**4.5 Goldenhar's syndrome (GS)**

*Special Considerations in Human Airway Management*

dysfunction and mouth opening limitation. TCS is associated with Cleft Palate in 30% of the cases, and pharyngeal hypoplasia is commonly associated as well. All these anomalies can disrupt several functions like breathing, swallowing, chewing, and speech. Systemic manifestations, namely cardiac, renal, and skeletal, especially

*Treacher Collins syndrome with characteristic facial features including downward and laterally slanting palpebral fissures, paucity of lashes and lack of naso-frontal angle, bird like appearance, micrognathia,* 

*Airway and anesthetic implications*: Treacher Collins syndrome (TCS) was firstly reported as a hazard for general anesthesia in 1963 by Edward Ross due to the difficulty in maintaining a free and adequate airway. Several associated mechanisms are the cause, namely mandibular hypoplasia, micrognathia, retrognathia, posterior displacement of the bulky tongue, and the pharyngeal hypoplasia, resulting in a small and narrow retromandibular space [29, 30]. The presence of a temporomandibular joint abnormality or a small mouth aperture may further worsen the case. Thus, upper airway obstruction is common at induction of anesthesia and may require an airway manual maneuver (a two-hand mask ventilation, chin lift, and jaw thrust) and insertion of an oropharyngeal/nasopharyngeal airway. If unsuccessful, insertion of a supraglottic airway device such as a laryngeal mask airway before intubation is indicated. In addition, the failure rate of direct intubation is extremely high owing to the difficulty in alignment of the three axes (oral, pharyngeal, and laryngeal axes), which complicates further the visualization of the glottis. The degree of severity worsens with increasing age mainly because of the decreased

cervical vertebral defects, may also be observed [1, 4, 6, 7, 17].

*microtia (deformed pinna), macrostomia, and large tongue [27].*

**166**

**Figure 4.**

mandibular growth.

*Overview*: Goldenhar's syndrome, also called Facio-auriculo-vertebral syndrome or Oculo-Auriculo-Vertebral syndrome, is a variant of hemifacial microsomia disorders that affect the eye, ear, nose, lip, soft palate, and mandible, and often associated with vertebral and cardiac anomalies. It was initially reported in 1952 by a Belgian-American ophthalmologist Maurice Goldenhar. In 1963, Gorlin introduced the term "Oculo-Auriculo-Vertebral syndrome" due to the presence of associated vertebral anomalies (**Figure 5**).

*Genetics*: the exact etiology remains unclear. Most cases are sporadic, but some rare familial cases were reported suggesting autosomal dominant or recessive inheritance [33].

*Incidence*: Goldenhar's syndrome occurs in about 1 in 3000 to 1 in 5000 live births, affecting males predominantly. Male to female ratio is 2:1.

*Pathophysiology*: this hemifacial microsomia is caused by the underdevelopment of the first and second branchial arches during the 4th week of gestation resulting in craniofacial anomalies, ocular anomalies, vertebral anomalies, and cardiac defects.

*Clinical considerations*: Goldenhar's syndrome (GS) is a multisystem syndrome with a wide spectrum of clinical features. Craniofacial anomalies are unilateral in 90% of the cases and include mandibular hypoplasia, hypoplastic zygomatic arch, micrognathia, macrostomia, external and middle ear malformations (microtia, preauricular appendages, and atresia) often with sensorineural hearing loss, and eye anomalies (epibulbar dermoids, lipodermoids, microphthalmos, and coloboma). Nevertheless, in 10% of cases both facial sides may be affected with one side typically more affected than the other. Oral cavity anomalies like palate anomalies

#### **Figure 5.**

*Goldenhar's syndrome before and after surgical procedure for lip and palatoplasty, correction of macrostomia and nasal septum deformity, excision of pre-auricular tags: (A) preoperative; (B) postoperative [32].*

(high arch or cleft) and tongue anomalies are often associated. Additionally, almost 50% of children with Goldenhar's syndrome may have vertebral defects, especially at the cervical level, such as hemivertebrae or hypoplasia, along with a potential risk of subluxation at the atlanto-occipital joint. Neck movements are limited during flexion and extension, increasing the rate of difficult or failed tracheal intubation procedure. Congenital cardiac structural malformations (Tetralogy of Fallot and septal defects) are present in about one third of the cases. In rare cases, some degrees of mental retardation and other systemic anomalies, mainly genitourinary, pulmonary, and vascular are reported [4, 6, 7, 17].

