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Congenital posterior choanal atresia
Congenital ARAM ROOPENIAN,M.D. AND
Posterior
AUGUST L. STEMMER, D.M.D., M.D.,
OSTERIOR choana1 atresia, a congenita1 formerly regarded as rare, is now becoming recognized as one which occurs more frequently than generalIy appreciated. In the past twenty years, due to a more informed medica profession, an increasing number of cases have been presented in the Iiterature. In addition, many recognized cases go unreported. It is thought by many that there is also a Iarge’ group that are unrecognized, resuIting in asphyxia1 death in the newborn or very young infant, and cIassified as asphyxia neonatorum, asphyxia cause unknown, bronchopneumonia or ateIectasis. To determine the incidence of this anomaIy more accurateIy, it wouId behoove the pathoIogist to examine the nasopharynx in every case of obscure infantile asphyxia1 death. It wouId better behoove the obstetrician to estabIish the patency of the choanae in the process of aspirating mucus from the mouth and nose of the newborn infant by simpIy attempting to pass the cathether through the nose into the nasopharynx. EmbryoIogicaIIy, the obstruction is due to a persistence of the bucconasa1 and buccopharyngea1 membranes and the intervening mesodermal eIements. This partition between the nasa1 cavity and nasopharynx may be thin or thick depending on the quantity of persisting mesoderm. It may be membranous, bony or a combination of ihe two, with a centra1 membranous area surrounded by a b ony rim. An atresia membranous at birth may become bony at a Iater date. In a study of I 15 cases, Frasier found that go per cent were bony. The obstruction may be compIete or partia1. ApproximateIy 300 cases have been reported in the Iiterature and about 36 per cent of these were biIatera1. As with a11 congenita1 anomaIies, one must be aIert to the possibiIity of muItipIe anomaIies, and severa such cases have been reported, 802 American Journalof Surgery. Voiume 96, December, rg28
P anomaIy
Choanal
Atresia Boston, Massachusetts
incIuding congenita1 heart disease. A hereditary tendency is reported. DIAGNOSIS It is imperative to estabIish the diagnosis of choanal atresia as soon as possibIe, since thk infant’s Iife may depend on this. The newborn infant instinctiveIy breathes through the nose. Mouth breathing is a habit acquired weeks or months Iater, probably during the crying experience. The biIatera1 case of atresia causes troubIe immediateIy after birth and the symptoms are usuaIIy most urgent. If the mouth happens to be open, the chiId wiI1 be comfortabIe; but when the mouth cIoses, an immediate emergency ensues during which the chiId struggIes for breath, exhibits suprasterna1 and infrasternal retraction and becomes cyanotic. The infant tightens the lips together thereby depriving itseIf of any possibIe air suppIy. If at this stage the chiId begins to cry, the episode ends favorabIy. However, if he does not cry and if the emergency is not discovered and treated immediateIy by the establishment of an airway, the infant wiI1 die of asphyxia. It is evident that such cases need constant attention until mouth breathing is learned, which some infants accomplish earIy and others late. OccasionaIIy an infant wiI1 be a mouth breather at birth, which probabIy expIains why some cases of biIatera1 atresia are not discovered for years. Feeding, of course, is another probIem. The chiId must be interrupted frequentIy at meaIs. Aspiration of miIk and coughing are common, sometimes resuIting in bronchopneumonia. MaInutrition and dehydration deveIop often. In the oIder chiId with biIatera1 atresia there is a constant viscid, gIairy nasal discharge, inabiIity to bIow the nose, anosmia, constant mouth breathing and a voice quaIity characteristic of nasal obstruction.
