|Year : 2014 | Volume
| Issue : 2 | Page : 73-78
Craniofacial and maxillary anomalies: Anesthetic implications and management
Sukhminder Jit Singh Bajwa1, Ashish Kulshrestha2
1 Department of Anaesthesiology and Intensive Care, Gian Sagar Medical College & Hospital, Banur, Punjab, India
2 Department of Anaesthesiology and Intensive Care, Government Medical College and Hospital, Chandigarh, India
|Date of Web Publication||20-May-2014|
Sukhminder Jit Singh Bajwa
Department of Anaesthesiology and Intensive Care, Gian Sagar Medical College & Hospital, Ram Nagar, Banur, Punjab
Source of Support: None, Conflict of Interest: None
The advancement in the craniofacial surgery has imposed challenges on the attending anesthesiologist for the successful conduct reconstructive surgery. The anesthesiologist remains a key person in the multidisciplinary tasks involved in such surgery. Most of these patients belong to smaller age group and have difficult airway due to various syndromes associated with it. The other major problems faced by the anesthesiologist in such surgery are intra-operative hypothermia due to prolonged surgery and significant blood loss as well as fluid shifts associated with it. A well-equipped intensive care unit is a must for the post-operative care of such patients. Even the adult patients coming for maxillofacial trauma surgery require careful vigilance both intra-operatively as well as post-operatively due to frequent difficult airway associated with it. A careful pre-operative evaluation and discussion with the surgeons, proper planning for airway management, intra-operative care and post-operative intensive care backup is required for the successful outcome in these surgeries. The current review is an attempt to describe in short the important anesthesia aspects and challenges related to various cranio-maxillary lesions.
Keywords: Airway management, anesthesia, craniofacial surgery, maxillofacial trauma
|How to cite this article:|
Bajwa SS, Kulshrestha A. Craniofacial and maxillary anomalies: Anesthetic implications and management. J Sci Soc 2014;41:73-8
| Introduction|| |
Cranio-maxillary lesions have always been a matter for social, behavioral, psychological and clinical concern. In the past, the presence of defects of face leading to appearance of a "monster" led to the social boycott of the person. These challenges get accentuated when such patients present for surgical intervention. ,,,,, With the advent of modern craniofacial surgical interventions, these facial defects can be corrected to a large extent and can help the person to lead a normal life. Paul Tessier, the father of craniofacial surgery, has suggested various surgical techniques for repair of these defects involving a multidisciplinary approach involving plastic surgeon, neurosurgeon, maxillofacial surgeon, an ophthalmologist, an otolaryngologist, orthodontist, pedodontist, a pediatrician, speech specialist and nursing specialist.  The role of an anesthesiologist is central and is mandatory for proper coordination among these specialists. The anesthesiologist's role starts from the pre-operative period and extend majority of times into the post-operative care in intensive care unit where frequently these patients are admitted post-operatively. ,,
| Literature Search Strategies|| |
Articles in various international and national bibliographic indices were extensively searched with emphasis on cranial and maxillary anomalies, cranio-maxillary trauma, airway management in facial trauma, congenital cranio-facial lesions, maxillary and mandibular malformations. The various search sites included Entrez (including PubMed), NIH.gov, Medscape.com, Scopus, Science Direct, WebMD.com, MedHelp.org, Searchmedica, yahoo.com and google.com. Manual search was also carried out and various articles in journals and textbooks related to cranio-maxillary diseases, plastic surgery, airway management in facial lesions critical care, cranio-facial trauma were also searched.
| Congenital Malformations|| |
The craniofacial area consists of skull, orbits, forehead, nose, maxilla, mandible, ears, tongue and lips. This area may be involved in congenital malformations, inflammation, neoplasms and trauma. ,,,,,,
Numerous congenital and traumatic cranio-maxillary malformations can be encountered in clinical practice, but we are limiting our discussion to the commonly faced anomalies.
These clefts are mainly due to the mal developments at an embryonic stage. These clefts can be further divided into predominantly cranial, predominantly facial and a combination, i.e., craniofacial with the palpebral tissue as the reference line. The common types of facial clefts encountered in clinical anesthesia practice are described in [Table 1]. ,,
The common types of craniofacial clefts encountered are described in [Table 2].