*Airway and anesthetic implications*: anesthesia or sedation may be provided for children with Goldenhar's syndrome for various procedures (ear reconstruction, distraction osteogenesis, soft tissue reconstruction, skin graft, cardiac, etc.).

The anesthetic management is risky because of the difficult airway. The degree of difficulties depends directly on the severity of craniofacial anomalies and associated vertebral defects and tends to worsen progressively with increasing age. Detecting patients at risk in the preoperative setting may anticipate challenging airway situations. Consultation of previous anesthesia records is of a valuable help; however, the airway must be reassessed before any new airway manipulation. The degree of severity of mandibular hypoplasia correlates with difficult tracheal intubation. Virtual imaging using 3D CT or cone-beam computed tomography may be indicated for selected patients to assess the airway anatomy looking for anomalies [34].

Facemask ventilation is challenging in children with Goldenhar's syndrome due to a poor mask seal, often requiring the use of a gauze with self-adhesive tape to provide an adequate seal. The reason for this poor mask fit is the facial asymmetry and the presence of a soft tissue slit extended from the side of the mouth to the middle of the cheek on the abnormal hemi-face.

**169**

laryngoscope.

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

Tracheal intubation is usually more difficult to achieve than maintaining airway

Although fiberoptic intubation (FOB) technique is one of the popular options for securing difficult airways of patients with GS, various strategies can be adopted successfully to perform tracheal intubation; however, it is recommended that the airway operator sticks with the tool and technique that is the most familiar to his practice. These include: FOB through LMA (video-assisted fiberoptic intubation (VAFI)), GlideScope, Air-Q, Airtraq, C-MAC D-blade or C-MAC Miller-blade videolaryngoscopy, Pentax-AWS Airwayscope with tracheal introducer, Laryngeal Mask Airway helped by Pediatric Boussignac Bougie or retrograde tracheal intubation. The Truview PCD® laryngoscopy has proven its effectiveness and offers the advantage of continuously supplying oxygen via its oxygenation side port during the procedure. When the intubation is not required, LMA or nasopharyngeal airway

*Overview:* it was initially described by Klippel and Feil in 1912 as a clinical triad

*Genetics*: Klippel-Feil syndrome is an inherited autosomal dominant condition. It is the result of an anomalous partition of the cervical somites during embryogenesis

*Clinical considerations*: KFS is classified into three classes according to the level and severity of the spine fusion, with type 1 representing a considerable fusion of numerous cervical vertebrae, type 2 representing fusions of one or two cervical vertebrae, and type 3 representing cervical vertebrae fusions combined with thoracic

KPS may be related to other anomalies such as cranio-facial deformities includ-

Neurological deficits with hyperlaxed cervical spines and neuro-degeneration

*Airway and anesthetic implications*: KFS is often associated with difficult intubation, evidently because of the fixed neck which does not allow proper extension of the atlanto-axial joint during laryngoscopy and the subsequent advanced Cormack-Lehane view. Many reports describe a grade 4 view with a conventional

ing craniosynostosis, cleft lip, micrognathia, and laryngeal defects. Thoracic deformities such as scoliosis may be present in addition to other skeletal deformities like scapular elevation (Sprengel's). Uro-genital malformations are commonly pres-

Screening patients at risk for cervical spine instability is a crucial step before airway management in order to avoid spinal injury, especially those with

ent as well as congenital heart defects most commonly the VSD.

with fused spines are commonly associated with KPS [17].

of an extremely short "compressed" neck, a low hairline at the rear end of the skull, and a limited mobility of the neck caused by fusion of two or more cervical vertebrae. Despite being discovered as a triad, it is not uncommon for patients with KFS to meet just one clinical criterion of the above. It may be associated with other

