Introduction: Recurrence following the open repair of craniosynostosis is a known event that has been described since 1974. However, the prevalence of this complication is poorly reported in the literature. The purpose of this study is to report the prevalence of symptomatic resynostosis following the repair of nonsyndromic craniosynostosis and elucidate any factors associated with this outcome over a sixteen year time period.

Methods: All nonsyndromic patients undergoing open surgical repair of craniosynostosis between 1997 and 2012 were identified. This population was then examined for symptomatic resynostosis requiring correction in addition to associated factors (such as gender, age at initial surgery, and affected cranial suture[s]). Patients requiring reoperation for other cosmetic issues (such as persistent cranial defects) were not included.

Results: 257 patients with nonsyndromic craniosynostosis underwent surgical repair at a mean age of 10.9 months (2.2-82.9 months). Fourteen (5.4%) patients required further surgery at an average age of 35.4 months (16.6-93.1 months) for a combination of clinical, cosmetic, radiographic, and ophthalmologic evidence of resynostosis. The average length of time between the initial procedure and subsequent reoperation was 29.0 months (11.6-69.5 months). Patients with bicoronal synostosis were significantly more likely to experience recurrent synostosis (p < 0.05). Additionally, all patients requiring reoperation had an acceptable cosmetic outcome (defined as a Whitaker Class I or II result) with an average follow-up of 6.2 years in this cohort.

Conclusions: This study of recurrence following open surgical repair of nonsyndomic craniosynostosis represents the largest to date. The prevalence in this study of 5.4% is consistent with prior reports. Given the prolonged time interval between initial surgery and recurrence, long-term surveillance for symptomatic resynostosis up to a decade post-operatively should be highly encouraged. Additionally, parents of patients with nonsyndromic bicoronal synostosis should be informed regarding the increased risk of symptomatic recurrence in this population.

Patient Care: This study improves patient care by highlighting the need for long-term follow-up to evaluate for symptomatic recurrence of craniosynostosis. Additionally, it illustrates the need for parents of patients with bicoronal synostosis to be aware of the higher recurrence rate in this population.

Learning Objectives: By the conclusion of this session, participants should be able to: 1) Describe the importance of symptomatic recurrence of nonsyndromic craniosynostosis and factors associated with its occurrence. 2) Realize the importance of long-term follow-up given the long time frame of recurrence.