It results from the premature fusion of cranial sutures leading to abnormal shape of the skull which depends upon the site and number of sutures involved. ,,, It may be classified into various types, e.g., brachycephaly (short skull) seen in bilateral coronal synostosis, scaphocephaly (boat shaped skull) seen in sagittal synostosis, trigonocephaly seen in metopic synostosis, plagiocephaly seen in unilateral coronal synostosis etc. It results in mal development of the vault and base of the skull with a raised intracranial tension (ICT). The associated anomalies may be hypoplasia of maxilla, hypertelorism, rhinomegaly, prognathia and ptosis. These anatomic alterations lead to impaired breathing, difficulty in swallowing and keratitis. The various syndromes associated with faciocraniosynostosis are described in [Table 3].
|Table 3: Association of various syndromes associated with faciocraniosynostosis|
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The main aim of treatment in faciocraniosynostosis is surgical correction of backwardly placed forehead and mid face in forward direction. This can be accomplished by frontal advancement where the forehead is advanced and fixed to the nasal and lateral orbital bones or by facial advancement, which involves advancement of lower third of orbit, nasal bone, malar bones and the upper maxillae by performing Le Fort III facial osteotomies. A combined approach may be used in severe abnormalities but carries a high risk of post-operative complications such as osteitis and meningitis. These surgeries are usually performed at an early age of 5 months to 5 years. The other types of procedures involved are multiple craniotomies, release of cranial base and sutures, bony elongation and phased distraction using special devices with the bony sections being made endoscopically thereby reducing the need for surgery. 
| Mandibular Micrognathia|| |
This condition leads to receding mandible leading to breathing and feeding problems. It is corrected by elongation osteotomy in the body of mandible followed by bone grafting. It is performed at 6-9 years of age to prevent any dental malocclusion. 
Other facial lesions requiring surgical correction are microstomia, microchelia, lymphangioma, neurofibroma involving the lips, ankyloglossia, macroglossia or macroglossia involving the tongue and ranula, Ludwig's angina, retropharyngeal abscess involving the floor of the mouth.
| Maxillofacial Trauma|| |
With the ever increasing vehicular population, the incidence of serious maxillofacial trauma has risen tremendously. , The maxillofacial trauma is considered more dangerous due to close proximity of the cranium and involvement of airway leading to breathing abnormalities. , These maxillofacial fractures can be classified according to their anatomical position and displacement as:
The various parts of the mandible fractured with incidences are condylar neck (35%), angle (20%), parasymphysis (13%), symphysis (11%) and coronoid (1%). 
Mid face fractures
The bones involved in trauma to mid face are maxilla, zygoma, palatine, nasal, vomer, ethmoid, lacrimal and pterygoid plate of sphenoid. Rene Le Fort has described these fractures depending on the anatomic location as:
- Le Fort I : Horizontal fracture separating the palate and upper alveolar ridge from the rest of the face.
- Le Fort II : Fracture line separates the maxilla and medial orbit from the zygomatic arch and skull.
- Le Fort III : Fracture line extends from lateral orbit to the zygomatic arch and to the pterygoid plate separating the mid face from the cranium.
The Le Fort fractures type II and III are often associated with basilar skull fractures with damage to the cribriform plate.
These patients pose great challenges to the attending anesthesiologist due to limitation of mouth opening due to pain or trismus and development of upper airway edema due to injury leading to breathing difficulties.
| Pre-Operative Assessment|| |
This is an extremely important part of anesthetic management that helps the anesthesiologist to familiarize with the anatomic alterations in the patient and it also helps to allay anxiety in adult patients regarding the surgical procedure. A detailed history from parents or the closest relatives is mandatory with special considerations to the recent upper respiratory tract infection, previous anesthetic exposure, features of raised ICT, history of seizures and their control by anticonvulsant medication and in case of maxillofacial trauma, the history of loss of consciousness with history of any breathing problem.
The general physical examinations should include the assessment of mental status and any neurological deficits should be documented. The airway examination is the key in these patients and should include mouth opening, thyromental distance and adequacy of neck movements and visualization of pharyngeal structures. The diagnosis of structural airway difficulties and management of difficult airway can be further helped by suitable radiologic investigations. These patients often have limited mouth opening with limited visualization of pharyngeal structures due to abnormalities of tongue and mandible. Patients may have long standing airway obstruction with chronic hypoxia leading to changes in heart, i.e., pulmonary hypertension and corpulmonale. The patients with maxillofacial trauma can have associated cervical spine injury which should be ruled out pre-operatively as it can cause dislocation of vertebra or injury to the spinal cord during intubation.
The pre-operative investigations should include a complete blood count and hemoglobin estimation to know the general status of the patient. The estimation of renal functions and serum electrolytes is beneficial if pre-operative therapy for raised ICT is started. As these patients often have associated cardiac anomalies, so a chest radiograph and an echocardiogram may be beneficial. The patients with maxillofacial trauma may also require a computed tomogram of the brain and neck to rule out intracranial or cervical spine injuries.
