surgical failure were age <6 months (P = .004),

weight <5 kg (P = .007), and bilateral choanal atresia (P < .001). The type of atresia, presence

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of associated abnormalities, surgical approach, stenting, and use of mitomycin were not

significantly linked with the surgical results.

Conclusions: This series highlights the importance of the timing of surgery, which is guided

by the clinical ability of the infant to tolerate the procedure. Endoscopic approaches are

widely performed, but a transpalatal approach, necessary in some cases of bilateral choanal

atresia, does not alter the results. Unilateral choanal atresia surgery should be delayed after

age 6 months and/or weight >5 kg when possible.

2. Clinical retrospective analysis of 15 cases of choanal atresia – Our experience

Objective: Choanal atresia is a rare congenital disorder due to failed recanalization of the

nasal fossae during fetal development. This article focuses on our experience in dealing with

choanal atresia and its management. Here we discuss the varied clinical symptoms that the

patients presented with, the clinical tests and investigations that were specific in diagnosing

this condition and surgical management of these cases with endoscopic transnasal

choanaplasty with stenting and follow up topical Mitomycin C application.

Material and methods: This is a retrospective study based on computerized medical record

review of the patients born in Department of ENT of Sur Hospital between 2002 and 2017.

The patients were assessed with detailed history, presentation of clinical symptoms and all

underwent nasal endoscopy and CT scans for assessing the atretic type. These patients

underwent transnasal endoscopic choanaplasty under general anesthesia using microdebrider

and stented using endotracheal tube. The patients were regularly followed up for review with

nasal endoscopy after discharge between 4th and 7th postoperative period. The parents were

educated on nursing care and the stent was removed in 4 weeks, all patients had Mitomycin C

applied to the neochoana and were on regular follow up for a year with no recurrence.

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Results: Fifteen patient records were analyzed, 10 females and 5 males, ages varying from

newborns up to 14 years old. Unilateral to bilateral choanal atresia was 4:1 ratio and female-

male showed 2:1 ratio. Right malformation was predominant in both sexes in unilateral

atresia. Mixed imperforation (bone-membranous) was the most frequently observed type,

followed by bone malformation. The commonest symptom during diagnosis was rhinorrhea

and the least one was respiratory failure. Majority of cases were diagnosed by CT scans of

sinuses. Around 10% patients presented with cardiac problems. None had restenosis in one

year follow up.

Conclusions: Neonates with acute respiratory insufficiency due to choanal atresia can be

diagnosed with simple bedside tests like cold spatula test, less invasive tests like failure to

pass intranasal catheter, CT scan. Surgical correction with endoscopic intranasal choanaplasty

is the way to address this problem and could avoid radical palatal approach, less morbidity

and high

success rate.

CONCLUSION

When faced with a neonate or other aged child with signs of nasal/upper airway obstruction

or respiratory distress, one must consider the possibility of a unilateral or bilateral choanal

atresia. If choanal atresia is identified, it is necessary to investigate other possible associated

anomalies such as those of CHARGE syndrome. If bilateral atresia is found, this is

considered an upper airway emergency and the proper measures should be taken to stabilize

the patient first before considering surgical intervention.

REFERENCES

1. Marlow DR. Redding B.A.Testbook of paediatric nursing edition, Noida, 2008

2. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMFH0002608/

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3. http://en.wikipedia.org/wiki/Choanal_atresia

4. Gleason CA, Juul SE. Avery’s diseases of the newborn: First south Asia edition. New

Delhi, India: Elsevier; 2018.

5. Hansen AR, Stark AR, Eichenwald EC, Martin CR. Cloherty and stark’s manual of

neonatal care. 9th ed. Baltimore, MD: Wolters Kluwer Health; 2023.

6. Moreddu E, Rossi M-E, Nicollas R, Triglia J-M. Prognostic factors and management

of patients with choanal atresia. J Pediatr [Internet]. 2019;204:234-239.e1. Available

from: http://dx.doi.org/10.1016/j.jpeds.2018.08.074

7. Abdul Cader SH, Shah FA, Reghunandanan N. Clinical retrospective analysis of 15

cases of choanal atresia – Our experience. World J Otorhinolaryngol-head Neck Surg

[Internet]. 2019;5(4):188–92. Available from:

http://dx.doi.org/10.1016/j.wjorl.2019.03.003

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