OF ROENTGENOLOGY RADIUM THERAPY AND NUCLEAR M EDICINE VOL. 112 M AY, i 7i No. I POSTERIOR CHOANAL ATRESIA* By HUGH J. W ILLIAM S, M .D. ST. PAUL, M INNESOTA 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 W hitehouse 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. W hile 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 childrens Hospital, Inc., St. Paul, M innesota. 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 M ost 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. D o w n l o a d e d
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2 Hugh j. W illiams M AY, 1971 Associated abnormalities of the surround- ing bony structures, including a high arched 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 the nasal fossa and the nasophar nx. The frequent 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 patients with unilateral choanal atresia the occur- rence was predominantly right-sided (over 2: i). SYM PTOM S 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 that oropharyngeal breathing is an acquired function, which normally takes several days to weeks to learn. This aversion to mouth breathing 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 by M oss5 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 the nose is the best avenue for respiration. It may be that this structural basis for obligatory nasal breathing in the newborn plays a protective role against the aspira- tion of food. Because most neonates are obligate nasal breathers, bilateral choanal atresia 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 from asphyxia annually in the U.S.A. In those infants who escape diagnosis in the first hours 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 seldom achieved until age 3-6 weeks. All infants with bilateral choanal atresia develop a thick, glairy, mucoid discharge from 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 small sleeping 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. M ore 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 bilateral choanal 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- D o w n l o a d e d
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VOL. 112, No. i Posterior Choanal Atresia 3 TABLE I SUM M ARY 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 II 3. 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) Omphalocele 7. B.G.l). F Bilateral Ill 8. J.H. F Left Ventricular septal defect and congenital deformity of external right 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 Left 13. K.S. F Right 14. L.S.J. F Left M ongolism i . J.A. M Bilateral I i6. C.D. F Right 17. T.M . F Bilateral I i8. B.W . I Bilateral I 19. R.S. F Right tion, by stressing the clinical rather than the anatomic aspects of the disease, will lead to a better understanding of the various manifestations of bilateral choanal atresia and hence to an earlier diagnosis. Type I. These infants lack the ability to rapidly 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 infants will 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 after birth than do infants of Type i. Their respiratory 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. D o w n l o a d e d
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No. of cases Type 1 5 Type II Type III Type IV 0 Time required to master mouth breathing Prolonged (weeks) Hours to days Immediate to a few hours None Time required to develop co-ordination between oral breathing and sucking 3-6 weeks usually -6 weeks usually 3-6 weeks None Airway requirements Immediate, prolonged Intermittent (hours to weeks) None Type of neonatal respiratory distress Immediate, severe and prolonged; usually ag- gravated by feedings Less severe and intermit- tent; occurs at rest and usually dur- ing feedings Nlild at rest; threatening only during feedings Little or none Need for gavage or specialized nipple feedings Irequent 1requent les None None 4 Hugh J. W illiams TABLE II CLASSIFICATION OF I1ILATERAL CHOANAL ATRESIA M AY, 1971 Type III. This still smaller group of infants rapidly masters the technique of oral breathing and any presenting respiratory symptoms are mild. An artificial airway is not necessary in this group. Nevertheless, the necessary co-ordination between mouth breathing and sucking has not yet been learned. Air hunger and cyanosis, which are only mild and occasional during ordi- nary activities, become severe during at- tempts at feeding (Case ). Because of this relationship between feedings and respira- tory distress, an investigation of the esophagus for vascular ring, ectopic left pulmonary artery or esophageal stenosis is often mistakenly undertaken. Gavage feed- ing, until such time as oropharyngeal breathing 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 and the essential co-ordination between mouth breathing and sucking. Infants in this group 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 a variable period. None of the infants in this series fall in this category. DIAGNOSIS The diagnosis of bilateral choanal atresia is established clinically by the inability to pass a firm rubber catheter or probe through the nose into the nasopharynx. Positive confirmation can be made roent- genologically by obtaining lateral skull roentgenograms, following the instillation of an opaque medium such as oily dionosil into each nasal vestibule. Preliminary instillation of a pediatric strength nasal D o w n l o a d e d
