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Long-term sequelae of normocephalic pansynostosis: a rare but insidious entity

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Abstract

Objective

Patients with normocephalic pansynostosis, who have a grossly normal head shape, are often overlooked early in life and present late with elevated intracranial pressure (ICP) requiring timely cranial vault expansion. This study evaluates the long-term clinical outcomes of patients with normocephalic pansynostosis.

Methods

We retrospectively reviewed patients with a clinical and radiographic diagnosis of primary pansynostosis who underwent vault reconstruction between 2000 and 2023. Clinical and treatment course after craniofacial interventions was followed in patients with normocephaly to assess outcomes.

Results

Thirty-five patients with primary pansynostosis were identified, of which eight (23.5%) had normocephaly and underwent initial vault expansion at a mean age of 5.0 ± 2.4 years. All eight patients (50.0% male) presented with symptoms of elevated ICP including headaches (50.0%), nausea and vomiting (50.0%), and developmental delay (62.5%) and/or signs of elevated ICP including papilledema (75.0%) and radiologic thumbprinting on head computed tomography scan (87.5%). Three of the four normocephalic patients who had over 7 years of postoperative follow-up developed subjective headaches, vision changes, or learning and behavioral issues in the long-term despite successful vault reconstruction.

Conclusions

Our longitudinal experience with this rare but insidious entity demonstrates the importance of timely intervention and frequent postoperative monitoring, which are critical to limiting long-term neurological sequelae. Multidisciplinary care by craniofacial surgery, neurosurgery, ophthalmology, and neuropsychology with follow-up into adolescence are recommended to assess for possible recurrence of elevated ICP secondary to cranio-cerebral disproportion.

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References

  1. Blount JP, Louis RG, Tubbs RS, Grant JH (2007) Pansynostosis: a review. Child’s Nerv Syst 23(10):1103–1109. https://doi.org/10.1007/s00381-007-0362-1

    Article  Google Scholar 

  2. Chumas PD, Cinalli G, Arnaud E, Marchac D, Renier D (1997) Classification of previously unclassified cases of craniosynostosis. J Neurosurg 86(2):177–181. https://doi.org/10.3171/jns.1997.86.2.0177

    Article  CAS  PubMed  Google Scholar 

  3. Foo R, Whitaker LA, Bartlett SP (2010) Normocephalic pancraniosynostosis resulting in late presentation of elevated intracranial pressures. Plast Reconstr Surg 125(5):1493–1502. https://doi.org/10.1097/PRS.0b013e3181d62b48

    Article  CAS  PubMed  Google Scholar 

  4. Connolly JP, Gruss J, Seto ML et al (2004) Progressive postnatal craniosynostosis and increased intracranial pressure. Plast Reconstr Surg 113(5):1313–1323. https://doi.org/10.1097/01.PRS.0000111593.96440.30

    Article  PubMed  Google Scholar 

  5. Renier D, Sainte-Rose C, Marchac D, Hirsch J-F (1982) Intracranial pressure in craniostenosis. J Neurosurg 57(3):370–377. https://doi.org/10.3171/jns.1982.57.3.0370

    Article  CAS  PubMed  Google Scholar 

  6. Collmann H, Sörensen N, Krauß J (2005) Hydrocephalus in craniosynostosis: a review. Child’s Nerv Syst 21(10):902–912. https://doi.org/10.1007/s00381-004-1116-y

    Article  CAS  Google Scholar 

  7. Reddy K, Hoffman H, Armstrong D (1990) Delayed and progressive multiple suture craniosynostosis. Neurosurgery. https://doi.org/10.1097/00006123-199003000-00011

    Article  PubMed  Google Scholar 

  8. Tan AP (2018) Primary normocephalic pancraniosynostosis detected incidentally after an accidental head injury: a case report and review of the literature. Child’s Nerv Syst 34(7):1427–1431. https://doi.org/10.1007/s00381-018-3768-z

    Article  Google Scholar 

  9. Killion EA, McEwan TW, Muzaffar AR (2013) Normocephalic pancraniosynostosis. J Craniofac Surg 24(5):1665–1668. https://doi.org/10.1097/SCS.0b013e3182997d8f

    Article  PubMed  Google Scholar 

  10. Wong S, Nagengast ES, Miller J (2016) Anterior cranial vault distraction for the treatment of normocephalic pancraniosynostosis. J Craniofac Surg 27(3):e283–e288. https://doi.org/10.1097/SCS.0000000000002452

    Article  PubMed  Google Scholar 

  11. Czerwinski M, Monsivais S (2017) Normocephalic pancraniosynostosis. J Craniofac Surg 28(5):1291–1292. https://doi.org/10.1097/SCS.0000000000003638

    Article  PubMed  Google Scholar 

  12. Chen EH, Gilardino MS, Whitaker LA, Bartlett SP (2009) Evaluation of the safety of posterior cranial vault reconstruction. Plast Reconstr Surg 123(3):995–1001. https://doi.org/10.1097/PRS.0b013e318199f4b4

