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Medical management of bilateral choanal atresia
T h e Journal of P E D I A T R I C S
561
Medical management of bilateral cboanal atresia Report of 4 cases
Experience with 4 cases of bilateral choanal atresia in a newborn in[ant is recorded. The patients were managed conservatively by inserting a small oropharyngeal airway [or a Jew weeks until the in[ant was capable o[ breathing through the mouth. This method obviates the need for immediate surgery. The method has been success[ul in our hands and is recommended [or the initial management of bilateral ehoanal atresia.
Darroll
J.
Erickson, Captain, MC, USA,
James L. Lodge, Major, USAF ( M C ) , * and
Edward J. Tomsovic, Lieutenant Colonel, MC, USA
T H E currently accepted management of bilateral choanal atresia is emergency surgery. Our approach to this problem has been to teach these infants to breathe by mouth and to eat without special devices. We are thus able to delay surgery until the child is larger, thereby avoiding the inherent problems and often poor results associated with surgery during the newborn period. Our success with 4 infants has been so impressive that this method is now preferred on the Pediatric Service of this hospital.
From the Pediatric Service, Walter Reed General Hospital, Walter Reed Army Medical Center, Washington 12, D.C. eAddress, Chief of Pediatrics, 7625th United States Air Force Hos#ital, United States Air Force Academy, Colorado.
The literature cites several examples of bilateral atresia where emergency repair was not employed for various reasons, a' a, 7 McGovern 5 mentioned that infants can be trained to breathe by mouth by 3 to 4 weeks of age and described a nipple device so successfully used by one patient that surgery was postponed. The purpose of this article is to describe our method and briefly) review recent case material. The embryology of choanal atresia, its clinical manifestations, and surgical experience with the condition have been adequately reviewed elsewhere, a, 2, 6, 7
METHOD 1. An oropharyngeal airway is inserted (Fig. 1) and, if necessary, secured in place.
56 2
Erickson, Lodge, and Tomsovic
Fig. 1. Infant with bilateral choanal atresia. Oropharyngeal airway in place. A string tie attaches the airway to the patient's clothing so it will not be lost.
October 1963
days the device is used by the infant as a pacifier. Neonatal nutrition is maintained by gavage. 2. In 2 to 3 days, the infant tolerates short absences of the airway during which cautious nipple feeding is begun. The ability to alternate breathing and feeding from the nipple is quickly learned. By l to 2 weeks of age supplemental gavage feedings may be discontinued. By 4 weeks of age the airway is no longer needed. Good nursing care is essential during this period to insure the presence of the airway when necessary and to guard against aspiration during the learning phase of eating. We have encountered no trouble in this regard and find that the nursing care involved is much less than that necessary in postoperative management of the newborn. 3. The parents are encouraged to observe the progress and are kept fully informed. Prior to discharge, the parents feed the infant until they feel secure in caring for the child at home. 4. Care thereafter is as for normal infants. Solids were introduced in our patients by the age of 3 months. 5. Our present plan is to operate at 5 to 6 years of age unless indicated earlier by facial structural alterations. CASE SUMMARIES
Fig. 2. Berman plastic oropharyngeal airway. We prefer a plastic airway of the type shown in Fig. 2. Some gagging usually occurs but tolerance is quickly attained. Within a few
Case 1. A Caucasian infant female had respiratory distress and cyanosis at birth. A diagnosis of bilateral choanal atresia was made after failure to pass nasal catheters. Distress was immediately alleviated by insertion of an oropharyngeal airway. Management was as described before. The airway was employed intermittently for 4 weeks. Garage supplementswere required for 1 week. After a hospital stay of 6 weeks she was returned to her home in Italy accompanied only by her mother. When the infant was 17 mouths old, the family returned to the United States from their European assignment. Her growth and development had been normal. She had had only one illness, a postvaccinal febrile episode. Her only symptom was a moderate, persistent nasal discharge. On physical examination, it was noted that
Volume 63 Number 4 part 1
she was unable to breathe through her nose. Mild hypertelorism was present (Figs. 3 and 4) as was a smalt well-healed ectodermal defect of the scalp, present since birth. Her weight was 24 pounds and her crown-heel length was 32I,/2 inches. Some widening of the nasal cavities was present bilaterally. H e r nasal turbinates were of normal size and the nasal mucosa was of normal
Bilateral choanal atresia
563
color. X-rays of the skull showed normal sinusoidal development. Case 2. A Caucasian female was referred to our care at the age of 10 days. Shortly after birth a stuffy nose was observed as well as mild respiratory difficulty during feedings. A clinical diagnosis of bilateral choanal atresia was made and confirmed by contrast x-ray study.
