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Indications for tracheostomy in children
PAEDIATRIC RESPIRATORY REVIEWS (2006) 7, 162–168
MINI-SYMPOSIUM: TRACHEOSTOMY IN CHILDREN
Indications for tracheostomy in children Daniel Trachsel* and Ju¨rg Hammer Division of Paediatric Intensive Care and Pulmonology, University Children’s Hospital Basel, Ro¨mergasse 8, CH-4059 Basel, Switzerland KEYWORDS tracheotomy; tracheostomy; infant; child; respiratory insufficiency; airway obstruction; respiration; artificial
Summary Vaccination programs, improvements in material engineering and anaesthetic skills have dramatically reduced the number of emergency tracheostomies performed for acute upper airway obstruction. Today, the indication to tracheotomise a child is generally ruled by the anticipation of long-term (cardio)respiratory compromise due to chronic ventilatory or, more rarely, cardiac insufficiency, or by the presence of a fixed upper airway obstruction that is unlikely to resolve for a significant period of time. As many of the younger candidates for tracheostomy have complex medical conditions, the indication for this intervention is often complicated by ethical, funding and socioeconomic concerns that necessitate a multidisciplinary approach. Unfortunately, these considerations are frequently not made until the first catastrophe has occurred, even in those patients in whom imminent cardiorespiratory failure has been foreseeable. Noninvasive ventilation via a face mask and newer developments such as the in-exsufflator device have gained importance as an alternative to tracheostomy in selected patients. ß 2006 Elsevier Ltd. All rights reserved.
INTRODUCTION The history of tracheostomy portrays the history of medical advance and of progress in material engineering. Depicted on Egyptian tablets as early as 3600 BC, tracheostomy has been a subject of medical debate ever since. Despite this, it did not enter routine medical practice until the 19th century, when doctors became increasingly open-minded towards the procedure as a means of providing immediate relief to patients with acute laryngeal obstruction, the majority of cases at that time being related to diphtheria.1,2 Although a variety of indications for tracheostomy, for example ‘pulmonary toilet’ or application of anaesthetics, were sporadically proposed, scepticism prevailed in view of an unacceptably high mortality. The procedure therefore remained the last resort for acute life-threatening upper
* Corresponding author. Tel.: +41 61 685 6565; Fax: +41 61 685 5004. E-mail address: [email protected] (D. Trachsel). 1526-0542/$ – see front matter ß 2006 Elsevier Ltd. All rights reserved. doi:10.1016/j.prrv.2006.06.004
airway obstruction.3 This only changed in the early 20th century, when Chevalier Jackson standardised the procedure and its after care measures, reducing morbidity and mortality related to the intervention.4 In the years to come, the list of indications for tracheostomy gradually increased to comprise diseases of the entire respiratory apparatus, a development that gained momentum with the famous Copenhagen poliomyelitis epidemic of 1952.5 Tracheostomy became standard of care for basically all patients with respiratory insufficiency.1 Although the idea of endotracheal intubation performed through the mouth dates back to Hippocrates (460–375 BC), who objected to the idea of tracheostomy for fear of injuring the carotid arteries, attempts to do this were not undertaken until the 19th century.6 Advances in material engineering and technical refinements, however, have since allowed a circumvention of tracheostomy in various areas of respiratory care. The replacement of rubber and sterling silver tubes by thermosensitive polyvinyl chloride tubes reduced the discomfort and laryngeal damage associated
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with prolonged transpharyngeal intubation.7 Thus, modern high-volume, low-pressure cuffed tubes have rendered endotracheal intubation via the oropharyngeal or the nasopharyngeal route an attractive and feasible alternative for even extended periods of intubation, and the decision to proceed to tracheostomy is generally guided by the anticipation of long-term respiratory compromise. Similarly, the ability to modulate face masks individually to each patient’s facial contours, along with the excellent tolerance towards the new materials, provides opportunity for longterm non-invasive positive-pressure ventilation in many patients with neuromuscular diseases.8,9 In addition, the introduction of fibreoptic intubation has made nasopharyngeal intubation a valuable alternative in difficult airway management.10 As a consequence, the indications for tracheostomy have changed during the years, and they continue to do so.
range of underlying conditions that may warrant tracheostomy (Table 1). Upper airway obstruction as an indication is decreasing in frequency and ventilator-dependence is the evolving indication in developed countries. Congenital upper airway anomalies resulting in a difficult airway that cannot be secured by other means frequently necessitate transient or permanent tracheostomy, especially in infants and young children with compromised nasal breathing (Fig. 1).13,14 Chronic lung disease of infancy and complex congenital heart disease are other important entities associated with the occasional need for tracheostomy, in heart disease mostly for long-term ventilation because of ongoing cardiac failure, tracheobronchomalacia or postoperative diaphragmatic paresis. Most of these children can eventually be decannulated.15,16 Most cohorts of tracheotomised children contain a significant number of infants (32%).17 A recent survey estimated the US-wide caseload of tracheostomy in infancy to be around 40 tracheostomy-related hospital discharges per 100 000 child–years.17 Adolescents represent a second important subset, contributing another third to the total. Although pulmonary disease and congenital anomalies account for the majority of underlying diagnoses in infancy, more than three-quarters of tracheostomies performed in adolescents are on injury victims, a third of them having suffered severe traumatic brain injury.17 There is a preponderance of males (64%) subjected to tracheostomy –
INDICATIONS Publications suggest that the range of indications for tracheostomy has broadened over the past decades. This is strictly speaking not true because tracheostomy in children is indicated for the same reasons as before; i.e. either it serves as a bypass round an upper airway obstruction or it is being instituted for long-term respiratory support and/or pulmonary toilet.11,12 What has changed, however, is the
Table 1 Indications for tracheostomy and examples of underlying conditions (not including disorders occasionally requiring emergency tracheostomy) Indications for tracheostomy Upper airway obstruction Subglottic stenosis Tracheomalacia Tracheal stenosis Craniofacial syndromes
Craniofacial and laryngeal tumours Bilateral vocal cord paralysis Obstructive sleep apnoea Laryngeal trauma Long-term ventilation/pulmonary toilet Pulmonary disease Congenital heart disease Neurological/neuromuscular disease
Adapted from references [12,26].
