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Objective:
To identify an additional objective measure to aid in the evaluation of children with isolated metopic craniosynostosis.
Design:
This is a retrospective study comparing specific computed tomography scan measurements between surgical and nonsurgical cohorts of children with isolated metopic craniosynostosis. Children were included if t...
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Zusammenfassung In dieser Untersuchung wurde der Zusam-menhang zwischen dem Maß der Brachyzephalie und transnasalen Druck-und Widerstandswerten ermittelt. Es wurden 64 Hunde unterschiedlicher Rassen untersucht. Von allen Tieren wurden zunächst normale Atmungskurven aufgenommen. Anschließend wurden unter Anästhesie die transnasalen Druckunterschiede...
Citations
... Metopic indices were taken from the CT and found to be within normal range. [3,5,6,16] e midfronto-zygomatic diameter was 70.4 mm, the eurioneurion diameter was 115, resulting in a metopic index of 0.61. In addition, the endocranial bifrontal angle was 139 degrees. ...
... Although there is currently no standard or widely accepted craniometric measurement for the diagnosis of metopic craniosynostoses, several have been proposed in the contemporary literature. [3,5,6,16] Many of our patient's measured craniofacial parameters align more closely with reported controls rather than those of patients with metopic craniosynostosis, which is consistent with the very mild, even subtle, features of metopic craniosynostosis she displayed. We certainly acknowledge that her case did not demonstrate a classic phenotypical appearance for metopic craniosynostosis. ...
Background
Marfan syndrome (MFS) is an autosomal dominant disorder of the connective tissues caused by mutations in the FBN1 gene which can result in widespread systemic involvement. Loeys-Dietz syndrome (LDS) is a related autosomal dominant disorder of connective tissue with widespread systemic involvement which has phenotypic overlap with MFS. LDS is caused by heterozygous pathogenic variants in six different genes, the most common of which involve transforming growth factor beta-receptor 1 or 2. While LDS is commonly associated with craniofacial manifestations, MFS is not typically characterized by craniosynostosis.
Case Description
We present a 7-month-old female patient with MFS and metopic craniosynostosis with an unusual clinical presentation who underwent cranial vault reconstruction with fronto-orbital advancement and anterior cranial vault remodeling. Her course was complicated by impaired wound healing after surgery, requiring return to the operating room.
Conclusion
Phenotypic overlap between genetic disorders can confound clinical diagnosis as illustrated in this case. Genetic testing can be highly valuable in the diagnosis of clinically variable disorders. Patients with MFS who undergo cranial surgery may be at increased risk for wound healing complications.
... McEwan et al 53 proposed the cephalic width-ICD ratio as an additional objective measure to aid in the evaluation of children with isolated metopic craniosynostosis, based on the studies by Waitzman et al. 54,55 Yan et al 10 recently described the Bitemporal lateral brow width to BP (biparietal width) ratio (BT-LB/BP) and the Bitemporal midforehead width to BP ratio (BT-MF/BP), finding them significantly higher for control patients and metopic ridge patients compared to metopic synostosis patients (where metopic patients were the ones diagnosed with isolated metopic synostosis resulting in trigonocephaly for whom the senior author recommended surgery). The authors 10 measured the distances using just a caliper, without any CT scanning. ...
... The CT scans should be obtained with the patient in a standardized position: the head in a neutral position with regard to rotation and the orbitomeatal line perpendicular to the CT table, as described by Waitzman and colleagues 54,55 and by McEwan and colleagues. 53 However, the risk of ionizing radiation to pediatric body tissues has been underscored and the need for each child with a suspected metopic craniosynostosis to have a CT scan has been questioned. 57,58 Important to remember, the metopic suture normally fuses before 9 months of age, therefore, a fused suture on a CT after 6 months of age is not by itself diagnostic of metopic synostosis. ...
Metopic craniosynostosis is the second most frequent type of craniosynostosis. When the phenotypic presentation has been deemed severe the treatment is surgical in nature and is performed in infancy with fronto-orbital advancement and cranial vault remodeling. At the time of this writing, there is no consensus regarding an objective evaluation system for severity, diagnostic criteria, or indications for surgery. This study aims to review the anthropometric cranial measurements and the relative diagnostic criteria/classification of severity/surgical indications proposed so far for this skull malformation, and to investigate if there is any scientific support for their utility.
... To date, studies evaluating surgical outcomes have focused on pre-and postoperative aesthetic markers aided by caliper-or CT-based anthropometric cranial measurements or laser scans by orthotic makers (Table 5) [2,8,14]. These methods include the interfrontal angle (CT) [13,16], cephalic width-intercoronal distance ratio (CT scans) [17], metopic index (CT and clinical) [27], metopic angle (laser scan) [9][10], frontal volumes (CT) [11,28], and 3D photography [18]. Each method has limitations. ...