References: 1. Agrawal D, Steinbok P, and Cochrane D. Recurrence of synostosis following surgery for isolated sagittal craniosynostosis. Can. J. Neurol. Sci. 32 (Suppl. 1): s9, 2005. 2. Cooper GM, Curry C, Barbano TE, Burrows AM, Vecchione L, Caccamese JF, Norbutt CS, Costello BJ, Losee JE, Moursi AM, Huard J, Mooney MP. Noggin inhibits postoperative resynostosis in craniosynostotic rabbits. J Bone Miner Res. 2007 Jul;22(7):1046-54. 3. Cooper GM, Usas A, Olshanski A, Mooney MP, Losee JE, Huard J. Ex vivo Noggin gene therapy inhibits bone formation in a mouse model of postoperative resynostosis. Plast Reconstr Surg. 2009 Feb;123(2 Suppl):94S-103S. 4. Esparza J, Hinojosa J, García-Recuero I, Romance A, Pascual B, Martínez de Aragón A. Surgical treatment of isolated and syndromic craniosynostosis. Results and complications in 283 consecutive cases. Neurocirugia (Astur). 2008 Dec;19(6):509-29. 5. Foster KA, Frim DM, McKinnon M. Recurrence of synostosis following surgical repair of craniosynostosis. Plast Reconstr Surg. 2008 Mar;121(3):70e-76e. 6. Gosain AK, Santoro TD, Song LS, Capel CC, Sudhakar PV, Matloub HS. Osteogenesis in calvarial defects: contribution of the dura, the pericranium, and the surrounding bone in adult versus infant animals. Plast Reconstr Surg. 2003 Aug;112(2):515-27. 7. Greene CS Jr. Pancraniosynostosis after surgery for single sutural craniosynostosis. Pediatr Neurosurg. 1998 Sep;29(3):127-32. 8. Hudgins RJ, Cohen SR, Burstein FD, Boydston WR. Multiple suture synostosis and increased intracranial pressure following repair of single suture, nonsyndromal craniosynostosis. Cleft Palate Craniofac J. 1998 Mar;35(2):167-72. 9. Marchac, D, Renier, D. Craniosynostosis. World J. Surg. 1989. 13: 358. 10. McCarthy JG, Glasberg SB, Cutting CB, Epstein FJ, Grayson BH, Ruff G, Thorne CH, Wisoff J, Zide BM. Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis--results and unsolved problems. Plast Reconstr Surg. 1995 Aug;96(2):272-83. 11. McCarthy JG, Glasberg SB, Cutting CB, Epstein FJ, Grayson BH, Ruff G, Thorne CH, Wisoff J, Zide BM. Twenty-year experience with early surgery for craniosynostosis: II. The craniofacial synostosis syndromes and pansynostosis--results and unsolved problems. Plast Reconstr Surg. 1995 Aug;96(2):284-95; discussion 296-8. 12. Mooney MP, Losken HW, Moursi AM, Bradley J, Azari K, Acarturk TO, Cooper GM, Thompson B, Opperman LA, Siegel MI. Anti-TGF-beta2 antibody therapy inhibits postoperative resynostosis in craniosynostotic rabbits. Plast Reconstr Surg. 2007 Apr 1;119(4):1200-12; discussion 1213-5. 13. Mooney MP, Losken HW, Moursi AM, Shand JM, Cooper GM, Curry C, Ho L, Burrows AM, Stelnicki EJ, Losee JE, Opperman LA, Siegel MI. Postoperative Anti-Tgf-beta2 Antibody Therapy Improves Intracranial Volume and Craniofacial Growth in Craniosynostotic Rabbits. J Craniofac Surg. 2007 Mar;18(2):336-46; discussion 347-9. 14. Mooney MP, Moursi AM, Opperman LA, Siegel MI. Cytokine therapy for craniosynostosis. Expert Opin Biol Ther. 2004 Mar;4(3):279-99 15. Norwood CW, Alexander E Jr, Davis CH Jr, Kelly DL Jr. Recurrent and multiple suture closures after craniectomy for craniosynostosis. J Neurosurg. 1974 Dec;41(6):715-9. 16. Renier D, El-Ghouzzi V, Bonaventure J, Le Merrer M, Lajeunie E. Fibroblast growth factor receptor 3 mutation in nonsyndromic coronal synostosis: clinical spectrum, prevalence, and surgical outcome. J Neurosurg. 2000 Apr;92(4):631-6. 17. Sloan GM, Wells KC, Raffel C, McComb JG. Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients. Pediatrics. 1997 Jul;100(1):E2. 18. Wagner JD, Cohen SR, Maher H, Dauser RC, Newman MH. Critical analysis of results of craniofacial surgery for nonsyndromic bicoronal synostosis. J Craniofac Surg. 1995 Jan;6(1):32-7; discussion 38-9. 19. Wall SA, Goldin JF, Hockly AD, Wake MJ, Poole MD, Briggs M. Fronto-orbital re-operation in craniosynostosis. Br. J. Plast. Surg. 1994; 47: 180. 20. Whitaker, L. A., Bartlett, S. P., Schut, L., and Bruce, D. Craniosynostosis: An analysis of the timing, treatment, and complications in 164 consecutive patients. Plast. Reconstr. Surg. 1987. 80:195. 21. Williams JK, Cohen SR, Burstein FD, Hudgins R, Boydston W, Simms C. A longitudinal, statistical study of reoperation rates in craniosynostosis. Plast Reconstr Surg. 1997 Aug;100(2):305-10.