Figs. 3 and 4. Frontal and lateral faclal v{ews of child 19 months of age with bilateral choanal atresla managed conservatively from birth. Same patient as Fig. 1.
5 64
Erickson, Lodge, and Tomsovic
The child tolerated the obstruction without need of an airway. By the age of 4 weeks she was eating well and had gained weight adequately. She was discharged to home care. Follow-up by correspondence revealed that the child, at 2 years of age, had experienced normal growth and development. The mother thought the infant's weight gain during the first 12 months was slow. She gained from 6 pounds, 15 ounces at birth to 19 pounds at 1 year of age. By 2 years, her weight was 27 pounds. Illnesses reported by the mother included two "colds" with fever of 104~ and an episode of pharyngitis. The child tolerated these infections w ~ o u t undue trouble and was not hospitalized. The only persistent symptom was a nasal discharge which was not troublesome. She is under the care of a local pediatrician who has advised surgery at about 4 years of age. Case 3. A Caucasian infant male was noted to have excessive nasal secretions at birth and respiratory distress if not fed slowly. A diagnosis of bilateral choanal atresia was confirmed by contrast x-rays. By frequent slow, cautious feeding, the infant did well without need of an airway. He was referred to our care at the age of 1 month for surgical evaluation. Since no difficulties were noted, surgery was deferred. At the age of t9 months, growth and development were normal. The child had had no illnesses. The only complaint offered was that of mild persistent nasal discharge. Case 4. A Caucasian female was noted shortly after birth to have excessive nasal mucus and respiratory difficulty if not fed slowly. A clinical diagnosis of bilateral choanal atresia was confirmed by x-ray contrast study. The difficulties encountered were so mild that she was discharged to home care at the age of 4 days to await transportation to Walter Reed General Hospital for surgical evaluation. No distress was encountered; the child ate and gained well during an observation period. She was returned home without surgery. Unfortunately, contact with the family has been lost. DISCUSSION A l t h o u g h initial nonoperative m a n a g e m e n t of bilateral choanal atresia is m e n t i o n e d in several articles, we think medical m a n a g e m e n t as a routine has not been previously advocated. M c G o v e r n 7 has established that bilateral atresia does not alter sinus formation, nasal physiology, or hearing. This was
October 1963
verified in o u r cases. A n o s m i a is a possible p e r m a n e n t e n d result of conservative m a n agement. Craig a n d Simpson I speculate that the nose b r e a t h i n g habit m a y never be relearned once the m o u t h b r e a t h i n g habit has been established. T h e "adenoid-facies" appearance would seem to be a logical consequence b u t m a y be avoidable with judiciously timed surgery. O u r m e t h o d allows the advantages of technically easier surgery because of the larger size of the operative field, a less complicated postoperative period, and the child benefits from the reduced anesthetic risk which accompanies increased age. We have encountered no increased incidence of infection a n d regurgitation has been no problem. Growth a n d development have been x
normal. T h e preferred routes for surgery are transpalatal a n d transnasal. 6' 8, 9 Perhaps the increased size of structures afforded by this methods of m a n a g e m e n t would facilitate employment of the transnasal approach. SUMMARY A technique of initial medical m a n a g e m e n t of bilateral choanal atresia is described. Advantage was taken of the fact that affected n e w b o r n infants can be taught to breathe through the m o u t h a n d can learn to eat without special devices. T h e m e t h o d described is now preferred by the Pediatric Service of Walter Reed General Hospital. W e no longer consider bilateral choanal atresia to w a r r a n t emergency surgery.
REFERENCES 1. Craig, D. H., and Simpson, N. M.: Posterior choanal atresia, J. Laryng. & Otol. 73: 603, 1959. 2. Campbell, W., Stokes, D., and Jennings, R.: Choanal atresia--Report of five cases and a review of recent literature, J. M. Soc. New Jersey 58: 478, 1961. 3. Baker, D., Jr., Waltner, J., and Novick, W.: Posterior choanal atresia, Ann. Otol., Rhinol. & Laryng. 69: 805, 1960. 4. Beinfield, H. H.: Bilateral ehoanal atresia in the newborn--Surgical dangers to be avoided, A. M. A. Arch. Otolaryng. 73: 659, 1961.
