Operative Techniques in Otolaryngology 32 (2021) 31–38

Surgery for congenital choanal atresia

Alice KY Siu, FRCSEd(ORL), FHKCORL, FHKAM(ORL), MMed(Paed),
MBChB a,b, Jacky FW Lo, FRCSEd(ORL), FHKCORL, FHKAM(ORL),
DCH(Syn), MBChB a,b

From the a Kowloon East Cluster, Hospital Authority, Hong Kong

b
Department of Otorhinolaryngology, Head and Neck Surgery, the Chinese University of Hong
Kong, China

KEYWORDS Congenital choanal atresia is rare congenital condition which may cause asphyxia in newborns.
Choanal atresia; Our team from Hong Kong, China, presented our endoscopic, endonasal, stentless approach to this
CHARGE syndrome; condition. In this review we also provided an overview of the evolution of various surgical techniques
Mitomycin; and accessory measures.
Vomer; © 2021 Elsevier Inc. All rights reserved.
Stents;
Nasal obstruction;
Airway obstruction;
Surgical flaps

Introduction niocentesis may have fetal MRI which is highly specific

Choanal atresia is narrowing or obliteration of the pos-
terior nasal aperture resulting in discontinuation of nasal
cavities and the nasopharynx. Unilateral atresia is more
common and may be asymptomatic until few years old.
Bilateral atresia on the contrary may render the affected
infant into respiratory distress: a newborn with cyanotic
spells only resolve with crying. The prevalence of con-
genital choanal atresia is estimated to be between 1:5000
and 1:9000 live births.1 Most series reported female domi-
nance. About 3/4 of choanal atresia is part of a congenital
syndrome. Posterior nasal aperture is hard to visualize on
antenatal ultrasound, though deviated nasal septum, cyst-
like dilated nasal cavities and signs of the underlying syn-
dromes may hint the problem.3 Fetus with abnormal am-
Address reprint requests and correspondence: Department of Otorhi-
nolaryngology, Head and Neck Surgery, the Chinese University of Hong
Kong, China.
E-mail addresses: sky167@ha.org.hk, siukwaiyee@gmail.com
for choanal problem.4 Newborn exams include passage of
a suction catheter to confirm the nasal patency, and it is
how most affected infants are diagnosed nowadays.
Burrow et al2 reviewed 130 cases treated between 1997
and 2007 in Cincinnati Children’s Hospital, about 50%
(n = 62) were unilateral disease. CHARGE (coloboma,
heart defect, atresia choanae, retarded growth, genitouri-
nary abnormalities and ear anomalies) is the most com-
mon syndromal diagnosis. Other syndromal diagnosis in-
cludes diabetic embryopathy, holoprosencephaly, Treacher
Collins syndrome, crainosynostosis spectrum disorders,
mandibulofacial dysostosis. For those who could not be
fitted into diagnostic criteria for a specific syndrome, there
were a high prevalence of airway, brain, and heart abnor-
malities.
There are 4 hypotheses for the cause of choanal atresia1 :
1. Persistence of the foregut buccopharyngeal membrane,
2. Persistence of mesodermal adhesions in the posterior
nasal aperture, 3. Persistence of the nasobuccal membrane