Adequate amount of cross matched blood and blood products should be arranged pre-operatively as most of the craniofacial reconstructive procedures involve massive blood loss.
| Premedication|| |
All the medications can be continued in these patients up until the morning of the day of surgery including the anticonvulsant medications and medications for raised ICT. The anesthesiologist should discuss the surgical procedure with surgeon pre-operatively and should enquire about the intra-operative positioning of the patient. The pre-operative sedative medications can be given to patients with no history of raised ICT or significant airway obstruction in the form of oral midazolam for children in a dose of 0.5-0.7 mg/kg while antianxiety premedication can be given to adults.
| Monitoring|| |
The monitoring should include all the standard monitors including five lead electrocardiogram, non-invasive blood pressure, pulse oximetry, end tidal carbon dioxide monitor, temperature and urine output. Invasive arterial blood pressure and central venous pressures are usually indicated in lengthy reconstructive procedures and in patients with significant comorbid conditions. Depending upon the availability, bispectral index monitoring system and electroencephalogram can be highly useful during peri-operative monitoring in this surgical population.
| Intra-Operative Concerns|| |
Intravenous induction is usually preferred in these patients if intravenous line is in situ which is also preferable in patients with raised ICT.  However, inhalational induction with short acting agents like sevoflurane is preferred in small children before securing an intravenous line. The anesthetic technique, however, has to be modified if a difficult airway is anticipated.
Difficult airway is commonly encountered in these children especially in craniofacial syndromes and a definite plan to secure airway should be made. All the airway adjuncts such as different sizes of endotracheal tubes, oropharyngeal airways, nasopharyngeal airway, different supraglottic airway devices and special laryngoscopes should be available and checked pre-operatively.  A good intravenous access with a wide bored cannula should be secured before induction in all the anticipated difficult airway situations. One option is careful inhalational induction with short acting inhalational agents like sevoflurane with direct laryngoscopy under deep level of anesthesia to secure the airway with spontaneous respiration. With ever increasing use of fiberoptic bronchoscope assisted awake intubation; its use is now advocated in all the anticipated difficult intubations.
Airway management in maxillofacial injuries pose significant challenges to the anesthesiologist as the intubation is usually rendered difficult due to disruption of facial structures and residual swelling, sharing of the airway and interference by the endotracheal tube in the surgical procedure.  The principles of airway management remain same as with the other craniofacial reconstructive procedures. An important concern is use of nasotracheal intubation desired by the operating surgeon to avoid interference in the surgical field. However, this route is best avoided in Le Fort III type of facial fractures as well as fractures of naso-ethmoidal complex due to frequent association of basilar skull fracture with them. Another important intra-operative concern is the use of intermaxillary fixation (IMF) by the surgeons to produce a template for fracture reduction which is also kept post-operatively and can cause airway obstruction and should be able to release in such a situation. Use of submental intubation is sometimes desired by the surgeons in patients with fracture of skull base in whom nasotracheal intubation is contraindicated. Elective tracheostomy can be performed in such patients depending upon the duration of hospital stay and severity of injury which may mandates prolonged mechanical ventilation. A careful intra-operative vigilance for kinking and accidental extubation of the endotracheal tube is required in these cases.
As most of these reconstructive procedures are time consuming, a special care should be undertaken for proper positioning these patients with careful padding of pressure points to avoid ischemic injury. Proper application of soft bolsters is required in prone or lateral position to evenly distribute the weight avoiding undue pressure on the abdomen or neck. A proper access to the intravenous lines under the drapes should be ensured before the surgery commences. Sometimes a tarsorrhaphy may be required in severely proptotic eyes to prevent exposure keratitis.
As these surgeries are time consuming and involve a large amount of blood loss, a careful attention should be provided toward fluid homeostasis. Most of these patients are children who have a larger surface area to volume ratio resulting in proportionately larger fluid and blood loss. The main indicators of intra-operative fluid status are urine output and central venous pressure but the urine output can be confounded by use of therapy for raised ICT like mannitol etc. The third space losses should be replaced by isotonic crystalloid solutions and the blood loss should be replaced by packed cells. The estimation of blood loss is often difficult due to loss under the drapes and in the irrigation solution so the blood transfusion should be guided by serial intra-operative hematocrit values and by the fluid status of the patient.
