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Background: Assessing health-related quality of life (HRQoL) allows acquisition of the subjective perspective of patients regarding their health and functioning; however, little is known about the experiences of patients living with treated craniosynostosis (CS). Methods: School-aged children (7–16 years) treated for non-syndromic CS were assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales via both self- and proxy reports.Results: Seventy-three patients and their parents responded to the PedsQL (response rate: 80.2%). Patients generally estimated HRQoL as high, with no difference in HRQoL found between treated sagittal (SS) or metopic (MS) synostosis. In the SS group, surgical methods involving spring-assisted surgery and pi-plasty were unrelated to HRQoL outcomes. Additionally, HRQoL was highly correlated with intelligence quotient (IQ) and adaptive behavior skills (ABAS). Furthermore, we observed differences in estimated HRQoL between self- and proxy reports (i.e., parents estimated child HRQoL as higher than did the children). Conclusions: Children treated for CS have a generally high HRQoL, and neither CS type nor surgical method influenced HRQoL outcomes. Moreover, children and parents estimated HRQoL differently, suggesting the importance of using both self- and proxy reporting in patient-reported measures. We found that HRQoL was strongly related to IQ and ABAS, indicating that the PedsQL can be used as a screening instrument to identify craniofacial patients in need of further psychological assessment.

In this retrospective study, the authors determined changes in intracranial volume (ICV) and cephalic index (CI) in patients with sagittal craniosynostosis and operated with craniotomy combined with either 2 or 3 springs. The authors included patients (n = 112) with complete follow-up that had undergone surgical correction for isolated sagittal craniosynostosis with craniotomy combined with springs between 2008 and 2017. All patients underwent computed tomography examination preoperative, at the time of spring extraction, and at 3 years of age. Intracranial volume was measured using a semiautomatic MATLAB program, and CI was calculated as the width/length of the skull. The authors found that craniotomy combined with 2 springs increased the ICV from a preoperative value of 792 ± 113 mL (mean ± standard deviation) to 1298 ± 181 mL at 3 years of age and increased the CI from 72.1 ± 4.1 to 74.6 ± 4.3, whereas craniotomy combined with 3 springs increased the ICV from 779 ± 128 mL to 1283 ± 136 mL and the CI from 70.7 ± 4.3 to 74.8 ± 3.7. The relative increase in ICV was 65 ± 21% in the two-spring group and 68 ± 34% in the three-spring group (P value = 0.559), and the relative increase in CI was 3.6 ± 3.3% in the two-spring group as compared with 6.0 ± 5.0% in the three-spring group (P = 0.004). These findings demonstrated that use of 3 springs resulted in additional absolute and relative CI-specific effects as compared with 2 springs during the time when the springs were in place, with this effect maintained at 3 years of age.

Since the development of spring-assisted techniques for corrective craniofacial surgery, routine postoperative admission to intensive care units (ICUs) has been questioned. However, close monitoring is necessary if the continuous infusion of morphine is used as recommended for better pain relief. In this study, the authors evaluated a simplified postoperative protocol without continuous morphine infusion and no indwelling urinary catheter following spring-assisted surgery (SAS) for sagittal synostosis. Ten children were cared for according to a standard protocol with postoperative intravenous (i.v.) infusion of morphine and an indwelling urinary catheter, and 11 consecutive children were treated according to a simplified protocol with pain relief based on intermittent injections of morphine and clonidine [according to Face, Legs, Activity, Cry, Consolability (FLACC) scores >4] without the indwelling catheter. A Mann-Whitney U test was used for comparison of distributions between the two groups. The results revealed no differences between groups regarding the proportion of FLACC scores >4, total amount of administered i.v. morphine and clonidine, total volume of buffered glucose infused, time to first feeding on breast milk or substitute, or the length of stay. Despite the inherent limitations of our small observational study, the authors concluded that at our institution, it was possible to exclude a standard continuous i.v. infusion of morphine and an indwelling urinary catheter from our postoperative care protocol without decreasing the quality of pain relief in children submitted to SAS for sagittal synostosis. This finding supports downgrading the level of care from the ICU to a regular ward after limited immediate postoperative observation.

Premature craniosynostosis is a rare condition, with a wide range of incidence estimations in the literature. The aim of this study was to establish the current incidence among the Swedish population. Since the surgical care for these children is centralized to the 2 centers of Sahlgrenska University Hospital and Uppsala University Hospital, the 2 craniofacial hospital registries were examined for surgically treated children, all having a computed tomography verified diagnosis. Results show an incidence of 7.7 cases per 10,000 live births, including 0.60/10,000 syndromic craniosynostosis. Due to information programs among health care staff and a system for early diagnosis through rapid communication, these results seem to mirror the true incidence of craniosynostosis in the Swedish population. The updated incidence data will facilitate healthcare planning and make future studies of possible changes in craniosynostosis incidence more accurate.

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...

