Isolated sagittal craniosynostosis: definition, classification, and surgical indications

Massimi, Luca; Caldarelli, Massimo; Tamburrini, Gianpiero; Paternoster, Giovanna; Di Rocco, Concezio

doi:10.1007/s00381-012-1834-5

Cited by 76Craniofacial Disorders and TreatmentsPectus Deformity Diagnosis and TreatmentReconstructive Facial Surgery Techniques

Isolated sagittal craniosynostosis: definition, classification, and surgical indications

Massimi, Luca, Caldarelli, Massimo, Tamburrini, Gianpiero, Paternoster, Giovanna, Di Rocco, Concezio·2012·Child s Nervous System

DOI 10.1007/s00381-012-1834-5

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Abstract

Sagittal craniosynostosis (SC) remains the most common type of synostosis, accounting for about a half of all forms. It would result from a mesenchymal disorder involving the intramembranous ossification of the sagittal suture and leading to its early fusion. No specific data on the etiologic factors are currently available. The premature ossification of the sagittal suture can result in three main types of SC, according to the different segment prevalently involved: anterior, posterior, and complete SC. The diagnosis is easily obtained by clinical examination. However, a radiological work up (3D CT scan) may be necessary to rule out hidden venous or cranial anomalies possibly associated with most severe cases, or for the surgical planning. The most common indication for surgery is the improvement of the cosmetic appearance of the skull, since a cranial deformation may have a significant psychological impact on affected subjects. To relieve from raised intracranial pressure is a further indication to surgery. Although an increased intracranial pressure can be demonstrated in a minority of affected children at diagnosis, indeed, it can present later (usually after the second/third year of life) with chronic symptoms. The role of surgery in the preservation of cognitive functions in scaphocephalic patients does not seem to be relevant, since minor anomalies of the cerebral development associated with SC would occur independently from the cranial shape. On the other hand, the surgical correction may show a protective effect on some visual skills, like the ability to fix and follow, and the fixation shift.

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Pathophysiological factors that may explain neurocognitive problems in ISS include increased intracranial pressure, deformation of the brain due to skull deformation, problems with normal brain development, and compression of venous sinuses with impaired venous outflow.
“A number of pathophysiological factors, alone or in combination, may explain the occurrence of neurocognitive problems associated with ISS:increased intracranial pressure [26],deformation of the brain as a result of deformation of the skull [27],problems with the normal development of the brain,compression of the venous sinuses with resulting impairment of venous outflow [14,28].”
From Isolated Sagittal Craniosynostosis: A Comprehensive Review (2024) · cites this paper as [28]

