CHOANAL ATRESIA

EMBRYOLOGY

Choanal atresia occurs in about 1 in 7000 births, so it is not rare. It occurs when the nasobuccal
membrane of Hochstetter fails to involute, leaving a plate of tissue across the back of the nose, closing it
off completely and separating it from the pharynx. The nose looks normal from the outside, but it is
completely blocked to airflow on one or both sides. The atretic plate is made of bone, thick at the edges
but quite thin in the middle. The central part may in fact be membranous rather than bony in 70 per cent
of cases, although this membranous part is often quite small. Just over half of all cases are unilateral the
rest bilateral.

Choanal atresia often presents as an airway emergency soon after birth. It is such a serious problem
because newborn babies are unable to breathe through their mouths by choice; they are obligate nasal
breathers. This occurs because the larynx of the neonate is positioned very high, so that it is in line with
the back of the nose. The uvula and epiglottis sit on top of one another, directing the food stream
around the sides of the larynx to the piriform fossae, while the air goes in the midline through the nose
to the larynx. This allows the infant to suckle and breathe at the same time. Subsequent descent of the
larynx into its normal adult position is a uniquely human phenomenon that facilitates mouth-breathing
and speech.

ASSOCIATED CONDITIONS

Around 75 percent of children with choanal atresia have other congenital abnormalities, and in half
there is a named syndrome. The most common of these is CHARGE (coloboma, heart defects, choanal
atresia, retarded growth and development, genital hypoplasia, ear anomalies and deafness), which is
present in approximately 25 per cent of children with choanal atresia

CLINICAL PRESENTATION

Infants with nasal obstruction will be very snuffly and may have difficulties with feeding, leading to a
referral to the otolaryngologist in the neonatal period. Most bilateral cases and some unilateral ones
present in this way. The classical presentation of choanal atresia is with cyclical hypoxia: The baby cries,
taking in air through the mouth (which relieves the hypoxia) but then settles down, closes the mouth,
struggles to breathe and becomes hypoxic again. Repeated oxygen desaturations occur with consequent
growth failure and effects on brain development. In many cases, the presentation is the same as for any
airway emergency with noisy breathing (stertor), respiratory distress and persistent hypoxia.

Some children with unilateral (and occasionally bilateral) atresia will make it through the neonatal period
without any intervention. They present in their preschool (toddler) years with unilateral nasal discharge,
or complaining of nasal obstruction in later childhood
PHYSICAL EXAMINATION

It is important to make an assessment of the urgency of the situation based on signs of respiratory
distress, including stertor, intercostal recession, sternal recession, tracheal tug, respiratory rate,
peripheral perfusion, conscious level and oxygen saturation. Clinical examination of the infant’s nose is
easy, and in most cases the diagnosis can be made by means of a few simple manoeuvres. First, examine
the nose with an otoscope. This gives excellent illumination and magnification and will allow you to see if
there is a nasal mass causing obstruction. Next, look for airflow through both nostrils by holding a cold
metal spatula under the nose. Misting excludes choanal atresia affecting that nostril. Finally, attempt to
pass a nasogastric tube through each nostril in turn. Failure to get past the nostril suggests piriform
aperture stenosis (see discussion that follows), while a tube that passes into the nasal cavity but stops at
the back of the nose suggests choanal atresia.

DIFFERENTIAL DIAGNOSIS NASAL MASSES

These can cause nasal blockage and therefore present in the same way as choanal atresia. The most
common nasal masses in infants are meningoencephalocoeles and gliomas, which develop from
herniations of meninges and brain tissue through the anterior skull base down into the nose.
Meningoencephalocoeles are still connected to the meninges and brain, while nasal gliomas are isolated
lumps of brain tissue in the nose, cut off from the brain by the formation of the anterior skull base. They
should be obvious on clinical inspection of the nose. The important point is to remember that congenital
nasal masses may be connected to the brain, so they should always be scanned before any kind of
surgical intervention such as a biopsy. Transnasal excision with repair of the skull base is the approach of
choice.

Haemangiomas (strawberry naevi) can occur in the nose and may cause nasal obstruction and poor
feeding. They are obvious on nasal examination. It may be worth getting a scan to define the extent of
the lesion. Without any treatment they grow, plateau and then eventually disappear, usually over 2 to 5
years, but if they are causing symptoms, they may need treatment. Corticosteroids, propranolol and
surgery all have a role, depending on the site and size of the lesion.

