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Pitfalls in the treatment of the Pierre Robin Syndrome
Pitfalls in the Treatment of the Pierre Robin Syndrome By RONALDB. BERGGRENAND ROBERT J. DURAN HE METHOD FOR RELIEVING THE GLOSSOPTOSIS and resulting airway obstruction in infants with micrognathia is still debated in the literature.l-” The confusion resulting can lead to treatment that is inadequate or misdirected, and resultant complications may result in treatment failure and death. The clinical records of 28 children with micrognathia were reviewed. Particular attention was given to the records of patients who died as a result of treatment failure. These failures were usually due to incomplete understanding of the clinical problem or failure to diagnose associated anomalies.
T
CLINICAL
MATERIAL
Between July 1960 and December 1969, 28 patients with the diagnosis of micrognathia were admitted to either Children’s HospitaI or The Ohio State University Hospitals in Columbus. The children admitted could be divided into three age groups. A few who had no neonatal complications were admitted after the age of one year for repair of a cleft palate. A second group of patients was admitted between the ages of 4 months and 12 months. Although some of these children had had neonatal respiratory difficulty, the reasons for admission were failure to thrive, feeding problems, or recurrent pulmonary infections. Seventeen infants admitted under 4 months, all with respiratory obstruction, composed the third group. While 23 patients were reported to have had “respiratory distress” early in life, cyanosis was reported in only 12. Nineteen patients had an associated cleft palate. Five patients had multiple congenital anomalies and three of the deaths occurred as a result of other anomalies incompatible with Iife in these infants. Five patients died as a result of treatment failure: case histories will be presented in detail. CASE REPORTS Cnse I: This child was born with an obviously small mandible and obstruction of the upper airway. Attempts to deliver the tongue from the pharynx using a stitch placed through the tip of the tongue failed and the patient was transferred to us. We planned a tongue-lip adhesion (Beverly-Douglas procedure). After the insertion of the endotracheal tube, the respiratory distress was relieved. When the floor of the mouth was inspected, a tight frenulum was found and released. At this point it was possible to protract the previously tethered tongue out of the mouth. The patient died the next day from the complications of prolonged hypoxia, caused by unrelieved airway obstruction before the diagnosis of tongue-tie was made. Crrse 2: A child admitted the day after birth because of cyanotic episodes associated with feedings had a stitch inserted through the tip of the tongue and taped to the child’s chest. This technique afforded adequate relief of the airway obstruction. On the third From the Division of Plastic Surgery, Deparfment of Surgery, Ohio Skzte Uttiversily Hospilals and Children’s Hospital, Columbus, Ohio. RONALD B. BERGGREN, M.D.: Associate Professor of Surgery; Director, Di\~isiotl of Plastic Surgery, Ohio State University College of Medicine and ChiLdren’s Hospiral, Colutn bus, Ohio. ROBERT J. DURAN, M.D.: Clinical Ass&ant Professor of Surgery, Ohio Stare University, and Children’s Hospital, Columbus, Ohio. JOURNAL OF PEDIATRIC SURGERY, VOL. 5. No. 5 (OCTOBER).
1970
539
540
BERGGRENANDDURAN
day of life the tongue suture pulled out and was accompanied baby aspirated the blood and died from this complication.
by some bleeding.