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“Boys and multiple births are overrepresented and genetic causes are becoming known [28, 29].”
Background
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Background
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“Massimi clinically differentiated these anatomical features into three forms: anterior, the anterior third or half of the sagittal suture; posterior, posterior part of the suture closed with narrowing of posterior regions; complete, the most severe form is a fusion of the entire suture.(28) Our experience was differen…”
Result
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Background
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“However, detection and classification of exact craniofacial type as well as quantifying the condition rely on more clinical methods like Computed Tomography (CT), plain radiography and morphometric evaluation 1,3,6–8 .”
Method
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“Such deregulation would probably affect the undifferentiated mesenchymal cells occupying the mid-suture space [18].”
Background
Is There an Advantage to Minimizing Surgery in Scaphocephaly? A Study on Extended Strip Craniotomy Versus Extensive Cranial Vault Remodeling
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The Incidence of Chiari Malformations in Patients with Isolated Sagittal Synostosis
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Personalized Bone Reconstruction and Regeneration in the Treatment of Craniosynostosis
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A systematic quantitative morpho-volumetric analysis in infants with sagittal craniosynostosis and relationship with the severity of scaphocephalic deformity
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“4,5 11-15 There is no prospective investigation of ICP at the time of primary surgical intervention in the literature.”
Background
Forehead Widening in Nonsyndromic Scaphocephaly Operated After 12 Months of Age
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“Craniosynostosis is a premature fusion of cranial vault sutures, resulting in abnormal head shape and compensatory growth in the region of functionally intact sutures [1, 2].”
Background
Diagnosis and treatment of craniosynostosis: Vilnius team experience
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“Clinical and neurological examination is very important as craniosynostosis can result in raised intracranial pressure (11) and in untreated cases can lead to some neurological deficits or increased irritability (12).”
Background
Pityriasis rosea in pregnancy: report of a spousal occurrence and craniosynostosis in the healthy newborn
Loh, T. Y., & Cohen, P. R. (2016) · Dermatology Practical & Conceptual · doi:10.5826/dpc.0603a08 · Cited by 8 · see citing works
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“(J Craniofac Surg 2021;32: 2273–2276) P remature fusion of the sagittal suture is the most common form of craniosynostosis.(1) The skull morphology usually results in an elongated head shape (dolichocephaly).”
Background
Delayed Presentation of Isolated Sagittal Synostosis With Raised Intracranial Pressure and Secondary Chiari Malformation With Cervical Syringomyelia
Sofos, S. S., Robertson, B., Duncan, C., & Sinha, A. (2017) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000003734 · Cited by 7 · see citing works
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“The timing of surgery is typically within the first six to 12 months of life [3].”
BackgroundInfluential
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Gutierrez-Pineda, F., Franklin, B. A., Punukollu, A., García, G. A. G., Duque, O. E. M., Renteria, H. A., Perez, J. F. P., & Gonzalez, I. A. (2024) · Child s Nervous System · doi:10.1007/s00381-024-06472-y · Cited by 6 · see citing works
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Persistent Cranial Defects After Endoscopic Sagittal Synostosis Surgery
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Unilateral Coronal Craniosynostosis
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“The etiology of sagittal craniosynostosis is still largely unknown; however, environmental and genetic factors are thought to be implicated.(3) Controversy in the literature exists with regards to the cranial vault and base involvement in craniosynostosis.”
Background
Quantifying Sagittal Craniosynostosis Severity: A Machine Learning Approach With CranioRate
Tao, W., Somorin, T. J., Kueper, J., Dixon, A., Kass, N. M., Khan, N., Iyer, K., Wagoner, J., Rogers, A., Whitaker, R., et al. (2025) · The Cleft Palate-Craniofacial Journal · doi:10.1177/10556656251347366 · Cited by 4 · see citing works
“As Figure 3A shows, SSS-captured differences align with empirical observations of regional deformities, 4 offering clear clinical insights.”
A novel sagittal craniosynostosis classification system based on multi-view learning algorithm
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“[5] further classified sagittal CSO into three main”
Background
Cranial sutures ultrasonography as a valid diagnostic tool in isolated craniosynostoses: a pilot study
Saponaro, G., Bernardo, S., Curzio, P. D., Basile, E., Rastelli, E., Martini, S., Vinci, V., Saldari, M., Manganaro, L., & Cascone, P. (2013) · European Journal of Plastic Surgery · doi:10.1007/s00238-013-0898-0 · Cited by 4 · see citing works
Single sagittal craniosynostosis surgical treatment with the “Peau d́ours” technique. Single-center experience in Mexico
Arenas-Ruiz, J. A., Martínez-Maldonado, H., Hernández-Segura, N. E., Villarreal-Silva, E. E., González-Carranza, V., Torres-García, S., & Chico-Ponce de León, F. (2022) · Journal of Clinical Neuroscience · doi:10.1016/j.jocn.2022.02.016 · Cited by 3 · see citing works
