Choanal atresia is the developmental failure of the
nasal cavity to communicate with the nasopharynx.
Choanal atresia is a relatively rare congenital
anomaly and occurs in approximately 1 in 5000 to
8000 live births, with a female to male ratio of 2:1 [1,2].
Generally, 65% to 75% of patients with choanal
atresia are unilateral, whereas the rest are bilateral3.
About 30% are pure bony, whereas 70% are mixed
bony-membranous [2,4]. No patients were found to
have a purely membranous atresia .
Fifty percent of all patients with choanal atresia
and up to 75% of patients with bilateral disease have
other associated congenital anomalies . Bilateral
choanal atresia is very rare in adults [6-8].
Bilateral choanal atresia will present as an
acute respiratory emergency at birth as newborns
are obligate nasal breathers. Placement of
an appropriately sized oral airway usually resolves
the distress. However, surgical correction is usually
necessary early in life .
An 18 year old female presented to us with the
complaints of total bilateral nasal blockage, bilateral
nasal discharge and anosmia since birth. History
revealed cyanotic attacks at rest and during feeding
in childhood which were relieved on crying. Parents
gave the history of evaluation of her nasal blockage
and radiological investigation in neonatal period
when she was diagnosed for bilateral complete choanal atresia and surgery was advised. However,
parents refused to choose surgery at that time.
Patient survived her neonatal period without any
active management. She did not have remarkable
complaints during early childhood or adolescence.
On clinical examination, thick tenacious secretion
was present in both nostrils without any evidence
of bubbling of nasal secretions with respirations.
There was lack of movement of cotton wool wick on
holding it in front of nostrils. No misting was seen
on cold spatula test. Nasal endoscopy with 0 degree
telescope revealed bilateral complete choanal
atresia (Figures 1 and 2). Definite diagnosis of the
condition was made on CT PNS, which showed a
mixed bony membranous type of choanal atresia
(Figures 3 and 4). There was no other associated
Figure 1: Pre-operative endoscopic image of right
side complete choanal atresia.
Figure 2: Pre-operative endoscopic image of left
side complete choanal atresia.
Figure 3: CT PNS axial view showing bilateral bony
membranous choanal atresia. Arrow pointing
towards both side atretic plates.
Figure 4: CT PNS sagittal view showing atretic plate in
posterior choana. Arrow points a small membranous
area of atresia.
Figure 4: CT PNS sagittal view showing atretic
plate in posterior choana. Arrow points a small
membranous area of atresia.
Patient underwent a trans-nasal endoscopic
approach using a zero degree endoscope, under
general anaesthesia with endotracheal intubation.
Bilateral nasal cavities were full of thick secretions,
after clearing the secretions, infiltration was given
with 2% lignocaine with adrenaline on the mucosa
over the anterior face of atretic area, lateral wall and
posterior portion of septum. A T-shaped mucosal
incision was taken with a pointed cautery tip, with
the horizontal limb of the T concave upwards and
vertical limb more towards septum. Mucosal flaps
were raised superiorly, laterally and small flap
medially (Figure 5).
Figure 5: Diagrammatic representation of T-shaped incision over mucosa of Right side choanal atresia.
After lifting the flaps, bony atretic plate was
visualised and only a small area in the centre of the
atresia was membranous. The bone from the atretic
plate, lateral nasal wall (medial aspect of pterygoid
plate), and lateral portion of the posterior septum
was drilled by microdebrider burr drill 15 degree,
3 mm rough diamond nasal burr. On the floor the
bony atretic plate was drilled till the edge of hard
palate. The mucosal flaps were replaced where the
bony atresia was drilled so that the nasal cavity is
in smooth continuity with the nasopharynx and
there was no exposed bone. Same procedure was
repeated on the other side. No stent or mitomycin
C was applied as the nasal cavities were wide and
there was no exposed bone left after replacing the
raised mucosal flaps. The posterior end of septum
and soft palate were normal. Bilateral glove finger
nasal packs to make sure it’s easy removal were
placed under endoscopic guidance taking care of
mucosal flaps (Figure 6). Packs were removed after
Figure 6: Nasal pack covered with cut finger glove secured with ties and few perforations made in the glove finger.
