International Journal of Pediatric Otorhinolatyngology, 22 (1991) 29-37 0 1991 Elsevier Science Publishers B.V. 016.5-5876/91/$03.50
PEDOT
29
00722
Management
of airway obstruction in the Pierre Robin sequence Bruce Benjamin
and Paul Walker
Royal Alexandra Hospital for Children, Camperdown, N.S. W 2050 (Australia i
(Revised
Key words: Pierre
Robin
sequence;
(Received 8 June 1990) version received 25 January (Accepted 30 January 1991)
Airway
obstruction;
1991)
Tracheotomy
Abstract
A lo-year retrospective study of the management of airway obstruction in 26 infants with Pierre Robin sequence was made. It was not possible when the infant was first seen to assess the future severity of the airway obstnlction but later 3 distinct groups could be identified according to the airway management which had become necessary. The deaths from respiratory obstruction indicate the prime importance of airway management. Laryngoscopy for intubation or endoscopic evaluation was often difficult and sometimes could not be achieved. ‘Awake intubation’ without general anesthesia has proved to be safer and less difficult using a special purpose slotted laryngoscope. Airway management should be individualized following the progressive sequence of posturing in the prone position, nasopharyngeal tube, endotracheal intubation and tracheotomy until successful control is achieved as indicated by the clinical features and pulse oximetry.
Introduction
The diagnostic criteria for the Pierre Robin sequence (PRS) are micrognathia, glossoptosis, and incomplete cleft palate [21], although an occasional patient may not have one of these features. PRS presents as an isolated anomaly, as part of a syndrome or as Stickler’s syndrome. Estimates of incidence range from 1 : 2000 to 1 : 50000 births [lo]. Upper airway obstruction in infants with PRS is common and usually considered to be due to mechanical factors [6,11]. The severity is less in the prone Correspondence:
B. Benjamin,
231 Macquarie
Street,
Sydney,
N.S.W. 2050, Australia
30
position as the tongue falls forward partly relieving pharyngeal obstruction. Severe obstruction may cause cyanotic episodes, cerebral hypoxia, aspiration pneumonitis, pulmonary hypertension, car pulmonale, or fatal asphyxia [10,20,22]. Robin [21] described the airway obstruction as a passive phenomenon in which the retroposed tongue flops into the posterior oropharynx to occlude the airway. Those who support the widely accepted ‘mechanistic’ explanation of airway obstruction postulate that the tongue is actively ‘sucked’ posteriorly by negative hypopharyngeal pressure generated during inspiration and swallowing [4,6,11,12,19]. Wedging of the tongue into the cleft palate may further contribute to the airway obstruction [22]. Others challenge the ‘mechanistic’ hypothesis and support the view that there is a central factor contributing to apnoea, obstruction and hypoxia with vagal overactivity and esophagogastric motor abnormalities [7], possibly from brainstem immaturity. The larynx in an infant with micrognathia and glossoptosis is under the base of the tongue (Fig. 1) making direct laryngoscopy and intubation difficult and sometimes impossible [1,2]. Many procedures to manage the airway obstruction have been described [4,8,9,13-16,19,23,24] but no one technique is satisfactory for all cases. In some infants tracheotomy may prove to be the only dependable means to relieve obstruction. This study was to reassess policy for the management of airway obstruction in PRS based on experience gained over a lo-year period and from this to suggest a rational progressive sequence for airway management.
Materials
and methods
The records of all infants with PRS admitted to the Royal Alexandra Hospital for Children for management of airway obstruction during the lo-year period 1977-1987 were reviewed. Data examined included complications during pregnancy, family history of micrognathia or cleft palate, birth weight, presence of a cleft soft or hard palate, other congenital anomalies and in particular time of onset of airway obstruction, airway management, duration of treatment, complications caused by airway obstruction and morbidity and mortality. Although the ‘severity’ of micrognathia was usually recorded, no uniform objective measurements were documented; we therefore concluded that there was serious doubt about the validity of such random subjective assessment. All infants were admitted to a neonatal intensive care ward. The methods used to support the airway included nursing in the prone position, nasopharyngeal tube, endotracheal tube, glossopexy and tracheotomy.
Results
Twenty-six infants with PRS were admitted for management tion. There were 15 males and 11 females.
of their obstruc-
31
Fig. 1. Lateral xerogram showing glossoptosis and a hypermobile tongue with a narrow and unstable origin.
