New diagnostic criteria for metopic ridges and trigonocephaly: a 3D geometric approach.

BACKGROUND: Trigonocephaly occurs due to the premature fusion of the metopic suture, leading to a triangular forehead and hypotelorism. This condition often requires surgical correction for morphological and functional indications. Metopic ridges also originate from premature metopic closure but are only associated with mid-frontal bulging; their surgical correction is rarely required. Differential diagnosis between these two conditions can be challenging, especially in minor trigonocephaly. METHODS: Two hundred seven scans of patients with trigonocephaly (90), metopic rigdes (27), and controls (90) were collected. Geometric morphometrics were used to quantify skull and orbital morphology as well as the interfrontal angle and the cephalic index. An innovative method was developed to automatically compute the frontal curvature along the metopic suture. Different machine-learning algorithms were tested to assess the predictive power of morphological data in terms of classification. RESULTS: We showed that control patients, trigonocephaly and metopic rigdes have distinctive skull and orbital shapes. The 3D frontal curvature enabled a clear discrimination between groups (sensitivity and specificity > 92%). Furthermore, we reached an accuracy of 100% in group discrimination when combining 6 univariate measures. CONCLUSION: Two diagnostic tools were proposed and demonstrated to be successful in assisting differential diagnosis for patients with trigonocephaly or metopic ridges. Further clinical assessments are required to validate the practical clinical relevance of these tools.

Prevalence and Severity of Positional Posterior Plagiocephaly and Positional Posterior Brachycephaly in Children and Adolescents in Japan.

This study aimed to assess the prevalence, severity, and natural history of positional posterior plagiocephaly (PPP) and positional posterior brachycephaly in Japan. We conducted a cross-sectional study of pediatric patients, ranging from 0 to 15 years old, evaluated for head trauma with negative computed tomography (CT) findings. The cranial vault asymmetry index (CVAI) was calculated using CT images at the superior orbital rim. Asymmetry according to CVAI values was subcategorized as follows: mild (3.5%-7%), moderate (7%-12%), and severe (>12%). The results were analyzed according to different age groups: group 1, 2-23 months (54 patients); group 2, 2-6 years (123 patients); and group 3, 7-15 years (123 patients). Overall, 300 patients were included (109 [36.3%] girls and 191 [63.7%] boys). The overall prevalence of PPP in the 300 patients was 46.7% (140 patients). PPP prevalence decreased consistently with age group: group 1, 57.4%; group 2, 47.2%; and group 3, 41.5%. Severe asymmetry was seen in all age groups. The overall mean cephalic index (CI) was 85.2. Cephalic index scores decreased consistently with age: group 1, 87.4; group 2, 85.1; and group 3, 84.3. The prevalence of PPP in Japan was higher than that reported in other countries. Although there was an overall decrease in the prevalence and severity of PPP with increasing patient age, PPP does not necessarily resolve spontaneously in all children. Furthermore, severe asymmetry was seen across all age groups.

Association of cranial base suture/synchondrosis fusion with severity of increased intracranial pressure in Crouzon syndrome.

This study investigated how the fusion states of the cranial base is related to the degree of increased intracranial pressure (ICP) in patients with Crouzon syndrome. This retrospective cohort study enrolled patients who were diagnosed with Crouzon syndrome between May 2007 and April 2022. We categorized the patients into three groups: A, B, and C, according to the severity of increased ICP and the number of cranial vault remodeling procedures for corrective operation. The preoperative fusion states of the cranial base sutures/synchondroses were examined using facial bone computed tomography and compared between groups. Overall, 22 patients were included in Groups A, B, and C, including 8, 7, and 7 patients, respectively. The preoperative average grades of the total cranial base suture/synchondrosis fusion appeared to significantly increase with severity, except for the frontoethmoidal suture, which showed the opposite tendency. In the subgroup analysis, frontosphenoidal, sphenoparietal, sphenosquamosal, parietomastoid, and occipitomastoid suture and petro-occipital synchondrosis were associated with earlier fusion in the more severe group. Premature closure of the cranial base sutures/synchodroses seems to be associated with increased ICP severity in patients with Crouzon syndrome. Precise evaluation of minor sutures/synchondroses at the first visit might help build subsequent operative plans and predict disease prognosis.