The
Case 3: This child experienced severe respiratory distress and cyanosis immediately after birth and was transferred to our care within 24 hours. She too had a minor tongue-tie which was relieved. There was some improvement in the protraction of the tongue, but her symptoms were not completely relieved. She was maintained for several days with a nasotracheal tube. At 48 hours she had some difficulty with swallowing as well as weakness of the pharyngeal muscles. It was thought that brain damage had occurred by this time because of the repeated bouts of anoxia. She continued to have trouble with aspiration until the fourth month of life when recurrent pneumonia caused her death. It seems probable that this child’s brain damage was caused by the repeated episodes of airway obstruction and consequent anoxia. Case 4: A child born in The University Hospital and diagnosed as having micrognathia had minor airway obstruction, easily relieved by keeping him in a prone position. He had some slight difficulty when feeding, but did not aspirate and gained weight well. He was kept in the hospital for 4 weeks until it was apparent that his problem was not increasing in severity. The mother was taught to care for the child and was feeding the child without difficulty in the hospital. Two weeks after discharge the child was found dead in his crib. No cause of death could be found and an autopsy was not performed. It was assumed that death was caused by airway obstruction in the presence of a marginal upper airway. Case 5: The patient was born with an obvious micrognathia, upper airway obstruction, and agenesis of the sternum. The anterior chest was flail and the usual abnormalities in pulmonary mechanics were present. Hypoxia and cyanosis were felt to have resulted from upper airway obstruction and attempts were made to relieve this with tongue traction and the insertion of a palate prosthesis with an extension to hold the tongue forward. All of these measures failed and the child eventually developed pneumonia before being transferred to Children’s Hospital at the age of 5 weeks. The patient was severely ill at this time and failed to recover from the pneumonia. DISCUSSION
The mortality of micrognathia in newborn children has been reported to range from 20 to 65 per cent,2 but many of these deaths result from concomitant anomalies of even greater severity. A significant number of babies die because treatment for the mechanical airway obstruction fails. The obstetrician or pediatrician must always be alert to the possibility of upper airway obstruction in cases of micrognathia. This can be relieved by protraction of the tongue from the oropharynx with a sharp-pointed towel clip, taking precautions to grasp a good mass of tissue. If the tongue cannot be delivered from the mouth, other problems should be suspected and, in particular, the lingular frenulum should be checked. If tongue traction succeeds in relieving the obstruction, a more permanent method should be devised. While minimum degrees of obstruction can be relieved by placing the baby in the prone position, more severe deformities require the placement of a large silk tongue suture well back in the body of the tongue. A large bite of tissue is taken perpendicular to the long axis of the tongue and immediately anterior to the circumvallate papillae. The base of the tongue is thus moved forward, clearing the upper airway. The tongue stitch should not be used for more than 36 to 48 hours because it is likely to pull out of the tongue, allowing the obstruction to recur and perhaps add the complication of bleeding, as in Case 2. After 24 to 36 hours, a more permanent tether is necessary, and a tongue-lip
PIERRE
ROBIN
541
SYNDROME
adhesion should be carried out. This operation requires the development of flaps on the lower lip and inferior surface of the tongue, which are interdigitated. A horizontal mattress suture of 3-O nylon should be passed from beneath the chin, over a button at the base of the tongue and tied over a button beneath the chin. as described by Randall.6 To obtain optimal results, these two procedures must be used together. The adhesion can be released in 6 months, although the horizontal mattress suture can be removed earlier. Hadley and Johnson7 described the use of a Kirschner wire to transfix the tongue.
Later
Schatten
and Tidmore”
reported
its use in four cases with good
results. It is a simpler procedure than the tongue-lip adhesion, but must be done with great care to avoid injuring important contiguous structures and to insure successful tongue position. Two recent advances have been recommended for the care of these patients. Extended nasotracheal intubation has been suggested but cannot be recommended for more than a few days because the incidence of associated tracheal stenosis increases. Although the newer plastic tubes are better tolerated, the additional supervision required and the complications that occur would seem to prohibit their use.” It has been suggested that a palate prosthesis be fashioned with a posterior extension to hold the base of the tongue forward,g but there appears to be an inherent danger in this technique. In patients with a severe deformity, the mouth is crowded with tissue for which there is too little room. The introduction of more material may further compromise the airway and significantly interfere
with swallowing
be recommended. Although these children
and feeding. may require
At the present a tracheostomy,
time this method
cannot
this procedure
must be
undertaken with full knowledge and understanding of the immediate and delayed problems. Failure to diagnose abnormalities in respiratory function not associated with the upper airway obstruction can lead to death of the child as in Case 5. In this instance it probably would have been wiser to insert a tracheostomy and place the patient on positive pressure respiratory assistance to overcome the abnormalities of respiratory mechanics secondary to the flail chest. In spite of the fact that the jaw tends to grow less rapidly than the tongue during the first 2 months of life, the occasional child can be maintained satisfactorily under close supervision in the hospita1.r After discharge from the hospital he may get into significant difficulty in a less well-controlled environment. The child cannot be considered to be out of danger of airway obstruction until he is 6 to 8 weeks of age. Therefore, a tongue-lip adhesion should seriously be considered in all children who have had some degree of respiratory obstruction, cyanosis, and an intra-alveolar difference of greater than 1 cm. Case 4 represents such a patient. In general, the choice of treatment for these patients must be made upon careful observation and good clinical judgment. If the child can maintain his airway while asleep and has had no episodes of cyanosis, surgery is probably not necessary.
