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Introduction: Prevalence of intracranial aneurysms in children with Apert syndrome has not been described, and development of an aneurysm as a complication secondary to craniofacial surgery has never been reported. Case Presentation: We report the rare case of a 10-year-old boy with Apert syndrome who underwent craniofacial reconstruction surgery consisting of subcranial Le Fort III osteotomies, bilateral lateral canthopexies, and nasal nares dilations for midfacial hypoplasia and resultant obstructive sleep apnea, and on routine follow-up magnetic resonance imaging (MRI) 1 year later, he was found to have a large left ophthalmic internal carotid artery (ICA) aneurysm that was not seen on MRI obtained 2 years prior. Immediately after the craniofacial surgery, the patient experienced a severe headache behind his left eye and extraocular movement abnormalities that subsided over the next days to months. Given the new and rapid growth of the aneurysm on follow-up MRI, the patient underwent a diagnostic cerebral angiogram followed by successful flow diversion treatment of the aneurysm with the pipeline embolization device (Medtronic, Dublin, Ireland). Conclusion: Post-procedurally, over the next year, the patient developed word-finding difficulty and stuttering speech. He was found to have in-stent ICA stenosis and middle cerebral artery (MCA) stenosis at the first follow-up and underwent an initial angioplasty. After several weeks, ICA, MCA, and anterior cerebral artery stenoses were identified, and the patient underwent angioplasties for the ICA and MCA stenoses. On follow-up examination after the second procedure, the patient had tremendous improvement in his speech difficulties and was doing well clinically.

Numerous children born with syndromic craniosynostosis will develop visual impairments. Based on the hypothesis that elevations in intracranial pressure might have greater impacts on vision than development, this review sought to ascertain the prevalence of optic nerve atrophy in syndromic craniosynostosis and to look for potential predictive factors.The authors conducted a retrospective chart review of all children with syndromic craniosynostosis treated at a single center.Of 442 patients with syndromic craniosynostosis, complete ophthalmologic records were available for 253. Although no instances of optic nerve atrophy were noted among those with Saethre-Chotzen or Muenke syndromes, an overall 14.7 percent prevalence was noted among those with Apert (7.8 percent), Crouzon (27.9 percent), and Pfeiffer syndromes (23.1 percent), with initial diagnoses occurring at a mean age of 10 years. The presence of a Chiari malformation was found to significantly correlate with the subsequent diagnosis of optic nerve atrophy (OR, 3.544; p = 0.002); however, the timing of the first cranial vault procedure, presence of a ventriculoperitoneal shunt, degree of brachycephaly, number of vault expansions, and diagnosis of sleep apnea, did not show significant associations.A substantial percentage of children with Apert, Crouzon, and Pfeiffer syndromes were found to develop optic nerve atrophy, with a prevalence likely to trend higher with longer follow-up. Chiari malformations were the only significant potential predictor for optic nerve atrophy. With the goal of preventing visual losses, more frequent monitoring for raised intracranial pressure with ophthalmologic evaluations and magnetic resonance imaging measurements of optic nerve sheath diameters should be considered.Risk, III.

BACKGROUND Does bifrontal width change with growth following trigonocephaly corrections? Postoperative long-term growth was assessed, along with the impacts of phenotypic severity, surgical timing, and operative technique, to determine how wide to surgically set bifrontal width. METHODS A retrospective review of all trigonocephaly repairs was performed. Exclusion criteria included syndromic conditions, incomplete records, and follow-up under 1 year. Anthropometric measurements taken through completion of growth were evaluated and compared to sex- and age-matched normal standards for Z score conversion. RESULTS Of 370 consecutive patients undergoing repairs, 95 had sufficient anthropometric data. The mean surgical age was 10.8 months (range, 2 months to 7 years) and mean follow-up was 54.3 months (range, 12 months to 17.8 years). Sequential measurements revealed progressive increases in bifrontal width. However, following conversion to Z scores, the initial overcorrection (mean overcorrection, 8.7 mm; mean Z score, +2.3) steadily diminished to an undercorrection (mean measurement, -5.5 mm; mean Z score, -1.1). Compared to treatment at an older age (10 to 12 months), repairs performed in those younger than 8 months showed worse growth (p = 0.004). Those more severely affected (lowest bifrontal Z scores) had growth similar to that of those more mildly impacted. Only two patients (2.1 percent) underwent secondary procedures for recurrences. No correlation was found between anthropologic measurements and observers' severity assessments. CONCLUSIONS Subnormal bifrontal growth occurs following trigonocephaly corrections, especially with earlier corrections. Repairs performed at approximately 11 months of age had to be overcorrected by approximately 1.5 cm to produce a normal bifrontal width at maturity. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.

