I THE AMERICAN JOURNAL OF ROENTGENOLOGY RADIUM THERAPY AND NUCLEAR MEDICINE VOL. 112 MAY, i7i No. I POSTERIOR CHOANAL ATRESIA* By HUGH J. WILLIAMS, M.D. ST. PAUL, MINNESOTA T HERE is scant reference to posterior choanal atresia in the roentgenologic literature, and a recent review of the 2 major radiologic journals published in this country yielded no articles on this subject since that of Whitehouse and Holt in 1952.12 The purpose of this communication is twofold. The first is to review the clinical features in 19 cases of this uncommon anomaly, and to emphasize the importance of considering the bilateral form of this disease in the differential diagnosis of severe respiratory distress in the newborn. The second purpose is to propose a clinical classification of bilateral choanal atresia which the author feels is more meaningful than the hitherto traditional anatomic classification. Failure to appreciate that bilateral choanal atresia often presents as an emer- gency in the newborn period can be disas- trous and result in tragic death from asphyxia. While the initial diagnosis of bilateral choanal atresia is usually sus- pected and confirmed by the obstetrician or pediatrician, occasionally the radiologist may be in a position to suggest the possi- bility of this diagnosis. In other suspected *From the children’s Hospital, Inc., St. Paul, Minnesota. cases he can readily confirm the diagnosis roen tgenographicallv. Schwartz and Isaacs1#{176} found that the earliest published description of this con- dition was by R#{246}derer in Gottingen, Ger- manv, around 1755. The first corrective surgery was performed in 1851 by Emmert who punctured the obstruction with a trocar.2 ETIOLOGY AND INCIDENCE Most embryologists believe that pos- terior choanal atresia results from a failure of rupture between the 35th and 38th day of fetal life of the partition which separates the bucconasal or buccopharyngeal mem- branes. The resultant choanal atresia may be unilateral or bilateral, bony or mem- branous, complete or incomplete. In over 90 per cent of cases the obstruction is bony, while in the remainder it is membranous.9 The bony type of atresia is commonly located 1-2 mm. anterior to the posterior edge of the hard palate, and the osseous septum varies in thickness from i to JO mm. In the membranous form of choanal atresia the obstruction usually occurs fur- ther posteriorly. In approximately one- third of cases the atresia is bilateral. Downloaded from www.ajronline.org by 36.83.104.88 on 05/02/14 from IP address 36.83.104.88. Copyright ARRS. For personal use only; all rights reserved
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I
THE AMERICAN JOURNAL
OF ROENTGENOLOGYRADIUM THERAPY AND
NUCLEAR MEDICINE
VOL. 112 MAY, i�7i No. I
POSTERIOR CHOANAL ATRESIA*
By HUGH J. WILLIAMS, M.D.ST. PAUL, MINNESOTA
T HERE is scant reference to posteriorchoanal atresia in the roentgenologic
literature, and a recent review of the 2
major radiologic journals published in this
country yielded no articles on this subject
since that of Whitehouse and Holt in1952.12
The purpose of this communication is
twofold. The first is to review the clinicalfeatures in 19 cases of this uncommonanomaly, and to emphasize the importance
of considering the bilateral form of this
disease in the differential diagnosis ofsevere respiratory distress in the newborn.The second purpose is to propose a clinicalclassification of bilateral choanal atresia
which the author feels is more meaningful
than the hitherto traditional anatomicclassification.
Failure to appreciate that bilateral
choanal atresia often presents as an emer-gency in the newborn period can be disas-
trous and result in tragic death fromasphyxia. While the initial diagnosis of
bilateral choanal atresia is usually sus-
pected and confirmed by the obstetrician orpediatrician, occasionally the radiologist
may be in a position to suggest the possi-
bility of this diagnosis. In other suspected
*From the children’s Hospital, Inc., St. Paul, Minnesota.
cases he can readily confirm the diagnosisroen tgenographicallv.
Schwartz and Isaacs1#{176} found that the
earliest published description of this con-
dition was by R#{246}derer in Gottingen, Ger-manv, around 1755. The first corrective
surgery was performed in 1851 by Emmert
who punctured the obstruction with a
trocar.2
ETIOLOGY AND INCIDENCE
Most embryologists believe that pos-
terior choanal atresia results from a failureof rupture between the 35th and 38th day
of fetal life of the partition which separates
the bucconasal or buccopharyngeal mem-branes. The resultant choanal atresia may
be unilateral or bilateral, bony or mem-
branous, complete or incomplete. In over
90 per cent of cases the obstruction is bony,while in the remainder it is membranous.9The bony type of atresia is commonly
located 1-2 mm. anterior to the posterioredge of the hard palate, and the osseousseptum varies in thickness from i to JO
mm. In the membranous form of choanalatresia the obstruction usually occurs fur-
ther posteriorly. In approximately one-
third of cases the atresia is bilateral.
