What to do with an incidental finding of a fused sagittal suture: a modified Delphi study.

OBJECTIVE: As many as 5% of normocephalic children may have a prematurely fused sagittal suture, yet the clinical significance and best course of management of this finding remain unclear. Providers in the Synostosis Research Group were surveyed to create a multicenter consensus on an optimal treatment and monitoring algorithm for this condition. METHODS: A four-round modified Delphi method was utilized. The first two rounds consisted of anonymous surveys distributed to 10 neurosurgeons and 9 plastic surgeons with expertise in craniosynostosis across 9 institutions, and presented 3 patients (aged 3 years, 2 years, and 2 months) with incidentally discovered fused sagittal sutures, normal cephalic indices, and no parietal dysmorphology. Surgeons were queried about their preferred term for this entity and how best to manage these patients. Results were synthesized to create a treatment algorithm. The third and fourth feedback rounds consisted of open discussion of the algorithm until no further concerns arose. RESULTS: Most surgeons preferred the term "premature fusion of the sagittal suture" (93%). At the conclusion of the final round, all surgeons agreed to not operate on the 3- and 2-year-old patients unless symptoms of intracranial hypertension or papilledema were present. In contrast, 50% preferred to operate on the 2-month-old. However, all agreed to utilize shared decision-making, taking into account any concerns about future head shape and neurodevelopment. Panelists agreed that patients over 18 months of age without signs or symptoms suggesting elevated intracranial pressure (ICP) should not undergo surgical treatment. CONCLUSIONS: Through the Delphi method, a consensus regarding management of premature fusion of the sagittal suture was obtained from a panel of North American craniofacial surgeons. Without signs or symptoms of ICP elevation, surgery is not recommended in patients over 18 months of age. However, for children younger than 18 months, surgery should be discussed with caregivers using a shared decision-making process.

Eyeing Risks: A Critical Analysis of the Use of Periorbital Steroids in Fronto-orbital Advancement.

OBJECTIVE: To evaluate the outcomes associated with two techniques of periorbital steroid administration in bilateral fronto-orbital advancement (FOA). DESIGN: Multi-institutional retrospective chart review. SETTING: Two high volume, tertiary US craniofacial centers. PATIENTS, PARTICIPANTS: Patients who underwent FOA between 2012 and 2021. INTERVENTIONS: Patients were divided into three cohorts based on method of steroid administration. Groups GEL and INJ represent those who received steroids in the form of triamcinolone soaked gelfoam or direct injection of dilute triamcinolone to the frontal/periorbital region, respectively. Group NON did not receive any periorbital steroids. MAIN OUTCOME MEASURE(S): Peri-operative outcomes including hospital length of stay and complications were evaluated based on method of periorbital steroid administration. Variables predictive of infectious complications were assessed using stepwise logistic regression. RESULTS: Four hundred and twelve patients were included in our sample (INJ:249, GEL:87, NON:76). Patients in the INJ group had a higher ASA class (P < .001) while patients in the NON group were significantly more likely to be syndromic (P < .001) and have multisuture craniosynostosis (P < .001). Rate of infectious complications for each cohort were NON: 2.6%, INJ: 4.4%, and GEL: 10.3%. There was no significant difference between groups in hospital length of stay (P = .654) or rate of post-operative infectious complications (P = .061). Increased ASA class (P = .021), increased length of stay (P = .016), and increased intraoperative narcotics (P = .011) were independent predictors of infectious complications. CONCLUSIONS: We identified a dose-dependent relationship between periorbital steroids and rate of postoperative infections, with key contributions from ASA class, hospital length of stay, and dose of intraoperative narcotics.

Pediatric Panfacial Fractures.

Pediatric panfacial trauma is a rare occurrence with poorly understood implications for the growing child. Treatment algorithms largely mirror adult panfacial protocols with notable exceptions including augmented healing and remodeling capacities that favor nonoperative management, limited exposure to avoid disruption of osseous suture and synchondroses growth centers, and creative fracture fixation techniques in the setting of an immature craniomaxillofacial skeleton. The following article provides a review of our institutional philosophy in the management of these challenges injuries with important anatomic, epidemiologic, examination, sequencing, and postoperative considerations.

