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Background Endoscopic suturectomy and helmeting represents a successful first-line surgical treatment for bilateral coronal craniosynostosis. Its effect on cranial morphology has not been previously described. Methods Patients were identified who had bilateral coronal craniosynostosis treated with endoscopic suturectomy and postoperative helmeting at Boston Children's Hospital between 2005 and 2013 and who underwent preoperative and postoperative computed tomography. Two normative patient populations were identified from our trauma registry with computed tomographic scans completed at the same age as our pretreatment and posttreatment scans. Craniometric indices were used to quantify the effect of treatment. Results Twenty-seven patients were identified who underwent bilateral coronal suturectomy. Twelve patients had preoperative and postoperative computed tomographic studies. Eight patients (66.7 percent) were syndromic. The average ages for preoperative and postoperative computed tomographic scan were 1.1 months (range, 0.03 to 2.6 months) and 19.6 months (range, 10.8 to 37.5 months). Thirteen patients with an average age of 1.1 months (range, 0.5 to 1.6 months) were identified as a preoperative control group. Fourteen patients with an average age of 18.5 months (range, 15.5 to 22.9 months) were identified as a postoperative control group. The anterior cranial height stabilized with treatment and the anterior cranial base length increased. The anterior cranial height-to-anterior cranial base length ratio significantly decreased with treatment (p = 0.128). Frontal bossing normalized with endoscopic suturectomy (craniosynostosis versus control: preoperatively, p = 0.001; postoperatively, p = 0.8). Cephalic indices also normalized with treatment (craniosynostosis versus control: preoperatively, p = 0.02; postoperatively, p = 0.13). No cases of hydrocephalus were observed. Conclusion Endoscopic suturectomy and helmeting improves anterior turricephaly and corrects frontal bossing and brachycephaly in patients with bilateral coronal craniosynostosis. Clinical question/level of evidence Therapeutic, IV.

Background The purpose of this study was to quantify change in cranioorbital morphology from presentation, after fronto-orbital advancement, and at 2-year follow-up. Methods Volumetric, linear, and angular analyses were performed on computed tomographic scans of consecutive bilateral coronal synostosis patients. Comparisons were made across three time points, between syndromic and nonsyndromic cases, and against normal controls. Significance was set at p Results Twenty-five patients were included: 11 were nonsyndromic, eight had Saethre-Chotzen syndrome, and six had Muenke syndrome. Total cranial volume was comparable to normal, age-matched control subjects before and 2 years after surgery despite an expansion during surgery. Axial and sagittal vector analyses showed advancement and widening of the lower forehead beyond control values with surgery and comparable anterior position, but increased width compared to controls at 2 years. Frontal bossing decreased with a drop in anterior cranial height and advanced lower forehead position. Middle vault height was not normalized and turricephaly persisted at follow-up. Posterior fossa volume remained lower at all three time points compared to control subjects. Supraorbital retrusion relative to anterior corneal position was overcorrected by surgery, with values comparable to those of control subjects at 2 years because of differential growth. There was no difference at 2 years between syndromic and nonsyndromic groups. Conclusions Open fronto-orbital advancement successfully remodels the anterior forehead but requires overcorrection to be comparable to normal at 2 years. Although there are differences in syndromic cases at presentation, they do not result in significant morphometric differences on follow-up. Posterior fossa volume remains lower at all time points. Clinical question/level of evidence Therapeutic, IV.

Changes made to the forehead and periorbital region can have dramatic effects in gender-affirmation surgery. Removal of frontal bossing and alteration of orbital shape can result in significant facial feminization. This elective surgical intervention must be safe, reliable, and aesthetically effective. The described technique of frontal and periorbital bone contouring allows for a safe and consistent surgical outcome in properly selected patients. Nuances in specific technical maneuvers in this operation can have profound effects on safety and aesthetic outcomes. In this article, specific points are detailed in text and video describing the senior author's (E.D.R) surgical approach.

BACKGROUND The combination of sagittal and metopic synostosis is rare, resulting in a scaphocephalic shape, but with an absence of frontal bossing and therefore varying degrees of trigonocephaly and occipital prominence. Treatment is primarily surgical, with a combination of procedures to address both the scaphocephaly and trigonocephaly required involving multiple operations. The authors discuss their experience of treating combined trigonoscaphocephaly in a single-stage procedure and propose a management strategy based on the severity of the presenting deformity. METHODS The Oxford Craniofacial Unit database was searched from inception in October of 2004 to August of 2013 to identify all patients with combined sagittal and metopic synostosis. Case notes were then manually searched to identify those patients who had true trigonoscaphocephaly. RESULTS Of 2856 patients in the authors' database, a total of nine were identified as having had true trigonoscaphocephaly. Seven of these patients underwent a combined single-stage procedure with an average cephalic index of 68.7 percent preoperatively and 80.3 percent postoperatively. CONCLUSIONS Management of trigonoscaphocephaly has been traditionally performed by multiple, staged surgical procedures. The authors propose that it can instead be managed in a single surgical procedure, with the choice of procedure determined by the severity of the deformity. If the deformity is mild to moderate with no occipital bullet, a combined fronto-orbital advancement remodeling and subtotal calvarial remodeling can be performed; however, if there is an occipital bullet, the authors propose the combination of fronto-orbital advancement remodeling and total calvarial remodeling performed in one operation with the patient turned from prone to supine intraoperatively. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.