PURPOSE The Pediatric Craniofacial Collaborative Group recently reported pooled perioperative data from 31 North American centers performing open cranial vault remodeling procedures. The authors sought to determine if outcomes were different at a single higher-volume center and if identified, ascertain reasons for any differences and propose strategies for improvement. METHODS A retrospective review was performed of all open pediatric cranial vault procedures performed at our center during the identical 3.25-year period reported by the Collaborative group, including demographic, perioperative management and outcome data, to permit multiple comparative analyses. RESULTS The 310 procedures were performed by our center during this time period, compared to 1223 by the combined 31 institutions (median: 29.5 cases/center; interquartile range: 12-54.5). Multiple outcome differences were found: our higher-volume center had a significantly lower overall red blood cell transfusion rate (≤2 years: 7.5 percent vs 91 percent, P

Background The authors sought to ascertain the upper limits of secondary skull defect size amenable to autogenous reconstructions and to examine outcomes of a surgical series. Published data for autogenous and alloplastic skull reconstructions were also examined to explore associations that might guide treatment. Methods A retrospective review of autogenously reconstructed secondary skull defects was undertaken. A structured literature review was also performed to assess potential differences in reported outcomes between autogenous bone and synthetic alloplastic skull reconstructions. Weighted risks were calculated for statistical testing. Results Ninety-six patients underwent autogenous skull reconstruction for an average defect size of 93 cm (range, 4 to 506 cm) at a mean age of 12.9 years. The mean operative time was 3.4 hours, 2 percent required allogeneic blood transfusions, and the average length of stay was less than 3 days. The mean length of follow-up was 28 months. There were no postoperative infections requiring surgery, but one patient underwent secondary grafting for partial bone resorption. An analysis of 34 studies revealed that complications, infections, and reoperations were more commonly reported with alloplastic than with autogenous reconstructions (relative risk, 1.57, 4.8, and 1.48, respectively). Conclusions Autogenous reconstructions are feasible, with minimal associated morbidity, for patients with skull defect sizes as large as 500 cm. A structured literature review suggests that autogenous bone reconstructions are associated with lower reported infection, complication, and reoperation rates compared with synthetic alloplasts. Based on these findings, surgeons might consider using autogenous reconstructions even for larger skull defects. Clinical question/level of evidence Therapeutic, IV.

BACKGROUND: Ocular pathology in craniosynostosis is a persistent concern for patients and providers, and some surgeons feel that early surgical intervention for synostosis alleviates the progression of ophthalmologic abnormalities. In contradistinction, we hypothesize that operating early will have no bearing on post-operative ophthalmologic outcomes. METHODS: Single-suture craniosynostosis patients who underwent surgical correction between 1989 and 2015 were reviewed. Patients with multi-suture craniosynostosis, syndromic diagnoses, no pre-operative ophthalmology evaluation, and less than two years of follow-up were excluded. Logistic regression was used to determine odds of pre- and post-operative ophthalmologic abnormalities by age, while controlling for patient-level covariates. RESULTS: 172 patients met inclusion criteria. The median age at surgery was 10 months (IQR 7–12.9 months). Increasing age at the time of surgery was associated with increased odds of pre-operative ophthalmologic diagnoses (OR: 1.06; p=0.037) but not post-operative diagnoses (OR: 1.00; p=0.91). Increasing age at surgery was also not associated with increased odds of ophthalmologic diagnoses, regardless of timing (OR 1.04, p=0.08). Patients with coronal synostosis (OR 3.94, p=0.036) had significantly higher odds of pre-operative ophthalmologic diagnoses. Patients with metopic (OR: 5.60; p

Background:. Although the natural history of nonsyndromic unilateral coronal craniosynostosis has been extensively described, optimal management remains controversial due to lack of Level 1 evidence. This study aims to assess the current state of practice among craniofacial surgeons. Methods:. Ninety-four craniofacial surgeons were approached to complete a survey consisting of 15 questions. Data were collected assessing surgeons’ primary surgical indication, timing of intervention, preoperative imaging, and choice of technique for patients presenting with nonsyndromic unilateral coronal craniosynostosis. Choice of technique and timing of intervention in case of recurrence were also investigated. Results:. After 5 mailings, the response rate was 61%. The combination of both appearance and raised intracranial pressure was the primary indication for treatment for 73.2% of surgeons. Preoperative CT scan of the skull was “always” performed by 70.1% of respondents. Open surgical management was most commonly performed at 8–10 months of age (38.6%). Bilateral frontal craniectomy with remodeling of the supraorbital bandeau and frontal bone was the most common choice of procedure (84.2%). In case of mild to moderate and moderate to severe recurrences at 1 year of age, 89.5% and 47.4% of surgeons opted for conservative management, respectively. Optimal timing for repeat cranioplasty was after 4 years of age (65.5%). Overall, 43.4% quoted lack of evidence as the greatest obstacle to clinical decision-making when dealing with unilateral synostosis. Conclusion:. This survey exposes the lack of consensus and the disparity of opinion among craniofacial surgeons regarding the management of nonsyndromic coronal synostosis, particularly in the setting of recurrence.