CongenitaI
Posterior
In the uniIatera1 case there may be no respiratory difficulty or feeding diffrcuhy until the patent airway becomes occIuded by nasal infection or mechanicahv obstructed by pIacing the baby in the crib in such a fashion as to compress the nostri1 on the patent side. NasaI obstruction, discharge and anosmia are present only on the affected side. These cases, of course, do not usuaIIy present the compeIIing probIems of the biIatera1 case. The diagnosis may be made by attempting to pass a fIexibIe catheter or meta probe through the nose into the nasopharynx. It has been recommended that methylene bIue dye be dropped into the nose and the pharynx inspected for staining, or radiopaque oi1 used and traced by x-ray studies. These diagnostic methods, aIthough usefu1, are not fooIproof, as a smaI1 inadequate choana1 opening may admit the passage of dye or oil into the nasopharynx and be misleading. TREATMENT
OF
BILATERAL
ChoanaI an opening
Atresia
in each choana via the nares, using knives, curets, chiseIs, drills, trochars, caustics or eIectrocautery for this forceps, purpose and maintaining this opening by the use of rubber or pIastic tubes for \veeks or months. Much care must be exercised to avoid penetrating the base of the skull. Periodic diIatation is usually necessary to avoid stricture. Secondary surgery ma)- be nccessar)’ at a later age. The transpalatine approach is more practical in Iater months or years and there are many reports of cases treated successfull!by this route after the chiId is six months of age. Some operators prefer to make a midIine incision through the mucoperiosteum of the hard palate extending into and through a portion of the soft paIate, retracting the soft tissues IateraIIy, thus exposing the defective area to direct vision. Others prefer to make a transverse, anteriorIy curved incision, rellecting the mucoperiosteum of the hard paIate as a posteriorIy hinged flap, exposing the region. It shouId be noted at this point that if the Iatter procedure is foIlowed care must be taken to make the incision cIose to the bases of the teeth, preserving both posterior paIatine arteries. FaiIure to observe these precautions may result in formation of :I fistula or necrosrs of the paIata1 flap, as has been reported. Additiona working room ma? be obtained by extending the incision around the maxihary tuberosities and carefulI?- stretching the posterior palatine arteries from their canals. The atretic membrane and bone are then resected aIong with a section of the posterior \-omer and posterior edge of the hard paIate, preserving and manipulating enough mucous membrane to cover denuded areas. The paIata1 Aap is then sutured back in its anatomica position. The ends of the sutures should be Ieft Iong enough to allon tying them over a dressing which will maintain the paIata1 flap against the palatal bone. This dressing can be removed in a few days, after the ffap has adhered. If the molar teeth are present, they can be used as anchorage points for sutures to maintain a dressing. Rubber or plastic tubes of caIiber sufficient to Ii11the newly made choanae are then inserted through the nose into the nasopharynx. Some surgeons have used one long tube, entering passing through each choana one nostril, at the posterior end of the vomer, and emerging
ATRESIA
Once the diagnosis has been established, treatment depends on the severity of the symptoms, the age of the patient and the type of atresia present. In the biIatera1 case in the newborn, steps must be taken immediately. Medovy and Beckman [zz] recommend the use of a feeding nippIe with a Iarge opening in it, as an airway, placed in one corner of the patient’s mouth and through which the infant can be fed with a medicine dropper w-ithout serious respiratory diffrcuIty. Feedings of course must be interrupted frequentty. Hough [7] recommends the passage of a plastic tube through the mouth into the stomach and a smaI1 catheter between the paIate and tongue. SeveraI other types of airways have been used with success, but in any case airways or stomach tubes cannot be tolerated Iong and the nutrition suffers rapidI?. Therefore in these cases surgery must be initiated very shortly. Tracheotomy has been recommended, but this procedure is fraught with danger in the newborn and the postoperative care is demanding. The genera1 surgica1 approaches described for correction of bilatera1 atresia in the infant are (I) transnasa1 and (2) transpaIatine. For the newborn, opinion favors the transnasal approach as the more feasibIe one. The operation consists essentiaIIy of making 803
Roopenian
and Stemmer
through the other nostri1, and each end fastened together in front of the nasa1 coIumeIIa in the shape of a doughnut. After heaIing is compIete the tube(s) is removed. Periodic dilatation may be necessary, aIthough not frequently. The choice of operative approach, whether transnasa1 or transpaIatine, should be inAuenced by the thickness of the obstructing membrane. This may be diffrcuIt to ascertain but at times can be done by paIpating the nasa1 side with a probe and at the same time examining the nasopharyngeal side with the finger. If one can be sure that the partition, whether bony or membranous, is thin, it can be satisfactorily treated by the intranasa1 approach; but if the Ithickness cannot be determined or if the partition is known to be thick, the transpalatine approach offers more chance of success since it aIIows resection of the mesodermic structures under direct view and covering of the resuIting raw areas with epithelium to prevent subsequent stricture. SimpIy breaking through a thick atretic membrane or removing it piecemea1 and haphazardIy through the nose wiI1 onIy result in coIIapse of the soft tissues after the tubes are removed, and in eventual stricture. TREATMENT
OF UNILATERAL
technic which proved to be efficacious in a case of bilateral choanaI atresia in an adult. CASE
REPORT