PurposeTo assess intermediate-term (> 3 years) outcomes of endoscopic strip craniectomy with postoperative helmet therapy (ESC + HT) for the treatment of infants with severe trigonocephaly.Methods
This retrospective study examined cranial morphology of consecutive patients with severe trigonocephaly treated with minimally invasive ESC + HT. Preoperative and follow-up clinical parameters were collected from patient charts. Interfrontal divergence angle (IFDA), a validated and accurate measure of forehead narrowing, was measured on preoperative CT scans and on preoperative and postoperative 2D photographs.ResultsSeven patients (4 male, 3 female) were included with a mean age at surgery of 2.76 months (range 1.8 to 4.1 months) and mean clinical follow-up of 3.71 years (photographic follow-up 2.73 years). The mean operative time was 91.4 min, with a mean estimated blood loss (EBL) of 57.1 ml and mean hospital length of stay of 1.14 days. IFDA improved from 118.8° to 135.9° (p < 0.01), with the mean final measurement falling within normal limits. The head circumference percentile was not significantly changed in follow-up. There was a statistically significant improvement in the inner-to-outer canthal distance ratio (p = 0.01) in follow-up, showing an improvement in hypotelorism. There were no dural tears, CSF leaks, infections, or other significant surgical morbidities, and there were no serious complications related to the use of helmet therapy. All patients achieved excellent aesthetic results judged by photographic comparison.Conclusion
This study demonstrated that patients treated with ESC + HT for metopic craniosynostosis showed measurable and significant improvement in forehead shape. This technique is a safe and effective alternative to more invasive surgical interventions.
... ICD represents the distance between the outer (i.e., superficial) surfaces of the coronal sutures; IPD represents the biparietal posterior maximum distance. [31,37]. These distances were computed by using the F plane because it was reported to be an accurate level [36]. ...
Purpose
Craniofacial dysmorphology varies significantly along a wide spectrum of severity in metopic cranial synostosis (MCS). This study aimed to quantify craniofacial changes, in MCS, to investigate their relationships with the severity of trigonocephaly.
Methods
By combining the metopic ridge and interfrontal angles, we identified three groups of trigonocephaly severity (mild group n.14, moderate group n.19, severe group n.18). We perform a quantitative analysis using high-resolution CT images evaluating (1) cranial fossae dimensions; (2) vault indices and ratios: interparietal/ intercoronal (IPD/ICD), interparietal/intertemporal (IPD/ITD), cephalic index, vertico-longitudinal index; (3) orbito-facial distances (midfacial depth, maxillary height, upper facial index, orbital distances, globe protrusions), maxilla and orbital volumes; (4) supratentorial (ICV) and infratentorial (PCFV) cranial volumes and supratentorial (WBV) and infratentorial (PCFBV) brain volumes.
Results
In all groups, middle skull base lengths and upper midface index were increased. In moderate and severe groups: anterior hemifossa lengths were reduced, IPD/ICD and vertico-longitudinal index were changed; midfacial depth, anterior, mild, and lateral interorbital distances were reduced; globe protrusions were increased. The comparison between moderate and severe groups showed an increase of both globe protrusions and IPD/ICD. Among all groups, ICV and WBV were reduced in the severe group.
Conclusion
This morpho-volumetric study provides new insights in understanding the craniofacial changes occurring in infants at different severity of trigonocephaly. The increase of globe protrusions and the reduction of supratentorial volumes found in the severe group reflect the severity of trigonocephaly; these findings might have a clinical and surgical relevance.
... Although these studies tend to factor in surgical technique as a variable, they did not always control for the initial severity of the condition and the magnitude of surgical correction. Even when the severity was measured, the metrics used were simplistic and did not represent the full spectrum of disorder (Heller et al. 2008;Dvoracek et al. 2015;McEwan et al. 2016;Fearon et al. 2017). One study that used the facial appearance of children with CS who underwent surgery found that lay-people did not perceive them as 'normal' compared with unaffected children, even after surgery (Collett et al. 2013). ...