Examples Congenital/acquired Congenital/acquired Congenital/acquired Pierre-Robin sequence CHARGE syndrome Treacher–Collins syndrome Beckwith–Wiedemann syndrome Cystic hygroma, haemangioma Hydrocephalus internus Moebius syndrome Burn, fracture Bronchopulmonary dysplasia Scoliosis with restrictive pneumopathy Postoperative diaphragmatic paresis Duchenne muscular dystrophy Spinal muscle atrophy type I Congenital central hypoventilation syndrome Cerebral palsy Traumatic brain and spinal injury Spina bifida
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D. TRACHSEL, AND J. HAMMER
Figure 1 Upper panels: An infant with severe cervical kyphosis and dwarfism, resulting in a highly critical airway until surgical stabilisation. A magnetic resonance imaging scan of the same infant on the right demonstrates the narrow upper airway anatomy. Lower panels: An infant with cystic hygroma resulting in a critical airway. A magnetic resonance imaging scan of the same child on the right demonstrates impressive infiltration of the upper airway structures. (Reproduced with parental consent.)
X-linked inherited diseases and high-risk behaviour in males may contribute to this gender distribution.
Tracheostomy as a bypass for upper airway obstruction For the past 150 years, acute life-threatening upper airway obstruction in a child who cannot be intubated via the pharyngeal route for any reason, whether technical or anatomical, has indicated an emergency tracheostomy. As epiglottitis and laryngotracheitis were the most important causes of severe acute upper airway obstruction, the
widespread implementation of vaccination programmes against diphtheria and Haemophilus influenzae type b, as well as refinements in anaesthetic techniques, have dramatically reduced the number of emergency tracheostomies.18,19 Studies reporting on changing trends in paediatric tracheostomy have described a significant proportional decrease in tracheostomies performed for infectious upper airway obstruction from more than 50% in the early 1970s to almost none today.19–26 Forty years ago, children with infectious acute upper airway obstruction were frequently tracheotomised. Now the airway can be secured mostly via the transpharyngeal
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route. Consequently, several recent studies listed no emergency tracheostomy among their cohorts.23,27 In the rare instances where emergency tracheostomy became necessary, injury of the larynx and the mid-face or congenital upper airway anomalies usually necessitated the intervention.28–31 The inherent downside of this development is that many paediatricians have little opportunity to gain experience in emergency tracheostomy. Training in animal laboratories might help to improve skills but will be difficult to implement on a broad scale.32 Today, the main indication for tracheostomy in acquired upper airway obstruction has shifted to causes allowing an elective intervention, such as subglottic stenosis from prolonged intubation in the neonatal period, bilateral vocal cord paralysis and upper airway trauma, i.e. burns and fractures. These tracheostomies are often temporary measures until corrective surgery can be performed or the infant has outgrown his or her susceptibility to critical airway compromise.
before day 10 after the burn was associated with less subglottic stenosis (15%) compared with delayed tracheostomy from day 10 onwards (>50%).28 Although this suggests that pressure and friction on the burned laryngeal tissue contribute to constrictive scarring, the pathogenesis of subglottic stenosis has not been clearly elucidated.28 Other arguments enforced by proponents of early tracheostomy in severely burned paediatric patients include easier handling during dressing changes, superiority in the management of severe respiratory failure and mucus plugging, and safety, bearing in mind the potentially disastrous results of accidental extubation in those in whom significant upper airway swelling precludes rapid reintubation.29,41 Others prefer a more conservative approach indicating a low overall incidence of complications with transpharyngeal intubation.42 A reasonable practice may be to perform elective tracheostomy if significant (supra)glottic oedema is present on laryngoscopy, because this subgroup is at high risk of critical airway incidences.30
Tracheostomy in the paediatric intensive care unit
Tracheostomy for long-term ventilation/ pulmonary toilet
Early reports of long-term intubation in adults suggested an increasing risk of laryngeal damage from the second week of intubation onwards.33,34 Although no official guidelines exist, it is a frequently adopted policy in adult intensive care units to tracheotomise patients after 1–2 weeks of intubation if a need for long-term invasive respiratory support is expected. The rationale for this, however, is not only based on worries about laryngeal complications, but also on economic constraints requiring patients to be moved rapidly from intensive care.35 Managing an adult patient on prolonged respiratory support via tracheostomy is more cost-effective as less sedation, less monitoring and hence fewer personnel are needed. In paediatric patients, the risks and implications of tracheostomy are not the same as they are in adults. No time limit for the transition to tracheostomy has ever met with wide acceptance. Transpharyngeal intubation is usually continued for prolonged periods as long as neuromuscular or pulmonary recovery is expected. Positive experience with prolonged transpharyngeal intubation was gained early in the discipline of paediatric intensive care.36,37 In a retrospective study, Lee et al. showed that the duration of intubation was not predictive of the likelihood of tracheostomy in children, and that the decision to tracheotomise a patient was made on an individual basis.38 This concurs with the observation that prolonged intubation does not seem to be a significant risk factor for post-extubational laryngeal complications in children, thereby negating the argument for early tracheostomy.39,40 Whether this holds true in burn patients remains controversial. An adverse impact of prolonged transpharyngeal intubation has been described in 36 severely burned paediatric patients, in whom transition to tracheostomy
