**2. Pediatric difficult airway evaluation**

#### **2.1 General airway assessment**

General clinical history should focus on the presence of any problem affecting the airway or the respiratory system including history of snoring or apneas, upper respiratory tract infection (URTI), croup, stridor, voice hoarseness, recurrent aspirations, asthma, parental smoking, and most importantly a history of previous difficult airway management [2].

The general airway examination must include: a baseline oxygen saturation on room air, respiratory rate, preferred body position (prone position must be redflagged as upper airway obstruction), mouth breathing, existence of any obstruction manifested by intercostal/suprasternal retractions, oral examination of the mouth opening, teeth, tongue size and Mallampati score, shape and position of the mandible, hyo-mental distance, and neck length/mobility.

Nevertheless, the airway examination in children may be difficult to perform in detail especially with an uncooperative child, thus it may be restricted to the general observation of the face, mandible, and breathing pattern in such situations.

The most critical step in airway examination of children pertains at taking a lateral "profile" look of the mandible which could spot a micrognathia or a retracted mandible that might be masked with the frontal look.

General diagnostic tests: they are seldom required especially when further details of the airway are needed. An X-ray of the head and neck can show the place and the level of upper airway obstruction; however, a CT scan/MRI can provide further details (especially in tumors and vascular malformations of the airway). CT virtual endoscopy (VE) is an excellent tool used to obtain an anatomically similar representation of the intraluminal geography of the airway, including supraglottic, glottic, and subglottic structures without the risk of exposure to ionizing radiation. Compared to conventional 3-D reconstructions, the images obtained through virtual endoscopy create the impression of a true endoscopic image allowing for a tailored approach toward the airway management. A flexible fiberoptic endoscopy maybe required in children with airway pathology especially children with an unexplained hoarse voice, suprasternal/intercostal retractions, and chronic aspirations.

### **2.2 Focused airway evaluation**

The clinical evaluation should focus on risk factors which may potentially contribute to difficult airway management including (**Table 1**) [3]:


**155**

advisable.

airway.

potential difficult airway.

cranio-facial anomalies.

*An Approach to the Airway Management in Children with Craniofacial Anomalies*

Overbite Pierre Robin-Treacher Collins-Goldenhar

Small mouth Pierre-Robin, Treacher-Collins, Goldenhar, Down's

Palate (high arched) Down, Crouzon, Apert, Pierre Robin, Treacher Collins,

Cleft Palate Pierre Robin, Treacher Collins, Goldenhar, Down's,

Tongue abnormality (Large or Glossoptosis) Beckwith-Wiedemann, Mucopolysaccharidosis, Pierre Robin.

Cervical spine abnormalities Down's syndrome, Klippel-Feil, Mucopolysaccharidosis, Goldenhar

Large mouth Mucopolysaccharidosis, Beckwith-Wiedemann

syndrome

Mucopolysaccharidosis

Pierre Robin, Treacher Collins, Goldenhar,

Apert, Crouzon

*DOI: http://dx.doi.org/10.5772/intechopen.93426*

**Anatomical malformation Syndrome**

Mandibular prognathism Apert

8.History of a previous failed airway;

Short thyromental distance (less than 3 finger breadths used by the own child's fingers)

Subglottic narrowing Down's syndrome Tracheal abnormality (tortuosity) Mucopolysaccharidosis

*Anatomical malformations related to difficult airway in craniofacial syndromes.*

quent plan B and C in case of failure of the former.

the airway.

**Table 1.**

9.Soft tissue tumors and vascular malformations with significant obstruction of

**3. General airway management in patients with craniofacial syndromes**

has many proposed algorithms but not unified as in the American Society of Anaesthesiologists (ASA) adult difficult airway algorithm [4–7]. The awake intubation is no more a popular option in pediatric intubations except for some emergency situations where the patient is in severe distress and obstruction, as it carries its own disadvantages (increase in intracranial pressure ICP and intracerebral hemorrhage ICH, gagging, uncooperative kid), hence induction of anesthesia with preservation of spontaneous breathing is the cornerstone for a safe airway management in patients with craniofacial syndromes with suspected difficult

The airway management plan of the infants and children with difficult airway

The airway provider should set a structured strategy for the management of the airway. Common practice is to have a "Plan A" as an initial approach with subse-

