Premature sagittal synostosis results in an elongated, narrow skull shape, scaphocephaly. It has been unclear whether the intracranial volume (ICV) of these children is different from that of normal children. The aim of the present study was to precisely determine the ICV in a large cohort of children with premature sagittal synostosis and to compare it to the ICV of a sex- and age-matched control group. All patients (n = 143) with isolated sagittal synostosis registered in the Göteborg Craniofacial Registry until the end of 2012 with a preoperative CT examination were identified. For each case, a sex- and age- (±30 days) matched control was identified from children who had undergone CT for post-traumatic or neurological reasons. The ICV was measured in a semi-automatic MATLAB program with functions such as region growing, watershed, and thresholding in axial CT slices. The ICV was calculated using the Cavalieri principle. The mean (± SEM) values of ICV for children with sagittal synostosis and for corresponding controls were 866 ± 13 ml and 870 ± 15 ml, respectively. The mean ages of these groups were 173 ± 8 days and 172 ± 8 days, respectively. Subgroup analysis of sex and age at CT (≤180 days and >180 days) did not reveal any differences in ICV between cases and controls. Precise determination of ICV in addition to the use of adequate controls has made it possible to conclude that children with premature isolated sagittal synostosis have a normal ICV.

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.

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.

Crouzon syndrome presents with craniosynostosis, maxillary hypoplasia, exophtalmus, and sometimes hampered neuropsychological development. The aim of the present study was to evaluate the quality of life for adult patients with Crouzon syndrome. Forty patients with Crouzon syndrome born before 1990 could be identified. A questionnaire addressing education, employment, social relations, and quality-of-life was used. A matched control group was created for comparison. Logistic regression, correcting for the influence of age and sex, was used to compare patients and controls. Thirty-one patients and 285 controls answered the questionnaire. The level of education was lower in patients than in controls (p < 0.015). Patients were less often married or had a partner (p = 0.059), had fewer children of their own (p = 0.004), and had less experience of a sexual relationship (p < 0.001). The difference in housing was not significant, and only one patient lived in a care centre and three patients required a personal assistant to manage activities of daily living. The patients' estimation of their somatic health was equal to that of the controls, but the patients more often used anti-epileptic medication (p = 0.003). Periods of depressive mood were more common in patients (p = 0.001), but there was no difference between the groups regarding a general positive attitude to life. In conclusion, patients with Crouzon syndrome often have intellectual and social shortcomings that negatively affect their lives. However, the range of abilities is wide in this group.