Coagulation abnormalities may develop due to massive blood transfusion for which fresh frozen plasma is required and platelet transfusion may be required in cases of dilutional thrombocytopenia. The blood loss can be reduced by various techniques such as positioning of head at 30 degree angle above the heart, use of vasopressors and use of controlled hypotension by various new drugs like dexmedetomidine etc. Dexmedetomidine not only reduces blood loss but also decreases the peri-operative anesthetic requirement besides attenuating the stress response. ,
Due to prolonged duration of these surgeries and a larger surface area of the children, there is considerable loss of heat leading to hypothermia. The maintenance of normothermia is important as hypothermia may have deleterious effects on coagulation. The heat loss can be prevented by use of fluid warmers, placing the child on heating mattresses, covering the child with heated blankets and wrapping the exposed body parts in cotton or plastic sheets. The use of heated humidifiers is desirable in the breathing circuits to prevent heat loss from the respiratory tract.
Venous air embolism (VAE)
VAE can occur in craniofacial reconstruction procedures due to opening of various non-collapsible venous channels in the skull during craniotomy in a head up position. It results in hemodynamic instability, hypoxia and in smaller children paradoxical embolism can occur through a patent foramen ovale.  A high degree of suspicion together with monitoring of end tidal carbon dioxide and precordial Doppler can help in detecting air embolism. In the event of VAE, immediate information of surgeon, flooding the operative field with saline, lowering the head, compressing the jugular veins, discontinuing nitrous oxide and supporting the hemodynamic parameters is required. A central venous catheter with the tip placed at the junction of the right atrium with the superior vena cava is helpful in aspirating the embolized air.
| Post-Operative Care|| |
Due to the prolonged nature of these reconstructive procedures with high incidence of hypothermia, it is desirable to continue mechanical ventilation in these patients for 24-48 h in an intensive care unit. , These patients are slowly weaned and the extubation is performed after assessment of level of consciousness, airway edema, recovery of protective airway reflexes, normothermia, hemodynamic stability and adequacy of blood loss replacement and circulatory volume. The post-operative complications commonly seen are infection, meningitis and loss of bone segments.  The most common causes of deterioration in these patients post-operatively are hypoxia, hypercapnia, cerebral edema, intracranial bleeding, electrolyte imbalance and hypoglycemia. ,
The extubation of patients of maxillofacial trauma should be done keeping in mind all the general principles of extubation as described above. In addition, use of IMF may cause airway obstruction so tools to release IMF should be readily available at the time of extubation and nasopharyngeal airway should be used to maintain airway patency in post-extubation period.
Post-operative analgesia plays an important role in success of these extensive reconstructive procedures as these are often associated with severe pain due to extensive tissue and bony dissection. A titrating dose of opioids is usually used to avoid respiratory depression in these patients and use of multi-modal analgesia with opioids along with non-steroidal anti-inflammatory drugs is preferred. A patient-controlled analgesia using intravenous morphine can be effectively used post-operatively for control of pain. Post-operative nausea and vomiting can be successfully treated with peri-operative administration of palonosetron. 
| Conclusion|| |
The craniofacial surgery is a separate discipline of surgery which requires a multidisciplinary team with a dedicated anesthesiologist for successful outcome. Most of these patients are of smaller age group who present with various syndromes associated with craniofacial malformations. The anesthesiologist is faced with many challenges in anesthetizing these children in the form of difficult airway, raised ICT, significant blood loss and fluid shifts and prolonged duration of surgery. Thus a multidisciplinary approach is required for these surgeries for craniofacial lesions.
