**4. Complications**

Craniofacial surgery is one of the established multidisciplinary specialty to produce safe surgery and good surgical outcome whilst minimizing the complications following surgical intervention for syndromic craniofacial patients. Although the results can be satisfying and there is general agreement on surgical indications, the potential remains for unwanted complications. The craniofacial surgery is unique because it involves exploration of the areas that allow very little margin of error. An inadequate knowledge of the anatomy, lack of training and surgical expertise can lead to not only disastrous results but even to the death of the patient. Any team that cares for craniofacial patients must take steps to avoid potential complications and be ready to deal with postoperative complications. Development of craniofacial surgery pioneered by Paul Tessier was a crucial step towards the paradigm shift in treating major craniofacial syndromic deformities [35].

#### **4.1 Mortality and morbidity rate following craniofacial surgical intervention**

The complications are the events that occurs during the management of craniofacial syndromes patients and may associated with any permanent deleterious effect on the patient. However, unfavourable outcomes generally are unexpected by the patients or surgeons [36].

The platinum rule to avoid any unwanted complications in any performed surgical procedures is to follow the dictum "Primum Non Nocere".

Many authors attempted to classify the complications of craniofacial surgery that arise during intraoperative and postoperative, but these complications might differ with different craniofacial syndromes. Intraoperative and post-operative complications pertaining to cranial vault surgery was described and classified into early, immediate post-operative and late postoperative complications [37–42]. Given the uniqueness presentation of patients with craniofacial syndromes, each patient presented with their

**137**

*Craniofacial Corrective Surgery in Syndromic Craniosynostosis*

own problems and complications following surgical intervention might be different

One of the easiest way to classify the complications following surgical interven-

*Type 1*: Minor events without any damaging effects on the patient. They include minor wound infections, poorly placed scars, minor cerebrospinal fluid leaks, and seromas/hematomas. Most of the time, this classification refers to less serious and

This is most of the time seen after hypertelorism correction but the reported incidence is quite low which is about 0.6% [42]. This is due to any procedure that involves dissection around the medial and inferomedial orbital floor may poten-

Extensive stripping of periorbita may lead to reattachment of periorbita at low level eventually resulting in enhanced antimongoloid slant. This is most frequently

seen on syndromic rather than nonsyndromic craniofacial syndrome [22].

Hardware can be considered as a foreign body thus occasionally can be infected, exposed or even palpable postoperatively. Infection and exposure of fixation material are rare in paediatric cranial vault surgery [42]. Metal fixation carry a possibility of intracranial fixation due to appositional cranial growth. Some reported translocation of hardware into calvarial bone in 14% and 6.6% with intracranial translocation and commonly occur in younger and syndromic patients [28]. Therefore, usage of resorbable hardware has now become more popular due to concerns about constriction of growth by metal fixation and the possibility of

Pneumothorax incidence of 3% after rib harvesting in their experience but

*Type 2*: Moderate-to-severe events that compromise the results and might need another surgical intervention for a successful outcome. They include exposure keratitis, diplopia, contour deformities, warping, non/malunion, and exposed hardware. Strabismus and temporary ptosis are frequently seen after cranial vault procedures that involve periorbita stripping [37]. McCarthy et al. noted preoperative strabismus in hypertelorism cases, which often worsened after surgery then stabi-

Cerebrospinal fluid (CSF) leak following craniofacial surgery and sequalae of neurosurgical infection is common. Obvious risk for infection with CSF was noted but also associated with impairment of wound healing. Predisposing factor that might lead to easy tear of dura are due to scar from previous surgery or abnormal bony contour with dural adhesion [36–39]. Some tears may go unnoticed, and CSF may manifest postoperatively either as rhinorrhoea or leakage through the scalp

Transient hyponatremia is one of the reported complications caused by secretion of inappropriate antidiuretic hormone (diabetes insipidus) has been reported [27, 40–44]. Researchers believe that it results from traction on the frontal lobes. Unexpected airway issues are other complications following craniofacial surgery, in which emergency reintubation or even prolonged ventilation is needed due to severe upper airway oedema. Decision for steroid covers for upper airway oedema is most of the time anecdotal but some reported beneficial in preventing

emphasized that the rates vary from 5–30% in other series [33].

lized approximately 6 months after surgery [38].

from each other thus the need for comprehensive classification systems.

tion are by Sharma et al. 2013 [41] which was divided into four types:

minor complications following craniofacial surgery that consists of:

*DOI: http://dx.doi.org/10.5772/intechopen.94584*

tially damage the lacrimal drainage system. Lateral canthal ligament dystopia

Epiphora

Hardware issues

implant translocation [29–32]. Bone graft donor morbidity

wounds or within the drain itself.

facial oedema postoperatively [45–50].