Ethical questions need to be answered before the indications for long-term ventilation and tracheostomy can be discussed. Physicians tend to underestimate their patients’ quality of life, their prediction of what life-saving procedures a patient would want more closely reflecting their own personal preferences than those of their patients.43,44 When a decision to tracheotomise a child is made, the goal must be clearly defined and discussed with the family beforehand. The issue is complicated because ventilation is often commenced in an acute crisis, leaving no opportunity to discuss the possibility of long-term ventilation in advance and thereby offering little choice to the child and the family. Unfortunately, even when the need for ventilatory support can be anticipated early, as in older paediatric patients with progressive disorders, long-term ventilation and end-of-life care are often not discussed with the patient and the family. There are no comprehensive recommendations for an invasive versus a non-invasive mode of ventilatory support in ventilator-dependent children. Recent surveys suggest that young age, ventilator-dependency for most of the day and frequent episodes of respiratory exacerbation with need for intubation increase the likelihood of being tracheotomised.45–47 An increasing number of studies, however, report successful long-term, non-invasive ventilation via a nasal or face mask in infants and young children.8,9,48,49 Experience in the non-invasive ventilation of young children and a proficient technical service capable of providing individually adjusted interfaces are prerequisites for success because ventilator–patient asynchrony and pressure sores are not uncommon problems.9,50 In addition, secondary mid-face hypoplasia has emerged as a worrisome complication of long-term mask application in young children.9,51 This is not only a cosmetic issue: it also
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has a significant effect on dentition and on nasopharyngeal patency in patients susceptible to upper airway obstruction. The amount and duration of pressure applied by strapping the mask to the face as well as periods of growth spurt, may have an impact on the incidence and extent of this complication. Diaphragmatic pacing has the potential to reduce the need for a bulky mechanical ventilator with certain causes of ventilator-dependence.52 Technological refinements, enhanced expertise and reasonable pricing would increase its role in managing ventilator-dependent children. Most children will, however, still require a tracheostomy or continuous positive airway pressure to prevent upper airway obstruction during electrically stimulated inspirations. Other developments, for example the mechanical in-exsufflator, might decrease the need for invasive mechanical ventilation by facilitating and prolonging the non-invasive management of chronic respiratory failure in children with neuromuscular diseases.53 In accordance with common practice elsewhere,45–47 the authors currently favour primary tracheostomy in young children and infants who are ventilator-dependent, while older children (over 8 years of age) who are able to sustain spontaneous breathing during the daytime are good candidates for non-invasive ventilation. This age limit, however, is by no means absolute. Instead, it represents an arbitrary consensus aiming at minimising the risk of mid-facial hypoplasia.
How to approach the indication for tracheostomy With the increasing complexity of the underlying medical problems, the decision to tracheotomise a child has become an interdisciplinary process involving intensive care and pulmonology specialists, paediatric otorhinolaryngologists, paediatric surgeons, social care workers and respiratory nurses. The attitudes of patients and caregivers influence the choice of treatment just as much as socioeconomic factors, funding and the availability of professional support at home. Questions that may help the decision to undertake a tracheostomy are proposed in Table 2. Rare relative contraindications – local skin infections, refractory eczema, the presence of an engorged pretracheal venous plexus in patients with advanced superior vena cava syndrome, and self-mutilation in mentally handicapped children – are generally insufficient reasons not to undertake a tracheostomy.
FUTURE DEVELOPMENTS Percutaneous dilational tracheostomy has increased in popularity for temporary respiratory management in adult intensive care units.35,54 Little experience, however, exists in children.55 Provided that dilators and cannulas of appropriate sizes are available, percutaneous dilational tracheostomy might be an alternative in selected patients, for
D. TRACHSEL, AND J. HAMMER
Table 2
Criteria favouring tracheostomy in children
The child with upper airway obstruction U Low chance of definitive, spontaneous resolution within a reasonable time (weeks) U Low probability that surgery can definitely correct the cause U High risk of critical upper airway obstruction with simple respiratory tract infections or minor bleeding (epistaxis) U High risk of or previous history of difficulties in airway management in case of an emergency U Difficult-to-control gastro-oesophageal reflux The child requiring long-term ventilation/ pulmonary toilet U Young age with a high risk of mid-facial deformation from mask pressure U Ventilator dependency for most of the day (more than 12 hours per day) U Inability to cope with a mask (full face or nasal mask) U Recurrent aspirations (gastro-oesophageal reflux, laryngeal incompetence) with significant benefit from pulmonary toilet U Safety-measures and local experience highly in favour of invasive ventilation
example children with Guillain-Barre´ syndrome or traumatic brain injury. Similarly, transtracheal oxygen catheters used for long-term oxygen therapy in adult patients have not gained widespread acceptance in paediatric practice; these are not without complications (dislodging, mucus plugging, local infection).56 Reported advantages include cosmetic benefits, a reduction in oxygen flow rates compared with nasal cannulae, and an avoidance of conventional tracheotomy.56,57
CONCLUSION The indications for tracheostomy have changed over time. Few children are now tracheotomised for airway emergencies; instead, the procedure is mostly performed for long-term problems. As a consequence, there is in most clinical situations no unity of approach, and the indication for tracheostomy is influenced by multiple circumstantial factors.