Background Several patients with ocular dystopia and craniofacial differences have been found to have orbital dysplasia and a frontal bone defect. This deformity is characteristically different from differential diagnoses of encephalocele, sphenoid dysplasia, craniofacial dysostoses, or atypical clefting. Methods The authors retrospectively reviewed the craniofacial registries of two pediatric centers for patients presenting with ocular dystopia or orbitofrontal anomalies between 2000 and 2014. The features and treatment of these patients were analyzed. Results Four patients with congenital orbitofrontal bone dysplasia were identified, three with unilateral and one with bilateral frontal bone defects. Clinical signs of hypoglobus and vertical ocular dystopia of an average of 5.0 mm on the affected side were noted shortly after birth. The transversely oriented bony defect had an average surface area of 3.9 cm in unilateral cases and 10.7 cm in bilateral cases. Patients showed a characteristic orbital vertical elongation with an average orbital height-to-width ratio of 1.30-in excess of the average normal 1.14 by 14 percent-and inferior rim displacement. Cranial contour demonstrated frontal bossing and borderline dolichocephaly. Fronto-orbital reconstruction was performed in three patients, using cranial bone grafting to obliterate the orbital roof defect and elevate the orbital floor, which was successful in reducing ocular dystopia and preserving vision in each patient. One patient followed for 11 years postoperatively has a durable result with no surgical revision. Conclusions Orbitofrontal bone dysplasia has not been previously reported, and includes a frontal bone defect and ocular dystopia. Single-stage fronto-orbital reconstruction appears to adequately correct it. Clinical question/level of evidence Therapeutic, IV.

Background Aims of surgical correction for isolated sagittal synostosis are functional and aesthetic. Multiple surgical techniques exist; however, reliable assessment of aesthetic outcome is poorly documented, limiting direct comparisons. The pinched appearance of the temporal regions is particularly challenging to correct. A visual analogue scale was designed to grade skull shape in patients who had total or subtotal calvarial remodeling for isolated sagittal synostosis. Methods Twenty-two assessors graded preoperative and postoperative photographs from 42 consecutive cases of sagittal synostosis under a single surgeon. Five aspects were graded (i.e., narrow elongated skull, frontal bossing, temporal pinching, occipital bullet, and overall shape) from 0 (normal) to 100 (severe). Interobserver and intraobserver agreement were analyzed by calculating within-subject standard deviation, coefficient of variation, and intraclass correlation coefficient. Linear regression analysis determined predictors of outcome. Results Surgery improved outcome dramatically across all five aspects of skull shape, with a 72.6 to 76.4 percent decrease in severity score. Improvements in severity score were greater after total calvarial remodeling, and type of calvarial remodeling (total versus subtotal) was an independent predictor of outcome in all aspects of skull shape (p≤0.001). Temporal pinching was improved in a subset of patients who also had onlay bone grafts in this region. Conclusions Calvarial remodeling is a powerful technique for improving skull shape. A panel can detect gross and subtle aesthetic changes after surgical correction of sagittal synostosis using a visual analogue scale, with moderate interobserver and intraobserver agreement. This provides a tool for future outcome assessment. Clinical question/level of evidence Therapeutic, IV.

Frontal bossing is a displeasing prominence of the supraorbital area. Aesthetic correction has been described as an open surgical technique. This report introduces an endoscopic approach to correction.A retrospective review was performed on all patients who underwent endoscopic repair of frontal bossing from 2002 to 2009. Demographics, intraoperative and postoperative course, and outcome variables were collected. Standardized preoperative and postoperative photographs were used for analysis. Aesthetic improvement of the forehead was assessed with a four-point scale (1 = no improvement at all, 4 = significant improvement) by six randomly selected observers. Preoperative facial imaging (lateral skull radiography or computed tomography) was obtained on all patients to assess the thickness of the anterior wall of the frontal sinus.Ten patients met the study inclusion criteria. Overall, the observers assessed the degree of frontal bossing correction as moderate improvement (2.67 on a four-point scale survey). All endoscopic frontal bossing corrections were performed on an outpatient basis by the senior author (B.G.). No patient was admitted to the hospital for postoperative complications. No complications (such as alopecia, hematoma, contour deformities, or penetration into the frontal sinus) were seen. The limiting factor in achieving optimal outcomes was inadequate thickness of the anterior wall of the frontal sinus. All patients had various concurrent facial rejuvenation procedures.Endoscopic correction of frontal bossing is an effective and safe surgical technique in forehead aesthetics. This technique is ideal for patients with a mild frontal bossing deformity who have an adequately thick anterior frontal sinus wall.