542
BERGGREN AND DURAN SUMMARY
Five possibly preventable deaths occurred in a series of 28 children with the Pierre Robin Syndrome. The primary goal in management is to move the base of the tongue from the pharynx. If this cannot be done easily, the reason, such as a tight lingular frenulum, should be found. A tongue traction stitch may be adequate to relieve the obstruction, but depending upon it for long periods of time is dangerous. The most adequate tonguelip adhesion includes a mattress suture from the base of the tongue to beneath the chin. Patients with severe micrognathia who can be maintained in a prone position should have tongue-lip adhesion before discharge from the hospital to prevent acute airway obstruction subsequently. The Pierre Robin Syndrome is not incompatible with life, but does require good clinical judgement and careful attention to detail if treatment is to be successful. REFERENCES 1. Routledge, R. T.: The Pierre Robin syndrome: A surgical emergency in the neonatal period. Brit. I. Plast. Surg. 13:204, 1960. 2. Goldberg, M. H., and Echblum, R. H.: The treatment of the Pierre Robin syndrome. Pediatrics 36:336, 1965. 3. Robin, P.: Glossoptosis due to atresia and hypertrophy of the mandible. Amer. J. Dis. Child. 48:541, 1934. 4. Dennison, W. M.: The Pierre Robin syndrome. Pediatrics 36:336, 1965. 5. Pielow, W. D.: Non-surgical management of Pierre Robin syndrome. Arch. Dis. Child. 42:20, 1967. 6. Randall, P.: Micrognathia and glossop-
tosis with airway obstruction: The Pierre Robin syndrome. In Converse, J. M. (Ed.): Reconstructive Plastic Surgery, Vol. III. Philadelphia, Saunders, 1965, pp. 14521462. 7. Hadley, R. C., and Johnson, J. B.: Utilization of the Kirschner wire in Pierre Robin syndrome. Plast. Reconstr. Surg. 31: 587, 1963. 8. Schatten, W. E., and Tidmore, T. L., Jr.: Airway management in patients with Pierre Robin syndrome. Plast. Reconstr. Surg. 38:309, 1966. 9. VerMeulen, V. R., and Birck, H.: Prolonged intubation versus tracheotomy in children. Arch. Otolaryng. 87:56, 1968.
T
CLINICAL
MATERIAL
Between July 1960 and December 1969, 28 patients with the diagnosis of micrognathia were admitted to either Children’s HospitaI or The Ohio State University Hospitals in Columbus. The children admitted could be divided into three age groups. A few who had no neonatal complications were admitted after the age of one year for repair of a cleft palate. A second group of patients was admitted between the ages of 4 months and 12 months. Although some of these children had had neonatal respiratory difficulty, the reasons for admission were failure to thrive, feeding problems, or recurrent pulmonary infections. Seventeen infants admitted under 4 months, all with respiratory obstruction, composed the third group. While 23 patients were reported to have had “respiratory distress” early in life, cyanosis was reported in only 12. Nineteen patients had an associated cleft palate. Five patients had multiple congenital anomalies and three of the deaths occurred as a result of other anomalies incompatible with Iife in these infants. Five patients died as a result of treatment failure: case histories will be presented in detail. CASE REPORTS Cnse I: This child was born with an obviously small mandible and obstruction of the upper airway. Attempts to deliver the tongue from the pharynx using a stitch placed through the tip of the tongue failed and the patient was transferred to us. We planned a tongue-lip adhesion (Beverly-Douglas procedure). After the insertion of the endotracheal tube, the respiratory distress was relieved. When the floor of the mouth was inspected, a tight frenulum was found and released. At this point it was possible to protract the previously tethered tongue out of the mouth. The patient died the next day from the complications of prolonged hypoxia, caused by unrelieved airway obstruction before the diagnosis of tongue-tie was made. Crrse 2: A child admitted the day after birth because of cyanotic episodes associated with feedings had a stitch inserted through the tip of the tongue and taped to the child’s chest. This technique afforded adequate relief of the airway obstruction. On the third From the Division of Plastic Surgery, Deparfment of Surgery, Ohio Skzte Uttiversily Hospilals and Children’s Hospital, Columbus, Ohio. RONALD B. BERGGREN, M.D.: Associate Professor of Surgery; Director, Di\~isiotl of Plastic Surgery, Ohio State University College of Medicine and ChiLdren’s Hospiral, Colutn bus, Ohio. ROBERT J. DURAN, M.D.: Clinical Ass&ant Professor of Surgery, Ohio Stare University, and Children’s Hospital, Columbus, Ohio. JOURNAL OF PEDIATRIC SURGERY, VOL. 5. No. 5 (OCTOBER).