PRACTICE POINTS In patients with progressive neuromuscular or respiratory disease, the possibility of tracheostomy should be discussed with the caregivers and the patient before a foreseeable emergency situation arises. Tracheostomy in burn patients should be considered early if significant mucosal damage is seen by laryngoscopy.
INDICATIONS FOR TRACHEOSTOMY IN CHILDREN
REFERENCES 1. Graamans K, Pirsig W, Biefel K. The shift in the indications for the tracheotomy between 1940 and 1955: an historical review. J Laryngol Otol 1999; 113: 624–627. 2. Pahor AL. Ear, nose and throat in Ancient Egypt. J Laryngol Otol 1992; 106: 773–779. 3. Kost KM. Endoscopic percutaneous dilatational tracheotomy: a prospective evaluation of 500 consecutive cases. Laryngoscope 2005; 115: S1–S30. 4. Jackson C. Tracheotomy. Laryngoscope 1909; 19: 285–290. 5. Lassen HC. The poliomyelitis epidemic of 1952 in Copenhagen: 349 cases with respiratory insufficiency and deglutition paralysis. Presse Med 1953; 19: 1667–1670. 6. O’Dwyer J. Intubation of the larynx. N Y Med J 1885; 42: 147. 7. Shann FA, Duncan AW, Brandstater B. Prolonged per-laryngeal endotracheal intubation in children: 40 years on. Anaesth Intensive Care 2003; 31: 664–666. 8. Fauroux B, Boffa C, Desguerre I, Estournet B, Trang H. Long-term noninvasive mechanical ventilation for children at home: a national survey. Pediatr Pulmonol 2003; 35: 119–125. 9. Simonds AK, Ward S, Heather S, Bush A, Muntoni F. Outcome of paediatric domiciliary mask ventilation in neuromuscular and skeletal disease. Eur Respir J 2000; 16: 476–481. 10. Wood RE. Clinical applications of ultrathin flexible bronchoscopes. Pediatr Pulmonol 1985; 1: 244–248. 11. Donelly MJ, Lacey PD, Maguire AJ. A twenty year (1971–1990) review of tracheotomies in a major paediatric hospital. Int J Pediatr Otorhinolaryngol 1996; 35: 1–9. 12. Midwinter KI, Carrie S, Bull PD. Paediatric tracheostomy: Sheffield experience 1979–1999. J Laryngol Otol 2002; 116: 532–535. 13. Roger G, Morisseau-Durand MP, Van Den Abbeele T et al. The CHARGE association: the role of tracheostomy. Arch Otolaryngol Head Neck Surg 1999; 125: 33–38. 14. Sculerati N, Gottlieb MD, Zimbler MS, Chibbaro PD, McCarthy JG. Airway management in children with major craniofacial anomalies. Laryngoscope 1998; 108: 1806–1812. 15. Hoskote A, Cohen G, Goldman A, Shekerdemian L. Tracheostomy in infants and children after cardiothoracic surgery: Indications, associated risk factors, and timing. J Thorac Cardiovasc Surg 2005; 130: 1086–1093. 16. LoTempio MM, Shapiro NL. Tracheotomy tube placement in children following cardiothoracic surgery: Indications and outcomes. Am J Otolaryngol 2002; 23: 337–340. 17. Lewis CW, Carron JD, Perkins JA, Sie KCY, Feudtner C. Tracheotomy in pediatric patients. A national perspective. Arch Otolaryngol Head Neck Surg 2003; 129: 523–529. 18. Mayer TA. Emergency pediatric tracheostomy: a technique in search of an indication. Ann Emerg Med 1987; 16: 606. 19. Sendi K, Crysdale WS. Acute epiglottitis: a decade of change – a 10year experience with 242 children. J Otolaryngol 1987; 16: 196–202. 20. Arcand P, Granger J. Pediatric tracheostomies: changing trends. J Otolaryngol 1988; 17: 121–124. 21. Carter P, Benjamin B. Ten-year review of pediatric tracheotomy. Ann Otol Rhinol Laryngol 1983; 92: 389–400. 22. Crysdale WS, Feldman RI, Naito K. Tracheotomies: a 10-year experience in 319 children. Ann Otol Rhinol Laryngol 1988; 97: 439–443. 23. Hadfield PJ, Lloyd-Faulconbridge RV, Almeyda J, Albert DM, Bailey CM. The changing indications for paediatric tracheostomy. Int J Pediatr Otorhinolaryngol 2003; 67: 7–10. 24. Line WS Jr, Hawkins DB, Kahlstrom JL, MacLaughlin EF, Ensley JL. Tracheotomy in infants and young children: the changing perspective 1970–1985. Laryngoscope 1986; 96: 510–515. 25. Kimmons HC Jr, Peterson BM. Management of acute epiglottitis in pediatric patients. Crit Care Med 1986; 14: 278–279. 26. Carron JD, Derkay CS, Strope GL, Nosonchuk JE, Darrow DH. Pediatric tracheotomies: changing indications and outcomes. Laryngoscope 2000; 110: 1099–1104.