Changes in mandibular position during midface distraction in patients with syndromic craniosynostosis.

The purpose of this study was to evaluate changes in mandibular position during midface distraction. Midface distraction was performed in patients with syndromic craniosynostosis to increase upper airway volume. Although this treatment resulted in changes in occlusion, the concomitant changes in mandibular position were poorly understood. In this retrospective study, three-dimensional (3D) cephalograms were obtained before and after midface distraction in 15 patients with syndromic craniosynostosis. Perioperative polysomnography scores and changes in maxillary and mandibular position, mandibular volume, and upper airway volume were analyzed. Results showed a significant improvement in apnea-hypopnea index (AHI) (from 20.6 ± 21.3 to 6.9 ± 5.1, p < 0.05) and upper airway volume (from 2951.65 ± 2286.38 to 5218.04 ± 3150.05 mm3, p < 0.001). When the lowest point of the sella turcica was set as the reference point, the mandible moved significantly in an anterior direction (from 47.9 ± 11.5 to 51.9 ± 9.8 mm, p < 0.05). Mandibular volume did not change significantly perioperatively (from 32530.19 ± 10726.01 to 35590.50 ± 14879.21 mm3, p = 0.10). There were positive correlations between the rates of improvement in AHI and the amount of mandibular movement in the anterior and inferior directions (both p < 0.05). Within the limitations of the study, it seems that the mandible moved in the anterior-inferior direction after midface distraction, and the amount of movement correlated with improvement in respiratory function. Therefore, it is important to consider the position of the mandible when determining the direction of midface distraction, as it may influence the therapeutic effect.

Nasal monobloc osteotomy for correction of late nasal and orbital asymmetry of unicoronal synostosis: A morphometric and outcomes study.

BACKGROUND: Craniofacial asymmetry associated with unicoronal synostosis (UCS) may persist into the teenage years despite surgery in infancy. This study evaluated outcomes following a nasal monobloc procedure by mobilizing a united nasomaxillary and bilateral medial orbital segment of bone (nasal monobloc) to perform corrective translational and rotational movement for secondary correction of residual nasal-orbital asymmetry associated with UCS. METHODS: A retrospective review of all UCS patients treated with nasal monobloc at our institution was performed. Demographic information was recorded, and pre- and postoperative 2D imaging was used for morphometric outcome analysis. Outcomes and complications were tabulated. RESULTS: The study included 14 patients (5 males, 9 females; mean age 14.6 years; range 9.6 to 22.5 years; mean follow-up 70.6 months range 12 to 132 months). Ancillary procedures (scar revision, forehead/orbital contouring, MEDPOR® augmentation) were performed in all patients at the time of the nasal monobloc. One patient underwent a repeat procedure 6 years later following technique modification. Additionally, another patient experienced late overgrowth of the frontal sinus with forehead asymmetry. The morphometric analysis demonstrated significant (p < 0.05) pre-op to post-op improvements in naso-orbital asymmetry, as demonstrated by horizontal orbital aperture ratio (0.88 vs 0.99), midline to exocanthion ratio (0.91 vs 0.98), orbital index ratio (1.15 vs 1.01), and midline discrepancy (7.1 degrees vs 2.7 degrees). CONCLUSION: Nasal monobloc osteotomy provides a reasonable surgical treatment to improve both the nasal and orbital asymmetries associated with unicoronal synostosis, including frontal nasal deviation, basal nasal deviation, and orbital aperture asymmetry. It is important to note that confounding anatomic variables such as globe dystopia, strabismus, and scleral show may affect the perception of orbital symmetry.

