A systematic review of surgical simulation in gender affirmation surgery.

The burgeoning field of gender affirmation surgery (GAS) has become increasingly complex, challenging plastic surgeons to meet high standards for their patients. During the COVID-19 pandemic, the emphasis on remote learning ushered in the increased use of surgical simulation training, offering residents the opportunity to trial challenging procedures before treating patients. This systematic review seeks to summarize current simulation training models used in GAS. A systematic review was conducted according to PRISMA-P guidelines using the following databases: PubMed, Medline, Scopus, Embase, Web of Science, and Cochrane. Inclusion criteria were English-language peer-reviewed articles on surgical simulation techniques or training related to the field of gender surgery. Skills and techniques taught and assessed, model type, equipment, and cost were abstracted from articles. Our search criteria identified 1650 articles, 10 of which met the inclusion criteria for data extraction. Simulation models included those that involved cadavers (n = 2), synthetic benchtop (n = 5), augmented/virtual reality (n = 2), and 3D-printed interfaces (n = 1). The most common procedure involved breast or pectoral reconstruction and/or augmentation (n = 5), followed by vaginal reconstruction (n = 3). One simulation model involved facial GAS. All models focused on surgical technique and anatomy, three on suture skills or knot-tying, and one on surgical decision-making. The evolving field of GAS requires that plastic surgery trainees be knowledgeable on surgical techniques surrounding this scope of practice. Surgical simulation not only teaches residents how to master techniques but also helps address the sensitive nature of GAS.

Narrative review of facial gender surgery: approaches and techniques for the frontal sinus and upper third of the face.

Facial gender confirmation surgery (FGCS) is a series of procedures which seek to harmonize a patient's face with his/her self-image and gender identity. Originally described in San Francisco in the 1980s, FGCS has evolved to encompass all elements of the craniofacial skeleton and facial soft tissue. This field in plastic and reconstructive surgery has quickly gained more attention in the past decade due to the pioneering work of groups around the world along with increased social acceptance and medical care of the transgender community. This narrative review focuses on the upper third of the face. Key differences in the forehead and the hairline of cis men and women are discussed which inform pharmacologic and surgical interventions. Hairline modifying therapies including pharmacotherapy and hair transplantation are explained. Virtual surgical planning (VSP), a tool broadly used in surgical fields, has a special role in FGCS and we offer advice in using VSP when addressing the frontal sinus. Use of VSP allows the surgeon to provide reproducible and accurate results. We then discuss the history of the frontal sinus setback and offer our algorithmic approach to recontouring the forehead with detailed description of the operative steps and decision making. Finally, postoperative care and complications considered.

Long-Term Neurocognitive Outcomes in Sagittal Synostosis: The Impact of Reoperation.

INTRODUCTION: Optimal age at surgery in nonsyndromic sagittal craniosynostosis continues to be debated. Previous reports suggest that earlier age at whole vault cranioplasty more frequently requires reoperation. It is unknown, however, whether reoperation affects neurocognitive outcome. This study examined the impact of reoperation on neurocognitive outcome in children with nonsyndromic sagittal craniosynostosis using comprehensive neurocognitive testing. METHODS: Forty-seven school-age children (age 5-16 years) with nonsyndromic sagittal craniosynostosis who underwent whole-vault cranioplasty were included in this analysis. Participants were administered a battery of standardized neuropsychological testing to measure neurocognitive outcomes. RESULTS: Thirteen of the 47 participants underwent reoperation (27.7%); 11 out of the 13 reoperations were minor revisions while 2 reoperations were cranioplasties. Reoperation rate was not statistically different between patients who had earlier surgery (at age ≤6 months) versus later surgery (at age >6 months) (P > 0.05). Nonreoperated patients who had only one later-in-life surgery did not perform statistically better than reoperated patients on any outcome measure of neurocognitive function, including IQ, academic achievement, visuomotor integration, executive function, and behavior. Comparing reoperated earlier surgery patients with nonreoperated later surgery patients, reoperated earlier surgery patients had higher full-scale and verbal IQ (P < 0.05), scored higher on word reading, reading comprehension, spelling, numerical operations, and visuomotor integration (P < 0.05), and had fewer indicators of suspected learning disabilities (P < 0.01) compared to nonreoperated later surgery patients. CONCLUSION: Reoperation rate after whole vault cranioplasty was 27.7%, with few cases of repeat cranioplasty (4.2% of all patients). Reoperation was not associated with worse neurocognitive outcome. Reoperated earlier surgery patients in fact performed better in IQ, academic achievement and visuomotor integration when compared to nonreoperated later surgery patients.

