Intracranial Volume In Patients Research Articles

A Review on Variability in Treatment Protocols With Posterior Cranial Vault Distraction.

Prior studies have confirmed the ability of posterior cranial vault distraction osteogenesis (PVDO) to expand the intracranial volume in patients with craniosynostosis. To date, there is scant literature on the optimal distraction protocol for PVDO. The authors sought to review the literature and define a common protocol for posterior cranial vault distraction. The authors performed a systematic review for published PVDO protocols. The data collected from these studies included age at the time of PVDO, number of distraction devices placed, time for latency, rate and rhythm of distraction, distraction length, time for consolidation, and surgical outcomes. A total of 286 patients were identified within 24 studies from 2011 to 2019. The mean age of patients identified was 25.34 months. After application of distractors, latency period ranged between 1 and 7 days, with most patients undergoing 5 to 7 days of latency. Once distraction was begun, the majority of patients (77.4%) underwent 1 mm of distraction daily. Total lengths of distraction ranged between 13 and 35 mm, with the largest cohort of patients undergoing 26 to 30 mm of total distraction. A total of 60 complications were reported for a total of 212 patients, yielding an overall complication rate of 28.3%. Although there is variability in reported PVDO protocols, the majority are similar to distraction osteogenesis protocols described for long bone sites. Increased patient age correlates with selection of a greater latency period and total distraction length, while frequency of complications is also increased.

Comparison of Intracranial Volumes in Patients With Isolated Metopic Ridge, Metopic Craniosynostosis, and Normal Children

Comparison of Intracranial Volumes in Patients With Isolated Metopic Ridge, Metopic Craniosynostosis, and Normal Children

Can Head Circumference Be Used as a Proxy for Intracranial Volume in Patients With Craniosynostosis?

Calculation of intracranial volume from neuroimaging can be complex and time consuming. In the adult population, there is evidence suggesting that owing to its strong correlation, head circumference (HC) may be used as a surrogate for intracranial volume (ICV). We were interested in studying the correlation between HC and ICV in patients with craniosynostosis. After institutional review board approval, a retrospective review was performed on patients with craniosynostosis. GE Healthcare AdW 4.3 volume assessment software was used to calculate ICV and HC based on preoperative computed tomographic scans. Pearson correlation was used to estimate correlation coefficients between ICV and HC for this patient population, with 0 to 0.3 considered a weak correlation, 0.4 to 0.6 considered a moderate correlation, 0.7 to 1 considered a strong correlation, and P < 0.05 was considered statistically significant. A total of 196 craniosynostosis patients were included in this study. There were 121 male and 75 female patients. Seventy-nine patients had metopic, 45 had coronal, 64 had sagittal, and 8 had lambdoid synostosis. Mean age was 8.2 months. Mean HC and ICV were 42.9 cm and 829 cm, respectively. Overall, there was a strong correlation between HC and ICV (r = 0.81). Patients were further categorized by craniosynostosis type. Very strong correlation was obtained for patients with coronal (0.89), metopic (0.98), and lambdoid craniosynostosis (0.97). Strong correlation was obtained for patients with sagittal synostosis (0.73). When categorized by sex, a stronger correlation was obtained for female patients (0.84) compared with male patients (0.80). Statistical significance was reached for all reported correlations. Our preliminary data suggest that a very strong correlation exists between HC and ICV for male and female patients with all types of craniosynostosis, making HC a useful surrogate for ICV in this patient population.

Intracranial volume (ICV) in isolated sagittal craniosynostosis: a retrospective case-matched-control study.

Children with sagittal craniosynostosis (SC) are at risk of developing raised intracranial pressure (ICP). This is thought to result from cephalocranial disproportion-the restriction of normal cerebral development by a small cranial vault. It remains unclear whether intracranial volume (ICV) is altered in SC. This study offers a novel volumetric analysis of the scaphocephalic skull, comparing supratentorial (ST) volume, infratentorial (IT) volume, and total ICV of patients with sagittal synostosis to normal controls. ICVs of 32 consecutive patients undergoing total calvarial vault remodelling (TCVR) for isolated SC were compared to 32 age- and sex-matched normal controls. ICV was measured with manual techniques on head computerised tomographic (CT) scans using OsiriX software. A paired t test was used to compare data between cases and controls. Mean total ICV, ST volume and IT volume were larger in SC than in controls, except in females > 6months of age. There was no statistical significance. Regression analysis demonstrated larger ICVs in diseased children than in controls younger than 10months, at which age trend lines intersected and the reverse became true for older children. This likely represents an evolving risk of cephalocranial disproportion beyond 10months of age. The IT/ST volume ratio was conserved in scaphocephaly, and very closely approximated that of controls. Sagittal craniosynostosis appears to be associated with a larger cranial vault at less than 10months and a smaller vault at greater than 10months, although statistical significance was not achieved.