Endoscopy-Assisted Craniosynostosis Surgery Versus Cranial Vault Remodeling for Non-Syndromic Craniosynostosis: Experience of a Single Center.

AIM: To evaluate and compare open cranial vault remodeling (OCVR) and endoscopy-assisted craniosynostosis surgery (EACS) in patients with non-syndromic craniosynostosis and to develop an algorithm to determine the most appropriate surgery for each patient. MATERIAL AND METHODS: Eighty-five children with craniosynostosis who underwent surgery between 2010 and 2022 were retrospectively analyzed. Demographic data, comorbidities, and peri-operative findings of the patients were recorded. Pre- and post-operative comparisons were made between predetermined measurement techniques for each deformation. In addition, measurements were obtained by computed tomography (CT) or 3D stereophotogrammetric (3DSPG) methods from eligible patients and compared with one another. RESULTS: In our study, 61 patients underwent EACS, whereas 24 underwent OCVR. The operating time of OCVR was approximately 54.4 minutes longer than that of EACS (p < 0.001). The intra-operative blood loss was around 139 ml higher in OCVR (p < 0.001). The length of hospital stay for patients who underwent EACS was shorter at 8.4 days on average (p < 0.001). Surprisingly, 5 complications were observed in OCVR compared with 7 in EACS. While the cosmetic outcome of EACS was superior in most of the pathologyspecific measurement techniques, the metopic index increased only in patients with metopic synostosis after both surgical operations. Still, this increase was lower in EACS than in OCVR. CONCLUSION: This study suggests that endoscopic craniosynostosis surgery has lower estimated blood loss and operation and hospitalization times, as well as comparable cosmetic results compared with open vault surgeries on long-term follow-up. CT and 3DSPG methods can help distinguish between different types of measurement techniques for synostoses. However, no significant differences were found in the comparisons since 3DSPG can also provide reliable measurements comparable to those on CT during follow-up.

The influence of orbital architecture on strabismus in craniosynostosis.

PURPOSE: To better characterize the correlation of bony orbital dysmorphology with strabismus in craniosynostosis. METHODS: The medical records of patients with craniosynostosis with and without strabismus seen at Rady Children's Hospital (San Diego, CA) from March 2020 to January 2022 were reviewed retrospectively in this masked, case-control study. Computed tomography scans of the orbits were analyzed to obtain dimensions of the orbital entrance and orbital cone. Primary outcome was correlation of strabismus with orbital measurements. RESULTS: A total of 30 orbits from 15 patients with strabismus and 15 controls were included. Craniofacial disorders included in the study were nonsyndromic craniosynostosis (63%), Crouzon syndrome (13%), Apert syndrome (13%), and Pfeiffer syndrome (10%). Orbital index (height:width ratio) (P = 0.01) and medial orbital wall angle (P = 0.04) were found to differ significantly between the strabismus and control groups. CONCLUSIONS: In our small cohort, bony orbital dimensions, including the ratio of orbital height to width and bowing of the medial orbital wall, were associated with strabismus in craniosynostosis.

What can a morphometric study of unoperated children teach us about the natural history of metopic synostosis?

OBJECTIVE: Outcomes of surgical repair of trigonocephaly are well reported in the literature, but there is a paucity of information on the natural history of unoperated children. The authors evaluated a group of unoperated children with metopic synostosis to describe the natural change in head shape over time. METHODS: A database was screened for scans of children with unoperated trigonocephaly (2010-2021). Multisuture cases and those with a metopic ridge were excluded. Three-dimensional surface scans (3D stereophotogrammetry/CT) were used for morphological analysis. Nine previously published parameters were used: frontal angle (FA30°), anteroposterior (AP) volume ratio (APVR), AP area ratio (APAR), AP width ratios 1 and 2 (APWR1 and APWR2), and 4 AP diagonal ratios (30° right APDR [rAPDR30], 30° left APDR [lAPDR30], 60° right APDR [rAPDR60], and 60° left APDR [lAPDR60]). RESULTS: Ninety-seven scans were identified from a cohort of 316 patients with a single metopic suture, in which the male-to-female ratio was 2.7:1. Ages at the time of the scan ranged from 9 days to 11 years and were stratified into 4 groups: group 1, < 6 months; group 2, 6-12 months; group 3, 1-3 years; and group 4, > 3 years. Significant improvements were detected in 5 parameters (APVR, APAR, APWR1, rAPDR30, and lAPDR30) over time, whereas no significant differences were found in FA30, APWR2, rAPDR60, and lAPDR60 between age groups. CONCLUSIONS: Forehead shape (surface area and volume), as well as narrowing and anterolateral contour at the frontal points, differed significantly over time without surgery. However, forehead angulation, narrowing, and anterolateral contour at temporal points did not show significant differences. This knowledge will aid in surgical and parental decision-making.