Background Adaptive behavior skills are important when assessing cognitive functions related to daily life; however, few studies have assessed these skills in patients treated for nonsyndromic craniosynostosis. In this study, the authors assessed the adaptive behavior skills of children treated for craniosynostosis and examined whether their outcomes are related to surgical technique. Methods The Adaptive Behavior Assessment System, 2nd Edition, parent report was used for children (age, 7 to 16 years) treated for sagittal (n = 41), metopic (n = 24), and other rare synostoses (n = 8). Background data, including intelligence quotient, were controlled for confounders. Results All evaluated children treated for craniosynostosis were estimated as lower in all aspects of adaptive behavior skills (full-scale, conceptual, social, and practical composites; effect size, 0.36 to 0.44) as compared with norms. The sagittal group showed shortcomings in social composite (effect size, 0.48) and subscales measuring self-care and self-direction, although no difference was observed between spring-assisted surgery and pi-plasty regarding outcomes of adaptive behavior skills. In addition, children treated for metopic synostosis showed results indicating shortcomings with adaptive behavior according to the full-scale, conceptual, and social composites (effect size, 0.53 to 0.61) relative to norms. Furthermore, attrition analysis revealed no significant differences between responders (rate, 80.2 percent) and nonresponders. Conclusion These results found that children treated for craniosynostosis display average adaptive behavior skills, and that the two surgical techniques used to treat sagittal synostosis did not differ in their behavioral outcomes. Clinical question/level of evidence Therapeutic, III.

The formation of the cranial sutures, in utero, occurs when the ossification of the skull bones reaches predestined positions around gestational week 15 to 20. Craniosynostosis, and the consequent skull shape deformities, is treated with surgery including osteotomies of the fused sutures. The occasional appearance of a new suture in the osteotomy lines has previously been described as sporadic events. In this retrospective study, a 4-year consecutive series of osteotomies combined with springs for craniosynostosis were systematically analysed regarding the appearance of neosutures. In total, 84 patients were included and in 16 patients (19%) a new radiologically normal suture appeared in a part of the suture that was completely closed preoperatively. Additionally, in 7 patients (8%) a new suture appeared in a part of the suture that had a discernible suture prior to surgery.In conclusion, in this consecutive and well-defined patient cohort operated for craniosynostosis, the formation of a neosuture is not a rare, and speculatively not a random, event. The appearance of a new suture long after the normal time period for suture formation in utero indicates that the craniosynostosis may just as well be caused by disturbed formation of the suture as actual premature closure.

The temporal contour deformity typical of metopic synostosis is often referred to as temporal hollowing, but has not been quantitatively defined. This deformity is present before surgery and remains to a varying extent at long-term follow-up. The present study aimed to objectively evaluate the degree of this contour deformity in metopic synostosis before and after surgical correction.All children surgically treated for metopic synostosis at Sahlgrenska University Hospital between 2002 and 2014 (n = 120) with appropriate computed tomography scans (n = 160) performed preoperatively and/or at follow-up at 3 years of age were included. Depending on age, 1 of 2 surgical techniques was used. Children presenting before the age of 6 months were treated with frontal remodeling in combination with a spring (S group), whereas children older than 6 months were treated with a bone transplant (BT group). The bony temporal deformity was measured with a semiautomatic MATLAB program and patients were compared to sex- and age-matched controls.The deformity was significantly reduced in both groups (P < 0.001). In the S group, it was reduced from a mean ± standard deviation of 3.6 ± 1.9% to 1.0 ± 1.2% and in the BT group, it was reduced from 3.3% ± 1.4% to 1.1% ± 0.8%.The contour deformity in metopic synostosis is present both before and after surgery and should therefore be termed temporal retrusion (TR). This assessment method enables objective comparison of TR before and after surgical correction and is a potential tool to evaluate TR in metopic synostosis.

INTRODUCTION Spring-assisted cranioplasty to correct sagittal synostosis is based on midline craniotomy through the closed sagittal suture, over the superior sagittal sinus (SSS). The aim of the present study was to evaluate the perioperative safety of this technique. MATERIALS AND METHODS This is a retrospective study of all patients operated with median craniotomy and springs from 1998 to the end of 2015. For comparison, all Pi-plasties performed during the same time interval were also evaluated. The safety measures were evaluated based on incidence of damage to SSS, incidence of dural tears, perioperative blood loss, operative time, and hospital stay. RESULTS In the group that had undergone midline craniotomy combined with springs (n = 225), 4 perioperative damages to SSS and 1 dural tear were seen. The perioperative blood loss was 62.8 ± 65.3 mL (mean ± standard deviation). The operative time was 67.9 ± 21.5 minutes and the hospital stay was 4.8 ± 1.1 days. In the group that had undergone pi-plasty (n = 105), no damages to SSS but 3 dural tears were seen. The perioperative blood loss was 352.8 ± 174.4 mL. The operative time was 126.0 ± 31.7 minutes and the hospital stay was 7.1 ± 1.4 days. CONCLUSION Craniotomy SSS in sagittal synostosis is a safe procedure with low morbidity in terms of damage to the SSS. Midline craniotomy combined with springs has significantly lower preoperative blood loss, operative time, and hospital stay (P