Two Year Comparison of Sagittal Synostosis Morphometric Outcomes Following Open Posterior Expansion Versus Endoscopic Strip Craniectomy with Helmet Molding Performed before Four Months of Age.

BACKGROUND: Open middle and posterior cranial vault expansion (OPVE) or endoscopic (ES) strip craniectomy are two surgical techniques for normalization of head shape in isolated sagittal synostosis. This study aims to compare two-year cranial morphometrics after these two approaches. METHODS: We performed morphometric analysis on preoperative (t0), immediately post-operative (t1) and 2-year (t2) postoperative CT scans of patients who underwent OPVE or ES prior to 4 months of age. Perioperative data and morphometrics were compared between the two groups and age-matched controls. RESULTS: Nineteen patients were included in the ES cohort, 19 age-matched patients in the OPVE cohort, and 57 as controls. Median surgery time and blood transfusion volume were less for the ES approach (118 min; 0cc) compared to OPVE (204 min; 250cc). Anthropometric measurements after OPVE were closer normal controls at t1 compared to ES, but the skull shapes were comparable at t2. In the mid-sagittal plane, anterior vault was higher after OPVE at t2 compared to both ES and controls, but the posterior length was shorter and closer to controls than the ES cohort. Cranial volumes were like controls for both cohorts at t2. There was no difference in complication rate. CONCLUSIONS: Both OPVE and ES techniques result in normalization of cranial shape in patients with isolated sagittal synostosis after two years with minimal morphometric differences. Family decision-making between the two approaches should be based on age at presentation, avoidance of blood transfusion, scar pattern, and availability of helmet molding and not on expected outcome. LEVEL OF EVIDENCE: III.

Morphometric Outcomes of Nonsyndromic Sagittal Synostosis following Open Middle and Posterior Cranial Vault Expansion.

BACKGROUND: This study aimed to quantify the change in three-dimensional skull morphometrics for patients with sagittal synostosis at presentation, after surgery, and at 2-year follow-up. METHODS: Computed tomography scans from 91 patients with isolated SS were age-, sex-, and race-matched with those from 273 controls. The authors performed vector analysis with linear regressions to model the effect of open middle and posterior cranial vault remodeling on cranial shape and growth. RESULTS: Anterior cranial volume, bossing angle, and frontal shape were not changed by surgery but normalized without surgical intervention by 2 years. Biparietal narrowing and middle cranial volume were corrected after surgery and maintained at 2 years. Occipital protuberance was improved after surgery and normalized at 2 years. Posterior cranial volume was decreased by occipital remodeling and remained slightly lower than control volumes at 2 years, whereas middle vault volume was larger than in controls. Residual deformities that persisted at 2 years were decreased superolateral width at the level of opisthion and increased anterosuperior height (vertex bulge). Linear models suggested older age at surgery resulted in more scaphocephaly and enlarged posterior cranial vault volumes at 2 years but did not affect other volume outcomes. Preoperative severity was the variable most predictive of 2-year morphometrics. CONCLUSIONS: Initial severity of sagittal synostosis deformity was the best predictor of 2-year morphometric outcomes. Upper posterior cranial width decreases with time after surgery and an anterior vertex bulge can persist after open surgery, but frontal dysmorphology self-corrects without surgical intervention. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Cranial Shape Changes in Nonsyndromic Unilateral Lambdoid Synostosis after Open Posterior Vault Remodeling.

BACKGROUND: The purpose of this study was to quantify change in cranial morphology in patients with nonsyndromic unilateral lambdoid craniosynostosis (ULC) from presentation (t0), after open posterior switch-cranioplasty (t1), and at 2-year follow-up (t2). METHODS: Volumetric, linear, and angular analysis were performed on computed tomographic scans at the three time points and against normal control subjects. Significance was set at P < 0.05. RESULTS: Twenty-two patients were included. ULC cranial vault asymmetry index was higher than in control subjects before surgery (6.22 ± 3.55) but decreased after surgery (3.00 ± 2.53) to become comparable with the normal asymmetry range present in the controls. After surgery, both diagonals increased, but more on the fused side. In the 2 years after surgery, both diagonals in patients with ULC grew proportionately, but the fused diagonal remained slightly shorter than the patent side. Total cranial volume was higher in patients with ULC than in control subjects after surgery but became comparable at t2. Cranial base angulation improved by t2 but did not approach normal, and ear position remained unchanged. The facial twist was higher than in controls at t0 and t1 but was comparable at t2. Coronal asymmetry improved with surgery but remained undercorrected at t2, with the greatest residual asymmetry at opisthion. CONCLUSIONS: Open-switch cranioplasty normalizes cranial vault asymmetry index by increasing the fused cranial diagonal more than the patent side and is stable at 2 years. Skull base twist does not normalize, but facial twist approaches normal. Technique improvement should focus on residual coronal asymmetry present at opisthion. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Syndromic Craniosynostosis: Cranial Vault Expansion in Infancy.