Sagittal craniosynostosis (SCS), the most common type of premature perinatal cranial suture fusion, results in abnormal head shape that requires extensive surgery to correct. It is important to find objective and repeatable measures of severity and surgical outcome to examine the effect of timing and technique on different SCS surgeries. The purpose of this study was to develop statistical models of infant (0–6 months old) skull growth in both normative and SCS subjects (prior to surgery). Our goal was to apply these models to the assessment of differences between these two groups in overall post‐natal growth patterns and sutural growth rates as a first step to develop methods for predictive models of surgical outcome. We identified 81 patients with isolated, non‐syndromic SCS from Seattle Children's Craniofacial Center patient database who had a preoperative CT exam before the age of 6 months. As a control group, we identified 117 CT exams without any craniofacial abnormalities or bone fractures in the same age group. We first created population‐level templates from the CT images of the SCS and normal groups. All CT images from both groups, as well as the canonical templates of both cohorts, were annotated with anatomical landmarks, which were used in a growth model that predicted the locations of these landmarks at a given age based on each population. Using the template images and the landmark positions predicted by the growth models, we created 3D meshes for each week of age up to 6 months for both populations. To analyze the growth patterns at the suture sites, we annotated both templates with additional semi‐landmarks equally spaced along the metopic, coronal, sagittal and lambdoidal cranial sutures. By transferring these semi‐landmarks to meshes produced from the growth model, we measured the displacement of the bone borders and suture closure rates. We found that the growth at the metopic and coronal sutures were more rapid in the SCS cohort than in the normal cohort. The antero‐posterior displacement of the semi‐landmarks also indicated a more rapid growth in the sagittal plane in the SCS model than in the normal model. Statistical templates and geometric morphometrics are promising tools for understanding the growth patterns in normal and synostotic populations and to produce objective and reproducible measurements of severity and outcome. Our study is the first of its kind to quantify the bone growth for the first 6 months of life in both normal and sagittal synostosis patients. Normal growth vs. growth in sagittal craniosynostosis. (A) Mediolateral displacement: positive values indicate medial movement (can be interpreted as suture closure) and negative values indicate lateral movement (mostly associated with the overall growth of the skull). (B) Superoinferior displacement: positive values indicate a superior movement. (C) Anteroposterior displacement: positive values indicate anterior, and negative values indicate posterior movement with respect to basion.
... In scientific papers the evaluation of the skull of infants and young children with premature atresia of cranial sutures typically refers to the cranial index (CI), brain volume, and the measurements of the jaw or of the skull base [15,17,[22][23][24][25]. The considerable majority of the articles evaluate these measurements in children with syndromic craniosynostosis. ...
Craniosynostosis is caused by premature fusion of one or more cranial sutures, restricting skull, brain and face growth. Nonsyndromic craniosynostosis could disturb the proportions of face. Although morphometric diameters of nasal cavity in healthy children are already known, they have not been established yet in children with nonsyndromic craniosynostosis. The aim our study was to check whether diameters of bone structures of nasal cavity in children with nonsyndromic craniosynostosis measured in CT are within normal range. 249 children aged 0–36 months (96 with clinical diagnosis of nonsyndromic craniosynostosis and 153 in control group) were included into the study. The following diameters were measured on head CT scans: anterior bony width (ABW), bony choanal aperture width (BCAW), right and left posterior bony width (between bone sidewall and nasal cavity septum—RPBW and LPBW). The study group has been divided into 4 categories, depending on child’s age. The dimensions measured between bone structures of nasal cavity were statistically significantly lower in comparison to the control group. They did not depend on the sex for ABW, nor on age in groups 7–12 months and < 2 years for BCAW, RPBW and LPBW. The measured dimensions increased with age. In children with nonsyndromic craniosynostosis the diameter of pyriform aperture and bony choanal aperture were lower than in controls, what may be described as fronto-orbital anomalies. Morphometric measurements of anthropometric indicators on CT scans could be used as standards in the clinical identification of craniosynostosis type and may help in planning surgical procedures, particularly in the facial skeleton in children.
Objective:
To systematically review the published comparative aesthetic outcomes, and its determinants, for craniosynostoses surgically treated by minimally-invasive cranial procedures and open cranial vault remodeling (CVR).
Design:
PRISMA-compliant systematic review.
Setting:
Not-applicable.
Patients/participants:
Articles were included if they compared spring cranioplasty, strip minimally-invasive craniectomy or CVR for outcomes related to aesthetics or head shape. Forty-two studies were included, comprising 2402 patients.
Interventions:
None.
Main outcome measure(s):
The craniometric and PROM used to determine surgical outcomes.
Results:
Twenty-five studies (59%) evaluated sagittal craniosynostosis, with metopic (7;17%) and unicoronal (4;10%) the next most prevalent. Thirty-eight studies (90%) included CVR, 24 (57%) included strip craniectomy with helmeting, 9 (22%) included strip craniectomy without helmeting, 11 (26%) included spring cranioplasty, and 3 (7%) included vault distraction. A majority of studies only used 1 (43%) or 2 (14%) craniometric measures to compare techniques. In sagittal synostosis, 13 (59%) studies showed no difference in craniometric outcomes, 5 (23%) showed better results with CVR, 3 (14%) with strip craniectomy, and 1 (5%) with springs. In studies describing other synostoses, 10/14 (71%) were equivocal. Subjective outcome measures followed similar trends. Meta-analysis shows no significant difference in cranial index (CI) outcomes between CVR and less invasive procedures in patients with sagittal synostosis.
