Download

AJOPS logo

Type: Review article
Published: 15-03-2019

The management of craniosynostosis in Australia and New Zealand

William A Ziaziaris MBBS MPhil,1,2 Damian D Marucci MBBS PhD FRACS1

1 

Children’s Hospital at Westmead Clinical School
Faculty of Medicine and Health
The University of Sydney
Westmead, New South Wales
AUSTRALIA

2

 

Royal North Shore Hospital
St Leonards, New South Wales
AUSTRALIA

OPEN ACCESS
Correspondence

Name
: Damian D Marucci
Clinical senior lecturer and craniofacial surgeon
Address
: Children’s Hospital at Westmead Clinical School
Faculty of Medicine and Health
The University of Sydney
Locked Bag 4001
Westmead, New South Wales, 2145
AUSTRALIA Email: damian.marucci@sydney.edu.au
Phone
: +61 9845 0918
Citation
: Ziaziaris W, Marucci DD. The management of craniosynostosis in Australia and New Zealand. Aust J Plast Surg. 2019;2(1):78–87. https://doi.org/10.34239/ajops.v2i1.98
Accepted for publication
: 25 January 2019
Copyright © 2019
. Authors retain their copyright in the article. This is an open access article distributed under the Creative Commons Attribution Licence which permits unrestricted use, distribution and reproduction in any medium, provided the original work is properly cited.

Section: Craniomaxillofacial


Abstract

Introduction: The management of syndromic and non-syndromic craniosynostosis is controversial with different units performing various procedures at different points in time and for different reasons. This study was performed to compare protocols of assessment and surgery in craniofacial units in Australasia.

Method: All paediatric craniofacial surgery centres in Australia and New Zealand were surveyed with respect to their current protocols for the preoperative assessment, intraoperative management and postoperative follow-up of syndromic and non-syndromic craniosynostosis patients.

Results: All eight centres completed the survey with 100 per cent response rate. There was considerable homogeneity in the management of the various synostoses. Differences in management were notable in the preoperative assessments that were offered and the management of midface retrusion in syndromic craniosynostoses.

Conclusion: Single-suture management among surveyed centres was relatively homogenous. Developmental assessment is not routinely performed for single-suture disease but is desired by the majority of surveyed centres. Postoperative CT scans are not commonly used but a number of centres desired routine use of 3D photometry for outcome assessment. In multi-suture cases, expansion of the posterior vault was performed in all cases but management of midface retrusion was variable.

Keywords: craniosynostosis, scaphocephaly, 3D photography, Le Fort III, monobloc


Introduction

Craniosynostosis involves the premature fusion of one or more cranial sutures. This leads to cranial deformity and potentially raised intra-cranial pressure or other deleterious long-term neurological sequelae.1–3 Synostosis can be ‘simple’, involving only one cranial suture or ‘complex’, involving multiple sutures and/or being related to a genetic syndrome with other limb, neurological, respiratory or cardiac abnormalities. The incidence of craniosynostosis in the population is one in 2000–4000 live births with single-suture involvement by far the most prevalent.4,5

The management of craniosynostosis is primarily surgical, however, the timing and choice of surgical intervention varies between units.6–10 There are few high-level evidence studies that compare the different management options for craniosynostoses to support a single model of treatment. Most studies are retrospective, have small cohorts and inconsistently compare outcomes, making comparison of protocols problematic.7,9,11–16 Some studies advocate for early surgical correction of synostosis to improve long-term neurocognitive outcomes whereas other centres perform surgery at a later stage. Moreover, some units propose major surgical procedures such as total calvarial remodeling (TCVR) whereas other units treat similar conditions with minimally invasive techniques, with or without adjuncts, such as cranial springs or helmets.11,17,18 Arguments for delaying operations include: potential for reduced blood product requirement, less disruption to growth and avoidance of deleterious neurocognitive effects of anaesthesia performed at a young age.17,19–24

The aim of this study was to survey paediatric craniomaxillofacial centres in Australia and New Zealand to document current practices with respect to preoperative assessment, surgical management and postoperative follow-up for patients with both syndromic and non-syndromic craniosynostosis.

Method

Institutional review board approval from the Sydney University was obtained (project number 2017/354) to conduct a survey of the assessment and management of craniosynostosis. All members of the Australian and New Zealand Society of Craniomaxillofacial Surgeons (ANZSCMFS) were asked about their usual protocols for the preoperative assessment, intraoperative management and postoperative follow-up of patients with syndromic and non-syndromic craniosynostosis. Specifically, the survey noted which craniofacial multi-disciplinary team members assessed the patients preoperatively, what investigations were performed, the choice and timing of surgical procedures for the different patterns of craniosynostosis and finally, the nature and frequency of follow-up. Surgeons were also surveyed as to what assessments they currently do not perform but would like to in the future. Where there was unclear or missing information provided, surgeons were contacted directly by email to clarify their responses to the survey. Data was collated, de-identified and statistical analysis performed using IBM® SPSS® Version 25 (SPSS Statistics for Windows, IBM Corp, Armonk, New York, USA, 2017).

Results

All eight centres of craniofacial surgery in Australia and New Zealand who are members of the Australian and New Zealand Society of Craniomaxillofacial Surgeons completed the survey (100% participation).

Single-suture craniosynostosis

Preoperative assessment

One centre stated no preoperative investigations were routinely performed, instead being decided on a case-by-case basis. Head circumference was the most uniformly performed preoperative assessment with seven of eight centres using this in single-suture synostoses. Computed tomography scanning and fundoscopy were the next most commonly performed assessment, used in six of eight centres. In the instances where preoperative CT scanning was not performed, elevated intracranial pressure was monitored using head circumference and fundoscopy. Where fundoscopy was not performed, CT scanning and head circumference were routine.