Preferences of Transgender and Gender-Nonconforming Persons in Gender-Confirming Surgical Care: A Cross-Sectional Study.

INTRODUCTION: Increased awareness for transgender and gender-nonconforming individuals may lead to increased demand for surgical interventions in gender-confirming care. However, limited literature exists regarding transgender and gender-nonconforming preferences and experiences with medical or surgical care. The authors aim to characterize the medical and surgical care sought by this population, as well as their surgical preferences, motivations, and barriers to care. METHODS: An online questionnaire about opinions and personal experiences with medical and surgical care during gender transition was publicized via regional online social networking forums in Connecticut and surrounding areas catering to transgender communities. RESULTS: Responses were received from 313 participants. Participants were 97% male gender at birth and 92% white with an average (SD) age of 51.6 (13.5) years. Fifty-nine percent identified as male-to-female transgender and 20% as gender nonconforming. Respondents were aware of their gender identity at a mean (SD) age of 9.6 (9.0) years, but did not begin transitioning until a mean (SD) age of 38.9 (20.8) years, with gender-nonconforming respondents choosing to transition at a significantly younger age as compared with transgender respondents (29.8 vs 41.4 years; P = 0.0061, unpaired t test). Only 42% of all respondents, with a significantly greater number of transgender as opposed to gender-nonconforming individuals, had previously met with a physician to discuss transitioning (49% vs 21%, P = 0.002, χ test). Eight percent of the study population had undergone gender confirmation surgery (GCS), 52% were interested in GCS, and 40% were not interested in GCS. Primary motivation for GCS included discomfort in one's current body (28%), and barriers to GCS included cost (40%) and reactions of family (40%), partners (32%), and friends (25%). CONCLUSIONS: Transgender and gender-nonconforming individuals lack medical support for gender transition, with fewer than half of survey respondents reporting a prior meeting with a physician to discuss transitioning. The reported perspectives offer important insight into transgender preferences that should act as the basis of future efforts to improve the efficacy of gender-confirming care.

Racial Disparities in Cleft Palate Repair.

BACKGROUND: Various factors can influence outcomes in cleft palate care. This study sought to determine the impact of race on admissions, hospital costs, and short-term complications in cleft palate repair. METHODS: Cleft palate operations were identified in the Kids' Inpatient Database data, from 2000 to 2009. Data were combed for demographics, perioperatives, complications, and hospital characteristics. Bivariate and multivariate analyses were performed between races in total, primary, and revision cohorts. RESULTS: There were 3464 white, 1428 Hispanic, 413 black, 398 Asian/Pacific-Islander, and 470 patients of other races captured. Black patients experienced more emergent admissions (p = 0.005) and increased length of stay (p = 0.029). Hospital charges were highest for black and Hispanic patients and lowest for white patients (p = 0.019). Black patients had more total complications than non-black patients (p = 0.039), including higher rates of postoperative fistula (p = 0.020) and nonspecific complications among revision repairs (p = 0.003). Asian/Pacific Islander in the primary cohort experienced higher rates of accidental puncture (p = 0.031) and fistula (p < 0.001). Other patients had the highest rates of wound disruption (p = 0.013). After controlling for race, diagnosis, Charlson Comorbidity Index score, region, elective/nonelective, payer, and income quartile, length of stay (p < 0.001) and age (p < 0.001) were associated with increases in both total complications and costs. CONCLUSIONS: Race may play a significant role in cleft palate repair, as white patients had fewer complications, shorter length of stay, and lower costs following repair. Delayed age at treatment may predispose patients to adverse sequelae in minority populations, in terms of influencing length of stay and costs. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Normalization of Speech Processing After Whole-Vault Cranioplasty in Sagittal Synostosis.

