A protocol of situation-dependent transfusion, erythropoietin and tranexamic acid reduces transfusion in fronto-orbital advancement for metopic and coronal craniosynostosis.

PURPOSE: Assess the effect of a protocol of preoperative erythropoietin (EPO) and ferrous sulfate in addition to perioperative tranexamic acid (TXA) on blood transfusions in patients with coronal or metopic craniosynostosis undergoing cranial vault remodeling (CVR) with fronto-orbital advancement (FOA). METHODS: Retrospective review of all coronal and metopic craniosynostosis patients undergoing CVR and FOA from March 2010 to June 2019 was performed. Before 2014 ("Control group"), all patients received blood transfusion at the start of surgery. In 2014, a protocol of preoperative EPO and ferrous sulfate with perioperative TXA and non-automatic transfusion was instituted ("Study group"). Patient demographics and anthropometrics, perioperative hemoglobin (Hb) levels, and transfusion details were collected and compared. RESULTS: Thirty-six patients met inclusion criteria. Twenty-one patients were in the control group, and 15 in the Study group. Nineteen patients had metopic synostosis, 11 had unicoronal synostosis, and 6 had bicoronal synostosis. There were no significant differences between groups in demographics, operative time, intraoperative crystalloid volume, craniofacial syndromes, or sutures affected. The Study group had higher preoperative Hb (13.9 ± 1.0 vs. 12.6 ± 0.8 g/dL, p < 0.001), lower intraoperative Hb nadir (7.4 ± 1.8 vs. 9.2 ± 1.2 g/dL) lower intraoperative transfusion rate (66.7% vs. 100%, p = 0.008), lower postoperative transfusion rate (0% vs 28.6%, p = 0.03), and exposure to fewer unique units of packed red blood cells (0.7 ± 0.6 vs. 1.5 ± 0.9 units). CONCLUSION: Our protocol resulted in decreased transfusion needs. These results add valuable information to the growing body of work on transfusion reduction in craniosynostosis surgery.

Quantitative and qualitative comparison of morphometric outcomes after endoscopic and conventional correction of sagittal and metopic craniosynostosis versus control groups.

OBJECTIVE: Surgical correction for sagittal and metopic craniosynostosis (SCS and MCS) aims to alter the abnormal cranial shape to resemble that of the normal population. The achieved correction can be assessed by morphometric parameters. The purpose of the presented study was to compare craniometric parameters of control groups to those same parameters after endoscopic and conventional (open) correction. METHODS: The authors identified 4 groups of children undergoing surgical treatment for either SCS or MCS, with either endoscopic (SCS, n = 17; MCS, n = 16) or conventional (SCS, n = 29; MCS, n = 18) correction. In addition, normal control groups of nonaffected children who were 6 (n = 30) and 24 (n = 18) months old were evaluated. For all groups, several craniometric indices calculated from 3D photographs were compared for quantitative analysis. For qualitative comparison, averages of all 3D photographs were generated for all groups and superimposed to visualize relative changes. RESULTS: For children with SCS, the cephalic index and coronal circumference index significantly differed preoperatively from those of the 6-month normal controls. The respective postoperative values were similar to those of the 24-month normal controls after both endoscopic and conventional correction. Similarly, for children with MCS, indices for circumference and diagonal dimension that were significantly different preoperatively became nonsignificantly different from those of 24-month normal controls after both endoscopic and conventional correction. The qualitative evaluation of superimposed average 3D head shapes confirmed changes toward normal controls after both treatment modalities for SCS and MCS. However, in SCS, the volume gain, especially in the biparietal area, was more noticeable after endoscopic correction, while in MCS, relative volume gain of the bilateral forehead was more pronounced after conventional correction. The average 3D head shapes matched more homogeneously with the average of normal controls after endoscopic correction for SCS and after conventional correction for MCS. CONCLUSIONS: This quantitative analysis confirms that the performed surgical techniques of endoscopic and conventional correction of SCS and MCS alter the head shape toward those of normal controls. However, in a qualitative evaluation, the average head shape after endoscopic technique for SCS and conventional correction for MCS appears to be closer to that of normal controls than after the alternative technique. This study reports on morphometric outcomes after craniosynostosis correction. Only an assessment of the whole multiplicity of outcome parameters based on multicenter data acquisition will allow conclusions of superiority of one surgical technique.