Conversely, developmental assessment, sleep studies, MRI and visual evoked potentials (VEPs) were far less common, each being performed in one of eight surveyed centres. Developmental assessment was the most desired service not currently routinely offered for single-suture disease within the remaining six of seven centres. Table 1 shows common investigations performed during preoperative assessments and most desired investigations not currently offered.

The management of single-suture craniosynostosis across all centres is summarised in Table 2.

Table 1: Preoperative investigations single-suture craniosynostosis
Investigation Number of centres offered (desired)
Head circumference 7 (0)
Developmental assessment 1 (6)
3D photography 4 (3)
Plain skull x-ray 3 (0)
Sleep study 1 (0)
Fundosopy 6 (1)
MRI 1 (0)
Visual evoked potentials 1 (1)
Genetic testing 3 (1)
CT scan 6 (0)
Table 2: Management of single-suture craniosynostoses
Suture involved Most common preferred surgical procedure Proportion of centres Timing of procedure
Sagittal SMC  5/8 < 6 months
Metopic FOAR  7/8 6–12 months
Lambdoid PCVR  5/8 < 12 months
Unicoronal FOAR  8/8 6–12 months
Bicoronal FOAR  5/8 6–12 months

Sagittal synostosis management

Management of sagittal synostosis varied with age in six of eight centres. The preferred operation was spring-mediated cranioplasty (SMC) for five centres and total calvarial vault remodelling (TCVR) for the remaining three centres. Centres performing SMC did so at <six months while TCVR was performed between six and 12 months of age. All of the centres preferring SMC performed TCVR for patients presenting after six months of age.

Metopic synostosis management

In seven of eight centres, fronto-orbital advancement remodelling (FOAR) was the preferred procedure. In the remaining centre, SMC performed prior to six months of age is employed. All eight centres performed FOAR for metopic synostosis beyond six months of age.

Lambdoid synostosis management

Spring-mediated cranioplasty is the preferred management at one centre, performed at less than six months of age. TCVR was the next most common procedure, being the preferred intervention at two centres, performed at six–12 months. Lastly, posterior cranial vault remodelling (PCVR) was the most commonly performed procedure, used in five centres. The timing of PCVR varied across these five centres with four operating at or prior to 12 months and one centre after 12 months of age.

Unicoronal synostosis management

Unicoronal synostosis was collectively managed with FOAR or similar operation with patients between six and 12 months of age. One centre stated they occasionally performed TCVR in lieu of FOAR in the same age group.

Bicoronal synostosis management

Bicoronal synostosis was managed with a single procedure in six centres, while the remaining two centres used a two-stage surgical approach. Of those performing a single procedure, timing of surgery was between six–12 months. FOAR was most common, used in five centres. The remaining centre performed SMC. For the two centres who perform two-stage procedure, a posterior vault distraction was performed at six months of age followed by FOAR thereafter at 12 months of age.

Follow-up for single-suture craniosynostosis

All centres followed patients annually or bi-annually one year postoperatively. There was some heterogeneity in the length of follow-up with three centres reviewing patients until skeletal maturity, two until teenage years, one until ten years of age, one for five years of age and one centre reviewing patients indefinitely. Table 3 shows common investigations performed at follow-up for single-suture craniosynostosis.

Table 3: Common investigations performed at follow-up for single-suture craniosynostosis
Investigation Number of centres offered
Head circumference 6
Developmental assessment 1
3D photography 4
Plain skull x-ray 1
Sleep study 0

Multi-suture craniosynostosis

Preoperative assessment

The mean preoperative investigations offered per centre for multi-suture craniosynostosis was greater than the mean investigations for simple synostosis (7.50 vs. 4.13, p=0.003). Head circumference and CT scan are routinely performed preoperatively in all multi-suture/syndromic craniosynostosis patients across all eight centres. Three dimensional photography is routinely offered at four centres with three of the remaining four stating they wish to offer this in the future. Table 4 summarises preoperative assessments for multi-suture craniosynostosis.

Table 4: Preoperative investigations multi-suture/syndromic craniosynostosis
Investigation Number of centres offered (desired)
Head circumference 8 (0)
Developmental assessment 6 (2)
3D photography 4 (3)
Plain skull x-ray 3 (0)
Sleep study 8 (0)
Fundosopy 7 (0)
MRI 4 (1)
Visual evoked potentials 4 (1)
Genetic testing 8 (0)
CT scan 8 (0)

Management of syndromic craniosynostosis without midface retrusion

In all eight centres, volume expansion of the posterior vault was performed initially. Five centres performed posterior vault distractions, two performed spring-mediated posterior vault cranioplasty and one performed formal posterior vault remodelling. All centres followed expansion of the posterior vault with FOAR with one centre additionally performing tarsorrhaphy. Table 5 summarises the management of multi-suture craniosynostosis syndromic without midface retrusion across eight centres.
Management of syndromic craniosynostosis with midface retrusion.

Management of syndromic craniosynostosis with midface retrusion is summarised in Table 6. All eight centres performed an initial procedure to increase the volume of the posterior vault. Five centres performed posterior vault distraction, three performed spring-mediated posterior vault cranioplasy and one performed formal posterior vault remodelling. Subsequent procedures were Le Fort III and monobloc osteotomy in three centres each and FOAR in the remaining two centres. Table 7 shows common follow-up investigations multi-suture craniosynostosis.