There is controversy regarding whether the frontal bossing associated with sagittal synostosis requires direct surgical correction or spontaneously remodels after isolated posterior cranial expansion. The authors retrospectively measured changes in frontal bone morphology in patients with isolated sagittal synostosis 2 years after open posterior and midvault cranial expansion and compared these changes with those occurring in age-comparable healthy control groups.Forty-three patients age 1 year or younger (mean, 6 months) with sagittal synostosis underwent computed tomography scan digital analysis immediately after and 2 years after posterior-middle cranial vault expansion. Quantitative angular and linear measures were taken along the midsagittal and axial planes to capture both aspects of frontal bossing. The change in values over the 2 years were compared with healthy controls with normal computed tomography scans taken to rule out head trauma.All measures indicative of frontal bossing decreased significantly from the time of posterior-middle vault expansion to 2 years postoperatively. Whereas the majority of patients at time of the operation had frontal bossing measures greater than two standard deviations outside the age-comparable control mean, almost all patients were within two standard deviations of the norm 2 years later. Lateral forehead bossing and anterior cranial growth was greater the older the patient was at the time of the operation, suggesting that the more time that passed before the operation, the more compensatory anterior fossa growth occurred. Central forehead position relative to the anterior cranial base was greatest in the younger patients at the time of operation, suggesting that a central forehead bulge was an early compensatory response to premature sagittal fusion.As a group, patients with sagittal synostosis start to normalize their forehead morphology within 2 years if an isolated posterior operation is performed at 1 year of age or younger, and this occurs by a combination of restriction of growth and reduction relative to patients without synostosis. This protocol decreases the risks of intraoperative positioning, forehead contour deformities, and two-stage operations.Therapeutic, III.

Background: The authors present a technique for correction of sagittal synostosis addressing the transverse constriction and providing foreshortening of the skull without the use of hardware. The design of the osteotomies combined with strict postoperative supine positioning allows foreshortening of the skull, with concomitant expansion. Methods: Twenty-two patients were operated on using the foreshortening and lateral expansion activated by gravity technique. Postoperative follow-up included clinical evaluation and three-dimensional computed tomographic scanning at 4 to 6 weeks, 1 year, and yearly afterward. These scans were analyzed using three-dimensional surface analysis software, according to a protocol the authors describe in this article. Results: The cephalic index improved from 66.4 preoperatively to 74.5 at 1 year postoperatively (normal, 72 to 87). Transverse growth continued to be the dominant vector of growth up to 1 year (until ossification-bridging of the vertex craniectomy). Beyond 1 year, the main area of transverse growth was overlying the temporal suture, producing a distinctive temporal bossing in patients. The parietal areas showed minimal growth afterward. Growth was mainly occipital between 1 and 3 years of age, and frontal afterward, up to 5 years of age. A low frontal bossing also occurred in some of our patients and resolved spontaneously. Conclusions: The authors have described a technique for correction of scaphocephaly that avoids the use of hardware, with acceptable operative times and transfusion needs. Postoperative improvement in shape is satisfactory. Analysis of the three-dimensional scans of these patients shows abnormal skull growth patterns up to 5 years of age.

An algorithm for the management of sagittal synostosis in older children who underwent delayed cranial vault reconstruction is presented. This algorithm tailors the surgical approach to the specific craniofacial deformity present in each case. The scaphocephalic deformity characteristic of sagittal synostosis varies significantly when presentation is delayed beyond the first year of life, the time during which reconstruction is usually performed. Sixteen patients with sagittal synostosis who presented after 12 months of age, and were a mean of 3.2 years of age at the time of cranial vault reconstruction, were reviewed. Four patients demonstrated preoperative symptoms and objective findings indicative of increased intracranial pressure, including frequent headaches and emesis, papilledema, or digital markings on computed tomographic scan. Each of the 16 patients underwent either (1) single-stage total vault reconstruction with or without concomitant fronto-orbital expansion; (2) two-stage total vault reconstruction with anterior two-thirds vault expansion followed by transverse occipital expansion and recession a mean of 8.7 months later; or (3) anterior two-thirds vault reconstruction with or without fronto-orbital expansion. In each case, the extent of the scaphocephalic deformity determined the procedure used. The presence of severe frontal bossing associated with transverse restriction of the orbitotemporal region was an indication for fronto-orbital expansion in addition to vault reconstruction, whereas significant occipital protrusion was an indication for transverse posterior vault expansion and recession in addition to anterior two-thirds vault reconstruction. Excellent aesthetic results were obtained in all cases regardless of the type of reconstruction performed. However, it is essential that the extent of the deformity be carefully evaluated preoperatively to permit selection of the appropriate technique for reconstruction.

An unusual case of frontal bossing due to excess subcutaneous fibroadipose tissue in a 38-year-old woman with Möbius syndrome is presented. A relatively simple transcoronal resection of soft tissue resulted in successful elimination of this type of frontal bossing.