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, 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 [24]
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Management of Facial Asymmetry
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Zhang, R. S., Wes, A. M., Naran, S., Hoppe, I. C., Sun, J. J., Mazzaferro, D. M., Bartlett, S. P. P., & Taylor, J. A. (2018) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000004230 · Cited by 33 · see citing works
“Nonsyndromic patients with multisutural involvement are more prone to developing increased ICP, and the frequency doubles after 1 year of age.(4,12) Debate regarding optimal timing of surgery continues, although delayed intervention is associated with increased morbidity.”
Study, T. N. B. D. P., Justice, C. M., Cuellar, A., Bala, K., Sabourin, J., Cunningham, M. L., Crawford, K. L., Phipps, J. M., Zhou, Y., Cilliers, D. D., et al. (2020) · Human Genetics · doi:10.1007/s00439-020-02157-z · Cited by 32 · see citing works
Self-citationSurgical Correction of Nonsyndromic Sagittal Craniosynostosis
Simpson, A. M., Wong, A. L., & Bezuhly, M. (2016) · Annals of Plastic Surgery · doi:10.1097/sap.0000000000000713 · Cited by 33 · see citing works
Management of Craniosynostosis at an Advanced Age
Iyengar, R. J., Klinge, P. M., Chen, W. S., Boxerman, J. L., Sullivan, S. R., & Taylor, H. O. B. (2016) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000002725 · Cited by 32 · see citing works
“%) underwent ICP measurements either at the time of surgery (epidural monitoring) or prior to surgery (intraparenchymal monitoring), and 3 of these patients (30% of total, and 75% of those measured) had documented elevations in ICP.”
BackgroundInfluentialHaredy, M. M., Zuccoli, G., Tamber, M. S., Davis, A. A., Nischal, K. K., & Goldstein, J. A. (2018) · Child s Nervous System · doi:10.1007/s00381-018-3728-7 · Cited by 32 · see citing works
“Intracranial hypertension has been estimated to affect 15 to 20% of patients with single suture craniosynostosis [2, 3] and 50 to 75% of those with multisutural craniosynostosis”
BackgroundSwanson, J. W., Xu, W., Ying, G., Pan, W., Lang, S., Heuer, G. G., Bartlett, S. P. P., & Taylor, J. A. (2019) · Child s Nervous System · doi:10.1007/s00381-019-04448-x · Cited by 30 · see citing works
Elevated Intracranial Pressure in Patients with Craniosynostosis by Optical Coherence Tomography
Kalmar, C. L., Humphries, L. S., McGeehan, B., Ying, G., Heuer, G. G., Liu, G. T., Avery, R. A., Bartlett, S. P. P., Taylor, J. A., Lang, S., et al. (2022) · Plastic & Reconstructive Surgery · doi:10.1097/prs.0000000000008821 · Cited by 30 · see citing works
Flanagan, M. F. (2015) · Neurology Research International · doi:10.1155/2015/794829 · Cited by 29 · see citing works
Endoscopic-assisted osteotomies for the treatment of craniosynostosis
Hinojosa, J., Esparza, J., & Muñoz, M. J. (2007) · Child s Nervous System · doi:10.1007/s00381-007-0473-8 · Cited by 30 · see citing works
“Concern of parents and the fact understood by pediatricians that some of these cases may be followed by increased intracranial pressure when left uncorrected [24, 25] have led to a rising awareness in the follow-up and treatment of these children.”
BackgroundCraniosynostosis and hydrocephalus: relevance and treatment modalities
Frassanito, P., Palombi, D., & Tamburrini, G. (2021) · Child s Nervous System · doi:10.1007/s00381-021-05158-z · Cited by 29 · see citing works
“Therefore, careful monitoring of intracranial pressure and ventricular size in the pre- and postoperative period is a diagnostic mainstay [29].”