Improved Facial and Skull-Base Symmetry following Osteotomy and Distraction of Unilateral Coronal Synostosis.

BACKGROUND: Unilateral coronal synostosis (UCS) results in a surgically demanding deformation, as the deformity is asymmetric in the calvaria but also presents with facial scoliosis and orbital dystopia. Traditional cranioplasties correct the forehead but have little effect on the face and orbits. In this article, the authors describe a consecutive series of patients operated on for UCS with osteotomy of the fused suture combined with distraction osteogenesis. METHODS: Fourteen patients (mean age, 8.0 months; range, 4.3 to 16.6 months) were included in this study. The authors measured and compared the orbital dystopia angle, anterior cranial fossa deviation, and anterior cranial fossa cant between preoperative computed tomography results and those at distractor removal. RESULTS: Blood loss was 6.1 mL/kg (range, 2.0 to 15.2 mL/kg), and length of stay was 4.4 days (range, 3.0 to 6.0 days). The authors observed significant improvements in the median orbital dystopia angle from 9.8 degrees (95% CI, 7.0 to 12.6 degrees) to 1.1 degrees (95% CI, -1.5 to 3.7 degrees) ( P < 0.001), anterior cranial fossa deviation from 12.9 degrees (95% CI, 9.2 to 16.6 degrees) to 4.7 degrees (95% CI, 1.5 to 7.9 degrees) ( P < 0.001), and anterior cranial fossa cant from 2.5 degrees (95% CI, 1.5 to 3.5 degrees) to 1.7 degrees (95% CI, 0.0 to 3.4 degrees) ( P = 0.003). CONCLUSIONS: Osteotomy combined with a distractor for UCS straightened the face and relieved orbital dystopia by affecting the nose angle relative to the orbits, correcting the deviation of the cranial base in the anterior fossa, and lowering the orbit on the affected side. Furthermore, this technique demonstrated a favorable morbidity profile with low perioperative bleeding and a short inpatient period, suggesting its potential to improve the surgical treatment of UCS. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Computed tomography quantitative analysis of cranial vault dysmorphology and severity of facial complex changes in posterior synostotic plagiocephaly patients.

BACKGROUND: Posterior synostotic plagiocephaly (PSP) impacts craniofacial skeleton. Study quantifies facial changes in children with PSP to investigate the impact of age and PSP severity at diagnosis on the facial dysmorphology. MATERIAL AND METHODS: High-resolution preoperative CT images of 22 infants with PSP were analyzed. They were divided according to the early or late age at time of diagnosis. Each group was further subdivided according to the severity of PSP evaluated by the cranial vault asymmetry index (CVAI): mild-moderate PSP (CVAI between 3 and 12%) and severe PSP (CVAI > 12%). Analysis of the facial complex was performed. Each group was compared with age-matched healthy subjects. RESULTS: All children exhibited unilateral lambdoid suture synostosis. The "early" diagnosis group consisted of 7 children with mild-moderate PSP while the "late" diagnosis group of 15 children in which 6 children had mild-moderate and 9 children severe PSP. All children showed altered position of glenoid fossae and mandibular asymmetry characterized by reduced mandibular diagonal distance length on the affected side while the subgroup of children with severe PSP detected in "late" diagnosis group had also altered mandibular inclination and reduced midfacial depth on both sides. CONCLUSIONS: PSP causes cranial base dysmorphology which drives changes in facial complex growth; the severity of facial changes mainly depends on the severity of cranial vault dysmorphology detected by CVAI. Mandible reshapes early under the stress of altered biomechanical forces of the skull base while changes in the maxilla are secondary to the asymmetric growth of the mandible and occur only in severe cases.