#### *Craniofacial Corrective Surgery in Syndromic Craniosynostosis DOI: http://dx.doi.org/10.5772/intechopen.94584*

own problems and complications following surgical intervention might be different from each other thus the need for comprehensive classification systems.

One of the easiest way to classify the complications following surgical intervention are by Sharma et al. 2013 [41] which was divided into four types:

*Type 1*: Minor events without any damaging effects on the patient. They include minor wound infections, poorly placed scars, minor cerebrospinal fluid leaks, and seromas/hematomas. Most of the time, this classification refers to less serious and minor complications following craniofacial surgery that consists of:

Epiphora

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

preventing the increase of ICP, and treating airway deficiency [30–34].

*3.2.3 Le Fort III osteotomy*

*3.2.4 Le Fort I osteotomy*

**4. Complications**

syndromic deformities [35].

patients or surgeons [36].

advancement and the experience of the team.

airway such as tonsillectomy and adenoidectomy.

include marked improvements in functional parameters involving eye protection,

Le Fort III advancement is aimed on improving the proptotic condition as well as opening the space for the upper airway. Syndromic craniosynostosis patients may presented with restricted upper airway thus causing obstructive sleep apnoea and shallow orbital floor. This allows the floor of the orbit to be advanced while opening the upper airway region on the nasal and maxillary region. This technique can be performed via conventional advancement or via DO depending on the amount of

This is usually indicated in a later stage when the patient is more stable in growth to correct skeletal discrepancies such as retruded maxilla thus causing OSA. Therefore, the maxilla is advanced to gain space for airway. This procedure may be combined with other soft tissues surgery to gain optimum results in opening the

Craniofacial surgery is one of the established multidisciplinary specialty to produce safe surgery and good surgical outcome whilst minimizing the complications following surgical intervention for syndromic craniofacial patients. Although the results can be satisfying and there is general agreement on surgical indications, the potential remains for unwanted complications. The craniofacial surgery is unique because it involves exploration of the areas that allow very little margin of error. An inadequate knowledge of the anatomy, lack of training and surgical expertise can lead to not only disastrous results but even to the death of the patient. Any team that cares for craniofacial patients must take steps to avoid potential complications and be ready to deal with postoperative complications. Development of craniofacial surgery pioneered by Paul Tessier was a crucial step towards the paradigm shift in treating major craniofacial

**4.1 Mortality and morbidity rate following craniofacial surgical intervention**

The platinum rule to avoid any unwanted complications in any performed

Many authors attempted to classify the complications of craniofacial surgery that arise during intraoperative and postoperative, but these complications might differ with different craniofacial syndromes. Intraoperative and post-operative complications pertaining to cranial vault surgery was described and classified into early, immediate post-operative and late postoperative complications [37–42]. Given the uniqueness presentation of patients with craniofacial syndromes, each patient presented with their

surgical procedures is to follow the dictum "Primum Non Nocere".

The complications are the events that occurs during the management of craniofacial syndromes patients and may associated with any permanent deleterious effect on the patient. However, unfavourable outcomes generally are unexpected by the

**136**

This is most of the time seen after hypertelorism correction but the reported incidence is quite low which is about 0.6% [42]. This is due to any procedure that involves dissection around the medial and inferomedial orbital floor may potentially damage the lacrimal drainage system.

Lateral canthal ligament dystopia

Extensive stripping of periorbita may lead to reattachment of periorbita at low level eventually resulting in enhanced antimongoloid slant. This is most frequently seen on syndromic rather than nonsyndromic craniofacial syndrome [22].

Hardware issues

Hardware can be considered as a foreign body thus occasionally can be infected, exposed or even palpable postoperatively. Infection and exposure of fixation material are rare in paediatric cranial vault surgery [42]. Metal fixation carry a possibility of intracranial fixation due to appositional cranial growth. Some reported translocation of hardware into calvarial bone in 14% and 6.6% with intracranial translocation and commonly occur in younger and syndromic patients [28]. Therefore, usage of resorbable hardware has now become more popular due to concerns about constriction of growth by metal fixation and the possibility of implant translocation [29–32].

Bone graft donor morbidity

Pneumothorax incidence of 3% after rib harvesting in their experience but emphasized that the rates vary from 5–30% in other series [33].

*Type 2*: Moderate-to-severe events that compromise the results and might need another surgical intervention for a successful outcome. They include exposure keratitis, diplopia, contour deformities, warping, non/malunion, and exposed hardware.

