Outcomes of 40 Nonsyndromic Sagittal Craniosynostosis Patients as Adults: A Case-Control Study With 26 Years of Postoperative Follow-up

Outcomes of 40 Nonsyndromic Sagittal Craniosynostosis Patients as Adults: A Case-Control Study... Abstract BACKGROUND While sagittal synostosis is the most common craniosynostosis, long-term follow-up of these patients is lacking. OBJECTIVE To evaluate the results of surgical management of those patients with sagittal synostosis who attain adulthood. METHODS An outcome study of surgically treated isolated sagittal synostosis patients operated between 1977 and 1998 was conducted at the Craniofacial Center of Oulu University Hospital, Oulu, Finland with an average follow-up time of 26.5 yr. Patients’ socioeconomic situation, satisfaction with their own facial appearance and attractiveness as rated by 2 independent panels was evaluated and compared to controls. RESULTS The self-satisfaction with the patients’ own appearance scored a mean of 75 mm on a visual analog scale of 100 mm between the patients and 76 mm with the control group. The subjective satisfaction of the patients with their own appearance failed to correlate with the rating of their appearance by the panels. The panels rated the patients’ appearance to be on average 6 to 7 mm out of 100 mm visual analog scale less attractive than the controls. Data on socioeconomic situation, including marital status, housing, education, employment of the patients, and controls are presented. CONCLUSION Isolated sagittal synostosis patients treated surgically were as happy with their facial appearance as were individuals in an age and gender-matched control group. Two independent panels found the patients’ appearance to be only somewhat less attractive. Analysis of the socioeconomic situation and general health revealed that patients equaled that of controls. Adult, Craniosynostoses, Aesthetics, Outcome assessment, Sagittal synostosis, Scaphocephaly ABBREVIATIONS ABBREVIATIONS MCID minimally clinically important difference QOL quality of life VAS visual analog scale Premature fusion of the sagittal suture is the most common type of craniosynostoses, comprising 40% to 60% of all cases.1 The patients have a classic scaphocephalic head shape resulting from a diminished skull height and width with compensatory anteroposterior growth.2 Surgical procedures to manage sagittal craniosynostosis have changed over the decades. Originally, the fused suture was released by making strip craniotomy lines on both sides of the suture. Later strip craniectomy of the ossified suture itself was performed.3,4 Currently more extensive calvarial vault reconstructions are preferred5,6 or endoscopic strip craniectomies are done followed by postoperative molding therapy with helmets.3,7 At Oulu University Hospital the early operative method included 2 parallel strip craniotomies lateral to the midline, while a strip of bone was left over the sagittal sinus according to Lannelongue.4 Later additional silicone membranes were used on the craniotomy lines8 (Figure 1A). This technique was supplanted by suturectomy, usually supplemented with split dural interpositioning, in the early 1980s. For dural split, the outer layer of the dura was cut parallel to the sagittal sinus on both sides of it, then dissected from the deeper layer, turned over the newly cut bony edges and sutured to the periosteum (Figure 1B).9 This method was replaced by the “H-technique” (Figure 1C).5 FIGURE 1. View largeDownload slide Different techniques used for the treatment of sagittal craniosynostosis in the Oulu University Hospital. A, The Lannelongue and Ingraham suture “unlocking” technique with and without silicone membrane interposition. B, Lane strip craniectomy with and without dural splitting. C, “H” cranioplasty with and without additional cuts to the temporal bone. FIGURE 1. View largeDownload slide Different techniques used for the treatment of sagittal craniosynostosis in the Oulu University Hospital. A, The Lannelongue and Ingraham suture “unlocking” technique with and without silicone membrane interposition. B, Lane strip craniectomy with and without dural splitting. C, “H” cranioplasty with and without additional cuts to the temporal bone. From a clinical viewpoint, surgically treated sagittal craniosynostosis patients are expected to have a minimal risk of late sequelae. Clinical follow-up is usually continued no later than school age. There are several studies suggesting single suture craniosynostoses are associated with mild to moderate developmental and educational difficulties that become apparent at school age.10-12 However, patients with sagittal synostosis have the best performance in neuropsychological tests.12 Furthermore, all craniosynostosis may have secondary psychosocial effects. Some patients retain residual aesthetic facial differences that might attract unfavorable attention from strangers and rejection from peers. Such attention may negatively influence social behavior and result in poor school performance with difficulties in educational environments.13 When studying persons treated for severe malocclusions, it was found that the quality of life (QOL) and self-perceived aesthetic satisfaction improve extensively after treatment.14 These variables may be determinant of the outcomes among craniosynostoses patients, as well, in the future. However, in cases with syndromic craniosynostosis, facial differences may not influence the subjective QOL.15 The current study aimed to evaluate the aesthetic outcomes in adulthood of cases treated surgically in early childhood for sagittal craniosynostosis. The evaluation of the social situation was another goal of this study. METHODS Study Patients This was a cohort outcome study of patients and controls operated for sagittal synostosis at the Craniofacial Center of Oulu University Hospital, Oulu, Finland between 1977 and 1998. Only cases of patients older than 18 yr of age at the time of the follow-up examination were included in the study. Patients with any craniofacial syndromes or associated neurological disease were excluded. There were 171 operations for craniosynostosis during the study period. Of these, 115 patients satisfied the inclusion criteria and attempts were made to reach them (Figure 2). Contact was made with 83 patients and 61 agreed to participate in the study. Twenty of these patients had either metopic, coronal, lambdoid, or multiple suture synostosis and were not included. A single patient with both sagittal synostosis and hydrocephalus was excluded from the study. The mean age of the 40 patients (25 males, 15 females) with nonsyndromic sagittal synostosis was 27.4 yr (range 18-41). The follow-up time after operation was a mean 26.5 yr (range 17-37). Controls who were age and gender matched (n = 40) were selected randomly from Finnish Population Register Centre. Invitations were sent to both patients and controls to attend a follow-up visit by which time the subjects had completed their questionnaires as requested. The facial appearance of the patients was examined during the control visit that also included evaluation of their head shape, the scar, and palpation of the head. Standardized photographs were obtained (Figure 3). The follow-up visits took place between November 2014 and February 2016. FIGURE 2. View largeDownload slide Flow chart of the patients included into the study. FIGURE 2. View largeDownload slide Flow chart of the patients included into the study. FIGURE 3. View largeDownload slide An example of the slide with photographs as presented for the evaluation of aesthetic results of the surgery by panelists. An image of a healthy person not included in the study is present with his consent. On the slides presented to the panels the eyes of the study persons were not blinded. FIGURE 3. View largeDownload slide An example of the slide with photographs as presented for the evaluation of aesthetic results of the surgery by panelists. An image of a healthy person not included in the study is present with his consent. On the slides presented to the panels the eyes of the study persons were not blinded. Operative Treatment of the Patients Operative treatment included linear parasagittal craniotomies with silicone membrane interposition (n = 9), suturectomy together with dural split (n = 4), suturectomy without dural split (n = 3), and various forms of H-plasty with or without barrel stave osteotomies of the temporal bone (n = 24; Figure 1). The coronal and lambdoid sutures were kept intact in all cases. Mean age when surgery was performed was 5.7 mo (ranging from 9 d up to 45 mo). While 38 patients were operated only once, 2 cases required reoperation because of residual scaphocephalic head