BACKGROUND: Neurocognitive studies have found impairments in language-related abilities in nonsyndromic craniosynostosis, highlighting clinical importance of early language processing. In this study, neural response to speech sounds in infants with nonsyndromic sagittal craniosynostosis (NSC) is compared, preoperatively and postoperatively, using event-related potentials (ERPs) to objectively characterize development in language processing. METHODS: Electroencephalogram was recorded while 39 infants (12 NSC and 27 controls; ages 73-283 days) listened to the Hindi dental /(Equation is included in full-text article.)a/ and retroflex /da/ phonemes (non-native phonemic discrimination task). The mismatch negativity (MMN) ERP was extracted as the peak amplitude of the largest negative deflection in the difference wave over 80 to 300 milliseconds poststimulus. Differences in MMN were analyzed using repeated measures analysis of variance. RESULTS: The MMN amplitude was attenuated in the infants with NSC preoperatively compared with controls (P = 0.047). A significant region by group interaction (P = 0.045) was observed, and infants with NSC displayed attenuated MMN in the frontal electrodes compared with controls (P = 0.010). Comparing the preoperative and postoperative MMN, a time by group interaction trend (P = 0.070) was observed. Pair-wise comparisons showed a trend for increase in MMN amplitude from preoperatively to postoperatively in the infants with NSC (P = 0.059). At the postoperative time point, infants with NSC showed no significant difference in MMN from controls (P = 0.344). CONCLUSION: Infants with NSC demonstrated atypical neural response to language preoperatively. After undergoing surgery, infants with NSC showed increased MMN amplitude which was not significantly different from controls. These findings support the idea that whole vault cranioplasty may improve neurocognitive outcomes in sagittal craniosynostosis.

Fibrin Tissue Sealant as an Adjunct to Cleft Palate Repair.

PURPOSE: Fibrin glue is a common tissue sealant used to promote hemostasis, adhere tissues, and accelerate healing. Cleft palate repair can be technically challenging, creating dead space between tissue planes, and can be prone to complications such as would dehiscence or bleeding. The purpose of this study is to assess the role of fibrin glue as an adjunct to cleft palate repair. The authors hypothesize a beneficial impact on complication rates, including bleeding, dehiscence, and fistula formation, among others. METHODS: Primary cleft palate repairs using fibrin glue were retrospectively analyzed. Demographic, intraoperative, perioperative, and postoperative data were combed for outcome variables. Complication rates were calculated in percentages and the results were compared to the published literature. Z-test statistics were performed for comparison. RESULTS: A total of 45 patients, 21 females and 24 males, who underwent primary cleft palate repair with fibrin glue between 2011 and 2014, had sufficient data to be reviewed. There were no instances of bleeding, dehiscence, airway obstruction, infection, oronasal fistula, or return to the operating room in any patients. One patient exhibited mild postoperative coughing and secretions that resolved with conservative measures. Another patient displayed postoperative seizure activity due to a pre-existing condition. All complication rates in our fibrin glue series were lower than those reported without the use of fibrin glue. Overall complication rates with fibrin sealant are significantly lower than overall complication rates without. CONCLUSION: Our data suggest that fibrin sealant is a beneficial adjunct to cleft palate repair. Its application is well-tolerated and the complication profile in our cohort was much less than the reported rates. The results of this preliminary study should be vetted with a prospective analysis involving a control group.

Does Cleft Palate Width Correlate With Veau Classification and Outcome?