J. G. R., a twenty-two year oId AmericanPortuguese woman, was seen at the Massachusetts Eye and Ear Infirmary complaining of inability to breathe through the nose since birth. History of respiratory distress in infancy was vague, but persistent nasal discharge and slow feedings were noted. Tonsillectomy and adenoidectomy when she was six years of age had not heIped. The cause of the nasa1 obstruction evidently was not discovered prior to the present examination, which reveaIed gIairy, viscid nasal discharge, paIe nasal mucosa and compIete absence of nasa1 respiratory exchange. A rubber catheter passed through the nares met with obstruction on each side in the region of the posterior choanae. Posterior mirror rhinoscopy was unsatisfactory. Radiopaque studies reveaIed complete biIatera1 choana1 atresia; a thin plate of bone couId be seen extending from the sphenoid sinuses to the posterior edge of the hard paIate. Sinus fdms were negative. Genera1 physica examination showed no other congenital anomalies. Operation was performed on October 19, 1957, under genera1 anesthesia. A Brown-Davis mouth gag was appIied with the patient in the recumbent position and the surgeon at the head of the table. (Fig. I.) DigitaI examination revealed the nasopharynx to be funnel-shaped with the apex of the funnel toward the nasa1 cavity. The bony outline of the posterior choanae could not be palpated, and it was feIt that a thick pad of soft tissue existed between the examining finger and the occluding bony pIate seen on x-ray film, representing thus a thick choana1 atresia. (Fig. 2.) A horseshoe-shaped incision was made through the palatal mucosa and extended around each maxiIIary tuberosity, outlining a tongue-shaped flap which was eIevated from the bone. The posterior paIatine arteries were carefulIy stretched from their canaIs to supply additional working room. (Fig. 3.) At the posterior edge of the hard palate the direction of the bone abruptty changed toward the sphenoid sinus. The soft tissues were easily eIevated exposing a continuous pIate of bone compIeteIy occluding both choanae. (Fig. 4.) This bone was removed without difficulty with maIIet and chisel, bringing into view the bucconasal membrane. The dimensions of the choanae were found to be infantile. Therefore the bony openings were enlarged in a11 directions, incIuding removal of the posterior edge of the vomer and hard paIate. The bucconasa1 membrane was incised along the periphery of each bony opening but Ieft attached at the floor of the choanae, thus creating two mucosa1
ATRESIA
This type may go unrecognized for a considerabIe Iength of time, with respiratory diffIcuIty being noticed onIy when the open nasa1 passage is occIuded. Most operators agree that surgery in the uniIatera1 case may be postponed unti1 the chiId reaches the age of nine or ten years of age or Iater. The transnasa1 route is very effective. One may remove the posterior haIf of the nasal septum along with. its mucoperiosteum, close to the ffoor of the nose, creating in effect a Iarge posterior perforation of the nasa1 septum through which the occluded side communicates with the open side, using the one patent choana for both sides. One may carry remova of bone and membrane IateraIly when the posterior end of the septum has been reached in an attempt to make the posterior opening Iarger if necessary. A transepta1 approach accomplishing the same result has been described. The transpaIatine approach may aIso be used depending on the surgeon’s preference. The foIIowing case iIlustrates a surgical 804
Congenitd
Posterior
Chwtnal
Atresia
3
2
I
FIG. I. Diagram illustrating the condition found at operation. Note the thickness of the obstructing partition, due mainly to the mesoderma1 soft tissues. This type of case is best approached by the transpalata1 route. FIG. 2. The horseshoe-shaped incision affords excellent exposure. There is no danger of necrosis of the paIata1 flap if both posterior palatine arteries are preserved, and hence no possibility of formation of a fistula. FIG. 3. The palatal case.
flap is eIevated
from the bone and the bony atretic
4
5
partition
is exposed with
0
FIG. 4. The obstructing bone is resected along with the vomer and a transverse section of the posterior edge of the hard paIate. The nasobuccal mucosa is then incised at the roof and sides of each choanal opening and the resulting flaps reflected back over the edge of the hard palate. Resection of the stippled area is a “must.” FIG 5. The bucconasal flap on each side is maintained over the posterior edge of the bony paIatc by a suture passed through a drill hoIe. The buccopharyngeal mucosa is incised at its junction with the soft palate and sutured to the mucosal edge at the roof of each choana. FIG. 6. AIthough manipulation of mucous membrane flaps minimizes raw areas, it is wise to cover any remaining raw arcas with a split-thickness skin graft, utiIizing a postnasal pack as a carrier for the graft. Rubber or plastic tubes are best introduced at this time.
flaps, each of which was retIected back over the posterior bony paIata1 edge and heId in this position by a suture passed through a dril1 hole made near the edge of the bony palate. The soft tissue partition remaining on the nasopharyngea1 side of the original bony obstruction was now examined and found to be about 94 inch thick. Although technically difficuIt, it was thinned by carefully resecting the mesodermal tissues from
the mucous membrane. (Fig. 5.) It was then possible to incise the buccopharyngeal mucosa transversely at its junction with the nasopharyngeal side of the soft palate. This was facilitated by tenting up the mucosa into the operative wound by a finger in the nasopharynx. This mucosal flap with its pedicle on the posterior nasopharyngeal waII was brought forward and sutured to the mucosal edge at the roof of each
805
Roopenian
and Stemmer 2. The bony choana1 openings shouId be enIarged as much as possible. This necessitates removal of a transverse section of the posterior edge of the hard paIate and a portion of the vomer. 3. When the obstructing partition is thick, the mesoderma1 tissues responsibIe for the thickness shouId be resected compIeteIy, Ieaving a thin mucous membrane on the nasa1 side and a thin mucous membrane on the nasopharyngea1 side which shouId be used judiciously to cover denuded areas. Whenever possibIe, any remaining denuded areas shouId be skin grafted to prevent stricture.