Strabismus and temporary ptosis are frequently seen after cranial vault procedures that involve periorbita stripping [37]. McCarthy et al. noted preoperative strabismus in hypertelorism cases, which often worsened after surgery then stabilized approximately 6 months after surgery [38].

Cerebrospinal fluid (CSF) leak following craniofacial surgery and sequalae of neurosurgical infection is common. Obvious risk for infection with CSF was noted but also associated with impairment of wound healing. Predisposing factor that might lead to easy tear of dura are due to scar from previous surgery or abnormal bony contour with dural adhesion [36–39]. Some tears may go unnoticed, and CSF may manifest postoperatively either as rhinorrhoea or leakage through the scalp wounds or within the drain itself.

Transient hyponatremia is one of the reported complications caused by secretion of inappropriate antidiuretic hormone (diabetes insipidus) has been reported [27, 40–44]. Researchers believe that it results from traction on the frontal lobes.

Unexpected airway issues are other complications following craniofacial surgery, in which emergency reintubation or even prolonged ventilation is needed due to severe upper airway oedema. Decision for steroid covers for upper airway oedema is most of the time anecdotal but some reported beneficial in preventing facial oedema postoperatively [45–50].

*Type 3*: Serious events with unfavourable result which can or cannot be successfully managed. They include nerve palsies and infection leading to bone loss and partial loss of vision.

Infection is the most common complication in the form of osteitis/osteomyelitis, meningitis, or an intracranial abscess, occurring in 6.2% of transcranial cases [25]. Overall reported infection rates ranging from 1–14% in large centre series [27, 32, 39–42, 47–51].

Permanent neurological deficit is another complication in craniofacial surgery that fall into type III classification. Majority of craniofacial surgery confined to extradural showed lower incidence of neurologic impairment. Several reports from major craniofacial centers has shown very low or no permanent neurologic deficits directly attributable to surgery [27, 30, 37, 50–52]. Blindness for example, is an unwanted complication in craniofacial surgery that post a real risk of blindness following surgery. Munro and Sabatier noted four cases of permanent blindness in 1092 procedures [54].

*Type 4*: Serious events that may even lead to death. They include postoperative infection, perioperative bleeding, respiratory compromise, or other serious anesthesia-related events.

Since 1970's to 1988, rate of mortality in craniofacial surgery were reported ranging from 1 to 2% all over the world [47–51]. However, with development of the technology, deeper understanding of the craniofacial surgery and its risks with emphasis on multidisciplinary approach, the numbers of mortality have dropped to 0.1–0.8% in some centers [24–29].

### **5. Research and development in corrective surgery**

With the recent medical and technology advancement, patient management was more promising and in a well-controlled manner. The surgical management of craniosynostosis patient resurfaces again in the mid-20th century as a completely stand-alone surgical specialty. A more well defined surgical procedure, better anaesthetic protocols, together with the help of advanced technology, this group of patients now enjoy a safer surgical outcome [52–57].

#### **5.1 Optimal age of surgery**

Surgical procedures advocated in this group of patients are aim to make sure normal growth of the brain and skull, and near to normal development to their adulthood. Controversy still on-going with the best timing of surgical intervention in this group of patients between early versus late surgical intervention. Early surgical intervention is always aiming for better corneal protection and to create spaces for constricted brain. Late surgical intervention conversely aiming for more stable bony correction and less likely for subsequent surgical intervention [50]. However, our center practice on more indication and need-based approach, surgical intervention at the best possible timing for patients in term of growing stage, the indication need for the surgery and patient's general health condition [4].

#### **5.2 Choices of osteotomy**

Surgical procedures evolved from strip craniectomy, monobloc osteotomy, fronto orbital advancement and recently, posterior vault expansion. All these procedures are indicated to release the fused cranial suture, re-create

**139**

**Table 1.**

**Figure 6.**

*Craniofacial Corrective Surgery in Syndromic Craniosynostosis*

more spaces for brain development and to make sure the bilateral eye globe is well protected as shown in **Figures 5** and **6**. The current treatment can be

*Posterior vault distraction distraction osteogenesis indicated for only increased ICP with no other symptoms.*

*Le Fort III advancement indicated for management of ICP, airway and orbital globe.*

*Treatment proposed for management of syndromic craniosynostosis issues.*

**Issues to manage Treatment proposed**

Only increase in ICP Posterior cranial vault distraction Increase in ICP and shallow orbit Fronto orbital advancement Reduced airway and shallow orbit Le Fort III advancement Reduced airway, shallow orbit and increased ICP Monobloc advancement

*DOI: http://dx.doi.org/10.5772/intechopen.94584*

summarized in **Table 1**.