shapes. Of these 2 cases, 1 was first operated at the age 9 d and then reoperated at the age of 10.5 mo. The other case was first operated at the age of 3 mo and then reoperated at the age 2 yr. Aesthetic Evaluation using Photographs Long-term aesthetic evaluation of the clinical outcome was the major goal of the clinical evaluation. Facial aesthetic appearance was rated by 2 independent panels. The panel members did not know which of the evaluated persons had undergone surgery and who were controls. One panel consisted of either orthodontic residents or consultants (1 male and 3 females) and 1 (female) orthognathic surgeon. This panel was named the “dentists’ panel.” Then a second panel that consisted of lay persons had no health care education (1 male and 3 females) were called the “lay panel.” Panelist evaluators were presented with a series of randomly ordered slides with photographs of the patients and the controls. Each slide consisted of 4 images in standardized projections comprising: anterior view smiling and not smiling, with lateral and oblique views as well (Figure 3). Slides were displayed for exactly 10 s, in order to acquire the immediate initial panelist impression. A 100 mm visual analog scale (VAS) with 0 mm as least attractive and 100 mm representing most attractive was used to determine the cosmetic outcomes. Self-reported Questionnaire The self-reported questionnaire contained questions regarding the participants’ education level, housing status, marital or relationship status, employment status, general health with attention to their history of headaches, and the presence of mental disturbances. The education level was classified into 3 groups: no professional education, professional secondary education, and professional tertiary education. The first group included those with no more than secondary nonvocational education accomplished. The second group included participants who had completed lower level vocational education, or any academic education with the degree below Bachelor. The third group included those with higher level vocational education or academic education of at least Bachelor level. Participants were also directed to express their subjective satisfaction with their own facial aesthetics using a 100 mm VAS. The question was: “How satisfied are you with your current facial appearance?” A VAS of 0 mm referred to “very unsatisfied” whereas a VAS of 100 mm corresponded to “very satisfied.” Another question was “Is there something that bothers you in your facial appearance?” The patients were also asked whether their scar bothered them. Statistical Analysis Analyses were performed between the matched case-control pairs using paired samples t-test and McNemar tests. When comparing the difference between the groups, Pearson chi-square tests and Fischer exact test were used. The degree of the panels’ interobserver reliability was studied using intraclass correlation coefficient both between the panels and also inside the panels amongst the panel members. When comparing 2 panels, the reliability coefficient was almost perfect (0.857) when the patient groups were rated and substantial (0.777) for control group, and 0.840 when whole group of participants rating was analyzed. The reliability within the 2 panels among panel members was moderate being P = .573 in the case of the dentist panel and P = .555 with the lay panel. Statistical significances were set at P < .05. All statistical analyses were performed using the commercially available SPSS software for Windows 14.0 (IBM Inc, Armonk, New York). Ethical Aspects The study was conducted in accordance with the Helsinki declaration. The study was reviewed and accepted by the Ethical Review Board of the Northern Ostrobothnia Hospital District (number 86/2013). Both the patients and their controls signed informed consent forms. RESULTS Aesthetic Outcome According to Panel Evaluation In the case of facial appearance evaluation by the dentist panel, the patients received lower ratings than their controls (VAS 62 vs 69, P = .002). The lay panel found a similar difference with lower ratings for patients than for controls (VAS 60 vs 66, P = .011; Table 1, Figure 4). The correlation of age of the study persons with the rating of the facial appearance by panels was not significant. FIGURE 4. View largeDownload slide Ratings of facial appearance as evaluated by the panels and subjects’ self-satisfaction with facial appearance on the 100 mm VAS. FIGURE 4. View largeDownload slide Ratings of facial appearance as evaluated by the panels and subjects’ self-satisfaction with facial appearance on the 100 mm VAS. TABLE 1. Aesthetic Evaluation by the Panels and Subjects’ Own Self-Satisfaction With Facial Appearance on the 100 mm VAS Scale Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 VAS, visual Analog scale View Large TABLE 1. Aesthetic Evaluation by the Panels and Subjects’ Own Self-Satisfaction With Facial Appearance on the 100 mm VAS Scale Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 VAS, visual Analog scale View Large Subjective Facial Appearance Satisfaction The subjective grade of the patients’ own satisfaction with facial appearance compared to controls (P = .662) was not significant. The mean VAS score was 75 mm for the patients and 76 mm with controls (Table 1). Subjective satisfaction with the patients’ own appearance did not correlate with the rating by the panels (being P = .775 with the dentist panel compared to P = .396 for the lay panel). When the patients and controls were asked, “Is there something that bothers you in your facial appearance (other than the scar)?” 13 patients (32.5%) and 11 controls (27.5%) answered “Yes” (McNemar P = .804). Those factors that were reported to have disturbed study participants regarding their facial appearance are summarized in the Table 2. Answers for 2 patients were not available. TABLE 2. Factors That Were Mentioned to Disturb Study Subjects Regarding Their Own Facial Appearance Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 View Large TABLE 2. Factors That Were Mentioned to Disturb Study Subjects Regarding Their Own Facial Appearance Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 View Large The Effect of Surgical Technique on the Outcomes Differences between older and newer cranioplasty techniques (Table 3) regarding the mean patient age at operation and age at follow-up visit were statistically significant (P < .001). However, there was no association with operative techniques and subjective satisfaction with one's own appearance (P = .801) or the evaluation of the panels (P = .671 and P = .922) noted. TABLE 3. Comparison Between the Old and the New Operative Techniques Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 VAS, visual analog scale View Large TABLE 3. Comparison Between the Old and the New Operative Techniques Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 VAS, visual analog scale View Large The Postoperative Scar Every patient who had a bothersome postoperative scar (n = 4), had been operated with the H-cranioplasty using a bicoronal skin incision. One reoperated patient had a painful scar on palpation where there was an area of incomplete ossification, but the patient was satisfied with the scar appearance. The scars were visible on photographs in 6 cases, but only 1 of these patients found the scar to be bothersome. General Somatic Health Nine patients and 15 controls reported having migraine. Other varieties of occasional headaches were mentioned by 23 patients and 22 controls. A total of 32 of patients and 28 of controls reported having no other medical concerns. Not one case reported a history of epilepsy. Eleven (27.5%) patients and 8 (20%) controls revealed that they have or have had a history of mental health problems. Their satisfaction with appearance did not differ from the other participants in their groups. None of these variables differed significantly (McNemar P > .05). Family and Socioeconomic Status There were no significant differences between either the patients and controls in their education level, housing situation, marital situation, or employment status (Tables 4 and 5). One-half of the patients (n = 21) and the controls (n = 20) were involved in a permanent relationship. Likewise, the same number of the patients and their controls had children with an average of 1.7 children per family among patients and 2 children per family among controls. Likewise, the same number of persons in each group lived in privately owned apartments, not as tenants (Table 4). TABLE 4. Socioeconomic Situation of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 View Large TABLE 4. Socioeconomic Situation of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 