http://doi.org/10.1016/j.otot.2021.01.006
1043-1810/© 2021 Elsevier Inc. All rights reserved.
32
Table 1 Diagnostic criteria for CHARGE (4 major or 3 major + 3 minor)
Major (4C)
1. Ocular coloboma (80%-90%)
2. Choanal atresia (50%-60%)
3. Characteristic ears (80%-100%)
4. Cranial nerve abnormalities
CN 1 hyposmia/anosmia
CN 7 facial palsy (40%)
CN 8 Sensorineural hearing loss
CN 8/9/10 dysphagia, reflux,
velopharyngeal insufficiency (70%-90%)
Figure 1 A CHARGE newborn with cleft palate (arrow),
choanal stenosis with thickened vomer/medialized medial ptey-
gopid plates, and bilateral vestibular organ atresia. (Color version
of figure is available online.)
of Hochstetter, 4. Disruption of neural crest cell migration
and subsequent mesodermal migration.
CHARGE was first described in 1979. Blake et al5 de-
scribed the diagnostic criteria of CHARGE with 4 major or
3 major plus 3 minor characters in 2006. (Table 1) Around
60% of CHARGE cases has autosomal dominant mutation
of chromodomain helicase DNA binding protein 7 (CHD7)
gene located on chromosome 8q12.1-q12.2. CHD7 is in-
volved in the cranial neural crest cells migration during
embryogenesis. CHD7 mutation in CHARGE is usually de
novo with 100% penetrance. Most patients have normal
karyotype, with balanced translocation, rearrangement or
interstitial deletion of 8q. Semaphorin-3E gene (SEMA3E)
on chromosome 7q21.11 has also been reported to be as-
sociated with CHARGE phenotype. Animal study relates
CHD7 regulation to SEMA3E expression, implicating a
common pathological pathway.6 Figure 1 shows the coro-
nal CT of a CHARGE newborn with choanal atresia, cleft
palate and inner ear deformities.
Operative Techniques in Otolaryngology, Vol 32, 2021
Minor
5. Cardiovascular malformations (75%-85%)
6. Genital hypoplasia (50%-60%)
7. Cleft lip/ palate (15%-20%)
8. Tracheoesophageal fistula (15%-20%)
9. Hypothalamo-hypophyseal dysfunction
(70%-80%)
10. Distinctive CHARGE facies (70%-80%)
11. Developmental delay (100%)
The fibroblast growth factor (FGF) pathway also plays
a role in the pathogenesis of choanal atresia. Craniosyn-
stosis syndromes including Antley-Bixler, Apert, Beare-
Stevenson, Crouzon and Pfeiffer have been reported to be
associated with choanal stenosis or atresia; and FGF muta-
tion has been implicated in about 44% of cases. 7 Retinoic
acid, which regulates FGF-8 expression, has been shown to
prevent choanal atresia in diseased mice models.1 In the
early 2000s there were an observation of nonsyndromal
choanal atresia infants born to mother with hyperthyroi-
disim receiving treatment; Thionamides (eg, carbimizole)
is blamed to be the cause. Subsequent animal and human
studies suggested elevated level of thyroid stimulating hor-
mone (TSH) per se has shown to alter the expression of
FGF and its receptors.8 Both the underlying disease and
thionamides may contribute to the congenital disease.
Surgery for choanal atresia
Timing
The timing for surgery for isolated unilateral choanal
atresia may not be much of a concern and can be de-
layed to 6 months or later.9 A diagnosis is usually straight
forward after computer tomography (CT) scan of the
paranasal sinus. However, in syndromal, bilateral choanal
atresia, who presented with neonatal airway emergencies,
the timing for choanal recanalization needs to be evalu-
ated against comorbidities, body size and blood loss. If
the affected infant has pulmonary prematurity or complex
cardiac malformations, early tracheostomy may be a safer
alternative to allow correction of cardiopulmonary condi-
tions and avoid prolonged endotracheal intubation. Chil-
dren with crainosynstosis tend to have thick bony choanal
atresia and small nasal volume, 7 , recanalization surgery
may be delayed due to blood loss concerns and instrument
limitations.
Preoperative assessment
A plain CT scan of the paranasal sinus with 1 mm thick-
ness is usually sufficient for the Preoperative assessment.
Ideally the nasal cavities should be suctioned clean, with
Alice KY Siu and Jacky FW Lo
Figure 2 Coronal CT of a newborn with bilateral choanal atre-
sia, with classical unfused, thick tri-layer vomer; and medialized
lateral nasal wall.
Figure 3 Sagittal CT of the same newborn, in para-median
plane to show the composition and thickness of the atrestic seg-
ment. Measurement and relevance to landmarks are noted. (Color
version of figure is available online.)
a dose of nasal decongestant (such as 0.5% oxymetazolin)
applied before the scan. Both coronal and sagittal views
give ideas on the consistence and thickness of the stenotic
segment (Figures 2 and 3). Choanal stenosis or atresia are
commonly classified into membranous, bony or mixed. CT
findings include narrowing of the posterior nasal choana
with medialization of the lateral nasal wall at the level of
medial pterygoid plate. Posterior vomer is often enlarged
and unfused with a central membranous connection.10 In
crainosynostosis children, the clivus of the sphenoid may
appear low lying, but not in children with CHARGE.11 Ra-
diological studies comparing affected and normal sides of
unilateral choanal atresia showed that the turbinates and the
sinuses are usually not differed.12 The mean width of the
posterior choanal airspace of newborns is 6.7 mm (mea-
33
sured from the lateral nasal wall to vomer, at the level of
maximal transverse diameter of the pyriform aperture). A
measurement less than 3.4 mm is considered stenotic. The
width of vomer is between 2.3 and 3.4mm in children less
than 8 years old, any measurement more than 5.5 mm is
pathological.10
If the infant is sedated for the CT, it is a good idea
to examine the nasal cavities as well with Hopkin rods to
gain an idea on what instruments to use at the time of
surgery. Differential diagnosis of neonatal bilateral nasal
obstruction includes pyriform aperture stenosis and bilat-
eral nasolacrimal duct cysts. Unilateral nasal obstruction
can be a result of nasal foreign body, septal deviation,
antrochoanal poly and nasal tumor.
Recanalization surgery
The approach to choanal atresia recanalization has been
evolving as a result of our increasing understanding of the
developing nose and the advancement of surgical instru-
ments. There is no objective numeral definition of posterior
choanal patency. Outcome of different surgical approaches
are reported in terms of blood loss, operative time, rate
of reoperation and complication rates. Table 2 summa-
rized the contemporary surgical approaches. Transantral
approach had replaced but sometimes included in litera-
tures for the sake of completeness.
Transnasal puncture
Transnasal puncture and dilatation has been a traditional
and safe approach in small infants with membranous or
thin bony atresia. Round tip, cured dilators such as Fearon
dilatators, or female urethral sounds are the instruments of
choice. The baby is anaesthetized and ventilated with oro-
tracheal intubation. The nasopharynx is then visualized us-
ing 120-degree telescope, or microscope with mirror. The
dilator is then passed nasally, following the septum and
nasal floor, and puncture the atretic plate under vision. Af-
ter the first puncture, the dilator size is gradually upgraded
until it reaches the size of the anterior nases. Fearon dilata-
tors have the merit being a hollow tube allowing suction.
Stents fashioned from endotracheal tubes are almost al-
ways used in puncture series. Gujrathi et al13 described
in details their 52 cases transnasal puncture from Toronto,
with the mean age of patients receiving surgery at just 11
days. They stented for 3 months and their revision rate was
3.8%. However, higher failure rates were reported by other
groups: Samadi et al15 reported an average of 4.9 proce-
dures in bilateral atresia infants; Hengerer et al16 reported
50% failure with an average of 3 additional procedures per
case.
Transpalatal resection
Transpalatal approach has gone hand in hand with
transnasal dilatation to cater for cases with thick bony atre-
sia. The palates are exposed using Boyle Davis or Dingman
34 Operative Techniques in Otolaryngology, Vol 32, 2021