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Posterior Choanal Atresia VOL. 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 is particularly important where the mother has been receiving reserpine. Nasal con- gestion occurs in approximately 10 per cent of 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 can be determined by measuring the distance separating the opaque medium in the posterior nasal cavity from the air column in the nasopharynx. The opaque medium must be flushed from the initially examined nare before proceeding to examine the opposite nare. If prior use of an airway has been found essential to prevent respiratory distress, it should be left in place during the roentgenographic examination. Unilateral choanal atresia is usually a benign condition with minimal symptoms, and is seldom diagnosed until later in childhood or adult life (Case ). The chief symptoms consist of a persistent nasal obstruction 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 other cause 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). TREATM ENT Since bilateral choanal atresia often presents as a neonatal emergency, immedi- ate diagnosis and treatment are essential to 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 deaths annually. In cases of Type i bilateral choanal atresia, immediate establishment and maintenance of an adequate oropharyngeal airway is essential and life- saving. Gavage feedings are often necessary subsequently in such infants. Corrective surgery, the definitive treatment, can be performed later as an elective procedure. In Type II infants, intermittent use of an airway and gavage feedings should be employed. Type III cases require only gavage feedings during the neonatal period and these may be discontinued when the afflicted infar t learns to co-ordinate oral breathing and swallowing. Type iv cases require no therapy initially. The definitive treatment in all types of bilateral bony choanal atresia is surgical resection of the septa. Surgical resection of the obstructing bone via the transpalatal approach is most commonly employed and this operation can be successfully per- formed during the neonatal period. The posterior choanal diameter doubles during the first year of life and for this reason surgery is often delayed until after 6 months of age, at which time it is tech- nically simpler. M ore recently M cGovern4 has described the use of a large holed rubber nipple which is strapped in the in- fants 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 choanal atresia. By employing the M cGovern apparatus, definitive surgery may be safely delayed for several months, when it be- comes technically simpler because of the increased size of the operative area. A modification of the M cGovern nipple has been successfully employed in several of the more recent cases at our hospital (Case 7). In cases of bilateral membranous atresia, transnasal puncture of the occluding mem- branes with a curved hemostat, followed by insertion of hollow tubing through each nostril into the nasopharynx, can be readily performed 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 D o w n l o a d e d
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FIG. i. Case 1. (4 and B) Newborn infant with Type i bilateral choanal atresia. Opaque medium injecte(l into 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 to that 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 able to breathe readily through the catheters and tolerated 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 bilateral choanal 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 the airway (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 M cGovern 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 formula orally, although sternal retraction and cyanosis D o w n l o a d e d
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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 obstruction and 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, born 4 days prior to admission in another hospital. Numerous episodes of mild dyspnea and cya- nosis occurred during the first hours of life but an airway was never required. Bronchoscopy on 3 occasions was negative. Attempts at feeding were accompanied by severe cyanosis and choking spells. The diagnosis of an overly re- laxed, floppy tongue was made and the baby was fed by gavage. There was a persistent, thick, tenacious mucoid discharge from both nostrils and at times the mucus contained small amounts of blood. Attempts to feed the baby orally by nipple following admission to Chil- drens Hospital provoked chest retraction and marked cyanosis. Gavage feedings were insti- tuted, and the subsequent feedings were com- plicated by only mild cyanosis. The diagnosis of bilateral choanal atresia was established clinically at i week of age by failure to pass a catheter 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 choanal atresia, with complete relief of symptoms. CASE 5. A.L., a 12 year old female, was seen 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 electively 6 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 Childrens 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- D o w n l o a d e d