A secured peripheral IV line prior to induction is recommended in patients with

Presence of two airway experts (pediatric anesthesiologists) during induction is

An otolaryngologist attendance for emergency backup surgical access (Bronchoscopy/Tracheostomy) is recommended during the management of the *An Approach to the Airway Management in Children with Craniofacial Anomalies DOI: http://dx.doi.org/10.5772/intechopen.93426*


#### **Table 1.**

*Special Considerations in Human Airway Management*

mandible, hyo-mental distance, and neck length/mobility.

mandible that might be masked with the frontal look.

difficult airway management [2].

and chronic aspirations.

syndrome;

syndrome;

difficult ventilation);

maintain in prone positioning;

**2.2 Focused airway evaluation**

respiratory tract infection (URTI), croup, stridor, voice hoarseness, recurrent aspirations, asthma, parental smoking, and most importantly a history of previous

The general airway examination must include: a baseline oxygen saturation on room air, respiratory rate, preferred body position (prone position must be redflagged as upper airway obstruction), mouth breathing, existence of any obstruction manifested by intercostal/suprasternal retractions, oral examination of the mouth opening, teeth, tongue size and Mallampati score, shape and position of the

Nevertheless, the airway examination in children may be difficult to perform in detail especially with an uncooperative child, thus it may be restricted to the general

The most critical step in airway examination of children pertains at taking a lateral "profile" look of the mandible which could spot a micrognathia or a retracted

General diagnostic tests: they are seldom required especially when further details of the airway are needed. An X-ray of the head and neck can show the place and the level of upper airway obstruction; however, a CT scan/MRI can provide further details (especially in tumors and vascular malformations of the airway). CT virtual endoscopy (VE) is an excellent tool used to obtain an anatomically similar representation of the intraluminal geography of the airway, including supraglottic, glottic, and subglottic structures without the risk of exposure to ionizing radiation. Compared to conventional 3-D reconstructions, the images obtained through virtual endoscopy create the impression of a true endoscopic image allowing for a tailored approach toward the airway management. A flexible fiberoptic endoscopy maybe required in children with airway pathology especially children with an unexplained hoarse voice, suprasternal/intercostal retractions,

The clinical evaluation should focus on risk factors which may potentially

1.An extremely short thyro-mental distance with an overbite, with micro/ retrognathia such as seen in Pierre Robin sequence and Treacher-Collins

3.A small oral opening and large tongue such as seen in Beckwith-Wiedemann

5.Stiff subcutaneous tissues as seen in Mucopolysaccharidosis (high risk of

7.Obstruction of the airway when in supine position and the need to continuously

4.Obstructive sleep apnea +/− secondary pulmonary hypertension;

6.Midface hypoplasia as seen in Apert and Crouzon syndromes;

contribute to difficult airway management including (**Table 1**) [3]:

2.A fixed neck such as in Klippel-Feil syndrome;

observation of the face, mandible, and breathing pattern in such situations.

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*Anatomical malformations related to difficult airway in craniofacial syndromes.*


#### **3. General airway management in patients with craniofacial syndromes**

The airway management plan of the infants and children with difficult airway has many proposed algorithms but not unified as in the American Society of Anaesthesiologists (ASA) adult difficult airway algorithm [4–7]. The awake intubation is no more a popular option in pediatric intubations except for some emergency situations where the patient is in severe distress and obstruction, as it carries its own disadvantages (increase in intracranial pressure ICP and intracerebral hemorrhage ICH, gagging, uncooperative kid), hence induction of anesthesia with preservation of spontaneous breathing is the cornerstone for a safe airway management in patients with craniofacial syndromes with suspected difficult airway.

The airway provider should set a structured strategy for the management of the airway. Common practice is to have a "Plan A" as an initial approach with subsequent plan B and C in case of failure of the former.

An otolaryngologist attendance for emergency backup surgical access (Bronchoscopy/Tracheostomy) is recommended during the management of the potential difficult airway.

A secured peripheral IV line prior to induction is recommended in patients with cranio-facial anomalies.

Presence of two airway experts (pediatric anesthesiologists) during induction is advisable.

**Plan A:** Preserve spontaneous ventilation:

• Induction using IV sedative medication: propofol infusion or boluses (also dexmedetomidine, ketamine)

Or

• Inhalation induction with Sevoflurane

While patient is spontaneously breathing, a careful insertion of the laryngoscope blade may be attempted provided that the patient tolerates without bucking norcoughing.