FIG. 7. The paIata1 flap is maintained against the DaIataIbone bv softened denta comoound aDDIiedover g bridge of wire extending between the fnst r&Jar teeth. Vaselined gauze is interposed between the compound and the mucosa.
SUMMARY
The incidence of congenita1 posterior choana1 atresia is higher than generaIIy appreciated and one shouId suspect this condition in any infant with obscure respiratory diffIcuIty. The diagnosis is made simpIy when suspected. Treatment depends on the severity of the symptoms, the age of the patient and the character of the obstructing partition. The two genera1 surgica1 approaches are described and a case of biIatera1 choanaI atresia in an aduIt is reported with detaiIed operative notes.
choana. AIthough the raw areas were minimized by this procedure, scattered areas needing epitheIium stiI1 existed, aIong with a Iarger area on the superior surface of the soft palate. (Fig. 6.) EpitheIial covering was supplied to these regions by a split-thickness skin graft around a postnasal pack. Two No. 6 DavoI (polyethyIene) tubes were oassed through the anterior nares and extended through the posterior choanae, each passing superior to the postnasal pack. The paIata1 ffap was then sutured in its anatomical position and maintained against the paIata1 bone by softened denta compound over a bridge of wire extending between both upper first moIar teeth. (Fig. 7.) The postnasa1 pack was removed on the eighth postoperative day, and the nasa1 tubes on the twelfth day. The patient has been abIe to breathe comfortably through both sides ever since. Postoperative dilatation of the choanae has not been necessary, as there have been no signs of appreciable stricture. 1
REFERENCES I. BAKER,
M. C. CongenitaI atresia of posterior nares. Arch. Otolaryng., 58: 431-434, 1953. 2. BEINFIELD, H. H. UniIateraI congenital incomplete bony atresia of the posterior nares (choanae). Arch. Otolaryng., 59: 65-70, 1954. 3. BEINFIELD, H. H. Atresia of the posterior nares in the infant. Eye, Ear, Nose CYThroat Montbly, 34: 575-579. 1955. 4. BEINFIELD, H. H. The unsoIved probIem of infant suffocation. New York State J: Med., 57: 31603161, 1957. 5. DENNY, W. R. and WILSON, C. P. Naso-pharyngea1 stenosis. J. Laryng. CYOtol., 71: 645-654, 1957. 6. ERSNER, M. S. Operative and postoperative management of biIatera1 bony atresia of the choana. Arch. Otolaryng., 58: 96-103, 1953. 7. HOUGH, J. V. D. The mechanism of asphyxia in biIatera1 choana1 atresia: the technic of its surgicaI correction in the newborn, Soutb. M. J..
COMMENTS
SeveraI technica points seem worthy of emphasis : I. The midIine vertica1 transpalatine incision, aIthough it affords good exposure, may resuIt in a fistuIa. The transverse curved incision cIose to the posterior edge of the hard paIate has aIso been reported as frequentIy resuIting in formation of a fistuIa. The horseshoe-shaped incision, preserving both posterior paIatine arteries as described herein, resuIts in a Ionger paIata1 Aap which is very viabIe and generousIy overIaps the paIata1 bone when repIaced. Exposure by this method is good and there are no compIications in heaIing of the ffap.
48: 588-594, 1955. 8. KAZANJIAN, V. H. The treatment of congenital atresia of the choanae. Ann. Otol., Rbin. u Laryng., 51: 704, 1942. g. KAZANJIAN, V. H. CoIIective review: secondary deformities of cleft paIates. Plast. H Reconstruct. Surg., 8: 477-489, 1951. IO. KNIGHT, C. H. CongenitaI bony occIusion of the posterior nares. Medical News) Nov. IO, 1888.
806
CongenitaI
Posterior
ChoanaI
Atresia
atresia of the choanae in the smaI1 child or the infant. Arch. OtO&‘ng., 41 : 432-438, 1945. I 4. WALKER, .I. S. Transpatatine surgery for congenital biIatera1 choanal atresia. J. A. M. A., 154: ?53-
I I. LICGOVERN, F. H. The association of congenitat choanal atresia and congenita1 heart disease. Ann. Otol., Rbin. w Laryng., 62: 894, 1953. 12. hlEDOvY, H. and BECKMAN, I. H. Asphyxial attacks in the newborn infant due to congenita1 occIusion of the posterior nares; report of five cases. Pediatrics, 8: 195 I. 13. RUDDY, L. A transpalatine operation for congenital
755, ‘954. 15. WILSON, C. P. Observations on the surgery of the nasopharynx. Ann. Otol., Rbin. CT Laryng.. 66: 5, ‘957.
