Craniosynostosis - Recognition, clinical characteristics, and treatment
Open accessAbstract
Craniosynostosis is a developmental craniofacial anomaly, resulting in impairment of brain development and abnormally shaped skull. The main cause of craniosynostosis is premature closure of one or more cranial sutures. It usually occurs as an isolated condition, but may also be associated with other malformations as part of complex syndromes. When left untreated, craniosynostosis can cause serious complications, such as developmental delay, facial abnormality, sensory, respiratory and neurological dysfunction, anomalies affecting the eye, and psychological disturbances. Thus, early diagnosis, expert surgical techniques, postoperative care, and adequate follow-up are of vital importance in treating craniosynostosis.
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ISS is the most common form of craniosynostosis, with an incidence of 1:5000 births, and accounts for 40 to 60% of all cases.
“ISS is the most common form of craniosynostosis occurring in clinical practise (incidence 1:5000 births) and accounts for 40 to 60% of all cases [3,10].”
From Isolated Sagittal Craniosynostosis: A Comprehensive Review (2024) · cites this paper as [3]
The CRANIOSYNOSTOSIS section (p. 1) lists sagittal synostosis as the most frequent type, at approximately 60% of cases. It does not give an incidence of 1 in 5,000 births for ISS; its figure of 1 in 2,100–2,500 births is for craniosynostosis overall. (LLM, conf=0.88)
Experimental — accuracy not yet measuredWith appropriate and successful surgical treatment of ISS, the likelihood of neurocognitive impairment in children is very low and has been reported to be up to 9% in a mild form.
“With appropriate and successful surgical treatment of ISS, the likelihood of neurocognitive impairment in children is very low and has been reported to be up to 9% in a mild form [3].”
From Isolated Sagittal Craniosynostosis: A Comprehensive Review (2024) · cites this paper as [3]
The Influence on Child Development section (p. 4) reports mild cognitive and academic deficits in children with simple craniosynostosis and a lower likelihood of learning disabilities with sagittal synostosis than with some other subtypes. The Treatment and Complications section (p. 5) describes normal brain development as a goal of surgery, but the paper does not report a post-surgical neurocognitive impairment rate of up to 9% for ISS. (LLM, conf=0.82)
Experimental — accuracy not yet measuredEnvironmental influences, including in utero mechanical constraints, maternal health conditions, medication exposure, and other prenatal risk factors, have been proposed as contributing factors particularly in non-syndromic cases.
“Environmental influences have also been proposed as contributing factors, particularly in non-syndromic cases. These include in utero mechanical constraints, maternal health conditions, medication exposure, and other prenatal risk factors [1, 2, 11, 22–25].”
From Genetics versus environment in the pathophysiology of sagittal synostosis (2026) · cites this paper as [11]
The CRANIOSYNOSTOSIS section (p. 1) identifies intrauterine constraint, maternal smoking, prenatal teratogen exposure, and antiepileptic drugs as possible predisposing factors. It discusses these factors for craniosynostosis generally, however, and does not say they contribute particularly to non-syndromic cases. (LLM, conf=0.86)
Experimental — accuracy not yet measuredISS occurs more frequently in boys than in girls.
“It occurs more frequently in boys than in girls.”
From Isolated Sagittal Craniosynostosis: A Comprehensive Review (2024) · cites this paper as [3]
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References 61 · Cited by 213
Research works printed but not linked: 1
- [13][13] Opperman LA. Cranial sutures as intramembranous bone growth sites. Dev Dyn 2000;219(4):472-85. https: //doi.org/10.1002/ 1097-0177 (2000) 9999:9999 <:AID-DVDY1073 > 3.0.CO;2-F. -
- Authors
- Kajdic, N., Spazzapan, P., & Velnar, T.
- Affiliations
- Ljubljana University Medical Centre (SI)
- ORCID
- Spazzapan, Peter
- Venue
- Bosnian Journal of Basic Medical Sciences
- Published
- 2017-06-17
- Volume
- 18
- Issue
- 2
- Pages
- 110-116
- ISSN
- 1512-8601 (ISSN-L)
- 1840-4812
- PMC
- PMC5988529
- OpenAlex
- W2626233920
- Cited by
- 213
- References
- 61
- Citations by year
- 2026: 14 · 2025: 44 · 2024: 58 · 2023: 29 · 2022: 30 · 2021: 26 · 2020: 6 · 2019: 3 · 2018: 1
- FWCI
- 1.82
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by
- Locations
- Bosnian Journal of Basic Medical Sciences (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Bosnian Journal of Basic Medical Sciences (submittedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- 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
- craniosynostosis, cranial sutures, premature suture closure, craniofacial anomalies, brain development, developmental delay, craniosynostosis syndromes, craniosynostosis surgery, postoperative care, follow-up
- MeSH terms
- Brain, Developmental Disabilities, Cranial Sutures, Craniosynostoses, Humans, Infant, Infant, Newborn, Magnetic Resonance Imaging, Skull, Prevalence
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