View Large TABLE 5. Educational Level of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 View Large TABLE 5. Educational Level of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 View Large DISCUSSION The major observation in this study was that after a mean of 26.5 yr following surgery for scaphocephaly, patients were equally satisfied with their appearance as the controls. The same applies to their general physical health. Their family status was also equal. The predominance of males among sagittal synostosis patients is in agreement with previous studies, though the ratio of males to females was 1.7:1 in our study population, which was lower than the 2.6 to 3.8:1 ratio from previous studies.1,16 Demographic data in controls such as living with parents, being unemployed or education were in accordance with average numbers for the Finnish population, confirming that the control cohort was representative.17 Panel Evaluation of Appearance Both panels rated the patients’ appearance to be worse than the appearance of the controls. The difference appeared to be statistically significant, but was only 7 mm out of a 100 mm of VAS as rated by the panel of dentists and 6 mm as rated by the panel of lay persons (Table 1, Figure 4). Few studies evaluate craniosynostosis surgical outcomes from photographs using independent panels of evaluators.18-20 Though this method is widely used in orthodontic and cleft-lip-palate surgery evaluations,14,21 it is just being introduced into the craniosynostosis surgical community. Since different follow-up times, imaging sets, scoring systems were used and different size and background of panelists in every study so far, the results cannot be directly compared. A lay panel was used for the first time in the assessment of operative results following craniosynostosis surgery from photographs in a study by Metzler et al.20 They found no significant difference between the aesthetic scores among panels of professionals and lay persons when full-size facial images were evaluated.20 This finding is in agreement with our study. But the score used in the study by Metzler et al20 had only 3 grades with no patients rated to be in the worst grade 3 when full-size images were evaluated. This finding speaks for the need to use more precise tools and scores able to detect changes of smaller magnitude. Aesthetic evaluation of operative results is highly subjective and possible influenced by many factors. This probably explains why in spite of almost perfect agreement between the panels evaluations, there was only moderate agreement between individual panelists. Studies are needed to define standards for image set, scores to be used, definition of questions, panel size, and panel constituents. The difference in magnitude between panel evaluation of patients and controls of less than 10 mm in the VAS raises the question of minimally clinically important difference (MCID).22 Though this difference is statistically significant, it is less than 10% and it could be of no clinical importance. In the literature, no standard is reported for determining MCID when VAS scales are used in the evaluating aesthetic surgical outcomes. Self-Perceived Satisfaction with Facial Appearance Patients appeared to be just as satisfied with their facial appearance as compared to the control group. The appearance of the postoperative scar bothered only few patients (10%). All of these patients graded their satisfaction with their general appearance below average as well. Also, all 4 of these patients were operated using a bicoronal incision with the H-cranioplasty technique. While the sagittal direction of skin incision is not applicable when performing modern extended calvarial remodeling surgery, this direction of the scar seems to bear less cosmetic concern. Surprisingly, the visibility of the scar seemed not to correlate with the patients’ satisfaction with own appearance. There was no significant difference between the groups regarding the issue of unsatisfactory facial appearance, with teeth, shape of head, or face being the most common subjects to complain about in both patients and controls (Table 2). The majority of patient complaints (8 out of 13) were not related to the craniosynostosis itself. No correlation was revealed between their self-perceived satisfaction in facial appearance and the results of panels’ evaluations neither in patients nor was this noted in the control group. Contrasting with the current findings, when self-perceived satisfaction with appearance was compared to panel evaluations of patients presenting for severe malocclusion treatments or other aesthetic facial surgery, there was usually some moderate correlation found.14,23 Unlike those studies, our patients were not independently seeking treatment, but were invited for a follow-up visit years after treatment. Our findings were in agreement with previously published studies on body image of persons with visible differences (eg, other craniofacial malformations, amputations of limbs, or burn injuries), where the degree of disfigurement does not predict the magnitude of negative body image or how people experienced their bodies.24 The Effect of Operative Technique on the Outcomes The clinical mileu in which these patients were treated has changed during the years. Modern surgical techniques have been developed and the medical community and public in general have become more aware of craniosynostoses, leading to a decrease in delayed diagnoses.1 This trend is demonstrated in our data. Also, all 3 of the cases in our study group that were operated after 1 yr of age were operated in the 1970s. Older techniques of linear craniotomy and suturectomy were abandoned in 1985, and replaced by modifications of the H-technique. Accordingly, the direction of the scar was changed as well, from sagittal to bicoronal. Thus, the patients who were operated with new methods were about 10 yr younger at follow-up visits. It could be argued that this could influence the results of self-evaluation or the panels’ evaluation. However, the authors found no correlation of any aesthetic or self-evaluation parameters with age in this study. Surprisingly, the authors found no differences in outcomes between the different operative techniques (Table 3). However, lacking data on the preoperative severity of the deformities, the authors do not know how decision making in favor of surgery changed over the years. It is possible that patients operated 30 to 40 yr ago with this method had less significant degrees of scaphocephalic deformity preoperatively than the patients that were operated during the 1990s. Since this study employs historical cohorts with different operative methods used during certain time periods independently of the severity of scaphocephalic deformation, there is likely no patient selection bias when choosing operative method. Somatic and Mental Health Isolated sagittal synostosis usually has no influence on somatic health. This was confirmed by the current findings. The authors also found no correlation with surgically treated sagittal synostosis and the self-reported prevalence of headaches or mental problems. Having a history of mental problems did not appear to influence any study participant's satisfaction with their facial appearance. Family and Socioeconomic Situation Having surgery for sagittal craniosynostosis did not have an influence on a patients’ level of education, status in employment, the finding of a life partner, or the having of children later in life (Tables 4 and 5). According to the European Union statistics data, the proportion of young people aged 25 to 34 yr who still live with their parents is 4% in Finland. In the present study, 2 persons among the controls (5%) and 5 of the patients (12.5%) still continued to live together with their parents.17 This difference between the patients and controls was not significant, and could be explained by a relatively small-sized population and young age, since none of those still living with parents was over 23 yr of age. A total of 42.7% of persons aged 30 to 34 yr in Finland had a tertiary level of education.17 Notably 30% of patients and corresponding 32.5% of controls had professional tertiary level education. However, the participants of this study were younger than the reference population and some were still engaged in studying. There were 5 unemployed patients (12.5%) and 3 controls (7.5%). The general level of unemployment in the year 2015 in Northern Finland ranged from 10% to 15% in persons with ages ranging from 15 to 74 yr.17 Thus, the study results were in agreement with these numbers. While there is a growing amount of data on late follow-up of syndromic cases, data on adult single-suture synostoses are still scarce. Our study intended to fill this gap and to give craniofacial surgeons as well as patients and their families more accurate information on expected long-term results of the surgery, and that also from the patients’ point of view. CONCLUSION Adult patients treated for sagittal craniosynostosis in early childhood managed as well in adult life as controls. Patients had equal headache frequencies, rates of mental health problems, or issues with general health as the control group. Patients were also as satisfied with their own facial appearance as their age and gender matched control groups. 