Sagittal synostosis

Sagittal craniosynostosis or scaphocephaly is the premature fusion of the suture at the top of the head (sagittal suture) that forces the head to grow long and narrow, rather than wide.

Not ever isolated absence of the sagittal suture does not produce a scaphocephalic skull shape 1).

While sagittal synostosis is the most common craniosynostosis, long-term follow-up of these patients is lacking.

The data in the literature show that scaphocephaly has an incidence of 0.4 out of 1000 new-born, it has male preponderance M/F = 3,5/1 and familial case occurrence – rare 2), being 40–60% of all nonsyndromic forms 3) 4).

Relationship to gender is observed: 70–90% of all cases are found in boys 5).

If differential progressive deformity occurs between the vault and base, this might affect decision making regarding the timing of surgical intervention.

Atypical sagittal craniosynostosis can be detected initially or occur with a delay in apparently standard SCS. Leptocephaly is a specific entity. Because of the implications on the management and risk for the patient, preoperative evaluation of patients with SCS with CT scanner and prolonged follow-up are necessary 6).

Association of achondroplasia with sagittal synostosis and scaphocephaly in two patients is reported 7).

The etiology is often described as premature fusion of the sagittal suture.

The study of Sakamoto et al., observed wave-like deformations in abnormally long and narrow skulls typical of scaphocephaly, and these deformations were divided into two types: type I deformations (one wave) and type II deformations (two waves). However, the pathogeneses of these deformations are unknown. Computed tomographic data sets were retrospectively analyzed from 18 patients with scaphocephaly who were admitted to the hospital between 2000-2010. Using three-dimensional reconstructions of the computed tomographic images, the relationship was analysed between the wave deformation types and the state of the sutures and fontanelles. The results demonstrate that the type of wave deformation was dependent on the location of the sagittal suture closure. Specifically, the premature closure of the posterior half of the sagittal suture caused a type I deformation, while total closure resulted in a type II deformation (p < 0.001). It is hypothesized that restricted growth of the fused suture causes billowing, which results in a waving deformation. The deformities that are often observed in sagittal synostosis can be explained more accurately 8).

Sinus pericranii association 9).

Sagittal synostosis diagnosis.

see Sagittal synostosis treatment.

see Sagittal craniosynostosis outcome.

see Sagittal craniosynostosis case series.

Rao et al. reported a patient with development of sagittal craniosynostosis after birth, which we term postnatal sagittal craniosynostosis (PSC). This is a rare occurrence in which management considerations are critical but are not well discussed. A 3-year old boy presented with concerns of a metopic ridge. Work-up revealed metopic ridging and an open sagittal suture. The patient later developed signs of increased intracranial pressure (ICP) and repeat CT scan 14 months later identified a newly fused sagittal suture. The patient underwent open posterior cranial vault expansion, resulting in resolution of symptoms. PSC is a rare condition and should be considered in otherwise unexplained increases in ICP among pediatric patients. Open posterior cranial vault expansion represents a safe and effective method to treat this condition 10).

Padmalayam D, Tubbs RS, Loukas M, Cohen-Gadol AA. Absence of the sagittal suture does not result in scaphocephaly. Childs Nerv Syst. 2013 Apr;29(4):673-7. doi: 10.1007/s00381-012-2005-4. Epub 2012 Dec 21. PubMed PMID: 23263744.
Shillito J Jr, Matson DD. Craniosynostosis: a review of 519 surgical patients. Pediatrics. 1968 Apr;41(4):829-53. PubMed PMID: 5643989.
Komotar R.J., Zacharia B.E., Ellis J.A., Feldstein N.A., Anderson R.C. Pitfalls for the pediatrician: positional molding or craniosynostosis? Pediatr Ann 2006; 35: 5: 365—375.
Shuper A., Merlob P., Grunebaum M., Reisner S.H. The incidence of isolated craniosynostosis in the newborn infant. Am J Dis Child 1985; 139: 1: 85—86.
Komotar R.J., Zacharia B.E., Ellis J.A., Feldstein N.A., Anderson R.C. Pitfalls for the pediatrician: positional molding or craniosynostosis? Pediatr Ann 2006; 35: 5: 365—375.
Vinchon M, Pellerin P, Guerreschi P, Baroncini M, Dhellemmes P. Atypical scaphocephaly: a review. Childs Nerv Syst. 2012 Sep;28(9):1319-25. doi: 10.1007/s00381-012-1807-8. Epub 2012 Aug 8. Review. PubMed PMID: 22872243.
Accogli A, Pacetti M, Fiaschi P, Pavanello M, Piatelli G, Nuzzi D, Baldi M, Tassano E, Severino MS, Allegri A, Capra V. Association of achondroplasia with sagittal synostosis and scaphocephaly in two patients, an underestimated condition? Am J Med Genet A. 2015 Mar;167(3):646-52. doi: 10.1002/ajmg.a.36933. PubMed PMID: 25691418.
Sakamoto Y, Nakajima H, Tamada I, Miwa T, Kishi K, Yoshida K. New pathogenesis and the classification in scaphocephaly. J Plast Surg Hand Surg. 2014 Feb;48(1):24-7. doi: 10.3109/2000656X.2013.793602. Epub 2013 Apr 30. PubMed PMID: 23627629.
Kimiwada T, Hayashi T, Sanada T, Shirane R, Tominaga T. Surgical treatment of scaphocephaly with sinus pericranii. Neurol Med Chir (Tokyo). 2013;53(2):121-5. PubMed PMID: 23438666.
Rao V, Ali R, Roussel LO, Crozier JW, Svokos K, Woo AS. Postnatal Sagittal Craniosynostosis: A Novel Presentation and Considerations in Diagnosis and Management. J Craniofac Surg. 2021 Mar 1. doi: 10.1097/SCS.0000000000007599. Epub ahead of print. PMID: 33654043.
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