Eight were born following a complicated pregnancy-2 oligohydramnios, 2 polyhydramnios, 1 threatened miscarriage, 1 pre-eclampsia, 1 with falling oestriol levels requiring induction, and 1 ante-partum hemorrhage. A family history of micrognathia or cleft palate was present in 8. Birth weight ranged from 900 to 4300 g (mean 3000 g). Three were premature births. A cleft soft palate was present in 25 and of these, 13 also had a cleft hard palate. In 14 the syndrome was regarded as an isolated anomaly and in 12 there were other anomalies (Table I). (It now seems likely, that in the light of current knowledge a more comprehensive evaluation of the congenital anomalies could be made.)
32 TABLE I Presence or absence of associated anomaliqs
14
PRS the only anomaly Associated syndrome Stickler Foetal alcohol Deletion chrom. 4
3 1 1
5 7
Associated non-syndromal defects
26
Total
TABLE II Time after birth to onset of symptoms Duration of time
n of patients
Within 2 hours Within 6 hours Within 12 hours Within 5 days Not noted
16 I 1 1 1
Total
26
Airway obstruction
Airway obstruction developed within 6 h of birth in 23 patients (Table II). Three distinct groups were identified according to the airway management which ultimately became necessary-these groups can be said to represent the severity of airway obstruction according to the eventual outcome. (Table III). In the mild group the age at which the infant could be nursed supine without significant obstruction while awake ranged from 1 to 7 months (mean 2.9 months). The age at which the infant could sleep supine ranged from 3 to 12 months (mean 5.6 months). In the moderate group the time at which a nasopharyngeal airway was first used ranged from one hour to 20 days and duration of placement of the tube ranged
TABLE III Secerity and management of airway obstruction according to eventual outcome Group
Management
Mild
Posture alone
8 (31)
Moderate
Nasopharyngeal tube
7 (27)
Severe
Endotracheal
n (%)
intubation or tracheotomy or death
11 (42)
33
from 14 days to 14 weeks. After tube removal the infants were nursed prone. The age at which the infants could sleep supine without significant obstruction ranged from 5 to 6 months. In the severe group of 11 patients 5 were managed by endotracheal intubation, 3 by tracheotomy and 3 by other means. Of the 5 managed by endotracheal intubation 3 were extubated after 10 days to 6 weeks and 2 died while still intubated. Three underwent tracheotomy; 2 later died of other causes and 1 survived. One of these had had a glossopexy but the airway obstruction was not relieved and a tracheotomy became necessary. The remaining 3 in the severe group were assessed as requiring more than nasopharyngeal intubation. In 2 the trachea could not be intubated; one was cyanosed at birth and died of hypoxic cerebral damage, and the other had a planned glossopexy abandoned after failure to intubate. In the third patient tracheotomy was planned but not performed because ‘general anesthesia was considered too great a risk’, yet eventually the infant survived. The 11 patients in the severe group proved to be difficult management problems and accounted for all 5 deaths in the series. The severe group were more likely to have been recorded as severe micrognathia (8 of ll>, to have associated congenital anomalies (5 of 11) and to develop complications due to airway obstruction (10 of 11). Early onset of airway obstruction showed no association with either severity of obstruction nor development of complications. Laryngoscopy for intubation was difficult in all patients on whom it was attempted and was not achieved in two patients. The trachea and main bronchi were examined with telescopes in most infants who had laryngoscopy, but no tracheal abnormalities were observed. Complications
Twelve infants developed complications. Eight had car pulmonale and of these, 5 had known congenital heart disease. Three newborn babies required resuscitation for cyanosis and intubation proved very difficult or impossible-all 3 suffered hypoxic cerebral damage. A fourth baby had multiple respiratory arrests due to obstruction, but no neurological sequelae. Deaths
There were 5 deaths. Age at death ranged from 4 days to 6 months. Three deaths were due to respiratory failure, 1 was due to heart failure, and 1 followed withdrawal of ventilatory support in a premature neonate with a massive intracerebra1 hemorrhage.