Clinical study and some molecular features of Mexican patients with syndromic craniosynostosis
Ibarra‐Arce, A., Almaraz‐Salinas, M., Martínez‐Rosas, V., de Zárate‐Alarcón, G. O., Flores‐Peña, L., Romero‐Valdovinos, M., & Olivo‐Díaz, A. (2020) · Molecular Genetics & Genomic Medicine · doi:10.1002/mgg3.1266 · Cited by 3 · see citing works
Correlation between degree of sagittal suture fusion and surrogates of elevated intracranial pressure in sagittal craniosynostosis
Villavisanis, D. F., Blum, J. D., Barrero, C. E., Kalmar, C. L., Plana, N. M., Cho, D., Shakir, S., Bartlett, S. P. P., Taylor, J. A., & Swanson, J. W. (2023) · Journal of Neurosurgery Pediatrics · doi:10.3171/2023.4.peds22539 · Cited by 3 · see citing works
Evaluating Cephalic Index Changes in Sagittal Synostosis Surgery: A Retrospective Study of Subtotal Versus Total Vault Remodeling
Kurzbuch, A. R., Cooper, B., Duncan, C. A., Ellenbogen, J. R., Vakharia, V. N., Hennedige, A. A., Parks, C., & Sinha, A. (2024) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000010922 · Cited by 3 · see citing works
Disorders of Head Shape and Size
Elgamal, E. A., & Salih, M. A. M. (2020) · Springer eBooks · doi:10.1007/978-3-319-43153-6_33 · Cited by 2 · see citing works
Novel Method of Lateral Vault Modification in Scaphocephaly
Madaree, A., & Morris, W. M. M. (2021) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000007874 · Cited by 1 · see citing works
An Exploration of the Practice of CT Modalities to Evaluate Anterior Cranial Deformities in Craniosynostosis
Madaree, A., Bisetty, V., Mohan, N., Barnes, C., & Lazarus, L. (2023) · Biomedical visualization · doi:10.1007/978-3-031-36850-9_7 · Cited by 1 · see citing works
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Multi-directional Cranial Distraction Osteogenesis for Treating Sagittal Synostosis with Frontometaphyseal Dysplasia: A Case Report
Morishita, Y., Sunaga, A., Gomi, A., Hatade, A., Morita, Y., & Yoshimura, K. (2021) · Plastic & Reconstructive Surgery Global Open · doi:10.1097/gox.0000000000003551
[Simulation method of skull remodellingsurgeryfor infant with craniosynostosis].
Lu, S., Luo, Y., Zheng, W., & Gong, J. F. (2021) · PubMed Central · doi:10.7507/1001-5515.202101046
Craniosynostosis
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Unilateral Coronal Craniosynostosis
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Craniostenoses
(2016) · Elsevier eBooks · doi:10.1016/b978-0-323-37754-6.50328-6
Craniosynostosis
Ghizoni, E., do Amaral, C. E. R., & Denadai, R. (2019) · Springer eBooks · doi:10.1007/978-3-030-17649-5_12
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Craniosynostosis in Isfahan, Iran: A Cross-Sectional Study
Abdali, H., Anaraki, A. G., & Mahdipour, S. (2022) · Journal of Maxillofacial and Oral Surgery · doi:10.1007/s12663-022-01794-3
“In sagittal type, maternal smoking [21], age, race, paternal industrial job, fertility assistant treatments, non-singleton pregnancy [27], and male gender, and in metopic type, advanced maternal age, non-singleton pregnancy, fertility assistant treatments, and male gender are shown to be involved…”
Sagittal Synostectomy With Tension Band Sutures for Correction of Sagittal Craniosynostosis
Hersh, A. M., & Cohen, A. (2023) · Operative Neurosurgery · doi:10.1227/ons.0000000000000851
Preoperative Frontal and Parietal Bone Thickness Assessment to Predict Blood Loss and Transfusion During Extended Suturectomy for Isolated Sagittal Craniosynostosis
Grove, A. M., Kirsch, H. M., Kurnik, N. M., Bristol, R. E., Sitzman, T. J., Pfeifer, C. M., & Singh, D. (2023) · The Cleft Palate-Craniofacial Journal · doi:10.1177/10556656231202840
Genetische Ursachen syndromaler und nicht-syndromaler Formen der Lippen-Kiefer-Gaumenspalte und funktionelle Analyse möglicher Kandidatengene
Fiedler, J. H. (2023) · doi:10.53846/goediss-10186
Craniosynostosis
Fakhroo, F. A., Allehaibi, M. H., Ahmed, F. O., & Ramadan, A. H. A. (2023) · Springer eBooks · doi:10.1007/978-3-031-49573-1_6
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Cranial morphology in 3-dimensional laser scans before and after open craniectomy for scaphocephaly
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A Novel Noninvasive Screening Method for Early Detection of Sagittal Craniosynostosis Using the Surface Cranial Index
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Sagittal craniosynostosis with Moebius syndrome; case illustration: first case in the literatüre
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“It accounts for approximately half of all synostosis cases, with an estimated incidence of 1 in 2000 [7].”
CRANIOSYNOSTOSIS AS A PUBLIC HEALTH CHALLENGE – CURRENT KNOWLEDGE ON ETIOLOGY, SYNDROMIC FORMS, AND MANAGEMENT STRATEGIES
Rejman, P., Stachura, P., Wilk, B., Witkowska, E., Pszczoła, K., Ciechański, M., Cholewiński, S., Wilk, K., Jurkiewicz, K., & Kasprzyk, A. (2025) · International Journal of Innovative Technologies in Social Science · doi:10.31435/ijitss.3(47).2025.3968
“Helmet therapy is continued postoperatively to maintain correction and promote symmetrical cranial development [4, 29, 30, 31].”
Influential
A new method for the treatment of scaphocephaly by porcupine quill like diverging longitudinal parietal flaps
Yasonov, S. A., & Vasilyev, I. A. (2026) · Plastic Surgery and Aesthetic Medicine · doi:10.17116/plast.hirurgia202601156
What is the impact of age at surgery on perioperative medical care in sagittal craniosynostosis ?
Riffaud, L., Sorlin, M., Bras, E. L., Rousseau, C., Aillet, S., Nardi, N., & Eustache, G. (2026) · Journal of Cranio-Maxillofacial Surgery · doi:10.1016/j.jcms.2026.104567
Investigation of the Effectiveness of Cranial Helmets Produced With 3D Printing Technology in Positional Cranial Deformities
Uncu, A., Alsancak, S., & Onganlar, Y. H. (2026) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000012891