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The quality of life in adult patients with syndromic craniosynostosis from their perspective . J Craniofac Surg . 2016 ; 27 ( 6 ): 1510 - 1514 . Google Scholar CrossRef Search ADS PubMed 16. Singer S , Bower C , Southall P , Goldblatt J . Craniosynostosis in western australia, 1980–1994: A population-based study . Am J Med Genet . 1999 ; 83 ( 5 ): 382 - 387 . Google Scholar CrossRef Search ADS PubMed 17. Kotzeva M , Brandmüller T , Önnerfors Å , eds. Eurostat Regional Yearbook . 2016th ed . Luxemburg : European Union ; 2016 . 10.2785/29084 . 18. Bendon CL , Johnson HP , Judge AD , Wall SA , Johnson D . The aesthetic outcome of surgical correction for sagittal synostosis can be reliably scored by a novel method of preoperative and postoperative visual assessment . Plast Reconstr Surg . 2014 ; 134 ( 5 ): 775e - 786e . Google Scholar CrossRef Search ADS PubMed 19. Panchal J , Marsh JL , Park TS , Kaufman B , Pilgram T . Photographic assessment of head shape following sagittal synostosis surgery . Plast Reconstr Surg . 1999 ; 103 ( 6 ): 1585 - 1591 . Google Scholar CrossRef Search ADS PubMed 20. Metzler P , Zemann W , Jacobsen C , Lubbers HT , Gratz KW , Obwegeser JA . Assessing aesthetic outcomes after trigonocephaly correction . Oral Maxillofac Surg . 2014 ; 18 ( 2 ): 181 - 186 . Google Scholar CrossRef Search ADS PubMed 21. Asher-McDade C Roberts C , Shaw WC , Gallager C . Development of a method for rating nasolabial appearance in patients with clefts of the lip and palate . Cleft Palate Craniofac J . 1991 ; 28 ( 4 ): 385 - 390 ; discussion 390-1 . Google Scholar CrossRef Search ADS PubMed 22. Thoma A , Ignacy TA . Health services research: Impact of quality of life instruments on craniofacial surgery . J Craniofac Surg . 2012 ; 23 ( 1 ): 283 - 287 . Google Scholar CrossRef Search ADS PubMed 23. Badran SA . The effect of malocclusion and self-perceived aesthetics on the self-esteem of a sample of jordanian adolescents . Eur J Orthod . 2010 ; 32 ( 6 ): 638 - 644 . Google Scholar CrossRef Search ADS PubMed 24. Tiggemann M . Considerations of positive body image across various social identities and special populations . Body Image . 2015 ; 14 : 168 - 176 . Google Scholar CrossRef Search ADS PubMed COMMENTS Overall this is a very interesting and rare long-term follow-up study in patients treated in infancy for non-syndromic sagittal craniosynostosis. The results are that patients treated for this condition had very good self-image, were doing well intellectually and socially, and were essentially on par with controls. Professional and lay panels found the patients to look slightly worse than controls, but the differences were minor. Patients' self-ratings were no different from those of the controls. The types of surgery were variable, and preoperative severity of the condition was obviously not available, but it is quite reassuring to know how well the patients are doing in long-term follow-up regardless of type of treatment or severity of the presenting condition. Interestingly, for many patients, the scar was the biggest aesthetic concern regardless of the technique. This would be very supportive of the more minimally invasive approaches being used these days, which avoid the large bicoronal incision. Mark Proctor Boston, Massachusetts This is an interesting study with a novel approach to outcome measurements in adults who underwent surgery for sagittal synostosis in childhood. It is difficult to quantify “success” in assessment of cosmetic or aesthetic outcomes in surgeries for craniosynostosis. While the methodology in this report has been previous used in orthodontic and cleft-lip-palate surgery population, this is a relatively new approach to evaluating outcomes in craniosynostosis surgery. This study used dentist (orthodontic residents/orthognathic surgeon) and lay public panels to evaluate cosmesis in patient and control photographs using visual analog scales. Patients also self-reported their satisfaction with aesthetic outcomes with visual analog scales and provided information on additional demographic information. While the dental and lay panels rated the appearance of surgical group lower than controls, the patients’ self-reported ratings did not differ from controls. The patients were also no different from controls in other assessment such as their somatic health, education level, employment status, or finding a life partner. This is encouraging information for both patients and neurosurgeon's performing these procedures. Laurie L. Ackerman Indianapolis, Indiana The authors provide an important study examining the long-term surgical outcomes of adults who underwent sagittal craniosynostosis repair as children. The study fills a void in the literature and is timely, especially today when successful results are determined by patient-report outcomes rather than those assigned by the surgeon. The authors utilize a visual analog scale (VAS) to quantify aesthetic satisfaction in the surgically treated subset and a control group. Interestingly, from a third person's perspective, cosmetic outcomes are poorer in the craniosynostosis repair group compared to controls; however, individuals in the treatment group did not perceive worse cosmetic outcomes compared to individuals in the control group. Hence, beauty is truly in the eye of the beholder – as it should be. Andrew Jea Indianapolis, Indiana Copyright © 2018 by the Congress of Neurological Surgeons http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Operative Neurosurgery Oxford University Press

Outcomes of 40 Nonsyndromic Sagittal Craniosynostosis Patients as Adults: A Case-Control Study With 26 Years of Postoperative Follow-up

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Oxford University Press
Copyright
Copyright © 2018 by the Congress of Neurological Surgeons
ISSN
2332-4252
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2332-4260
D.O.I.
10.1093/ons/opy047
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Abstract

Abstract BACKGROUND While sagittal synostosis is the most common craniosynostosis, long-term follow-up of these patients is lacking. OBJECTIVE To evaluate the results of surgical management of those patients with sagittal synostosis who attain adulthood. METHODS An outcome study of surgically treated isolated sagittal synostosis patients operated between 1977 and 1998 was conducted at the Craniofacial Center of Oulu University Hospital, Oulu, Finland with an average follow-up time of 26.5 yr. Patients’ socioeconomic situation, satisfaction with their own facial appearance and attractiveness as rated by 2 independent panels was evaluated and compared to controls. RESULTS The self-satisfaction with the patients’ own appearance scored a mean of 75 mm on a visual analog scale of 100 mm between the patients and 76 mm with the control group. The subjective satisfaction of the patients with their own appearance failed to correlate with the rating of their appearance by the panels. The panels rated the patients’ appearance to be on average 6 to 7 mm out of 100 mm visual analog scale less attractive than the controls. Data on socioeconomic situation, including marital status, housing, education, employment of the patients, and controls are presented. CONCLUSION Isolated sagittal synostosis patients treated surgically were as happy with their facial appearance as were individuals in an age and gender-matched control group. Two independent panels found the patients’ appearance to be only somewhat less attractive. Analysis of the socioeconomic situation and general health revealed that patients equaled that of controls. Adult, Craniosynostoses, Aesthetics, Outcome assessment, Sagittal synostosis, Scaphocephaly ABBREVIATIONS ABBREVIATIONS MCID minimally clinically important difference QOL quality of life VAS visual analog scale Premature fusion of the sagittal suture is the most common type of craniosynostoses, comprising 40% to 60% of all cases.1 The patients have a classic scaphocephalic head shape resulting from a diminished skull