**5. Craniofacial vascular malformations (VMs)**

Vascular anomalies are disorders of abnormal vasculogenesis or lymphogenesis. They can involve any part of body and can present in any phase of development. They include different types according to histopathology and anatomical site.

VMs may be with other syndromic malformations such as Parkes-Weber, Sturge-Weber, Klippel-Trenaunay Servelle-Martorell, PHACE and LUMBAR syndromes.

#### **5.1 Subglottic hemangiomas**

*Overview*: infantile hemangioma (IH) is the most prevalent vascular anomaly of the head and neck. It may cause a life-threatening airway obstruction if located in the glottic/subglottic area.

*Special Considerations in Human Airway Management*

and intubation difficult to perform [39, 40].

airway must be immediately implemented.

**4.8 Mucopolysaccharidosis (MPS)**

the airway operator.

phalos repair or hepatectomy.

intubation.

interventions in infancy such as tongue reduction surgeries, hernia repair, exom-

must be evaluated in addition to the preoperative glucose homeostasis.

Preoperative assessment should include a careful evaluation of the airway focusing on the mouth opening, tongue size, and preoperative head and neck imaging if required. It should focus also on other systems involvement such as the heart, hence an echocardiography is recommended. The genito-urinary system and the liver

The major challenge during airway management of a patient with Beckwith-Wiedemann syndrome pertains to the huge tongue which might completely obstruct the airway with anesthetic induction rendering the bag-mask ventilation

Hence a careful plan A, with a backup plan B and a plan C must be organized beforehand. An intravenous line is recommended to be secured before induction. Keeping spontaneous ventilation is considered to be the safest approach to the airway that can be achieved with intravenous anesthetics or inhalational Sevoflurane and by avoiding non-depolarizing neuro-muscular blockers. Some practices may use muscle relaxants after making sure of the ability to ventilate by a trial of ventilation after induction. Sugammadex muscle (rocuronium antidote) has allowed more confidence for anesthesiologists to give muscle relaxants in order to facilitate

A variety of tools can be used for intubation ranging from the direct laryngoscopes aided by a Gum elastic Bougie or a stylet, to the Videoscopes including the

Extubation must only occur when patient is fully awake and may be helped with a nasopharyngeal airway "in-situ" to help overcoming the tongue's obstruction in the immediate post-operative period. Patients with BWS must have a post-operative monitoring after surgery in the high dependency area or the intensive care unit.

A plan B consists of inserting a laryngeal mask airway should ventilation become problematic, and in case of the latter's failure, a plan C with a surgical

*Overview*: the mucopolysaccharidoses (MPS) are a set of storage diseases caused by a disordered or absent lysosomal hydroxylase enzyme leading to the build-up of muco-polysaccharides (glycosaminoglycans (GAG)) in the connective tissues, the musculo-skeletal system and the visceral organs. They are classified as seven syndromes with each involving a mix of 11 enzymatic disorders [41]. Mucopolysaccharidoses patients are associated with a high incidence of difficult bag-mask ventilation which represents the "nightmare" for the anesthesiologist or

*Incidence*: as a rare set of conditions, MPS accounts for less than 0.1% of all genetic but have been reported throughout the world in various forms. Region and

*Pathophysiology*: the mutation in lysosomal hydroxylase enzyme leads to an impaired metabolism of mucopolysaccharides (GAG) which will accumulate in multiple body tissues leading to macroglossia, adenotonsillar hypertrophy, and

Some types of MPS (Type1: Hurler, Type 4: Morquio, and Type 6: Maroteaux-Lamy) may be associated with atlantoaxial subluxation and odontoid hypoplasia which may lead to spinal cord compression with subsequent neurological deficit. Accumulation in the cervical spines may lead to a short and fixed neck. The

C-MAC and the Glidescope, to the fiberoptic bronchoscope.

ethnic background may affect the phenotype of MPS. *Genetic*: inheritance autosomal recessive.

laryngeal/tracheal tissue distortion and narrowing.

**172**

*Incidence*: its prevalence is approximately 4–5% mainly in fair-skinned kids. It is more common in girls and commonly associated with cutaneous hemangiomas.

*Pathophysiology*: the infantile hemangioma is a benign proliferative disorder of the vascular endothelial-like cells. It is believed to be due to a dysregulated angiogenesis resulting in neovascularization caused by the disordered differentiation of the embryonic mesenchymal cells. It has two phases, a proliferative one which starts as early as the first few weeks of life and continues progression and growth in size until late childhood, and an involution phase where the tumor begins shrinking in size until complete disappearance which is eventually due to the replacement of the endothelial cells with fibroblasts and fat cells. IH may occur at any place in the head and neck, starting as a small lump and then enlarging in size. Patients with IH are usually asymptomatic unless the tumor is present in the airway and reaches considerable size to cause obstructive symptoms. Hemangiomas of the airway may cause snoring, hemoptysis, hoarseness, stridor, and respiratory distress, which requires early intervention. Propranolol therapy plays a great role in its shrinkage [43].