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27. Simma B, Spehler D, Burger R et al. Tracheostomy in children. Eur J Pediatr 1994; 153: 291–296. 28. Barret JP, Desai MH, Herndon DN. Effects of tracheostomies on infection and airway complications in pediatric burn patients. Burns 2000; 26: 190–193. 29. Coln CE, Purdue GF, Hunt JL. Tracheostomy in the young pediatric burn patient. Arch Surg 1998; 133: 537–540. 30. Prater ME, Deskin RW. Bronchoscopy and laryngoscopy findings as indications for tracheotomy in the burned child. Arch Otolaryngol Head Neck Surg 1998; 124: 1115–1117. 31. Tantinikorn W, Alper CM, Bluestone CD, Casselbrant ML. Outcome in pediatric tracheotomy. Am J Otolaryngol 2003; 24: 131–137. 32. Custalow CB, Kline JA, Marx JA, Baylor MR. Emergency department resuscitative procedures: animal laboratory training improves procedural competency and speed. Acad Emerg Med 2002; 9: 575–586. 33. Dunham CM, LaMonica C. Prolonged tracheal intubation in the trauma patient. J Trauma 1984; 24: 120–124. 34. Whited RE. A prospective study of laryngotracheal sequelae in longterm intubation. Laryngoscope 1984; 94: 367–377. 35. Rumbak MJ, Newton M, Truncale T, Schwartz SW, Adams JW, Hazard PB. A prospective, randomized, study comparing early percutaneous dilational tracheotomy to prolonged translaryngeal intubation (delayed tracheotomy) in critically ill medical patients. Crit Care Med 2004; 32: 1689–1694. 36. Battersby EF, Hatch DJ, Towey RM. The effects of prolonged nasoendotracheal intubation in children. A study in infants and young children after cardiopulmonary bypass. Anaesthesia 1977; 32: 154– 157. 37. Allen TH, Steven IM. Prolonged endotracheal intubation in infants and children. Br J Anaesth 1965; 37: 566–573. 38. Lee W, Koltai P, Harrison AM et al. Indications for tracheotomy in the pediatric intensive care unit population. Arch Otolaryngol Head Neck Surg 2002; 128: 1249–1252. 39. Kemper KJ, Benson MS, Bishop MJ. Predictors of postextubation stridor in pediatric trauma patients. Crit Care Med 1991; 19: 352– 355. 40. Watier-Launey C, Buronfosse A, Saliba E, Bertrand P, Ployet MJ. Predictive factors for complications in children with laryngeal damage at extubation. Laryngoscope 2000; 110: 328–331. 41. Palmieri TL, Jackson W, Greenhalgh DG. Benefits of early tracheostomy in severely burned children. Crit Care Med 2002; 30: 922–924. 42. Kadilak PR, Vanasse S, Sheridan RL. Favorable short- and long-term outcomes of prolonged translaryngeal intubation in critically ill children. J Burn Care Rehabil 2004; 25: 262–265. 43. Bach JR, Campagnolo DI, Hoeman S. Life satisfaction of individuals with Duchenne muscular dystrophy using long-term mechanical ventilatory support. Am J Phys Med Rehabil 1991; 70: 129–135. 44. Schneiderman LJ, Kaplan RM, Pearlman RA, Teetzel H. Do physicians’ own preferences for life-sustaining treatment influence their perceptions of patients’ preferences? J Clin Ethics 1993; 4: 28–33. 45. Edwards EA, O’Toole M, Wallis C. Sending children home on tracheostomy dependent ventilation: pitfalls and outcomes. Arch Dis Child 2004; 89: 251–255. 46. Ioos C, Leclair-Richard D, Mrad S, Barois A, Estournet-Mathiaud B. Respiratory capacity course in patients with infantile muscular atrophy. Chest 2004; 126: 831–837. 47. Kamm M, Burger R, Rimensberger P, Knoblauch A, Hammer J. Survey of children supported by long-term mechanical ventilation in Switzerland. Swiss Med Wkly 2001; 131: 261–266. 48. Bach JR, Baird JS, Plosky D, Navado J, Weaver B. Spinal muscular atrophy type 1: management and outcomes. Pediatr Pulmonol 2002; 34: 16–22. 49. Vanderlaan M, Holbrook CR, Wang M, Tuell A, Gozal D. Epidemiologic survey of 196 patients with congenital central hypoventilation syndrome. Pediatr Pulmonol 2004; 37: 217–229. 50. Essouri S, Nicot F, Cle´ment A et al. Noninvasive positive pressure ventilation in infants with upper airway obstruction: Comparison of
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continuous and bilevel positive pressure. Intensive Care Med 2005; 31: 574–580. 51. Li KK, Riley RW, Guilleminault C. An unreported risk in the use of home nasal continuous positive airway pressure and home nasal ventilation children. Mid-face hypoplasia. Chest 2000; 117: 916–918. 52. Shaul DB, Danielson PD, McComb JG, Keens TG. Thoracoscopic placement of phrenic nerve electrodes for diaphragmatic pacing in children. J Pediatr Surg 2002; 37: 974–978. 53. Miske LJ, Hickey EM, Kolb SM, Weiner DJ, Panitch HB. Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough. Chest 2004; 125: 1406–1412.
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54. Gru¨ndling M, Quintel M. Percutaneous dilational tracheostomy. Indications – techniques – complications. Anaesthesist 2005; 54: 929–944. 55. Toursarkissian B, Fowler CL, Zweng TN, Kearney PA. Percutaneous dilational tracheostomy in children and teenagers. J Pediatr Surg 1994; 29: 1421–1424. 56. Preciado DA, Thatcher G, Panitch HB, Rimell FL. Transtracheal oxygen catheters in a pediatric population. Ann Otol Rhinol Laryngol 2002; 111: 310–314. 57. Kampelmacher MJ, Deenstra M, van Kesteren RG, Melissant CF, Douze JM, Lammers JW. Transtracheal oxygen therapy: an effective and safe alternative to nasal oxygen administration. Eur Respir J 1997; 10: 828–833.