Discussion
PRS is a congenital anomaly categorized as isolated Pierre Robin sequence, as Pierre Robin with multiple anomalies or as Sticklers sequence and often associated
34
with serious upper airway obstruction which may be life-threatening. The obstruction is usually manifest within a few hours of birth but the onset may be delayed. It is not possible at first to assess the future severity of the airway problem and repeated observation in the neonatal intensive care ward for hours or many days is needed to determine which method of management is required. Monitoring by pulse oximetry is performed in every case [12]. A reasonable guide seems to be to have the oxygen saturation in room air over 90% for at least 90% of the time. Airway management must be individualized until successful control is achieved and should follow the progressive sequence of: (11 Posturing in the prone position. (2) Nasopharyngeal tube. (3) Endotracheal intubation. (4) Tracheotomy. The method may partly depend on the severity of associated cardiac and other anomalies. Posturing prone is the most important initial part of treatment; the tongue falls forward partly clearing the oropharyngeal airway. Posturing can be discontinued when the child is able to maintain the airway while sleeping supine-at about 5 or 6 months of age in the mild to moderate groups. Nasogastric tube feeding to minimize aspiration may be necessary in some patients. When posturing does not control the airway, a nasopharyngeal tube should be used [19]. The tube is passed through one nasal cavity and positioned by clinical testing and X-ray control so the tip is below the level of the base of the tongue but above the larynx (Fig. 2). The tube not only provides an airway through its own lumen but also creates a space at the base of the tongue on either side of the tube minimising the build-up of negative hypopharyngeal pressure. Posturing may still be necessary. Regular review and trial removal determine the appropriate time to discontinue use of the tube. Olson et al. 1191 state that with cooperative parents early home management of a baby with a nasopharyngeal tube is safe and acceptable. A variety of operative techniques to secure the tongue forward have been described, using sutures [8,9,18], wire [13], various obturators, traction devices [16,24] and fixation to the hyoid [14]. We have had little experience with these methods. If positioning prone and use of a nasopharyngeal tube fail to provide a reliable airway, prolonged endotracheal intubation or tracheotomy should be considered. Endotracheal intubation performed under controlled conditions will provide an excellent temporary airway but there are disadvantages. Laryngoscopy for intubation is always difficult and sometimes impossible without special purpose laryngoscopes. The patency of the tube must be carefully maintained, accidental extubation may occur with failure to reintubate, and laryngeal trauma from prolonged intubation is a possibility. We are now convinced that endotracheal intubation is more safely performed as an ‘awake intubation’ without general anesthesia but with cooperation between the anesthetist and the endoscopist to ensure adequate oxygenation. Intubation using standard pediatric laryngoscopes is difficult. Visualisation of the larynx for intubation is less difficult using a special purpose slotted laryngoscope with a slender barrel-the Holinger pediatric anterior commissure laryngoscope (Karl Storz,
Fig. 2. Lateral
X-ray
showing small mandible positioned
and a nasopharyngeal
tube supporting
the tongue and
so that the distal tip lies above the larynx.
Tuttlingen, F.R.G.) for term, normal weight babies or the smaller HolingerTucker-Benjamin laryngoscope (Karl Storz, Tuttlingen, F.R.G.) for premature or small babies. In an emergency, traction using a toothed forcep or a suture to pull the tongue forward will immediately improve the airway. If other techniques have failed tracheotomy will successfully bypass the obstruction. It is a safe and deuendable orocedure in small infants i3.51. The decision for
36
tracheotomy is based upon episodes of cyanosis observed clinically and desaturation measured objectively and there should be no reluctance or delay in performing the tracheotomy. Age at successful decannulation ranges from 3 to 18 months [17]. Management of the airway in PRS requires constant careful observation by nursing and medical staff experienced in assessing the severity of airway obstruction.
Conclusion The onset of airway obstruction in newborn infants with PRS usually occurs within a few hours of birth but it is not possible at first to assess the future severity. Monitoring clinically and by pulse oximetry should be done in a neonatal intensive care unit. A reasonable guide is to have the oxygen saturation above 90% for at least 90% of the time. Management of airway obstruction requires frequent reassessment, according to the difficulty with each patient, following the sequence of posture, nasopharyngeal tube or endotracheal tube. If these techniques fail, tracheotomy becomes necessary. Laryngoscopy and intubation is always difficult and is more safely performed without general anesthesia, using special purpose slotted laryngoscopes. In an emergency use of an oro-pharyngeal tube or an oro-esophageal tube or tongue traction using a forcep or a suture will be effective to restore an airway. Posturing in the prone position may be needed until 6 months of age. With optimal care there should be a low incidence of hypoxic cerebral damage and death.