BackgroundSelf-citationCurrent Controversies in Metopic Suture Craniosynostosis
Jaskolka, M. S. (2017) · Oral and Maxillofacial Surgery Clinics of North America · doi:10.1016/j.coms.2017.07.003 · Cited by 29 · see citing works
van der Meulen, J., van der Vlugt, J., Okkerse, J. M. E., & Albert Hofman, D. (2008) · Journal of Neurosurgery Pediatrics · doi:10.3171/ped-08/01/025 · Cited by 29 · see citing works
Shruthi, N. M., & Gulati, S. (2022) · Journal of Pediatric Neurosciences · doi:10.4103/jpn.jpn_25_22 · Cited by 27 · see citing works
Kunz, M., Lehner, M., Heger, A., Armbruster, L., Weigand, H., Mast, G., & Peraud, A. (2014) · Child's Nervous System · doi:10.1007/s00381-013-2340-0 · Cited by 30 · see citing works
Langvatn, E. A., Frič, R., Due‐Tønnessen, B. J., & Eide, P. K. (2019) · Journal of Neurosurgery Pediatrics · doi:10.3171/2019.2.peds18767 · Cited by 25 · see citing works
Rattani, A., Riordan, C. P., Meara, J. G., & Proctor, M. R. (2020) · Journal of Neurosurgery Pediatrics · doi:10.3171/2020.2.peds19522 · Cited by 26 · see citing works
Park, D. H., Chung, J., & Yoon, S. H. (2010) · Pediatric Neurosurgery · doi:10.1159/000319005 · Cited by 25 · see citing works
Craniofacial reconstruction as a treatment for elevated intracranial pressure
Baird, L. C., Gonda, D. D., Cohen, S. R., Evers, L. H., LeFloch, N., Levy, M. L., & Meltzer, H. S. (2011) · Child s Nervous System · doi:10.1007/s00381-011-1615-6 · Cited by 25 · see citing works
“Others have argued that this scale is not relevant in the setting of craniosynostosis [24].”
BackgroundInfluentialNormocephalic Pancraniosynostosis Resulting in Late Presentation of Elevated Intracranial Pressures
Foo, R., Whitáker, L. A., & Bartlett, S. P. P. (2010) · Plastic & Reconstructive Surgery · doi:10.1097/prs.0b013e3181d62b48 · Cited by 24 · see citing works
Cranial Fossa Volume and Morphology Development in Apert Syndrome
Lu, X., Forte, A. J., Wilson, A. T., Steinbacher, D. M., Alperovich, M. A., Alonso, N., & Persing, J. A. (2020) · Plastic & Reconstructive Surgery · doi:10.1097/prs.0000000000006679 · Cited by 22 · see citing works
Frame-based cranial reconstruction
Hochfeld, M. S., Lamecker, H., Thomale, U., Schulz, M., Stefan Zachow, A., & Haberl, H. (2014) · Journal of Neurosurgery Pediatrics · doi:10.3171/2013.11.peds1369 · Cited by 22 · see citing works
Airway anomalies in patients with craniosynostosis
Mathews, F., Shaffer, A. D., Georg, M. W., Ford, M. D., Goldstein, J. A., Jabbour, N., & Simons, J. P. (2018) · The Laryngoscope · doi:10.1002/lary.27589 · Cited by 21 · see citing works
Trigonocephaly: Long-term results after surgical correction of metopic suture synostosis
Wójcicki, P., & Prudel, B. (2019) · Advances in Clinical and Experimental Medicine · doi:10.17219/acem/90763 · Cited by 21 · see citing works
Posterior calvarial distraction for complex craniosynostosis and cerebellar tonsillar herniation
Lo, W. B., Thant, K. Z., Kaderbhai, J., White, N., Nishikawa, H., Dover, M. S., Evans, M., & Rodrigues, D. (2020) · Journal of Neurosurgery Pediatrics · doi:10.3171/2020.4.peds19742 · Cited by 21 · see citing works
Kunz, A., & Iliadis, C. (2007) · Child s Nervous System · doi:10.1007/s00381-007-0468-5 · Cited by 21 · see citing works
“For example, in sagittal synostosis, there is the well-known compression of the superior sagittal sinus [29], and in craniosynostosis-related syndromes or the non-syndromic multiple suture synostosis, a stenotic or no-flow of the sigmoid-jugular sinus complex is seen, again producing this hypertensive state [30].”
BackgroundSpruijt, B., Mathijssen, I., Bredero-Boelhouwer, H., Cherian, P., Corel, L., van Veelen, M. V., Hayward, R., Tasker, R., & Joosten, K. (2016) · Plastic and Reconstructive Surgery · doi:10.1097/prs.0000000000002741 · Cited by 22 · see citing works
Rufai, S. R., Jeelani, O., & McLean, R. J. (2020) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000006771 · Cited by 20 · see citing works
“Direct intraparenchymal measurement represents the gold standard, but involves hospital admission and carries risks of infection, bleeding, and device failure.(6) Noninvasive methods such as ophthalmoscopy,(9) transorbital ultrasound,(10) and radiography(11) deliver inadequate sensitivity to be used for screening.”
