Multicenter comparison of head shape outcomes for three minimally invasive strip craniectomy techniques for sagittal craniosynostosis.

OBJECTIVE: A literature gap exists comparing whole head shape outcome following correction of sagittal craniosynostosis. The objective of this multicenter study was to provide an analysis of long-term results following three different endoscopic strip craniectomy techniques for correction of sagittal craniosynostosis: 1) spring-assisted strip craniectomy, 2) wide-strip craniectomy with biparietal and bitemporal barrel-stave wedge osteotomies plus helmet orthosis, and 3) narrow-strip craniectomy plus orthosis without barrel staves. METHODS: Pre- and postoperative 3D stereophotogrammetric images were collected from patients who underwent craniosynostosis surgery. Procedures were divided among institutions as follows: spring-assisted strip craniectomies were performed at Atrium Health Wake Forest Baptist Hospital; narrow-strip craniectomies were performed at St. Louis Children's Hospital by one craniofacial surgeon; and wide-vertex craniectomies were performed at St. Louis Children's Hospital prior to 2010, and then continued at Children's Medical Center Dallas. Pre- and postoperative 3D whole-head composite images were generated for each procedure to visually represent outcomes at final follow-up and compared with age-matched normal controls. RESULTS: Patients in the spring-assisted strip craniectomy group showed normalization of frontal bossing and skull height compared with age-matched controls, whereas patients undergoing wide-strip craniectomy showed greater correction of occipital protrusion. Patients in the narrow-strip craniectomy cohort had intermediate results between these outcomes. Nested aggregate head shapes showed good correction of head shapes from all techniques. CONCLUSIONS: This large, retrospective, multicenter study illustrated whole head shape outcomes from three different craniectomy procedures. Although each procedure showed some differences in loci of primary correction, all three surgical methods demonstrated good correction of primary scaphocephalic deformity.

Three-dimensional Analysis of Facial Asymmetry in Unilateral Lambdoid Craniosynostosis.

OBJECTIVE: Unilateral lambdoid synostosis (ULS) is characterized by occipital flattening, mastoid bulging, and contralateral parietal bossing. Anterior craniofacial features are less well-defined. This study utilizes volumetric, craniometric, and composite heat maps of three-dimensional (3D) rendered CT scans to analyze anterior craniofacial asymmetry in ULS and compared to controls. DESIGN: A retrospective review of three-dimensional CT scans. SETTING: Tertiary care pediatric institution. PATIENTS, PARTICIPANTS: 30 ULS and 30 control patients. MAIN OUTCOME MEASURE(S): Volumetric and craniometric analysis of the anterior fossa, orbits, zygomas, maxilla, and mandible was performed. RESULTS: The anterior fossa volume was greater bilaterally (0.047, 0.038), and the fossa angle was more anterior contralaterally (<0.001) and more anterior bilaterally than controls (0.038, 0.033). The orbits had greater height and lesser depth bilaterally compared to controls (0.006, 0.009; < 0.001, < 0.001). Zygoma length was significantly greater on the contralateral side than controls (0.048; < 0.001). Nasal contralateral deviation of 3.57 ± 1.97°. The maxillary length was longer on the contralateral side (0.045). The mandibular angle was more anterior on the ipsilateral side and posterior on the contralateral side (<0.001) compared to controls (0.042, < 0.001). Chin had a contralateral deviation of 1.04 ± 3.74°. CONCLUSIONS: ULS has significant asymmetry in the anterior craniofacial skeleton. There is a bilateral expansion of the anterior cranial fossa with greater frontal bossing on the contralateral side. Increased orbital height and decreased depth. Contralateral zygomatic and mandibular body lengthening with posterior mandibular deviation. These features may provide more effective diagnosis and potential clinical management strategies.

Alar Asymmetry in Patients with Unilateral Cleft Lip: Implications for Secondary Rhinoplasty.