*Airway evaluation and management*: history, imaging (MRI), and endoscopic studies are essential in the preoperative evaluation. Patients with glottic/subglottic lesions may easily get airway obstruction upon anesthetic induction. An LMA/ supraglottic for rescue ventilation may not be an option if the obstruction is infraglottic. Laryngoscopy and intubation may be complicated by bleeding if the tumor is traumatized which may completely impede the view and render airway management more challenging. Preserving spontaneous breathing with either inhalational or intravenous anesthetic and avoiding muscle relaxants is a safe approach until the airway is secured. Postoperative extubation should not be attempted until resolution of the airway edema. Intensive care (PICU) and postoperative steroids are indicated after major airway lesions endoscopic excision.

#### **5.2 Lymphatic/lymphaticovenous malformations (cystic hygroma)**

*Overview*: also called cavernous hemangioma. It is a multi-loculated benign congenital tumor affecting preferably the head and neck. It can present as a neck mass on prenatal ultrasound [5]. Airway compromise can be the sequelae of direct compression from the mass and/or bony changes following mandibulomaxillary hypertrophy (**Figure 8**).

*Incidence*: approximately 1 in 6000 live births, 70–80% occur in the neck, usually in the post cervical triangle.

*Pathophysiology*: it is a combination of lymphatic and venous cells. They may present at different sizes and progress in growth after birth. It might get complicated with bleeding or infection which may considerably increase their size. The clinical presentations are related to its size and the anatomy involved.

*Clinical evaluation and airway management*: large masses involving the airway can cause potential airway compromise according to the location and the size of the tumor. Tracheal compressions can be present with neck and mediastinal lesions. Tongue, pharyngeal or laryngeal involvement may present with significant airway obstruction. The treatment is often LASER or surgical excision and always requires a pre-inserted tracheostomy or an "in place" endotracheal tube prior to the procedure. Imaging is required in all cases, and tumors located in the mediastinum require further investigation. Sedatives should be used with caution because of their potential to aggravate the airway obstruction. Maintaining spontaneous ventilation via inhalational or intravenous induction is the cornerstone of a safe anesthetic approach when managing the airway of patients with CH. In case of complex and high-risk lesions, a tracheostomy under local anesthesia may be indicated prior to the procedure. Aspirating the cyst to shrink its size prior to

**175**

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

facilitate intubation has been reported but at the expense of complicating further the surgery. Post-operative intensive care (PICU) monitoring is indicated for

Arterio-venous malformations (AVM) involving the face and the airway are rare to occur. The main issue with airway management is the risk of massive bleeding that can occur if the AVM has been traumatized when managing the airway.

*Clinical evaluation and airway management of children with vascular malformations involving the airway*: a thorough preoperative history and physical exam are of utmost importance in anticipating a potentially difficult airway in children with vascular malformations. Clinical imaging (CT, MRI and 3-D reconstruction) and endoscopic evaluation may be necessary to establish the diagnosis and identify the size and location of the pathology. Even with a meticulous airway evaluation, an unanticipated difficult airway can still occur, hence an effective preparation before airway evaluation is crucial. An airway management strategy should be tailored according to each patient's airway pathology including a back-up approach should

General anesthesia with an inhaled agent such as Sevoflurane or an intravenous agent (such as propofol, ketamine or dexmedetomidine) while maintaining spontaneous ventilation is the technique of choice. Video laryngoscopy, FOB-guided endotracheal intubation, and endotracheal intubation through an LMA are the most

A pre-procedure tracheostomy inserted under local anesthesia may be indicated

The airway management of children with craniofacial abnormalities imposes great challenges owing to the difficulties in ventilation and intubation potentially encountered in most of the syndromes as well as the other systems involvement. A careful anticipation, preparation, and planning are key elements for a safe and successful airway management. The approach to each patient should be tailored according to the extent of the relevant abnormalities following a strategy of a simple clear plan A with a backup plan B and C. Preserving spontaneous breathing is one

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

high-risk lesions [45, 46].

**Figure 8.**

**5.3 Arterio-venous malformations**

*Huge movable mass is found out on the left neck [44].*

popular strategies for intubation.

**6. Conclusion**

the initial one fails and an emergency rescue plan [47].

for high risk malformations with severe airway obstruction [48].

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

**Figure 8.** *Huge movable mass is found out on the left neck [44].*

*Special Considerations in Human Airway Management*

indicated after major airway lesions endoscopic excision.

hypertrophy (**Figure 8**).

in the post cervical triangle.

**5.2 Lymphatic/lymphaticovenous malformations (cystic hygroma)**

clinical presentations are related to its size and the anatomy involved.