A wide range of airway tools and techniques have been described for the intubation. However, it is crucial that the airway operator sticks with the tool that he is mostly familiar with. It is noteworthy to keep in mind that an airway tool is not a plan.

Techniques used for intubation of syndromic children with difficult airway include:

1.**Direct laryngoscopy**: a regular laryngoscope blade (a Macintosh curved or a Miller straight blade).

The paraglossal approach with a straight blade is a well-described technique for intubation in children with difficult airway where conventional laryngoscopy technique fails. Using this technique may allow the operator to avoid the large tongue commonly encountered in cranio-facial syndromes via inserting the laryngoscope's straight blade through the trench between the tonsils and the base of the tongue. This technique allows a better exposure of an anterior larynx; however, it does not provide an adequate space for tube manipulation, hence it requires a high level of skills by the operator.

Intubation with direct laryngoscopy may be facilitated with gum elastic bougie or a stylet.


It is also challenging to perform F.O.B intubation in neonates who have a small airway as the field of vision in the distal lens is very narrow and might get easily obstructed whenever touching any obstacle, hence it requires a high-level skill set and expertise.

4.**Fiber-optic bronchoscope intubation through LMA**: F.O.B intubation through LMA is a popular technique of intubation for children with difficult airway. The LMA can serve for ventilation/oxygenation during the process

**157**

*An Approach to the Airway Management in Children with Craniofacial Anomalies*

of intubation which is a very favorable advantage especially in children with

However, the removal of the LMA might get complicated with accidental ETT extubation, thus many techniques have been described for the removal; however, the safest strategy remains keeping both the LMA and the ETT in

5.**Fiberoptic bronchoscope + GlideScope**: The fiberoptic bronchoscope can be used as a guiding bougie for intubation, while the view is provided by the Glidescope. This technique will require two personnel: with one handling the Glidescope while the other manipulating the F.O.B. It is called the video-assisted

**Plan B:** If Plan A fails and ventilation becomes problematic at any step of the airway management, then an LMA should be immediately inserted. If manual bag-mask ventilation gets possible after LMA insertion, then the operator has a choice whether to keep it if appropriate for the procedure, to proceed with F.O.B intubation through the LMA or to use other techniques for

If "Cannot ventilate" through LMA, then the operator should immediately move

**Plan C:** If attempts at ventilating via "Bag-Mask" and LMA fail, one attempt at

If all the previous attempts fail, then an emergency surgical airway must imminently be resorted to by the otolaryngologist with either an emergent rigid bron-

**Extubation and post-extubation strategies:** Extubation of patients with craniofacial anomalies directly at the end of the procedure should only be performed

direct laryngoscopy can be performed aiming at intubating if possible.

place after intubation and removing them together at extubation.

*DOI: http://dx.doi.org/10.5772/intechopen.93426*

difficult bag-mask ventilation.

fiberoptic intubation (VAFI) technique.

choscopy placement or an emergency tracheostomy.

1.An atraumatic intubation with minimal airway edema

3.Absence of a history of severe obstructive apnea

2.A minor short surgery not involving the airway (excluding adeno-

The extubation should be performed when the patient is fully awake with vigorous spontaneous breathing and resumption of airway reflexes. The anesthesiologist must be ready for a re-intubation with all the airway tools set up should the

Patients at risk of post-operative airway obstruction such as obstructive sleep apnea must be monitored overnight in a high dependency unit (Apnea

Patients with significant obstructive apneas undergoing major airway surgeries (such as mandibular distraction osteogenesis surgery) in which intubation might have been difficult or traumatic should not be extubated at the end of the procedure as they are at high risk of obstruction post-op which may necessitate an intubation that would likely be extremely difficult as exacerbated by the airway edema. Such patients should be kept intubated, properly sedated, and transferred to the ICU for post-monitoring. Intravenous steroids regimen should be given to minimize

intubation.

to plan C.

in the following conditions:

tonsillectomies)

extubation trial fail.

monitoring).

airway edema.

*An Approach to the Airway Management in Children with Craniofacial Anomalies DOI: http://dx.doi.org/10.5772/intechopen.93426*

of intubation which is a very favorable advantage especially in children with difficult bag-mask ventilation.

However, the removal of the LMA might get complicated with accidental ETT extubation, thus many techniques have been described for the removal; however, the safest strategy remains keeping both the LMA and the ETT in place after intubation and removing them together at extubation.