BackgroundThe Risk of Psychopathology in Children with Craniosynostosis
van der Vlugt, J., van der Meulen, J., Creemers, H., Willemse, S. P., Lequin, M. L., & Okkerse, J. (2009) · Plastic and Reconstructive Surgery · doi:10.1097/prs.0b013e3181bcf2dc · Cited by 16 · see citing works
Kadakia, S., Helman, S. N., Healy, N. J., Saman, M., & Wood‐Smith, D. (2014) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000001121 · Cited by 20 · see citing works
“Conclusions: This study collates surgical management data from CS and other related syndromes as a means of establishing a cohesive approach to the surgical treatment of CS. Key Words: Carpenter syndrome, craniofacial surgery, acrocephalopolysyndactyly, craniosynostosis, craniofacial reconstruction, syndactyly/brachyd…”
BackgroundInfluentialMa, R., Rowland, D., Judge, A., Calisto, A., Jayamohan, J., Johnson, D., Richards, P., Magdum, S. A., & Wall, S. A. (2018) · Journal of Neurosurgery Pediatrics · doi:10.3171/2017.9.peds17360 · Cited by 20 · see citing works
Pellicer, E., Siebold, B. S., Birgfeld, C. B., & Gallagher, E. R. (2018) · Plastic & Reconstructive Surgery · doi:10.1097/prs.0000000000004180 · Cited by 20 · see citing works
Brookes, C. D., Golden, B. A., & Turvey, T. A. (2014) · Atlas of the Oral and Maxillofacial Surgery Clinics · doi:10.1016/j.cxom.2014.04.001 · Cited by 20 · see citing works
“Venous congestion, hydrocephalus, and upper airway obstruction likely all contribute to intracranial hypertension.(16,59)”
BackgroundKuta, V., Curry, L. C., McNeely, D., Walling, S. A., Chorney, J. M. L., & Bezuhly, M. (2020) · BMJ Open · doi:10.1136/bmjopen-2019-033403 · Cited by 19 · see citing works
Sader, N., Mehta, V., Hart, S., Bliss, L., Moore, H., DaSilva, M., Rashid, R., & Riva-Cambrin, J. (2021) · Journal of Neurosurgery Pediatrics · doi:10.3171/2021.5.peds2136 · Cited by 20 · see citing works
Electrophysiological and fundoscopic detection of intracranial hypertension in craniosynostosis
Rufai, S. R., Marmoy, O. R., Thompson, D., van de Lande, L. S., Breakey, R. W. F., Bunce, C., Panteli, V., Schwiebert, K., Mohamed, S., Proudlock, F. A., et al. (2022) · Eye · doi:10.1038/s41433-021-01839-w · Cited by 18 · see citing works
“IH occurs in 30–40% [4, 5] of the syndromic cases, most commonly in patients diagnosed with Apert (71%) [6], Crouzon (61%) [7] and Pfeiffer (60%) [8] syndromes.”
BackgroundCognitive Development in Single-Suture Craniosynostosis – A Systematic Review
Olsson, K., Engman, J. H., Nowinski, D. J., Ramklint, M., & Frick, M. A. (2023) · Developmental Neuropsychology · doi:10.1080/87565641.2023.2225662 · Cited by 18 · see citing works
“There is a well-known risk for raised intracranial pressure in some forms of CS, particularly in certain genetic syndromes such as Crouzon and Apert (Tamburrini et al., 2005).”
BackgroundPitfalls for the Pediatrician: Positional Molding or Craniosynostosis?
Komotar, R. J., Zacharia, B. E., Ellis, J. A., & Feldstein, N. A. (2006) · Pediatric Annals · doi:10.3928/0090-4481-20060501-08 · Cited by 18 · see citing works
Saethre–Chotzen syndrome: long‐term outcome of a syndrome‐specific management protocol
den Ottelander, B. K., van Veelen, M. C., de Goederen, R., van de Beeten, S. D. C., Dremmen, M. H. G., Loudon, S. E., Versnel, S. L., van den Ouweland, A. M., van Dooren, M. F., Joosten, K. F. M., et al. (2020) · Developmental Medicine & Child Neurology · doi:10.1111/dmcn.14670 · Cited by 17 · see citing works
“The latest available test results were evaluated, since papilledema may have a long-term effect on vision.(13) Confounding caused by strabismus and/or amblyopia was minimalized by using the eye with the best visual acuity.”