Table 5: Management of multi-suture craniosynostosis syndromic without midface retrusion
Centre Timing Single or multiple procedures Procedure
1 6–12m multi Posterior vault distraction/FOAR
2 6–12m multi SMC/FOAR
3 4–7m multi Posterior vault distraction/FOAR /tarsorrhaphy
4 1–12m multi Posterior vault distraction/FOAR
5 6–12m multi Posterior vault distraction/FOAR
6 6–12m multi Posterior vault distraction/FOAR
7 6–12m multi PVR/FOAR
8 6–12m multi SMC/PVR/FOAR
Table 6: Management of multi-suture craniosynostosis syndromic with midface retrusion
Centre Initial procedure Subsequent procedure(s)
1 Posterior vault distraction/FOAR Le Fort III
2 SMC Monobloc distraction, bimax surgery
3 Posterior vault distraction/FOAR +/- tarsorrhaphy Le Fort III
4 Posterior vault distraction/FOAR Le Fort III
5 Posterior vault distraction Monobloc distraction
6 SMC Monobloc distraction
7 PVR FOAR
8 SMC FOAR
Table 7: Common investigations performed at follow-up for multi-suture craniosynostosis
Investigation Number of centres offered
Head circumference 4
Developmental assessment 1
3D photography 4
Plain skull x-ray 1
Sleep study 4
Fundosopy 8
MRI 1
Visual evoked potentials 1
Genetic testing 0
CT scan 1
Photography 4

Discussion

Craniosynostosis may present as simple, single-suture disease, involve multiple sutures or be secondary to complex genetic syndromes where multiple sutures and organs are affected. Craniofacial clinics are necessarily multidisciplinary as the management of these patients may be multi-modal. Craniofacial multi-disciplinary teams commonly include neurosurgeons, plastic surgeons, speech therapists, geneticists, ear, nose and throat surgeons, orthodontists, ophthalmologists, sleep physicians, general paediatricians, neuropsychologists and specialist nursing staff.25

This is the first Australasian survey of the preoperative assessment, intraoperative management and postoperative follow-up of patients with craniosynostosis. This is of particular relevance due to the variety of preoperative screening tools and surgical techniques available and the current lack of a definitive management guidelines for any or all variants of craniosynostosis. We demonstrated relative homogeneity of management of the various types of craniosynostosis between surveyed centres with major differences coming in the preoperative screening investigations offered and the management of midface retrusion in syndromic synostosis.

However, the current standard of care and the use of CT scanning for the diagnosis and evaluation of synostosis is controversial. Computed tomography is said to have a role in diagnosis, screening for intracranial complications of craniosynostosis, surgical planning and for use as a baseline for later comparison.26–28 The advent of 3D reconstructions has also added further benefit to diagnosis and preoperative planning.29 Our survey demonstrates that a high proportion of centres across Australia and New Zealand routinely use CT for the preoperative evaluation of patients with single-suture craniosynostosis. All centres routinely performed preoperative CT scanning for multi-suture and syndromic craniosynostosis.

Whether the detailed information provided by CT scanning is useful in the management of single-suture disease is questionable. Some reports suggest clinical examination alone should be sufficient for diagnosis and that the decreased likelihood of intracranial anomalies makes CT less useful in this population.26,30 Some studies have outlined the potential long-term detriments associated with ionising radiation exposure in infancy.31–35 A non-ionising alternative to CT, ‘Black Bone’ MRI, has been shown to accurately identify cranial sutures and diagnose craniosynostosis but lacks widespread availability and would represent a significant pressure on hospital resources.36 Further analysis with multi-centre studies is warranted to ascertain its clinical and cost effectiveness. In our survey, only one centre routinely performed MRI in lieu of CT for single-suture craniosynostosis. Magnetic resonance imaging is a safer alternative for young patients and provides greater detail of neuroanatomy to rule out structural brain anomalies. However, CT with 3D reconstructions with its superior bone depiction remains the gold standard imaging technique for craniosynostosis.

Three dimensional photography is another non-ionising, non-invasive alternative to CT scanning and has been successfully implemented for preoperative phenotype screening, surgical planning and postoperative follow-up to quantify cranial volumes and facial proportions.37–39 Four of the surveyed centres routinely used 3D cameras in the preoperative assessment of both single-suture and multi-suture/syndromic craniosynostoses. Of the remaining four centres, three stated they wished to offer 3D photography to patients in the future. Sharing of data obtained from 3D cameras would allow for objectively comparing outcomes following synostosis surgery while avoiding the long-term detriments of repeat CT scanning.

Neuropsychology or developmental assessments of patients with synostosis is not routine. However, there is a significant body of evidence that demonstrates significant deficits in memory, language, processing speed and attention in patients with craniosynostosis even with surgical correction.17,40–44 Across Australasia, developmental assessments were offered at two centres for simple, and six centres for multi-suture/syndromic, synostosis respectively. For the preoperative assessment of simple synostosis, developmental assessment was the most desired service not currently offered.