OBJECTIVE: Alar asymmetry in unilateral cleft lip (UCL) nasal deformity is a well-recognized clinical feature. However, there is a lack of comprehensive quantitative analysis of this asymmetry. This study compares the shape, volume, and axis rotation between the cleft and non-cleft ala in skeletally mature patients with UCL. DESIGN: A retrospective comparative study utilizing three-dimensional rendered CT scans. SETTING: Tertiary care pediatric institution. PATIENTS, PARTICIPANTS: This study included 18 patients with UCL nasal deformity at skeletal maturity. MAIN OUTCOME MEASURE(S): Cleft and non-cleft side ala volume, surface area, and axis to the midsagittal plane. RESULTS: The cleft-side ala was significantly lesser in volume by 27.3%, significantly lesser in surface area by 17.6%, and significantly greater in surface area to volume ratio by 14.6% than the non-cleft ala. The cleft-side ala was significantly greater by 43.1% horizontal axis to the midsagittal plane. In patients with primary rhinoplasty, the cleft-side ala had 28.0% less volume and 18.7% less surface area. In intermediate rhinoplasty, the cleft-side ala had 39.1% less volume and 23.5% less surface area than the non-cleft ala. CONCLUSIONS: Significant asymmetry exists between the cleft-side and non-cleft ala in patients with UCL. The cleft-side ala is significantly smaller in volume and surface area than the non-cleft ala. Additionally, the cleft-side ala demonstrates a significantly greater horizontal axis that contributes considerably to nasal asymmetry, supporting the need to restore a normal vertical axis to the clef-side ala.

Three-dimensional Anthropometric Analysis of Racial and Ethnic Differences in Unilateral and Bilateral Cleft Nasal Deformity.

This study used three-dimensional measurements to provide a nasolabial analysis of patients with unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), and controls across different races and ethnicities.A retrospective comparative study.Tertiary care pediatric institution.The study included 90 patients with UCLP, 43 patients with BCLP, and 90 matched controls. Patients are separated as self-identified Caucasian, Hispanic, or African American.Nasal length, nasal protrusion, columellar height, columellar width, tip width, alar width, alar base width, nasolabial angle, upper lip length, philtrum length, nostril height, and nostril width.All UCLP groups had significantly greater columella and tip widths and decreased nasolabial angles than controls. All BCLP groups had significantly greater columella width, tip width, nasolabial angle, and nostril widths. Upper lip length, philtrum length, and nostril height were significantly decreased in BCLP compared to controls. Across UCLP groups, African Americans had significantly decreased nasal protrusion and columella height and a significantly increased columella width compared to Caucasians and Hispanics. Alar and alar base widths were significantly different between all groups. Across BCLP groups, the Caucasian nostril width was significantly less than the African Americans.These findings suggest that when correcting nasolabial characteristics in patients with cleft lip, it is important to consider racial and ethnic differences to achieve a normal appearance. Specifically, goals for alar width, alar base width, nasal tip, and projection should be tailored to the patient's race and ethnicity.

Three-Dimensional Assessment of Frontal Bossing and Temporal Pinching in Patients with Sagittal Craniosynostosis Using Curvature Analysis.

BACKGROUND: Sagittal craniosynostosis results in varying degrees of frontal bossing and bilateral temporal pinching. This study assessed the three-dimensional changes in these regions using curvature analysis and volumetric analysis before and 1 year after extended sagittal strip craniectomy (ESC) with postoperative helmet therapy. METHODS: A retrospective review of three-dimensional photographs of 50 subjects treated with ESC with postoperative helmet therapy and 50 age-matched controls was performed. Images were collected preoperatively and 1 year postoperatively. Forehead convexity and temple concavity were quantified. Computed tomographic scans of subjects with and without sagittal synostosis were analyzed to assess the percentage of total intracranial volume (ICV) in the anterior cranial fossa before and after ESC with postoperative helmet therapy. RESULTS: Forehead convexity in the ESC with postoperative helmet therapy group preoperatively (24.49 ± 3.16 m -1 ) was significantly greater than controls (22.48 ± 3.84 m -1 ; P = 0.005). Forehead convexity significantly decreased after ESC with postoperative helmet therapy (18.79 ± 2.43 m -1 ; P < 0.001) and did not differ from controls (19.67 ± 3.08 m -1 ; P = 0.115). The ESC group had more concave temples preoperatively (-10.27 ± 4.37 m -1 ) as compared with controls (-6.99 ± 3.55 m -1 ; P < 0.001). Temple concavity significantly decreased after ESC (-4.82 ± 3.17 m -1 ; P < 0.001) and did not differ from controls (-5.64 ± 3.27 m -1 ; P = 0.075). In the ESC group, the percentage ICV in the anterior cranial fossa decreased from 22.03% to 18.99% after surgery, whereas the anterior volume in controls was stable (17.74% to 16.81%). CONCLUSIONS: The ESC group had significantly greater forehead convexity, temple concavity and anterior cranial fossa volume compared with controls. One year after ESC with postoperative helmet therapy, forehead convexity, temple concavity, and percentage ICV in the anterior fossa were comparable to controls. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Three-Dimensional Treatment Outcomes of a Virtual Helmet Design Protocol for Sagittal Strip Craniectomy.