MINI-SYMPOSIUM: TRACHEOSTOMY IN CHILDREN
Indications for tracheostomy in children Daniel Trachsel* and Ju¨rg Hammer Division of Paediatric Intensive Care and Pulmonology, University Children’s Hospital Basel, Ro¨mergasse 8, CH-4059 Basel, Switzerland KEYWORDS tracheotomy; tracheostomy; infant; child; respiratory insufficiency; airway obstruction; respiration; artificial
Summary Vaccination programs, improvements in material engineering and anaesthetic skills have dramatically reduced the number of emergency tracheostomies performed for acute upper airway obstruction. Today, the indication to tracheotomise a child is generally ruled by the anticipation of long-term (cardio)respiratory compromise due to chronic ventilatory or, more rarely, cardiac insufficiency, or by the presence of a fixed upper airway obstruction that is unlikely to resolve for a significant period of time. As many of the younger candidates for tracheostomy have complex medical conditions, the indication for this intervention is often complicated by ethical, funding and socioeconomic concerns that necessitate a multidisciplinary approach. Unfortunately, these considerations are frequently not made until the first catastrophe has occurred, even in those patients in whom imminent cardiorespiratory failure has been foreseeable. Noninvasive ventilation via a face mask and newer developments such as the in-exsufflator device have gained importance as an alternative to tracheostomy in selected patients. ß 2006 Elsevier Ltd. All rights reserved.
INTRODUCTION The history of tracheostomy portrays the history of medical advance and of progress in material engineering. Depicted on Egyptian tablets as early as 3600 BC, tracheostomy has been a subject of medical debate ever since. Despite this, it did not enter routine medical practice until the 19th century, when doctors became increasingly open-minded towards the procedure as a means of providing immediate relief to patients with acute laryngeal obstruction, the majority of cases at that time being related to diphtheria.1,2 Although a variety of indications for tracheostomy, for example ‘pulmonary toilet’ or application of anaesthetics, were sporadically proposed, scepticism prevailed in view of an unacceptably high mortality. The procedure therefore remained the last resort for acute life-threatening upper
* Corresponding author. Tel.: +41 61 685 6565; Fax: +41 61 685 5004. E-mail address: [email protected] (D. Trachsel). 1526-0542/$ – see front matter ß 2006 Elsevier Ltd. All rights reserved. doi:10.1016/j.prrv.2006.06.004
airway obstruction.3 This only changed in the early 20th century, when Chevalier Jackson standardised the procedure and its after care measures, reducing morbidity and mortality related to the intervention.4 In the years to come, the list of indications for tracheostomy gradually increased to comprise diseases of the entire respiratory apparatus, a development that gained momentum with the famous Copenhagen poliomyelitis epidemic of 1952.5 Tracheostomy became standard of care for basically all patients with respiratory insufficiency.1 Although the idea of endotracheal intubation performed through the mouth dates back to Hippocrates (460–375 BC), who objected to the idea of tracheostomy for fear of injuring the carotid arteries, attempts to do this were not undertaken until the 19th century.6 Advances in material engineering and technical refinements, however, have since allowed a circumvention of tracheostomy in various areas of respiratory care. The replacement of rubber and sterling silver tubes by thermosensitive polyvinyl chloride tubes reduced the discomfort and laryngeal damage associated
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with prolonged transpharyngeal intubation.7 Thus, modern high-volume, low-pressure cuffed tubes have rendered endotracheal intubation via the oropharyngeal or the nasopharyngeal route an attractive and feasible alternative for even extended periods of intubation, and the decision to proceed to tracheostomy is generally guided by the anticipation of long-term respiratory compromise. Similarly, the ability to modulate face masks individually to each patient’s facial contours, along with the excellent tolerance towards the new materials, provides opportunity for longterm non-invasive positive-pressure ventilation in many patients with neuromuscular diseases.8,9 In addition, the introduction of fibreoptic intubation has made nasopharyngeal intubation a valuable alternative in difficult airway management.10 As a consequence, the indications for tracheostomy have changed during the years, and they continue to do so.
range of underlying conditions that may warrant tracheostomy (Table 1). Upper airway obstruction as an indication is decreasing in frequency and ventilator-dependence is the evolving indication in developed countries. Congenital upper airway anomalies resulting in a difficult airway that cannot be secured by other means frequently necessitate transient or permanent tracheostomy, especially in infants and young children with compromised nasal breathing (Fig. 1).13,14 Chronic lung disease of infancy and complex congenital heart disease are other important entities associated with the occasional need for tracheostomy, in heart disease mostly for long-term ventilation because of ongoing cardiac failure, tracheobronchomalacia or postoperative diaphragmatic paresis. Most of these children can eventually be decannulated.15,16 Most cohorts of tracheotomised children contain a significant number of infants (32%).17 A recent survey estimated the US-wide caseload of tracheostomy in infancy to be around 40 tracheostomy-related hospital discharges per 100 000 child–years.17 Adolescents represent a second important subset, contributing another third to the total. Although pulmonary disease and congenital anomalies account for the majority of underlying diagnoses in infancy, more than three-quarters of tracheostomies performed in adolescents are on injury victims, a third of them having suffered severe traumatic brain injury.17 There is a preponderance of males (64%) subjected to tracheostomy –
INDICATIONS Publications suggest that the range of indications for tracheostomy has broadened over the past decades. This is strictly speaking not true because tracheostomy in children is indicated for the same reasons as before; i.e. either it serves as a bypass round an upper airway obstruction or it is being instituted for long-term respiratory support and/or pulmonary toilet.11,12 What has changed, however, is the
Table 1 Indications for tracheostomy and examples of underlying conditions (not including disorders occasionally requiring emergency tracheostomy) Indications for tracheostomy Upper airway obstruction Subglottic stenosis Tracheomalacia Tracheal stenosis Craniofacial syndromes
Craniofacial and laryngeal tumours Bilateral vocal cord paralysis Obstructive sleep apnoea Laryngeal trauma Long-term ventilation/pulmonary toilet Pulmonary disease Congenital heart disease Neurological/neuromuscular disease
Adapted from references [12,26].
