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Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review
Abstract
Introduction One third of patients with craniofacial dysostosis syndromes and about 15-20% of children with single suture craniostenosis have a documented increase in intracranial pressure (ICP). The early detection of intracranial hypertension is important in order to reduce the risks for brain development and visual function. However, in children with craniosynostosis, the clinical manifestations of abnormally increased ICP are difficult to detect, as the majority of patients may have neither warning signs nor symptoms for a long period of time.Review Moreover, data from the literature suggest that neither fundoscopic nor radiological findings are necessarily related to intracranial pressure recordings in this type of pathology. In this context prolonged ICP monitoring seems actually to be the most valuable diagnostic tool. Extradural, subdural, and intraparenchymal devices have been used, and different softwares have been employed for PC storage and analysis of the data obtained. Most recent series consider mean ICP combined with plateaux waves as the most sensitive indicators in the final evaluation of the results.Conclusions The analysis of the literature demonstrates a significant difference in the prevalence of raised ICP preoperatively between non-syndromic and syndromic patients. Among the non-syndromic children, a direct relation between the number of sutures involved and raised ICP is documented. The analysis of plateaux waves seems to be particularly important in children with borderline mean ICP values. Persistent postoperative raised ICP has been described in 6-15% of patients with craniofacial dysostosis. It must be related to the multifactorial etiology of increased ICP in these patients, which includes cerebral venous congestion, upper airway obstruction, and hydrocephalus.
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“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 [24]
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“Among these, the raised ICP is a major complication (5, 6).”
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“Approximately 1 in 5 patients with single suture scaphocephaly (SSS) will develop intracranial hypertension later in childhood [2].”
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Dibbs, R. P., Beh, H. Z., Donoho, D. A., Davies, L. W., Ferry, A. M., Bauer, D. F., & Buchanan, E. P. (2021) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000007888 · Cited by 3
“The most accurate method for confirming elevated ICP is via intraparenchymal pressure monitoring; however, this method is invasive and given the risks involved, not useful as a screening tool.(19) Anomalies in intracranial vessels have also been associated with syndromic craniosynostosis resulting in venous hypertensi…”
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den Ottelander, B. K., van de Beeten, S. D. C., Yang, S., van Veelen, M. C., Tasker, R. C., Loudon, S. E., & Mathijssen, I. M. J. (2023) · Plastic & Reconstructive Surgery · doi:10.1097/prs.0000000000011177 · Cited by 3
“4”
BackgroundPrevalence and treatment outcomes of hydrocephalus among children with craniofacial syndromes
Jönsson, S., Nilsson, D., Tarnow, P., Maltese, G., Bhatti-Søfteland, M., Kölby, L., & Hallén, T. (2025) · Journal of Plastic Surgery and Hand Surgery · doi:10.2340/jphs.v60.42957 · Cited by 3
Current Understanding of Crouzon Syndrome Pathophysiology and New Therapeutic Approaches
Tiberio, F., Polito, L., Salvati, M., Pietro, L. D., Massimi, L., Parolini, O., Tamburrini, G., & Lattanzi, W. (2025) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000011376 · Cited by 4
Self-citationSevere Deformational Plagiocephaly
Rogers, G. F. (2010) · Journal of Craniofacial Surgery · doi:10.1097/scs.0b013e3181fb8919 · Cited by 3
“Pressure elevation in patients with craniosynostosis has been found to occur in up to 20% of single sutural fusions and between 48% and 75% of patients with multiple fusions.(4) Recent studies have failed to establish that intracranial expansion for single-suture craniosynostosis results in improved cognitive developm…”
Yamada, S., Park, Y.-S., Nakagawa, I., Nishimura, F., Motoyama, Y., & Nakase, H. (2019) · Interdisciplinary Neurosurgery · doi:10.1016/j.inat.2019.100603 · Cited by 3
Types of Craniosynostosis and their Etiology, Pathophysiology and Epidemiology