Volume 63 Number 4 part 1
5. McGovern, F. H.: Bilateral choanal atresia in the newborn, A new method of medical management, Laryngoscope 71: 480, 1961. 6. McGovern, F. H., and Fitz-Hugh, G. S.: Surgical management of congenital choanal atresia, A. M. A. Arch. Otolaryng. 73: 627, 1961. 7. McGovern, F. H.: Congenital choanal atresia, Laryngoscope 60: 815, I950. 8. Wilson, C. P.: Treatment of choanal atresia, J. Laryng. & Otol. 71: 616, 1957. 9. Beinfield, H. H.: Surgery for bilateral bony atresia of the posterior nares in the newborn, A. M. A. Arch. Otolaryng. 70: 1, 1959. 10. Rickharn, P. P.: Diagnosis of congenital anomalies incompatible with life, but amenable to surgical correction, Pediat. Clin. North America 955: 977, 1959. 11. Morrow, R. C.: Bilateral congenital ehoanal atresia--A report of three cases corrected surgically in the newborn, Ann. Otol., Rhinol. & Laryng. 66: 135, 1957.
Bilateral choanal atresia
565
12. Beinfield, It. H.: Treatment of complete unilateral bony atresia of the posterior nares, A. M. A. Arch. Ototaryng. 53: 530, 1951. 13. Beinfield, H. H.: Congenital bilateral bony atresia of the posterior nares in a one-month old premature infant who survived, J. PEDIAT. 45: 679, 1954. 14. Beinfield, H. H.: A forgotten cause of infant suffocation, J. Internat. Coll. Surgeons 22: 447, 1954.
ADDENDUM The child shown in Figs. 1 and 2 was operated upon by the transpalatine route in 1963 by Dr. Joseph H. Hersh of New York City. A hard, bony lamina was present bilaterally. Results have been good postoperatively and the child has definite smell discrimination.
561
Medical management of bilateral cboanal atresia Report of 4 cases
Experience with 4 cases of bilateral choanal atresia in a newborn in[ant is recorded. The patients were managed conservatively by inserting a small oropharyngeal airway [or a Jew weeks until the in[ant was capable o[ breathing through the mouth. This method obviates the need for immediate surgery. The method has been success[ul in our hands and is recommended [or the initial management of bilateral ehoanal atresia.
Darroll
J.
Erickson, Captain, MC, USA,
James L. Lodge, Major, USAF ( M C ) , * and
Edward J. Tomsovic, Lieutenant Colonel, MC, USA
T H E currently accepted management of bilateral choanal atresia is emergency surgery. Our approach to this problem has been to teach these infants to breathe by mouth and to eat without special devices. We are thus able to delay surgery until the child is larger, thereby avoiding the inherent problems and often poor results associated with surgery during the newborn period. Our success with 4 infants has been so impressive that this method is now preferred on the Pediatric Service of this hospital.
From the Pediatric Service, Walter Reed General Hospital, Walter Reed Army Medical Center, Washington 12, D.C. eAddress, Chief of Pediatrics, 7625th United States Air Force Hos#ital, United States Air Force Academy, Colorado.
The literature cites several examples of bilateral atresia where emergency repair was not employed for various reasons, a' a, 7 McGovern 5 mentioned that infants can be trained to breathe by mouth by 3 to 4 weeks of age and described a nipple device so successfully used by one patient that surgery was postponed. The purpose of this article is to describe our method and briefly) review recent case material. The embryology of choanal atresia, its clinical manifestations, and surgical experience with the condition have been adequately reviewed elsewhere, a, 2, 6, 7
METHOD 1. An oropharyngeal airway is inserted (Fig. 1) and, if necessary, secured in place.
56 2
Erickson, Lodge, and Tomsovic
Fig. 1. Infant with bilateral choanal atresia. Oropharyngeal airway in place. A string tie attaches the airway to the patient's clothing so it will not be lost.