References 1 Benjamin, B.N.P., Laryngoscopy. In S.L. Gans (Ed.), Pediatric Endoscopy, Grune and Stratton, New York, 1983, pp. 17-36. 2 Benjamin, B.N.P., Technique of laryngoscopy, Int. J. Pediatr. Otorhinolaryngol., 13 (1987) 299-313. 3 Benjamin, B.N.P. and Curley, J., Infant tracheotomy: endoscopy and decannulation, Int. J. Pediatr. Otorhinolaryngol., 20 (1990) 113-121. 4 Bull, M.J., Givan, D.C., Savove, A.M., Bixter, D. and Hearn, D., Improved outcome in Pierre Robin sequence: effect of multidisciplinary evaluation and management, Pediatrics, 86 (1990) 294-301. 5 Carter, P. and Benjamin, B.N.P., Ten-year review of pediatric tracheotomy, Ann. Otol. Rhinol. Laryngol., 92 (1983) 398-400. 6 Clarke, L., Hepworth, W.B., Carey, J.C. and Seegmiller, R.E., Chondrodystrophic mice with coincidental agnathia: evidence for the tongue obstruction hypothesis in cleft palate, Teratology, 38 (1988) 565-570. 7 Couly, G., Cheron, G., de-Blic, J., Despres, C., Cloup, M. and Hubert, P., Le syndrome de Pierre Robin. Classification et nouvelle approche therapeutique, Arch. Fr. Pediatr., 45 (1988) 553-559. 8 Douglas, B., A further report on the treatment of micrognathia associated with obstruction by a plastic procedure, Plast. Reconstr. Surg., 5 (1950) 113-122.
37 9 Duhamel, B., Chirurgie du Nouveau-& du Not&son, Masson et Cie., Paris, 1953, p. 55. 10 Dykes, E.H., Raine, P.A.M., Arthur, D.S., Drainer, I.K. and Young, D.G., Pierre Robin syndrome and pulmonary hypertension, J. Ped. Surg., 20 (1985) 49952. 11 Fletcher, M.M., Blum, S.L. and Blanchard, C.L., Pierre Robin syndrome pathophysiology of obstructive episodes, Laryngoscope, 79 (1969) 547-560. 12 Freed, G., Pearlman, M.A., Brown, AS. and Barot, L.R., Polysomnographic indications for surgical intervention in Pierre Robin sequence: acute airway management and follow-up studies after repair and take-down of tongue-lip adhesion, Cleft Palate J., 25 (1988) 151-155. 13 Hadley, R.C. and Johnson, J.B., Utilisation of the Kirschner wire in Pierre Robin syndrome, with case report, Plast. Reconstr. Surg., 31 (1963) 587-596. 14 Lapidot, A., Resvanti, F., Terrefe, D. and Ben-Hur, N., A new functional approach to the surgical management of Pierre Robin syndrome: experimental and clinical report, Laryngoscope, 86 (1976) 979-983. 15 Lewis, M.B. and Pashayan, H.M., Management of infants with Robin anomaly, Clin. Pediatr., 19 ( 1980) 5 19-528. 16 Longmire Jr., W.P. and Sanford, M.C., Stimulation of mandibular growth in congenital micrognathia by traction, Am. J. Dis. Child., 78 (1949) 750-754. 17 McEvitt, W.G.. Micrognathia and its management, Plast. Reconstr. Surg., 41 (1968) 450-455. 18 Oeconomopoulos, CT., The value of glossopexy in Pierre Robin syndrome, N. Engl. J. Med., 262 (1960) 1267-1268. 19 Olson, T.S., Kearns, D.B., Pransky, S.M. and Seid, A.B., Early home management of patients with Pierre Robin sequence, Int. J. Pediatr. Otorhinolaryngol., 20 (1990) 45-49. 20 Pashayan, H.M. and Lewis, M.B., Clinical experience with the Robin sequence, Cleft Palate J.. 21 (I 984) 270-276. 21 Robin, P., Glossoptosis due to atresia and hypotrophy of the mandible, Am. J. Dis. Child.. 48 (1934) 541-547. 22 Routledge, R.T., The Pierre Robin syndrome: a surgical emergency in the neonatal period, Br. J. Plast. Surg., 13 (1960) 204-218. 23 Stern, L..M., Fonkalsrud, E.W., Hassakis, P. and Jones, M.H., Management of Pierre Robin syndrome by prolonged nasoesophageal intubation, Am. J. Dis. Child., 124 (1972) 78-80. 24 Wada, T., Ishi, T., Sugai, T., Molla, M.R., Matsuya, T., Miyazaki, T. and Koh, Y., Mandibular traction for relieving respiratory distress in the Pierre Robin anomaly, J. Max.-fat. Surg., I1 (1983) 187-190.