The management options for sagittal synostosis, the most common type of single-suture craniosynostosis, remain broad. Sagittal synostosis was originally treated with simple excision of the fused suture (a sagittal ‘strip’) however, results were inconsistent and the procedure fell out of favour. Some units prefer traditional total calvarial vault remodelling procedures involving the removal of most of the calvarial vault, reshaping and replacing it in order to correct the scaphocephalic head shape.45 Total calvarial vault remodelling is a major surgical undertaking that may involve significant blood loss, long operating time and associated increased hospital length of stay.46 Spring-mediated cranioplasty is a popular alternative to TCVR and, in this study, was used by the most centres for the management of sagittal synostosis. Spring-mediated cranioplasty is usually conducted in patients less than six–seven months of age with an advantage being that it is minimally invasive and provides active expansion of the cranial vault.47 However, once springs are placed, expansion of the vault cannot be controlled and a second procedure is required to remove the springs. No units in Australasia were using endoscopic suturectomy and helmet therapy to treat craniosynostosis, although this technique is practiced elsewhere.18,48

For other synostoses, our study demonstrated that the vast majority of surveyed centres used FOAR for metopic and unicoronal craniosynostosis and PCVR for the rarer lambdoid synostosis. With fusion of the metopic suture, the sagittal and lambdoid sutures compensate producing increased skull width posteriorly and a narrowed forehead and creating a characteristic triangular appearance when viewed from above.49 In some centres, the management of metopic synostosis is dictated by the severity of disease with mildly affected patients undergoing burring of the affected metopic ridge while more severe cases undergo remodeling.50

In unicoronal synostosis, the ipsilateral forehead flattens, the supraorbital rim is elevated and the contralateral forehead undergoes compensatory bossing.49 Fronto-orbital advancement remodelling is the current standard of care for unilateral coronal synostosis.51

Lambdoid synostosis is rare with the characteristic deformity being an ipsilateral, flat posterior skull with ipsilateral loss of skull height and compensatory parietal bone bossing. In their recent systematic review, Al-Jabri et al showed that an overwhelming majority of published reports on the management of lambdoid synostosis favour more extensive remodelling techniques, similar to the preferences demonstrated across Australasia in this study.8,46,52–59 Some centres however, advocate for minimally invasive strip craniectomy.18

The approach to patients with syndromic multi-suture craniosynostosis was relatively consistent between the surveyed centres. Multi-suture syndromic synostosis without midface retrusion was uniformly managed with an initial procedure to increase the volume of the posterior vault, followed by FOAR. Expansion of the posterior vault is considered the ideal first stage procedure in syndromic multi-suture synostoses as it significantly increases intracranial volume and thus reduces the incidence of raised intracranial hypertension and its sequelae.60 An additional advantage of commencing with posterior expansion is that the fronto-orbital region remains untouched, facilitating future monobloc or Le Fort III advancements as appropriate to the case.61

Over 150 syndromes associated with craniosynostosis have been described.49,62 Many of the more common types of syndromic craniosynostoses, such as Crouzon and Apert syndromes, may be associated with significant midfacial hypoplasia, which may be secondary to compensatory growth of the cranium parallel to synostosed sutures and/or a primary developmental abnormality of the cranial base.63–65 The sequelae of the midfacial hypoplasia of syndromic craniosynostosis may include exophthalmos, obstructive sleep apnoea (OSA) and class III malocclusion. Left untreated, exophthalmos may lead to corneal ulceration and visual loss while OSA is associated with daytime somnolence, hypercapnia and raised ICP.63,66–68

The deformity of midfacial hypoplasia in the setting of syndromic craniosynostosis can be addressed in two ways. The first, a two-stage procedure with early fronto-orbital advancement to address brachycephaly and a later Le Fort III advancement to address the midfacial hypoplasia. Alternatively, the forehead and midface can be advanced simultaneously as a ‘monobloc’ or single piece, combining the Le Fort III osteotomy with a frontal bone advancement in patients with severe exophthalmos.49,63,69,70 A perceived advantage of monobloc distraction over Le Fort III is the increased average midface advancement achieved.69 Previous studies have demonstrated improvements in respiratory function, exophthalmos and facial profile with an average of 25 mm advancement, while the average advancement achieved with Le Fort III osteotomies is reportedly in the range of 6–17 mm.66,69,71,72 In the published literature, orbital deficiency (how shallow the syndromic craniosynostosis patient’s hypoplastic orbit lies with resultant exophthalmos) approaches 24 mm.66,69,71,72 While Le Fort III has been successful in expanding orbital cavity volume, monobloc osteotomy may provide an alternative for severe exophthalmos.66

The use of distraction techniques in either Le Fort III or monobloc advancements limits the retrofrontal dead space, allows for brain expansion anteriorly and slow soft tissue accommodation and thus reduces complication rates.61,73 Prior to distraction osteogenesis, midface advancement was performed as a single-stage procedure and was associated with increased rates of haematoma, ascending meningitis and frontal bone loss.62,74 The advent of internal/external distraction allows midface advancement to occur in smaller increments over time and hence may improve the safety of procedure.74,75 Distraction is generally well tolerated, supports newly forming bone and avoids the need for bone grafting.63 However, long term data pertaining to changes in rates of secondary procedures post distraction osteogenesis is lacking and currently, despite best efforts, a large proportion of children with midface hypoplasia will require subsequent operations as they reach adolescence and young adulthood.76 Thus far, it seems morbidity and mortality between patients undergoing Le Fort III or monobloc advancements is similar.69

There are several limitations of this study. Responses may reflect the ideal but not actual management of craniosynostosis at various centres. Moreover, this survey does not account for individual variations in treatment protocols resulting from atypical presentations or individual surgeon preference. For the most desired preoperative investigations, ambiguity exists as to whether or not providing a service represents a limited resource or if centres did not think it indicated. Lastly, this survey is a snapshot into the practices of craniomaxillofacial centres across Australia and New Zealand at this time with trends in patient management not well represented. Future surveys conducted into the management of craniofacial pathologies should include questions on changing or evolving surgical practice as well as rationale for specific investigations and procedures.

Conclusion

This study represents the first attempt to summarise the management of craniosynostosis across a regional program. Despite significant variability in treatment options, the management of synostoses was relatively similar between centres. It is hoped that this work may stimulate collaborative and comparative research between the different units in Australasia.

Acknowledgement

The authors would like to formally acknowledge the support and endorsement of the Australian and New Zealand Society of Craniomaxillofacial Surgeons for this project.