Introduction Infantile myofibroma (IM) is a benign mesenchymal disorder. Although it is the most common tumor of infancy, it is a relatively rare pathological entity. IM occurs predominantly before the age of two. It is characterized by tumor formation in skin, muscle, viscera, bone and subcutaneous tissues. The tumor can occur in either solitary or multicentric forms. The solitary form without visceral involvement usually has a benign course. Male gender predominates in the solitary form. Case presentation We report a clinical case of an osseous infantile myofibroma of the zygomatico-maxillo-orbital complex in a 4 months old male. We also show the radiological spontaneous regression without surgical intervention. Clinical, radiological, and histopathological examinations established the diagnosis. Our treatment was conservative with clinical follow-up. No surgical intervention was carried out during the course of the disease. Significant spontaneous regression occurred after a year and was confirmed by CT scan. Conclusion Radiologically aggressive infantile myofibroma has been previously treated by surgical intervention. In this case report there was a significant spontaneous regression. Conservative treatment and follow-up may be an appropriate alternative.

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. The aim of the study was to assess the sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis. To reduce selection bias, we included children that had previously undergone surgery for craniosynostosis, were between 8 and 16 years, and lived close to the craniofacial centre. The Connors Continuous Performance Test (3

Comparing complication rates between centres is difficult due to the lack of unanimous criteria regarding what adverse events should be defined as complications and how these events should be compiled. This study analysed all adverse events in a cohort of craniofacial (CF) operations over a 10-year period and applied three different scales (Clavien-Dindo, Leeds and Oxford) for systematic comparison. A total of 1023 consecutive CF procedures in 641 patients was identified. The Clavien-Dindo scale captured 74 complications in 74 procedures (7.2%), whereas the Leeds and Oxford scales captured 163 complications in 134 procedures (13.1%) and 85 complications in 83 procedures (8.1%), respectively. The Clavien-Dindo scale appeared less suitable for CF surgery, because it is predominantly adapted to severe complications and also regards blood transfusion as a complication. The Leeds scale provided a detailed picture of all complications, as well as minor events, whereas the Oxford scale captured all major complications well but applied less accurate definitions for the minor events. Our findings contribute to the benchmarking of complications between CF centres and suggest that both the Leeds and the Oxford scale appear relevant, depending on the emphasis required for major and minor complications and inter-centre audits, respectively.

BACKGROUND Long-term neuropsychological and cognitive outcomes in patients with nonsyndromic craniosynostosis have proven difficult to evaluate objectively because of methodologic problems with published studies based on their small and biased samples of patients, wide age ranges, and testing with unacceptable psychometric properties. This study evaluated the Full-Scale Intelligence Quotient and its subscales in a cohort with a small selection bias. METHODS Patients aged 7 to 16 years, born with nonsyndromic craniosynostosis and surgically treated, were tested using the Wechsler Intelligence Scale for Children, Fourth Edition. Ninety-one patients were invited, and 73 patients were tested. RESULTS There was no difference in Full-Scale Intelligence Quotient score between patients who had undergone operations for sagittal synostosis or metopic synostosis and norms provided by the test. Patients operated on for sagittal synostosis showed a significantly higher perceptual reasoning intelligence quotient, but also significantly lower working-memory intelligence quotient and processing-speed intelligence quotient compared with the norms. Patients operated on for metopic synostosis showed no differences in any intelligence quotient index compared with the norm. In addition, attrition analysis showed no differences in background factors between responders and nonresponders. CONCLUSIONS These results derived from a group of patients with uniform age range, and tested using an established tool, revealed that nonsyndromic children having undergone surgery for craniosynostosis exhibited average intellectual ability. However, the analysis indicated possible issues with working memory and processing speed in patients operated on for sagittal synostosis, highlighting impairments potentially associated with neuropsychological problems and that might contribute to learning disabilities.

INTRODUCTION Craniosynostosis is a premature closure of a cranial suture. Cranioplasty is indicated to correct skull deformity, relieve increased intracranial pressure, and promote homogenous cranial growth. Different techniques have been adopted to achieve optimal outcomes. Although surgical benefits are widely accepted, this intervention might also affect cranial skeletal growth. METHODS The authors conducted a retrospective case-control study including patients operated for isolated metopic or sagittal synostosis. These patients had undergone a computed tomography (CT) scan before surgery and/or at 3 years of age postoperatively. These were operated between 2002 and 2012. Intracranial volume was measured using a MATLAB application. The control group was age and sex-matched individuals who had CT scans for trauma or neurological indications. All results with P value