*Overview*: also called cavernous hemangioma. It is a multi-loculated benign congenital tumor affecting preferably the head and neck. It can present as a neck mass on prenatal ultrasound [5]. Airway compromise can be the sequelae of direct compression from the mass and/or bony changes following mandibulomaxillary

*Incidence*: approximately 1 in 6000 live births, 70–80% occur in the neck, usually

*Pathophysiology*: it is a combination of lymphatic and venous cells. They may present at different sizes and progress in growth after birth. It might get complicated with bleeding or infection which may considerably increase their size. The

*Clinical evaluation and airway management*: large masses involving the airway can cause potential airway compromise according to the location and the size of the tumor. Tracheal compressions can be present with neck and mediastinal lesions. Tongue, pharyngeal or laryngeal involvement may present with significant airway obstruction. The treatment is often LASER or surgical excision and always requires a pre-inserted tracheostomy or an "in place" endotracheal tube prior to the procedure. Imaging is required in all cases, and tumors located in the mediastinum require further investigation. Sedatives should be used with caution because of their potential to aggravate the airway obstruction. Maintaining spontaneous ventilation via inhalational or intravenous induction is the cornerstone of a safe anesthetic approach when managing the airway of patients with CH. In case of complex and high-risk lesions, a tracheostomy under local anesthesia may be indicated prior to the procedure. Aspirating the cyst to shrink its size prior to

*Incidence*: its prevalence is approximately 4–5% mainly in fair-skinned kids. It is more common in girls and commonly associated with cutaneous hemangiomas. *Pathophysiology*: the infantile hemangioma is a benign proliferative disorder of the vascular endothelial-like cells. It is believed to be due to a dysregulated angiogenesis resulting in neovascularization caused by the disordered differentiation of the embryonic mesenchymal cells. It has two phases, a proliferative one which starts as early as the first few weeks of life and continues progression and growth in size until late childhood, and an involution phase where the tumor begins shrinking in size until complete disappearance which is eventually due to the replacement of the endothelial cells with fibroblasts and fat cells. IH may occur at any place in the head and neck, starting as a small lump and then enlarging in size. Patients with IH are usually asymptomatic unless the tumor is present in the airway and reaches considerable size to cause obstructive symptoms. Hemangiomas of the airway may cause snoring, hemoptysis, hoarseness, stridor, and respiratory distress, which requires early intervention. Propranolol therapy plays a great role in its shrinkage [43]. *Airway evaluation and management*: history, imaging (MRI), and endoscopic studies are essential in the preoperative evaluation. Patients with glottic/subglottic lesions may easily get airway obstruction upon anesthetic induction. An LMA/ supraglottic for rescue ventilation may not be an option if the obstruction is infraglottic. Laryngoscopy and intubation may be complicated by bleeding if the tumor is traumatized which may completely impede the view and render airway management more challenging. Preserving spontaneous breathing with either inhalational or intravenous anesthetic and avoiding muscle relaxants is a safe approach until the airway is secured. Postoperative extubation should not be attempted until resolution of the airway edema. Intensive care (PICU) and postoperative steroids are

**174**

facilitate intubation has been reported but at the expense of complicating further the surgery. Post-operative intensive care (PICU) monitoring is indicated for high-risk lesions [45, 46].

#### **5.3 Arterio-venous malformations**

Arterio-venous malformations (AVM) involving the face and the airway are rare to occur. The main issue with airway management is the risk of massive bleeding that can occur if the AVM has been traumatized when managing the airway.

*Clinical evaluation and airway management of children with vascular malformations involving the airway*: a thorough preoperative history and physical exam are of utmost importance in anticipating a potentially difficult airway in children with vascular malformations. Clinical imaging (CT, MRI and 3-D reconstruction) and endoscopic evaluation may be necessary to establish the diagnosis and identify the size and location of the pathology. Even with a meticulous airway evaluation, an unanticipated difficult airway can still occur, hence an effective preparation before airway evaluation is crucial. An airway management strategy should be tailored according to each patient's airway pathology including a back-up approach should the initial one fails and an emergency rescue plan [47].

General anesthesia with an inhaled agent such as Sevoflurane or an intravenous agent (such as propofol, ketamine or dexmedetomidine) while maintaining spontaneous ventilation is the technique of choice. Video laryngoscopy, FOB-guided endotracheal intubation, and endotracheal intubation through an LMA are the most popular strategies for intubation.

A pre-procedure tracheostomy inserted under local anesthesia may be indicated for high risk malformations with severe airway obstruction [48].

#### **6. Conclusion**

The airway management of children with craniofacial abnormalities imposes great challenges owing to the difficulties in ventilation and intubation potentially encountered in most of the syndromes as well as the other systems involvement. A careful anticipation, preparation, and planning are key elements for a safe and successful airway management. The approach to each patient should be tailored according to the extent of the relevant abnormalities following a strategy of a simple clear plan A with a backup plan B and C. Preserving spontaneous breathing is one

of the safe practices when patients are at risk of difficult ventilation. The use of the most familiar airway tools is pivotal in achieving favorable results. Extubation is an integral part of the airway management and should only be performed when the child meets its criteria and in a controlled environment. Post-extubation monitoring in a critical care or an observation area must be carried out for patients at risk of airway obstruction.