Table 2 Different approaches of choanal atresia surgery.

Approach
Transnasal puncture
Transpalatal resection
Transseptal resection
Endoscopic Endonasal
resection
How
Fearon dilatators or urethral sounds through
nostrils to dilate the posterior choana. Visualize
from nasopharynx using 120-degree telescope or
mirror.
Transoral surgery with palatal flap based on
greater palatine artery, open resection of the
atretic plate and vomer.
Endonasal or sublabial incision and enter
submucosal plane of nasal septum, resect
posterior vomer and atretic plate with punches or
drill. Excise or reposition the atretic pouch
mucosa as flaps.
Without flap – endoscopic removal of atretic
plate and posterior vomer using endoscopic bone
cutting forceps and drills
With flap – preserve the atretic pouch mucosa
prior to bone work and reposition as flaps.
Remarks
Easy for membranous or thin bony atresia.
High recurrence and re-operation rate.
Stent or dilators left in situ postoperatively for
weeks to months.
Definitive or salvage surgery for thick atresia.
High successful rate.
Risks of palatal fistula, transfusion, possible
midface deformity.
High successful rate in smaller case series.
Narrow operative field for infants.
High successful rate compared to conventional
transnasal dilatation, even better with flaps.
Straight forward but dependent on availability of
instruments.