height and width with compensatory anteroposterior growth.2 Surgical procedures to manage sagittal craniosynostosis have changed over the decades. Originally, the fused suture was released by making strip craniotomy lines on both sides of the suture. Later strip craniectomy of the ossified suture itself was performed.3,4 Currently more extensive calvarial vault reconstructions are preferred5,6 or endoscopic strip craniectomies are done followed by postoperative molding therapy with helmets.3,7 At Oulu University Hospital the early operative method included 2 parallel strip craniotomies lateral to the midline, while a strip of bone was left over the sagittal sinus according to Lannelongue.4 Later additional silicone membranes were used on the craniotomy lines8 (Figure 1A). This technique was supplanted by suturectomy, usually supplemented with split dural interpositioning, in the early 1980s. For dural split, the outer layer of the dura was cut parallel to the sagittal sinus on both sides of it, then dissected from the deeper layer, turned over the newly cut bony edges and sutured to the periosteum (Figure 1B).9 This method was replaced by the “H-technique” (Figure 1C).5 FIGURE 1. View largeDownload slide Different techniques used for the treatment of sagittal craniosynostosis in the Oulu University Hospital. A, The Lannelongue and Ingraham suture “unlocking” technique with and without silicone membrane interposition. B, Lane strip craniectomy with and without dural splitting. C, “H” cranioplasty with and without additional cuts to the temporal bone. FIGURE 1. View largeDownload slide Different techniques used for the treatment of sagittal craniosynostosis in the Oulu University Hospital. A, The Lannelongue and Ingraham suture “unlocking” technique with and without silicone membrane interposition. B, Lane strip craniectomy with and without dural splitting. C, “H” cranioplasty with and without additional cuts to the temporal bone. From a clinical viewpoint, surgically treated sagittal craniosynostosis patients are expected to have a minimal risk of late sequelae. Clinical follow-up is usually continued no later than school age. There are several studies suggesting single suture craniosynostoses are associated with mild to moderate developmental and educational difficulties that become apparent at school age.10-12 However, patients with sagittal synostosis have the best performance in neuropsychological tests.12 Furthermore, all craniosynostosis may have secondary psychosocial effects. Some patients retain residual aesthetic facial differences that might attract unfavorable attention from strangers and rejection from peers. Such attention may negatively influence social behavior and result in poor school performance with difficulties in educational environments.13 When studying persons treated for severe malocclusions, it was found that the quality of life (QOL) and self-perceived aesthetic satisfaction improve extensively after treatment.14 These variables may be determinant of the outcomes among craniosynostoses patients, as well, in the future. However, in cases with syndromic craniosynostosis, facial differences may not influence the subjective QOL.15 The current study aimed to evaluate the aesthetic outcomes in adulthood of cases treated surgically in early childhood for sagittal craniosynostosis. The evaluation of the social situation was another goal of this study. METHODS Study Patients This was a cohort outcome study of patients and controls operated for sagittal synostosis at the Craniofacial Center of Oulu University Hospital, Oulu, Finland between 1977 and 1998. Only cases of patients older than 18 yr of age at the time of the follow-up examination were included in the study. Patients with any craniofacial syndromes or associated neurological disease were excluded. There were 171 operations for craniosynostosis during the study period. Of these, 115 patients satisfied the inclusion criteria and attempts were made to reach them (Figure 2). Contact was made with 83 patients and 61 agreed to participate in the study. Twenty of these patients had either metopic, coronal, lambdoid, or multiple suture synostosis and were not included. A single patient with both sagittal synostosis and hydrocephalus was excluded from the study. The mean age of the 40 patients (25 males, 15 females) with nonsyndromic sagittal synostosis was 27.4 yr (range 18-41). The follow-up time after operation was a mean 26.5 yr (range 17-37). Controls who were age and gender matched (n = 40) were selected randomly from Finnish Population Register Centre. Invitations were sent to both patients and controls to attend a follow-up visit by which time the subjects had completed their questionnaires as requested. The facial appearance of the patients was examined during the control visit that also included evaluation of their head shape, the scar, and palpation of the head. Standardized photographs were obtained (Figure 3). The follow-up visits took place between November 2014 and February 2016. FIGURE 2. View largeDownload slide Flow chart of the patients included into the study. FIGURE 2. View largeDownload slide Flow chart of the patients included into the study. FIGURE 3. View largeDownload slide An example of the slide with photographs as presented for the evaluation of aesthetic results of the surgery by panelists. An image of a healthy person not included in the study is present with his consent. On the slides presented to the panels the eyes of the study persons were not blinded. FIGURE 3. View largeDownload slide An example of the slide with photographs as presented for the evaluation of aesthetic results of the surgery by panelists. An image of a healthy person not included in the study is present with his consent. On the slides presented to the panels the eyes of the study persons were not blinded. Operative Treatment of the Patients Operative treatment included linear parasagittal craniotomies with silicone membrane interposition (n = 9), suturectomy together with dural split (n = 4), suturectomy without dural split (n = 3), and various forms of H-plasty with or without barrel stave osteotomies of the temporal bone (n = 24; Figure 1). The coronal and lambdoid sutures were kept intact in all cases. Mean age when surgery was performed was 5.7 mo (ranging from 9 d up to 45 mo). While 38 patients were operated only once, 2 cases required reoperation because of residual scaphocephalic head shapes. Of these 2 cases, 1 was first operated at the age 9 d and then reoperated at the age of 10.5 mo. The other case was first operated at the age of 3 mo and then reoperated at the age 2 yr. Aesthetic Evaluation using Photographs Long-term aesthetic evaluation of the clinical outcome was the major goal of the clinical evaluation. Facial aesthetic appearance was rated by 2 independent panels. The panel members did not know which of the evaluated persons had undergone surgery and who were controls. One panel consisted of either orthodontic residents or consultants (1 male and 3 females) and 1 (female) orthognathic surgeon. This panel was named the “dentists’ panel.” Then a second panel that consisted of lay persons had no health care education (1 male and 3 females) were called the “lay panel.” Panelist evaluators were presented with a series of randomly ordered slides with photographs of the patients and the controls. Each slide consisted of 4 images in standardized projections comprising: anterior view smiling and not smiling, with lateral and oblique views as well (Figure 3). Slides were displayed for exactly 10 s, in order to acquire the immediate initial panelist impression. A 100 mm visual analog scale (VAS) with 0 mm as least attractive and 100 mm representing most attractive was used to determine the cosmetic outcomes. Self-reported Questionnaire The self-reported questionnaire contained questions regarding the participants’ education level, housing status, marital or relationship status, employment status, general health with attention to their history of headaches, and the presence of mental disturbances. The education level was classified into 3 groups: no professional education, professional secondary education, and professional tertiary education. The first group included those with no more than secondary nonvocational education accomplished. The second group included participants who had completed lower level vocational education, or any academic education with the degree below Bachelor. The third group included those with higher level vocational education or academic education of at least Bachelor level. Participants were also directed to express their subjective satisfaction with their own facial aesthetics using a 100 mm VAS. The question