Background The aim of the current study was to determine the intracranial volume (ICV) and cephalic index (CI) in patients operated for sagittal synostosis, and to compare the outcome of 2 different surgical techniques: craniotomy combined with springs and modified pi-plasty. Methods The authors studied all patients who had been operated for isolated sagittal synostosis and registered in the Gothenburg Craniofacial Registry until the end of 2012 and who had undergone a preoperative and/or postoperative (at 3 years of age) computed tomography examination. Sex- and age-matched controls were identified from children who had undergone computed tomography for other reasons. Results Craniotomy combined with springs increased the ICV and CI from 802 ± 127 mL (mean ± SD) and 70.1 ± 4.0 to 1300 ± 158 mL and 73.1 ± 3.3, respectively. The corresponding values for controls were 796 ± 136 mL and 83.6 ± 7.3 preoperatively and 1334 ± 136 mL and 80.0 ± 4.5 at 3 years of age. Pi-plasty increased the ICV and CI from 1014 ± 115 mL and 69.7 ± 3.3 to 1286 ± 122 mL and 74.1 ± 2.6, respectively. Corresponding values for controls were 1043 ± 153 mL and 83.4 ± 7.0 preoperatively and 1362 ± 122 mL and 79.6 ± 3.9 at 3 years of age. Conclusions There was no significant difference between craniotomy combined with springs in children younger than 6 months and pi-plasty in older children regarding the efficacy of improving ICV and CI. Neither of the techniques fully normalized the head shape.

Metopic synostosis results in a keel-shaped forehead, reduced frontal intracranial volume (ICV), and lower frontal to total volume ratio. The ratio improves with cranioplasty, but at 3 years of age, the ratio is still not normalized when compared to that in normal children. The aim of the present study was to investigate whether a low frontal to total ICV ratio at 3 years of age was due to relapse or insufficient correction.All children surgically treated for metopic synostosis in combination with a spring at Sahlgrenska University Hospital with subsequent spring extraction between 2002 and 2008 (n = 20) were included. A MATLAB program was used to measure frontal and total ICV.Preoperatively, the frontal to total ICV ratio was 9.8 ± 1.3% (mean ± standard deviation). At spring removal, 6 months after cranioplasty, the ratio had increased to 11.8 ± 2.4%. At 3 years of age, the ratio was 11.6 ± 1.9%. In age-matched normal children, the ratio was 14.4 ± 1.9% preoperatively, 15.3 ± 2.2% at time of spring extraction, and 13.4 ± 1.4% at 3 years of age.Cranioplasty thus improved the frontal to total ICV ratio, but did not normalize it. The ratio did not change from 6 months after the cranioplasty to 3 years of age. These results indicate that a more pronounced frontal volume correction during cranioplasty is necessary to achieve a normalized distribution of ICV in metopic synostosis.

Multisutural nonsyndromic craniosynostosis is a rare group of malformations, whose frequency has been reported between 3% and 7% of all craniosynostosis. The clinical diagnosis can be difficult and computed tomography is usually required. Surgical treatment is challenging and staged procedures are performed in up to 80% of patients. The aim of the present study was to determine the reoperation rate and to evaluate the surgical outcomes by measuring intracranial volume (ICV) preoperatively and at follow-up, and comparing it to a control group. Perioperative variables and reoperation rate were recorded. Fifty-one patients presented with a complex pattern of synostosis without a recognizable syndrome (5% of cases of total patients evaluated). Fifteen patients have been treated with spring-assisted surgery, either alone or in combination with a foreheadplasty. The mean follow-up was 6.2 years. The mean preoperative ICV of the patients was smaller, but not significantly, than in the normal population (P = 0.13). Postoperatively, the mean ICV was similar to that of the control group at 1 year (P = 0.92), while at 3 years it was appreciably smaller, although not significantly different (P = 0.06). Five patients (33%) went through a secondary skull expansion for either raised intracranial pressure or cosmetic reasons. Spring-assisted surgery seems to temporarily expand ICV in children with complex synostosis and lower the reoperation rate, thus reducing the need for a second procedure. A longer follow-up would be necessary to further investigate the effects of springs over time.

Background/Aims: Cranioplasty is often accompanied by a substantial oozing bleeding from the bone surface and bone edges. Our aim was to measure if strict subgaleal dissection without any periosteal release reduces blood loss during pi-plasty surgery for sagittal synostosis. Method: A group of 32 children who underwent pi-plasty surgery at the Sahlgrenska University Hospital between 2010 and 2014 for premature sagittal synostosis with traditional subgaleal dissection combined with incision and release of the periosteum adjacent to the osteotomy lines was compared to a group of 7 children who underwent pi-plasty with strict subgaleal dissection and osteotomy through the bone with the periosteum attached. Information about blood loss and body weight was extracted from medical records. Results: The blood loss in the group of 7 children with strict subgaleal dissection was 102 ± 86 mL (mean ± SD) (10 ± 7 mL/kg) compared to 320 ± 119 mL (32 ± 12 mL/kg) in the control group with traditional periosteal release (p < 0.001). Conclusion: Intact periosteum at the osteotomy lines significantly reduces blood loss in pi-plasty surgery for sagittal synostosis. The mechanism is likely because of preserved veins between the bone surface and periosteum.

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.

