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Atypical sagittal suture craniosynostosis: pathological considerations for early closure of the anterior part of the sagittal suture

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Abstract

Sagittal suture synostosis is one of the most common craniosynostoses and is often diagnosed by characteristic narrow and long skull shape, scaphocephaly. However, some patients with sagittal suture synostosis do not present with typical scaphocephaly, making early diagnosis difficult. In this study, five cases of characteristic skull deformity showing a narrowing of the cranium posterior to the coronal suture on computed tomography (CT) are presented. The three older children presented with papilledema and intellectual disability and a closed sagittal suture on CT. The two infant cases were diagnosed with the characteristic cranial deformities with aggravation of the deformity over time, but sagittal suture closure was not evident on CT. All patients underwent cranial remodeling surgery. In the two infant cases, the histopathological findings showed that the anterior part of the sagittal suture was firmly fused with fibrous tissue without bony fusion. These findings suggested that narrowing of the cranium posterior to the coronal suture might be due to functional fusion of the anterior portion of the sagittal suture prior to bony fusion. In an infant presenting with such a deformity that shows aggravation of the deformity over time, surgical treatment should be considered.

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Authors and Affiliations

  1. Department of Neurosurgery, Miyagi Children’s Hospital, 4-3-17 Ochiai, Aoba-ku, Sendai, Miyagi, 989-3126, Japan

    Inukai Madoka, Hayashi Toshiaki & Kimiwada Tomomi

  2. Department of Neurosurgery, Kitasato University School of Medicine, Sagamihara, Japan

    Inukai Madoka & Kumabe Toshihiro

  3. Department of Pathology, Miyagi Children’s Hospital, Sendai, Japan

    Takeyama Junji

  4. Department of Plastic Surgery, Miyagi Children’s Hospital, Sendai, Japan

    Sanada Takehiko

  5. Department of Radiology, Miyagi Children’s Hospital, Sendai, Japan

    Shimanuki Yoshihisa & Kitami Masahiro

  6. Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan

    Endo Hidenori

Authors
  1. Inukai Madoka
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  2. Hayashi Toshiaki
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  3. Kimiwada Tomomi
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  4. Takeyama Junji
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  5. Sanada Takehiko
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  6. Shimanuki Yoshihisa
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  7. Kitami Masahiro
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  8. Kumabe Toshihiro
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  9. Endo Hidenori
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Contributions

Inukai Madoka and Hayashi Toshiaki wrote the main manuscript and text. Kimiwada Tomomi, Sanada Takehiko and Hayashi Toshiaki collected data. Shimanuki Yoshihisa and Kitami Masahiro performed radiological review. Takeyama Junji performed pathological review. Kumabe Toshihiro and Endo Hidenori supervised the study. All authors reviewed the manuscript.

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Correspondence to Hayashi Toshiaki.

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Madoka, I., Toshiaki, H., Tomomi, K. et al. Atypical sagittal suture craniosynostosis: pathological considerations for early closure of the anterior part of the sagittal suture. Childs Nerv Syst 40, 575–580 (2024). https://doi.org/10.1007/s00381-023-06141-6

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  • DOI: https://doi.org/10.1007/s00381-023-06141-6

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