Prenatal diagnostic approaches diagnosed craniosynostosis and identified a novel nonsense variant in SMAD6 in a Chinese fetus.

Craniosynostosis is one of the most common congenital craniofacial birth defects. The genetic etiology is complex, involving syndromic developmental diseases, chromosomal abnormalities, and monogenic non-syndromic diseases. Herein, we presented a proband of craniosynostosis, who firstly displayed structural abnormalities. This research conducted dynamic ultrasound monitoring a fetus with gradually developing intrauterine growth retardation (IUGR). A novel de novo variant c.41G > A: p.W14* in SMAD6 was identified by pedigree analysis and genetic examination approaches. Recombinant plasmid carrying wild-type sequence and mutant that carries c.41G > A in SMAD6 were constructed and transfected into HEK293T cells. mRNA and protein expression of SMAD6 were reduced in SMAD6 mutants compared to the wild type. Cycloheximide (CHX) treatment and si-UPF1 transfection rescued the SMAD6 mRNA expression in the mutant construct, indicating that c.41G > A: p.W14* in SMAD6 triggered nonsense-mediated mRNA degradation (NMD) process and thus led to haploinsufficiency of the protein product. Our study demonstrated that whole-exome sequencing (WES) was a powerful tool for further diagnosis and etiological identification once fetal malformation was detected by ultrasound. Novel de novo c.41G > A: p.W14* in SMAD6 is pathogenic and potentially leads to craniosynostosis via NMD process.

Surgical Treatment of Trigonocephaly, Simplified Technique for Moderate Cases.

BACKGROUND: The prevalence of trigonocephaly has increased worldwide over the past 2 decades. Early identification and appropriate treatment are critical. The aim of this study is to evaluate the outcomes and the effect of metopic suture excision, perisutural frontal bone shave, and bilateral pericranial flap method on the shape of the forehead after surgical correction in infants with moderate trigonocephaly. METHODS: The present study was performed as a cross-sectional study on 40 infants of 3 to 12 months old with trigonocephalus who underwent metopic suture excision and pericardial flap surgery in Mofid Pediatric Hospital from 2016 to 2022. The definitive diagnosis of patients' trigonocephaly was made based on clinical signs and computed tomography scan findings by a plastic surgeon. RESULTS: Overall in 40 patients operated by this technique, 23 (57.5%) of cases were males, and 17 (42.5%) were females. The mean age of patients was 7.86 ± 2.22 months. Hospital stay was 2 to 4 days (mean: 3 d), intensive care unit admission was in 33 cases for 24 hours, and no intensive care unit admission for 7 cases. Blood was transfused during surgery for 25 patients, and 15 patients did not require blood transfusion use. Results were evaluated in 6 to 12 months after surgery by 3 independent plastic surgeons, with pre and postoperative photos. Satisfaction with the results of forehead shape was excellent for 60% of patients, good for 37.5%, and moderate for 2.5%. Only one female patient had a recurrence after the surgery. CONCLUSION: This study showed that the pericranial flap method after full metopic suture excision and frontal shave was very effective in the treatment of infants with moderate trigonocephaly.

Endoscopy-Assisted Craniosynostosis Surgery without Postoperative Helmet Molding Therapy.