PURPOSE: Wider cleft palates are thought to be associated with increased complications and poorer outcomes following cleft palate repair. Objective cleft palate photographic measurement and assessment of complications have not been previously performed. The purpose of this study is to quantitatively characterize a series of cleft palate dimensions and to investigate possible correlations with Veau classification and intra-, peri-, and postoperative outcomes. METHODS: The analytic sample included primary cleft palate repairs performed by the senior author over a 2-year period. Standard photographs of clefts taken at the time of repair were analyzed using Image-J software. Demographic, intraoperative, perioperative, and postoperative information were collected. Width measurements were correlated with Veau classification, intraoperative variables, perioperative variables, and adverse outcomes. Statistical tests performed included simple regression analyses and multiple regression analysis. RESULTS: Out of 70 patients, 50 had adequate photographic documentation for inclusion in the study; 44% of patients were classified as Veau I with an average cleft width of 5.4 mm, 28% Veau II with an average of 8.9 mm, 16% Veau III with an average of 11.3 mm, and 12% Veau IV with an average of 10.0 mm. No patients exhibited postoperative bleeding, dehiscence, airway problems, infection, fistula formation, or return to the operating room. The authors found that increasing cleft width significantly predicts increasing Veau classification (P < 0.01), increasing operating time (P < 0.05), increased hypernasality (P < 0.05), and speech delay (P < 0.001). Additionally, the presence of an intentional alveolar fistula (Veau III or Veau IV clefts) significantly predicts fluid emission (P < 0.001) but cleft width did not predict fluid emission. Increased cleft width did not significantly predict length of stay. CONCLUSION: Our data demonstrate that wider preoperative cleft palates correlate with Veau classification, increased operating time, and slightly worsened postoperative sequela. There were no perioperative instances of bleeding, dehiscence, respiratory complications, infection, fistula formation, and return to operating room. Hypernasality and speech delay were associated with increased cleft palate width. Length of stay did not correlate with cleft palate width.

The Severity of Deformity in Metopic Craniosynostosis Is Correlated with the Degree of Neurologic Dysfunction.

BACKGROUND: In recent years, metopic synostosis has represented up to 25 percent of all nonsyndromic craniosynostosis cases, manifesting with varying degrees of trigonocephaly. It is unknown whether the degree of severity of anterior dysmorphology is proportionate to neurologic dysfunction. This knowledge is essential to guide future surgical treatment recommendations. Given the reported correlation of abnormal auditory processing with future language dysfunction and poorer academic performance in craniosynostosis patients, the present study aims to evaluate auditory processing in preoperative patients with moderate and severe metopic synostosis, and in control infants, to discern the neurologic risk carried by different severities of trigonocephaly. METHODS: Thirty-eight infants underwent evaluation of auditory processing: nine patients with severe metopic synostosis, seven with moderate metopic synostosis, and 22 controls. Brain activity was recorded by electroencephalography while the participants listened to language-based stimuli. Electroencephalographic data were analyzed to extract event-related potentials evoked by the speech sounds. RESULTS: Severe metopic synostosis patients demonstrated significantly attenuated event-related potential responses in the left frontal scalp region overlying the left frontal lobe compared with controls (p < 0.05). The moderate metopic synostosis patients did not show significantly different language processing compared to the control infants or the severe metopic synostosis patients in the frontal scalp region. CONCLUSIONS: The results of this study suggest that untreated severe metopic synostosis is associated with reduced language response in the frontal cortex. Less severe (moderate) forms are indeterminate, and mild forms do not show calculable irregularity by the event-related potential method of analysis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Direct brain recordings reveal impaired neural function in infants with single-suture craniosynostosis: a future modality for guiding management?