Syndromic craniosynostosis (CS) represents a relatively uncommon disease process that poses significant reconstructive challenges for the craniofacial surgeon. Although there is considerable overlap in clinical features associated with various forms of syndromic CS, key extracranial features and close examination of the extremities help to distinguish the subtypes. While Virchow's law can easily guide the diagnosis of single suture, nonsyndromic CS, syndromic CS traditionally results in atypical presentations inherent to multiple suture fusion. Coronal ring involvement in isolation or associated with additional suture fusion is the most common pattern in syndromic CS often resulting in turribrachycephaly.

Multidisciplinary Care Considerations for Patients with Craniosynostosis.

Infants and children with craniosynostosis require multidisciplinary care, and this is best accomplished when care is provided on a craniofacial team. Most patients with craniosynostosis will have non-syndromic presentations; however, longitudinal care remains critical to ensure appropriate growth and development throughout childhood. In patients with syndromic craniosynostoses, coordinated longitudinal care becomes even more paramount because of the high level of complexity across many different specialties or disciplines. Care delivery that includes perspective and expertise from multiple disciplines is important to help patients reach their full potential and optimal outcomes.

Management of Sagittal and Lambdoid Craniosynostosis: Minimally Invasive Approaches.

The resurgence of strip craniectomies began in the mid-1990s with advances in surgical technique and anesthesia coupled with the critical observation that earlier interventions benefitted from an easily molded skull. Jimenez and Barone's pioneering introduction of endoscopic approaches to strip craniectomies coupled with postoperative helmeting in newborns and young infants and Claes Lauritzen's introduction of spring-mediated cranioplasty began the era of minimally invasive approaches in the surgical correction of craniosynostosis. This article provides technical descriptions of these treatment modalities, a comparative literature review, and our institutional algorithms for the correction of sagittal craniosynostosis and unilambdoid craniosynostosis.

Morphologic Differences in Sagittal Synostosis with Age before Surgery.

BACKGROUND: It is important to determine whether sagittal synostosis-associated scaphocephaly is static in the presurgical period, or whether there are morphologic differences with time to include in surgical decision-making. The authors' purpose was to perform cross-sectional analysis of cranial morphology before any surgical intervention in children with sagittal synostosis younger than 9 months compared to matched controls. METHODS: The authors performed morphometric analysis on computed tomographic scans from 111 untreated isolated sagittal synostosis patients younger than 9 months and 37 age-matched normal controls. The authors divided the patients into three age groups and performed statistical comparison between sagittal synostosis and controls for each group. RESULTS: Sagittal synostosis cephalic indices were stable and lower in patients than in controls across groups. Total cranial volume was equivalent, but sagittal synostosis patients had a greater posterior volume than controls at all ages and a smaller middle fossa volume at older ages. Pterional width was greater in sagittal synostosis patients than in controls for each age group. Frontal bossing vectors were most severe in the youngest age groups and least in the older group. Occipital protuberance was consistent across the age groups. CONCLUSIONS: Upper parietal narrowing and occipital protuberance were the consistent deformities across age groups, with the most parietal constriction seen in older patients. Frontal bossing was not consistent and was more severe in the younger patients. The authors did not detect significant pterional constriction, and the appearance of constriction is relative to adjacent morphology and not absolute. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Evaluating the Utility of Routine Computed Tomography Scans after Cranial Vault Reconstruction for Children with Craniosynostosis.