    Article  CAS  PubMed  Google Scholar 

  13. Steinbacher DM, Skirpan J, Puchała J, Bartlett SP (2011) Expansion of the posterior cranial vault using distraction osteogenesis. Plast Reconstr Surg 127(2):792–801. https://doi.org/10.1097/PRS.0b013e318200ab83

    Article  CAS  PubMed  Google Scholar 

  14. Marchac D (1978) Radical forehead remodeling for craniostenosis. Plast Reconstr Surg 61(6):823–835. http://www.ncbi.nlm.nih.gov/pubmed/662945

  15. Whitaker LA, Bartlett SP, Schut L, Bruce D (1987) Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg 80(2):195–212. http://www.ncbi.nlm.nih.gov/pubmed/3602170

  16. Goldstein JA, Paliga JT, Wink JD, Low DW, Bartlett SP, Taylor JA (2013) A craniometric analysis of posterior cranial vault distraction osteogenesis. Plast Reconstr Surg 131(6):1367–1375. https://doi.org/10.1097/PRS.0b013e31828bd541

    Article  CAS  PubMed  Google Scholar 

  17. Derderian CA, Wink JD, McGrath JL, Collinsworth A, Bartlett SP, Taylor JA (2015) Volumetric changes in cranial vault expansion: comparison of fronto-orbital advancement and posterior cranial vault distraction osteogenesis. Plast Reconstr Surg 135(6):1665–1672. https://doi.org/10.1097/PRS.0000000000001294

    Article  CAS  PubMed  Google Scholar 

  18. Zapatero ZD, Kosyk MS, Kalmar CL et al (2022) How low should we go? Safety and craniometric impact of the low occipital osteotomy in posterior vault remodeling. Plast Reconstr Surg 150(5):1037e–1048e. https://doi.org/10.1097/PRS.0000000000009626

    Article  CAS  PubMed  Google Scholar 

  19. Humphries LS, Zapatero ZD, Vu GH et al (2022) Ten years of posterior cranial vault expansion by means of distraction osteogenesis: an update and critical evaluation. Plast Reconstr Surg 150(2):379–391. https://doi.org/10.1097/PRS.0000000000009336

    Article  CAS  PubMed  Google Scholar 

  20. Swanson JW, Samra F, Bauder A, Mitchell BT, Taylor JA, Bartlett SP (2016) An algorithm for managing syndromic craniosynostosis using posterior vault distraction osteogenesis. Plast Reconstr Surg 137(5):829e–841e. https://doi.org/10.1097/PRS.0000000000002127

    Article  CAS  PubMed  Google Scholar 

  21. van der Meulen J, van der Vlugt J, Okkerse J, Hofman B (2008) Early beaten-copper pattern: its long-term effect on intelligence quotients in 95 children with craniosynostosis. J Neurosurg Pediatr 1(1):25–30. https://doi.org/10.3171/PED-08/01/025

    Article  PubMed  Google Scholar 

  22. Arnaud E, Renier D, Marchac D (1995) Prognosis for mental function in scaphocephaly. J Neurosurg 83(3):476–479. https://doi.org/10.3171/jns.1995.83.3.0476

    Article  CAS  PubMed  Google Scholar 

  23. Gault DT, Renier D, Marchac D, Jones BM (1992) Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg 90(3):377–381. https://doi.org/10.1097/00006534-199209000-00003

    Article  CAS  PubMed  Google Scholar 

  24. Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C (2005) Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review. Child’s Nerv Syst 21(10):913–921. https://doi.org/10.1007/s00381-004-1117-x

    Article  CAS  Google Scholar 

  25. Pijpers M, Poels PJP, Vaandrager JM et al (2004) Undiagnosed obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis. J Craniofac Surg 15(4):670–674. https://doi.org/10.1097/00001665-200407000-00026

    Article  PubMed  Google Scholar 

  26. Baron ME, Dvoracek LA, Bykowski MR, Goldstein JA, Losee JE (2021) Undiagnosed normocephalic pancraniosynostosis presenting as bilateral abducens nerve palsy. J Craniofac Surg 32(1):270–272. https://doi.org/10.1097/SCS.0000000000007018

    Article  PubMed  Google Scholar 

  27. Bonfield CM, Tamber MS, Losee JE (2014) Blindness from late presenting undiagnosed pancraniosynostosis mimicking pseudotumor cerebri. J Child Neurol 29(8):NP24–NP27. https://doi.org/10.1177/0883073813495307

    Article  PubMed  Google Scholar 

  28. Persing JA (2008) MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis. Plast Reconstr Surg 121(Supplement):1–11. https://doi.org/10.1097/01.prs.0000305929.40363.bf

    Article  PubMed  Google Scholar 

  29. Kalmar CL, Lang S-S, Heuer GG et al (2022) Neurocognitive outcomes of children with non-syndromic single-suture craniosynostosis. Child’s Nerv Syst 38(5):893–901. https://doi.org/10.1007/s00381-022-05448-0