OBJECTIVE This study examines a series of patients with hypophosphatemic rickets and craniosynostosis to characterize the clinical course and associated craniofacial anomalies. METHODS A 20-year retrospective review identified patients with hypophosphatemic rickets and secondary craniosynostosis at 3 major craniofacial centers. Parameters examined included sex, age at diagnosis of head shape anomaly, affected sutures, etiology of rickets, presenting symptoms, number and type of surgical interventions, and associated diagnoses. A review of the literature was performed to optimize treatment recommendations. RESULTS Ten patients were identified (8 males, 2 females). Age at presentation ranged from 1 to 9 years. The most commonly affected suture was the sagittal (6/10 patients). Etiologies included antacid-induced rickets, autosomal dominant hypophosphatemic rickets, and X-linked hypophosphatemic (XLH) rickets. Nine patients had undergone at least 1 cranial vault remodeling (CVR) surgery. Three patients underwent subsequent surgeries in later years. Four patients underwent formal intracranial pressure (ICP) monitoring, 3 of which revealed elevated ICP. Three patients were diagnosed with a Chiari Type I malformation. CONCLUSIONS Secondary craniosynostosis develops postnatally due to metabolic or mechanical factors. The most common metabolic cause is hypophosphatemic rickets, which has a variety of etiologies. Head shape changes occur later and with a more heterogeneous presentation compared with that of primary craniosynostosis. CVR may be required to prevent or relieve elevated ICP and abnormalities of the cranial vault. Children with hypophosphatemic rickets who develop head shape abnormalities should be promptly referred to a craniofacial specialist.

Reconstruction of the craniosynostosis deformity is a relatively safe operation with low overall complication risks. Despite expected risk of significant blood loss, life-threatening bleeding is relatively rare, and there is a low incidence of reported deaths in the literature. Several modalities have been described for perioperative mitigation of blood loss and transfusion requirements. Due to the low overall risk of life-threatening bleeding and circulatory collapse, it is judicious that any potential causes of such unusual but potentially significant fatal bleeding complication be evaluated and reported to increase awareness for craniofacial surgeons treating these conditions. In this report and literature review, the authors present a highly unusual patient with significant bone bleeding and circulatory collapse in a metopic craniosynostosis patient with guanine nucleotide-binding protein alpha stimulating (GNAS) mutation; perform a literature review regarding bleeding diathesis in craniosynostosis patients with GNAS mutations; and suggest guidelines to potentially prevent mortality in such patients.

BACKGROUND Following correction of anterior sutural fusions, long-term forehead irregularities may arise. Based on the premise that frontal reconstructions using a seamless construct might produce better long-term aesthetic results, the supraorbital bandeau was abandoned for a single-piece frontal reconstruction. The purpose of this review was to compare outcomes and complications between children who underwent reconstruction with the traditional supraorbital bandeau and those who underwent reconstruction with a single bony segment. METHODS A retrospective sequential chart review was performed of all children undergoing anterior sutural fusion repairs to compare supraorbital bandeau to single-segment reconstructions. Length of surgery, blood loss, hospital stay, and complications were assessed. Aesthetic outcomes and reoperation rates were also examined. RESULTS Over 10 years, 199 patients with anterior sutural fusions underwent correction: 124 with a single-piece frontal reconstruction and 75 with traditional supraorbital bandeaus. Outcome analyses revealed no significant differences between techniques with respect to blood loss, surgical times, complication rates, and hospital length of stay. A preliminary analysis suggested lower reoperation rates (0 percent versus 2.7 percent) and more favorable aesthetic rankings for those treated with a single segment, although the follow-up for this group was significantly shorter compared with those treated with a supraorbital bandeau (16 months versus 43 months). CONCLUSIONS Abandoning the traditional supraorbital bandeau for a simplified single-segment frontal reconstruction has the potential to result in improved forehead aesthetics, although longer term evaluations are needed. This review suggests that this technical variation does not appear to be associated with longer surgical times, increased blood loss, or higher complication rates. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, III.

BACKGROUND Understanding mandibular growth in children with fibroblast growth factor receptor 2 (FGFR2) mutations is important for planning the degree of midface advancement, and for determining the best treatment for obstructive sleep apnea. Yet, relatively little is known about growth of the unoperated mandible in affected children. The purpose of this study was to evaluate mandibular growth through skeletal maturity in Apert, Crouzon, and Pfeiffer syndromes. METHODS A retrospective chart review was performed. Long-term, unoperated mandibular growth was assessed using multiple anthropologic measurements including: mandibular width, height, depth, and the cranial base width (approximating bicondylar width). Measurements were compared over 3 age intervals: 6 to 7 years, 10 years, and at skeletal maturity (15 years+). RESULTS Out of 327 treated patients with FGFR2 mutations, 21 were found to have complete mandibular measurements through skeletal maturity (11 Apert, 7 Crouzon, and 3 Pfeiffer). Initial measurements revealed that mandibular height and bigonial breadth were slightly larger than normal, but sagittal depth and cranial base width were deficient. Early growth was slightly accelerated along the vertical and sagittal axes, stable across the bigonial distance, and marked deficient at the cranial base. At skeletal maturity, vertical height and bigonial width remained above average, but mandibular depth (forward sagittal growth) and cranial base width, remained deficient. CONCLUSIONS Mandibular growth in children with FGFR2 mutations is not normal with impairments found in forward sagittal growth and skull base widening. Knowledge of these deficiencies has significant implications for both planning the degree of midfacial advancements, as well as treating obstructive sleep apnea.