PEDOT
29
00722
Management
of airway obstruction in the Pierre Robin sequence Bruce Benjamin
and Paul Walker
Royal Alexandra Hospital for Children, Camperdown, N.S. W 2050 (Australia i
(Revised
Key words: Pierre
Robin
sequence;
(Received 8 June 1990) version received 25 January (Accepted 30 January 1991)
Airway
obstruction;
1991)
Tracheotomy
Abstract
A lo-year retrospective study of the management of airway obstruction in 26 infants with Pierre Robin sequence was made. It was not possible when the infant was first seen to assess the future severity of the airway obstnlction but later 3 distinct groups could be identified according to the airway management which had become necessary. The deaths from respiratory obstruction indicate the prime importance of airway management. Laryngoscopy for intubation or endoscopic evaluation was often difficult and sometimes could not be achieved. ‘Awake intubation’ without general anesthesia has proved to be safer and less difficult using a special purpose slotted laryngoscope. Airway management should be individualized following the progressive sequence of posturing in the prone position, nasopharyngeal tube, endotracheal intubation and tracheotomy until successful control is achieved as indicated by the clinical features and pulse oximetry.
Introduction
The diagnostic criteria for the Pierre Robin sequence (PRS) are micrognathia, glossoptosis, and incomplete cleft palate [21], although an occasional patient may not have one of these features. PRS presents as an isolated anomaly, as part of a syndrome or as Stickler’s syndrome. Estimates of incidence range from 1 : 2000 to 1 : 50000 births [lo]. Upper airway obstruction in infants with PRS is common and usually considered to be due to mechanical factors [6,11]. The severity is less in the prone Correspondence:
B. Benjamin,
231 Macquarie
Street,
Sydney,
N.S.W. 2050, Australia
30
position as the tongue falls forward partly relieving pharyngeal obstruction. Severe obstruction may cause cyanotic episodes, cerebral hypoxia, aspiration pneumonitis, pulmonary hypertension, car pulmonale, or fatal asphyxia [10,20,22]. Robin [21] described the airway obstruction as a passive phenomenon in which the retroposed tongue flops into the posterior oropharynx to occlude the airway. Those who support the widely accepted ‘mechanistic’ explanation of airway obstruction postulate that the tongue is actively ‘sucked’ posteriorly by negative hypopharyngeal pressure generated during inspiration and swallowing [4,6,11,12,19]. Wedging of the tongue into the cleft palate may further contribute to the airway obstruction [22]. Others challenge the ‘mechanistic’ hypothesis and support the view that there is a central factor contributing to apnoea, obstruction and hypoxia with vagal overactivity and esophagogastric motor abnormalities [7], possibly from brainstem immaturity. The larynx in an infant with micrognathia and glossoptosis is under the base of the tongue (Fig. 1) making direct laryngoscopy and intubation difficult and sometimes impossible [1,2]. Many procedures to manage the airway obstruction have been described [4,8,9,13-16,19,23,24] but no one technique is satisfactory for all cases. In some infants tracheotomy may prove to be the only dependable means to relieve obstruction. This study was to reassess policy for the management of airway obstruction in PRS based on experience gained over a lo-year period and from this to suggest a rational progressive sequence for airway management.
Materials
and methods
The records of all infants with PRS admitted to the Royal Alexandra Hospital for Children for management of airway obstruction during the lo-year period 1977-1987 were reviewed. Data examined included complications during pregnancy, family history of micrognathia or cleft palate, birth weight, presence of a cleft soft or hard palate, other congenital anomalies and in particular time of onset of airway obstruction, airway management, duration of treatment, complications caused by airway obstruction and morbidity and mortality. Although the ‘severity’ of micrognathia was usually recorded, no uniform objective measurements were documented; we therefore concluded that there was serious doubt about the validity of such random subjective assessment. All infants were admitted to a neonatal intensive care ward. The methods used to support the airway included nursing in the prone position, nasopharyngeal tube, endotracheal tube, glossopexy and tracheotomy.
Results
Twenty-six infants with PRS were admitted for management tion. There were 15 males and 11 females.
of their obstruc-
31
Fig. 1. Lateral xerogram showing glossoptosis and a hypermobile tongue with a narrow and unstable origin.