BACKGROUND: Patients with single-suture craniosynostosis (SSC) are at an elevated risk for long-term learning disabilities. Such adverse outcomes indicate that the early development of neural processing in SSC may be abnormal. At present, however, the precise functional derangements of the developing brain remain largely unknown. Event-related potentials (ERPs) are a form of noninvasive neuroimaging that provide direct measurements of cortical activity and have shown value in predicting long-term cognitive functioning. The current study used ERPs to examine auditory processing in infants with SSC to help clarify the developmental onset of delays in this population. METHODS: Fifteen infants with untreated SSC and 23 typically developing controls were evaluated. ERPs were recorded during the presentation of speech sounds. Analyses focused on the P150 and N450 components of auditory processing. RESULTS: Infants with SSC demonstrated attenuated P150 amplitudes relative to typically developing controls. No differences in the N450 component were identified between untreated SSC and controls. CONCLUSIONS: Infants with untreated SSC demonstrate abnormal speech sound processing. Atypicalities are detectable as early as 6 months of age and may represent precursors to long-term language delay. Electrophysiological assessments provide a precise examination of neural processing in SSC and hold potential as a future modality to examine the effects of surgical treatment on brain development.

The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis.

BACKGROUND: The relationship between surgical age and long-term neuropsychological outcomes in sagittal-suture craniosynostosis remains equivocal. Whole-vault cranioplasty and strip craniectomy are performed at various times in individuals with sagittal-suture craniosynostosis. This study used comprehensive neurological testing to examine the relationship between age at time of surgery and long-term neuropsychological function. METHODS: Seventy sagittal-suture craniosynostosis patients who had previously undergone either whole-vault cranioplasty or strip craniectomy were examined divided into three groups: treatment before 6 months (n = 41), between 6 and 12 months (n = 21), and after 12 months (n = 8). To examine long-term cognitive functioning, participants between the ages of 5 and 25 years underwent neurodevelopmental tests to evaluate intelligence, achievement, and learning disabilities. RESULTS: Compared with those treated between 6 and 12 months and after 12 months, patients who underwent surgery before 6 months demonstrated higher full-scale IQ (p < 0.01) and verbal IQ (p < 0.01). Patients who received surgery before 6 months also demonstrated superior abilities in word reading (p < 0.01), reading comprehension (p < 0.01), spelling (p < 0.01), and numerical operations (p < 0.05) relative to those who had surgery between 6 and 12 months old. A statistically significant higher percentage of patients treated after 6 months had one or more reading-related learning disabilities as compared with those undergoing earlier surgery. CONCLUSIONS: This study suggests that surgery before 6 months old results in improved long-term neurological outcomes. Future studies should examine how the technique of surgery impacts these neuropsychological measures. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Brain electrophysiology reveals intact processing of speech sounds in deformational plagiocephaly.

BACKGROUND: The prevalence of deformational plagiocephaly has risen dramatically in recent years, now affecting 15 percent or more of infants. Prior research using developmental scales suggests that these children may be at elevated risk for developmental delays. However, the low positive predictive value of such instruments in identifying long-term impairment, coupled with their poor reliability in infants, warrants the development of methods to more precisely measure brain function in craniofacial patients. Event-related potentials offer a direct measure of cortical activity that is highly applicable to young populations and has been implemented in other disorders to predict long-term cognitive functioning. The current study used event-related potentials to contrast neural correlates of auditory perception in infants with deformational plagiocephaly and typically developing children. METHODS: Event-related potentials were recorded while 16 infants with deformational plagiocephaly and 18 nonaffected controls passively listened to speech sounds. Given prior research suggesting their association with subsequent functioning, analyses focused on the P150 and N450 event-related potential components. RESULTS: Deformational plagiocephaly patients and normal controls showed comparable cortical responses to speech sounds at both auditory event-related potential components. CONCLUSIONS: Children with deformational plagiocephaly demonstrate neural responses to language that are consistent with normative expectations and comparable to those of typical children. These results indicate that head shape deformity secondary to supine sleep is not associated with impairments in auditory processing. The applicability of the current methods in early infancy suggests that electrophysiologic brain recordings represent a promising method of monitoring brain development in children with cranial disorders. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

The effects of whole-vault cranioplasty versus strip craniectomy on long-term neuropsychological outcomes in sagittal craniosynostosis.