Craniosynostosis affecting the lambdoid suture is uncommon. The definition of lambdoid craniosynostosis solely applies to those cases demonstrating true suture obliteration, similar to other forms of craniosynostosis. In patients presenting with posterior plagiocephaly, true lambdoid craniosynostosis must be differentiated from the much more common positional molding. It can occur in a unilateral form, a bilateral form, or as part of a complex craniosynostosis. In children with craniofacial syndromes, synostosis of the lambdoid suture most often is seen within the context of a pansynostotic picture. Chiari malformations are commonly seen in multisutural and syndromic types of craniosynostosis that affect the lambdoid sutures. Posterior cranial vault remodeling is recommended to provide adequate intracranial volume to allow for brain growth and to normalize the skull shape. Although many techniques have been described for the correction of lambdoid synostosis, optimal outcomes may result from those techniques based on the concept of occipital advancement.

BACKGROUND Hypotensive anesthesia is routinely used during craniosynostosis corrections to reduce blood loss. Noting that cerebral oxygenation levels often fell below recommended levels, the authors sought to measure the effects of hypotensive versus standard anesthesia on blood transfusion rates. METHODS One hundred children undergoing craniosynostosis corrections were randomized prospectively into two groups: a target mean arterial pressure of either 50 mm Hg or 60 mm Hg. Aside from anesthesiologists, caregivers were blinded and strict transfusion criteria were followed. Multiple variables were analyzed, and appropriate statistical testing was performed. RESULTS The hypotensive and standard groups appeared similar, with no statistically significant differences in mean age (46.5 months versus 46.5 months), weight (19.25 kg versus 19.49 kg), procedure [anterior remodeling (34 versus 31) versus posterior (19 versus 16)], or preoperative hemoglobin level (13 g/dl versus 12.9 g/dl). Intraoperative mean arterial pressures differed significantly (56 mm Hg versus 66 mm Hg; p < 0.001). The captured cell saver amount was lower in the hypotensive group (163 cc versus 204 cc; p = 0.02), yet no significant differences were noted in postoperative hemoglobin levels (8.8 g/dl versus 9.3 g/dl). Fifteen of 100 patients (15 percent) received allogenic transfusions, but no statistically significant differences were noted in transfusion rates between the hypotensive [nine of 53 (17.0 percent)] and standard anesthesia [six of 47 (13 percent)] group (p = 0.056). CONCLUSIONS No significant difference in transfusion requirements was found between hypotensive and standard anesthesia during craniosynostosis corrections. Considering potential benefits of improved cerebral blood flow and total body perfusion, surgeons might consider performing craniosynostosis corrections without hypotension. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, II.

Object The optimal management of Chiari malformations in the setting of craniosynostosis is not well established. In this report the authors describe their outcomes with the combined technique of simultaneous suboccipital decompression (SOD) during posterior cranial vault remodeling (PCVR). Methods A retrospective review was performed of all patients undergoing PCVR and simultaneous SOD. Demographic data, diagnosis, imaging studies, operative intervention, and clinical follow-up were evaluated. Results Thirty-four patients were identified as having undergone a simultaneous PCVR/SOD for Chiari malformation associated with craniosynostosis. Eighty-eight percent of these patients had syndromic, multisutural craniosynostosis, and the remaining patients had unilateral lambdoid craniosynostosis. There were no postoperative complications as a direct result from this combined procedure. Two patients required a subsequent direct approach for decompression of the Chiari malformation. The interval between these subsequent surgeries was 3 years and 19 months. Conclusions Chiari malformations are commonly associated with syndromic, complex craniosynostosis and isolated lambdoid craniosynostosis. In appropriately selected patients, a combined posterior cranial vault enlargement and SOD of the foramen magnum is associated with a low complication rate and appears to be an effective procedure.

The authors catalogued phenotypic variability among children with Apert syndrome, reviewed surgical outcomes (particularly with respect to their treatment goals of avoiding preventable developmental delays and reducing operative interventions), and examined correlations that might stimulate improved treatment paradigms.A case series review of all Apert syndrome patients, treated by a single surgeon, including phenotypic variations, mutational analyses, developmental assessments, and surgical treatments, was performed.Over a 20-year period, 135 Apert syndrome patients were treated (32 percent from birth). A fairly even distribution of mutations was noted (S252W, n = 20; P253R, n = 18). Of 268 hands, 60 percent were type I, 21 percent were type II, and 19 percent were type III. Fifty percent had palatal anomalies. Three separate skull configuration types were identified, and 29 percent had acquired Chiari malformations, 24 percent had anomalies of the septum pellucidum, and 12 percent had anomalies of the corpus callosum. Cranial and midfacial procedures were performed significantly earlier at outside centers (6.2 months versus 12.6 months, and 5.3 years versus 7.5 years). No significant correlations were noted between development and gene mutation, hand or skull phenotypes, intracranial anomalies, and timing of initial skull surgery. A significant correlation was noted between adverse development and ventriculoperitoneal shunts, tracheostomies, and more operative interventions. Higher development strongly correlated with treatment at our center from birth.Treatment goals focused on the prevention of avoidable developmental delays (from raised intracranial pressure and sleep apnea) and reducing operative interventions may potentially improve developmental outcomes.Therapeutic, III.