OBJECTIVE: Endoscopy-assisted craniosynostosis surgery (EACS) yields excellent surgical outcomes by minimizing blood loss, operative time, and hospital stays. Postoperative helmet therapy (PHT), commonly employed for head shape correction, involves frequent adjustments, potential complications, and high costs. Given the rising cost of helmet therapy, reduced insurance coverage, and limited availability in low- and middle-income countries, understanding success rates without helmet use is crucial. The present study analyses the anthropometric results of the first EACS series without PHT. METHODS: A retrospective analysis of a single-center series involving 90 consecutive patients who underwent EACS without PHT from 2012 to 2022 was conducted, with a follow-up exceeding 3 years. The study exclusively included patients with nonsyndromic isolated sagittal synostosis, with 33 meeting the criteria. Craniometric measurements were obtained from preoperative, 1-year postoperative, and the latest computed tomography scans. For isolated sagittal synostosis cases, the cephalic index (CI) was calculated (CI >75 for excellent results, CI 70-75 for good results, and <70 for poor results). Collected data encompassed patient sex, age, and follow-up time. RESULTS: The mean age was 84.8 ± 45.3 days (2.79 ± 1.49 months) within a range of 3-172 days. The preoperative mean CI was 68 ± 42, increasing to 76 ± 6 1 year postoperatively (mean difference +8 ± 6.3; P = 0.0001). Seventy-one percent of patients achieved excellent results, 23% good (CI = 70-75), and 6% poor. Reintervention was unnecessary. CONCLUSIONS: EACS without PHT demonstrates favorable anthropometric results, cost reduction, and simplified postoperative management.

A Suture-specific Photo Score for Metopic Synostosis.

Head shape assessments in children with metopic synostosis are a relevant outcome measure in addition to functional measures, such as neurocognitive outcomes, behavioral outcomes, and visual functioning outcomes. However, consensus on head shape assessments in children with metopic synostosis is lacking. The aim of this study is to develop a reproducible and reliable suture-specific photo score that can be used for cross-center comparison of phenotypical severity of metopic synostosis and evaluation of esthetic outcome of treatment later in childhood. We conducted a retrospective study among nonsyndromic metopic synostosis patients aged <18 years. Preoperative and postoperative photosets of patients with metopic synostosis from 6 expert centers were included. The photo score was discussed in the group of expert craniofacial plastic surgeons and pediatric neurosurgeons. Interrater reliability was determined with modified weighted Fleiss' kappa and intraclass correlation coefficients. Correlation between individual photo score items with overall phenotype was assessed using Spearman correlation analyses. The metopic synostosis photo score contained the following items: "wedging of the forehead", "hypotelorism", "temporal hollowing", "biparietal widening,"and an assessment of "overall phenotype". Items were scored on a 4-point ordinal scale ranging from normal to severe. We found moderate interrater reliability for all items, but substantial agreement for the summed scores. Correlation with overall phenotype was lowest for biparietal widening. To conclude, although agreement on individual photo score items was suboptimal, the agreement on the summed score was substantial, which indicates there is consensus on the overall severity of the metopic synostosis phenotype.

CranioRate: An Image-Based, Deep-Phenotyping Analysis Toolset and Online Clinician Interface for Metopic Craniosynostosis.

BACKGROUND: The diagnosis and management of metopic craniosynostosis involve subjective decision-making at the point of care. The purpose of this work was to describe a quantitative severity metric and point-of-care user interface to aid clinicians in the management of metopic craniosynostosis and to provide a platform for future research through deep phenotyping. METHODS: Two machine-learning algorithms were developed that quantify the severity of craniosynostosis-a supervised model specific to metopic craniosynostosis [Metopic Severity Score (MSS)] and an unsupervised model used for cranial morphology in general [Cranial Morphology Deviation (CMD)]. Computed tomographic (CT) images from multiple institutions were compiled to establish the spectrum of severity, and a point-of-care tool was developed and validated. RESULTS: Over the study period (2019 to 2021), 254 patients with metopic craniosynostosis and 92 control patients who underwent CT scanning between the ages of 6 and 18 months were included. CT scans were processed using an unsupervised machine-learning based dysmorphology quantification tool, CranioRate. The average MSS was 0.0 ± 1.0 for normal controls and 4.9 ± 2.3 ( P < 0.001) for those with metopic synostosis. The average CMD was 85.2 ± 19.2 for normal controls and 189.9 ± 43.4 ( P < 0.001) for those with metopic synostosis. A point-of-care user interface (craniorate.org) has processed 46 CT images from 10 institutions. CONCLUSIONS: The resulting quantification of severity using MSS and CMD has shown an improved capacity, relative to conventional measures, to automatically classify normal controls versus patients with metopic synostosis. The authors have mathematically described, in an objective and quantifiable manner, the distribution of phenotypes in metopic craniosynostosis.