de León, F. C.-P., & Franco-Jiménez, J. A. (2021) · Springer eBooks · doi:10.1007/978-3-030-72338-5_14 · Cited by 3
Khormi, Y. H., Chiu, M., Tyndall, R. G., Mortenson, P., Smith, D. J., & Steinbok, P. (2019) · Child s Nervous System · doi:10.1007/s00381-019-04400-z · Cited by 3
Mandera, M. (2017) · Springer eBooks · doi:10.1007/978-3-319-31512-6_64-1 · Cited by 3
The clinical picture of nonsyndromic craniosynostosis in children
Sufianov, A. A., Gaibov, S. S., Sufianov, R. A., & Иванова, Н. Е. (2015) · S S Korsakov Journal of Neurology and Psychiatry · doi:10.17116/jnevro20151158118-22 · Cited by 3
de Goederen, R., Joosten, K. F. M., den Ottelander, B. K., van der Oest, M. J. W., Bröker-Schenk, E. M. M., van Veelen, M. C., Wolvius, E. B., Versnel, S. L., Tasker, R. C., & Mathijssen, I. M. J. (2019) · Plastic & Reconstructive Surgery Global Open · doi:10.1097/gox.0000000000002419 · Cited by 4
Persing, J. A., Jane, J. A., & Piatt, J. H. (2011) · Elsevier eBooks · doi:10.1016/b978-1-4160-5316-3.00185-4 · Cited by 3
Crompton, J. L., & Black, J. (2013) · Elsevier eBooks · doi:10.1016/b978-0-7020-4691-9.00028-5 · Cited by 3
Multisuture craniosynostosis: a case report of unusual presentation of chromosome 14q32 deletion
Chaisrisawadisuk, S., Vatanavicharn, N., Khampalikit, I., & Moore, M. H. (2022) · Child s Nervous System · doi:10.1007/s00381-022-05788-x · Cited by 2
Anesthetic Considerations for Pediatric Patients with Craniofacial Anomalies
Tran, L. T., Hoffmann, P., Parry, S., Davis, P. J., & Soliman, D. E. (2025) · Clinics in Plastic Surgery · doi:10.1016/j.cps.2024.08.010 · Cited by 2
Severity of Unilateral Coronal Synostosis Linked to Intracranial Volume
Kronig, S. A. J., Kronig, O. D. M., & van Adrichem, L. N. A. (2021) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000007842 · Cited by 2
“For example, only 25% of patients with raised ICP have evidence of papilledema on fundoscopy.(21,22,27) Therefore, the percentage of UCS patients with bilateral papilledema and raised ICP in the present study is possibly an underestimation of the problem.”
BackgroundChaij, J. M., Liu, J., French, B. M., Mirsky, D. M., Miles, R. C., Linguraru, M. G., Nguyen, P. D., Alexander, A., Görg, C., & Porras, A. R. (2025) · Plastic & Reconstructive Surgery Global Open · doi:10.1097/gox.0000000000006618 · Cited by 2
“7,12,13,23,24 In patients with syndromic CS (SCS), prevalence of IIP is reported to range from 38% 25 to 83%.”
The role of ICP monitoring in paediatric IIH
Srinivasan, H. L., Richetta, C., Manoim, N., Zipori, A. B., Shiran, S. I., Meirson, H., Roth, J., & Constantini, S. (2020) · Child s Nervous System · doi:10.1007/s00381-020-04618-2 · Cited by 2
Plastic and Reconstructive Surgery
Engelhardt, T., Crawford, M. W., & Lerman, J. (2009) · Elsevier eBooks · doi:10.1016/b978-141603134-5.50037-8 · Cited by 2
Hinojosa, J. (2017) · Springer eBooks · doi:10.1007/978-3-319-31512-6_63-1 · Cited by 2
Recognising a child with isolated craniosynostosis
Ching, R. C., Palmer, S. C., Jones, K. L., & Johnson, D. (2023) · BMJ · doi:10.1136/bmj-2022-073906 · Cited by 2
Endoscopic Treatment of Arachnoid Cysts
Cinalli, G., Spennato, P., Martino, G. D., Mirone, G., & Cascone, D. (2015) · Springer eBooks · doi:10.1007/978-3-319-21533-4_16 · Cited by 2
Intracranial Pressure Monitoring
Oertel, J., & Antes, S. (2018) · Springer eBooks · doi:10.1007/978-3-319-31889-9_71-1 · Cited by 2
Classification of craniofacial anomalies
Jagadeesan, J., & Nishikawa, H. (2021) · Oxford University Press eBooks · doi:10.1093/med/9780199682874.003.0180 · Cited by 2
Intracranial Pressure Monitoring
Oertel, J., & Antes, S. (2019) · Springer eBooks · doi:10.1007/978-3-319-27250-4_71 · Cited by 2
Genetics versus environment in the pathophysiology of sagittal synostosis
De Gregorio, V., Tosi, D. D., Vita, A., Salvati, M., Massimi, L., Tamburrini, G., Tiberio, F., & Lattanzi, W. (2026) · Child's Nervous System · doi:10.1007/s00381-026-07396-5 · Cited by 2
Self-citationLiang, Q., Chen, X., Yang, B., Song, Y., Sun, S.-Q., Jing, J.-J., Vito, A. D., Hall, W. A., Ganau, M., & Bao, N. (2023) · Translational Pediatrics · doi:10.21037/tp-23-495 · Cited by 1
“Of note, single-suture craniosynostosis may also result in a significant volumetric reduction of the skull cavity on the affected side with consequent functional impact due to potential increases in intracranial pressure (ICP) in about 15–20% of children (2).”