retractors as in the case of palatoplasty. The hard palate
is then exposed using a median or a U-shaped incision,
preserving the greater palatine neurovascular bundles. The
atretic segment, which arises from the posterior part of hor-
izontal plate of palatine bone, posterior nasal spine, vomer
and medial pterygoid plates are resected with rongeurs or
cutting drills. The palate is then closed with absorbable
sutures. Successful rate (patency more than a year later)
as a single surgery is more than 80%.16 Blood loss and
palatal fistula are occasional complications. A major con-
cern of this technique originates from a report by Freng
et al17 who compared 2 groups of Norwegian children with
choanal atresia: 35 with transpalatal resection, and 20 with
no surgery. He reported 52% occurrence of crossbite in
those with surgery (4% in nonoperated group), affected
individuals have a significant reduction of the width of
the upper dental arch and the maxilla. Contemporary sur-
geons almost never apply this technique as the first line
of surgery for children with choanal atresia. A few con-
sider such approach when the noses are too small to admit
endoscope and instruments.9
Trans-septal Resection
Trans-septal approach was described in the late 80s as
an alternative to the aforementioned traditional approaches.
Trans-septal approach involves elevating mucoperichon-
drial/mucoperiosteial flaps of the septum, as in septoplasty,
and tackles the atretic structures submucosally using mi-
croscopes and nasal speculums. Krespi et al18 utilized a
sublabial incision, while McIntosh et al19 adopted a Kil-
lian incision at the anteirorinferior septum extended to the
anterior nasal floor. This approach gives a novel access to
the posterior vomer but the mucosal flap limits the access
to the medial pterygoid plates. With the advance of endo-
scopic instruments, Wormald et al 14 reported a make-over
of this approach with the assistance of endoscope in 2016.
They elevated the mucoperichondrial flaps bilaterally using
transfixion incision, then develop the submucosal planes
all the way to the atresia segment lifting a pouch of pos-
terior nasal mucosa. The posterior vomer, palatine crests
and medial pterygoid plates are removed using Blakesley
forceps and 1mm diamond blur. They then make cuts on
the blind pouch mucosa to fashion a pair of swing-door
flaps. The flaps are secured with fibrin glue and they do
not use stents. Their result is impressive with no revision
surgery necessary, though only 2 of the 17 reported cases
were neonates.
Endoscopic endonasal approach
With the advancement of high definition cameras and
small caliber endoscopes, endoscopic endonasal resection
has now become the standard for primary choanal atresia
recanalization. With blooming of literatures since 1990s,
this straight forward approach has shown replicability and
earned confidence from surgeons worldwide. Cedin et al20
published a Cochrane review in 2012, endoscopic approach
shown a slight reduction in the absolute risk for not requir-
ing reoperation (0.75, compared to 0.84 for nonendoscopic
approaches). Yet the use of endoscope allows meticulous
dissection in small operative fields, and leads to the evo-
lution of mucosal preservation techniques. The absolute
risk for not requiring reoperation of endoscopic cases with
flaps is down to 0.09 (compared to 0.3 for endoscopic ap-
proach without flaps). Different flaps have been described.
Figure 4 summarized flap designs by various authors, the
list is not exclusive.
Alice KY Siu and Jacky FW Lo
Figure 4 A summary of flap designs by various authors.
How we do it
Preparation
There is no age limitation for endoscopic choanal atre-
sia surgery. If bilateral choanal atresia is considered to
be the sole cause of respiratory distress, recanalization
can be offered to babies as light as 2.5 kg. Instrument
size is the main concern. Ear endoscopes (3 mm width,
11 or 14 cm length, 0 degree) used in endoscopic ear
surgery provides superb view for neonatal noses. Nasoen-
doscope (4 mm width, 18 cm length, 0 and 30 degree)
can be used for older children. Pediatric functional en-
doscopic sinus surgery (FESS) sets with cutting Blake-
35
ley forceps, straight Stammberger mushroom punches and
backbites forceps are ideal for children. In neonates, how-
ever, tympanoplasty sets are often more practical. Drills
are chosen again based on the limitation of nasal cavity
sizes. Otological drills such as the Medtronic Visao system
or the Skeeter drills (Medtronic Xomed Inc., Jacksonville,
Florida, USA) offer precise removal of atretic fragments.
For neonatal cases, the Viaso 1.5 mm curved fluted cutting
burs (31151598E) or the 2 mm curved fine diamond burs
(31182090E) are preferred, for these burs have slim shafts
and end-on drilling configuration. Other otological drills
can also be used, minding to protect the nostril skins with
thin silicone sheets or aluminum foils.
36 Operative Techniques in Otolaryngology, Vol 32, 2021