Disclosure

The authors have no financial or commercial conflicts of interest to disclose.

References

  1. Bristol RE, Lekovic GP, Rekate HL, editors. The effects of craniosynostosis on the brain with respect to intracranial pressure. Semin Pediatr Neurol. 2004;11(4):262–67. https://doi.org/10.1016/j.spen.2004.11.001 PMid:15828710
  2. Renier D, Brunet L, Marchac D. IQ and craniostenosis. In: Marchac D (ed). Craniofacial surgery. Berlin, Heidelberg: Springer;1987. p 114–17. https://doi.org/10.1007/978-3-642-82875-1_25
  3. Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostoses. Child’s Nerv Syst. 2000;16:645–58. https://doi.org/10.1007/s003810000320 PMid:11151714
  4. Kolar JC. An epidemiological study of nonsyndromal craniosynostoses. J Craniofac Surg. 2011;22:47–49. https://doi.org/10.1097/SCS.0b013e3181f6c2fb PMid:21187784
  5. Selber J, Reid RR, Chike-Obi CJ, Sutton LN, Zackai EH, McDonald-McGinn D, Sonnad SS, Whitaker LA, Bartlett SP. The changing epidemiologic spectrum of single-suture synostoses. Plast Reconstr Surg. 2008;122(2):527–33. https://doi.org/10.1097/PRS.0b013e31817d548c PMid:18626371
  6. Gerety PA, Basta MN, Fischer JP, Taylor JA. Operative management of nonsyndromic sagittal synostosis: a head-to-head meta-analysis of outcomes comparing 3 techniques. J Craniofac Surg. 2015;26:1251–57. https://doi.org/10.1097/SCS.0000000000001651 PMid:26080168
  7. Chan JW, Stewart CL, Stalder MW, Hilaire HS, McBride L, Moses MH. Endoscope-assisted versus open repair of craniosynostosis: a comparison of perioperative cost and risk. J Craniofac Surg. 2013;24:170–74. https://doi.org/10.1097/SCS.0b013e3182646ab8 PMid:23348279
  8. David LR, Plikaitis CM, Couture D, Glazier SS, Argenta LC. Outcome analysis of our first 75 spring-assisted surgeries for scaphocephaly. J Craniofac Surg. 2010;21:3–9. https://doi.org/10.1097/SCS.0b013e3181c3469d PMid:20061981
  9. Guimarães-Ferreira J, Gewalli F, David L, Olsson R, Friede H, Lauritzen CG. Spring-mediated cranioplasty compared with the modified pi-plasty for sagittal synostosis. Scand J Plast Recons. 2003;37:208–15. https://doi.org/10.1080/02844310310001823
  10. Honeycutt JH. Endoscopic-assisted craniosynostosis surgery. Semin Plast Surg. 2014;28(3):144–49. https://doi.org/10.1055/s-0034-1384810 PMid:25210508 PMCid:PMC4154971
  11. Jimenez DF, Barone CM. Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg. 1998;88:77–81. https://doi.org/10.3171/jns.1998.88.1.0077 PMid:9420076
  12. Lauritzen CG, Davis C, Ivarsson A, Sanger C, Hewitt TD. The evolving role of springs in craniofacial surgery: the first 100 clinical cases. Plast Reconstr Surg. 2008;121:545–54. https://doi.org/10.1097/01.prs.0000297638.76602.de PMid:18300975
  13. Marsh J, Jenny A, Galic M, Picker S, Vannier M. Surgical management of sagittal synostosis. A quantitative evaluation of two techniques. Neurosurg Clin N Am. 1991;2:629–40. https://doi.org/10.1016/S1042-3680(18)30724-1
  14. Maugans TA, McComb G, Levy ML. Surgical management of sagittal synostosis: a comparative analysis of strip craniectomy and calvarial vault remodeling. Pediatr Neurosurg. 1997;27:137–48. https://doi.org/10.1159/000121241 PMid:9548524
  15. Ridgway EB, Berry-Candelario J, Grondin RT, Rogers GF, Proctor MR. The management of sagittal synostosis using endoscopic suturectomy and postoperative helmet therapy. J Neuros-Pediatr. 2011;7:620–26. https://doi.org/10.3171/2011.3.PEDS10418 PMid:21631199
  16. Seymour-Dempsey K, Baumgartner JE, Teichgraeber JF, Xia JJ, Waller AL, Gateno J. Molding helmet therapy in the management of sagittal synostosis. J Craniofac Surg. 2002;13:631–35. https://doi.org/10.1097/00001665-200209000-00007 PMid:12218789
  17. Hashim PW, Patel A, Yang JF, Travieso R, Terner J, Losee JE, Pollack I, Jane J Sr, Jane J Jr, Kanev P, Mayes L, Duncan C, Bridgett DJ, Persing JA. The effects of whole-vault cranioplasty versus strip craniectomy on long-term neuropsychological outcomes in sagittal craniosynostosis. Plast Reconstr Surg. 2014;134(3):491–501. https://doi.org/10.1097/PRS.0000000000000420 PMid:24804639
  18. Jimenez DF, Barone CM, Cartwright CC, Baker L. Early management of craniosynostosis using endoscopic-assisted strip craniectomies and cranial orthotic molding therapy. Pediatrics. 2002;110:97–104. https://doi.org/10.1542/peds.110.1.97 PMid:12093953
  19. Patel A, Yang JF, Hashim PW, Travieso R, Terner J, Mayes LC, Kanev P, Duncan C, Jane J Jr, Jane J Sr, Pollack I, Losee JE, Bridgett DJ, Persing JA. The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis. Plast Reconstr Surg. 2014;134(4):608e–17e. https://doi.org/10.1097/PRS.0000000000000511 PMid:25357055