A relationship between lambdoid craniosynostosis and Chiari deformations has been suggested, but the true extent of this association remains uncertain. The authors reviewed a single center's experience treating lambdoid synostosis to further elucidate this relationship, examine surgical outcomes, and identify associations that might impact future treatments.A retrospective chart review was performed of all patients treated for lambdoid craniosynostosis, excluding the syndromic craniosynostoses. Operative data, scans, hospitalization, and subsequent surgical procedures were tracked. All patients were treated with remodeling procedures, but those with Chiari deformations underwent additional simultaneous suboccipital decompressions.Over 22 years, 1006 nonsyndromic craniosynostosis patients were treated, 45 of whom (4.5 percent) presented with lambdoid involvement: 25 single-suture and 20 multiple-suture (complex craniosynostosis). Magnetic resonance imaging revealed that 60 percent of children with unilateral synostosis and 71 percent with a complex synostosis had associated Chiari deformations. The mean surgical age was 12 months, and the average follow-up was 5.7 years: two patients developed syringomyelia requiring transcervical decompressions and two underwent secondary posterior remodeling procedures (one unilateral and one complex synostosis) while undergoing later Chiari decompressions. No patients treated with initial suboccipital decompressions have subsequently developed symptoms requiring treatment.The majority of children with lambdoid synostosis develop Chiari deformations; therefore, routine preoperative and postoperative magnetic resonance imaging should be considered. The treatment of lambdoid craniosynostosis with cranial remodeling procedures, including incontinuity suboccipital decompressions when Chiari deformations were present, was associated with few complications. Ninety-six percent of those with isolated fusions were managed with a single procedure.Therapeutic, IV.

BACKGROUND: Hydrocephalic macrocephaly, occurring despite adequate cerebrospinal fluid shunting, is rare, and most publications advocate near-total cranial vault reduction procedures. The authors reviewed our series of limited reductions (designed to minimize complications while still providing functional benefits) to evaluate outcomes. METHODS: All patients undergoing posterior reduction cranioplasties were retrospectively reviewed for outcomes, including operative data, length of stay, preoperative and postoperative anthropometrics, and complications. In addition, preoperative and postoperative motor function was assessed using a novel scale. These data were then compared with published series. RESULTS: Ten patients (five male, five female) underwent reduction cranioplasties for macrocephaly at an average age of 17.9 months (range, 6 to 53 months) and were followed for an average of 41.5 months. The mean operative time was 4.9 hours (range, 4.3 to 6.5 hours), estimated blood loss was 530 ml (range, 200 to 1500 ml), and 78 percent received blood transfusions. The average length of hospitalization was 2.6 days. Three patients experienced complications, including one shunt revision. The mean functional assessment scores increased from 2.3 to 3.9 (p = 0.022), with all patients able to support their heads postoperatively. CONCLUSIONS: Use of a limited cranioplasty technique was associated with a hospitalization that was over 7 days shorter than has been reported in the literature for total cranial vault reductions and with a much lower shunt revision rate. Measurable improvements in motor function and subjective benefits in appearance were noted, despite a more limited reduction. Surgeons faced with this unusual condition may wish to consider performing this smaller procedure.

Background: The first combined evaluation of morbidity and mortality rates in craniofacial surgery was published 30 years ago; many surgeons believe these procedures have since become safer. The authors performed a contemporary evaluation of craniofacial morbidity and mortality rates to help surgeons more accurately counsel families about current risks, and to gain insight into reducing future incidences. Methods: This study used two methodologies to capture all serious morbidities and mortalities associated with major craniofacial procedures between 1990 and 2008: a comprehensive two-center retrospective review (Dallas and Seattle), and an Internet-based survey sent to all North American craniofacial centers. Results: Combining the two-center data with the survey results yielded a database of 7328 intracranial and 773 subcranial procedures, for a total of 8101 major craniofacial procedures. The combined intracranial major morbidity rate was less than 0.1 percent, and the combined mortality rate was 0.1 percent. Of the eight perioperative deaths following intracranial procedures, four (50 percent) intracranial mortalities were directly attributed to blood loss. The combined subcranial procedure major morbidity rate was 0.1 percent and the mortality rate was 0.3 percent (airway related). Comparing the earliest published intracranial mortality rate to our current review revealed a statistically significant reduction in incidence (p < 0.001). Conclusions: The incidence rates for serious morbidities and mortalities among major craniofacial procedures have significantly fallen since first published. On the basis of these analyses, the authors believe that a greater focus on protocols for airway management, blood salvage and replacement, age-appropriate deep venous thrombosis prophylaxis, and timing of subcranial midfacial advancements might result in further reduction in craniofacial mortality rates.