807
Posterior
AUGUST L. STEMMER, D.M.D., M.D.,
OSTERIOR choana1 atresia, a congenita1 formerly regarded as rare, is now becoming recognized as one which occurs more frequently than generalIy appreciated. In the past twenty years, due to a more informed medica profession, an increasing number of cases have been presented in the Iiterature. In addition, many recognized cases go unreported. It is thought by many that there is also a Iarge’ group that are unrecognized, resuIting in asphyxia1 death in the newborn or very young infant, and cIassified as asphyxia neonatorum, asphyxia cause unknown, bronchopneumonia or ateIectasis. To determine the incidence of this anomaIy more accurateIy, it wouId behoove the pathoIogist to examine the nasopharynx in every case of obscure infantile asphyxia1 death. It wouId better behoove the obstetrician to estabIish the patency of the choanae in the process of aspirating mucus from the mouth and nose of the newborn infant by simpIy attempting to pass the cathether through the nose into the nasopharynx. EmbryoIogicaIIy, the obstruction is due to a persistence of the bucconasa1 and buccopharyngea1 membranes and the intervening mesodermal eIements. This partition between the nasa1 cavity and nasopharynx may be thin or thick depending on the quantity of persisting mesoderm. It may be membranous, bony or a combination of ihe two, with a centra1 membranous area surrounded by a b ony rim. An atresia membranous at birth may become bony at a Iater date. In a study of I 15 cases, Frasier found that go per cent were bony. The obstruction may be compIete or partia1. ApproximateIy 300 cases have been reported in the Iiterature and about 36 per cent of these were biIatera1. As with a11 congenita1 anomaIies, one must be aIert to the possibiIity of muItipIe anomaIies, and severa such cases have been reported, 802 American Journalof Surgery. Voiume 96, December, rg28
P anomaIy
Choanal
Atresia Boston, Massachusetts
incIuding congenita1 heart disease. A hereditary tendency is reported. DIAGNOSIS It is imperative to estabIish the diagnosis of choanal atresia as soon as possibIe, since thk infant’s Iife may depend on this. The newborn infant instinctiveIy breathes through the nose. Mouth breathing is a habit acquired weeks or months Iater, probably during the crying experience. The biIatera1 case of atresia causes troubIe immediateIy after birth and the symptoms are usuaIIy most urgent. If the mouth happens to be open, the chiId wiI1 be comfortabIe; but when the mouth cIoses, an immediate emergency ensues during which the chiId struggIes for breath, exhibits suprasterna1 and infrasternal retraction and becomes cyanotic. The infant tightens the lips together thereby depriving itseIf of any possibIe air suppIy. If at this stage the chiId begins to cry, the episode ends favorabIy. However, if he does not cry and if the emergency is not discovered and treated immediateIy by the establishment of an airway, the infant wiI1 die of asphyxia. It is evident that such cases need constant attention until mouth breathing is learned, which some infants accomplish earIy and others late. OccasionaIIy an infant wiI1 be a mouth breather at birth, which probabIy expIains why some cases of biIatera1 atresia are not discovered for years. Feeding, of course, is another probIem. The chiId must be interrupted frequentIy at meaIs. Aspiration of miIk and coughing are common, sometimes resuIting in bronchopneumonia. MaInutrition and dehydration deveIop often. In the oIder chiId with biIatera1 atresia there is a constant viscid, gIairy nasal discharge, inabiIity to bIow the nose, anosmia, constant mouth breathing and a voice quaIity characteristic of nasal obstruction.