**Figure 5.**

more spaces for brain development and to make sure the bilateral eye globe is well protected as shown in **Figures 5** and **6**. The current treatment can be summarized in **Table 1**.

#### **Figure 5.**

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

partial loss of vision.

1092 procedures [54].

anesthesia-related events.

0.1–0.8% in some centers [24–29].

**5.1 Optimal age of surgery**

**5.2 Choices of osteotomy**

**5. Research and development in corrective surgery**

patients now enjoy a safer surgical outcome [52–57].

need for the surgery and patient's general health condition [4].

39–42, 47–51].

*Type 3*: Serious events with unfavourable result which can or cannot be successfully managed. They include nerve palsies and infection leading to bone loss and

Infection is the most common complication in the form of osteitis/osteomyelitis, meningitis, or an intracranial abscess, occurring in 6.2% of transcranial cases [25]. Overall reported infection rates ranging from 1–14% in large centre series [27, 32,

Permanent neurological deficit is another complication in craniofacial surgery that fall into type III classification. Majority of craniofacial surgery confined to extradural showed lower incidence of neurologic impairment. Several reports from major craniofacial centers has shown very low or no permanent neurologic deficits directly attributable to surgery [27, 30, 37, 50–52]. Blindness for example, is an unwanted complication in craniofacial surgery that post a real risk of blindness following surgery. Munro and Sabatier noted four cases of permanent blindness in

*Type 4*: Serious events that may even lead to death. They include postoperative infection, perioperative bleeding, respiratory compromise, or other serious

Since 1970's to 1988, rate of mortality in craniofacial surgery were reported ranging from 1 to 2% all over the world [47–51]. However, with development of the technology, deeper understanding of the craniofacial surgery and its risks with emphasis on multidisciplinary approach, the numbers of mortality have dropped to

With the recent medical and technology advancement, patient management was more promising and in a well-controlled manner. The surgical management of craniosynostosis patient resurfaces again in the mid-20th century as a completely stand-alone surgical specialty. A more well defined surgical procedure, better anaesthetic protocols, together with the help of advanced technology, this group of

Surgical procedures advocated in this group of patients are aim to make sure normal growth of the brain and skull, and near to normal development to their adulthood. Controversy still on-going with the best timing of surgical intervention in this group of patients between early versus late surgical intervention. Early surgical intervention is always aiming for better corneal protection and to create spaces for constricted brain. Late surgical intervention conversely aiming for more stable bony correction and less likely for subsequent surgical intervention [50]. However, our center practice on more indication and need-based approach, surgical intervention at the best possible timing for patients in term of growing stage, the indication

Surgical procedures evolved from strip craniectomy, monobloc osteotomy,

fronto orbital advancement and recently, posterior vault expansion. All these procedures are indicated to release the fused cranial suture, re-create

**138**

*Posterior vault distraction distraction osteogenesis indicated for only increased ICP with no other symptoms.*

#### **Figure 6.**

*Le Fort III advancement indicated for management of ICP, airway and orbital globe.*


#### **Table 1.**

*Treatment proposed for management of syndromic craniosynostosis issues.*

Different surgeon will advocate different surgical technique at different timing, either with direct osteotomy and surgical plating or through distraction osteogenesis procedure [2, 4, 9, 49, 50, 58, 59]. There is no uniform surgical algorithm internationally, but more on surgeon or surgical center preferences. Obviously, these surgeries need collaboration of neurosurgeon, oral maxillofacial surgeon and otorhinolaryngologists.

#### **5.3 Research**

The ultimate goals for craniosynostosis treatment is mainly to restore function, improve facial aesthetic and ensure a healthy psychological development. More research is needed in this syndromic craniosynostosis in term of treatment algorithm, utilisation of latest and advancement of computer planning, computer navigation, 3-dimensional printing and usage of the cheaper, user friendly and effective surgical device in making the surgery more safer and more predictable outcome. The treatment focus not only on the fused suture of the skull, but also make an effort to address issue like intracranial pressure, strabismus, abnormally positioned orbit and dentofacial deformities [60–62]. Another area of future development will be in the molecular genetic testing in the field of genetic counselling.

#### **6. Conclusions**

Corrective surgery in syndromic craniosynostosis was formerly regarded as formidable however currently performed as a routine by major craniofacial center in the world due to advancement of technology and multidisciplinary approaches. Certain types of deformity, particularly those patients with Crouzon's or Apert's syndrome, require more than one functional intervention to achieve maximum correction.