BACKGROUND: Unicoronal synostosis presents with cranial asymmetry. Fixed points are difficult to identify; surgical results are therefore difficult to evaluate. The aim of this study was to develop a computer-based method for evaluation of forehead symmetry to enable evaluation of surgical results in unicoronal synostosis. METHODS: The MATLAB tool was programmed to segment computed tomographic images, leaving the outermost contour. Cephalometric images were segmented manually due to lower contrast. A center-point (O) and an end-point were manually defined in the midline of the forehead and at the nonfused coronal suture, respectively. The program then found a point (p) on the fused side, at the same distance from the O as the end-point. The contours of the left and right side of the forehead were thereafter superimposed, and the position of minimal area mismatch of the sides was identified. To correct for growth between preoperative images and follow-up, the number of mismatching pixels was related to the area outlined by the contour of the forehead, the end-point and p. Two quantities, the relative symmetry change and the absolute symmetry change, were defined and evaluated by repeated measurements on spherical and elliptical phantoms and 15 patients. RESULTS: Measurements with the MATLAB program were reliable with an SD of 0.26% to 5.39% for the expected range of differences. The SD was lower for measurements on computed tomographic images than for measurements on cephalometric images. The SD was also lower in patients with large surgical improvement than in patients with little improvement. The results support the use of relative symmetry change to evaluate surgical results. CONCLUSIONS: Our new computer-based method is capable of measuring forehead symmetry with good precision. This method can be used for systematic evaluation of surgical outcome for unicoronal synostosis and other asymmetric skull deformities.

Premature craniosynostosis is a congenital disorder causing a skull deformity. For both functional and cosmetic reasons, the deformity is surgically treated with a cranioplasty before the age of 1 year. Temporal hollowing is a common and undesirable remaining deformity after cranioplasty for metopic synostosis. The most common method to determine the degree of temporal hollowing is subjective judgement of the temporal region. The aim of the present project was to develop a quantitative semi-automatic computer tool for objective measurement of bony temporal hollowing.Using MATLAB, a tool was developed to segment computed tomography images, defining the outermost contour. The images were dorsally limited to the widest point of the head. In each case, a sex- and age-matched control was identified and the contours compared. The bony temporal hollowing of the cases was calculated.The intra-user coefficient of variation (CV) was 5.0% (95% CI = 4.2%-6.2%) and the inter-user CV was 3.0% (95% CI = 2.1%-8.6%). For clinical testing purposes, the tool was used in 14 patients, seven of whom had been operated on with a spring-assisted cranioplasty and seven with a cranioplasty using a bone graft.In summary, this study presents a new tool for objective measurement of the surgical result after cranioplasty for metopic 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.

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 Goteborg 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.

Metopic synostosis, apart from the pointed forehead, typically is characterized by hypotelorism with egg-shaped orbits on cephalography and the frontoorbital axis parallel or even converging superiorly. The frontoorbital axis angle is a novel parameter for analyzing and describing the orientation of the orbits. Current methods of surgery often result in undercorrection of the almost ever-present hypotelorism. The present study was performed to analyze a new technique, capable in this respect, using steel wire springs in conjunction with a cranioplasty.A retrospective study of 23 metopic synostosis patients operated on between 1999 and 2004 was conducted. A strip midline craniectomy and frontal reshaping were combined with the insertion of a steel wire spring across the midline craniectomy, forcing lateral displacement of the orbits. Preoperative and postoperative follow-up cephalograms were obtained, and the bony medial interorbital distance was measured and compared with the bony medial interorbital distance of a control group. Perioperative data and complications were noted.Preoperative mean bony interorbital distance was 10.6 mm (range, 7.7 to 13.2 mm). It increased to 15.7 mm (range, 10.4 to 22 mm) at 1.5 months postoperatively and to 16.2 mm (range, 10.9 to 24.5 mm) 5 months postoperatively. Results as judged clinically ranged from little effect to a definitive overcorrection. The frontoorbital axis was improved in every case. Average frontoorbital axis was -4 degrees (range, -33 to 23 degrees) preoperatively and 28 degrees (range, 11 to 46 degrees) postoperatively.It was concluded that a spring used together with a cranioplasty is a powerful tool for the correction of both hypotelorism and orbital shape in trigonocephaly.

Premature sagittal synostosis (SS) can be surgically corrected using extensive cranioplasties or using less invasive methods, e.g. spring-assisted surgery (SAS).The aim of the present study was to perform a proper systematic review of springs in the treatment of SS.A literature search was performed with the assistance of a professional librarian in the databases PubMed, EMBASE, and The Cochrane Library between 1997 and September 2013. Studies that fulfilled the PICO (patients, interventions, controls, outcome) criteria were included. All studies were graded for methodological quality according to MINORS, and all retrospective studies were assessed according to a scale developed for retrospective studies in paediatric surgery. The quality of evidence was rated according to GRADE.A total of 241 abstracts were extracted in the literature search. Five studies met the PICO criteria. Two of these five were considered as preliminary reports and excluded. Assessment according to MINORS showed a mean score of 21, i.e. fair quality. The clinical outcome regarding cephalic index did not differ between the surgical techniques, but the quality of evidence, according to GRADE, that SAS was equally efficient, was very low. Clinical outcome regarding operation time, blood loss, ICU stay and hospital stay was in favour for SAS, but the quality of evidence was low.This systematic review has revealed that the level of evidence for SAS being an equally efficient surgical method as more extensive cranioplasties for SS is low or very low. The results point out the need for well-designed prospective studies within craniofacial surgery.