*Incidence*: KFS has a prevalence of 1:40000 to 1:42000 of live births.

patency, especially in the case right-sided hemifacial microsomia. Several combined conditions may contribute to difficult intubation, including retrognathia, micrognathia, asymmetrical hypoplasia of the mandible, limited mouth opening, palatal anomalies, potentially associated vertebral defects, and limited neck motion. The hypoplastic mandible reduces the retromandibular space. The relatively bulky tongue is displaced posteriorly overhanging the larynx, thus making visualization of the vocal inlets difficult, even nearly impossible during conventional

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

constitutes a possible alternative [1, 35, 36].

between the third and eighth week of gestation.

**4.6 Klippel-Feil syndrome (KFS)**

and/or lumbar vertebral fusions.

anomalies (**Figure 6**).

laryngoscopy.

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

Tracheal intubation is usually more difficult to achieve than maintaining airway patency, especially in the case right-sided hemifacial microsomia. Several combined conditions may contribute to difficult intubation, including retrognathia, micrognathia, asymmetrical hypoplasia of the mandible, limited mouth opening, palatal anomalies, potentially associated vertebral defects, and limited neck motion. The hypoplastic mandible reduces the retromandibular space. The relatively bulky tongue is displaced posteriorly overhanging the larynx, thus making visualization of the vocal inlets difficult, even nearly impossible during conventional laryngoscopy.

Although fiberoptic intubation (FOB) technique is one of the popular options for securing difficult airways of patients with GS, various strategies can be adopted successfully to perform tracheal intubation; however, it is recommended that the airway operator sticks with the tool and technique that is the most familiar to his practice. These include: FOB through LMA (video-assisted fiberoptic intubation (VAFI)), GlideScope, Air-Q, Airtraq, C-MAC D-blade or C-MAC Miller-blade videolaryngoscopy, Pentax-AWS Airwayscope with tracheal introducer, Laryngeal Mask Airway helped by Pediatric Boussignac Bougie or retrograde tracheal intubation. The Truview PCD® laryngoscopy has proven its effectiveness and offers the advantage of continuously supplying oxygen via its oxygenation side port during the procedure. When the intubation is not required, LMA or nasopharyngeal airway constitutes a possible alternative [1, 35, 36].

#### **4.6 Klippel-Feil syndrome (KFS)**

*Special Considerations in Human Airway Management*

(high arch or cleft) and tongue anomalies are often associated. Additionally, almost 50% of children with Goldenhar's syndrome may have vertebral defects, especially at the cervical level, such as hemivertebrae or hypoplasia, along with a potential risk of subluxation at the atlanto-occipital joint. Neck movements are limited during flexion and extension, increasing the rate of difficult or failed tracheal intubation procedure. Congenital cardiac structural malformations (Tetralogy of Fallot and septal defects) are present in about one third of the cases. In rare cases, some degrees of mental retardation and other systemic anomalies, mainly genitourinary,

*Goldenhar's syndrome before and after surgical procedure for lip and palatoplasty, correction of macrostomia and nasal septum deformity, excision of pre-auricular tags: (A) preoperative; (B) postoperative [32].*

*Airway and anesthetic implications*: anesthesia or sedation may be provided for children with Goldenhar's syndrome for various procedures (ear reconstruction, distraction osteogenesis, soft tissue reconstruction, skin graft, cardiac, etc.).

The anesthetic management is risky because of the difficult airway. The degree of difficulties depends directly on the severity of craniofacial anomalies and associated vertebral defects and tends to worsen progressively with increasing age. Detecting patients at risk in the preoperative setting may anticipate challenging airway situations. Consultation of previous anesthesia records is of a valuable help; however, the airway must be reassessed before any new airway manipulation. The degree of severity of mandibular hypoplasia correlates with difficult tracheal intubation. Virtual imaging using 3D CT or cone-beam computed tomography may be indicated

Facemask ventilation is challenging in children with Goldenhar's syndrome due to a poor mask seal, often requiring the use of a gauze with self-adhesive tape to provide an adequate seal. The reason for this poor mask fit is the facial asymmetry and the presence of a soft tissue slit extended from the side of the mouth to the

for selected patients to assess the airway anatomy looking for anomalies [34].

pulmonary, and vascular are reported [4, 6, 7, 17].

middle of the cheek on the abnormal hemi-face.