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2 Hugh j. Williams MAY, 1971
Associated abnormalities of the surround-
ing bony structures, including a higharched hard palate, and an increased thick-
ness of the vomer, nasal septum and the
lateral nasal structures are often present,thereby reducing the diameters of thenasal fossa and the nasophar��nx. Thefrequent association of other congenital
anomalies has been well documented4 and a
familial incidence has been noted by some
authors.13 In the large series of Flake and
Ferguson,3 there was a twofold predomi-
nance of females over males. In patientswith unilateral choanal atresia the occur-rence was predominantly right-sided (over2: i).
SYM PTOMS
Ronaldson in 1 88 1 � recorded the first
clinical description of choanal atresia and
it has never been surpassed in clarity.Despite the classic description of bi-
lateral choanal atresia some 89 years ago,
many physicians are still unaware of the
lethal potential of this uncommon anomal��of the newborn. It is not generally appreci-ated that the newborn infant instinctively
breathes only through the nose, and thatoropharyngeal breathing is an acquired
function, which normally takes several daysto weeks to learn. This aversion to mouthbreathing has been attributed to an evolu-
tionary need for air currents to pass over
the olfactory area.7 The anatomic cross sec-tions of the infant skull performed byMoss5 demonstrate that the relatively
higher position of the cervical viscera
during infancy impedes access of air from
the mouth to the lower airway and that thenose is the best avenue for respiration. It
may be that this structural basis forobligatory nasal breathing in the newborn
plays a protective role against the aspira-tion of food. Because most neonates are
obligate nasal breathers, bilateral choanalatresia usually precipitates respiratory dis-
tress immediately after birth. This anom-aly, therefore, must be included in the
differential diagnosis of any newborn infant
suffering from respiratory distress. Failure
to consider this life threatening entity,establish the correct diagnosis, and
promptly institute appropriate therapy is
1.1 ndou b tedly responsi ble for an u nde ter-mined number of neonatal deaths fromasphyxia annually in the U.S.A. In those
infants who escape diagnosis in the firsthours of life because they rapidly learn
mou th-breathi ng, respi ratorv distress is
su ally precipitated subsequently by
attempts at feeding, since the ability to
simultaneously breathe and suck is seldomachieved until age 3-6 weeks.
All infants with bilateral choanal atresia
develop a thick, glairy, mucoid dischargefrom the nostrils. The copious, leathery
mucus may occasionally become blood
tinged but secondary infection seldom
supervenes. The external nares and upper
lip may become excoriated from the con-
stant mucus discharge from the nose.
Affected patients are unable to blow the
nose and the voice develops a nasal
quality. Anosmia and diminished taste are
present. The true diagnosis in those few
patients who do not manifest neonatal
respirator\� distress is often delayed, and
the nasal obstruction is erroneously attrib-
uted to allergy, rhinitis, deviate(l nasal
septum or enlarged adenoids.
The recent proposal � Shaw,” that
bilateral nasal obstruction from trivial
upper respiratory infection in the smallsleeping infant who does not instinctively
or voluntarily breathe through his mouth
is a significant factor in sudden unexpected
death of infancy, is an attractive one. More
than 15,000 infants under 6 months of age
die annually from this perplexing problem
and it constitutes the greatest cause of
mortality during the first year after the
neonatal period (2 to 3/1,000 live births).