    Article  Google Scholar 

  30. Kalmar CL, Humphries LS, McGeehan B et al (2022) Elevated intracranial pressure in patients with craniosynostosis by optical coherence tomography. Plast Reconstr Surg 149(3):677–690. https://doi.org/10.1097/PRS.0000000000008821

    Article  CAS  PubMed  Google Scholar 

  31. Xu W, Gerety P, Aleman T, Swanson J, Taylor J (2016) Noninvasive methods of detecting increased intracranial pressure. Child’s Nerv Syst 32(8):1371–1386. https://doi.org/10.1007/s00381-016-3143-x

    Article  Google Scholar 

  32. Nazir S, O’Brien M, Qureshi NH, Slape L, Green TJ, Phillips PH (2009) Sensitivity of papilledema as a sign of shunt failure in children. J Am Assoc Pediatr Ophthalmol Strabismus 13(1):63–66. https://doi.org/10.1016/j.jaapos.2008.08.003

    Article  Google Scholar 

  33. Aylward SC, Waslo CS, Au JN, Tanne E (2016) Manifestations of pediatric intracranial hypertension from the Intracranial Hypertension Registry. Pediatr Neurol 61:76–82. https://doi.org/10.1016/j.pediatrneurol.2016.04.007

    Article  PubMed  Google Scholar 

  34. Swanson JW, Aleman TS, Xu W et al (2017) Evaluation of optical coherence tomography to detect elevated intracranial pressure in children. JAMA Ophthalmol 135(4):320. https://doi.org/10.1001/jamaophthalmol.2017.0025

    Article  PubMed  PubMed Central  Google Scholar 

  35. Pellicer E, Siebold BS, Birgfeld CB, Gallagher ER (2018) Evaluating trends in headache and revision surgery following cranial vault remodeling for craniosynostosis. Plast Reconstr Surg 141(3):725–734. https://doi.org/10.1097/PRS.0000000000004180

    Article  CAS  PubMed  Google Scholar 

  36. Miller MT, Pasquale M, Kurek S et al (2004) Initial head computed tomographic scan characteristics have a linear relationship with initial intracranial pressure after trauma. J Trauma Inj Infect Crit Care 56(5):967–973. https://doi.org/10.1097/01.TA.0000123699.16465.8B

    Article  Google Scholar 

  37. Kouvarellis AJ, Rohlwink UK, Sood V, Van Breda D, Gowen MJ, Figaji AA (2011) The relationship between basal cisterns on CT and time-linked intracranial pressure in paediatric head injury. Child’s Nerv Syst 27(7):1139–1144. https://doi.org/10.1007/s00381-011-1464-3

    Article  Google Scholar 

  38. Hormozi AK, Kasbi AA, Hormozi HK, Davai NR (2022) Assessing long-term neurodevelopment among children with non-syndromic single suture craniosynostosis. World J Plast Surg 11(2):57–61. https://doi.org/10.52547/wjps.11.2.57

    Article  Google Scholar 

  39. Long AS, Hauc SC, Almeida MN et al (2023) Morphologic severity and age at surgery are associated with school age neurocognitive outcomes in metopic craniosynostosis. Plast Reconstr Surg. https://doi.org/10.1097/PRS.0000000000010999

    Article  PubMed  Google Scholar 

  40. Junn AH, Long AS, Hauc SC et al (2023) Long-term neurocognitive outcomes in 204 single-suture craniosynostosis patients. Child’s Nerv Syst 39(7):1921–1928. https://doi.org/10.1007/s00381-023-05908-1

    Article  Google Scholar 

  41. Zapatero ZD, Kalmar CL, Kosyk MS et al (2021) Craniometric and volumetric analysis of posterior vault distraction osteogenesis: 10 year update. J Craniofac Surg 32(7):2379–2383. https://doi.org/10.1097/SCS.0000000000007838

    Article  PubMed  Google Scholar 

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No funding was received to assist with the preparation of this manuscript.

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Authors and Affiliations

  1. Division of Plastic, Reconstructive, and Oral Surgery, Children’s Hospital of Philadelphia, Pennsylvania, PA, USA

    Meagan Wu, Benjamin B. Massenburg, Neil Reddy, Dominic J. Romeo, Jinggang J. Ng, Jesse A. Taylor, Jordan W. Swanson & Scott P. Bartlett

  2. Division of Neurosurgery, Children’s Hospital of Philadelphia, Pennsylvania, PA, USA

    Shih-Shan Lang

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  1. Meagan Wu
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Contributions

M.W. participated in conception and design of the project, data analysis, interpretation of data, drafting of the manuscript, critical revision of the manuscript, and final approval of the version to be published. B.B.M., N.R., D.J.R., J.J.N., J.A.T., and J.W.S. participated in interpretation of data and critical revision of the manuscript. S.P.B. and S.S.L. participated in conception and design of the project, interpretation of data, critical revision of the manuscript, and final approval of the version to be published.

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Correspondence to Shih-Shan Lang.

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Wu, M., Massenburg, B.B., Reddy, N. et al. Long-term sequelae of normocephalic pansynostosis: a rare but insidious entity. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06379-8

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