BACKGROUND: The helmet worn after sagittal strip craniectomy must be customized to the surgical procedure and the patient's anatomy to achieve optimal outcomes. This study compares three-dimensional head shape outcomes obtained from a novel virtual helmet design and from a traditional helmet design. METHODS: Twenty-four patients underwent extended sagittal strip craniectomy performed by a single surgeon and helmet management performed by a single orthotist. Eleven patients constitute the traditional helmet group, with helmet design based on laser scans. Thirteen patients constitute the virtual helmet group, with helmet design based on an overlay of a three-dimensional volume rendering of a low-radiation protocol computed tomographic scan and three-dimensional photograph. Cephalic index and vertical height were recorded from three-dimensional photographs. Three-dimensional whole-head composite images were generated to compare global head shape outcomes to those of age-matched controls. RESULTS: There was no significant difference in mean cephalic index between the virtual helmet group (83.70 ± 2.33) and controls (83.53 ± 2.40). The differences in mean cephalic index between the traditional helmet group (81.07 ± 3.37) and controls and in mean vertical height were each significant (p < 0.05). Three-dimensional analysis demonstrated normal biparietal and vertical dimensions in the virtual helmet group compared to controls. The traditional helmet group exhibited narrower biparietal dimension and greater vertical dimension compared to controls. CONCLUSIONS: Traditional and virtual helmet protocols improved mean cephalic index, but the virtual helmet group yielded more consistent and greater change in cephalic index. The virtual helmet design protocol yielded three-dimensional outcomes similar to those of age-matched controls. Traditional helmet design yielded a narrower biparietal dimension and greater vertical dimension to the cranial vault compared to the virtual helmet group and controls. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Three-Dimensional Analysis of Bilateral Cleft Lip and Palate Nasal Deformity.

OBJECTIVE: This cross-sectional study utilizes 3-dimensional analysis to assess nasal morphology in patients with bilateral cleft lip and palate (BCLP) compared to controls across the timeline of cleft care. DESIGN: Retrospective comparative cross-sectional study. SETTING: Tertiary pediatric academic institution. PATIENTS AND PARTICIPANTS: One hundred and twelve patients with BCLP and an equal number of age and sex-matched control participants. MAIN OUTCOME MEASURE(S): Nasolabial angle, nasal length, nasal protrusion, columella length, columella width, nasal tip width, alar width, and alar base width were collected at each time point. The measurements were collected pre-nasoalveolar molding (NAM) therapy, post-NAM therapy, post-primary cleft rhinoplasty, 1 year, 5 years, 10 years, and 15 years of age. RESULTS: Nasolabial angle and nasal tip width were significantly different from controls from pre-NAM through 15 years of age time points. Nasal length was not significantly different at any time point. Alar width and alar base width were significantly different from pre-NAM through 10 years of age time points. Nasal protrusion, columella length, and columella width were significantly different from pre-NAM through 5 years of age time points. CONCLUSIONS: This study demonstrates that three-dimensional photogrammetry is effective in assessing the changes in nasal morphology that occur throughout the course of care in patients with BCLP from before cleft lip repair to the completion of nasal growth.

The Pediatric Craniofacial Collaborative Group (PCCG) Consensus Conference Methodology.

OBJECTIVE: This article describes the methodology used for the Pediatric Craniofacial Collaborative Group (PCCG) Consensus Conference. DESIGN: This is a novel Consensus Conference of national experts in Pediatric Craniofacial Surgery and Anesthesia, who will follow standards set by the Institute of Medicine and using the Research and Development/University of California, Los Angeles appropriateness method, modeled after the Pediatric Critical Care Transfusion and Anemia Expertise Initiative. Topics related to pediatric craniofacial anesthesia for open cranial vault surgery were divided into twelve subgroups with a systematic review of the literature. SETTING: A group of 20 content experts met virtually between 2019 and 2020 and will collaborate in their selected topics related to perioperative management for pediatric open cranial vault surgery for craniosynostosis. These groups will also identify where future research is needed. CONCLUSIONS: Experts in pediatric craniofacial surgery and anesthesiology are developing recommendations on behalf of the Pediatric Craniofacial Collaborative Group for perioperative management of patients undergoing open cranial vault surgery for craniosynostosis and identifying future research priorities.

Congenital Inverted Conchal Bowl Deformity: Proposed Technique.

Congenital inverted conchal bowl is a rare deformity with unknown etiology. Its occurrence poses both functional and aesthetic difficulties for patients. We report 2 cases of congenital inverted conchal bowl deformity in an 8-year-old male and 17-year-old female. Both patients were first evaluated in a delayed fashion with concern for difficulty hearing, poor hygiene, and aesthetic concerns. Patient presentation, surgical management, and 6-month follow-up results are presented. Both patients had improved functional and aesthetic outcome after surgical correction.