AIM d-myo-inositol-1,2,6-trisphosphate (alpha-trinositol, PP56), is a synthetic isomer of the intracellular second messenger, d-myo-inositol-1,4,5-trisphospahate. The pharmacological actions of alpha-trinositol include potent anti-inflammatory properties and inhibition of the secretion induced by cholera toxin and obstructive ileus. In the present study, we investigated whether alpha-trinositol was able to influence the secretion induced by heat-stable ST-toxin from Escherichia coli in the rat jejunum. METHODS A midline abdominal incision was performed in anaesthetized male Sprague-Dawley rats and a 6-7 cm long jejunal segment was isolated with intact vascular supply and placed in a chamber suspended from a force displacement transducer connected to a Grass(R) polygraph. Intestinal net fluid transport was continuously monitored gravimetrically. Crystalline ST-toxin (120 mouse units) was introduced into the intestinal lumen and left there for the rest of the experiment. When a stable secretion was observed, alpha-trinositol (60 mg kg-1 h-1) or saline were infused during 2 h, followed by a 2-h control period. RESULTS alpha-Trinositol induced a significant (P < 0.001) inhibition of ST-toxin secretion within 30 min, lasting until 2 h after infusion had stopped. The agent also moderately increased (P < 0.05) net fluid absorption in normal jejunum. Mean arterial pressure (P < 0.001) and heart rate (P < 0.001) were reduced by alpha-trinositol. CONCLUSION The inhibition by alpha-trinositol of ST-toxin induced intestinal secretion is primarily secondary to inhibition of secretory mechanisms and only to lesser extent due to increased absorption. The detailed mechanisms of action have not been clarified but may involve suppression of inflammation possibly by means of cellular signal transduction.

The fire disaster in Gothenburg, Sweden, 1998 killing 63 and wounding 213 teenagers was caused by arson committed by a youth from the same community. The fire was started in the basement of an overcrowded discotheque and made, due to unfortunate circumstances, devastating progress. The ensuing rescue work performed by other youth, fire fighters, police and medical staff was prompt and must be seen in the light of a very difficult situation. As a result of these orchestrated efforts and the fact that this disaster occurred in a major city with substantial resources, all the injured were able to be hospitalized within 2h. The load on four local hospitals was initially severe due to the large number of injured and the limited number of staff on night duty. The situation was contained by relocating patients from the intensive care units to ordinary wards and by transporting several of the most severe burn injuries by helicopter to burn units in other parts of Sweden and to Norway. Hundreds of relatives and friends gathered at the local hospitals. This was a new experience for the hospitals and staff, involving many positive aspects as well as some negative aspects such as violence, threats and rumors. As a result of the large number of injuries vast psychosocial rehabilitation program was initiated by health care staff, religious communions, schools and the community, has continued over the past years. Such a disaster emphasises a requirement for extensive preparation not only in the rescue and medical services, but also in the ways and areas to rehabilitate patients in society.

Burn pain is often long-lasting, severe and intermittently excruciating due to repeated wound dressings, skin grafts, reconstructive surgery, or other interventional procedures [1]. The severity of pain has established the use of potent opioid analgesics as a standardised mean of inducing analgesia, although pain levels and analgesic requirements are often underestimated in burn patients [2]. A major side-effect encountered in burn patients receiving high doses of opioid analgesics is the pronounced respiratory depression induced by the agents [3], which prompt the use of ventilator support. In the aftermath of the discotheque fire disaster in Gothenburg, 213 patients were triaged within 2 h to four hospitals in the city after having suffered deep partialor full-thickness skin burns (31 patients) and/or smoke inhalation injuries (158 patients). A significant number of victims required ventilator care either to prevent respiratory failure due to administration of large doses of opioid analgesics and/or to maintain adequate oxygenation secondary to airway injuries, thereby surpassing ventilator capacity. This and similar situations, presenting a severe challenge to limited resources, has urged us to investigate alternative techniques for inducing efficient analgesia in burn patients without the troublesome side-effects of opioid analgesics [4]. A decade ago we presented results on the use of continuous intravenous infusions of lidocaine to produce potent analgesia in major burns without inducing respiratory depression and with few other side effects [5], thereby reducing the need for ventilator support. We present here a case from the discotheque fire disaster in Gothenburg having received this treatment and being particularly illustrative as the patient came to serve as his proper control.

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 and180 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.