Eight were born following a complicated pregnancy-2 oligohydramnios, 2 polyhydramnios, 1 threatened miscarriage, 1 pre-eclampsia, 1 with falling oestriol levels requiring induction, and 1 ante-partum hemorrhage. A family history of micrognathia or cleft palate was present in 8. Birth weight ranged from 900 to 4300 g (mean 3000 g). Three were premature births. A cleft soft palate was present in 25 and of these, 13 also had a cleft hard palate. In 14 the syndrome was regarded as an isolated anomaly and in 12 there were other anomalies (Table I). (It now seems likely, that in the light of current knowledge a more comprehensive evaluation of the congenital anomalies could be made.)
32 TABLE I Presence or absence of associated anomaliqs
14
PRS the only anomaly Associated syndrome Stickler Foetal alcohol Deletion chrom. 4
3 1 1
5 7
Associated non-syndromal defects
26
Total
TABLE II Time after birth to onset of symptoms Duration of time
n of patients
Within 2 hours Within 6 hours Within 12 hours Within 5 days Not noted
16 I 1 1 1
Total
26
Airway obstruction
Airway obstruction developed within 6 h of birth in 23 patients (Table II). Three distinct groups were identified according to the airway management which ultimately became necessary-these groups can be said to represent the severity of airway obstruction according to the eventual outcome. (Table III). In the mild group the age at which the infant could be nursed supine without significant obstruction while awake ranged from 1 to 7 months (mean 2.9 months). The age at which the infant could sleep supine ranged from 3 to 12 months (mean 5.6 months). In the moderate group the time at which a nasopharyngeal airway was first used ranged from one hour to 20 days and duration of placement of the tube ranged
TABLE III Secerity and management of airway obstruction according to eventual outcome Group
Management
Mild
Posture alone
8 (31)
Moderate
Nasopharyngeal tube
7 (27)
Severe
Endotracheal
n (%)
intubation or tracheotomy or death
11 (42)
33
from 14 days to 14 weeks. After tube removal the infants were nursed prone. The age at which the infants could sleep supine without significant obstruction ranged from 5 to 6 months. In the severe group of 11 patients 5 were managed by endotracheal intubation, 3 by tracheotomy and 3 by other means. Of the 5 managed by endotracheal intubation 3 were extubated after 10 days to 6 weeks and 2 died while still intubated. Three underwent tracheotomy; 2 later died of other causes and 1 survived. One of these had had a glossopexy but the airway obstruction was not relieved and a tracheotomy became necessary. The remaining 3 in the severe group were assessed as requiring more than nasopharyngeal intubation. In 2 the trachea could not be intubated; one was cyanosed at birth and died of hypoxic cerebral damage, and the other had a planned glossopexy abandoned after failure to intubate. In the third patient tracheotomy was planned but not performed because ‘general anesthesia was considered too great a risk’, yet eventually the infant survived. The 11 patients in the severe group proved to be difficult management problems and accounted for all 5 deaths in the series. The severe group were more likely to have been recorded as severe micrognathia (8 of ll>, to have associated congenital anomalies (5 of 11) and to develop complications due to airway obstruction (10 of 11). Early onset of airway obstruction showed no association with either severity of obstruction nor development of complications. Laryngoscopy for intubation was difficult in all patients on whom it was attempted and was not achieved in two patients. The trachea and main bronchi were examined with telescopes in most infants who had laryngoscopy, but no tracheal abnormalities were observed. Complications
Twelve infants developed complications. Eight had car pulmonale and of these, 5 had known congenital heart disease. Three newborn babies required resuscitation for cyanosis and intubation proved very difficult or impossible-all 3 suffered hypoxic cerebral damage. A fourth baby had multiple respiratory arrests due to obstruction, but no neurological sequelae. Deaths
There were 5 deaths. Age at death ranged from 4 days to 6 months. Three deaths were due to respiratory failure, 1 was due to heart failure, and 1 followed withdrawal of ventilatory support in a premature neonate with a massive intracerebra1 hemorrhage.