**168**

**Figure 5.**

*Overview:* it was initially described by Klippel and Feil in 1912 as a clinical triad of an extremely short "compressed" neck, a low hairline at the rear end of the skull, and a limited mobility of the neck caused by fusion of two or more cervical vertebrae. Despite being discovered as a triad, it is not uncommon for patients with KFS to meet just one clinical criterion of the above. It may be associated with other anomalies (**Figure 6**).

*Genetics*: Klippel-Feil syndrome is an inherited autosomal dominant condition. It is the result of an anomalous partition of the cervical somites during embryogenesis between the third and eighth week of gestation.

*Incidence*: KFS has a prevalence of 1:40000 to 1:42000 of live births.

*Clinical considerations*: KFS is classified into three classes according to the level and severity of the spine fusion, with type 1 representing a considerable fusion of numerous cervical vertebrae, type 2 representing fusions of one or two cervical vertebrae, and type 3 representing cervical vertebrae fusions combined with thoracic and/or lumbar vertebral fusions.

KPS may be related to other anomalies such as cranio-facial deformities including craniosynostosis, cleft lip, micrognathia, and laryngeal defects. Thoracic deformities such as scoliosis may be present in addition to other skeletal deformities like scapular elevation (Sprengel's). Uro-genital malformations are commonly present as well as congenital heart defects most commonly the VSD.

Neurological deficits with hyperlaxed cervical spines and neuro-degeneration with fused spines are commonly associated with KPS [17].

*Airway and anesthetic implications*: KFS is often associated with difficult intubation, evidently because of the fixed neck which does not allow proper extension of the atlanto-axial joint during laryngoscopy and the subsequent advanced Cormack-Lehane view. Many reports describe a grade 4 view with a conventional laryngoscope.

Screening patients at risk for cervical spine instability is a crucial step before airway management in order to avoid spinal injury, especially those with

#### **Figure 6.**

*Child with Klippel-Feil syndrome and anomaly of the Occipito-cervical junction. The images show an elevated left shoulder due to a Sprengel anomaly, a short, webbed neck, and a low hairline (scar on the thorax after surgical repair of nonrestrictive atrial septal defect) [37].*

hyperlaxed spines who may require C-spine precautions with in-line-stabilization during intubation. This is performed via a lateral neck X-ray in flexion and extension positions [1, 4–7].

Obstructive sleep apnea (OSA) is not uncommonly encountered in patients with KFS, hence a preoperative sleep study may be indicated along with postoperative apnea monitor. A preoperative echocardiography must be considered especially in patients with pulmonary hypertension secondary to OSA and scoliosis.

A history of previous smooth intubation in KPS patient does not implicate a future favorable outcome due to the progressive nature of the disease. Hence, a careful preoperative assessment and planning is critical for safe airway management.

Bag-mask ventilation is likely difficult in those patients as well as direct laryngoscopy owing to the fixed neck or the in-line neck stabilization.

Should the older child be cooperative enough to sustain an awake fiberoptic intubation (with light sedation) that would be the approach of choice avoiding both the risks of difficult ventilation and neck injury secondary to airway manipulation.

Otherwise, a plan to preserve spontaneous ventilation during anesthetic induction should be performed with either total intravenous anesthesia (TIVA) or inhaled Sevoflurane (IV Dexmedetomidine and IV Ketamine can be used as well) avoiding the use of neuro-muscular blockers.

A trial of manual bag-mask to assess for ventilation is used by some practitioners before airway manipulation. The various arrays of airway tools including videoscopes (Glidescope/C-MAC etc.) as well as the fiberoptic bronchoscope have been used successfully for intubation in patients with KFS. They are preferred as first line intubation technique due to the lower incidence of cervical spine mobilization when used.