Previous studies have shown that local anaesthetics possess a wide range of effects on the pathophysiology of burns, including inhibition of burn oedema and inhibition of progressive burn ischemia. The present randomised double-blind cross-over study in six volunteers investigated the effects of intravenous lidocaine infusion on partial thickness skin burns. A thermoprobe was used to induce a standardised thermal injury (1 cm(2)) on the flexor side of one forearm and was repeated on the opposite side 1 week later. Subjects received either an intravenous bolus dose of lidocaine (1 mg kg(-1)) immediately after the thermal trauma followed by continuous intravenous infusion of lidocaine (40 microg kg(-1) min(-1)) during 4 h or equal volumes of isotonic saline. Macrophotographs of the experimental skin area were taken preburn and 1, 2, 3, 4, and 12 h postburn and evaluated by computerised image colour analysis using normalised rgb (n-rgb) and Hue-Saturation-Intensity (HSI) colour systems as a quantitative measure of pathophysiological events. Maximum erythema occurred 2-3 h postburn. Differences between lidocaine- and placebo-treated burns were not significant during the first 4 h postburn. At 12 h postburn, the lidocaine-treated burn demonstrated a significantly faster restitution of residual erythema compared to control sites. The present study shows that intravenous lidocaine significantly inhibits the long-term inflammation-induced tissue responses to thermal trauma.

Vascular changes following deep skin burns are characterised by vasoconstriction and progressive ischemia. Nitric oxide (NO) has been shown to be a potent regulator of vascular smooth muscle tone and tissue perfusion. We assessed the importance of NO on post-burn skin perfusion in rats using laser Doppler. The present results show that neither the NO-synthase inhibitor, NG-nitro- l -arginine ( l -NNA) ( n =6) nor the NO precursor, l -arginine, significantly influenced skin perfusion in nonburned skin compared to saline-treated animals. In the area of full-thickness skin burn, neither l -arginine ( n =6) nor l -NNA ( n =6) had significant influence on post-burn perfusion compared to saline-treated controls ( n =6). Administration of l -NNA ( n =6) significantly impaired skin perfusion in the area adjacent to the contact burn representing a partial-thickness burn, while the NO precursor, l -arginine ( n =6) had no significant effect on burn perfusion as compared to saline-treated controls ( n =6). In conclusion, impairment of perfusion in a full thickness burn following administration of NO-synthase inhibitor suggests that nitric oxide is involved in the mechanisms responsible for maintaining adequate circulation post-burn. The lack of additional improvement of perfusion in response to l -arginine may suggest that NO synthesis in response to the thermal trauma is already at a peak.

Burn injuries trigger a pronounced inflammatory response in the burned skin, resulting in oedema formation and impaired circulation. This response involves activation of the nitric oxide (NO) synthetic pathway, which could play a key role in the complex hemodynamic and hemostatic changes occurring as a result of a burn trauma. The results presented in full-thickness skin burns of rats show that the NO-precursor, L-arginine (n = 10), inhibit burn-induced plasma extravasation as compared to saline-treated burned controls (n = 10) (p

Bacground: Previous studies have demonstrated potent inhibition of burn oedema and progressive ischaemia by local anaesthetics. Since eicosanoids have been suggested to play an important role in the pathophysiology of burns, we compared in the present ex vivo study the effects of topical lidocaine/prilocaine cream (EMLA®, ASTRA, Sweden) and intravenous lidocaine with that of saline on eicosanoid formation by normal and burned rat skin. Methods: A full-thickness burn trauma was induced in the abdominal skin. All the agents were given 5 min postburn until 2 h after the trauma. The experimental skin was subsequently removed and incubated in Krebs solution for 1 h. Eicosanoid concentrations in the solution were analysed by radioimmunoassay. Results: EMLA® cream induced a significant inhibition of TXB2 (P

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.

The anti-inflammatory agent D-myo-inositol-1,2,6-trisphosphate (1,2,6-IP3) has shown beneficial effects in experimental burns following systemic administration. The purpose of this study was to investigate the effect of topical 1,2,6-IP3 cream on a standardised full-thickness 1 cm2 burn injury in rats. The experimental cream contained a transcutaneous absorption enhancer, hexylbetaine. Five different treatment groups were used. Two experimental groups of burned rats received either 1,2,6-IP3 cream with hexylbetaine (n = 10) or without hexylbetaine (n = 10). Two burned control groups were treated either with hexylbetaine cream (n = 10) or placebo cream (n = 10), while a third control group was untreated (n = 14). The various creams (0.5 g) were administered to the experimental burn area and allowed to remain for 3 h covered with an occlusive dressing. Spectrophotometrical quantification of Evans blue albumin extravasation was used to evaluate the effect of the experimental creams on vascular permeability following the burn trauma. Results showed a significant reduction of albumin extravasation both by 1,2,6-IP3 (p0.05) and by hexylbetaine alone (p0.01), as compared to placebo cream-treated animals. The transcutaneous absorption enhancer hexylbetaine did not further improve the effect of 1,2,6-IP3 on burn oedema. In conclusion, both topical 1,2,6-IP3 and hexylbetaine induced a significant reduction of albumin extravasation in burned skin. The effect of 1,2,6-IP3 could be related to previously shown anti-inflammatory actions of the agent, while the mechanisms of actions of hexylbetaine remain to be investigated.