| References|| |
|1.||Bertossi D, Malchiodi L, Shideh E, Albanese M, Portelli M, Lucchese A, et al. Delayed progressive haematoma after Le Fort I osteotomy: A possible severe complication in orthognatic surgery. Dent Res J (Isfahan) 2012;9:S246-50. |
|2.||Bajwa SS, Panda A, Bajwa SK, Singh A, Parmar SS, Singh K. Anesthetic and airway management of a child with a large upper-lip hemangioma. Saudi J Anaesth 2011;5:82-4. |
|3.||Coupe MH, Johnson D, Seigne P, Hamlin B. Special article: Airway management in reconstructive surgery for noma (cancrum oris). Anesth Analg 2013;117:211-8. |
|4.||Bajwa SS, Kaur J, Singh A, Singh G. Post-burn facial contractures in pediatric patients: Challenging aspects of difficult airway management. Int J Health Allied Sci 2012;1:186-9. |
|5.||Park CD, Lee HK, Yim JY, Kang IH. Anesthetic management for a patient with severe mento-sternal contracture: Difficult airway and scarce venous access - A case report-. Korean J Anesthesiol 2013;64:61-4. |
|6.||Bajwa SJ, Gupta S, Kaur J, Panda A, Bajwa SK, Singh A, et al. Anesthetic considerations and difficult airway management in a case of Noonan syndrome. Saudi J Anaesth 2011;5:345-7. |
|7.||Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976;4:69-92. |
|8.||Bajwa SJ, Kaur J, Singh A, Kapoor V, Bindra GS, Ghai GS. Clinical and critical care concerns of cranio-facial trauma: A retrospective study in a tertiary care institute. Natl J Maxillofac Surg 2012;3:133-8. |
|9.||Sinkueakunkit A, Chowchuen B, Kantanabat C, Sriraj W, Wongswadiwat M, Bunsangjaroen P, et al. Outcome of anesthetic management for children with craniofacial deformities. Pediatr Int 2013;55:360-5. |
|10.||Bajwa SJ, Bajwa SK, Singh A, Khan B, Parmar SS, Singh G, et al. Anesthetic challenges and difficulties in the management of Treacher Collins syndrome. Anesth Essays Res 2011;5:111-3. |
|11.||Marston AP, Lander TA, Tibesar RJ, Sidman JD. Airway management for intubation in newborns with Pierre Robin sequence. Laryngoscope 2012;122:1401-4. |
|12.||Barnett S, Moloney C, Bingham R. Peri-operative complications in children with Apert syndrome: A review of 509 anesthetics. Paediatr Anaesth 2011;21:72-7. |
|13.||Forbes BJ. Congenital craniofacial anomalies. Curr Opin Ophthalmol. 2010;21(5):367-74. |
|14.||Bajwa SJ, Gupta SK, Kaur J, Singh A, Parmar SS. Anaesthetic management of a patient with Crouzon syndrome: Case study. South Afr J Anaesth Analg 2012;18:270-2. |
|15.||Caruselli M, Giretti R, Pallotto R, Rocchi G, Carboni L. Intubation using a "bonfils fiberscope" in a patient with Pfeiffer syndrome. J Bronchology Interv Pulmonol 2011;18:374-5. |
|16.||Jarrahy R, Cha ST, Berci G, Shahinian HK. Endoscopic transglabellar approach to the anterior fossa and paranasal sinuses. J Craniofac Surg 2000;11:412-7. |
|17.||Brooker GE, Cooper MG. Airway management for infants with severe micrognathia having mandibular distraction osteogenesis. Anaesth Intensive Care 2010;38:43-9. |
|18.||Bajwa SS, Kaur J, Bajwa SK, Kaur G, Singh A, Parmar SS, et al. Designing, managing and improving the operative and intensive care in polytrauma. J Emerg Trauma Shock 2011;4:494-500. |
|19.||Eskitaþcýoðlu T, Ozyazgan I, Coruh A, Günay GK, Yontar Y, Altýparmak M. Fractures of the mandible: A 20-year retrospective analysis of 753 patients. Ulus Travma Acil Cerrahi Derg 2013;19:348-56. |
|20.||Singh Bajwa SJ, Bajwa SK, Kaur J. Comparison of two drug combinations in total intravenous anesthesia: Propofol-ketamine and propofol-fentanyl. Saudi J Anaesth 2010;4:72-9. |
|21.||Osses H, Poblete M, Asenjo F. Laryngeal mask for difficult intubation in children. Paediatr Anaesth 2000;10:452. |
|22.||Bajwa SJ, Kaur J, Singh A, Parmar S, Singh G, Kulshrestha A, et al. Attenuation of pressor response and dose sparing of opioids and anaesthetics with pre-operative dexmedetomidine. Indian J Anaesth 2012;56:123-8. |
|23.||Shams T, El Bahnasawe NS, Abu-Samra M, El-Masry R. Induced hypotension for functional endoscopic sinus surgery: A comparative study of dexmedetomidine versus esmolol. Saudi J Anaesth 2013;7:175-80. |
|24.||Kececi Y, Ozek C, Gurler T, Guner U, Celik N, Mutluer S, et al. Complications in craniofacial surgery: The Turkish experience. J Craniofac Surg 2000;11:168-71. |
|25.||Bajwa SS, Bajwa SK, Kaur J, Sharma V, Singh A, Singh A, et al. Palonosetron: A novel approach to control post-operative nausea and vomiting in day care surgery. Saudi J Anaesth 2011;5:19-24. |
[Table 1], [Table 2], [Table 3]