Background: The complex craniosynostoses, which include all nonsyndromic multiple sutural fusions, represent a small fraction of patients presenting with craniosynostosis. Among these are a trisutural fusion, dubbed the "Mercedes Benz pattern," involving the sagittal and both lambdoid sutures. The purpose of this report is to review the authors' series of this unusual form of craniosynostosis, to identify associated anomalies, and to assess treatment outcomes. Methods: The authors conduced a retrospective clinical outcome assessment of all patients presenting with Mercedes Benz pattern craniosynostosis. Growth was assessed by direct anthropologic measurements, and significance was assessed by the t test. Results: Over a 17-year period, 11 of 802 patients presenting with craniosynostosis were identified with Mercedes Benz pattern synostosis (1.4 percent). Three patients had additional sutural involvement and two had identifiable genetic syndromes. Seven of 11 (64 percent) had cerebellar tonsillar herniation on preoperative imaging, and four symptomatic patients (36 percent) have required Chiari decompressions. Three patients have required more than one remodeling procedure. Serial postoperative anthropologic measurements identified progressive brachycephaly, with diminished growth in both head circumference and skull length (mean follow-up, 3.75 years). Cognitive function was grossly normal, except for one syndromic patient. Conclusions: Despite fusion of the sagittal suture, the surgical treatment for Mercedes Benz pattern craniosynostosis should include skull lengthening, not reduction. The authors' findings for diminished postoperative growth suggest that an overcorrection be considered. Fourth or fifth sutural involvement correlated with additional corrections. Given the observed high incidence for symptomatic cerebellar tonsillar herniation, routine magnetic resonance evaluations are recommended for affected individuals.

Background: Pfeiffer syndrome is rarely encountered, even at major craniofacial centers. Published reports indicate high mortality rates (25 to 85 percent) for severely affected subtypes. The authors reviewed their surgically treated patients to improve outcomes. Methods: The authors conducted a 17-year, single-center, retrospective outcome assessment of all children treated for Pfeiffer syndrome, with data summarized using descriptive statistics. Results: Of 802 patients treated for craniosynostosis, 28 were identified with Pfeiffer syndrome: 17 were classified as type I (61 percent), seven were classified as type II (25 percent), and four were classified as type III (14 percent). The mean age was 10 years (range, 12 months to 39 years), with an average of 9.3 operations per child (2.5 cranial vaults, 1.1 Le Fort III procedures). Fifty-nine percent had external auditory canal atresia (100 percent of type III patients), and 29 percent had some visual disturbance. Tracheostomies were recommended in 100 percent of type II and III patients, and two type II patients required tracheal stenosis repairs. Eighty-four percent had acquired Chiari malformations (100 percent of type II and III patients), and 61 percent required treatment for hydrocephalus. Fifty percent of shunted patients (mean age, 7 years) have required Chiari decompressions, but no patients undergoing endoscopic third ventriculostomies (mean age, < 3 yearns) have required treatment. The mortality rate was 7 percent, with both deaths occurring at home without proximity to surgery. Conclusions: The authors' mortality rates for type II and III Pfeiffer syndrome are lower than those previously published. The authors believe a preemptory tarsorrhaphy strategy can prevent visual loss and that further reductions in mortality rates are possible with aggressive airway management (early tracheostomies) and more frequent screening (e.g., magnetic resonance imaging, sleep studies) for Chiari malformations.

BACKGROUND This study was designed to examine long-term skull growth following single sutural synostosis corrections and to evaluate surgical outcomes. METHODS A retrospective outcome assessment of all children treated with a single-stage, open-remodeling procedure for sagittal, metopic, unilateral coronal, and unilateral lambdoid synostosis was performed. Long-term growth was assessed from serial anthropometric measurements taken up to 11 years postoperatively (mean, 4 years). RESULTS Of 296 consecutive patients, complete records were available for 248 operative procedures. The mean surgical age was 12.3 months (range, 2.5 months to 8 years), with 36 patients older than 12 months. Transfusion rates decreased from 81 to 19 percent following the institution of blood conservation strategies. The average hospitalization was 2.5 days. There was one nonsurgically treated infection (0.4 percent), and no major complications or deaths. Five patients underwent secondary remodeling procedures (2 percent). Serial anthropometric measurements, obtained in 75 patients, showed normalization of cranial indices 6 weeks postoperatively, but subsequent measurements revealed statistically significant diminished growth. Earlier surgical treatment of metopic synostosis (4 months) was associated with significantly more growth inhibition than seen in those treated at an older age (12 months), with the other synostoses showing similar tendencies. CONCLUSIONS Treatment of single sutural synostosis was extremely safe with very low reoperative rates, but subsequent calvarial growth was abnormal, with a tendency toward recapitulation of the primary deformity. Growth was less diminished in procedures performed in older infants. Surgeons treating single sutural craniosynostosis should consider expanding treatment goals beyond normalization to an overcorrection of the abnormal skull shape.