Postoperative recovery was uneventful. After
pack removal patient complains of inability to
speak loudly and nasal twang to voice, which was recovered in two days. Patient was adapted to
breathing from nose within seven days. However,
Sense of smell was absent. After endoscopic nasal
cleaning, patient was taught to do nasal wash with
normal saline after 15 days. She had been reviewed
by endoscopy weekly for one month and thereafter,
once in a month for 3 months. At the end of one year
and two months bilateral choanae are as wide as it
was intra operatively and well healed with normal
nasal mucosa (Figures 7,8).
Figure 7: Endoscopic image of left side wide patent
choana after one year of surgery.
Figure 8: Endoscopic image of right side wide
patent choana after one year of surgery.
There have been several theories regarding the
embryogenesis of choanal atresia, but it is generally
thought to be secondary to persistence of either
the nasobuccal membrane of Hochstetter or the
buccopharyngeal membrane from the foregut. This
membrane normally ruptures between the fifth
and sixth weeks of gestation to produce choanae.
Failure of this membrane to rupture causes atresia
of choanae [2,9].
Since a new-born child is an obligate nasal
breather, respiratory distress and collapse occurs
in patients with bilateral congenital choanal atresia
immediately after birth. If not promptly recognized, it can lead to severe asphyxia and death. They
usually present with similar pattern of cyclic cyanosis
which is relieved by crying. Feeding difficulties may
lead to failure to thrive. Most patients with bilateral
congenital choanal atresia are detected in neonatal
period. However, it can be diagnosed in adults with
long-term bilateral nasal obstruction with nasal
discharge .Very rarely neonates with complete
Bilateral Choanal Atresia can gain the ability to
breathe through the mouth .
Immediate management of bilateral choanal
atresia involves training the infant to breathe through
the mouth with the aid of an indwelling oral appliance
such as a McGovern nipple or an oropharyngeal
airway. If there is severe respiratory distress and
airway cannot be established by endotracheal
intubation, an emergency tracheotomy should be
performed until further evaluation and treatment
can be established. Nevertheless, surgical correction
is usually necessary early in life .
Tests such as CT of the paranasal sinuses and
nasal endoscopy are essential in diagnosis and
therapeutic plan –to know the extent, location and
distinctness of atresia bone, membrane, and mixed
(bony membranous) .
Better instrumentation in endoscopic sinus surgery
and advances in CT scans have made the trans-nasal
repair the most popular method. Nasal endoscopy
is beneficial in the management of choanal atresia
since it helps to confirm the diagnosis, characterize
the extent of lateral nasal wall contribution to the
stenosis or atresia, evaluate the composition of
the atresia (bony and/or membranous), and guide
There are several techniques: microscopic and
endoscopic trans-nasal, trans-palatal, trans-septal,
and dilations trans-antral intranasal [13-17].
A shift has occurred in the surgical philosophy
over the last few decades away from trans-palatal
and toward trans-nasal surgery5. The main advantage
of the trans-nasal procedure is minimally invasive,
quick (avoiding the need for prolonged anaesthetic
agents), less traumatic with minimal blood loss, and
provides excellent visualization and the ability to
perform exact surgery on patients of all ages [2,18].
Richardson and Osguthorpe has compared
transnasal repair with trans-palatal repair in 37 with
congenital atresia or severe stenosis of both posterior
nasal choanae. They found that trans-nasal repair
allowed correction with minimal blood loss and
without facial growth or occlusal abnormalities .
Paulo Tinoco et al in 2009, has reported a case of
34 year male managed with mucosal flaps without
stent placement .
In our case also, we preferred to use mucosal
flaps to cover the surgical area so as to prevent
restenosis and to avoid stent placement. Stent and Mitomycin C were not used as the exposed bone was
completely covered with the raised mucosal flaps.
With the use of stent, there is a fear of foreign body
reaction, skin necrosis in the columella, nasal alar
erosion and requirement of intense antimicrobial
therapy. We bypassed these by not using stents. At
the end of one year bilateral choana are wide open
with the surrounding healed mucosa.
Despite the nasal blockage was removed, and
patient was breathing through nose, she continued
to have anosmia. Gross-Isseroff et al. have studied
the postoperative olfactory function at their case
series of four of which 3 of them were Bilateral
Choanal Atresia, and concluded that bilateral cases
sustained olfactory deficit while unilateral case had
the normal olfactory function .
Bilateral complete congenital choanal atresia is
a life threatening condition with early diagnosis in
new born due to its symptoms and high degree of
suspicion by the paediatrician. It is very rarely found
in adults. Our patient survived in neonatal period
with bilateral complete congenital choanal atresia,
without any management.