Head Circumference Is Strongly Correlated with Intracranial Volume in Patients with Metopic Craniosynostosis

Cronin, Brendan J.; Brandel, Michael G.; Buckstaff, Taylor M.; Cahill, Gabrielle L.; Mannix, Emily M.; Hashmi, Asra MD; Reid, Chris M. MD; Lance, Samuel MD; Meltzer, Hal MD; Gosman, Amanda A. MD Author Information

Growth curves for intracranial volume in normal Asian children fortify management of craniosynostosis

BackgroundAlthough the charting of normal intracranial volume (ICV) is fundamental for managing craniosynostosis, Asian norms in this regard are unknown. The purpose of this study was to establish a growth curve for ICVs in a large series of normal Asian children, providing reference values to guide corrective surgery. MethodsA total of 124 normal children (male, 63; female, 61) and 41 children diagnosed with craniosynostoses were analyzed. Patients aged 0–8 years presenting to the emergency room and subjected to computed tomography (CT) for head trauma served as the reference cohort. Axial CT head scan data were obtained from radiographic archives at Jichi Medical University. Imaging was done on a Siemens CT scanner (5-mm slice thickness), using a DICOM viewer to measure ICVs. ResultsICVs were plotted against age, and best-fit logarithmic curves for normal subjects were generated, without and with gender stratification. Male and female growth curves were similar in shape but diverged past the age of 1 year (male > female). ICVs of patients with craniosynostoses were plotted to male and female growth curves by disease subset, revealing the following: sagittal synostosis, near normal (or marginally larger); metopic synostosis, below normal; other non-syndromic synostoses (unilateral, bilateral, and lambdoidal) and Crouzon syndrome, near normal; Apert syndrome, above normal; and Pfeiffer syndrome, variable. ConclusionICVs of early childhood were investigated in Asian subjects, creating growth curves that set criteria for timing, planning and goalsetting in surgical correction of craniosynostosis.

Quantifying Craniometric Change Early After Fronto-Orbital Advancement in Metopic Synostosis.

Frontal orbital advancement (FOA) for metopic synostosis results in cranial vault expansion, supporting underlying growth of the developing brain and improving head shape. Previous studies have shown that FOA in younger infants leads to a greater incidence of long-term growth restriction of intracranial volume; however, it is still unknown as to whether this is due to undercorrection at the time of surgery versus primary suture pathology. The purpose of our study is to provide a method for objective analyses of intracranial volumes in the early post-FOA period. A retrospective chart review was conducted on patients who underwent FOA over a 10-year period by a single surgeon. Data collected included patient characteristics and clinical outcomes. Radiological analysis of intracranial volumes pre- and post-FOA was determined using Amira volume-rendering software. Average increase in intracranial volume at 6 weeks post-FOA was 139.27 mL (80.01-225.25 mL) with average relative cranial vault expansion of 18.1% (3.3-48%). Patients who underwent FOA older than 12 months of age had an average increase in intracranial volume of 8.5% (3.3-13.1%). Patients younger than 12 months had a statistically higher average increase of 25% (12.8-48%). Frontal orbital advancement effectively increases intracranial volume in patients with metopic synostosis. When compared with older patients, patients younger than 1 year of age have a greater relative increase in intracranial volume in the immediate postoperative period. Quantifying cranial vault expansion after FOA can assist surgeons in surgical planning as well as in measuring and monitoring clinical outcomes within and across craniofacial centers regardless of technique.

Intracranial volume (ICV) in isolated sagittal craniosynostosis measured by 3D photocephalometry: A new perspective on a controversial issue.