Sagittal Suture Synostosis: Key Points for Surgeons and Management Principles (Diagnosis, Surgical Considerations and Timing)
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Sagittal suture craniosynostosis or craniosynostoses? The heterogeneity of the most common premature fusion of the cranial sutures
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Surgery for isolated sagittal synostosis: a neuro-developmental necessity or an isolated cosmetic challenge? Mini-survey and literature review
Soleman, J., Tamburrini, G., Roth, J., & Constantini, S. (2026) · Child s Nervous System · doi:10.1007/s00381-026-07313-w
Delayed Presentation of Isolated Sagittal Synostosis With Raised Intracranial Pressure and Secondary Chiari Malformation With Cervical Syringomyelia
Sofos, S. S., Robertson, B., Duncan, C., & Sinha, A. (2017) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000003734 · Cited by 7 · see citing works
Classification System for Sagittal Craniosynostosis
David, L. R., Glazier, S. S., Pyle, J. W., Thompson, J. T., & Argenta, L. C. (2009) · Journal of Craniofacial Surgery · doi:10.1097/scs.0b013e3181945ab0 · Cited by 56 · see citing works

Authors: Massimi, L., Caldarelli, M., Tamburrini, G., Paternoster, G., & Di Rocco, C.
Affiliations: Università Cattolica del Sacro Cuore (IT)
Corresponding author: Massimi, Luca
ORCID: Massimi, Luca
Caldarelli, Massimo
Tamburrini, Gianpiero
Venue: Child s Nervous System
Publisher: Springer Science and Business Media LLC
Published: 2012-08-08
Volume: 28
Issue: 9
Pages: 1311-1317
ISSN: 0256-7040 (ISSN-L)
1433-0350
DOI: 10.1007/s00381-012-1834-5
OpenAlex: W2073738974
Cited by: 76
References: 49
Citations by year: 2026: 3 · 2025: 5 · 2024: 4 · 2023: 9 · 2022: 11 · 2021: 11 · 2020: 9 · 2019: 5 · 2018: 2 · 2017: 3 · 2016: 8 · 2015: 3 · 2014: 1 · 2013: 2
FWCI: 1.15
Type: journal article
Language: en
Open access: closed
Licence: www.springer.com/tdm (tdm)
Locations: Child s Nervous System (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Craniofacial Disorders and Treatments
Health Sciences › Medicine › Surgery › Pectus Deformity Diagnosis and Treatment
Health Sciences › Medicine › Surgery › Reconstructive Facial Surgery Techniques
Keywords: sagittal craniosynostosis, intracranial pressure, cranial deformity, cranial deformity surgery, cognitive function, visual function, three-dimensional computed tomography, pediatric neurosurgery
MeSH terms: Craniosynostoses, Humans

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Isolated sagittal craniosynostosis: definition, classification, and surgical indications

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