Intracranial volume is normal in infants with sagittal synostosis.

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.

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.

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.

Psychosocial conditions in adults with Crouzon syndrome: a follow-up study of 31 Swedish patients.

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.

Correction of hypotelorism in isolated metopic synostosis.

Surgical correction of trigonocephaly is performed by fronto-orbital reshaping and advancement. Some authors supplement the fronto-orbital remodelling with direct surgical correction of the hypotelorism, but the role of this step of the procedure has been questioned. At Sahlgrenska University Hospital, hypotelorism in metopic synostosis is treated with spring-assisted surgery (S) in children below 6 months of age and with fronto-orbital advancement with the interposition of a bone graft (BG) in the fronto-orbital region at higher age. The aim of the present study was to evaluate the anterior bony interorbital distance (BIOD) preoperatively and at follow-up in patients operated on with the two techniques and to compare the results with adequate control groups. Preoperatively, the patients affected by metopic synostosis had a significantly reduced BIOD compared to their respective controls (S group: 13.8 ± 1.6 (mean ± SD) mm vs 18.6 ± 1.4 mm, p < 0.001, BG group: 14.7 ± 1.0 mm vs 18.8 ± 1.4 mm, p < 0.001). At 3 years follow-up, BIOD was improved in both groups, but only in the S group the mean BIOD did not differ from the control group (19.7 ± 2.9 mm vs 20.2 mm ± 1.3 mm, p = 0.3). In the BG group the BIOD was still significantly different between patients and controls (17.6 ± 1.8 vs 20.0 ± 1.1 mm, p < 0.001). Spring-assisted surgery performed before 6 months of age can normalise hypotelorism in metopic synostosis. Full correction in a large series of patients has now been achieved for the first time.

New objective measurement of forehead symmetry in unicoronal craniosynostosis - comparison between fronto-orbital advancement and forehead remodelling with a bone graft.

Patients with unicoronal synostosis (UCS) present with ipsilateral forehead flattening, contralateral frontal bossing, and rotation of the facial midline. Uni- or bilateral fronto-orbital advancement (FOA) techniques are the most common surgical approaches for correction of UCS. The purpose of this study was to objectively evaluate the surgical outcome in patients for UCS, using a new MATLAB computer tool programmed to measure the symmetry of the two halves of the forehead.Files were reviewed from a consecutive series of patients treated for UCS at the unit, from 1979-2008. The patients were grouped according to the method of operation used. The computer tool evaluated preoperative and postoperative cephalograms and CT scans. Eighty-eight patients were included. The male-to-female ratio was 1:2.4. Forty-six patients had been operated on with FOA and 42 with forehead remodelling using a calvarial bone graft. Forehead symmetry was significantly improved by both techniques (p < 0.001 for both), but the postoperative forehead symmetry was significantly better after forehead remodelling (p = 0.025). The reoperation rate was much lower for the second group (6.5 vs 37.2%, p < 0.001). It is concluded that forehead remodelling with a calvarial bone graft creates a more symmetrical forehead than FOA and may, therefore, be a better alternative for treatment of unicoronal synostosis.

Children with Apert syndrome as adults: a follow-up study of 28 Scandinavian patients.

BACKGROUND: Apert syndrome (acrocephalosyndactyly type 1) includes craniofacial deformities, malformations of the extremities and the central nervous system, and often mental retardation. The aim of this study was to investigate the life situation of adult patients with Apert syndrome. METHODS: Thirty-three patients with Apert syndrome born before 1990 were identified in the Göteborg craniofacial registry. The authors used a questionnaire mainly dealing with education, employment, social relations, and quality of life. A matched control group was created for comparison. RESULTS: Five of the patients had died. Twenty-four of the remaining patients answered the questionnaire. The level of education was lower in patients than in controls (p = 0.007), but there was no significant difference in the extent of employment between the two groups. The difference in housing was significant (p < 0.001) and the majority of patients lived with their parents. Patients were less often married (p < 0.001), had fewer friends (p < 0.001), and had less experience with sexual relationships (p < 0.001). The somatic health was lower in patients [e.g., they had more hearing problems (p < 0.001) and more epilepsy (p = 0.005)]. Depressive mood periods were more common in patients (p < 0.001), but there was no difference between the groups regarding a generally positive attitude toward life. CONCLUSIONS: This study shows that patients with Apert syndrome manage relatively well in adulthood. The discrepancy in social relations between the Apert patients and the healthy control group indicates that further improvement of the treatment is desirable.

Spring-assisted cranioplasty for bicoronal synostosis.

BACKGROUND: Numerous surgical techniques for cranial reconstruction of patients with bicoronal synostosis have been suggested. The outcome is, however, still often suboptimal. METHODS: Since 2005, we have, at the Craniofacial Unit, Sahlgrenska University Hospital, Gothenburg, used a standardized surgical technique with advancement and remodeling of the forehead combined with spring distraction of the occipital area. The aim of the current study was to evaluate this operative technique. Eighteen consecutive patients (9 boys and 9 girls) with bicoronal synostosis operated on using this technique were identified. Sixteen patients had syndromic bicoronal synostosis, and 2 had nonsyndromic bicoronal synostosis. Cephalic index was obtained from three-dimensional computed tomography scans, and photographs were analyzed for aesthetic evaluation. RESULTS: The preoperative calvarial shape was hyperbrachycephalic in all subjects. Postoperatively, the calvarial shape was, in general, much closer to the norm. The reduction in the mean cephalic index from the preoperative stage (94) to the 3-year follow-up (82) was statistically significant (P < 0.0001). The mean duration of surgery was 155 (SD, 32) minutes, with a mean perioperative bleeding of 237 (SD, 95) mL. The mean hospital stay was 6.3 (SD, 1.5) days, of which the mean intensive care unit stay was 1.6 (SD, 1.2) days. In 2 patients, one of the springs had to be reinserted because of postoperative dislocation. No other major complications were observed. CONCLUSIONS: Spring-assisted cranioplasty for bicoronal synostosis is a safe technique, is less invasive than many other cranioplasties, and results in marked improvement in the calvarial shape.

Parental estimation of early psychological development in children operated on for single suture synostosis.

Impaired psychological development in children with single suture craniosynostosis is often described in the literature. The authors' own experience was that these children appeared to have normal psychological development during their first years of life. The aim of the present study was, therefore, to evaluate if parental estimation would reveal any shortcomings in psychological development. All consecutive patients operated on for non-syndromal single suture synostosis between October 2002 and June 2006 were included (n = 66). A questionnaire was filled out by the parents when the child was 3 years old. The questions concerned development of speech, motor control, personal abilities, and emotional development. The results were compared with randomly selected controls of the same age (n = 180) whose parents answered the same questionnaire. The results showed that children operated on for single suture synostosis did not show any signs of lasting disadvantages due to craniofacial surgery. The children did not in any respect suffer from a hampered psychological development up to 3 years of age. In summary the psychological development of patients operated on for single suture synostosis seems to be normal up to 3 years of age as far as parental estimation can reveal.