NEW CLINICAL CLASSIFICATION
A new clinical classification of bilateralchoanal atresia, based on the rapidity with
which the newborn learns to both breathe
orally and to co-ordinate mouth breathing
and sucking, is proposed by the author
(Table II). It is hoped that this classifica-
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VOL. 112, No. i Posterior Choanal Atresia 3
TABLE I
SUMMARY OF CASES
Case Sex Location of Atresia Typ e (bilateral cases) Associated Anomalies
i. B.G.H. F Bilateral I (Fig. I, A and B)2. D.M. F Bilateral II3. G.J. NI Bilateral I Esophageal atresia
with fistula�. R.A.L. NI Bilateral III (Fig. 3, ii and B)�. A.L. I’ Left (Fig. �, A and B)6. J.M.C. NI Right (Fig. 4, 4 and B) Omphalocele7. B.G.l). F Bilateral Ill8. J.H. F Left Ventricular septal
defect and congenitaldeformity of externalright ear
�. M.H. Ni Right
io. L.C. F Bilateral II (Fig. 2, A and B)ii. M.W. M Right
12. G.W. M Left13. K.S. F’ Right14. L.S.J. F Left Mongolismi�. J.A. M Bilateral Ii6. C.D. F Right17. T.M. F Bilateral Ii8. B.W. I’ Bilateral I19. R.S. F Right
tion, by stressing the clinical rather thanthe anatomic aspects of the disease, willlead to a better understanding of thevarious manifestations of bilateral choanalatresia and hence to an earlier diagnosis.
Type I. These infants lack the ability torapidly learn the technique of oropharyn-
geal breathing and immediately develop air
hunger, cyanosis and chest retraction(Cases I, and 3). Type I comprises the
largest group (Table i) and these infantswill die from asphyxia unless an artificial
airway is immediately established. Pro-
longed use of such an airway is often re-
quired, but in many cases the infant can be
gradually weaned from it over a period of
weeks. These infants frequently cannot co-
ordinate mouth breathing and sucking and
attempts at feeding provoke respiratory
distress. Gavage feedings are usually neces-
sary in such infants and must be continued
after oropharyngeal breathing has been
mastered, until such time as the necessary
co-ordination between mouth breathing
and sucking develops.
Type II. This smaller group of infants
learns mouth breathing more rapidly afterbirth than do infants of Type i. Theirrespiratory distress is cyclic and less severe,
necessitating only transient or intermittent
use of an artificial airway (Case 2). How-
ever, even after the technique of oropharyn-geal breathing has been mastered (hours to
days), respiratory distress may still be
provoked by attempts at feeding. The
obviously ravenous infant will suckle only
spasmodically because the nipple causes
temporary occlusion of the oral airway.
Until such infants learn to co-ordinate oral
breathing and sucking (usually by the age
3-6 weeks), gavage feedings should be em-
ployed. Persistent, premature attempts to
orally feed such infants prior to develop-
ment of this co-ordination will result in
choking, cyanotic spells and aspiration
pneumonitis.
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No. ofcases
Type 1 5
Type II
Type III
Type IV 0
Time required
to mastermouth
breathing
Prolonged(weeks)
Hours to days
Immediate to
a few hours
None
Time required
to developco-ordinationbetween oral
breathing and
sucking
3-6 weeks
usually
�-6 weeks
usually
3-6 weeks
None
Airwayrequirements
Immediate,
prolonged
Intermittent
(hours to
weeks)
None
Type of
neonatalrespiratory
distress
Immediate,
severe andprolonged;
usually ag-
gravated by
feedings
Less severeand intermit-
tent; occurs
at rest and
usually dur-
ing feedings
Nlild at rest;
threateningonly during
feedings
Little or none
Need for
gavage orspecialized
nipple
feedings
I’requent
1’requent
les
None None
4 Hugh J. Williams
TABLE II
CLASSIFICATION OF I1ILATERAL CHOANAL ATRESIA
MAY, 1971
Type III. This still smaller group of infantsrapidly masters the technique of oralbreathing and any presenting respiratory
symptoms are mild. An artificial airway
is not necessary in this group. Nevertheless,
the necessary co-ordination between mouthbreathing and sucking has not yet been
learned. Air hunger and cyanosis, whichare only mild and occasional during ordi-
nary activities, become severe during at-
tempts at feeding (Case �). Because of thisrelationship between feedings and respira-
tory distress, an investigation of theesophagus for vascular ring, ectopic leftpulmonary artery or esophageal stenosis isoften mistakenly undertaken. Gavage feed-
ing, until such time as oropharyngealbreathing and sucking become co-ordi-
nated, is the only initial treatment required
in these infants.
Type IV. This very small group is com-posed of those few neonates who are for-
tunate enough to rapidly master both the
technique of oropharyngeal breathing andthe essential co-ordination between mouthbreathing and sucking. Infants in thisgroup manifest little or no respiratory dis-
tress and do not require use of an airway
or specialized feeding techniques. In in-fants with a complete absence of symptoms,
the condition may remain undetected for avariable period. None of the infants in thisseries fall in this category.
