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Attention problems are common in patients with craniosynostosis. Craniosynostosis is a rare condition, studies face challenges of selection bias, small sample sizes, and wide age ranges. Th...

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.

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.

The aim of this project was to develop a tool for systematic evaluation of volumetric changes after surgery for craniosynostosis. A computer program using MATLAB was developed to measure total intracranial volume and frontal volume, anterior to the coronary sutures, by multiplying the area with slice thickness of each slice from just above foramen magnum to just beneath the vertex in CT examinations. The ratio between frontal volume and total volume was used for evaluation of the clinical result in 12 patients operated on for metopic synostosis. In 0.625 and 5 millimetre slices the coefficients of variation were 0.00049 and 0.00058, respectively, for measurements of total volume. The highest coefficient of variation was found in postoperative measurements of the frontal volume and was 0.014 in 0.625 millimetre slices. Measurements in 5 millimetre slices resulted in 3.8% ± 1.5% (mean ± SD) lower total volumes and 5.8% ± 5.3% lower frontal volumes than measurements in 0.625 millimetre slices. In patients operated on for metopic synostosis the ratio between frontal volume and total volume increased 25% ± 16% for patients operated on with cranioplasty in combination with a spring (n = 6) and 20% ± 13% for patients operated on with cranioplasty in combination with a bone transplant (n = 6). In summary, this study has developed a tool that can determine frontal and total intracranial volume with little variation. This tool can be used for systematic evaluation of the result of the operation for metopic synostosis.

We have developed a new technique for reconstruction of full thickness eyelid defects. A composite three-layer skin-cartilage-mucosal unit is manufactured and tailored three-dimensionally. Use of this sandwich graft in our patients proved to be simple and safe for eyelid reconstruction and has given good results without complications in four patients.

D-myo-inositol-1,2,6-triphosphate (1,2,6-IP3) has beneficial effects in experimental, progressive burn-induced ischaemia and oedema. A 1 cm2 full-thickness burn was made in the skin of 20 rats with a hot aluminium rod followed by infusion of 1,2,6-IP3 (60 mg.kg.-1 h-1) or isotonic saline (n = 10 in each group). One hour later Evans blue was injected intravenously. Colour photographs of the area of the burn were taken in a standard manner before the burn and at intervals for three hours afterwards. The photographs were analysed by digital image colour analysis using normalised red-green-blue values. The increase in normalised blue values between 60 and 180 minutes after the burn was significantly reduced in animals treated with 1,2,6-IP3 compared with control animals (p0.001). Spectrophotometric analysis of extravasated Evans blue in the skin 180 minutes after the burn showed that it had been significantly inhibited by treatment with 1,2,6-IP3 (p0.001). In conclusion, digital image analysis allowed repeated evaluation over time and confirmed previous data about the ability of 1,2,6-IP3 to inhibit extravasation of plasma after burns.

The nasolabial flap is the classic flap for reconstruction of nasal defects. During the last five years we have used a modified nasolabial flap in which the distal part of the flap is defatted leaving only the dermis and epidermis intact. This distal part of the flap is then folded and used as inner or outer lining which creates a reconstruction that is thinner than the original folded flap. We have used this technique in 11 patients and the results are satisfactory, with only three patients requiring minor corrections.