BACKGROUND: Postoperative computed tomography scans allow for evaluation of the structural results of cranial vault reconstruction and potential surgical concerns. The authors evaluated the clinical utility of routine postoperative scans to identify relevant surgical findings in children treated for craniosynostosis. METHODS: The authors conducted a retrospective study of postoperative computed tomography reports for patients with craniosynostosis following cranial vault reconstruction during a 9-year period at their tertiary care pediatric hospital. They categorized postoperative computed tomography findings as typical, atypical, or indeterminate. Images with reported indeterminate or atypical findings were reviewed and verified by a pediatric neuroradiologist and a pediatric neurological surgeon. Clinical outcomes of patients with abnormal postoperative images were assessed with chart review for clinical relevance. RESULTS: Postoperative computed tomography radiology reports for 548 operations in 506 participants were included. Most participants had single-suture craniosynostosis (89 percent), were male (64 percent), and under 1 year of age (78 percent). Surgically concerning scans were described in 52 reports (<9.5 percent), and the research team's pediatric neuroradiologist confirmed abnormal findings in 36 (6.5 percent). Potentially relevant abnormal findings included subdural blood (n = 18), subarachnoid blood (n = 4), intraparenchymal findings (n = 6), bone abnormalities (n = 5), vascular injury (n = 3), and increased ventricular size (n = 2). Most cases with abnormal findings did not require additional observation nor intervention. Only three cases (of 548; 0.55 percent) required further intervention, which included additional medical management (n = 2) and return to the operating room (n = 1). CONCLUSION: Abnormal findings on routine computed tomography scans after cranial vault reconstruction are uncommon and rarely result in an urgent surgical or medical intervention. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, IV.

Evolution of Cranioorbital Shape in Nonsyndromic, Muenke, and Saethre-Chotzen Bilateral Coronal Synostosis: A Case-Control Study of 2-Year Outcomes.

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 < 0.05. 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.

A Comparison of Subgaleal Versus Subperiosteal Dissection in Open Cranial Vault Expansion for Sagittal Craniosynostosis.

BACKGROUND: The aim of this study was to evaluate surgical outcomes for patients with sagittal craniosynostosis undergoing open cranial vault remodeling with a modified pi procedure comparing subgaleal versus subperiosteal dissection. METHODS: A retrospective chart review was performed for children between the ages of 3 and 7 months with sagittal craniosynostosis undergoing open cranial vault expansion at Seattle Children's Hospital. Patient demographics, operative variables, and postoperative outcomes including the surface area of bony cranial defects at 2-year follow-up were evaluated. RESULTS: Over a 3-year period, 35 patients between the ages of 3 and 7 months underwent surgical correction of sagittal craniosynostosis using our institutional adaptation of the modified pi technique. Twenty-five patients underwent exposure via a subgaleal (SG) approach, 10 patients had a subpericranial (SP) exposure. Compared with the SP group, the SG group had significant lower estimated blood loss and a shorter operating time (P < 0.05). There were no significant differences with regard to hospital length of stay or postoperative complications (P ≥ 0.48). At 2 years postoperatively, there were no significant differences in the size of the largest cranial defects (SG: 1.1 ± 0.1 cm2 versus 3.7 ± 0.1 cm2, P = 0.40); no patients required a secondary cranioplasty. CONCLUSIONS: Open posterior and middle cranial vault expansion is a safe and efficient method of open cranial vault expansion in sagittal craniosynostosis regardless of the plane of dissection. Elevation of the scalp flaps in the SG plane is a minor technical modification that can reduce blood loss and operative times.

Pioneers in Modern Craniofacial Surgery: Assessing the Academic Impact of Drs. Joseph Gruss and Paul Manson.

BACKGROUND: The authors conducted this study to assess the impact that Drs. Joseph Gruss and Paul Manson have had on craniofacial surgery through their individual contributions and through their trainees. METHODS: This was a retrospective analysis of fellows trained by either Dr. Gruss or Dr. Manson. Demographic and bibliometric measures were recorded for each fellow. Demographic factors included years since completion of fellowship training, current practice of craniomaxillofacial surgery, academic practice, and academic leadership roles. Bibliometric measures included number of publications, number of citations, and h-index. To adjust for scholarly activity before fellowship training, only contributions published after fellowship training were included. RESULTS: Over a 39-year period, a total of 86 surgeons completed fellowship training with either of the two principal surgeons. The mean time since completion of training was 18.7 ± 11.4 years. Seventy-nine percent of surgeons had active practices in craniomaxillofacial surgery; 54 percent had academic practices. The mean number of publications was 26.4 ± 69.3, the mean number of citations was 582 ± 2406, and the average h-index was 6.7 ± 10.6. Among academic surgeons, the average h-index was 10.7 ± 13.1, 89 percent practiced in North America, 89 percent had active practices in craniomaxillofacial surgery, and nearly 50 percent had achieved a leadership role. CONCLUSIONS: Modern craniofacial reconstruction has evolved from principles used in trauma and correction of congenital differences. The extensive impact that Drs. Paul Manson and Joseph Gruss have had on the field, and plastic surgery at large, is evident through their primary contributions and the immense impact their trainees have had on the field.