DIAGNOSIS
The diagnosis of bilateral choanal atresiais established clinically by the inability topass a firm rubber catheter or probe
through the nose into the nasopharynx.Positive confirmation can be made roent-genologically by obtaining lateral skullroentgenograms, following the instillation
of an opaque medium such as oily dionosil
into each nasal vestibule. Preliminaryinstillation of a pediatric strength nasal
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Posterior Choanal AtresiaVOL. 112, No. 5
decongestant into each nostril will elimi-nate the possibility of mistakenly diagnosing
nasal obstruction by edematous, inflamed
nasal mucosa for choanal atresia. This isparticularly important where the mother
has been receiving reserpine. Nasal con-
gestion occurs in approximately 10 per centof such infants and when severe may
occlude the nasal passages, thereby simu-
lating bilateral choanal atresia.6 This drug-induced nasal congestion may persist for� to 6 days following delivery. If desired,
the thickness of the occluding septum canbe determined by measuring the distance
separating the opaque medium in theposterior nasal cavity from the air column
in the nasopharynx. The opaque mediummust be flushed from the initially examined
nare before proceeding to examine theopposite nare. If prior use of an airway hasbeen found essential to prevent respiratory
distress, it should be left in place duringthe roentgenographic examination.
Unilateral choanal atresia is usually abenign condition with minimal symptoms,and is seldom diagnosed until later in
childhood or adult life (Case �). The chief
symptoms consist of a persistent nasalobstruction and a constant discharge from
the affected nostril. Unilateral atresia,
however, may assume real importance in
early infancy should the unobstructed nos-tril become occluded from rhinitis or othercause prior to mastery of the technique of
mouth breathing. Under such circum-stances a unilateral choanal atresia be-comes functionally converted into a bi-
lateral choanal atresia and severe respira-
tory distress may develop (Case 6).
TREATMENT
Since bilateral choanal atresia often
presents as a neonatal emergency, immedi-ate diagnosis and treatment are essentialto prevent death from asphyxia. Failure
to consider this congenital anomaly in the
newborn infant with severe respiratory dis-tress is responsible for an indeterminate
number of readily preventable deathsannually. In cases of Type i bilateral
choanal atresia, immediate establishment
and maintenance of an adequateoropharyngeal airway is essential and life-
saving. Gavage feedings are often necessary
subsequently in such infants. Correctivesurgery, the definitive treatment, can be
performed later as an elective procedure.In Type II infants, intermittent use of an
airway and gavage feedings should beemployed. Type III cases require onlygavage feedings during the neonatal period
and these may be discontinued when theafflicted infar�t learns to co-ordinate oral
breathing and swallowing. Type ‘iv casesrequire no therapy initially.
The definitive treatment in all types
of bilateral bony choanal atresia is surgicalresection of the septa. Surgical resection of
the obstructing bone via the transpalatalapproach is most commonly employed and
this operation can be successfully per-
formed during the neonatal period.’ Theposterior choanal diameter doubles during
the first year of life and for this reasonsurgery is often delayed until after 6
months of age, at which time it is tech-nically simpler. More recently McGovern4
has described the use of a large holedrubber nipple which is strapped in the in-fant’s mouth as a temporary means of
treatment. This ingenious, yet simple de-vice was invented by the concerned parents
of a child born with bilateral choanalatresia. By employing the McGovern
apparatus, definitive surgery may be safely
delayed for several months, when it be-comes technically simpler because of theincreased size of the operative area. A
modification of the McGovern nipple hasbeen successfully employed in several of
the more recent cases at our hospital (Case7).
In cases of bilateral membranous atresia,
transnasal puncture of the occluding mem-branes with a curved hemostat, followedby insertion of hollow tubing through each
nostril into the nasopharynx, can be readilyperformed during the neonatal period.Should stenosis occur after this procedure,
it can be treated by periodic dilatations.In patients with unilateral atresia (Case
�) surgical correction of the anomaly can
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FIG. i. Case 1. (4 and B) Newborn infant with Type i bilateral choanal atresia. Opaque medium injecte(linto both the right (4) and left (B) nasal cavities does not enter the nasopharynx. Note the endotracheal
airway which was required to prevent death from asphyxia.