5.**Fiberoptic bronchoscope + GlideScope**: The fiberoptic bronchoscope can be used as a guiding bougie for intubation, while the view is provided by the Glidescope. This technique will require two personnel: with one handling the Glidescope while the other manipulating the F.O.B. It is called the video-assisted fiberoptic intubation (VAFI) technique.

**Plan B:** If Plan A fails and ventilation becomes problematic at any step of the airway management, then an LMA should be immediately inserted. If manual bag-mask ventilation gets possible after LMA insertion, then the operator has a choice whether to keep it if appropriate for the procedure, to proceed with F.O.B intubation through the LMA or to use other techniques for intubation.

If "Cannot ventilate" through LMA, then the operator should immediately move to plan C.

**Plan C:** If attempts at ventilating via "Bag-Mask" and LMA fail, one attempt at direct laryngoscopy can be performed aiming at intubating if possible.

If all the previous attempts fail, then an emergency surgical airway must imminently be resorted to by the otolaryngologist with either an emergent rigid bronchoscopy placement or an emergency tracheostomy.

**Extubation and post-extubation strategies:** Extubation of patients with craniofacial anomalies directly at the end of the procedure should only be performed in the following conditions:


The extubation should be performed when the patient is fully awake with vigorous spontaneous breathing and resumption of airway reflexes. The anesthesiologist must be ready for a re-intubation with all the airway tools set up should the extubation trial fail.

Patients at risk of post-operative airway obstruction such as obstructive sleep apnea must be monitored overnight in a high dependency unit (Apnea monitoring).

Patients with significant obstructive apneas undergoing major airway surgeries (such as mandibular distraction osteogenesis surgery) in which intubation might have been difficult or traumatic should not be extubated at the end of the procedure as they are at high risk of obstruction post-op which may necessitate an intubation that would likely be extremely difficult as exacerbated by the airway edema. Such patients should be kept intubated, properly sedated, and transferred to the ICU for post-monitoring. Intravenous steroids regimen should be given to minimize airway edema.

*Special Considerations in Human Airway Management*

**Plan A:** Preserve spontaneous ventilation:

• Inhalation induction with Sevoflurane

dexmedetomidine, ketamine)

Miller straight blade).

or a stylet.

patients group.

set and expertise.

accommodate the smallest F.O.B.

Or

norcoughing.

include:

• Induction using IV sedative medication: propofol infusion or boluses (also

While patient is spontaneously breathing, a careful insertion of the laryngoscope blade may be attempted provided that the patient tolerates without bucking

A wide range of airway tools and techniques have been described for the intubation. However, it is crucial that the airway operator sticks with the tool that he is mostly familiar with. It is noteworthy to keep in mind that an airway tool is not a plan. Techniques used for intubation of syndromic children with difficult airway

1.**Direct laryngoscopy**: a regular laryngoscope blade (a Macintosh curved or a

The paraglossal approach with a straight blade is a well-described technique for intubation in children with difficult airway where conventional laryngoscopy technique fails. Using this technique may allow the operator to avoid the large tongue commonly encountered in cranio-facial syndromes via inserting the laryngoscope's straight blade through the trench between the tonsils and the base of the tongue. This technique allows a better exposure of an anterior larynx; however, it does not provide an adequate space for tube manipulation,

Intubation with direct laryngoscopy may be facilitated with gum elastic bougie

2.**Video laryngoscopy**: video laryngoscopes such as the Storz video laryngoscope or the Glidescope have been successfully used for intubation in syndromic children with difficult airway. Despite the fact that the Glidescope provides an easy view, the intubation may be challenging due to the small "working" space and the curvature of the airway, especially in the smaller

3.**Fiberoptic bronchoscope intubation**: F.O.B intubation is a popular option as "first attempt" technique or as an alternative to a failed direct laryngoscopy and other techniques. It can be performed nasally, orally or through an LMA. However, its main disadvantage is that it might not be an option for neonates and smaller infants who require smaller endotracheal tubes that may barely

It is also challenging to perform F.O.B intubation in neonates who have a small airway as the field of vision in the distal lens is very narrow and might get easily obstructed whenever touching any obstacle, hence it requires a high-level skill

4.**Fiber-optic bronchoscope intubation through LMA**: F.O.B intubation through LMA is a popular technique of intubation for children with difficult airway. The LMA can serve for ventilation/oxygenation during the process

hence it requires a high level of skills by the operator.

**156**