**171**

**Figure 7.**

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

to the severity of their cardio-pulmonary and airway pathology.

impose challenges to airway management (**Figure 7**).

*Incidence:* 1 per 13,700–15,000 of live births.

and adrenal carcinoma has been observed [6, 7, 17].

*Macroglossia in the patient with Beckwith-Wiedemann syndrome after intubation [38].*

If the patient is on a home BIPAP, it will be required after extubation.

*Overview:* it was initially reported by J Bruce Beckwith and Hans-Rudolf Wiedemann. It is one of the syndromes associated with a huge tongue that may

*Genetics:* it is caused by genetic alterations on chromosome 11p15 region.

*Clinical considerations:* Beckwith-Wiedemann syndrome is characterized by a clinical tetrad of macroglossia (Most common), omphalocele, umbilical hernia, and

*Airway and anesthetic implications*: the main anesthetic considerations in BWS are abnormal airway anatomy, hypoglycemia, and cardiac anomalies (cardiomegaly and other cardiac structural defects). A detailed pre-operative assessment of the airway, the cardiac, and the urinary system is mandatory. Visceromegaly may shift the diaphragm upward reducing functional residual capacity. Associated congenital malformations, prematurity, and interventions early in life may complicate anesthetic management. These children frequently require corrective surgical

These children are associated with exomphalos, macroglossia, gigantism, macrosomia, visceromegaly, horizontal earlobe creases, renal medullary dysplasia, cardiac malformations, hypoglycemia, hypothyroidism, hyperlipidemia, polycythemia, hypercalciuria, and embryonal tumors. The presence of three features out of the above will confirm the clinical diagnosis of BWS after ruling out the clinical features of overgrowth syndromes. The risk for embryonal tumor development especially hepatoblastoma, neuroblastoma, rhabdomyosarcoma, gonadoblastoma,

It is important to keep in mind that the option of an emergency surgical airway such as cricothyrotomy/tracheostomy is very limited in patients with KFS due to the extremely short neck, thus the airway approach must be very carefully planned. Extubation must only be attempted when the patient is fully awake and in the presence of protective airway reflexes. An opioid-sparing anesthetic technique is recommended (regional anesthesia whenever possible) as the presence of OSA may increase narcotic-sensitivity. KFS patients should preferably be monitored in the post-operative setting in a high dependency unit or an intensive care unit according

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

**4.7 Beckwith-Wiedemann syndrome**

neonatal hypoglycemia.

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

It is important to keep in mind that the option of an emergency surgical airway such as cricothyrotomy/tracheostomy is very limited in patients with KFS due to the extremely short neck, thus the airway approach must be very carefully planned.

Extubation must only be attempted when the patient is fully awake and in the presence of protective airway reflexes. An opioid-sparing anesthetic technique is recommended (regional anesthesia whenever possible) as the presence of OSA may increase narcotic-sensitivity. KFS patients should preferably be monitored in the post-operative setting in a high dependency unit or an intensive care unit according to the severity of their cardio-pulmonary and airway pathology.

If the patient is on a home BIPAP, it will be required after extubation.

#### **4.7 Beckwith-Wiedemann syndrome**

*Special Considerations in Human Airway Management*

hyperlaxed spines who may require C-spine precautions with in-line-stabilization during intubation. This is performed via a lateral neck X-ray in flexion and exten-

*Child with Klippel-Feil syndrome and anomaly of the Occipito-cervical junction. The images show an elevated left shoulder due to a Sprengel anomaly, a short, webbed neck, and a low hairline (scar on the thorax after* 

A history of previous smooth intubation in KPS patient does not implicate a future favorable outcome due to the progressive nature of the disease. Hence, a careful preoperative assessment and planning is critical for safe airway

Bag-mask ventilation is likely difficult in those patients as well as direct laryn-

Should the older child be cooperative enough to sustain an awake fiberoptic intubation (with light sedation) that would be the approach of choice avoiding both the risks of difficult ventilation and neck injury secondary to airway