Computed tomographic scan evaluation is the current standard of care for diagnosing craniosynostosis. Recent publications, and the National Cancer Institute, have raised concerns about ionizing radiation associated with computed tomographic scans in children (e.g., developmental delays, tumor induction). The authors sought to ascertain the diagnostic accuracy of the physical examination in evaluating single-sutural craniosynostosis and assess the need for computed tomographic scans in surgical correction.This prospective, multicenter, outcome assessment included children clinically diagnosed with a single-sutural synostosis by craniofacial surgeons (with 1 to 18 years' experience) at four centers over a 1-year period. Clinical diagnoses were compared with computed tomographic scan evaluations. All surgeons scored the utility of computed tomographic scans during surgical repair.Sixty-seven patients were clinically diagnosed with single-sutural craniosynostosis (mean age, 7 months; range, 1 week to 48 months). Sixty-six of 67 patients were diagnosed with craniosynostosis by computed tomographic scan (sagittal, 40 percent; metopic, 31 percent; right unilateral coronal, 16 percent; left unilateral coronal, 6 percent; and right lambdoid, 6 percent), for a diagnostic accuracy exceeding 98 percent. One patient with suspected lambdoid synostosis was radiologically diagnosed with positional plagiocephaly. Three of four craniofacial surgeons scored computed tomographic scans as "unnecessary" for surgical correction; one scored scans as "sometimes helpful."Craniofacial surgeons with various experience levels were able to accurately diagnose single-sutural synostosis by physical examination alone. Considering potential side effects from ionizing radiation, risks of sedation, and costs, surgeons may wish to reserve computed tomographic scans only for infants with suspected single-sutural craniosynostosis in whom the physical examination is not clearly diagnostic.

The majority of infants with multiple sutural craniosynostosis have identifiable syndromes, which affect both coronal sutures (Apert, Crouzon, Pfeiffer, and so on), and multiple sutural fusions not involving both these sutures are exceeding rare. We report a highly unusual pattern of trisutural fusion, which to our knowledge has not been previously described. A retrospective clinical review was performed of all cases of craniosynostosis, which had presented to the Craniofacial Center in Dallas, over a 15-year period. Two patients were identified with a unique pattern of craniosynostosis involving the left coronal, sagittal, and right lambdoid sutures creating a "Z-pattern." Both patients were treated with staged cranial vault reconstructive procedures with an initial posterior remodeling (3 to 4 months) followed by an anterior repair (7 to 8 months). Both patients developed cerebellar tonsillar herniation, and one symptomatic patient required surgical decompression. With a follow up of greater than 2 years, normocephaly has been maintained with growth, and both children appear to have normal intellectual development. A unique pattern of trisutural "Z-patterned" craniosynostosis is presented. This distinctive pattern of craniosynostosis appears to be associated with cerebellar tonsillar herniation. In this small series, a two-staged procedure successfully normalized the calvarial dysmorphism, and early growth and development appear normal.

Background The authors' center uses a nonnarcotic postoperative regimen following craniosynostosis corrections. Despite opioid avoidance, the authors noted that some children still experienced nausea and vomiting following the oral administration of either acetaminophen or ibuprofen. This study sought to evaluate whether intravenous administration of these medications might reduce nausea and vomiting rates. Methods A total of 50 children undergoing craniosynostosis corrections were prospectively randomized to a control group given only oral ibuprofen (10 mg/kg) and acetaminophen (15 mg/kg), or a treatment group given only intravenous ketorolac (0.5 mg/kg) and acetaminophen (15 mg/kg). All patients were assessed for postoperative nausea and vomiting by a blinded research nurse. Results Twenty-eight patients randomized to the oral control group, and 22 to the intravenous treatment group. No statistically significant differences were identified between groups, including: age, weight, sex, before history of severe postoperative nausea and vomiting, or procedure. With similar anesthesia times there was significantly more vomiting episodes in the oral group (71% versus 41%). Using a multivariate logistic regression, controlling for age, weight and procedure, the odds ratio for vomiting in the oral control versus intravenous experimental groups was 3.61 (95% CI 1.11-1.76; P = 0.033), and for postoperative nausea was 14.0 (95% CI 1.40-71.69, P = 0.010). Conclusions The authors found a significant reduction in nausea and vomiting among children randomized to receive intravenous medications. In addition, the intravenous delivery of medications has the theoretical advantage of insuring an effective full dose delivery. Based on these findings, our standard process is to preferentially manage all children following craniosynostosis corrections with intravenous nonnarcotics.