Eye Tracking as a Proxy for Perceptual Evaluation of Repaired Cleft Lip.

Human interactions begin with near-instantaneous visual evaluation of the face, modifying emotional responses and social behavior toward the person. Eye-tracking technology can provide a potential window into how humans undertake this evaluation, and the threshold of whether a face containing a craniofacial difference is perceived as "normal." This study's main questions were whether gaze patterns differ from normal when evaluating children with craniofacial differences, and whether these gaze patterns vary with different levels of craniofacial deformity. Two experiments tested the feasibility of using eye tracking to study facial evaluation and whether there was a difference in the gaze pattern and characteristics, correlating with the Asher McDade Aesthetic Index. Fifty-three participants' eye movements were recorded as they gazed at photographs of children either with hemifacial microsomia or repaired cleft lip, with repaired cleft lip digitally corrected to symmetry, or with no craniofacial disorder. Recruitment and participation for this study occurred in a pediatric plastic surgery clinic and in a medical school student center. Participants gazed longer on the lip in the cleft-lip photographs compared with control photographs in the first experiment (207 ms; SD 75 versus 145 ms; SD 61, respectively; P = 0.04). This gaze bias was confirmed in the second experiment and found to correlate with severity (r = 0.042). Gaze patterns differ when individuals look at photographs with or without craniofacial differences. The degree to which these eye movement patterns differ correlates with the severity of craniofacial deformity.

Surgical Treatment of Nonsyndromic Unicoronal Craniosynostosis.

PURPOSE: Unicoronal synostosis (UCS) remains one of the most difficult craniofacial conditions to treat. This review attempts to consolidate all existing literature from the past 25 years that has investigated surgical treatment of nonsyndromic UCS. Additional attention is paid to specific areas of controversy regarding surgical management and evaluation of UCS: outcomes of fronto-orbital advancement (FOA) versus endoscopic strip craniectomy (ESC), emergence of spring-associated cranioplasty for craniosynostosis, and morphologic assessment metrics. METHODS: A literature search from 1992 to 2017 was performed with a defined search strategy and manual screening process. About 24 studies were included in the final review. RESULTS: There is inconsistency among studies in measuring outcomes. Despite wide utilization, FOA produces mixed results, especially when looking at long-term outcomes. Preliminary data suggest advantages to using ESC over FOA with regard to facial symmetry, postoperative strabismus, and operating time. Distraction osteogenesis has long been utilized in Asia and is now being actively studied in the United States. Data on spring-assisted cranioplasty are lacking. Long-term results at skeletal maturity for many of these techniques are lacking. DISCUSSION: Despite some promising results, methods of measurement are inconsistent and long-term data are lacking. All future research in this area would benefit from consistent and standardized reporting of data, including perioperative statistics and reproducible 3-dimensional computed tomography craniofacial measurements. Additionally, no definitive recommendations can be made until data at skeletal maturity are studied, and all studies would benefit from long-term follow-up data.

Behind the Black Box: The Evidence for the U.S. Food and Drug Administration Warning about the Risk of General Anesthesia in Children Younger than 3 Years.

On December 14, 2016, the U.S. Food and Drug Administration issued a Drug Safety Communication warning "that repeated or lengthy use of general anesthetic and sedation drugs during surgeries or procedures in children younger than 3 years or in pregnant women during their third trimester may affect the development of children's brains." Health care professionals were urged to "balance the benefits of appropriate anesthesia in young children and pregnant women against the potential risks, especially for procedures that may last more than 3 hours or if multiple procedures are required in children under 3 years." Surgeons must have an understanding of the evidence that led to the U.S. Food and Drug Administration warning and recommendations to appropriately weigh these risks and counsel their patients. In this article, the authors summarize the preclinical and clinical data that led to the U.S. Food and Drug Administration warning, discuss ongoing clinical studies, and provide strategies to reduce the risk of general anesthesia in patients younger than 3 years.

A Three-Dimensional Study of Midfacial Changes Following Le Fort II Distraction With Zygomatic Repositioning in Syndromic Patients.