Deep partial-thickness burn injury was induced in the abdominal skin of anesthetized rats. Dermal perfusion was assessed by laser Doppler flowmetry. In the first set of experiments, one group of rats (n = 15) was topically treated with a lidocaine-prilocaine cream 5% (25 mg of each in 1 g) for 6 hours, starting 5 minutes after inducing the burn injury. In one control group (n = 14), the thermal injury was treated with placebo cream. Results showed a markedly reduced perfusion in the skin of the control animals within the first hour after burn injury, with further decrease during the following 5 hours of observation. In animals treated with the lidocaine-prilocaine cream, skin perfusion in the burned area was significantly increased during the first 30 minutes after the burn injury compared to before the burn (p < 0.01), followed by a decrease to a level below the preburn stage but significantly higher than that of control animals during the first hour after burn injury (p < 0.05). As opposed to burned control animals, skin perfusion gradually recovered toward preburn levels at the end of the experiment in local anesthetic-treated animals. In the second experimental set, four groups of animals were burned and subsequently treated with a bolus dose of lidocaine intravenously (2 mg/kg), followed by continuous intravenous lidocaine infusions at a rate of 50 (n = 10), 100 (n = 11), or 150 (n = 10) micrograms.kg-1.min-1. The infusions were started 5 minutes after the burn injury and lasted for 6 hours. Corresponding volumes of saline solution were given to burned control animals (n = 10). Results showed a significantly improved skin perfusion in the lidocaine-treated group in a dose-response fashion as compared to control animals. A maximum improvement of dermal perfusion in the burned area was induced by intravenous lidocaine at an infusion rate of 150 micrograms.kg-1.min-1 as compared to burned controls treated with isotonic saline solution infusions (p < 0.01). Results showed that topical or systemic administration of local anesthetics can prevent progressive dermal ischemia after thermal injury.

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.

Surgical correction of trigonocephaly is performed by fronto-orbital reshaping and advancement. Some authors supplement the frontoorbital 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.

Full-thickness burn injury results in a continuous deterioration of blood flow due to vascular sludging, thrombosis formation and oedema leading to irreversible ischaemia and tissue necrosis. D-myo-inositol-1,2,6-trisphosphate (IP3) has previously been shown to reduce burn-induced oedema formation and inflammation involved in the pathophysiology of progressive ischaemia. A full-thickness burn injury (1 cm2) was induced in the abdominal skin of anaesthetized rats using an electrically heated thermoprobe. Blood flow in the experimental area was measured by laser Doppler flowmetry during 6.5 h postburn. The experiments included five groups. Three burned groups were treated intravenously with IP3 and received respectively: a bolus dose of 4 mg/kg followed by a continuous intravenous infusion of 20 mg/kg/h, 8 mg/kg + 40 mg/kg/h or 16 mg/kg + 60 mg/kg/h. One burned and one unburned control group received a corresponding bolus dose and infusion of saline. Results showed a significant inhibition of dermal ischaemia in the burned groups receiving IP3 at all dose intervals as compared to saline-treated burned rats (all P < 0.001). We conclude that IP3 improved local dermal perfusion in burned skin. Probable mechanisms of action could be the vasodilatory and anti-inflammatory properties of the agent.

This study investigated the effects of the anti-inflammatory agent D-myo-inositol-1,2,6-trisphosphate (IP3) on burn edema. Two sets of experiments were performed. In the first set, a full-thickness burn injury was induced in the abdominal skin of anesthetized rats. Postburn intravenous treatment was given with IP3, indomethacin or saline solution. Extravasation of Evans blue albumin in the burned tissue was quantified by a spectrophotometric technique. Results showed significant inhibition of albumin extravasation by IP3 in three of five different doses compared to saline-treated animals. In the second set of experiments, a deep full-thickness burn through the abdominal skin and rectus muscle was induced. The therapeutic window of IP3 could be more well-defined. Resulted showed a significant reduction of albumin extravasation in the skin at all four dose levels and in the abdominal muscle at three of four doses. Indomethacin had no significant effect on postburn edema formation. The mechanisms responsible for the inhibition of albumin leakage by IP3 could be secondary to reduced formation of edema-promoting inflammatory mediators by the agent, resulting in improved vascular patency.

In the field of maxillofacial reconstruction, additive manufacturing technologies, specifically electron beam melting (EBM), offer clinicians the potential for patient-customized design of jaw prostheses, which match both load-bearing and esthetic demands. The technique allows an innovative, functional design, combining integrated porous regions for bone ingrowth and secondary biological fixation with solid load-bearing regions ensuring the biomechanical performance. A patient-specific mandibular prosthesis manufactured using EBM was successfully used to reconstruct a patient's mandibular defect after en bloc resection. Over a 9-month follow-up period, the patient had no complications. A short operating time, good esthetic outcome, and high level of patient satisfaction as measured by quality-of-life questionnaires-the European Organisation for Research and Treatment of Cancer QLQ-C30 (30-item quality-of-life core questionnaire) and H&N35 (head and neck cancer module)-were reported for this case. Individually planned and designed EBM-produced prostheses may be suggested as a possible future alternative to fibular grafts or other reconstructive methods. However, the role of porosity, the role of geometry, and the optimal combination of solid and porous parts, as well as surface properties in relation to soft tissues, should be carefully evaluated in long-term clinical trials. (C) 2016 American Association of Oral and Maxillofacial Surgeons