# Developments in diagnostic and surgical techniques in children with sagittal suture craniosynostosis: a systematic review spanning the last 30 years

**Authors:** Julia Hermann, Christa K. Raak, Thomas Ostermann, Wolfram Scharbrodt

PMC · DOI: 10.1186/s13023-025-03978-9 · Orphanet Journal of Rare Diseases · 2025-08-17

## TL;DR

This systematic review examines how diagnostic and surgical approaches for sagittal suture craniosynostosis in children have evolved over the last 30 years.

## Contribution

The study provides a comprehensive overview of advancements in diagnosis and surgery for sagittal suture craniosynostosis over three decades.

## Key findings

- A total of 57 studies were included, mainly from the USA and the Netherlands.
- Evolution in surgical and diagnostic strategies is evident, with techniques tailored to patient-specific factors.
- Timely surgical intervention remains crucial for correcting cranial deformities and preventing complications.

## Abstract

Sagittal suture craniosynostosis is the most usual subtype of craniosynostosis which results from premature fusion of the sagittal suture. It leads to an elongated skull shape known as scaphocephaly. This condition necessitates timely surgical intervention to correct cranial deformities and prevent the associated complications. Over the past three decades, the use of advanced diagnostic methods and the refinement of surgical techniques have improved the understanding of this rare disease.

To analyse the development of surgical interventions and diagnostic methods in children suffering from sagittal suture craniosynostosis over the last three decades.

A comprehensive literature search was conducted in electronic databases Pubmed and online university libraries to identify articles, studies and case reports reporting on surgical interventions and diagnostic procedures for sagittal suture craniosynostosis the period from 1994 to 2024. Clinical studies, case reports, systematic reviews and meta-analyses were assessed and analysed according to inclusion and exclusion criteria. Prisma guidelines for systematic reviews were considered.

A systematic literature search identified 301, and a hand search identified 12 articles, of which a total of 57 met the inclusion criteria after careful evaluation. The reviewed studies, predominantly originated from the USA and the Netherlands and provided data on diagnostic methods, surgical techniques, patient-specific characteristics, and outcomes for non-syndromic sagittal craniosynostosis.

The evolutionary change in surgical and diagnostic strategies for sagittal suture craniosynostosis reflects the ongoing efforts of the medical community to achieve optimal outcomes for affected children. The selection of the appropriate technique remains an individualized decision, considering age, severity of craniosynostosis and other patient-specific factors.

## Full-text entities

- **Genes:** FGF13 (fibroblast growth factor 13) [NCBI Gene 2258] {aka DEE90, FGF-13, FGF2, FHF-2, FHF2, LINC00889}, TWIST1 (twist family bHLH transcription factor 1) [NCBI Gene 7291] {aka ACS3, BPES2, BPES3, CRS, CRS1, CSO}, SAT1 (spermidine/spermine N1-acetyltransferase 1) [NCBI Gene 6303] {aka DC21, KFSD, KFSDX, SAT, SSAT, SSAT-1}, FGFR3 (fibroblast growth factor receptor 3) [NCBI Gene 2261] {aka ACH, CD333, CEK2, HSFGFR3EX, JTK4}
- **Diseases:** pressure (MESH:D003668), increased (MESH:D000067251), frontal bossing (MESH:D020233), Apert, (MESH:D000168), syndromic genetic diseases (MESH:D030342), hyperthyroidism (MESH:D006980), leukaemia (MESH:D015458), frontal and occipital bossing (MESH:D006259), chromosomal abnormalities (MESH:D002869), bone defects (MESH:D001847), CSO (MESH:D003398), uterine deformities (MESH:D014591), hydrocephalus (MESH:D006849), smoking (MESH:D015208), headaches (MESH:D006261), synostosis (MESH:D013580), intracranial anomaly (MESH:D001932), deformity (MESH:D009140), rigidity (MESH:D009127), syndromic diseases (MESH:D004194), cognitive impairments (MESH:D003072), sickle cell anaemia (MESH:D000755), congenital disorder (MESH:D009358), WS (MESH:D018980), abnormal skull development (MESH:D002658), Blood loss (MESH:D016063), hypophosphatemia (MESH:D017674), restricted (MESH:D002313), blood (MESH:D006402), suture abnormalities (MESH:C563296), polycythaemia vera (MESH:D011087), BPD (MESH:D015875), skull deformities (MESH:D012888), trauma (MESH:D014947), cranial anomalies (MESH:C565666), CUS (MESH:D003389), thalassemia (MESH:D013789), cancer (MESH:D009369), cerebrospinal fluid leaks (MESH:D065634), cerebral malformations (MESH:D020786), premature ossification (MESH:C562735), microcephaly (MESH:D008831), single sutural synostosis (MESH:D012640), cerebral atrophy (MESH:D001284), brain abnormalities (MESH:D001927), infections (MESH:D007239), rickets (MESH:D012279), mucopolysaccharidoses (MESH:D009083), Disorders of bone metabolism (MESH:D001851), subarachnoid cysts (MESH:D013345), positional plagiocephalus (MESH:D000377)
- **Chemicals:** CVR (-), valproate (MESH:D014635), titanium (MESH:D014025)
- **Species:** Homo sapiens (human, species) [taxon 9606]
- **Mutations:** P250R

## Full text

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## Figures

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## References

1 references — full list in the complete paper: https://tomesphere.com/paper/PMC12358073/full.md

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Source: https://tomesphere.com/paper/PMC12358073