BACKGROUND In the craniofacial surgery literature, there is a wide disparity of opinions regarding the management of nonsyndromic metopic synostosis. With the lack of level I evidence to support a particular regimen, we aimed to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. METHODS A survey was sent to 102 craniofacial surgeons. The survey featured 2 parts: clinical scenarios and questions regarding the following: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated metopic synostosis. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. RESULTS The total response rate was 72% (73/102) for the clinical scenarios and 63% (64/102) for the complete survey. There was a large discrepancy when classifying and managing mild metopic synostosis, with between 16% and 35% of surgeons electing to operate on a mild case. All surgeons agreed to operate on moderate and severe cases. For 95% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management was most commonly performed at 6 months (29%) of age. Open frontal orbital advancement was the most commonly performed procedure in mild (27%), moderate (77%), and severe (89%) cases. Endoscopic approaches were more likely to be used in milder cases by 19% of surgeons. CONCLUSION Our survey demonstrates that there is a wide disparity of opinion among craniofacial surgeons regarding the diagnosis and management of mild nonsyndromic metopic synostosis. LEVEL OF EVIDENCE Diagnostic, level 5.

Background The authors sought to evaluate current craniofacial training in the United States to achieve perspective on changes over time and to gain insights into possible process improvements. Methods Following a review of the San Francisco Match listings and an Internet search, an anonymous online survey invitation was sent to all fellows finishing in 2013. Results Thirty-three fellows were identified in 29 listed programs and 30 responded (91 percent). All had completed plastic surgery training. A mean caseload of 380 cases (95 percent CI, 307 to 452) was reported. Case analyses permitted subclassification of fellowships into five areas of relative strengths: cleft/intracranial/midface, 35 percent; cleft/general pediatrics, 20 percent; cleft/adult plastics, 20 percent; cleft/facial trauma, 15 percent; and adult plastics/facial trauma, 10 percent of programs. Eighty-six percent were residency-type programs, whereas only 14 percent remained apprenticeships. Fellows cited confidence in any procedure following participation in more than 12 cases, but 20 percent reported not feeling adequately trained following fellowship. Over half (52 percent) thought training could be improved by establishing core areas of exposure and case category minimums. Conclusions Fellowships identified as "craniofacial" are actually fairly heterogeneous, offering diverse clinical experiences. Currently, only a minority emphasize traditional cleft, intracranial, and midfacial procedures, with the majority focused more on cleft care, general pediatric plastic surgery, and trauma. Concomitant with an increase in fellowship-trained surgeons has come a change in program structure from apprenticeships to residency-type models. Prospective fellows should consider matching their individual training goals with each program's unique clinical strengths.

Background Midfacial advancements improve function and normalize appearance in growing children with syndromic craniosynostosis yet carry substantial risks while often offering only transient benefits. The authors sought to evaluate outcomes and the number of advancements required, at skeletal maturity. Methods The authors performed a retrospective review of all children undergoing rigid external distraction Le Fort III advancement who had reached skeletal maturity, including examinations, photographic scoring, and cephalometric, anthropometric and polysomnography data. Results Over 15 years, 104 children underwent distraction and 32 met skeletal maturity criterion (mean age, 18.6 years). Thirty-four percent had prior conventional Le Fort III advancement (mean age, 5.6 years), primarily by other surgeons. Primary distractions were performed on average at 9.1 years. Ten children presented with obstructive sleep apnea; eight normalized, two improved, but 30 percent subsequently developed recurrent apnea. Per anthropometry, no sagittal growth was noted after a Le Fort III. With a mean follow-up of 9.4 years, almost half (15 of 32) underwent two midfacial advancements; however, following primary distractions, only 25 percent required repeated advancements. Associated with repeated procedures were a greater degree of midfacial hypoplasia (-2.4 versus -1.6, p = 0.01), advancement at a younger age (7.1 versus 9.5 years, p = 0.04), and failure to overcorrect midfacial position (p = 0.03). Conclusions These data suggest that sagittal midfacial growth ceases after advancement. Operation before 8 years old and failure to overadvance the midface were both associated with need for subsequent midfacial procedures. Although distraction effectively eliminated apnea, subsequent ventilatory degradation may occur, suggesting the need for continued surveillance.

Numerous publications have examined technique-specific results in treating sagittal synostosis, but there are scant data examining long-term outcomes. This retrospective study was designed to evaluate a large series of surgically repaired children with sagittal craniosynostosis and to examine long-term cranial growth following correction.From 1990 to 2003, 132 children with sagittal synostosis were evaluated and 89 surgically treated (single-stage posterior remodeling) patients were retrospectively reviewed (43 were excluded for multisutural involvement, incomplete records, or nonsurgical treatment). Long-term growth was assessed from anthropologic measurements taken from 3 to 11 years postoperatively (mean, 4.7 years).The surgical age ranged from 2 months to 5 years (mean, 8 months), and the hospital stay was less than 3 days. There were no deaths, major complications, infections, or repeated remodeling procedures performed. Postoperatively, the cephalic index was overcorrected. Subsequent growth of the cranial breadth and length was less than predicted. Moreover, growth was more deficient in cranial breadth than length, resulting in a regression of the cephalic index over time (p0.01). Frontal breadth improved immediately postoperatively but did not subsequently narrow over time. Head circumference also showed a statistically significant diminished capacity for growth (p0.001).This study demonstrated abnormal skull growth in children following the surgical correction of scaphocephaly. On the basis of these findings, surgeons treating sagittal synostosis may wish to expand their goals of treatment beyond normalization to an overcorrection of the abnormal skull shape.