In general, the surgical indication for paediatric CMF deformities can be classified into intermediate and definitive intervention. The intermediate intervention is performed at an early phase of patient's life and aimed to salvage vital tissue or organ function such as the brain, eye, airway or feeding which are essential for the child's development. The protocol of the management for syndromic craniosynostosis patients is summarized in **Figure 7**. These procedures include ventriculoperitoneal (VP) shunt, tarsorrhaphy, adenotonsillectomy and tracheostomy.

Therefore, these interventions in craniofacial syndromes is associated with multiple morbidities. It is important to understand the need and risks of these interventions prior making decision of treatment for each patient. Craniofacial teams should be cognizant to audit data on morbidity and mortality as well as surgical outcomes to monitor complication rates.

Surgical risk stratification involving the severity of patient's functional issues, age, co-morbidities, logistics, the timing and type of surgery and anticipated postoperative issues are in practiced to guide decision making consensus and serve as the index of precaution prior to any surgery [63]. This is in line with the recommendation of other centers which placed greater focus on protocols for airway management, blood salvage and replacement, age-appropriate deep venous thrombosis prophylaxis and timing of sub cranial midfacial advancements which might result in further reductions in craniofacial mortality rates.

**141**

**Acknowledgements**

**Figure 7.**

**Conflict of interest**

HKL, and SASMEC@IIUM, Malaysia.

The authors declare no conflict of interest.

*Summary of indications for intervention in craniofacial syndromes.*

We would like to thank everyone who were involved direct and indirectly in the making of this chapter, craniofacial team members, staff and patients in UMMC,

*Craniofacial Corrective Surgery in Syndromic Craniosynostosis*

*DOI: http://dx.doi.org/10.5772/intechopen.94584*

*Craniofacial Corrective Surgery in Syndromic Craniosynostosis DOI: http://dx.doi.org/10.5772/intechopen.94584*

#### **Figure 7.**

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

otorhinolaryngologists.

**5.3 Research**

counselling.

correction.

tracheostomy.

cal outcomes to monitor complication rates.

in further reductions in craniofacial mortality rates.

**6. Conclusions**

Different surgeon will advocate different surgical technique at different timing,

The ultimate goals for craniosynostosis treatment is mainly to restore function, improve facial aesthetic and ensure a healthy psychological development. More research is needed in this syndromic craniosynostosis in term of treatment algorithm, utilisation of latest and advancement of computer planning, computer navigation, 3-dimensional printing and usage of the cheaper, user friendly and effective surgical device in making the surgery more safer and more predictable outcome. The treatment focus not only on the fused suture of the skull, but also make an effort to address issue like intracranial pressure, strabismus, abnormally positioned orbit and dentofacial deformities [60–62]. Another area of future development will be in the molecular genetic testing in the field of genetic

Corrective surgery in syndromic craniosynostosis was formerly regarded as formidable however currently performed as a routine by major craniofacial center in the world due to advancement of technology and multidisciplinary approaches. Certain types of deformity, particularly those patients with Crouzon's or Apert's syndrome, require more than one functional intervention to achieve maximum

In general, the surgical indication for paediatric CMF deformities can be classified into intermediate and definitive intervention. The intermediate intervention is performed at an early phase of patient's life and aimed to salvage vital tissue or organ function such as the brain, eye, airway or feeding which are essential for the child's development. The protocol of the management for syndromic craniosynostosis patients is summarized in **Figure 7**. These procedures include ventriculoperitoneal (VP) shunt, tarsorrhaphy, adenotonsillectomy and

Therefore, these interventions in craniofacial syndromes is associated with multiple morbidities. It is important to understand the need and risks of these interventions prior making decision of treatment for each patient. Craniofacial teams should be cognizant to audit data on morbidity and mortality as well as surgi-

Surgical risk stratification involving the severity of patient's functional issues, age, co-morbidities, logistics, the timing and type of surgery and anticipated postoperative issues are in practiced to guide decision making consensus and serve as the index of precaution prior to any surgery [63]. This is in line with the recommendation of other centers which placed greater focus on protocols for airway management, blood salvage and replacement, age-appropriate deep venous thrombosis prophylaxis and timing of sub cranial midfacial advancements which might result

either with direct osteotomy and surgical plating or through distraction osteogenesis procedure [2, 4, 9, 49, 50, 58, 59]. There is no uniform surgical algorithm internationally, but more on surgeon or surgical center preferences. Obviously, these surgeries need collaboration of neurosurgeon, oral maxillofacial surgeon and

**140**

*Summary of indications for intervention in craniofacial syndromes.*