BACKGROUND: The optimal type of surgical management for isolated sagittal synostosis remains a source of significant debate. There is a paucity of data regarding possible differences in long-term neuropsychological outcomes following treatment with whole-vault cranioplasty or endoscopic strip craniectomy. This study provides the first comparative analysis examining the effects of the two techniques related to long-term intellectual functioning. METHODS: A total of 70 patients were enrolled in this multicenter study, 29 of whom had previously undergone endoscopic strip craniectomy and 41 of whom had previously undergone whole-vault cranioplasty. All patients completed a battery of neurodevelopmental tests (Beery-Buktenica Developmental Test of Visual-Motor Integration, Wechsler Abbreviated Scale of Intelligence, and Wechsler Fundamentals) to evaluate various domains of neuropsychological function. RESULTS: In a group comparison of those treated before 6 months of age, whole-vault patients obtained higher scores relative to endoscopic strip craniectomy patients on visuomotor integration, full-scale intelligence quotient, verbal intelligence quotient, word reading, and reading comprehension (p < 0.05 for all). When compared against strip craniectomy performed before 3 months of age, the whole-vault group still showed significantly higher scores in verbal intelligence quotient, reading comprehension, and word reading (p < 0.05 for all). CONCLUSIONS: The type of surgical intervention for isolated sagittal synostosis impacts long-term neuropsychological outcomes. Patients undergoing early whole-vault cranioplasty attained higher intelligence quotient and achievement scores relative to those undergoing strip craniectomy. Surgical management with whole-vault cranioplasty performed before 6 months of age provides the most favorable long-term intellectual outcomes in patients with isolated sagittal synostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

A range of malar and masseteric hypoplasia exists in Treacher Collins syndrome.

BACKGROUND: Treacher Collins syndrome (TCS) is a facial dysostosis, the hallmark being bilateral malar hypoplasia. The purpose of this study is to morphologically classify the TCS malar deformity and to volumetrically characterise both the TCS zygoma and masseter muscle, including for left-right symmetry, compared to controls. We hypothesise that the TCS zygoma will be smaller than controls and zygomatic deficiency will portend masseteric hypoplasia. METHODS: Demographic and computed tomography (CT) data were recorded. The CT scans were converted into three-dimensional facial renderings, and the zygomatic morphology was grossly evaluated. A classification was reported based on malar structure and presence/absence of normal zygomaticomaxillary complex articulations. The zygoma and masseter muscles were then digitally isolated using 3-D planning software (Materialise, Leuven, Belgium). Volumes and sidedness ratios were calculated and compared using two-sided t-tests. RESULTS: 58 sides were identified (24 TCS: 34 controls), mean age of 60.0 months and normally distributed. The phenotypic dysmorphology was graded as mild, moderate or severe (I, II and III, respectively). TCS malar and masseteric volumes were significantly smaller than controls (p < 0.0001 in both cases). The TCS zygomatic side-side symmetry ratio was 0.66 ± 0.28, compared to 0.97 ± 0.02 for controls (p = 0.002). The TCS masseteric side-side ratio was 0.74 ± 0.20, compared to 0.92 ± 0.09 for controls (p = 0.001). CONCLUSIONS: A range of zygomatic hypoplasia exists in TCS (mild-severe). The decrease in malar volume occurs in concert with masseteric hypoplasia, and the left and right sides are not equally affected.

A range of condylar hypoplasia exists in Treacher Collins syndrome.