6 Hugh .J. \Villianis M�’, 197!
be performed as an elective procedure any-
time following establishment of the diag-
nosis. The surgical technique is similar tothat in bilateral choanal atresia and the
transpalatal approach is most commonly
employed.
REPORT OF REPRESENTATIVE CASES
CASE 1. B.G.H. was a full term female in-
fant who breathed spontaneously at birth but
within seconds became cyanotic and struggled
to maintain respiration. The respiratory dis-tress was relieved by insertion of a pharyngeal
airway, but recurred immediately whenever the
airway was removed (Type i). The diagnosis
of bilateral choanal atresia was established
clinically by the inability to pass a rubber cath-
eter through either nostril and was confirmed
roentgenologically (Fig. i, A and B). Bilateral
transnasal perforation was carried out under
general anesthesia during the first day of life
and No. io French catheters were inserted
through both nostrils into the pharynx. On
awaking from the anesthetic the baby was ableto breathe readily through the catheters andtolerated its feedings well.
CASE 2. D.M., a full term female infant,
developed sternal retraction and cyanosis im-
mediately after delivery in another hospital.
This was felt to be due to prolapse of the tongue
into the throat and an airway was inserted,
with immediate relief of the symptoms. Fol-
lowing transfer at 6 hours of age, the airway
was removed and the baby breathed more easily,with only occasional episodes of sternal re-
traction (Type II). The diagnosis of bilateralchoanal atresia was established both clinically
and roen tgenologically. Cyanosis developed
whenever oral feedings were attempted, and
because of this gavage feedings were instituted.
By the age of 3 weeks, oral feedings could be
administered slowly by nipple without pro-
voking respiratory distress. Bilateral transpal-
atal repair was performed at the age of 8 weeks.
CASE 3. G.j. was a 2,500 gm. premature
male infant, the product of a pregnancy compli-
cated by toxemia and polyhydramnios. Res-
piratory distress and cyanosis began immedi-
ately after birth. The distress was relieved by
insertion of an airway, but recurred immediately
whenever an attempt was made to remove theairway (Type i). Neither a rubber catheter nor
opaque medium could be passed through either
nostril, confirming the diagnosis of bilateral
choanal atresia. A Type III esophageal atresia
with fistula was also present. In addition to the
above anomalies, the baby had low, deformed
ears and micrognathia. The esophageal atresia
was repaired immediately and a gastrostomy
performed. The treatment of the choanal atre-
sia consisted of a rigid pharyngeal airway for 3
days, followed by use of a McGovern nipple.
The infant gradually mastered mouth breath-
ing and by 3 weeks of age could tolerate short
periods without the nipple. By the age of �
weeks he could take small amounts of formulaorally, although sternal retraction and cyanosis
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VOL. 112, No. i Posterior Choanal Atresia 7
FIG. 2. Case 10. (A and B) Type II bilateral choanal atresia. The diagnosis was established both clinically
and roentgenologically during the first week of life. There was intermittent respiratory distress, aggra-vated by feeding, which responded to intermittent use of an airway and gavage feedings. At the time of
this contrast examination at the age of 2 years, the only symptoms present were bilateral nasal obstructionand a thick, persistent mucoid discharge from both nostrils. Oily dionosil injected into the right nare (A)
and left nare (B) fails to enter the nasopharynx. The child is now 5 years of age and has not had surgical
treatment.
sometimes occurred during feedings. At the age
of 7 weeks he was able to breath without the
open tip nipple and this was discontinued.
Large amounts of thick mucus were removed
by suction periodically from the occluded nos-
trils. He gained well until 5 months of age,
when he developed aspiration pneumonitis and
died of cardiac arrest. At autopsy the esopha-
geal anastomotic site was tightly stenotic.
CASE 4. R.A.L. was a full term male, born4 days prior to admission in another hospital.Numerous episodes of mild dyspnea and cya-
nosis occurred during the first hours of life butan airway was never required. Bronchoscopy on
3 occasions was negative. Attempts at feedingwere accompanied by severe cyanosis and
choking spells. The diagnosis of an overly re-laxed, floppy tongue was made and the babywas fed by gavage. There was a persistent,thick, tenacious mucoid discharge from bothnostrils and at times the mucus contained smallamounts of blood. Attempts to feed the babyorally by nipple following admission to Chil-
tuted, and the subsequent feedings were com-plicated by only mild cyanosis. The diagnosisof bilateral choanal atresia was established
clinically at i week of age by failure to pass acatheter through either nostril. This diagnosis
was confirmed roentgenologically using lipiodol
(Fig. 3, A and B). An esophagogram was nega-
tive. Bilateral transpalatal repair was per-
formed in this infant with Type III choanalatresia, with complete relief of symptoms.