Impact of One-Piece Fronto-Orbital Distraction Osteogenesis on Orbital Morphology and Strabismus Development in Unilateral Craniosynostosis.

BACKGROUND: Unicoronal craniosynostosis (UCS) is associated with orbital dysmorphologies that underlie ophthalmologic dysfunctions, such as strabismus. This study aimed to assess orbital dysmorphology in patients with UCS and how it changes after fronto-orbital distraction osteogenesis (FODO), and to analyze the features of new-onset strabismus. METHODS: A retrospective analysis was conducted on 19 patients with UCS who underwent FODO between May of 2008 and November of 2020. Ophthalmologic records and computed tomographic scans were reviewed. Seven parameters, including width, height, volume, and four-direction orbital angles were evaluated in patients with UCS and compared with those of age-matched control subjects. RESULTS: The superolateral angle and vertical angle of the ipsilateral orbit and the superomedial angle (SMA) of the contralateral orbit were more obtuse than those of the controls. Following FODO, the ipsilateral superolateral angle was decreased from 69.2 ± 5.4 degrees to 59.1 ± 4.2 degrees ( P = 0.001), and the contralateral SMA was decreased from 64.8 ± 5.8 degrees to 60.2 ± 6.0 degrees ( P = 0.003). Four of the 17 patients without strabismus in the preoperative period developed strabismus, and the horizontal type was the most common. Logistic regression analysis demonstrated a significant association between new-onset strabismus and SMA difference between both orbits (OR, 1.39; P = 0.041). CONCLUSIONS: Orbital dysmorphology in the UCS is bilateral, and the orbital roofs are dysmorphic. The bilateral orbital roofs are lifted toward the fused coronal suture and can be improved after FODO. Horizontal strabismus, such as esotropia and exotropia, is common after FODO, and superomedial orbital roof asymmetry may play a role in its development. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Midface hypoplasia in syndromic craniosynostosis: predicting craniofacial growth via a novel regression model from anatomical morphometric analysis.

Midface hypoplasia in syndromic craniosynostosis (SC) may lead to serious respiratory issues. The aim of this study was to analyse the morphometric correlation between midface and cranial base parameters in paediatric SC patients in order to formulate predictive regression models. The computed tomography scans of 18 SC patients and 20 control were imported into Materialise Mimics Medical version 21.0 software for the measurement of multiple craniofacial landmarks and correlation analysis. The results showed a strong correlation of anterior cranial base (SN), posterior cranial base (SBa), and total cranial base (NBa) (r = 0.935) to maxilla length and width (ZMR-ZML) (r = 0.864). The model of NBa = - 1.554 + 1.021(SN) + 0.753(SBa) with R2 = 0.875 is proposed to demonstrate the development of the cranial base that causes a certain degree of midface hypoplasia in SC patients. The formula is supported using a prediction model of ZMR-ZML = 5.762 + 0.920(NBa), with R2 = 0.746. The mean absolute difference and standard deviation between the predicted and true NBa and ZMR-ZML were 2.08 ± 1.50 mm and 3.11 ± 2.32 mm, respectively. The skeletal growth estimation models provide valuable foundation for further analysis and potential clinical application.