Intraoperative
The child is orotracheal intubated, preferably with oral-
Rae tube. In unilateral cases, the patent side gives a good
idea on the height and width of the posterior choana, thus
surgery is usually straight forward. In bilateral cases, how-
ever, perspectives are taken from the nasopharynx to es-
timate the dimension so as not to violate the skull base
which is commonly the cause of major bleeding. 120 tele-
scope viewing from the oropharynx can serve this purpose.
The noses are prepped with 0.5% oxymetazoline or 1 to
100,000 adrenaline soaked neurosurgical petties. Cuts are
made onto the atretic pouch mucosa with sharp elevators
(Duckbills or round knife), and the mucosa is reflected
using curved curettes (House curettes). In infants the “I”
incision are more feasible to construct fold-over circum-
ferential flaps. In older children, a cross over flap can be
used. The difficulty of mucosa preservation attempts is that
the mucosa often obscures the field for bone work, and
the small but continuous bleeding becomes disturbing in
infants’ small confined nasal cavities.

Bone work
After elevation of the mucosa, bone work can be started at
the posteroinferior vomer. The septum and nasal floor are
the most reliable landmarks. In unilateral case, it is possi-
ble to use backbite forceps. In bilateral cases, sharp Freer
elevators are used to make curvilinear cuts cracking the
vomer. The vomer in choanal atresia children are layered Figure 5 Endoscopic endonasal recanalization Surgery in a uni-
lateral choanal atresia case.
with a membrane sandwiched between the 2 bony plates.
(a) Preoperative picture showing right choanal atresia. (b) Lateral
Once the posteroinferior vomer plates on both sides are based mucoperiosteal flap raised. (c) Confirmation of atresia po-
removed, a posterior choanal airway is established. From sition with Transoral illumination using a 120-degree endoscope.
there the circumference can be enlarged by chipping the (d) Burring down of posteroinferior vomer with 2mm diamond
vomer superiorly, then burring the vomer alae and the me- burr. (e) Removal of vomer with Blakesley forceps. (f) Part of
dial pterygoid plates. The end of middle turbinate gives us vomer being removed. (g) Trimming and reposition of mucope-
an idea of the lateral and superior limits of the choana. riosteal flap. (h) Recanalized posterior choanae.
The sphenoid ostia are commonly used as the reference of
skull base – they certainly mark the body of the sphenoid,
breath nasally and discharge home in 2 days with normal
yet in infants there is no validated measurements taking
saline drops and a week course of empirical antibiotics.
reference from the roof of choana to the sphenoid ostium.
Many surgeons use nasal steroid sprays and proton pump
A preoperative detailed study of the CT helps the estima-
inhibitors postoperatively. 9
tion. Then it comes to the point whether CT navigation
is useful? Navigation systems are surely of value in older
children and revision cases, though in infants the operative
field would be too small to utilize the navigation-enabled Discussion: debate and controversies
instruments, and the accuracy for 0.5 mm measurements
was doubtful. Figure 5 illustrated one of our cases. Stents