CASE 5. A.L., a 12 year old female, wasseen because of a complete obstruction of the
left nasal cavity since birth. This had been ac-companied by an ipsilateral copious thick,
mucoid nasal discharge. On inspection, the left
nasal cavity contained a large amount of ten-
acious, glairy mucus. A catheter could not be
passed through the left nostril into the naso-
pharynx, but passed readily through the right
nostril. The diagnosis of unilateral left choanal
atresia was confirmed by a lipiodol study (Fig.
s, A and B). Surgery was performed electively6 months later and the thick bony septum was
resected through a transpalatal approach.
CASE 6. J.M.C. This full term, newborn
male infant was transferred to Children’s Hos-
pital because of a small omphalocele, which
was repaired uneventfully. There was no res-
piratory difficulty and the baby sucked well.
There was a large amount of thick mucoid dis-
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FIG. 3. Case �. (A and B) Type III bilateral choanal atresia. Numerous episodes of mild dyspnea and cyanosis
attributed to a lax tongue occurred during the first week of life, but did not necessitate an airway. Since
attempts at oral feedings resulted in choking and severe cyanosis, gavage feedings were utilized. Three
negative bronchoscopies were performed. Lipiodol examination at the age of I week of the right (A) andleft nares (B) revealed bilateral choanal atresia.
8 Hugh J. Williams MAY, 1971
charge from the right nostril. Choanal atresia
was suspected clinically, because a cousin had
been treated for this disease. A rubber catheter
could not be passed through the right nostril,
but passed readil through the left nostril into
the pharynx. The infant returned as an out-
patient 6 weeks later for contrast study confir-
mation of the previously diagnosed right choanal
atresia. Opaque medium injected into the right
nasal cavity failed to enter the nasopharvnx
(Fig. 4A). After flushing the medium from the
right nostril, the catheter was passed into the
nasopharynx (Fig. 4B), whereupon the infant
immediately developed severe respiratory dis-
tress, sternal retraction and cyanosis. Follow-
ing suction, mouth-to-mouth resuscitation, and
the administration of oxygen, normal breathing
resumed in several minutes. The baby was ad-
mitted for observation and discharged in good
condition some 24 hours later. Transpalatal
FIG. 4. Case 6. (A and B) The diagnosis of right choanal atresia was clinically established during the firstweek of life.Injection of contrast medium into the right nasal cavity at the age of 6 weeks confirmed the
diagnosis of unilateral choanal atresia (A). Contrast medium injected into the left nostril passed readilyinto the nasopharynx (B) and provoked immediate respiratory distress which necessitated emergency
measures. This case represents iatrogenic temporary conversion of a unilateral form of choanal atresiainto a Type I form of bilateral choanal atresia.
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VOL. 112, No. I Posterior Choanal Atresia 9
FIG. �. Case �. (A and B) This i 2 year old female presented with obstruction of the left nostril, accompanied
by a copious mucoid discharge from this nostril since birth. A catheter could not be passed through theleft nare into the pharynx. Lipiodol injected into the left nasal cavity did not enter the nasopharynx in
either the lateral (A) or submentovertical (B) views. A thick bony posterior septum was surgically re-
sected. Note the small amount of lipiodol which has entered the sphenoid sinus.
repair of the right choanal atresia was subse-
quently performed. Occlusion of the anatomi-
cally patent left choana by the puddled bolus of
opaque medium in the nasopharynx, in effect,converted this case of unilateral choanal atresia
into a Type I bilateral choanal atresia, pro-
ducing immediate, severe, respiratory distress.
CASE 7. B.G.D. This full term female in-
fant breathed promptly immediately after
birth and had a lusty cry. Shortly thereafter,
mild retraction developed but no airway assis-
tance was required. A chest roentgenogram re-
vealed a small pneumomediastinum. A catheter
could not be passed through either nostril, and
roentgenographic examination with a water
soluble medium confirmed the diagnosis of bi-
lateral choanal atresia. The pneumomediasti-
num resolved spontaneously and the infant had
no further difficulty in breathing at rest. At-
tempts at feeding through an ordinary nipple
produced a moderate degree of choking and
mild cyanosis. She was switched to a large
holed McGovern type nipple and the nurses
remarked on how well she mastered the tech-
nique of alternating breathing and sucking.