  20. Davidson AJ, Disma N, De Graaff JC, Withington DE, Dorris L, Bell G, Stargatt R, Bellinger DC, Schuster T, Arnup SJ, Hardy P, Hunt RW, Takagi MJ, Giribaldi G, Hartmann PL, Salvo I, Morton NS, von Ungern Sternberg BS, Locatelli BG, Wilton N, Lynn A, Thomas JJ, Polaner D, Bagshaw O, Szmuk P, Absalom AR, Frawley G, Berde C, Ormond GD, Marmor J, McCann ME; GAS consortium. Neurodevelopmental outcome at 2 years of age after general anaesthesia and awake-regional anaesthesia in infancy (GAS): an international multicentre, randomised controlled trial. Lancet. 2016;387(10015):239–50. https://doi.org/10.1016/S0140-6736(15)00608-X
  21. DiMaggio C, Sun LS, Ing C, Li G. Pediatric anesthesia and neurodevelopmental impairments: a Bayesian meta-analysis. J Neurosurg Anesth. 2012;24:376. https://doi.org/10.1097/ANA.0b013e31826a038d PMid:23076225 PMCid:PMC3475986
  22. Ing C, DiMaggio C, Whitehouse A, Hegarty MK, Brady J, von Ungern-Sternberg BS, Davidson A, Wood AJ, Li G, Sun LS. Long-term differences in language and cognitive function after childhood exposure to anesthesia. Pediatrics. 2012;130(3):e476–85. https://doi.org/10.1542/peds.2011-3822 PMid:22908104
  23. Flick RP, Katusic SK, Colligan RC, Wilder RT, Voigt RG, Olson MD, Sprung J, Weaver AL, Schroeder DR, Warner DO. Cognitive and behavioral outcomes after early exposure to anesthesia and surgery. Pediatrics. 2011;128(5):e1053-61. https://doi.org/10.1542/peds.2011-0351 PMid:21969289 PMCid:PMC3307194
  24. Hansen TG, Pedersen JK, Henneberg SW, Pedersen DA, Murray JC, Morton NS, Christensen K. Academic performance in adolescence after inguinal hernia repair in infancya nationwide cohort study. Anesthesiolog. 2011;114:1076–085. https://doi.org/10.1097/ALN.0b013e31820e77a0 PMid:21368654
  25. Capone RB, Sykes JM. The cleft and craniofacial team: the whole is greater than the sum of its parts. Facial Plast Surg. 2007;23:83–86. https://doi.org/10.1055/s-2007-979275 PMid:17516333
  26. Cerovac S, Neil-Dwyer J, Rich P, Jones B, Hayward R. Are routine preoperative CT scans necessary in the management of single suture craniosynostosis? Brit J Neurosurg. 2002;16:348–54. https://doi.org/10.1080/0268869021000007560 PMid:12389887
  27. Panchal J, Uttchin V. Management of craniosynostosis. Plast Reconstr Surg. 2003;111:2032–2048;quiz 2049. https://doi.org/10.1097/01.PRS.0000056839.94034.47 PMid:12711969
  28. Furuya Y, Edwards MS, Alpers CE, Tress BM, Norman D, Ousterhout DK. Computerized tomography of cranial sutures: Part 2: Abnormalities of sutures and skull deformity in craniosynostosis. J Neurosurg. 1984;61:59–70. https://doi.org/10.3171/jns.1984.61.1.0059 PMid:6726412
  29. Tartaro A, Larici A, Antonucci D, Merlino B, Colosimo C, Bonomo L. Optimization and diagnostic accuracy of computerized tomography with tridimensional spiral technique in the study of craniostenosis. Radiol Med. 1998;96:10–7.
  30. Fearon JA, Singh DJ, Beals SP, Jack CY. The diagnosis and treatment of single-sutural synostoses: are computed tomographic scans necessary? Plast Reconstr Surg. 2007;120:1327–331. https://doi.org/10.1097/01.prs.0000279477.56044.55 PMid:17898608
  31. Anderson V, Godber T, Smibert E, Weiskop S, Ekert H. Cognitive and academic outcome following cranial irradiation and chemotherapy in children:a longitudinal study. Br J Cancer. 2000;82:255. https://doi.org/10.1054/bjoc.1999.0912 PMid:10646874 PMCid:PMC2363266
  32. Brenner DJ, Elliston CD, Hall EJ, Berdon WE. Estimated risks of radiation-induced fatal cancer from pediatric CT. Am Rroentgenol. 2001;176:289–96. https://doi.org/10.2214/ajr.176.2.1760289 PMid:11159059
  33. Brenner DJ, Hall EJ. Computed tomography—an increasing source of radiation exposure. New Eng J Med. 2007;357:2277–284. https://doi.org/10.1056/NEJMra072149 PMid:18046031
  34. Hall EJ, Wuu CS. Radiation-induced second cancers: the impact of 3D-CRT and IMRT. Int J Radiat Oncol Biol Phys. 2003;56:83–88. https://doi.org/10.1016/S0360-3016(03)00073-7
  35. Schull WJ, Norton S, Jensh RP. Ionizing radiation and the developing brain. Neurotoxicol Teratol. 1990;12:249–60. https://doi.org/10.1016/0892-0362(90)90096-U
  36. Eley K, McIntyre A, Watt-Smith S, Golding S. ‘Black bone’ MRI: a partial flip angle technique for radiation reduction in craniofacial imaging. Br J Radiol. 2012;85:272–78. https://doi.org/10.1259/bjr/95110289 PMid:22391497 PMCid:PMC3473988