Examples Congenital/acquired Congenital/acquired Congenital/acquired Pierre-Robin sequence CHARGE syndrome Treacher–Collins syndrome Beckwith–Wiedemann syndrome Cystic hygroma, haemangioma Hydrocephalus internus Moebius syndrome Burn, fracture Bronchopulmonary dysplasia Scoliosis with restrictive pneumopathy Postoperative diaphragmatic paresis Duchenne muscular dystrophy Spinal muscle atrophy type I Congenital central hypoventilation syndrome Cerebral palsy Traumatic brain and spinal injury Spina bifida
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Figure 1 Upper panels: An infant with severe cervical kyphosis and dwarfism, resulting in a highly critical airway until surgical stabilisation. A magnetic resonance imaging scan of the same infant on the right demonstrates the narrow upper airway anatomy. Lower panels: An infant with cystic hygroma resulting in a critical airway. A magnetic resonance imaging scan of the same child on the right demonstrates impressive infiltration of the upper airway structures. (Reproduced with parental consent.)
X-linked inherited diseases and high-risk behaviour in males may contribute to this gender distribution.
Tracheostomy as a bypass for upper airway obstruction For the past 150 years, acute life-threatening upper airway obstruction in a child who cannot be intubated via the pharyngeal route for any reason, whether technical or anatomical, has indicated an emergency tracheostomy. As epiglottitis and laryngotracheitis were the most important causes of severe acute upper airway obstruction, the
widespread implementation of vaccination programmes against diphtheria and Haemophilus influenzae type b, as well as refinements in anaesthetic techniques, have dramatically reduced the number of emergency tracheostomies.18,19 Studies reporting on changing trends in paediatric tracheostomy have described a significant proportional decrease in tracheostomies performed for infectious upper airway obstruction from more than 50% in the early 1970s to almost none today.19–26 Forty years ago, children with infectious acute upper airway obstruction were frequently tracheotomised. Now the airway can be secured mostly via the transpharyngeal
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route. Consequently, several recent studies listed no emergency tracheostomy among their cohorts.23,27 In the rare instances where emergency tracheostomy became necessary, injury of the larynx and the mid-face or congenital upper airway anomalies usually necessitated the intervention.28–31 The inherent downside of this development is that many paediatricians have little opportunity to gain experience in emergency tracheostomy. Training in animal laboratories might help to improve skills but will be difficult to implement on a broad scale.32 Today, the main indication for tracheostomy in acquired upper airway obstruction has shifted to causes allowing an elective intervention, such as subglottic stenosis from prolonged intubation in the neonatal period, bilateral vocal cord paralysis and upper airway trauma, i.e. burns and fractures. These tracheostomies are often temporary measures until corrective surgery can be performed or the infant has outgrown his or her susceptibility to critical airway compromise.
before day 10 after the burn was associated with less subglottic stenosis (15%) compared with delayed tracheostomy from day 10 onwards (>50%).28 Although this suggests that pressure and friction on the burned laryngeal tissue contribute to constrictive scarring, the pathogenesis of subglottic stenosis has not been clearly elucidated.28 Other arguments enforced by proponents of early tracheostomy in severely burned paediatric patients include easier handling during dressing changes, superiority in the management of severe respiratory failure and mucus plugging, and safety, bearing in mind the potentially disastrous results of accidental extubation in those in whom significant upper airway swelling precludes rapid reintubation.29,41 Others prefer a more conservative approach indicating a low overall incidence of complications with transpharyngeal intubation.42 A reasonable practice may be to perform elective tracheostomy if significant (supra)glottic oedema is present on laryngoscopy, because this subgroup is at high risk of critical airway incidences.30
Tracheostomy in the paediatric intensive care unit
Tracheostomy for long-term ventilation/ pulmonary toilet
Early reports of long-term intubation in adults suggested an increasing risk of laryngeal damage from the second week of intubation onwards.33,34 Although no official guidelines exist, it is a frequently adopted policy in adult intensive care units to tracheotomise patients after 1–2 weeks of intubation if a need for long-term invasive respiratory support is expected. The rationale for this, however, is not only based on worries about laryngeal complications, but also on economic constraints requiring patients to be moved rapidly from intensive care.35 Managing an adult patient on prolonged respiratory support via tracheostomy is more cost-effective as less sedation, less monitoring and hence fewer personnel are needed. In paediatric patients, the risks and implications of tracheostomy are not the same as they are in adults. No time limit for the transition to tracheostomy has ever met with wide acceptance. Transpharyngeal intubation is usually continued for prolonged periods as long as neuromuscular or pulmonary recovery is expected. Positive experience with prolonged transpharyngeal intubation was gained early in the discipline of paediatric intensive care.36,37 In a retrospective study, Lee et al. showed that the duration of intubation was not predictive of the likelihood of tracheostomy in children, and that the decision to tracheotomise a patient was made on an individual basis.38 This concurs with the observation that prolonged intubation does not seem to be a significant risk factor for post-extubational laryngeal complications in children, thereby negating the argument for early tracheostomy.39,40 Whether this holds true in burn patients remains controversial. An adverse impact of prolonged transpharyngeal intubation has been described in 36 severely burned paediatric patients, in whom transition to tracheostomy