Postoperative care
The mucosal flaps are repositioned after the bone work,
and packed down with hemostasis petties. Often this is
enough to stick the flap down without fibrin glue nor nasal
packing. Many authors use stents and mitomycin C. In our
unit, stents are no longer used as they do not seem to alter
the reoperation rates. Mitomycin C use as local applica-
tion to prevent re-stenosis is off-labeled, we now change
to smear the wound with a mixture of 1% momethasone
plus 1% gentamycin ointment, as an orthodox practice bor-
rowed from our airway surgery. The babies are left to
Many articles had detailed the craft of tailor making bi-
lateral nasal stents from noncuffed endotracheal tubes.13 , 24
Postoperative stentings have been a tradition since the di-
latation/transpalatal era. There is no consensus on how
long a pair of stents should be kept in situ, though many
reported a duration of 2 to 8 weeks. Few series reported
the stents to be removed in 24-48 hours, these nasopha-
ryngeal tubes serve more as a transient airway. From the
2012 Cedin et al 20 Cochrane Library review, the absolute
risk of not requiring reoperation with or without stents
is the same (0.81/0.81). A more recent systemic review
Alice KY Siu and Jacky FW Lo
by Strychowsky et al30 has a similar conclusion: stented
and unstented series have similar reoperation rates (65%
in stented, 64% in un-stented patients are successful with
first surgery). The stented patients had a higher complica-
tion rate including alar injury, vestibular stenosis, columela
tear and stent dislodgement or blockage.
Mitomycin C
Mitomycin C is an antibiotic and chemotherapy agent
isolated from the Streptomuces caespitosus fungus. It is
used widely in different surgical fields to reduce scar,
for its antiproliferative property which inhibits the forma-
tion of progenitor cells to myofibroblasts. When used in
choanal atresia surgery, it is applied by packing petties
soaked with 0.4mg/mL dilution at the end of the proce-
dure. The evidence of its benefit has been questionable,
with only few cases series showing effects, all coupled
with the use of stents.1 With the possible risk of bone mar-
row toxicity and oncogenicity later in life, it has largely
fell out of favor.
How much vomer is safe to remove?
Posterior crossbite had been the holdback for
transpalatal approach (removal of vomer and horizontal
plate of palatine bone), and it gives rise to one question:
how much bone removal will affect the midface growth?
Removing the posteroinferior vomer has been an effec-
tive, safe step in choanal atresia surgery. It is tempting
to create a large posterior septal window as an alterna-
tive to lateral bone work. Yet aggressive removal of the
vomer should be restrained. The palatine bone has one
ossification center, in the perpendicular, but not the hor-
izontal, plate, while the vomer ossifies from the laminae
on both sides of a median plate of cartilage.31 This ossi-
fication patterns continues throughout childhood. It is rea-
sonable to believe that vomer resection contributes more
in the deformity observed in long-term patients who re-
ceived transpalatal surgery. There is no consensus on how
much vomer is safe to be removed by what age, nor an-
imal studies giving cues on the degree of acceptable re-
section. To preserve the growth potential, a circumferen-
tial approach to establish the choana (removing medial
pterygoid plates) rather than overzealous resection of the
vomer should be advocated. The posterior nasal spine and
transverse palatine suture are to be respected in every
incidence.
Comeback of transnasal dilatation: Balloons
With the success of re-visiting dilatation technique in
the care of subglottic stenosis using high-pressure, low
compliance balloons, new series have come up with at-
tempts to utilize balloons in the treatment of choanal atre-
sia. Riepl et al 32 first published in 2013 with a series of 6
neonates with primary transnasal balloon dilatation. They
37
coupled the use of bougies and stents, and averaged with
3.8 procedures per case. De Vincentis et al33 from Rome
utilized the balloons as a secondary measure to stabilized
their results in the early postoperative periods in their en-
doscopic endonasal stentless laser resection. They reported
an 85.7% successful rate with an average dilatation of 3.5
times. Both teams used the Relieva Solo Pro Sinus Balloon
Catheter by Acclarent (Menlo Park, California, USA) with
a diameter of 5-10mm (depends on age of the patients) and
a length of 16 mm. The balloon catheter was inflated twice
for 5 min at 8 Bar using the Acclarent Balloon Inflation
Device. Contrary to the laryngeal framework which con-
sists of solely cartilage, we shall evaluate the outcome of
balloon dilatation on the bony posterior choana carefully.
Author roles
Dr Alice KY Siu – manuscript preparation, tables and
radiology images.
Dr Jacky FW Lo – manuscript advice, operative photos
and description.
Disclosure
No disclosure.
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