  37. Chan FC, Kawamoto HK, Federico C, Bradley JP. Soft-tissue volumetric changes following monobloc distraction procedure: analysis using digital three-dimensional photogrammetry system (3dMD). J Craniofac Surg. 2013;24:416–20. https://doi.org/10.1097/SCS.0b013e31827ff296 PMid:23524705
  38. Schaaf H, Malik CY, Streckbein P, Pons-Kuehnemann J, Howaldt HP, Wilbrand JF. Three-dimensional photographic analysis of outcome after helmet treatment of a nonsynostotic cranial deformity. J Craniofac Surg. 2010;21:1677–682. https://doi.org/10.1097/SCS.0b013e3181f3c630 PMid:21119399
  39. Chen ZC, Albdour MN, Lizardo JA, Chen YA, Chen PKT. Precision of three-dimensional stereo-photogrammetry (3dMD™) in anthropometry of the auricle and its application in microtia reconstruction. J Plast Reconstr Aes. 2015;68:622–31. https://doi.org/10.1016/j.bjps.2015.02.020 PMid:25892285
  40. Boltshauser E, Ludwig S, Dietrich F, Landolt M. Sagittal craniosynostosis: cognitive development, behaviour, and quality of life in unoperated children. Neuropediatrics. 2003;34:293–300. https://doi.org/10.1055/s-2003-44667 PMid:14681754
  41. Shipster C, Hearst D, Somerville A, Stackhouse J, Hayward R, Wade A. Speech, language, and cognitive development in children with isolated sagittal synostosis. Dev Med Child Neurol. 2003;45:34–43. https://doi.org/10.1111/j.1469-8749.2003.tb00857.x PMid:12549753
  42. Virtanen R, Korhonen T, Fagerholm J, Viljanto J. Neurocognitive sequelae of scaphocephaly. Pediatrics. 1999;103:791–95. https://doi.org/10.1542/peds.103.4.791 PMid:10103304
  43. Magge SN, Westerveld M, Pruzinsky T, Persing JA. Long-term neuropsychological effects of sagittal craniosynostosis on child development. J Craniofac Surg. 2002;13:99–04. https://doi.org/10.1097/00001665-200201000-00023 PMid:11887004
  44. Chieffo D, Tamburrini G, Massimi L, Di Giovanni S, Giansanti C, Caldarelli M, Di Rocco C. Long-term neuropsychological development in single-suture craniosynostosis treated early. Journal of Neurosurgery: Pediatrics. 2010;5:232–37. https://doi.org/10.3171/2009.10.PEDS09231 PMid:20192638
  45. Panchal J, Marsh JL, Park T, Kaufman B, Pilgram T, Huang SH. Sagittal craniosynostosis outcome assessment for two methods and timings of intervention. Plast Reconstr Surg. 1999;103:1574–584. https://doi.org/10.1097/00006534-199905000-00004 PMid:10323690
  46. Seruya M, Oh AK, Boyajian MJ, Posnick JC, Myseros JS, Yaun AL, Keating RF. Long-term outcomes of primary craniofacial reconstruction for craniosynostosis: a 12-year experience. Plast Reconstr Surg. 2011;127:2397–406. https://doi.org/10.1097/PRS.0b013e318213a178 PMid:21311385
  47. van Veelen MLC, Mathijssen IM. Spring-assisted correction of sagittal suture synostosis. Childs Nerv Syst. 2012;28:1347–351. https://doi.org/10.1007/s00381-012-1850-5 PMid:22872247
  48. Shah MN, Kane AA, Petersen JD, Woo AS, Naidoo SD, Smyth MD. Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children’s Hospital experience. J Neurosurg Pediatr. 2011;8:165–70. https://doi.org/10.3171/2011.5.PEDS1128 PMid:21806358
  49. Fearon JA. Evidence-based medicine: craniosynostosis. Plast Reconstr Surg. 2014;133:1261–275. PMid:24776557
  50. Aryan HE, Jandial R, Ozgur BM, Hughes SA, Meltzer HS, Park MS, Levy ML. Surgical correction of metopic synostosis. Childs Nerv Syst. 2005;21:392–98. https://doi.org/10.1007/s00381-004-1108-y PMid:15714353
  51. Selber JC, Brooks C, Kurichi JE, Temmen T, Sonnad SS, Whitaker LA. Long-term results following fronto-orbital reconstruction in nonsyndromic unicoronal synostosis. Plast Reconstr Surg. 2008;121:251e–260e. https://doi.org/10.1097/PRS.0b013e31816a9f88 PMid:18453937
  52. Persing JA, Delashaw JB, Jane JA, Edgerton MT. Lambdoid synostosis: surgical considerations. Plast Reconstr Surg. 1988;81:852–60. https://doi.org/10.1097/00006534-198806000-00006 PMid:3375348
  53. Pearson GD, Havlik RJ, Eppley B, Nykiel M, Sadove AM. Craniosynostosis: a single institution’s outcome assessment from surgical reconstruction. J Craniofac Surg. 2008;19:65–71. https://doi.org/10.1097/SCS.0b013e31815c8aae PMid:18216667
  54. Sgouros S, Goldin J, Hockley A, Wake M. Posterior skull surgery in craniosynostosis. Childs Nerv Syst. 1996;12:727–33. https://doi.org/10.1007/BF00366158 PMid:9118138
  55. Fearon JA, Ruotolo RA, Kolar JC. Single sutural craniosynostoses: surgical outcomes and long-term growth. Plast Reconstr Surg. 2009;123:635–42. https://doi.org/10.1097/PRS.0b013e318195661a PMid:19182624