\Vithin 2 weeks her oral breathing and sucking
co-ordination had become so well developed
that she was able to feed through an ordinary
nipple without any respiratory distress. She
was discharged at the age of � weeks and elec-
tive surgery was planned at about i year of age.
There was a copious, thick, mucoid discharge
from both nares of this case of Type iii bilateral
choanal atresia during the period of hospital-
ization.
DISCUSSION
Choanal atresia is not rare, despite thepaucity of cases reported in the roentgeno-
logic literature. An incomplete search in
our community, prompted by the chrono-
logically rapid presentation of several cases
of choanal atresia at The Children’s Hos-pital of St. Paul, yielded 19 cases. All of
these were encountered in the past ioyears, and io of the patients presented
during the past 2 years. An analysis of
these cases (Table I) revealed that theatresia was bilateral in almost 50 per cent
of the patients (9 of 19). This incidence,however, is not truly representative of thebilateral frequency of this anomaly, since
many unilateral cases either escape diag-
nosis, or do not enter a hospital such as
ours following establishment of the diag-
nosis. The sex incidence in our series was
slightly higher in females than males (II
versus 8), but there was not the twofold
female predominance encountered in the
larger series of Flake and Ferguson.3 When
unilateral atresia was present in our series,there was no distinct preference for either
side, in contradistinction to the above
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10 MAY, 1971Hugh J. Williams
Illentioned series, where unilateral atresia
occurred twice as frequently on the right
side.’
Because of the ati thor’s dissatisfaction
with the anatomic classification of bilateral
choanal atresia which offers no assistanceto the clinician, a new classification of bi-
lateral choanal atresia has been devised
and is proposed (Table ii). This is a clinical
classification, based on the rapidity with
which the neonate learns to mouth-breathe
and to co-ordinate oral breathing with
sucking. It is hoped that this classification,as opposed to the traditional anatomic
classification, will orient the clinician
towards the varying clinical features ofbilateral choanal atresia. Hopefully thisapproach will lead to earlier diagnosis and
institution of appropriate treatment.The diagnosis of bilateral choanal atresia
is established clinically by failure to pass a
rubber catheter through either nostril into
the nasopharynx. Roentgenologic confirma-tion can be achieved by injecting an opaque
medium such as oily dionosil into eachnasal cavity. The initial treatment in
symptomatic infants consists of establish-ing an airway and employing specializedfeeding techniques. The recent introduc-
tion of the McGovern nipple has provided
an ingenious alternative form of temporary
treatment and allows definitive surgery tobe delayed until it is technically simpler.
This simple technique solves both therespiratory and feeding problems and obvi-
ates the need for an endotracheal tube or
tracheotomy in most cases.Unilateral atresia is usually a benign
condition and the diagnosis is frequentlynot established until later in life, when theaffected individual presents with the com-
plaint of unilateral chronic nasal obstruc-tion and discharge. Only rarely does uni-lateral choanal atresia produce a life
threatening situation, but this can occurif the uninvolved nostril becomes occludedby rhinitis or other cause before the infant
learns oral breathing (Case 6). Except inthese rare instances, the treatment ofunilateral choanal atresia is elective.
SUMMARY
I . Posterior choanal atresia is an tin-
common condition, but it is not as rare as
the scant radiologic literature would mdi-cate.
2. The findings in 19 cases of unilateral
and bilateral choanal atresia are presented,
with the emphasis on symptoms, diag-nosis, and treatment. It is stressed that the
newborn infant is an obligate nasal breatherand that oral breathing is an acquired
ability.
3. A new clinical classification of bi-
lateral choanal atresia, based on the easewith which the neonate learns both tobreathe orally and to co-ordinate mouth
breathing and sucking, is proposed as areplacement for the traditional anatomic
classification.
�. Bilateral choanal atresia must beruled out in any neonate with respiratory
distress. Failure to quickly establish the
diagnosis and institute immediate therapymay result in preventable death from
asphyxia.
�. Unilateral choanal atresia is usually abenign condition and elective therapysuffices.
The Children’s Hospital31! Pleasant Avenue
St. Paul, Minnesota 55102
The author wishes to express thanks tothe various radiologists and otorhinolaryn-
gologists in various Twin City Hospitalswho contributed cases and encouragement.
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