Discussion
PRS is a congenital anomaly categorized as isolated Pierre Robin sequence, as Pierre Robin with multiple anomalies or as Sticklers sequence and often associated
34
with serious upper airway obstruction which may be life-threatening. The obstruction is usually manifest within a few hours of birth but the onset may be delayed. It is not possible at first to assess the future severity of the airway problem and repeated observation in the neonatal intensive care ward for hours or many days is needed to determine which method of management is required. Monitoring by pulse oximetry is performed in every case [12]. A reasonable guide seems to be to have the oxygen saturation in room air over 90% for at least 90% of the time. Airway management must be individualized until successful control is achieved and should follow the progressive sequence of: (11 Posturing in the prone position. (2) Nasopharyngeal tube. (3) Endotracheal intubation. (4) Tracheotomy. The method may partly depend on the severity of associated cardiac and other anomalies. Posturing prone is the most important initial part of treatment; the tongue falls forward partly clearing the oropharyngeal airway. Posturing can be discontinued when the child is able to maintain the airway while sleeping supine-at about 5 or 6 months of age in the mild to moderate groups. Nasogastric tube feeding to minimize aspiration may be necessary in some patients. When posturing does not control the airway, a nasopharyngeal tube should be used [19]. The tube is passed through one nasal cavity and positioned by clinical testing and X-ray control so the tip is below the level of the base of the tongue but above the larynx (Fig. 2). The tube not only provides an airway through its own lumen but also creates a space at the base of the tongue on either side of the tube minimising the build-up of negative hypopharyngeal pressure. Posturing may still be necessary. Regular review and trial removal determine the appropriate time to discontinue use of the tube. Olson et al. 1191 state that with cooperative parents early home management of a baby with a nasopharyngeal tube is safe and acceptable. A variety of operative techniques to secure the tongue forward have been described, using sutures [8,9,18], wire [13], various obturators, traction devices [16,24] and fixation to the hyoid [14]. We have had little experience with these methods. If positioning prone and use of a nasopharyngeal tube fail to provide a reliable airway, prolonged endotracheal intubation or tracheotomy should be considered. Endotracheal intubation performed under controlled conditions will provide an excellent temporary airway but there are disadvantages. Laryngoscopy for intubation is always difficult and sometimes impossible without special purpose laryngoscopes. The patency of the tube must be carefully maintained, accidental extubation may occur with failure to reintubate, and laryngeal trauma from prolonged intubation is a possibility. We are now convinced that endotracheal intubation is more safely performed as an ‘awake intubation’ without general anesthesia but with cooperation between the anesthetist and the endoscopist to ensure adequate oxygenation. Intubation using standard pediatric laryngoscopes is difficult. Visualisation of the larynx for intubation is less difficult using a special purpose slotted laryngoscope with a slender barrel-the Holinger pediatric anterior commissure laryngoscope (Karl Storz,
Fig. 2. Lateral
X-ray
showing small mandible positioned
and a nasopharyngeal
tube supporting
the tongue and
so that the distal tip lies above the larynx.
Tuttlingen, F.R.G.) for term, normal weight babies or the smaller HolingerTucker-Benjamin laryngoscope (Karl Storz, Tuttlingen, F.R.G.) for premature or small babies. In an emergency, traction using a toothed forcep or a suture to pull the tongue forward will immediately improve the airway. If other techniques have failed tracheotomy will successfully bypass the obstruction. It is a safe and deuendable orocedure in small infants i3.51. The decision for
36
tracheotomy is based upon episodes of cyanosis observed clinically and desaturation measured objectively and there should be no reluctance or delay in performing the tracheotomy. Age at successful decannulation ranges from 3 to 18 months [17]. Management of the airway in PRS requires constant careful observation by nursing and medical staff experienced in assessing the severity of airway obstruction.
Conclusion The onset of airway obstruction in newborn infants with PRS usually occurs within a few hours of birth but it is not possible at first to assess the future severity. Monitoring clinically and by pulse oximetry should be done in a neonatal intensive care unit. A reasonable guide is to have the oxygen saturation above 90% for at least 90% of the time. Management of airway obstruction requires frequent reassessment, according to the difficulty with each patient, following the sequence of posture, nasopharyngeal tube or endotracheal tube. If these techniques fail, tracheotomy becomes necessary. Laryngoscopy and intubation is always difficult and is more safely performed without general anesthesia, using special purpose slotted laryngoscopes. In an emergency use of an oro-pharyngeal tube or an oro-esophageal tube or tongue traction using a forcep or a suture will be effective to restore an airway. Posturing in the prone position may be needed until 6 months of age. With optimal care there should be a low incidence of hypoxic cerebral damage and death.