Otherwise, a plan to preserve spontaneous ventilation during anesthetic induc-

A trial of manual bag-mask to assess for ventilation is used by some practitioners

tion should be performed with either total intravenous anesthesia (TIVA) or inhaled Sevoflurane (IV Dexmedetomidine and IV Ketamine can be used as well)

before airway manipulation. The various arrays of airway tools including videoscopes (Glidescope/C-MAC etc.) as well as the fiberoptic bronchoscope have been used successfully for intubation in patients with KFS. They are preferred as first line intubation technique due to the lower incidence of cervical spine mobilization

patients with pulmonary hypertension secondary to OSA and scoliosis.

goscopy owing to the fixed neck or the in-line neck stabilization.

avoiding the use of neuro-muscular blockers.

Obstructive sleep apnea (OSA) is not uncommonly encountered in patients with KFS, hence a preoperative sleep study may be indicated along with postoperative apnea monitor. A preoperative echocardiography must be considered especially in

**170**

when used.

sion positions [1, 4–7].

*surgical repair of nonrestrictive atrial septal defect) [37].*

management.

**Figure 6.**

manipulation.

*Overview:* it was initially reported by J Bruce Beckwith and Hans-Rudolf Wiedemann. It is one of the syndromes associated with a huge tongue that may impose challenges to airway management (**Figure 7**).

*Genetics:* it is caused by genetic alterations on chromosome 11p15 region. *Incidence:* 1 per 13,700–15,000 of live births.

*Clinical considerations:* Beckwith-Wiedemann syndrome is characterized by a clinical tetrad of macroglossia (Most common), omphalocele, umbilical hernia, and neonatal hypoglycemia.

These children are associated with exomphalos, macroglossia, gigantism, macrosomia, visceromegaly, horizontal earlobe creases, renal medullary dysplasia, cardiac malformations, hypoglycemia, hypothyroidism, hyperlipidemia, polycythemia, hypercalciuria, and embryonal tumors. The presence of three features out of the above will confirm the clinical diagnosis of BWS after ruling out the clinical features of overgrowth syndromes. The risk for embryonal tumor development especially hepatoblastoma, neuroblastoma, rhabdomyosarcoma, gonadoblastoma, and adrenal carcinoma has been observed [6, 7, 17].

*Airway and anesthetic implications*: the main anesthetic considerations in BWS are abnormal airway anatomy, hypoglycemia, and cardiac anomalies (cardiomegaly and other cardiac structural defects). A detailed pre-operative assessment of the airway, the cardiac, and the urinary system is mandatory. Visceromegaly may shift the diaphragm upward reducing functional residual capacity. Associated congenital malformations, prematurity, and interventions early in life may complicate anesthetic management. These children frequently require corrective surgical

interventions in infancy such as tongue reduction surgeries, hernia repair, exomphalos repair or hepatectomy.

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 must be evaluated in addition to the preoperative glucose homeostasis.

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 and intubation difficult to perform [39, 40].

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 intubation.

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

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 airway must be immediately implemented.

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.

#### **4.8 Mucopolysaccharidosis (MPS)**

*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 the airway operator.

*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 ethnic background may affect the phenotype of MPS.

*Genetic*: inheritance autosomal recessive.

*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 laryngeal/tracheal tissue distortion and narrowing.

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

**173**

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

temporo-mandibular joint may get affected with restricted mobility. Deposition of mucopolysaccharides in the heart and vascular tissues may lead to cardiomyopa-

*Clinical considerations and airway management*: difficult ventilation and intubation are common concerns in patients with mucopolysaccharidoses as the thick and uncompliant infiltrated tissues (skin, tongue, and mucous membranes of the airway, bones, and joints) may render the natural airway narrow and not distensible by the positive pressure ventilation applied by the bag-mask. Laryngoscopy is often challenging due to difficult manipulation of the laryngoscope as a result of the stiff and thick tissues. The large tongue, the hypertrophied tonsils, and the limited mouth opening are caused by the immobile temporo-mandibular joints [40]. This effect is compounded if there is a subluxation of the atlanto-axial joint or cervicalspine affection which mandates an in-line-stabilization during airway management. A narrow/distorted trachea may prevent an appropriately sized endotracheal