CongenitaI
Posterior
In the uniIatera1 case there may be no respiratory difficulty or feeding diffrcuhy until the patent airway becomes occIuded by nasal infection or mechanicahv obstructed by pIacing the baby in the crib in such a fashion as to compress the nostri1 on the patent side. NasaI obstruction, discharge and anosmia are present only on the affected side. These cases, of course, do not usuaIIy present the compeIIing probIems of the biIatera1 case. The diagnosis may be made by attempting to pass a fIexibIe catheter or meta probe through the nose into the nasopharynx. It has been recommended that methylene bIue dye be dropped into the nose and the pharynx inspected for staining, or radiopaque oi1 used and traced by x-ray studies. These diagnostic methods, aIthough usefu1, are not fooIproof, as a smaI1 inadequate choana1 opening may admit the passage of dye or oil into the nasopharynx and be misleading. TREATMENT
OF
BILATERAL
ChoanaI an opening
Atresia
in each choana via the nares, using knives, curets, chiseIs, drills, trochars, caustics or eIectrocautery for this forceps, purpose and maintaining this opening by the use of rubber or pIastic tubes for \veeks or months. Much care must be exercised to avoid penetrating the base of the skull. Periodic diIatation is usually necessary to avoid stricture. Secondary surgery ma)- be nccessar)’ at a later age. The transpalatine approach is more practical in Iater months or years and there are many reports of cases treated successfull!by this route after the chiId is six months of age. Some operators prefer to make a midIine incision through the mucoperiosteum of the hard palate extending into and through a portion of the soft paIate, retracting the soft tissues IateraIIy, thus exposing the defective area to direct vision. Others prefer to make a transverse, anteriorIy curved incision, rellecting the mucoperiosteum of the hard paIate as a posteriorIy hinged flap, exposing the region. It shouId be noted at this point that if the Iatter procedure is foIlowed care must be taken to make the incision cIose to the bases of the teeth, preserving both posterior paIatine arteries. FaiIure to observe these precautions may result in formation of :I fistula or necrosrs of the paIata1 flap, as has been reported. Additiona working room ma? be obtained by extending the incision around the maxihary tuberosities and carefulI?- stretching the posterior palatine arteries from their canals. The atretic membrane and bone are then resected aIong with a section of the posterior \-omer and posterior edge of the hard paIate, preserving and manipulating enough mucous membrane to cover denuded areas. The paIata1 Aap is then sutured back in its anatomica position. The ends of the sutures should be Ieft Iong enough to allon tying them over a dressing which will maintain the paIata1 flap against the palatal bone. This dressing can be removed in a few days, after the ffap has adhered. If the molar teeth are present, they can be used as anchorage points for sutures to maintain a dressing. Rubber or plastic tubes of caIiber sufficient to Ii11the newly made choanae are then inserted through the nose into the nasopharynx. Some surgeons have used one long tube, entering passing through each choana one nostril, at the posterior end of the vomer, and emerging
ATRESIA
Once the diagnosis has been established, treatment depends on the severity of the symptoms, the age of the patient and the type of atresia present. In the biIatera1 case in the newborn, steps must be taken immediately. Medovy and Beckman [zz] recommend the use of a feeding nippIe with a Iarge opening in it, as an airway, placed in one corner of the patient’s mouth and through which the infant can be fed with a medicine dropper w-ithout serious respiratory diffrcuIty. Feedings of course must be interrupted frequentty. Hough [7] recommends the passage of a plastic tube through the mouth into the stomach and a smaI1 catheter between the paIate and tongue. SeveraI other types of airways have been used with success, but in any case airways or stomach tubes cannot be tolerated Iong and the nutrition suffers rapidI?. Therefore in these cases surgery must be initiated very shortly. Tracheotomy has been recommended, but this procedure is fraught with danger in the newborn and the postoperative care is demanding. The genera1 surgica1 approaches described for correction of bilatera1 atresia in the infant are (I) transnasa1 and (2) transpaIatine. For the newborn, opinion favors the transnasal approach as the more feasibIe one. The operation consists essentiaIIy of making 803
Roopenian
and Stemmer
through the other nostri1, and each end fastened together in front of the nasa1 coIumeIIa in the shape of a doughnut. After heaIing is compIete the tube(s) is removed. Periodic dilatation may be necessary, aIthough not frequently. The choice of operative approach, whether transnasa1 or transpaIatine, should be inAuenced by the thickness of the obstructing membrane. This may be diffrcuIt to ascertain but at times can be done by paIpating the nasa1 side with a probe and at the same time examining the nasopharyngeal side with the finger. If one can be sure that the partition, whether bony or membranous, is thin, it can be satisfactorily treated by the intranasa1 approach; but if the Ithickness cannot be determined or if the partition is known to be thick, the transpalatine approach offers more chance of success since it aIIows resection of the mesodermic structures under direct view and covering of the resuIting raw areas with epithelium to prevent subsequent stricture. SimpIy breaking through a thick atretic membrane or removing it piecemea1 and haphazardIy through the nose wiI1 onIy result in coIIapse of the soft tissues after the tubes are removed, and in eventual stricture. TREATMENT
OF UNILATERAL
technic which proved to be efficacious in a case of bilateral choanaI atresia in an adult. CASE
REPORT