Ethical questions need to be answered before the indications for long-term ventilation and tracheostomy can be discussed. Physicians tend to underestimate their patients’ quality of life, their prediction of what life-saving procedures a patient would want more closely reflecting their own personal preferences than those of their patients.43,44 When a decision to tracheotomise a child is made, the goal must be clearly defined and discussed with the family beforehand. The issue is complicated because ventilation is often commenced in an acute crisis, leaving no opportunity to discuss the possibility of long-term ventilation in advance and thereby offering little choice to the child and the family. Unfortunately, even when the need for ventilatory support can be anticipated early, as in older paediatric patients with progressive disorders, long-term ventilation and end-of-life care are often not discussed with the patient and the family. There are no comprehensive recommendations for an invasive versus a non-invasive mode of ventilatory support in ventilator-dependent children. Recent surveys suggest that young age, ventilator-dependency for most of the day and frequent episodes of respiratory exacerbation with need for intubation increase the likelihood of being tracheotomised.45–47 An increasing number of studies, however, report successful long-term, non-invasive ventilation via a nasal or face mask in infants and young children.8,9,48,49 Experience in the non-invasive ventilation of young children and a proficient technical service capable of providing individually adjusted interfaces are prerequisites for success because ventilator–patient asynchrony and pressure sores are not uncommon problems.9,50 In addition, secondary mid-face hypoplasia has emerged as a worrisome complication of long-term mask application in young children.9,51 This is not only a cosmetic issue: it also
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has a significant effect on dentition and on nasopharyngeal patency in patients susceptible to upper airway obstruction. The amount and duration of pressure applied by strapping the mask to the face as well as periods of growth spurt, may have an impact on the incidence and extent of this complication. Diaphragmatic pacing has the potential to reduce the need for a bulky mechanical ventilator with certain causes of ventilator-dependence.52 Technological refinements, enhanced expertise and reasonable pricing would increase its role in managing ventilator-dependent children. Most children will, however, still require a tracheostomy or continuous positive airway pressure to prevent upper airway obstruction during electrically stimulated inspirations. Other developments, for example the mechanical in-exsufflator, might decrease the need for invasive mechanical ventilation by facilitating and prolonging the non-invasive management of chronic respiratory failure in children with neuromuscular diseases.53 In accordance with common practice elsewhere,45–47 the authors currently favour primary tracheostomy in young children and infants who are ventilator-dependent, while older children (over 8 years of age) who are able to sustain spontaneous breathing during the daytime are good candidates for non-invasive ventilation. This age limit, however, is by no means absolute. Instead, it represents an arbitrary consensus aiming at minimising the risk of mid-facial hypoplasia.
How to approach the indication for tracheostomy With the increasing complexity of the underlying medical problems, the decision to tracheotomise a child has become an interdisciplinary process involving intensive care and pulmonology specialists, paediatric otorhinolaryngologists, paediatric surgeons, social care workers and respiratory nurses. The attitudes of patients and caregivers influence the choice of treatment just as much as socioeconomic factors, funding and the availability of professional support at home. Questions that may help the decision to undertake a tracheostomy are proposed in Table 2. Rare relative contraindications – local skin infections, refractory eczema, the presence of an engorged pretracheal venous plexus in patients with advanced superior vena cava syndrome, and self-mutilation in mentally handicapped children – are generally insufficient reasons not to undertake a tracheostomy.
FUTURE DEVELOPMENTS Percutaneous dilational tracheostomy has increased in popularity for temporary respiratory management in adult intensive care units.35,54 Little experience, however, exists in children.55 Provided that dilators and cannulas of appropriate sizes are available, percutaneous dilational tracheostomy might be an alternative in selected patients, for
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Table 2
Criteria favouring tracheostomy in children
The child with upper airway obstruction U Low chance of definitive, spontaneous resolution within a reasonable time (weeks) U Low probability that surgery can definitely correct the cause U High risk of critical upper airway obstruction with simple respiratory tract infections or minor bleeding (epistaxis) U High risk of or previous history of difficulties in airway management in case of an emergency U Difficult-to-control gastro-oesophageal reflux The child requiring long-term ventilation/ pulmonary toilet U Young age with a high risk of mid-facial deformation from mask pressure U Ventilator dependency for most of the day (more than 12 hours per day) U Inability to cope with a mask (full face or nasal mask) U Recurrent aspirations (gastro-oesophageal reflux, laryngeal incompetence) with significant benefit from pulmonary toilet U Safety-measures and local experience highly in favour of invasive ventilation
example children with Guillain-Barre´ syndrome or traumatic brain injury. Similarly, transtracheal oxygen catheters used for long-term oxygen therapy in adult patients have not gained widespread acceptance in paediatric practice; these are not without complications (dislodging, mucus plugging, local infection).56 Reported advantages include cosmetic benefits, a reduction in oxygen flow rates compared with nasal cannulae, and an avoidance of conventional tracheotomy.56,57
CONCLUSION The indications for tracheostomy have changed over time. Few children are now tracheotomised for airway emergencies; instead, the procedure is mostly performed for long-term problems. As a consequence, there is in most clinical situations no unity of approach, and the indication for tracheostomy is influenced by multiple circumstantial factors.
PRACTICE POINTS In patients with progressive neuromuscular or respiratory disease, the possibility of tracheostomy should be discussed with the caregivers and the patient before a foreseeable emergency situation arises. Tracheostomy in burn patients should be considered early if significant mucosal damage is seen by laryngoscopy.
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