There are still controversies regarding the intracranial volumes in patients with isolated sagittal craniosynostosis compared to a healthy population. This study aimed to compare the intracranial volume of children with sagittal synostosis and scaphocephaly to an age- and gender-matched control cohort using three-dimensional (3D) photogrammetry. 62 boys and nine girls with sagittal craniosynostosis were included in this study. The intracranial volume was measured at the first clinical presentation. However, 3D photogrammetry was performed at children not younger than 3 months. The 3D photogrammetric data of 547 healthy boys and 287 healthy girls between the ages of 3-10 month was analyzed to establish an age- and gender-matched control group. Male patients with sagittal synostosis showed a significantly reduced intracranial volume compared to the reference group. For female patients, the intracranial volume was slightly lower compared to the norm group, but not significantly. Male children with sagittal synostosis showed significantly decreased intracranial volume between the age of 3 and 10 months compared to an age- and gender-matched control group. Female patients in the same age group presented a lower intracranial volume compared to the norm group. Measuring intracranial volume using 3D photogrammetry is a comparable and valuable alternative to CT scans that leads to a significant reduction of radiation exposure to the growing brain.

The occipitofrontal circumference: reliable prediction of the intracranial volume in children with syndromic and complex craniosynostosis.

OBJECT Patients with syndromic and complex craniosynostosis are characterized by the premature fusion of one or more cranial sutures. These patients are at risk for developing elevated intracranial pressure (ICP). There are several factors known to contribute to elevated ICP in these patients, including craniocerebral disproportion, hydrocephalus, venous hypertension, and obstructive sleep apnea. However, the causal mechanism is unknown, and patients develop elevated ICP even after skull surgery. In clinical practice, the occipitofrontal circumference (OFC) is used as an indirect measure for intracranial volume (ICV), to evaluate skull growth. However, it remains unknown whether OFC is a reliable predictor of ICV in patients with a severe skull deformity. Therefore, in this study the authors evaluated the relation between ICV and OFC. METHODS Eighty-four CT scans obtained in 69 patients with syndromic and complex craniosynostosis treated at the Erasmus University Medical Center-Sophia Children's Hospital were included. The ICV was calculated based on CT scans by using autosegmentation with an HU threshold < 150. The OFC was collected from electronic patient files. The CT scans and OFC measurements were matched based on a maximum amount of the time that was allowed between these examinations, which was dependent on age. A Pearson correlation coefficient was calculated to evaluate the correlations between OFC and ICV. The predictive value of OFC, age, and sex on ICV was then further evaluated using a univariate linear mixed model. The significant factors in the univariate analysis were subsequently entered in a multivariate mixed model. RESULTS The correlations found between OFC and ICV were r = 0.908 for the total group (p < 0.001), r = 0.981 for Apert (p < 0.001), r = 0.867 for Crouzon-Pfeiffer (p < 0.001), r = 0.989 for Muenke (p < 0.001), r = 0.858 for Saethre- Chotzen syndrome (p = 0.001), and r = 0.917 for complex craniosynostosis (p < 0.001). Age and OFC were significant predictors of ICV in the univariate linear mixed model (p < 0.001 for both factors). The OFC was the only predictor that remained significant in the multivariate analysis (p < 0.001). CONCLUSIONS The OFC is a significant predictor of ICV in patients with syndromic and complex craniosynostosis. Therefore, measuring the OFC during clinical practice is very useful in determining which patients are at risk for impaired skull growth.

Intracranial Volume in 15 Children with Bilateral Coronal Craniosynostosis

Background:Intracranial volume (ICV) growth in patients with bilateral coronal craniosynostosis (BCS) is not well described. It is therefore important to evaluate the consequences of cranial surgery in children with this condition. The aim of the present study was to evaluate ICVs in patients operated on for BCS.Methods:A consecutive series of patients with BCS were operated on using spring-assisted cranioplasty, with computed tomography scans in 0.6-mm slices, were included. A MATLAB-based computer program capable of measuring ICV was used. Patients were compared with an age- and gender-matched control group of healthy children. Student’s t test was used for statistical analysis.Results:Fifteen patients (7 girls and 8 boys) with 43 computed tomography scans were identified. The diagnoses were 13 syndromic BCS (3 Apert, 1 Crouzon, 6 Muenke, and 3 Saethre-Chotzen) and 2 nonsyndromic BCS. The mean preoperative volume at the age of 5 months (n = 15) was 887 mL (range, 687–1082). Mean volume at follow-up at the age of 3 years (n = 13) was 1369 mL (range, 1196–1616). In comparison, the mean ICVs for controls at the ages of 5 months (n = 30) and 3 years (n = 26) were 854 mL and 1358 mL, respectively. The differences were not statistically significant (P > 0.05).Conclusions:Patients with BCS were operated on with spring-assisted cranioplasty seem to maintain their age-related ICV at 3 years of age when compared to normal children.