October 1963
days the device is used by the infant as a pacifier. Neonatal nutrition is maintained by gavage. 2. In 2 to 3 days, the infant tolerates short absences of the airway during which cautious nipple feeding is begun. The ability to alternate breathing and feeding from the nipple is quickly learned. By l to 2 weeks of age supplemental gavage feedings may be discontinued. By 4 weeks of age the airway is no longer needed. Good nursing care is essential during this period to insure the presence of the airway when necessary and to guard against aspiration during the learning phase of eating. We have encountered no trouble in this regard and find that the nursing care involved is much less than that necessary in postoperative management of the newborn. 3. The parents are encouraged to observe the progress and are kept fully informed. Prior to discharge, the parents feed the infant until they feel secure in caring for the child at home. 4. Care thereafter is as for normal infants. Solids were introduced in our patients by the age of 3 months. 5. Our present plan is to operate at 5 to 6 years of age unless indicated earlier by facial structural alterations. CASE SUMMARIES
Fig. 2. Berman plastic oropharyngeal airway. We prefer a plastic airway of the type shown in Fig. 2. Some gagging usually occurs but tolerance is quickly attained. Within a few
Case 1. A Caucasian infant female had respiratory distress and cyanosis at birth. A diagnosis of bilateral choanal atresia was made after failure to pass nasal catheters. Distress was immediately alleviated by insertion of an oropharyngeal airway. Management was as described before. The airway was employed intermittently for 4 weeks. Garage supplementswere required for 1 week. After a hospital stay of 6 weeks she was returned to her home in Italy accompanied only by her mother. When the infant was 17 mouths old, the family returned to the United States from their European assignment. Her growth and development had been normal. She had had only one illness, a postvaccinal febrile episode. Her only symptom was a moderate, persistent nasal discharge. On physical examination, it was noted that
Volume 63 Number 4 part 1
she was unable to breathe through her nose. Mild hypertelorism was present (Figs. 3 and 4) as was a smalt well-healed ectodermal defect of the scalp, present since birth. Her weight was 24 pounds and her crown-heel length was 32I,/2 inches. Some widening of the nasal cavities was present bilaterally. H e r nasal turbinates were of normal size and the nasal mucosa was of normal
Bilateral choanal atresia
563
color. X-rays of the skull showed normal sinusoidal development. Case 2. A Caucasian female was referred to our care at the age of 10 days. Shortly after birth a stuffy nose was observed as well as mild respiratory difficulty during feedings. A clinical diagnosis of bilateral choanal atresia was made and confirmed by contrast x-ray study.
Figs. 3 and 4. Frontal and lateral faclal v{ews of child 19 months of age with bilateral choanal atresla managed conservatively from birth. Same patient as Fig. 1.
5 64
Erickson, Lodge, and Tomsovic
The child tolerated the obstruction without need of an airway. By the age of 4 weeks she was eating well and had gained weight adequately. She was discharged to home care. Follow-up by correspondence revealed that the child, at 2 years of age, had experienced normal growth and development. The mother thought the infant's weight gain during the first 12 months was slow. She gained from 6 pounds, 15 ounces at birth to 19 pounds at 1 year of age. By 2 years, her weight was 27 pounds. Illnesses reported by the mother included two "colds" with fever of 104~ and an episode of pharyngitis. The child tolerated these infections w ~ o u t undue trouble and was not hospitalized. The only persistent symptom was a nasal discharge which was not troublesome. She is under the care of a local pediatrician who has advised surgery at about 4 years of age. Case 3. A Caucasian infant male was noted to have excessive nasal secretions at birth and respiratory distress if not fed slowly. A diagnosis of bilateral choanal atresia was confirmed by contrast x-rays. By frequent slow, cautious feeding, the infant did well without need of an airway. He was referred to our care at the age of 1 month for surgical evaluation. Since no difficulties were noted, surgery was deferred. At the age of t9 months, growth and development were normal. The child had had no illnesses. The only complaint offered was that of mild persistent