1970
539
540
BERGGRENANDDURAN
day of life the tongue suture pulled out and was accompanied baby aspirated the blood and died from this complication.
by some bleeding.
The
Case 3: This child experienced severe respiratory distress and cyanosis immediately after birth and was transferred to our care within 24 hours. She too had a minor tongue-tie which was relieved. There was some improvement in the protraction of the tongue, but her symptoms were not completely relieved. She was maintained for several days with a nasotracheal tube. At 48 hours she had some difficulty with swallowing as well as weakness of the pharyngeal muscles. It was thought that brain damage had occurred by this time because of the repeated bouts of anoxia. She continued to have trouble with aspiration until the fourth month of life when recurrent pneumonia caused her death. It seems probable that this child’s brain damage was caused by the repeated episodes of airway obstruction and consequent anoxia. Case 4: A child born in The University Hospital and diagnosed as having micrognathia had minor airway obstruction, easily relieved by keeping him in a prone position. He had some slight difficulty when feeding, but did not aspirate and gained weight well. He was kept in the hospital for 4 weeks until it was apparent that his problem was not increasing in severity. The mother was taught to care for the child and was feeding the child without difficulty in the hospital. Two weeks after discharge the child was found dead in his crib. No cause of death could be found and an autopsy was not performed. It was assumed that death was caused by airway obstruction in the presence of a marginal upper airway. Case 5: The patient was born with an obvious micrognathia, upper airway obstruction, and agenesis of the sternum. The anterior chest was flail and the usual abnormalities in pulmonary mechanics were present. Hypoxia and cyanosis were felt to have resulted from upper airway obstruction and attempts were made to relieve this with tongue traction and the insertion of a palate prosthesis with an extension to hold the tongue forward. All of these measures failed and the child eventually developed pneumonia before being transferred to Children’s Hospital at the age of 5 weeks. The patient was severely ill at this time and failed to recover from the pneumonia. DISCUSSION
The mortality of micrognathia in newborn children has been reported to range from 20 to 65 per cent,2 but many of these deaths result from concomitant anomalies of even greater severity. A significant number of babies die because treatment for the mechanical airway obstruction fails. The obstetrician or pediatrician must always be alert to the possibility of upper airway obstruction in cases of micrognathia. This can be relieved by protraction of the tongue from the oropharynx with a sharp-pointed towel clip, taking precautions to grasp a good mass of tissue. If the tongue cannot be delivered from the mouth, other problems should be suspected and, in particular, the lingular frenulum should be checked. If tongue traction succeeds in relieving the obstruction, a more permanent method should be devised. While minimum degrees of obstruction can be relieved by placing the baby in the prone position, more severe deformities require the placement of a large silk tongue suture well back in the body of the tongue. A large bite of tissue is taken perpendicular to the long axis of the tongue and immediately anterior to the circumvallate papillae. The base of the tongue is thus moved forward, clearing the upper airway. The tongue stitch should not be used for more than 36 to 48 hours because it is likely to pull out of the tongue, allowing the obstruction to recur and perhaps add the complication of bleeding, as in Case 2. After 24 to 36 hours, a more permanent tether is necessary, and a tongue-lip
PIERRE
ROBIN
541
SYNDROME
adhesion should be carried out. This operation requires the development of flaps on the lower lip and inferior surface of the tongue, which are interdigitated. A horizontal mattress suture of 3-O nylon should be passed from beneath the chin, over a button at the base of the tongue and tied over a button beneath the chin. as described by Randall.6 To obtain optimal results, these two procedures must be used together. The adhesion can be released in 6 months, although the horizontal mattress suture can be removed earlier. Hadley and Johnson7 described the use of a Kirschner wire to transfix the tongue.