Atypical sagittal suture craniosynostosis: pathological considerations for early closure of the anterior part of the sagittal suture.

Sagittal suture synostosis is one of the most common craniosynostoses and is often diagnosed by characteristic narrow and long skull shape, scaphocephaly. However, some patients with sagittal suture synostosis do not present with typical scaphocephaly, making early diagnosis difficult. In this study, five cases of characteristic skull deformity showing a narrowing of the cranium posterior to the coronal suture on computed tomography (CT) are presented. The three older children presented with papilledema and intellectual disability and a closed sagittal suture on CT. The two infant cases were diagnosed with the characteristic cranial deformities with aggravation of the deformity over time, but sagittal suture closure was not evident on CT. All patients underwent cranial remodeling surgery. In the two infant cases, the histopathological findings showed that the anterior part of the sagittal suture was firmly fused with fibrous tissue without bony fusion. These findings suggested that narrowing of the cranium posterior to the coronal suture might be due to functional fusion of the anterior portion of the sagittal suture prior to bony fusion. In an infant presenting with such a deformity that shows aggravation of the deformity over time, surgical treatment should be considered.

The Accuracy of Classification Systems in Nonsyndromic Sagittal Craniosynostosis.

Numerous classification systems of nonsyndromic sagittal craniosynostosis (NSC) are applied but none has gained a wide acceptance, since each classification is focused on distinct aspects. The aim of the study was to assess the accuracy of 4 classifications of NSC discussed in the literature by defining the associations among the classifications, individual features (sex, age, cranial index), and objective morphologic criteria (frontal bossing, retrocoronal constriction, sagittal ridge, and occipital bulleting). The study was conducted on anonymized thin-cut CT scans of 133 children with NSC 1 to 12 months old (mean age 5.42 mo). The type of cranial dysmorphology was assessed using 4 classification systems, focusing on skull shape, pattern of sagittal suture closure (Heuzé classification), deformation of skull vault (Sakamoto classification), and a single-dominant feature (David classification). Each patient was also independently investigated for the presence of morphologic criteria. A multivariate analysis was performed to explore the relations among the classifications and assess their accuracy. In the analyzed cohort sphenocephaly (38.3%), CFF type by Heuzé (30.8%), type I by Sakamoto (72.9%), and a central type by David (42.9%) were dominant findings. Regarding the morphologic criteria, frontal bossing was observed the most frequently (91.7%). The age of patients and cranial index differed significantly among the shapes of skull and David classifications ( P <0.001). The shape-based system showed the strongest correlation with other classifications and with measurable variables. Other classifications have much in common and some overlap, but none of them constitutes a standalone system to define all aspects of cranial dysmorphology in NSC.

Biparietal remodelling and total vault remodelling in scaphocephaly-a comparative study using 3d stereophotogrammetry.

PURPOSE: The aim of the study was to compare the results of two surgical techniques for the treatment of isolated sagittal synostosis (ISS) by means of 3D stereophotogrammetry. One technique, the Renier's "H" technique (RHT) comprised a biparietal expansion, the other, the total vault remodeling (TVR) included also a frontal remodeling. METHODS: The two groups of operated children were compared with a third control group of normocephalic children. The 3D scanning was performed in all children between 12 and 245 months of age. On each 3D image six measurements and indices have been made, with the aim of evaluating not only length and width of the head, but also the height. The cranial index (CI) was measured in a plane parallel to the nasion-tragus plane, at the intersection with the opisthocranion. RESULTS: Each of the three groups (RHT, TVR, control group) included 28 children. The measurements that were influenced by the correction of the frontal bossing, namely the CI and the sagittal length, were closer to normocephaly after TVR than after RHT. Lesser or no statistical difference was documented in the measurements evaluating the biparietal aspect and the height of the vertex, indicating that the biparietal expansion is effective in both procedures. CONCLUSION: Based on our results TVR results in a better esthetical outcome, particularly in relation to the direct surgical remodeling of the frontal bossing.