Distinguishing Between Lambdoid Craniosynostosis and Deformational Plagiocephaly: A Review of This Paradigm Shift in Clinical Decision-Making and Lesson for the Future.

The cause of occipital asymmtery can be either extrinsic or intrinsic. Intrinsic causes include lambdoid craniosynsotosis. This condition is generally treated with cranial vault expansion surgery. Extrinsic causes include deformational plagiocephaly, which became commonplace after the "Back to Sleep Campaign" instituted in the 1980s by the American Academy of Pediatrics. The treatment of this condition is non surgical. Dr. Joseph Gruss was instumental in differentiating between these conditions and reducing the number of unnecessary surgeries that were previously being performed.

Unilateral Cleft Lip Nasal Deformity: Foundation-Based Approach to Primary Rhinoplasty.

BACKGROUND: Cleft lip results in disruption of the nasal foundation and collapse of tip structures. Most approaches to primary rhinoplasty focus on correction of lower lateral cartilages; however, recurrent deformity is common, and secondary revision is frequently required. The authors describe an alternate approach that focuses on the foundation to "upright the nose," without any nasal tip dissection. This study assessed changes with surgery and with growth. Secondary goals were to compare methods of sidewall reconstruction and septoplasty and to identify predictors of relapse. METHODS: Consecutive patients undergoing repair (n = 102) were assessed. Images were captured preoperatively, postoperatively, and at 5 years of age (when available) using three-dimensional stereophotogrammetry. Standard anthropometric and contemporary shape-based analysis (volume ratio, dorsal deviation, and alar-cheek definition) was performed to assess longitudinal changes. Images of age-matched normal control subjects were used for comparison. RESULTS: Significant changes in anthropometric and morphometric measurements occurred following surgery. Postoperative form was similar to controls immediately after surgery and at 5 years. Nasal corrections were satisfactory, and only two patients have elected to undergo revision. When subjects were grouped according to cleft type, we found the same trends. When comparing different methods of nasal sidewall reconstruction or septoplasty, we found no differences. Alveolar cleft width was a significant predictor of worse preoperative and postoperative form. CONCLUSIONS: Significant nasal correction can be achieved by means of reconstruction of nasal foundation, without nasal tip dissection. Preservation of tissue planes may allow for easier secondary revision, if necessary. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Practical Computed Tomography Scan Findings for Distinguishing Metopic Craniosynostosis from Metopic Ridging.

BACKGROUND: Premature fusion of the metopic suture (ie, metopic craniosynostosis) can be difficult to discriminate from physiological closure of the metopic suture with ridging (MR). Yet, MCS is treated surgically, whereas MR is treated nonsurgically. Often, the diagnosis can be made by physical examination alone, but in difficult cases, a computed tomography (CT) scan can add additional diagnostic information. METHODS: We de-identified, randomized, and analyzed the CT scans of patients with MCS (n = 52), MR (n = 20) and age-matched normative controls (n = 52) to identify specific findings helpful in distinguishing between MCS and MR. Four expert clinicians were blinded to the clinical diagnosis and assessed each CT for features of the orbits, frontal bones, and inner table of calvaria. RESULTS: Although no single feature was diagnostic of MCS, we identified several signs that were correlated with MCS, MR, or controls. Features such as "posteriorly displaced frontal bone" and "frontal bone tangent to mid-orbit or medial" demonstrated higher correlation with MCS than MR and the addition of other features improves the accuracy of diagnosis as did inclusion of the interfrontal divergence angle. CONCLUSION: The presence of a closed metopic suture in addition to other CT scan findings may improve the accuracy of diagnosing MCS, MR, and normocephaly.