was: “How satisfied are you with your current facial appearance?” A VAS of 0 mm referred to “very unsatisfied” whereas a VAS of 100 mm corresponded to “very satisfied.” Another question was “Is there something that bothers you in your facial appearance?” The patients were also asked whether their scar bothered them. Statistical Analysis Analyses were performed between the matched case-control pairs using paired samples t-test and McNemar tests. When comparing the difference between the groups, Pearson chi-square tests and Fischer exact test were used. The degree of the panels’ interobserver reliability was studied using intraclass correlation coefficient both between the panels and also inside the panels amongst the panel members. When comparing 2 panels, the reliability coefficient was almost perfect (0.857) when the patient groups were rated and substantial (0.777) for control group, and 0.840 when whole group of participants rating was analyzed. The reliability within the 2 panels among panel members was moderate being P = .573 in the case of the dentist panel and P = .555 with the lay panel. Statistical significances were set at P < .05. All statistical analyses were performed using the commercially available SPSS software for Windows 14.0 (IBM Inc, Armonk, New York). Ethical Aspects The study was conducted in accordance with the Helsinki declaration. The study was reviewed and accepted by the Ethical Review Board of the Northern Ostrobothnia Hospital District (number 86/2013). Both the patients and their controls signed informed consent forms. RESULTS Aesthetic Outcome According to Panel Evaluation In the case of facial appearance evaluation by the dentist panel, the patients received lower ratings than their controls (VAS 62 vs 69, P = .002). The lay panel found a similar difference with lower ratings for patients than for controls (VAS 60 vs 66, P = .011; Table 1, Figure 4). The correlation of age of the study persons with the rating of the facial appearance by panels was not significant. FIGURE 4. View largeDownload slide Ratings of facial appearance as evaluated by the panels and subjects’ self-satisfaction with facial appearance on the 100 mm VAS. FIGURE 4. View largeDownload slide Ratings of facial appearance as evaluated by the panels and subjects’ self-satisfaction with facial appearance on the 100 mm VAS. TABLE 1. Aesthetic Evaluation by the Panels and Subjects’ Own Self-Satisfaction With Facial Appearance on the 100 mm VAS Scale Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 VAS, visual Analog scale View Large TABLE 1. Aesthetic Evaluation by the Panels and Subjects’ Own Self-Satisfaction With Facial Appearance on the 100 mm VAS Scale Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 Variable Patients Controls P Self-satisfaction with appearance, mm VAS  Mean 75 76 .662  Range 29-100 29-98 Dentists panel, mm VAS  Mean 62 69 .002  Range 36-79 50-82 Lay panel, mm VAS  Mean 60 66 .011  Range 43-82 45-85 VAS, visual Analog scale View Large Subjective Facial Appearance Satisfaction The subjective grade of the patients’ own satisfaction with facial appearance compared to controls (P = .662) was not significant. The mean VAS score was 75 mm for the patients and 76 mm with controls (Table 1). Subjective satisfaction with the patients’ own appearance did not correlate with the rating by the panels (being P = .775 with the dentist panel compared to P = .396 for the lay panel). When the patients and controls were asked, “Is there something that bothers you in your facial appearance (other than the scar)?” 13 patients (32.5%) and 11 controls (27.5%) answered “Yes” (McNemar P = .804). Those factors that were reported to have disturbed study participants regarding their facial appearance are summarized in the Table 2. Answers for 2 patients were not available. TABLE 2. Factors That Were Mentioned to Disturb Study Subjects Regarding Their Own Facial Appearance Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 View Large TABLE 2. Factors That Were Mentioned to Disturb Study Subjects Regarding Their Own Facial Appearance Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 Patients Controls Head and face shape 6 5 Nose shape 1 1 Dental appearance 4 2 Skin condition 1 2 Hair condition 1 0 Eyelids 0 1 Total 13 11 Missing answer 2 0 View Large The Effect of Surgical Technique on the Outcomes Differences between older and newer cranioplasty techniques (Table 3) regarding the mean patient age at operation and age at follow-up visit were statistically significant (P < .001). However, there was no association with operative techniques and subjective satisfaction with one's own appearance (P = .801) or the evaluation of the panels (P = .671 and P = .922) noted. TABLE 3. Comparison Between the Old and the New Operative Techniques Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 VAS, visual analog scale View Large TABLE 3. Comparison Between the Old and the New Operative Techniques Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 Variables Old techniques (N16) New techniques (N24) Age at operation, mo  Mean 9.1 3.4  Range 0.3-45.3 1.3-9.9 Age at follow-up, yr  Mean 32.1 23.8  Range 24.2-41.0 18.0-29.9 Self-satisfaction, mm VAS  Mean 75 73  Range 29-100 30-96 Dentists panel rating, mm VAS  Mean 61 63  Range 36-79 40-78 Lay panels rating, mm VAS  Mean 60 61  Range 43-81 43-82 VAS, visual analog scale View Large The Postoperative Scar Every patient who had a bothersome postoperative scar (n = 4), had been operated with the H-cranioplasty using a bicoronal skin incision. One reoperated patient had a painful scar on palpation where there was an area of incomplete ossification, but the patient was satisfied with the scar appearance. The scars were visible on photographs in 6 cases, but only 1 of these patients found the scar to be bothersome. General Somatic Health Nine patients and 15 controls reported having migraine. Other varieties of occasional headaches were mentioned by 23 patients and 22 controls. A total of 32 of patients and 28 of controls reported having no other medical concerns. Not one case reported a history of epilepsy. Eleven (27.5%) patients and 8 (20%) controls revealed that they have or have had a history of mental health problems. Their satisfaction with appearance did not differ from the other participants in their groups. None of these variables differed significantly (McNemar P > .05). Family and Socioeconomic Status There were no significant differences between either the patients and controls in their education level, housing situation, marital situation, or employment status (Tables 4 and 5). One-half of the patients (n = 21) and the controls (n = 20) were involved in a permanent relationship. Likewise, the same number of the patients and their controls had children with an average of 1.7 children per family among patients and 2 children per family among controls. Likewise, the same number of persons in each group lived in privately owned apartments, not as tenants (Table 4). TABLE 4. Socioeconomic Situation of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 View Large TABLE 4. Socioeconomic Situation of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 Living with parents Living in own real estate In permanent relation Having children Students Unemployed Patients 5 17 21 12 10 5 Controls 2 17 20 12 8 3 View Large TABLE 5. Educational Level of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 View Large TABLE 5. Educational Level of the Patients and Controls (Number of Subjects Presented, McNemar Test P > .05 for All Variables) No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 No professional education Professional secondary Professional tertiary Patients 9 19 12 Controls 11 16 13 View Large DISCUSSION The major observation in this study was that after a mean of 26.5 yr following surgery for scaphocephaly, patients were equally satisfied with their appearance as the controls. The same applies to their general physical health. Their family status was also equal. The predominance of males among sagittal synostosis patients is in agreement with previous studies, though the ratio of males to females was 1.7:1 in our study population, which was lower than the 2.6 to 3.8:1 ratio from previous studies.1,16 Demographic data in controls such as living with parents, being unemployed or education were in accordance with average numbers for the Finnish population, confirming that the control cohort was representative.17 Panel Evaluation of Appearance Both panels rated the patients’ appearance to be worse than the appearance of the controls. The difference appeared to be statistically significant, but was only 7 mm out of a 100 mm of VAS as rated by the panel of dentists and 6 