PIRIFORM APERTURE STENOSIS

This is a rare abnormality of midline structures producing narrowing at the bony entrance to the nose It
is often associated with a single central mega-incisor tooth and abnormalities of the pituitary gland and
brain. It presents like choanal atresia, but on examination the nostrils are narrow and slit-like, and it is
difficult to pass a nasogastric tube into the front of the nose. The single central incisor tooth may be seen
or palpated through the upper gum. Computed tomography (CT) scans are helpful in diagnosis. An
endocrine specialist should assess pituitary function. The stenosis can be easily drilled wider through a
sublabial incision, and it may be worth stenting the nose for a few weeks. True bony stenosis of the nasal
cavity is rare. Stenosis at the level of the nasopharynx occurs largely as part of a syndrome where the
maxilla is hypoplastic, such as the syndromic craniosynostoses (Crouzon’s, Apert’s, Pfeiffer’s and Saethre
Chotzen syndromes).
NEONATAL RHINITIS

This is by far the most common cause of a snuffly nose in a baby. The diagnosis is made if there is
evidence of nasal congestion with noisy breathing and feeding difficulties, but air can be seen passing
through both sides of the nose (bilateral misting of a cold metal spatula, for example), or nasogastric
tubes can be passed through both sides, and there is no bony narrowing of the nostrils or nasal mass on
examination. The cause is usually unclear, although some cases may be caused by congenital infection
with chlamydia.5 Snuffly babies are very common, and most settle quickly with no specific treatment.
Saline douches may be helpful, as may regular suction with a handheld rubber bulb syringe.
Corticosteroid drops and decongestants are effective but prone to side effects. They should be used very
rarely, very sparingly and for a very short time.

INVESTIGATIONS

Examination with a paediatric flexible laryngoscope can be helpful if secretions are removed with suction
first. If you can pass the scope through the nose and see the larynx, there is no choanal atresia on that
side. The definitive investigation for choanal atresia is an axial CT scan This will reliably confirm or
exclude the diagnosis, and it will also exclude piriform aperture stenosis. The best pictures are achieved
by suctioning secretions from the nose and applying a few drops of decongestant immediately prior to
the scan; otherwise, secretions pool in the nose against the atretic plate, making it difficult to make out.
Good scans give the surgeon useful information about the amount of bone that needs to be removed.

It is vital to arrange a series of investigations, known as a CHARGE screen, for every child with choanal
atresia. Of these, the cardiac assessment is the only one that is urgent enough to need doing before
choanal atresia surgery; the rest can be done in the post-operative period.

MANAGEMENT

An infant who is struggling to breathe through the nose will need something to hold the mouth open. In
many cases, an orogastric feeding tube is enough. If not, a Guedel oropharyngeal airway works very well
as a short-term measure but can cause palatal ulceration if left in more than a couple of days. A
McGovern nipple is a softer alternative that resembles somewhat the teat of a feeding bottle.

Remember that these children are small and vulnerable, and often also medically complex, so the role of
the otolaryngologist is to work as part of a wider team including neonatologists, geneticists, cardiologists
and others. The surgery of choanal atresia involves perforating the central, thin, often membranous part
of the atretic plate, then widening the hole with a drill. It is important to be able to see what you are
doing so as to avoid injury to the palate or skull base. There are various ways of achieving this. Using a
zero-degree Hopkins rod telescope in the nostril together with instruments, as in sinus surgery, is fine in
older children, but space is very limited in neonates. A better view is obtained using a mouth gag on
suspension as for tonsillectomy and a 120° telescope passed through the mouth, looking up behind the
soft palate Instruments passed into the nose will appear to be coming towards you on screen. The palate
should be retracted out of the way with a retraction suture at the base of the uvula.
However, the surgery is done, the choanae will restenose to some extent. It helps to make the choanae
as large as possible by removing plenty of bone laterally and by removing the back of the vomer with
back-biting forceps It is also helpful to warn parents at the outset that some degree of restenosis is
inevitable and to schedule at least one procedure to dilate the choanae with a balloon over the first few
weeks. Regular balloon dilatation should keep the choanae from closing completely and forming a stable
opening. Nasal stents can be bought pre-formed or made from cut endotracheal tubes held in place with
a polypropylene suture encircling the septum. They provide a patent nasal airway for infants during the
early neonatal phase when they are obligate nasal breathers. It is important that they are sized and
placed very carefully: too large and they will cause pressure necrosis of the alar cartilage, too small and
they won’t provide an adequate airway, too tight and they cause necrosis of the columella, too loose and
they cause difficulties with suction. The stents are prone to block with dried secretions, so very frequent
suction and instillation of saline drops are required. Six to eight weeks of stenting is usually enough.