The evolution of a new Craniofacial Procedure: From Concept to established Intervention. The 8 year history of Posterior Calvarial Osseodistraction and its use in Craniofacial Surgery

Introduction: Posterior Calvarial Osseodistraction was first presented at the annual scientific meeting BAOMS, Aviemore, 2007 by the lead author. Its indication was to alleviate raised intracranial pressure in syndromic craniosynostotic patients. Since then the procedure has been accepted and used in many craniofacial surgery units worldwide. In addition in some patients with Chiari malformation at the craniocervical junction, there is regression of the Chiari and improvement in symptoms when this technique is used. Method: We prospectively collected data for the 38 cases who have undergone this intervention in our unit in the 8 years since the inception of the technique. Parameters measured included age, diagnosis, indication, distraction distance achieved and complications. Results: There have been modifications in the technique and distractors used in the study period.. 22 patients had a confirmed craniofacial syndrome. The majority of patients required the procedure for raised intracranial pressure (n=24); other indications were Chiari malformation (n= 2) and Chiari malformation with syrinx (n=2). This will be discussed further. Complications of the procedure are discussed and include cerebro spinal fluid leaks (n= 5) and distractor loosening (n=5). Conclusion: Posterior Calvarial Osseodistraction is an accepted intervention for increasing intracranial volume in patients who have premature sutural fusion. It is used in our unit as an adjunct in the management of complex craniofacial patients.

Intracranial Volume Before and After Surgical Treatment for Isolated Metopic Synostosis

Metopic synostosis results in a keel-shaped forehead, hypotelorism, and an increased interparietal width. This study aimed to measure the frontal and total intracranial volume in patients with metopic synostosis before and after surgery and to compare the effect of 2 different operation methods. All patients operated for isolated metopic synostosis between 2002 and 2008 at Sahlgrenska University Hospital who had undergone preoperative and/or postoperative computed tomographic examination (at 3 y of age) were included. The patients were grouped according to operation method: (1) forehead remodeling in combination with a bone graft or (2) forehead remodeling in combination with a spring. Sex- and age-matched controls were identified. A previously developed MATLAB computer program was used to measure the frontal and total intracranial volumes. Sixty patients and 198 controls were included. Preoperatively, the patients with metopic synostosis had significantly lower frontal volumes than those of the controls (P < 0.001) but equal total intracranial volumes. The operations redistributed the intracranial volume and resulted in an improved, frontal-total intracranial volume ratio. However, at 3 years of age, the frontal volume (P < 0.001), total intracranial volume (P ≤ 0.002), and ratio between the 2 (P < 0.001) were significantly lower in the patients than in the controls. The 2 operation methods were equally efficient in creating an improved frontal-total ratio. Surgery for metopic synostosis improves the distribution of the intracranial volume but does not result in normal total intracranial volume or frontal volume at 3 years of age.

Prnp gene and cerebellum volume in patients with refractory mesial temporal lobe epilepsy

The cellular prion protein, encoded by Prnp gene, is involved in neuroprotection, neuroplasticity and neurodevelopment. The variant allele Valine at codon 129 of the Prnp was associated with decreased brain volume in healthy volunteers and schizophrenic patients. We investigate the association between the cerebellum volume and the presence of variant allele Valine at codon 129 of the Prnp gene in patients with mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE-HS). The Prnp coding sequence was determined in 41 refractory MTLE-HS patients. The cerebellum volume corrected by the intracranial volume of patients with the normal Prnp genotypes was compared with that of patients presenting the variant alleles at codon 129. Twenty patients showed the Met129Met genotype, 16 showed Met129Val, and 5 had Val129Val. There were no association among clinical, demographic, electrophysiological, antiepileptic drugs used, and the presence of the Prnp variant alleles. The presence of Prnp variant allele at codon 129 was not associated with the analyzed cerebellum volume. Prnp variant alleles at codon 129 are not associated with cerebellum volume in patients with refractory MTLE-HS.