Conclusions:
There is no difference in CI outcomes between CVR and less invasive procedures. The majority of literature comparing craniometric and aesthetic outcomes between CVR and less invasive procedures shows equivocal results for sagittal synostosis. However, the heterogeneity of data for other craniosynostoses did not allow meta-analysis.
OBJECTIVE
Quantitative measurements of trigonocephaly can be used to characterize and track this phenotype, which is associated with metopic craniosynostosis. Traditionally, trigonocephaly metrics were extracted from CT scans; however, this method exposes patients to ionizing radiation. Three-dimensional optical scans are another option but are not routinely available in most outpatient settings. Recently, the authors developed semiautomated artificial intelligence algorithms that extract craniometric data from orthogonal 2D photographs. Although 2D photographs are safe, inexpensive, and straightforward to obtain, the accuracy of photograph-based craniometrics in comparison to CT and 3D optical scan correlates has not been established. In this study the authors compared the classification power of 2D photograph–based metrics of trigonocephaly with four CT-based metrics and one 3D optical scan–based metric in a heterogeneous series of patients who presented to an outpatient craniofacial clinic.
METHODS
In this study the authors performed retrospective craniometric analyses of patient 2D photographs, 3D optical scans, and CT scans. Imaging-derived craniometrics include the 2D photograph–based anterior arc angle (AAA 2D-photo ), anterior-posterior ratio (APR 2D-photo ), and anterior-middle ratio (AMR 2D-photo ); the CT-based anterior arc angle (AAA CT ), metopic index (MI CT ), endocranial-bifrontal angle (eBFA CT ), and interfrontal angle (IFA CT ); and the 3D optical scan–based anterior arc angle (AAA 3D-optical ). Receiver operating characteristics (ROCs) were used to identify craniometrics strongly descriptive of trigonocephaly. Interrater comparisons were made between paired trigonocephaly measurements obtained from photographs and either CT scans or 3D optical scans.
RESULTS
There were 13 photograph-based and CT-based pairs and 22 paired measurements from 2D photographs and 3D optical scans. AAA displayed the strongest classification capacity across all three imaging modalities. Significant agreement was observed between AAA CT and AAA 2D-photo (intraclass correlation coefficient [ICC] = 0.68 [95% CI 0.24–0.89], p = 0.0035), and AAA 3D-optical and AAA 2D-photo (ICC = 0.70 [95% CI 0.41–0.87], p < 0.0001). There was no significant correlation between APR 2D-photo or AMR 2D-photo and conventional CT-based metrics describing longitudinal width ratios (MI CT ).
CONCLUSIONS
Photograph-based craniometrics are powerful tools that can be used to quantify the severity of trigonocephaly and exhibit high concordance with standard measurements derived from CT scans and 3D optical scans. The authors developed and freely share a research-use application to calculate trigonocephaly metrics from 2D photographs. Given the availability of digital photography, lack of ionizing radiation, and low cost of photograph-based craniometric derivation, this technique may be useful to supplement routine ambulatory care and objectively track outcomes following treatment.
Early endoscopic-assisted correction of unicoronal and metopic synostosis is an excellent, safe, cost-effective, and highly effective option for affected patients. Although open calvarial remodeling has a place in the armamentarium of the craniofacial team, the skull base changes seen in endoscopic-assisted techniques are unparalleled. The procedures are associated with low morbidity and no mortality. There is minimal blood loss, decreased operating time, significantly reduced blood transfusion rates, decreased hospitalization length, decreased cost, and less pain and swelling. Early diagnosis and referral for surgical evaluation are critical to obtaining these results.
Objective:
To present and compare outcomes and complications of conventional open reconstruction and minimally invasive correction of metopic synostosis in patients who underwent treatment of trigonocephaly in our center between 2015 and 2019.
Materials and methods:
The hospital database was searched for hospitalization and surgical information, as well as imaging of individuals with trigonocephaly. Post-operation evaluation of the patients was performed during the follow-up sessions. The radiological evaluation was based on brain CT scans taken two years following the operation.
Results:
Sixty-four patients (19 females, 45 males) had their trigonocephaly corrected surgically. Thirty-five patients (9 females, 26 males) had complete vault reconstruction surgery (CVR), while 29 patients (10 females, 19 males) had minimally invasive surgery (MIS). The post-operation cephalic width /intercoronal distance ratio and interpupillary distance/interfrontozygomatic distance ratio assessment showed no differences in the outcome of both groups (p-value > 0.05). Minimally invasive techniques resulted in less intraoperative bleeding, a shorter stay in the ICU and hospital, and a shorter surgery and anesthesia duration. (p-value < 0.05) CONCLUSION: Surgical treatment of trigonocephaly can result in a satisfactory correction of the deformity. MIS delivers a comparable result to CVR with less invasiveness and hospitalization and can be considered a reasonable option for patients in their early months of life. Patients must, however, undergo long-term cosmetic, behavioral, and developmental evaluations.