Later
Schatten
and Tidmore”
reported
its use in four cases with good
results. It is a simpler procedure than the tongue-lip adhesion, but must be done with great care to avoid injuring important contiguous structures and to insure successful tongue position. Two recent advances have been recommended for the care of these patients. Extended nasotracheal intubation has been suggested but cannot be recommended for more than a few days because the incidence of associated tracheal stenosis increases. Although the newer plastic tubes are better tolerated, the additional supervision required and the complications that occur would seem to prohibit their use.” It has been suggested that a palate prosthesis be fashioned with a posterior extension to hold the base of the tongue forward,g but there appears to be an inherent danger in this technique. In patients with a severe deformity, the mouth is crowded with tissue for which there is too little room. The introduction of more material may further compromise the airway and significantly interfere
with swallowing
be recommended. Although these children
and feeding. may require
At the present a tracheostomy,
time this method
cannot
this procedure
must be
undertaken with full knowledge and understanding of the immediate and delayed problems. Failure to diagnose abnormalities in respiratory function not associated with the upper airway obstruction can lead to death of the child as in Case 5. In this instance it probably would have been wiser to insert a tracheostomy and place the patient on positive pressure respiratory assistance to overcome the abnormalities of respiratory mechanics secondary to the flail chest. In spite of the fact that the jaw tends to grow less rapidly than the tongue during the first 2 months of life, the occasional child can be maintained satisfactorily under close supervision in the hospita1.r After discharge from the hospital he may get into significant difficulty in a less well-controlled environment. The child cannot be considered to be out of danger of airway obstruction until he is 6 to 8 weeks of age. Therefore, a tongue-lip adhesion should seriously be considered in all children who have had some degree of respiratory obstruction, cyanosis, and an intra-alveolar difference of greater than 1 cm. Case 4 represents such a patient. In general, the choice of treatment for these patients must be made upon careful observation and good clinical judgment. If the child can maintain his airway while asleep and has had no episodes of cyanosis, surgery is probably not necessary.
542
BERGGREN AND DURAN SUMMARY
Five possibly preventable deaths occurred in a series of 28 children with the Pierre Robin Syndrome. The primary goal in management is to move the base of the tongue from the pharynx. If this cannot be done easily, the reason, such as a tight lingular frenulum, should be found. A tongue traction stitch may be adequate to relieve the obstruction, but depending upon it for long periods of time is dangerous. The most adequate tonguelip adhesion includes a mattress suture from the base of the tongue to beneath the chin. Patients with severe micrognathia who can be maintained in a prone position should have tongue-lip adhesion before discharge from the hospital to prevent acute airway obstruction subsequently. The Pierre Robin Syndrome is not incompatible with life, but does require good clinical judgement and careful attention to detail if treatment is to be successful. REFERENCES 1. Routledge, R. T.: The Pierre Robin syndrome: A surgical emergency in the neonatal period. Brit. I. Plast. Surg. 13:204, 1960. 2. Goldberg, M. H., and Echblum, R. H.: The treatment of the Pierre Robin syndrome. Pediatrics 36:336, 1965. 3. Robin, P.: Glossoptosis due to atresia and hypertrophy of the mandible. Amer. J. Dis. Child. 48:541, 1934. 4. Dennison, W. M.: The Pierre Robin syndrome. Pediatrics 36:336, 1965. 5. Pielow, W. D.: Non-surgical management of Pierre Robin syndrome. Arch. Dis. Child. 42:20, 1967. 6. Randall, P.: Micrognathia and glossop-
tosis with airway obstruction: The Pierre Robin syndrome. In Converse, J. M. (Ed.): Reconstructive Plastic Surgery, Vol. III. Philadelphia, Saunders, 1965, pp. 14521462. 7. Hadley, R. C., and Johnson, J. B.: Utilization of the Kirschner wire in Pierre Robin syndrome. Plast. Reconstr. Surg. 31: 587, 1963. 8. Schatten, W. E., and Tidmore, T. L., Jr.: Airway management in patients with Pierre Robin syndrome. Plast. Reconstr. Surg. 38:309, 1966. 9. VerMeulen, V. R., and Birck, H.: Prolonged intubation versus tracheotomy in children. Arch. Otolaryng. 87:56, 1968.