Fortunately, the enzymatic replacement therapy may hinder the progression of the disease, hence an adequately treated mucopolysaccharidosis patient may have a

Preoperatively, the MPS patient must be well assessed for the extent of the disease as well as a focused airway assessment including the mouth opening, tongue and tonsillar size, presence of obstructive sleep apnea (which is very common in MPS), and pulmonary hypertension. A preoperative echocardiography is essential to rule out any associated cardiomyopathy. Patients with MPS type 1 (Hurler), 4 (Morquio), and 6 must be checked for any cervical spine or atlanto-

Intravenous cannulation in MPS patients may be challenging due to the thick infiltrated skin, hence a preoperatively inserted intravenous line before anesthetic

Spontaneous ventilation must be maintained during induction of anesthesia due to the high risk of difficult ventilation in case of apnea. This could be achieved with either intravenous or inhalational induction agents and by avoiding neuro-muscular

An LMA must be used as a second plan whenever encountering difficulties in

Video-laryngoscopies are other favorable options for intubation in MPS patients especially those with unstable cervical spine owing to the lesser risk of neck mobilization when used. Establishment of a surgical airway access such as tracheostomy may be difficult due to the thick tissues and the associated tracheal deformities.

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.

*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

ventilation; it can also be used to facilitate fiberoptic intubation.

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

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

thies, arrhythmias, and pulmonary hypertension [5].

tube from passing throughout the vocal cords.

axial instability [42].

induction is preferable.

**5.1 Subglottic hemangiomas**

the glottic/subglottic area.

blockers.

lesser risk of difficult airway than the untreated patient.

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

temporo-mandibular joint may get affected with restricted mobility. Deposition of mucopolysaccharides in the heart and vascular tissues may lead to cardiomyopathies, arrhythmias, and pulmonary hypertension [5].

*Clinical considerations and airway management*: difficult ventilation and intubation are common concerns in patients with mucopolysaccharidoses as the thick and uncompliant infiltrated tissues (skin, tongue, and mucous membranes of the airway, bones, and joints) may render the natural airway narrow and not distensible by the positive pressure ventilation applied by the bag-mask. Laryngoscopy is often challenging due to difficult manipulation of the laryngoscope as a result of the stiff and thick tissues. The large tongue, the hypertrophied tonsils, and the limited mouth opening are caused by the immobile temporo-mandibular joints [40]. This effect is compounded if there is a subluxation of the atlanto-axial joint or cervicalspine affection which mandates an in-line-stabilization during airway management.

A narrow/distorted trachea may prevent an appropriately sized endotracheal tube from passing throughout the vocal cords.

Fortunately, the enzymatic replacement therapy may hinder the progression of the disease, hence an adequately treated mucopolysaccharidosis patient may have a lesser risk of difficult airway than the untreated patient.

Preoperatively, the MPS patient must be well assessed for the extent of the disease as well as a focused airway assessment including the mouth opening, tongue and tonsillar size, presence of obstructive sleep apnea (which is very common in MPS), and pulmonary hypertension. A preoperative echocardiography is essential to rule out any associated cardiomyopathy. Patients with MPS type 1 (Hurler), 4 (Morquio), and 6 must be checked for any cervical spine or atlantoaxial instability [42].

Intravenous cannulation in MPS patients may be challenging due to the thick infiltrated skin, hence a preoperatively inserted intravenous line before anesthetic induction is preferable.

Spontaneous ventilation must be maintained during induction of anesthesia due to the high risk of difficult ventilation in case of apnea. This could be achieved with either intravenous or inhalational induction agents and by avoiding neuro-muscular blockers.

An LMA must be used as a second plan whenever encountering difficulties in ventilation; it can also be used to facilitate fiberoptic intubation.

Video-laryngoscopies are other favorable options for intubation in MPS patients especially those with unstable cervical spine owing to the lesser risk of neck mobilization when used. Establishment of a surgical airway access such as tracheostomy may be difficult due to the thick tissues and the associated tracheal deformities.