mm as rated by the panel of lay persons (Table 1, Figure 4). Few studies evaluate craniosynostosis surgical outcomes from photographs using independent panels of evaluators.18-20 Though this method is widely used in orthodontic and cleft-lip-palate surgery evaluations,14,21 it is just being introduced into the craniosynostosis surgical community. Since different follow-up times, imaging sets, scoring systems were used and different size and background of panelists in every study so far, the results cannot be directly compared. A lay panel was used for the first time in the assessment of operative results following craniosynostosis surgery from photographs in a study by Metzler et al.20 They found no significant difference between the aesthetic scores among panels of professionals and lay persons when full-size facial images were evaluated.20 This finding is in agreement with our study. But the score used in the study by Metzler et al20 had only 3 grades with no patients rated to be in the worst grade 3 when full-size images were evaluated. This finding speaks for the need to use more precise tools and scores able to detect changes of smaller magnitude. Aesthetic evaluation of operative results is highly subjective and possible influenced by many factors. This probably explains why in spite of almost perfect agreement between the panels evaluations, there was only moderate agreement between individual panelists. Studies are needed to define standards for image set, scores to be used, definition of questions, panel size, and panel constituents. The difference in magnitude between panel evaluation of patients and controls of less than 10 mm in the VAS raises the question of minimally clinically important difference (MCID).22 Though this difference is statistically significant, it is less than 10% and it could be of no clinical importance. In the literature, no standard is reported for determining MCID when VAS scales are used in the evaluating aesthetic surgical outcomes. Self-Perceived Satisfaction with Facial Appearance Patients appeared to be just as satisfied with their facial appearance as compared to the control group. The appearance of the postoperative scar bothered only few patients (10%). All of these patients graded their satisfaction with their general appearance below average as well. Also, all 4 of these patients were operated using a bicoronal incision with the H-cranioplasty technique. While the sagittal direction of skin incision is not applicable when performing modern extended calvarial remodeling surgery, this direction of the scar seems to bear less cosmetic concern. Surprisingly, the visibility of the scar seemed not to correlate with the patients’ satisfaction with own appearance. There was no significant difference between the groups regarding the issue of unsatisfactory facial appearance, with teeth, shape of head, or face being the most common subjects to complain about in both patients and controls (Table 2). The majority of patient complaints (8 out of 13) were not related to the craniosynostosis itself. No correlation was revealed between their self-perceived satisfaction in facial appearance and the results of panels’ evaluations neither in patients nor was this noted in the control group. Contrasting with the current findings, when self-perceived satisfaction with appearance was compared to panel evaluations of patients presenting for severe malocclusion treatments or other aesthetic facial surgery, there was usually some moderate correlation found.14,23 Unlike those studies, our patients were not independently seeking treatment, but were invited for a follow-up visit years after treatment. Our findings were in agreement with previously published studies on body image of persons with visible differences (eg, other craniofacial malformations, amputations of limbs, or burn injuries), where the degree of disfigurement does not predict the magnitude of negative body image or how people experienced their bodies.24 The Effect of Operative Technique on the Outcomes The clinical mileu in which these patients were treated has changed during the years. Modern surgical techniques have been developed and the medical community and public in general have become more aware of craniosynostoses, leading to a decrease in delayed diagnoses.1 This trend is demonstrated in our data. Also, all 3 of the cases in our study group that were operated after 1 yr of age were operated in the 1970s. Older techniques of linear craniotomy and suturectomy were abandoned in 1985, and replaced by modifications of the H-technique. Accordingly, the direction of the scar was changed as well, from sagittal to bicoronal. Thus, the patients who were operated with new methods were about 10 yr younger at follow-up visits. It could be argued that this could influence the results of self-evaluation or the panels’ evaluation. However, the authors found no correlation of any aesthetic or self-evaluation parameters with age in this study. Surprisingly, the authors found no differences in outcomes between the different operative techniques (Table 3). However, lacking data on the preoperative severity of the deformities, the authors do not know how decision making in favor of surgery changed over the years. It is possible that patients operated 30 to 40 yr ago with this method had less significant degrees of scaphocephalic deformity preoperatively than the patients that were operated during the 1990s. Since this study employs historical cohorts with different operative methods used during certain time periods independently of the severity of scaphocephalic deformation, there is likely no patient selection bias when choosing operative method. Somatic and Mental Health Isolated sagittal synostosis usually has no influence on somatic health. This was confirmed by the current findings. The authors also found no correlation with surgically treated sagittal synostosis and the self-reported prevalence of headaches or mental problems. Having a history of mental problems did not appear to influence any study participant's satisfaction with their facial appearance. Family and Socioeconomic Situation Having surgery for sagittal craniosynostosis did not have an influence on a patients’ level of education, status in employment, the finding of a life partner, or the having of children later in life (Tables 4 and 5). According to the European Union statistics data, the proportion of young people aged 25 to 34 yr who still live with their parents is 4% in Finland. In the present study, 2 persons among the controls (5%) and 5 of the patients (12.5%) still continued to live together with their parents.17 This difference between the patients and controls was not significant, and could be explained by a relatively small-sized population and young age, since none of those still living with parents was over 23 yr of age. A total of 42.7% of persons aged 30 to 34 yr in Finland had a tertiary level of education.17 Notably 30% of patients and corresponding 32.5% of controls had professional tertiary level education. However, the participants of this study were younger than the reference population and some were still engaged in studying. There were 5 unemployed patients (12.5%) and 3 controls (7.5%). The general level of unemployment in the year 2015 in Northern Finland ranged from 10% to 15% in persons with ages ranging from 15 to 74 yr.17 Thus, the study results were in agreement with these numbers. While there is a growing amount of data on late follow-up of syndromic cases, data on adult single-suture synostoses are still scarce. Our study intended to fill this gap and to give craniofacial surgeons as well as patients and their families more accurate information on expected long-term results of the surgery, and that also from the patients’ point of view. CONCLUSION Adult patients treated for sagittal craniosynostosis in early childhood managed as well in adult life as controls. Patients had equal headache frequencies, rates of mental health problems, or issues with general health as the control group. Patients were also as satisfied with their own facial appearance as their age and gender matched control groups. Both panels of lay persons and dentists found the patients’ appearance to be slightly less attractive than controls, but this difference was slight, being less than 10 mm on a 100 mm VAS. There was no correlation between aesthetic self-satisfaction and third person's opinion regarding facial appearance. This study has been financially supported by The Foundation for Pediatric Research, Finland. Disclosures This study has been financially supported by The Foundation for Pediatric Research, Finland. The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article. REFERENCES 1. Kolar JC . An epidemiological study of nonsyndromal craniosynostoses . J Craniofac Surg . 