BackgroundNeuroimaging in the Craniosynostoses
Blasér, S., & Raybaud, C. (2024) · Springer eBooks · doi:10.1007/978-3-031-69386-1_21 · Cited by 1
Le, D. M., Hoffman, G. R., Le, T., Elkhill, C., Porras, A. R., French, B. M., Yu, J. W., Nguyen, P. D., Mathes, D. W., & Khechoyan, D. Y. (2026) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000012342 · Cited by 1
OPEN RECONSTRUCTIVE METHOD OF SURGICAL TREATMENT OF NONSYNDROMIC CRANIOSYNOSTOSIS IN CHILDREN
Летягин, Г. В., Kim, S. A., Danilin, V. E., Sysoeva, A. A., & Амелин, М. Н. (2018) · Russian journal of neurosurgery · doi:10.63769/1683-3295-2017-0-4-50-58 · Cited by 1
Khan, F. Z., Abdellah, S., Blum, M. J., Craig, J. N., Chin, M. G., Carbullido, M. K., Gupta, S., Patel, M., Garland, C., & Cho, D. (2026) · Seminars in Pediatric Neurology · doi:10.1016/j.spen.2026.101324 · Cited by 1
Craneosinostosis: diagnóstico intraútero y evolución posnatal
Padilla-Iserte, P., Folch, B. F., Pallarés, I. J., Portero, R. G., & Marín, A. P. (2014) · Clínica e Investigación en Ginecología y Obstetricia · doi:10.1016/j.gine.2013.12.010 · Cited by 1
Metadata
- Authors
- Tamburrini, G., Caldarelli, M., Massimi, L., Santini, P., & Di Rocco, C.
- Affiliations
- Agostino Gemelli University Polyclinic (IT)
- Corresponding author
- Tamburrini, G.
- Venue
- Child's Nervous System
- Published
- 2005-05-03
- Volume
- 21
- Issue
- 10
- Pages
- 913-921
- ISSN
- 0256-7040 (ISSN-L)
- 1433-0350
- OpenAlex
- W2044133352
- Cited by
- 261
- References
- 36
- Citations by year
- 2026: 14 · 2025: 8 · 2024: 19 · 2023: 11 · 2022: 23 · 2021: 21 · 2020: 21 · 2019: 17 · 2018: 19 · 2017: 12 · 2016: 16 · 2015: 12 · 2014: 13 · 2013: 6 · 2012: 12
- FWCI
- 2.70
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Child s Nervous System (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Craniofacial Disorders and Treatments
- Health Sciences › Medicine › Clinical Neurology › Traumatic Brain Injury and Neurovascular Disturbances
- Health Sciences › Medicine › Clinical Neurology › Cerebral Venous Sinus Thrombosis
- Keywords
- intracranial pressure monitoring, craniosynostosis, single-suture craniosynostosis, intracranial hypertension, cerebral venous congestion, upper airway obstruction, hydrocephalus, children
- MeSH terms
- Cerebrospinal Fluid Shunts, Child, Child, Preschool, Cranial Sutures, Craniosynostoses, Craniotomy, Humans, Intracranial Pressure, Monitoring, Physiologic
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