References 1 Benjamin, B.N.P., Laryngoscopy. In S.L. Gans (Ed.), Pediatric Endoscopy, Grune and Stratton, New York, 1983, pp. 17-36. 2 Benjamin, B.N.P., Technique of laryngoscopy, Int. J. Pediatr. Otorhinolaryngol., 13 (1987) 299-313. 3 Benjamin, B.N.P. and Curley, J., Infant tracheotomy: endoscopy and decannulation, Int. J. Pediatr. Otorhinolaryngol., 20 (1990) 113-121. 4 Bull, M.J., Givan, D.C., Savove, A.M., Bixter, D. and Hearn, D., Improved outcome in Pierre Robin sequence: effect of multidisciplinary evaluation and management, Pediatrics, 86 (1990) 294-301. 5 Carter, P. and Benjamin, B.N.P., Ten-year review of pediatric tracheotomy, Ann. Otol. Rhinol. Laryngol., 92 (1983) 398-400. 6 Clarke, L., Hepworth, W.B., Carey, J.C. and Seegmiller, R.E., Chondrodystrophic mice with coincidental agnathia: evidence for the tongue obstruction hypothesis in cleft palate, Teratology, 38 (1988) 565-570. 7 Couly, G., Cheron, G., de-Blic, J., Despres, C., Cloup, M. and Hubert, P., Le syndrome de Pierre Robin. Classification et nouvelle approche therapeutique, Arch. Fr. Pediatr., 45 (1988) 553-559. 8 Douglas, B., A further report on the treatment of micrognathia associated with obstruction by a plastic procedure, Plast. Reconstr. Surg., 5 (1950) 113-122.
37 9 Duhamel, B., Chirurgie du Nouveau-& du Not&son, Masson et Cie., Paris, 1953, p. 55. 10 Dykes, E.H., Raine, P.A.M., Arthur, D.S., Drainer, I.K. and Young, D.G., Pierre Robin syndrome and pulmonary hypertension, J. Ped. Surg., 20 (1985) 49952. 11 Fletcher, M.M., Blum, S.L. and Blanchard, C.L., Pierre Robin syndrome pathophysiology of obstructive episodes, Laryngoscope, 79 (1969) 547-560. 12 Freed, G., Pearlman, M.A., Brown, AS. and Barot, L.R., Polysomnographic indications for surgical intervention in Pierre Robin sequence: acute airway management and follow-up studies after repair and take-down of tongue-lip adhesion, Cleft Palate J., 25 (1988) 151-155. 13 Hadley, R.C. and Johnson, J.B., Utilisation of the Kirschner wire in Pierre Robin syndrome, with case report, Plast. Reconstr. Surg., 31 (1963) 587-596. 14 Lapidot, A., Resvanti, F., Terrefe, D. and Ben-Hur, N., A new functional approach to the surgical management of Pierre Robin syndrome: experimental and clinical report, Laryngoscope, 86 (1976) 979-983. 15 Lewis, M.B. and Pashayan, H.M., Management of infants with Robin anomaly, Clin. Pediatr., 19 ( 1980) 5 19-528. 16 Longmire Jr., W.P. and Sanford, M.C., Stimulation of mandibular growth in congenital micrognathia by traction, Am. J. Dis. Child., 78 (1949) 750-754. 17 McEvitt, W.G.. Micrognathia and its management, Plast. Reconstr. Surg., 41 (1968) 450-455. 18 Oeconomopoulos, CT., The value of glossopexy in Pierre Robin syndrome, N. Engl. J. Med., 262 (1960) 1267-1268. 19 Olson, T.S., Kearns, D.B., Pransky, S.M. and Seid, A.B., Early home management of patients with Pierre Robin sequence, Int. J. Pediatr. Otorhinolaryngol., 20 (1990) 45-49. 20 Pashayan, H.M. and Lewis, M.B., Clinical experience with the Robin sequence, Cleft Palate J.. 21 (I 984) 270-276. 21 Robin, P., Glossoptosis due to atresia and hypotrophy of the mandible, Am. J. Dis. Child.. 48 (1934) 541-547. 22 Routledge, R.T., The Pierre Robin syndrome: a surgical emergency in the neonatal period, Br. J. Plast. Surg., 13 (1960) 204-218. 23 Stern, L..M., Fonkalsrud, E.W., Hassakis, P. and Jones, M.H., Management of Pierre Robin syndrome by prolonged nasoesophageal intubation, Am. J. Dis. Child., 124 (1972) 78-80. 24 Wada, T., Ishi, T., Sugai, T., Molla, M.R., Matsuya, T., Miyazaki, T. and Koh, Y., Mandibular traction for relieving respiratory distress in the Pierre Robin anomaly, J. Max.-fat. Surg., I1 (1983) 187-190.
