The management of craniosynostosis in Australia and New Zealand

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William Andrew Ziaziaris
Damian D Marucci


Craniosynostosis, scaphocephaly, 3D photography, Monobloc, Le Fort III


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.

Methods: 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.


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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. 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.
3. Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostoses. Child’s Nerv Syst. 2000;16:645-58. PMid:11151714
4. Kolar JC. An epidemiological study of nonsyndromal craniosynostoses. J Craniofac Surg. 2011;22:47-49. 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. 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. 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. 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. 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.
10. Honeycutt JH. Endoscopic-assisted craniosynostosis surgery. Semin Plast Surg. 2014;28(3):144-49. PMid:25210508 PMCid:PMC4154971
11. Jimenez DF, Barone CM. Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg. 1998;88:77-81. 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. 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.
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. 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. 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. 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. 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. 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. 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.
21. DiMaggio C, Sun LS, Ing C, Li G. Pediatric anesthesia and neurodevelopmental impairments: a Bayesian meta-analysis. J Neurosurg Anesth. 2012;24:376. 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. 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. 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. 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. 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. PMid:12389887
27. Panchal J, Uttchin V. Management of craniosynostosis. Plast Reconstr Surg. 2003;111:2032-2048;quiz 2049. 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. 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. 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. 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. PMid:11159059
33. Brenner DJ, Hall EJ. Computed tomography—an increasing source of radiation exposure. New Eng J Med. 2007;357:2277-284. 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.
35. Schull WJ, Norton S, Jensh RP. Ionizing radiation and the developing brain. Neurotoxicol Teratol. 1990;12:249-60.
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. 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. 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. 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. 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. 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. PMid:12549753
42. Virtanen R, Korhonen T, Fagerholm J, Viljanto J. Neurocognitive sequelae of scaphocephaly. Pediatrics. 1999;103:791-95. 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. 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. 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. 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. PMid:21311385
47. van Veelen MLC, Mathijssen IM. Spring-assisted correction of sagittal suture synostosis. Childs Nerv Syst. 2012;28:1347-351. 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. 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. 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. PMid:18453937
52. Persing JA, Delashaw JB, Jane JA, Edgerton MT. Lambdoid synostosis: surgical considerations. Plast Reconstr Surg. 1988;81:852-60. 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. PMid:18216667
54. Sgouros S, Goldin J, Hockley A, Wake M. Posterior skull surgery in craniosynostosis. Childs Nerv Syst. 1996;12:727-33. 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. 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.
57. Goodrich JT, Argamaso R. Lambdoid stenosis (posterior plagiocephaly) and craniofacial asymmetry: long-term outcomes. Childs Nerv Syst. 1996;12:720-26.
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. PMid:12425988
59. Al-Jabri T, Eccles S. Surgical correction for unilateral lambdoid synostosis: a systematic review. J Craniofac Surg. 2014;25:1266-272. 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. 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. 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. 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.
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. PMid:15547389
67. Miller MT. Ocular abnormalities in craniofacial malformations. Int Ophthalmol Clin. 1984;24:143-63. PMid:6443567
68. Marucci DD, Dunaway DJ, Jones BM, Hayward RD. Raised intracranial pressure in Apert syndrome. Plast Reconstr Surg. 2008;122:1162-168. 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. 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.
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. 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. 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. 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. 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. PMid:21398092