Mesh-based method for measuring intracranial volume in patients with craniosynostosis

Craniosynostosis may lead to reduced intracranial volume (ICV) and disturb normal brain growth and development. Thus, ICV is an important parameter with respect to the surgical outcome. Current methods for ICV determination from computed tomography (CT) images have drawbacks. The aim of this study was to investigate the performance of the novel mesh-based method (MBM) for ICV determination with craniosynostosis patients. Twenty-two patients operated on for scaphocephaly were included in this study. ICVs from preoperative, one-week postoperative, and one-year postoperative CT images were measured with MBM. The level of agreement with the manual segmentation method (MSM) was determined for the measurements of preoperative and one-year postoperative datasets. Repeatability was determined with re-measurements of six datasets. Measurement time was recorded for MBM. Mean [Formula: see text] preoperative ICV values were 895.0 [Formula: see text] 153.1 [Formula: see text] and 896.4 [Formula: see text] 147.2 [Formula: see text] as measured with MBM and MSM, respectively. Corresponding one-year postoperative values were 1,238.3 [Formula: see text] 118.7 [Formula: see text] and 1,250.1 [Formula: see text] 117.5 [Formula: see text]. The MBM allowed ICV determination from one-week postoperative datasets. Measurement time with MBM was 4 CONCLUSIONS: MBM is an efficient method for determining the ICV of craniosynostosis patients, allowing the measurement of skulls with bony defects. The repeatability and short measurement time of MBM are attributable to the user interference and assessment of the measurement process.

Relationships Between Retinal Axonal and Neuronal Measures and Global Central Nervous System Pathology in Multiple Sclerosis

To determine the relationships between conventional and segmentation-derived optical coherence tomography (OCT) retinal layer thickness measures with intracranial volume (a surrogate of head size) and brain substructure volumes in multiple sclerosis (MS). Cross-sectional study. Johns Hopkins University, Baltimore, Maryland. A total of 84 patients with MS and 24 healthy control subjects. High-definition spectral-domain OCT conventional and automated segmentation-derived discrete retinal layer thicknesses and 3-T magnetic resonance imaging brain substructure volumes. Peripapillary retinal nerve fiber layer as well as composite ganglion cell layer+inner plexiform layer thicknesses in the eyes of patients with MS without a history of optic neuritis were associated with cortical gray matter (P=.01 and P=.04, respectively) and caudate (P=.04 and P=.03, respectively) volumes. Inner nuclear layer thickness, also in eyes without a history of optic neuritis, was associated with fluid-attenuated inversion recovery lesion volume (P=.007) and inversely associated with normal-appearing white matter volume (P=.005) in relapsing-remitting MS. As intracranial volume was found to be related with several of the OCT measures in patients with MS and healthy control subjects and is already known to be associated with brain substructure volumes, all OCT-brain substructure relationships were adjusted for intracranial volume. CONCLUSIONS Retinal measures reflect global central nervous system pathology in multiple sclerosis, with thicknesses of discrete retinal layers each appearing to be associated with distinct central nervous system processes. Moreover, OCT measures appear to correlate with intracranial volume in patients with MS and healthy control subjects, an important unexpected factor unaccounted for in prior studies examining the relationships between peripapillary retinal nerve fiber layer thickness and brain substructure volumes.

Combined metopic and sagittal craniosynostosis: is it worse than sagittal synostosis alone?

OBJECT Combined metopic and sagittal craniosynostosis is a common variant of the nonsyndromic, multiplesuture synostoses. It is unknown whether this combined form causes reduced intracranial volume (ICV) and potentially more brain dysfunction than sagittal synostosis alone. This study is a volumetric comparison of these 2 forms of craniosynostosis. METHODS The authors conducted a retrospective chart and CT review of 36 cases of isolated sagittal synostosis or combined metopic and sagittal synostosis, involving patients seen between 1998 and 2006. Values were obtained for the intracranial compartment, brain tissue, CSF space, and ventricular volumes. Patients with craniosynostosis were then compared on these measures to 39 age- and sex-matched controls. RESULTS In patients with isolated sagittal synostosis and in those with combined metopic and sagittal synostosis, there was a trend toward smaller ICV than in controls (p < 0.1). In female patients older than 4.5 months of age, there was also a trend toward smaller ICV in patients with the combined form than in those with sagittal synostosis alone (p < 0.1), and the ICV of patients with the combined form was significantly smaller than the volume in controls in the same age group (p < 0.05). Brain tissue volume was significantly smaller in both patient groups than in controls (p < 0.05). Ventricular volume was significantly increased (compared with controls) only in the patients with isolated sagittal synostosis who were younger than 4.5 months of age (p < 0.05). Overall CSF space, however, was significantly larger in both patient groups in patients younger than 4.5 months of age (p < 0.05). CONCLUSIONS These findings raise concerns about intracranial and brain volume reduction in patients with sagittal and combined metopic and sagittal synostoses and the possibility that this volume reduction may be associated with brain dysfunction. Because the ICV reduction is greater in combined metopic and sagittal synostosis in patients older than 4.5 months of age than in sagittal synostosis in this age group, the potential for brain dysfunction may be particularly true for these younger infants.