2011 ; 22 ( 1 ): 47 - 49 . Google Scholar CrossRef Search ADS PubMed 2. Fearon JA . Evidence-based medicine: Craniosynostosis . Plast Reconstr Surg . 2014 ; 133 ( 5 ): 1261 - 1275 . Google Scholar CrossRef Search ADS PubMed 3. Clayman MA , Murad GJ , Steele MH , Seagle MB , Pincus DW . History of craniosynostosis surgery and the evolution of minimally invasive endoscopic techniques . Ann Plast Surg . 2007 ; 58 ( 3 ): 285 - 287 . Google Scholar CrossRef Search ADS PubMed 4. Bir SC , Ambekar S , Notarianni C , Nanda A . Odilon marc lannelongue (1840-1911) and strip craniectomy for craniosynostosis . Neurosurg Focus . 2014 ; 36 ( 4 ): E16 . Google Scholar CrossRef Search ADS PubMed 5. Di Rocco F , Knoll BI , Arnaud E et al. Scaphocephaly correction with retrocoronal and prelambdoid craniotomies (renier's “H” technique) . Childs Nerv Syst . 2012 ; 28 ( 9 ): 1327 - 1332 . Google Scholar CrossRef Search ADS PubMed 6. Boop FA , Shewmake K , Chadduck WM . Synostectomy versus complex cranioplasty for the treatment of sagittal synostosis . Childs Nerv Syst . 1996 ; 12 ( 7 ): 371 - 375 . Google Scholar CrossRef Search ADS PubMed 7. Berry-Candelario J , Ridgway EB , Grondin RT , Rogers GF , Proctor MR . Endoscope-assisted strip craniectomy and postoperative helmet therapy for treatment of craniosynostosis . Neurosurg Focus . 2011 ; 31 ( 2 ): E5 . Google Scholar CrossRef Search ADS PubMed 8. Ingraham FD , Alexander E Jr , Matson DD . Clinical studies in craniosynostosis analysis of 50 cases and description of a method of surgical treatment . Surgery . 1948 ; 24 ( 3 ): 518 - 541 . Google Scholar PubMed 9. van der Werf AJ . Ten-year experience with a new method in the treatment of craniosynostosis . J Neurol Neurosurg Psychiatry . 1971 ; 34 ( 1 ): 105 - 105 . Google Scholar CrossRef Search ADS PubMed 10. Kapp-Simon KA , Collett BR , Barr-Schinzel MA et al. Behavioral adjustment of toddler and preschool-aged children with single-suture craniosynostosis . Plast Reconstr Surg . 2012 ; 130 ( 3 ): 635 - 647 . Google Scholar CrossRef Search ADS PubMed 11. Speltz ML , Kapp-Simon KA , Cunningham M , Marsh J , Dawson G . Single-suture craniosynostosis: a review of neurobehavioral research and theory . J Pediatr Psychol . 2004 ; 29 ( 8 ): 651 - 668 . Google Scholar CrossRef Search ADS PubMed 12. Bellew M , Chumas P . Long-term developmental follow-up in children with nonsyndromic craniosynostosis . J Neurosurg Pediatrics . 2015 ; 16 ( 4 ): 445 - 451 . Google Scholar CrossRef Search ADS 13. Becker DB , Petersen JD , Kane AA , Cradock MM , Pilgram TK , Marsh JL . Speech, cognitive, and behavioral outcomes in nonsyndromic craniosynostosis . Plast Reconstr Surg . 2005 ; 116 ( 2 ): 400 - 407 . Google Scholar CrossRef Search ADS PubMed 14. Silvola AS , Varimo M , Tolvanen M , Rusanen J , Lahti S , Pirttiniemi P . Dental esthetics and quality of life in adults with severe malocclusion before and after treatment . Angle Orthod . 2014 ; 84 ( 4 ): 594 - 599 . Google Scholar CrossRef Search ADS PubMed 15. Lloyd MS , Venugopal A , Horton J et al. The quality of life in adult patients with syndromic craniosynostosis from their perspective . J Craniofac Surg . 2016 ; 27 ( 6 ): 1510 - 1514 . Google Scholar CrossRef Search ADS PubMed 16. Singer S , Bower C , Southall P , Goldblatt J . Craniosynostosis in western australia, 1980–1994: A population-based study . Am J Med Genet . 1999 ; 83 ( 5 ): 382 - 387 . Google Scholar CrossRef Search ADS PubMed 17. Kotzeva M , Brandmüller T , Önnerfors Å , eds. Eurostat Regional Yearbook . 2016th ed . Luxemburg : European Union ; 2016 . 10.2785/29084 . 18. Bendon CL , Johnson HP , Judge AD , Wall SA , Johnson D . The aesthetic outcome of surgical correction for sagittal synostosis can be reliably scored by a novel method of preoperative and postoperative visual assessment . Plast Reconstr Surg . 2014 ; 134 ( 5 ): 775e - 786e . Google Scholar CrossRef Search ADS PubMed 19. Panchal J , Marsh JL , Park TS , Kaufman B , Pilgram T . Photographic assessment of head shape following sagittal synostosis surgery . Plast Reconstr Surg . 1999 ; 103 ( 6 ): 1585 - 1591 . Google Scholar CrossRef Search ADS PubMed 20. Metzler P , Zemann W , Jacobsen C , Lubbers HT , Gratz KW , Obwegeser JA . Assessing aesthetic outcomes after trigonocephaly correction . Oral Maxillofac Surg . 2014 ; 18 ( 2 ): 181 - 186 . Google Scholar CrossRef Search ADS PubMed 21. Asher-McDade C Roberts C , Shaw WC , Gallager C . Development of a method for rating nasolabial appearance in patients with clefts of the lip and palate . Cleft Palate Craniofac J . 1991 ; 28 ( 4 ): 385 - 390 ; discussion 390-1 . Google Scholar CrossRef Search ADS PubMed 22. Thoma A , Ignacy TA . Health services research: Impact of quality of life instruments on craniofacial surgery . J Craniofac Surg . 2012 ; 23 ( 1 ): 283 - 287 . Google Scholar CrossRef Search ADS PubMed 23. Badran SA . The effect of malocclusion and self-perceived aesthetics on the self-esteem of a sample of jordanian adolescents . Eur J Orthod . 2010 ; 32 ( 6 ): 638 - 644 . Google Scholar CrossRef Search ADS PubMed 24. Tiggemann M . Considerations of positive body image across various social identities and special populations . Body Image . 2015 ; 14 : 168 - 176 . Google Scholar CrossRef Search ADS PubMed COMMENTS Overall this is a very interesting and rare long-term follow-up study in patients treated in infancy for non-syndromic sagittal craniosynostosis. The results are that patients treated for this condition had very good self-image, were doing well intellectually and socially, and were essentially on par with controls. Professional and lay panels found the patients to look slightly worse than controls, but the differences were minor. Patients' self-ratings were no different from those of the controls. The types of surgery were variable, and preoperative severity of the condition was obviously not available, but it is quite reassuring to know how well the patients are doing in long-term follow-up regardless of type of treatment or severity of the presenting condition. Interestingly, for many patients, the scar was the biggest aesthetic concern regardless of the technique. This would be very supportive of the more minimally invasive approaches being used these days, which avoid the large bicoronal incision. Mark Proctor Boston, Massachusetts This is an interesting study with a novel approach to outcome measurements in adults who underwent surgery for sagittal synostosis in childhood. It is difficult to quantify “success” in assessment of cosmetic or aesthetic outcomes in surgeries for craniosynostosis. While the methodology in this report has been previous used in orthodontic and cleft-lip-palate surgery population, this is a relatively new approach to evaluating outcomes in craniosynostosis surgery. This study used dentist (orthodontic residents/orthognathic surgeon) and lay public panels to evaluate cosmesis in patient and control photographs using visual analog scales. Patients also self-reported their satisfaction with aesthetic outcomes with visual analog scales and provided information on additional demographic information. While the dental and lay panels rated the appearance of surgical group lower than controls, the patients’ self-reported ratings did not differ from controls. The patients were also no different from controls in other assessment such as their somatic health, education level, employment status, or finding a life partner. This is encouraging information for both patients and neurosurgeon's performing these procedures. Laurie L. Ackerman Indianapolis, Indiana The authors provide an important study examining the long-term surgical outcomes of adults who underwent sagittal craniosynostosis repair as children. The study fills a void in the literature and is timely, especially today when successful results are determined by patient-report outcomes rather than those assigned by the surgeon. The authors utilize a visual analog scale (VAS) to quantify aesthetic satisfaction in the surgically treated subset and a control group. Interestingly, from a third person's perspective, cosmetic outcomes are poorer in the craniosynostosis repair group compared to controls; however, individuals in the treatment group did not perceive worse cosmetic outcomes compared to individuals in the control group. Hence, beauty is truly in the eye of the beholder – as it should be. Andrew Jea Indianapolis, Indiana Copyright © 2018 by the Congress of Neurological Surgeons

Journal

Operative NeurosurgeryOxford University Press

Published: Apr 2, 2018

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