Le Fort II distraction with zygomatic repositioning introduced the ability to restore central midfacial height and convexity independent of changes in orbital morphology. This study analyzes midfacial and orbital morphology before and after Le Fort II distraction with zygomatic repositioning.All patients who underwent Le Fort II Distraction with zygomatic repositioning between 2013 and 2015 were included. Two- and 3-dimensional measurements were made using 3dMD Vultus software to assess canthal tilt, nasolabial angle, ratio of midfacial to lower facial height, and absolute change in nasal length. Presence of an open bite and Angle classification were assessed before and after surgery.Four patients underwent segmental midface advancement using Le Fort II distraction with zygomatic repositioning. Associated diagnoses included Apert syndrome, Goldenhar syndrome, and achondroplasia. Changes in facial dimensions included: 3.19° improvement in canthal tilt (range -4.7° to 8.4°), 9° change in nasolabial angle (range -1.0° to 19°), and 0.69 cm increase in absolute nasal length (range 0.2-0.94 cm). Mean ratio of midfacial to lower facial height was 0.79 preoperatively and 0.89 postoperatively. Preoperatively, all patients demonstrated Angle class III with 3 of 4 patients demonstrating anterior open bite. All achieved closure of open bite and demonstrated class I or II occlusion. No complications were observed.Le Fort II distraction with zygomatic repositioning resulted in normalization of midfacial soft tissue landmarks. This form of advancement demonstrates the ability to selectively improve midfacial height and canthal tilt while restoring normal occlusion.

Three-dimensional changes in head shape after extended sagittal strip craniectomy with wedge ostectomies and helmet therapy.

OBJECTIVE Outcome studies for sagittal strip craniectomy have largely relied on the 2D measure of the cephalic index (CI) as the primary indicator of head shape. The goal of this study was to measure the 2D and 3D changes in head shape that occur after sagittal strip craniectomy and postoperative helmet therapy. METHODS The authors performed a retrospective review of patients treated with sagittal strip craniectomy at their institution between January 2012 and October 2015. Inclusion criteria were as follows: 1) isolated sagittal synostosis; 2) age at surgery < 200 days; and 3) helmet management by a single orthotist. The CI was calculated from 3D images. Color maps and dot maps were generated from 3D images to demonstrate the regional differences in the magnitude of change in head shape over time. RESULTS Twenty-one patients met the study inclusion criteria. The mean CI was 71.9 (range 63.0-77.9) preoperatively and 81.1 (range 73.0-89.8) at the end of treatment. The mean time to stabilization of the CI after surgery was 57.2 ± 32.7 days. The mean maximum distances between the surfaces of the preoperative and 1-week postoperative and between the surfaces of the preoperative and end-of-treatment 3D images were 13.0 ± 4.1 mm and 24.71 ± 6.83 mm, respectively. The zone of maximum change was distributed equally in the transverse and vertical dimensions of the posterior vault. CONCLUSIONS The CI normalizes rapidly after sagittal strip craniectomy (57.2 days), with equal distribution of the change in CI occurring before and during helmet therapy. Three-dimensional analysis revealed significant vertical and transverse expansion of the posterior cranial vault. Further studies are needed to assess the 3D changes that occur after other sagittal strip craniectomy techniques.

Volumetric changes in cranial vault expansion: comparison of fronto-orbital advancement and posterior cranial vault distraction osteogenesis.

BACKGROUND: Posterior cranial vault distraction osteogenesis has recently been introduced to treat patients with multisuture syndromic craniosynostosis and is believed to provide greater gains in intracranial volume. This study provides volumetric analysis to determine the gains in intracranial volume produced by this modality. METHODS: This was a two-center retrospective study of preprocedure and postprocedure computed tomography scans of two groups of 15 patients each with syndromic multisuture craniosynostosis treated with either fronto-orbital advancement or posterior cranial vault distraction osteogenesis. Scan data were analyzed volumetrically with Mimics software. Volumetric gains attributable to growth between scans were controlled for. RESULTS: The mean advancements were 12.5 mm for fronto-orbital advancement and 24.8 mm for distraction osteogenesis. The mean difference in volume between the preoperative and postoperative scans was 144 cm(3) for fronto-orbital advancement and 274 cm(3) for (p = 0.009). After controlling for growth, the corrected mean volume difference was 66 cm(3) for fronto-orbital advancement and 142 cm(3) for distraction osteogenesis (p = 0.0017). The corrected mean volume difference per millimeter of advancement was 4.6 cm(3) for fronto-orbital advancement and 5.8 cm(3) for distraction (p = 0.357). CONCLUSIONS: In this retrospective study, posterior cranial vault distraction osteogenesis provided statistically greater intracranial volume expansion than fronto-orbital advancement. The volume gains per millimeter advancement were similar between groups, with a trend toward greater gains per millimeter with distraction osteogenesis. Gradual expansion of the overlying soft tissues with posterior cranial vault distraction osteogenesis appears to be the primary mechanism for greater volume gains with this technique. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.