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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) and left nares (B) revealed bilateral choanal atresia. 8 Hugh J. W illiams M AY, 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 first week 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 readily into the nasopharynx (B) and provoked immediate respiratory distress which necessitated emergency measures. This case represents iatrogenic temporary conversion of a unilateral form of choanal atresia into a Type I form of bilateral choanal atresia. D o w n l o a d e d
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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 the left 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 M cGovern 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 the paucity 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 Childrens Hos- pital of St. Paul, yielded 19 cases. All of these were encountered in the past io years, and io of the patients presented during the past 2 years. An analysis of these cases (Table I) revealed that the atresia was bilateral in almost 50 per cent of the patients (9 of 19). This incidence, however, is not truly representative of the bilateral 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 W hen unilateral atresia was present in our series, there was no distinct preference for either side, in contradistinction to the above D o w n l o a d e d
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10 M AY, 1971 Hugh J. W illiams Illentioned series, where unilateral atresia occurred twice as frequently on the right side. Because of the ati thors dissatisfaction with the anatomic classification of bilateral choanal atresia which offers no assistance to 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 of bilateral choanal atresia. Hopefully this approach 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 each nasal cavity. The initial treatment in symptomatic infants consists of establish- ing an airway and employing specialized feeding techniques. The recent introduc- tion of the M cGovern nipple has provided an ingenious alternative form of temporary treatment and allows definitive surgery to be delayed until it is technically simpler. This simple technique solves both the respiratory 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 frequently not established until later in life, when the affected 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 occur if the uninvolved nostril becomes occluded by rhinitis or other cause before the infant learns oral breathing (Case 6). Except in these rare instances, the treatment of unilateral choanal atresia is elective. SUM M ARY 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 breather and that oral breathing is an acquired ability. 3. A new clinical classification of bi- lateral choanal atresia, based on the ease with which the neonate learns both to breathe orally and to co-ordinate mouth breathing and sucking, is proposed as a replacement for the traditional anatomic classification. . Bilateral choanal atresia must be ruled out in any neonate with respiratory distress. Failure to quickly establish the diagnosis and institute immediate therapy may result in preventable death from asphyxia. . Unilateral choanal atresia is usually a benign condition and elective therapy suffices. The Childrens Hospital 31! Pleasant Avenue St. Paul, M innesota 55102 The author wishes to express thanks to the various radiologists and otorhinolaryn- gologists in various Twin City Hospitals who contributed cases and encouragement. REFERENCES CONNELLY, J. P., M ONTGOM ERY, W . W ., and ROBINSON, J. C. Choanal atresia. Clin. Pediat., 1965, 4, 65-70. 2. EM M ERT, C. F. Lehrbuch der Chirurgie. F.v.R. Dass, Stuttgart, 1853. 3. FLAKE, C. G., and FERGUSON, C. F. Congenital choanal atresia in infants and children. Ann. Otol., Rhin. &Laryng., 1964, 73,458-473. 4. M CGOVERN, F. H. Association of congenital choanal atresia and congenital heart disease: report of two cases. Ann. Otol., Rhin. & Laryng., 1953, 62, 894-895. D o w n l o a d e d
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VOL. 112, No. Posterior Choanal Atresia II . M oss, M . L. Veloepiglottic sphincter and obli- gate nose breathing in neonate. 7. Pediat., 1965, 67, 330-331. 6. M OYA, F., and THORNDIKE, V. Effects of drugs used in labor on fetus and newborn. Clin. Pharmacol. & Therap., 1963, 4, 628-653. 7. NEGU5, V. The Comparative Anatomy and Physiology of the Nare and Paranasal Sinuses. E. & S. Livingstone, Ltd., Edinburgh, 1958. 8. RONALD5ON, T. R. Note on a case of congenital closure of the posterior nares. Edinburgh M . 7., 1880-188 I, 26, 1035. 9. ROOPENIAS, A., and STERM ER, A. L. Congenital posterior choanal atresia. Am. 7. Surg., 1958, 96, 802-807. 10. SCHW ARTZ, A. A., and I5AAC5, M . J. Congenital atresia of posterior nares: report of two cases. Arch. Otolaryng., 1942, 35, 603-612. II. SHAW , E. B. Sudden unexpected death in in- fancy syndrome. A.M .A. Am. 7. Dis. Child., 1970, 119, 416418. i . W HITEHOUSE, % V. M ., and HOLT, J. F. Para- doxical ballooning of hypopharynx in siblings with bilateral choanal atresia. Radiology, 1952, 59, 216-220. 13. W ILKERSON, W . W ., and COYCE, L. E. Con- genital choanal occlusion. Trans. Am. Acad. Ophthal., 1948, 52, 234-236. D o w n l o a d e d