nasal discharge. Case 4. A Caucasian female was noted shortly after birth to have excessive nasal mucus and respiratory difficulty if not fed slowly. A clinical diagnosis of bilateral choanal atresia was confirmed by x-ray contrast study. The difficulties encountered were so mild that she was discharged to home care at the age of 4 days to await transportation to Walter Reed General Hospital for surgical evaluation. No distress was encountered; the child ate and gained well during an observation period. She was returned home without surgery. Unfortunately, contact with the family has been lost. DISCUSSION A l t h o u g h initial nonoperative m a n a g e m e n t of bilateral choanal atresia is m e n t i o n e d in several articles, we think medical m a n a g e m e n t as a routine has not been previously advocated. M c G o v e r n 7 has established that bilateral atresia does not alter sinus formation, nasal physiology, or hearing. This was
October 1963
verified in o u r cases. A n o s m i a is a possible p e r m a n e n t e n d result of conservative m a n agement. Craig a n d Simpson I speculate that the nose b r e a t h i n g habit m a y never be relearned once the m o u t h b r e a t h i n g habit has been established. T h e "adenoid-facies" appearance would seem to be a logical consequence b u t m a y be avoidable with judiciously timed surgery. O u r m e t h o d allows the advantages of technically easier surgery because of the larger size of the operative field, a less complicated postoperative period, and the child benefits from the reduced anesthetic risk which accompanies increased age. We have encountered no increased incidence of infection a n d regurgitation has been no problem. Growth a n d development have been x
normal. T h e preferred routes for surgery are transpalatal a n d transnasal. 6' 8, 9 Perhaps the increased size of structures afforded by this methods of m a n a g e m e n t would facilitate employment of the transnasal approach. SUMMARY A technique of initial medical m a n a g e m e n t of bilateral choanal atresia is described. Advantage was taken of the fact that affected n e w b o r n infants can be taught to breathe through the m o u t h a n d can learn to eat without special devices. T h e m e t h o d described is now preferred by the Pediatric Service of Walter Reed General Hospital. W e no longer consider bilateral choanal atresia to w a r r a n t emergency surgery.
REFERENCES 1. Craig, D. H., and Simpson, N. M.: Posterior choanal atresia, J. Laryng. & Otol. 73: 603, 1959. 2. Campbell, W., Stokes, D., and Jennings, R.: Choanal atresia--Report of five cases and a review of recent literature, J. M. Soc. New Jersey 58: 478, 1961. 3. Baker, D., Jr., Waltner, J., and Novick, W.: Posterior choanal atresia, Ann. Otol., Rhinol. & Laryng. 69: 805, 1960. 4. Beinfield, H. H.: Bilateral ehoanal atresia in the newborn--Surgical dangers to be avoided, A. M. A. Arch. Otolaryng. 73: 659, 1961.
Volume 63 Number 4 part 1
5. McGovern, F. H.: Bilateral choanal atresia in the newborn, A new method of medical management, Laryngoscope 71: 480, 1961. 6. McGovern, F. H., and Fitz-Hugh, G. S.: Surgical management of congenital choanal atresia, A. M. A. Arch. Otolaryng. 73: 627, 1961. 7. McGovern, F. H.: Congenital choanal atresia, Laryngoscope 60: 815, I950. 8. Wilson, C. P.: Treatment of choanal atresia, J. Laryng. & Otol. 71: 616, 1957. 9. Beinfield, H. H.: Surgery for bilateral bony atresia of the posterior nares in the newborn, A. M. A. Arch. Otolaryng. 70: 1, 1959. 10. Rickharn, P. P.: Diagnosis of congenital anomalies incompatible with life, but amenable to surgical correction, Pediat. Clin. North America 955: 977, 1959. 11. Morrow, R. C.: Bilateral congenital ehoanal atresia--A report of three cases corrected surgically in the newborn, Ann. Otol., Rhinol. & Laryng. 66: 135, 1957.
Bilateral choanal atresia
565
12. Beinfield, It. H.: Treatment of complete unilateral bony atresia of the posterior nares, A. M. A. Arch. Ototaryng. 53: 530, 1951. 13. Beinfield, H. H.: Congenital bilateral bony atresia of the posterior nares in a one-month old premature infant who survived, J. PEDIAT. 45: 679, 1954. 14. Beinfield, H. H.: A forgotten cause of infant suffocation, J. Internat. Coll. Surgeons 22: 447, 1954.
ADDENDUM The child shown in Figs. 1 and 2 was operated upon by the transpalatine route in 1963 by Dr. Joseph H. Hersh of New York City. A hard, bony lamina was present bilaterally. Results have been good postoperatively and the child has definite smell discrimination.