PURPOSE: Temporomandibular joint malformation is a complex deformity in Treacher-Collins syndrome (TCS); however, it is not well characterized. This study aimed to better clarify this pathology by volumetrically assessing the mandibular condyle in patients with TCS compared with normal controls and the relative contribution of the condyle to hemimandibular volume. MATERIALS AND METHODS: A retrospective, cross-sectional analysis of pediatric patients with TCS and unaffected controls was performed. The study sample was comprised of Treacher Collins patients. The predictor variable in this study was disease status (TCS diagnosis vs control), and the outcome variable was condylar volume. Demographic information was collected, and 3-dimensional computed tomographic data were analyzed by computerized segmentation (Materialise). Volumes were obtained for TCS condyles and compared with age-matched controls using the Student t test. RESULTS: Three-dimensional computed tomographic scans were identified in 10 patients with TCS (20 sides) and 14 control subjects (28 sides). The TCS group included 4 female and 6 male patients (age, 0.3 to 213 mo; average age, 66.5 mo). The control cohort included 7 female and 7 male subjects (average age, 68.8 mo). Evaluation of the mandibular condyle showed that patients with TCS had a significantly smaller condylar volume than control patients (TCS, 178.28 ± 182.74 mm(3); control, 863.55 ± 367.20 mm(3); P < .001). Additional intragroup analysis showed no significant differences between the left and right condylar volumes in the TCS group (P = .267). In addition, the condyle for patients with TCS represented a smaller proportion of hemimandibular volume compared with controls (1.37% vs 4.19%, respectively; P < .001). CONCLUSIONS: The results of the this study suggest that condylar volumes are significantly smaller in patients with TCS compared with age-matched controls, and the condyle represents a smaller fraction of the total mandibular volume for patients with TCS than in unaffected children. In addition, there is considerable variability of condylar size in patients with TCS. These facts portend treatment decisions because a functional temporomandibular joint is necessary and may need to be reconstructed as a first stage before effective implementation of distraction procedures.

On Bernard Sarnat's 100th birthday: pathology and management of craniosynostosis.

The focus on nonsyndromic craniosynostosis, the most common type of isolated craniosynostosis, is sagittal, followed by unilateral coronal, bilateral coronal, metopic, and lambdoid, in order of decreasing frequency. Certain forms of craniosynostosis display a sex predilection. For example, boys outnumber girls in a 4:1 ratio in sagittal synostosis, but girls outnumber boys in a 3:2 ratio in unilateral coronal synostosis. Other forms, such as metopic, lambdoid, and bilateral coronal synostosis, demonstrate no sex dominances tract.

Combined metopic and sagittal craniosynostosis: is it worse than sagittal synostosis alone?

OBJECT Combined metopic and sagittal craniosynostosis is a common variant of the nonsyndromic, multiplesuture synostoses. It is unknown whether this combined form causes reduced intracranial volume (ICV) and potentially more brain dysfunction than sagittal synostosis alone. This study is a volumetric comparison of these 2 forms of craniosynostosis. METHODS The authors conducted a retrospective chart and CT review of 36 cases of isolated sagittal synostosis or combined metopic and sagittal synostosis, involving patients seen between 1998 and 2006. Values were obtained for the intracranial compartment, brain tissue, CSF space, and ventricular volumes. Patients with craniosynostosis were then compared on these measures to 39 age- and sex-matched controls. RESULTS In patients with isolated sagittal synostosis and in those with combined metopic and sagittal synostosis, there was a trend toward smaller ICV than in controls (p < 0.1). In female patients older than 4.5 months of age, there was also a trend toward smaller ICV in patients with the combined form than in those with sagittal synostosis alone (p < 0.1), and the ICV of patients with the combined form was significantly smaller than the volume in controls in the same age group (p < 0.05). Brain tissue volume was significantly smaller in both patient groups than in controls (p < 0.05). Ventricular volume was significantly increased (compared with controls) only in the patients with isolated sagittal synostosis who were younger than 4.5 months of age (p < 0.05). Overall CSF space, however, was significantly larger in both patient groups in patients younger than 4.5 months of age (p < 0.05). CONCLUSIONS These findings raise concerns about intracranial and brain volume reduction in patients with sagittal and combined metopic and sagittal synostoses and the possibility that this volume reduction may be associated with brain dysfunction. Because the ICV reduction is greater in combined metopic and sagittal synostosis in patients older than 4.5 months of age than in sagittal synostosis in this age group, the potential for brain dysfunction may be particularly true for these younger infants.