Careful review of the computed tomography scan
and experience with endoscopic nasal surgery makes
the trans-nasal endoscopic treatment a safe and
effective approach for managing bilateral choanal
atresia. Stenting and Mitomycin C are not necessary
when the exposed bone is completely covered by
raising adequate mucosal flaps. Management of
choanal atresia with mucosal flaps alone has shown
good results in our patient.
- Theogaraj SD, Hoehn JG, Hagan KF (1983) Practical management of congenital choanal atresia. Plast Reconstr Surg 72: 634-42.
- Paraya A, Choakchai M (2009) Choanal atresia. J Med Assoc Thai 92: 699-706
- Dobrowski JM, Grundfast KM, Rosenbaum KN, Zajtchuk JT (1985) Otorhinolaryngic manifestations of CHARGE association. Otolaryngol Head Neck Surg 93: 798-803.
- Brown OE, Pownell P, Manning SC (1996) Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope 106: 97-101.
- Elluru RG & Wootten CT (2010) Congenital malformation of the nose. Cummings Otolaryngology Head and Neck Surgery. 5th ed. Philadelphia: Mosby Elsevier; page 2694
- Panda NK, Simhadri S, Ghosh S (2004) Bilateral choanal atresia in an adult: is it compatible with life? J Laryngol Otol 118: 244-5.
- El-Sawy H, Siddiq MA, Anbarasu A (2006) Bilateral choanal atresia and paranasal sinus hypoplasia in an adult patient with hypogammaglobulinaemia. Eur Arch Otorhinolaryngol, 263: 1136-8.
- Yasar H, Ozkul MH (2007) Bilateral congenital choanal atresia in a 51- year-old woman. Am J Rhinol 21: 716-8.
- Dunham ME, Miller RP (1992) Bilateral choanal atresia associated with malformation of the anterior skull base: embryogenesis and clinical implications. Ann Otol Rhinol Laryngol 101: 916-9.
- Voegels RL, Chung D, Lessa MM, Lorenzetti FT, Goto EY, Butugan O (2002) Bilateral congenital choanal atresia in a 13-year-old patient. Int J Pediatr Otorhinolaryngol 65: 53-7
- Baker DC Jr, Waltner JG, Novick W (1960) Posterior choanal atresia. Ann Otol Rhinol Laryngol 69: 805-9
- Schweinfurth JM (2002) Image guidance-assisted repair of bilateral choanal atresia. Laryngoscope., 112: 2096-8.
- Lazar RH, Younes RT (1995) Transnasal repair of choanal atresia using telescopes. Arch Otolaryngol Head Neck Surg., 121: 517-20.
- Samadi DS, Shah UK, Handler SD (2003) Choanal atresia: A twenty-year review of medical comorbiditied and surgical outcomes. Laryngoscope 113: 254-8.
- Vogels RL, Chung D, Lessa MM, Lorenzetti FTM, Goto EY, Butugan O (2002) Bilateral congenital choanal atresia in a 13-year-old patient. Int J Pediatr Otorhinolaryngol. 65: 53-7.
- Josephson GD, Vickery CL, Giles WC, Gross CW (1998) Transnasal endoscopic repair of congenital choanal atresia. Arch Otolaryngol Head Neck Surg 124: 537-40.
- Cedin AC, Rocha JrFP, Deppermann MB, Manzano PAM, Murao M, Shimuta AS (2002) Transnasal endoscopic surgery of choanal atresia without the use of stents. Laryngoscope., 112: 750-52.
- Stankiewicz JA (1990) The endoscopic repair of choanal atresia. Otolaryngol Head Neck Surg 103: 931-7.
- Richardson MA, Osguthorpe JD (1988) Surgical management of choanal atresia. Laryngoscope; 98: 915-8.
- Paulo T, José Carlos Oliveira Pereira, Rodolfo Caldas Lourenço Filho. Bilateral Choanal Atresia in 34 Year-old Patients, Intl. Arch. Otorhinolaryngol., São Paulo - Brazil, v.14, n.4, p. 481-484, Oct/Nov/ December – 2010.
- Gross-Isseroff R, Ophir D, Marshak G, Ganchrow JR, Beizer M, Lancet D (1989) Olfactory function following late repair of choanalatresia. Laryngoscope 99: 1165-6.