J. G. R., a twenty-two year oId AmericanPortuguese woman, was seen at the Massachusetts Eye and Ear Infirmary complaining of inability to breathe through the nose since birth. History of respiratory distress in infancy was vague, but persistent nasal discharge and slow feedings were noted. Tonsillectomy and adenoidectomy when she was six years of age had not heIped. The cause of the nasa1 obstruction evidently was not discovered prior to the present examination, which reveaIed gIairy, viscid nasal discharge, paIe nasal mucosa and compIete absence of nasa1 respiratory exchange. A rubber catheter passed through the nares met with obstruction on each side in the region of the posterior choanae. Posterior mirror rhinoscopy was unsatisfactory. Radiopaque studies reveaIed complete biIatera1 choana1 atresia; a thin plate of bone couId be seen extending from the sphenoid sinuses to the posterior edge of the hard paIate. Sinus fdms were negative. Genera1 physica examination showed no other congenital anomalies. Operation was performed on October 19, 1957, under genera1 anesthesia. A Brown-Davis mouth gag was appIied with the patient in the recumbent position and the surgeon at the head of the table. (Fig. I.) DigitaI examination revealed the nasopharynx to be funnel-shaped with the apex of the funnel toward the nasa1 cavity. The bony outline of the posterior choanae could not be palpated, and it was feIt that a thick pad of soft tissue existed between the examining finger and the occluding bony pIate seen on x-ray film, representing thus a thick choana1 atresia. (Fig. 2.) A horseshoe-shaped incision was made through the palatal mucosa and extended around each maxiIIary tuberosity, outlining a tongue-shaped flap which was eIevated from the bone. The posterior paIatine arteries were carefulIy stretched from their canaIs to supply additional working room. (Fig. 3.) At the posterior edge of the hard palate the direction of the bone abruptty changed toward the sphenoid sinus. The soft tissues were easily eIevated exposing a continuous pIate of bone compIeteIy occluding both choanae. (Fig. 4.) This bone was removed without difficulty with maIIet and chisel, bringing into view the bucconasal membrane. The dimensions of the choanae were found to be infantile. Therefore the bony openings were enlarged in a11 directions, incIuding removal of the posterior edge of the vomer and hard paIate. The bucconasa1 membrane was incised along the periphery of each bony opening but Ieft attached at the floor of the choanae, thus creating two mucosa1
ATRESIA
This type may go unrecognized for a considerabIe Iength of time, with respiratory diffIcuIty being noticed onIy when the open nasa1 passage is occIuded. Most operators agree that surgery in the uniIatera1 case may be postponed unti1 the chiId reaches the age of nine or ten years of age or Iater. The transnasa1 route is very effective. One may remove the posterior haIf of the nasal septum along with. its mucoperiosteum, close to the ffoor of the nose, creating in effect a Iarge posterior perforation of the nasa1 septum through which the occluded side communicates with the open side, using the one patent choana for both sides. One may carry remova of bone and membrane IateraIly when the posterior end of the septum has been reached in an attempt to make the posterior opening Iarger if necessary. A transepta1 approach accomplishing the same result has been described. The transpaIatine approach may aIso be used depending on the surgeon’s preference. The foIIowing case iIlustrates a surgical 804
Congenitd
Posterior
Chwtnal
Atresia
3
2
I
FIG. I. Diagram illustrating the condition found at operation. Note the thickness of the obstructing partition, due mainly to the mesoderma1 soft tissues. This type of case is best approached by the transpalata1 route. FIG. 2. The horseshoe-shaped incision affords excellent exposure. There is no danger of necrosis of the paIata1 flap if both posterior palatine arteries are preserved, and hence no possibility of formation of a fistula. FIG. 3. The palatal case.
flap is eIevated
from the bone and the bony atretic
4
5
partition
is exposed with
0
FIG. 4. The obstructing bone is resected along with the vomer and a transverse section of the posterior edge of the hard paIate. The nasobuccal mucosa is then incised at the roof and sides of each choanal opening and the resulting flaps reflected back over the edge of the hard palate. Resection of the stippled area is a “must.” FIG 5. The bucconasal flap on each side is maintained over the posterior edge of the bony paIatc by a suture passed through a drill hoIe. The buccopharyngeal mucosa is incised at its junction with the soft palate and sutured to the mucosal edge at the roof of each choana. FIG. 6. AIthough manipulation of mucous membrane flaps minimizes raw areas, it is wise to cover any remaining raw arcas with a split-thickness skin graft, utiIizing a postnasal pack as a carrier for the graft. Rubber or plastic tubes are best introduced at this time.
flaps, each of which was retIected back over the posterior bony paIata1 edge and heId in this position by a suture passed through a dril1 hole made near the edge of the bony palate. The soft tissue partition remaining on the nasopharyngea1 side of the original bony obstruction was now examined and found to be about 94 inch thick. Although technically difficuIt, it was thinned by carefully resecting the mesodermal tissues from
the mucous membrane. (Fig. 5.) It was then possible to incise the buccopharyngeal mucosa transversely at its junction with the nasopharyngeal side of the soft palate. This was facilitated by tenting up the mucosa into the operative wound by a finger in the nasopharynx. This mucosal flap with its pedicle on the posterior nasopharyngeal waII was brought forward and sutured to the mucosal edge at the roof of each
805
Roopenian
and Stemmer 2. The bony choana1 openings shouId be enIarged as much as possible. This necessitates removal of a transverse section of the posterior edge of the hard paIate and a portion of the vomer. 3. When the obstructing partition is thick, the mesoderma1 tissues responsibIe for the thickness shouId be resected compIeteIy, Ieaving a thin mucous membrane on the nasa1 side and a thin mucous membrane on the nasopharyngea1 side which shouId be used judiciously to cover denuded areas. Whenever possibIe, any remaining denuded areas shouId be skin grafted to prevent stricture.