MethodRufai, S. R., Bowman, R. J. C., Bunce, C., Panteli, V., McLean, R. J., Teli, S., Gottlob, I., Thomas, M. G., Jeelani, O., & Proudlock, F. A. (2021) · Translational Vision Science & Technology · doi:10.1167/tvst.10.8.24 · Cited by 16 · see citing works
Fronto-Orbital Advancement for Metopic and Unilateral Coronal Craniosynostoses
Massenburg, B. B., Tolley, P. D., Lee, A., & Susarla, S. M. (2022) · Oral and Maxillofacial Surgery Clinics of North America · doi:10.1016/j.coms.2022.01.001 · Cited by 16 · see citing works
The genetic overlap between osteoporosis and craniosynostosis
Kague, É., Medina‐Gómez, C., Boyadjiev, S. A., & Rivadeneira, F. (2022) · Frontiers in Endocrinology · doi:10.3389/fendo.2022.1020821 · Cited by 16 · see citing works
“Thus, affected children may experience ongoing medical complications, such as increased intracranial pressure (66, 67), vision problems (optic neuropathy secondary to elevated intracranial pressure, strabismus, refracture errors, exposure keratopathy from exorbitism) (68), and learning disabilities (69–71).”
BackgroundMarupudi, N. I., Sood, S., Rozzelle, A. A., & Ham, S. D. (2016) · Journal of Neurosurgery Pediatrics · doi:10.3171/2016.1.peds15569 · Cited by 16 · see citing works
Telemetric Intracranial Pressure Monitoring in Syndromic Craniosynostosis
Beez, T., O’Kane, R., Piper, I., Koppel, D. A., & Sangra, M. (2016) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000002556 · Cited by 16 · see citing works
“This risk is highest in patients of syndromic craniosynostosis involving multiple sutures, with 90% of patients having raised ICP.2 However, children with single suture craniosynostosis can also be affected and the incidence of raised Copyright © 2016 Mutaz B. Habal, MD. Unautho # 2016 Mutaz B. Habal, MD ICP in this s…”
MethodInfluentialThe role of ICP overnight monitoring (ONM) in children with suspected craniostenosis
Zipfel, J., Jager, B., Collmann, H., Czosnyka, Z. H., Schuhmann, M. U., & Schweitzer, T. (2019) · Child s Nervous System · doi:10.1007/s00381-019-04288-9 · Cited by 16 · see citing works
Rufai, S. R., Hisaund, M., Jeelani, O., & McLean, R. J. (2021) · BMJ Open · doi:10.1136/bmjopen-2020-046935 · Cited by 15 · see citing works
Rojas-Peña, M. L., Olivares‐Navarrete, R., Hyzy, S. L., Arafat, D., Schwartz, Z., Boyan, B. D., Williams, J. K., & Gibson, G. C. (2014) · Journal of Genomics · doi:10.7150/jgen.8833 · Cited by 15 · see citing works
“Atypical growth of the cranium could be related to aberrant mechanical pressure on the developing skull in utero, compromising cerebral blood flow (11).”
BackgroundDifferential spatial regulation of BMP molecules is associated with single-suture craniosynostosis
Whitton, A., Hyzy, S. L., Britt, C., Williams, J. K., Boyan, B. D., & Olivares‐Navarrete, R. (2016) · Journal of Neurosurgery Pediatrics · doi:10.3171/2015.12.peds15414 · Cited by 15 · see citing works
Normalization of Speech Processing After Whole-Vault Cranioplasty in Sagittal Synostosis
Chuang, C., Rolison, M. J., Yang, J. F., Brooks, E. D., Hashim, P. W., Travieso, R., Terner, J. S., Steinbacher, D. M., Landi, N., Stavropoulos, K. K. M., et al. (2018) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000004474 · Cited by 15 · see citing works
Yokote, A., Aihara, Y., Eguchi, S., & Okada, Y. (2013) · Child s Nervous System · doi:10.1007/s00381-013-2049-0 · Cited by 15 · see citing works
Letter to the Editor: Raised intracranial pressure and nonsyndromic sagittal craniosynostosis
Hayward, R. D., Britto, J. A., Dunaway, D. J., Evans, R. D., Jeelani, O., & Thompson, D. N. P. (2015) · Journal of Neurosurgery Pediatrics · doi:10.3171/2014.11.peds14625 · Cited by 15 · see citing works
Haredy, M. M., Liasis, A. C., Fu, V. L. N., Davis, A. A., Pollack, I. F., Losee, J. E., Saied, S. M. A., Nischal, K. K., & Goldstein, J. A. (2019) · Plastic & Reconstructive Surgery · doi:10.1097/prs.0000000000005935 · Cited by 14 · see citing works
van Nunen, D. P. F., Stubenitsky, B. M., Woerdeman, P. A., Han, K. S., Breugem, C. C., van der Molen, A. B. M., & de Graaff, J. C. (2016) · Journal of Craniofacial Surgery · doi:10.1097/scs.0000000000003072 · Cited by 14 · see citing works
- 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
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Craniofacial Disorders and Treatments
- Health Sciences › Medicine › Neurology › Traumatic Brain Injury and Neurovascular Disturbances
- Health Sciences › Medicine › 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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