PR02 *SPRING‐ASSISTED CRANIOPLASTY – RESULTS OF CHRONOLOGICAL CHANGES TO SKULL DIMENSIONS

Purpose: To demonstrate chronological changes of spring expansion in the treatment of scaphocephaly and brachycephaly. Spring‐assisted cranioplasty has become the first‐line treatment option for patients less than one year‐old with scaphocephaly and in some cases with brachycephaly. The speed of spring expansion and its in‐situ expansile behaviour has not been previously demonstrated in humans.Methodology: From January 2008 to December 2008, five patients underwent spring‐assisted Cranioplasty (4 scaphocephaly, 1 brachycephaly). The cephalic index, head circumference was measured chronologically until 3‐month postoperatively. Postoperative intracranial volume was calculated in one centre with post‐spring removal head CT. Prospective review of patient's operative time and blood loss, hospital time, changes in cephalic index (CI) and intracranial volume.Results: The average age of the patients were 5.2 months, operative time was averaged 70 minutes, operative blood loss was less than 50 ml, however three of five cases required blood transfusion day 1 postoperatively. Average hospital stay was 3 days, the preoperative CI was 70% for scaphocephalic patients and 83% for the brachycephalic patient. The 3‐month postoperative CI was 76% and 79%, respectively, the changes in intracranial volume was 356 ml.Conclusion: 30% of its total expansion occurs during the operation. The remaining spring's expansion capacity takes place in the first postoperative month as the skull lengthening occurs. Spring‐assisted cranioplasty is useful in increasing intracranial volume in patients with craniocerebral disproportion and high risk of developing increased intracranial pressure.

Intracranial volume in patients with nonsyndromal craniosynostosis

In recent years, comparisons between intracranial volumes (ICVs) in patients with craniosynostosis and healthy patients have given variable results, leading to questions regarding the validity of the normal reference material and the comparability of the measurement techniques. In this study, ICVs in patients with nonsyndromal craniosynostosis without previous surgical intervention were compared with the ICVs of a normal population of European descent determined using the same method for each group. Determination of ICV was based on measuring the area of intersection in each computerized tomography slice. For comparisons the ICV measurements for each patient were standardized with regard to age and sex by expressing them in terms of the standard deviation score. Only the group of boys with metopic synostosis had a tendency toward smaller ICV than did healthy boys (p = 0.04). Partitioning the male metopic data into age groups younger and older than 7 months of age revealed that the younger children had normal ICVs, whereas the older children had, on average, smaller ICVs (p = 0.02). Both the female sagittal synostosis and the male unilateral coronal synostosis groups had larger than normal ICVs, both with a probability value less than 0.001. No evidence was found that the ICVs of patients with nonsyndromal craniosynostosis are smaller than those of normal children, except for boys older than 7 months of age with metopic synostosis. This finding may have implications for the timing of surgical intervention for these patients. The indications are that interventions should be focused less on ICV and more on normalizing craniofacial shape and promoting normal development.

A study of intracranial volume in Apert syndrome.

The present study was undertaken to characterize the intracranial volume in patients with Apert syndrome. Radiographic and CT-based techniques for measuring intracranial volume were found to show close correlation when validated in dry normal adult skulls. A standard-deviation score of intracranial volume, determined from CT scans in 20 patients with Apert syndrome from birth to 30 years of age, was calculated with reference to a normative database of age- and sex-matched controls. Analysis of the data showed no significant difference in standard-deviation score between Apert patients with or without ventriculomegaly, before or after cranial vault surgery, or between male and female patients. The mean standard-deviation score among all Apert CT scans compared with normative data was 3.1, which was highly significant (p < 0.001). However, the growth curves showed no significant difference in intracranial volume between Apert and normal patients below 3.5 months of age. In summary, intracranial volume in Apert syndrome is within normal range at birth but increases to greater than 3 standard deviations above normal after 3.5 months of age, a finding independent of cranial vault surgery.