FIG. 7. The paIata1 flap is maintained against the DaIataIbone bv softened denta comoound aDDIiedover g bridge of wire extending between the fnst r&Jar teeth. Vaselined gauze is interposed between the compound and the mucosa.
SUMMARY
The incidence of congenita1 posterior choana1 atresia is higher than generaIIy appreciated and one shouId suspect this condition in any infant with obscure respiratory diffIcuIty. The diagnosis is made simpIy when suspected. Treatment depends on the severity of the symptoms, the age of the patient and the character of the obstructing partition. The two genera1 surgica1 approaches are described and a case of biIatera1 choanaI atresia in an aduIt is reported with detaiIed operative notes.
choana. AIthough the raw areas were minimized by this procedure, scattered areas needing epitheIium stiI1 existed, aIong with a Iarger area on the superior surface of the soft palate. (Fig. 6.) EpitheIial covering was supplied to these regions by a split-thickness skin graft around a postnasal pack. Two No. 6 DavoI (polyethyIene) tubes were oassed through the anterior nares and extended through the posterior choanae, each passing superior to the postnasal pack. The paIata1 ffap was then sutured in its anatomical position and maintained against the paIata1 bone by softened denta compound over a bridge of wire extending between both upper first moIar teeth. (Fig. 7.) The postnasa1 pack was removed on the eighth postoperative day, and the nasa1 tubes on the twelfth day. The patient has been abIe to breathe comfortably through both sides ever since. Postoperative dilatation of the choanae has not been necessary, as there have been no signs of appreciable stricture. 1
REFERENCES I. BAKER,
M. C. CongenitaI atresia of posterior nares. Arch. Otolaryng., 58: 431-434, 1953. 2. BEINFIELD, H. H. UniIateraI congenital incomplete bony atresia of the posterior nares (choanae). Arch. Otolaryng., 59: 65-70, 1954. 3. BEINFIELD, H. H. Atresia of the posterior nares in the infant. Eye, Ear, Nose CYThroat Montbly, 34: 575-579. 1955. 4. BEINFIELD, H. H. The unsoIved probIem of infant suffocation. New York State J: Med., 57: 31603161, 1957. 5. DENNY, W. R. and WILSON, C. P. Naso-pharyngea1 stenosis. J. Laryng. CYOtol., 71: 645-654, 1957. 6. ERSNER, M. S. Operative and postoperative management of biIatera1 bony atresia of the choana. Arch. Otolaryng., 58: 96-103, 1953. 7. HOUGH, J. V. D. The mechanism of asphyxia in biIatera1 choana1 atresia: the technic of its surgicaI correction in the newborn, Soutb. M. J..
COMMENTS
SeveraI technica points seem worthy of emphasis : I. The midIine vertica1 transpalatine incision, aIthough it affords good exposure, may resuIt in a fistuIa. The transverse curved incision cIose to the posterior edge of the hard paIate has aIso been reported as frequentIy resuIting in formation of a fistuIa. The horseshoe-shaped incision, preserving both posterior paIatine arteries as described herein, resuIts in a Ionger paIata1 Aap which is very viabIe and generousIy overIaps the paIata1 bone when repIaced. Exposure by this method is good and there are no compIications in heaIing of the ffap.
48: 588-594, 1955. 8. KAZANJIAN, V. H. The treatment of congenital atresia of the choanae. Ann. Otol., Rbin. u Laryng., 51: 704, 1942. g. KAZANJIAN, V. H. CoIIective review: secondary deformities of cleft paIates. Plast. H Reconstruct. Surg., 8: 477-489, 1951. IO. KNIGHT, C. H. CongenitaI bony occIusion of the posterior nares. Medical News) Nov. IO, 1888.
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CongenitaI
Posterior
ChoanaI
Atresia
atresia of the choanae in the smaI1 child or the infant. Arch. OtO&‘ng., 41 : 432-438, 1945. I 4. WALKER, .I. S. Transpatatine surgery for congenital biIatera1 choanal atresia. J. A. M. A., 154: ?53-
I I. LICGOVERN, F. H. The association of congenitat choanal atresia and congenita1 heart disease. Ann. Otol., Rbin. w Laryng., 62: 894, 1953. 12. hlEDOvY, H. and BECKMAN, I. H. Asphyxial attacks in the newborn infant due to congenita1 occIusion of the posterior nares; report of five cases. Pediatrics, 8: 195 I. 13. RUDDY, L. A transpalatine operation for congenital
755, ‘954. 15. WILSON, C. P. Observations on the surgery of the nasopharynx. Ann. Otol., Rbin. CT Laryng.. 66: 5, ‘957.
807