  56. Esparza J, Hinojosa J, Garcia-Recuero I, Romance A, Pascual B, de Aragón AM. Surgical treatment of isolated and syndromic craniosynostosis. Results and complications in 283 consecutive cases. Neurocirugía. 2008;19:509–29. https://doi.org/10.1016/S1130-1473(08)70201-X
  57. Goodrich JT, Argamaso R. Lambdoid stenosis (posterior plagiocephaly) and craniofacial asymmetry: long-term outcomes. Childs Nerv Syst. 1996;12:720–26. https://doi.org/10.1007/BF00366157
  58. Zöller JE, Mischkowski RA, Speder B. Preliminary results of standardized occipital advancement in the treatment of lambdoid synostosis. J Craniomaxillofac Surg. 2002;30:343–48. https://doi.org/10.1054/jcms.2002.0326 PMid:12425988
  59. Al-Jabri T, Eccles S. Surgical correction for unilateral lambdoid synostosis: a systematic review. J Craniofac Surg. 2014;25:1266–272. https://doi.org/10.1097/SCS.0000000000000961 PMid:24978452
  60. Spruijt B, Rijken BF, den Ottelander BK, Joosten KF, Lequin MH, Loudon SE, van Veelen ML, Mathijssen IM. First vault expansion in Apert and Crouzon-Pfeiffer syndromes: front or back? Plast Reconstr Surg. 2016;137:112e–121e. https://doi.org/10.1097/PRS.0000000000001894 PMid:26368328
  61. Posnick J. Craniofacial dysostosis syndromes: a staged reconstructive approach. facial clefts and craniosynostosis: principles and management. Philadelphia: WB Saunders Co, 1996. pp 630–85.
  62. Cohen SR, Boydston W, Hudgins R, Burstein FD. Monobloc and facial bipartition distraction with internal devices. J Craniofac Surg. 1999;10:244–51. https://doi.org/10.1097/00001665-199905000-00013 PMid:10530235
  63. Toth BA, Kim JW, Chin M, Cedars M. Distraction osteogenesis and its application to the midface and bony orbit in craniosynostosis syndromes. J Craniofac Surg. 1998;9:100–113;discussion 119–22. https://doi.org/10.1097/00001665-199803000-00003 PMid:9586536
  64. Virchow R. Uber den cretinismus, namentlich in franken, and uber patholoische schadelformen. Verh Phys Med Ges Würzburg. 1851;2:230.
  65. Moss ML. The pathogenesis of premature cranial synostosis in man. Cells Tissues Organs. 1959;37:351–70. https://doi.org/10.1159/000141479
  66. Meling TR, Due-Tønnessen BJ, Høgevold HE, Skjelbred P, Arctander K. Monobloc distraction osteogenesis in pediatric patients with severe syndromal craniosynostosis. J Craniofac Surg. 2004;15:990–1000. https://doi.org/10.1097/00001665-200411000-00020 PMid:15547389
  67. Miller MT. Ocular abnormalities in craniofacial malformations. Int Ophthalmol Clin. 1984;24:143–63. https://doi.org/10.1097/00004397-198402410-00012 PMid:6443567
  68. Marucci DD, Dunaway DJ, Jones BM, Hayward RD. Raised intracranial pressure in Apert syndrome. Plast Reconstr Surg. 2008;122:1162–168. https://doi.org/10.1097/PRS.0b013e31818458f0 PMid:18827651
  69. Meling TR, Høgevold HE, Due-Tønnessen BJ, Skjelbred P. Comparison of perioperative morbidity after LeFort III and monobloc distraction osteogenesis. Br J Oral Maxillofac Surg. 2011;49:131–34. https://doi.org/10.1016/j.bjoms.2009.11.015 PMid:20226576
  70. Gillies H, Harrison SH. Operative correction by osteotomy of recessed malar maxillary compound in a case of oxycephaly. Br J Plast Surg. 1950;3:123–27. https://doi.org/10.1016/S0007-1226(50)80019-X
  71. McCarthy JG, Staffenberg DA, Wood RJ, Cutting CB, Grayson BH, Thorne CH. Introduction of an intraoral bone-lengthening device. Plast Reconstr Surg. 1995;96:978–81. https://doi.org/10.1097/00006534-199509001-00034 PMid:7652076
  72. Ousterhout DK, Vargervik K, Clark S. Stability of the maxilla after Le Fort III advancement in craniosynostosis syndromes. Cleft Palate J. 1986;23:91–101. PMid:3469047
  73. Arnaud E, Marchac D, Renier D. Reduction of morbidity of the frontofacial monobloc advancement in children by the use of internal distraction. Plast Reconstr Surg. 2007;120:1009–026. https://doi.org/10.1097/01.prs.0000278068.99643.8e PMid:17805131
  74. McCarthy J, Schreiber J, Karp N, Thorne C, Grayson B. Lengthening the human mandible by gradual distraction. Plast Reconstr Surg. 1992;89(1):1–8;discussion 9–10. https://doi.org/10.1097/00006534-199289010-00001 PMid:1727238
  75. Witherow H, Dunaway D, Evans R, Nischal KK, Shipster C, Pereira V, et al. Functional outcomes in monobloc advancement by distraction using the rigid external distractor device. Plast Reconstr Surg. 2008;121:1311–322. https://doi.org/10.1097/01.prs.0000305538.75347.52 PMid:18349650
  76. Nout E, Koudstaal M, Wolvius E, Van der Wal K. Additional orthognathic surgery following Le Fort III and monobloc advancement. Int J Oral Maxillofac Surg. 2011;40:679–84. https://doi.org/10.1016/j.ijom.2011.02.014 PMid:21398092