Statistical shape analyses of trigonocephaly patients.

PURPOSE: Surgery is the first treatment option for patients with metopic craniosynostosis. Fronto-orbital advancement is the preferred method for correction of isolated trigonocephaly, but it is hard to understand whether surgery has been successful mainly in an early period. We aim to investigate the shape differences in the head shapes of trigonocephaly patients compared between preoperative and postoperative term. METHODS: Cranial shape data were collected from the two-dimensional digital images. The Generalized Procrustes analysis was used to obtain mean shapes of the preoperative and postoperative term. The shape deformation of the frontal calvarium from preoperative to the postoperative term was evaluated using the thin-plate spline (TPS) method. RESULTS: There was significant cranial shape difference between preoperative and postoperative term. The high-level deformations for preoperative to postoperative term determined seen in TPS graphic. Highest deformation was observed at the bifrontal dimension especially at nasion and posterior edge of the forehead. CONCLUSIONS: In this study, we showed that the shape difference and structural deformation of the calvarium were correlated with the metopic craniosynostosis. The present study also shows that preoperative and postoperative head shapes of patients with trigonocephaly can be compared using the landmark-based geometrical morphometric method by taking into consideration the topographic distribution.

Orbito-facial dysmorphology in patients with different degrees of trigonocephaly severity: quantitative morpho-volumetric analysis in infants with non-syndromic metopic craniosynostosis.

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.

Surgical Correction of Metopic Craniosynostosis: A 3-D Photogrammetric Analysis of Cranial Vault Outcomes.

OBJECTIVE: To evaluate 3-dimensional (3-D) photogrammetry as a tool for assessing the postoperative head shape of patients who had undergone cranial vault remodeling for metopic synostosis. DESIGN: We prospectively analyzed images of patients with metopic craniosynostosis who had undergone anterior cranial vault remodeling and age-matched controls. To ensure standardized facial orientation, each 3-D image was positioned to "best fit" the preoperative face by aligning 6 soft tissue landmarks. Forehead measurements were taken from a standardized position behind the surface of the face to landmarks placed in a ray configuration across the forehead. SETTING: Academic teaching hospital. PATIENTS, PARTICIPANTS: Thirteen pediatric patients with metopic craniosynostosis who had undergone anterior cranial vault remodeling and age-matched controls. INTERVENTIONS: Images were taken preoperatively, immediately postoperatively, and over 1-year postoperatively. MAIN OUTCOME MEASURES: Forehead contours preoperatively and postoperatively, with statistics performed using a multivariate analysis of variance shape analysis. RESULTS: Mean postoperative follow-up was 1.8 (0.6) years. The average distance from the origin to forehead landmarks was 55.1 (3.4) mm preoperatively, 59.3 (0.7) mm immediate postoperatively, 59.1 (1.0) mm 1-year postoperatively, and 59.4 (0.6) mm in controls. Postoperative metopic forehead contours varied significantly from preoperative contours ( P < .01), while there was no statistical difference between the 2 postoperative time points ( P = .70). One-year postoperative patients were not significantly different from their age-matched controls ( P > .99). CONCLUSIONS: Preoperative metopic forehead contours varied significantly from postoperative contours. Cranial reconstructions approximated the foreheads of normal controls, and reconstructions were stable at more than 1-year follow-up.

Incidence of Amblyopia and Its Risk Factors in Children With Isolated Metopic Craniosynostosis.

OBJECTIVE: Ophthalmic abnormalities in children with syndromic craniosynostosis have been reported previously, and referral of these children to a pediatric ophthalmologist is recommended. However, it is not as clear whether a child with nonsyndromic synostosis needs to be referred to a pediatric ophthalmologist. The aim of this study is to report the incidence of amblyopia and its risk factors in children with isolated metopic craniosynostosis. DESIGN: An institutional review board-approved, retrospective review was performed on 91 children diagnosed with isolated metopic craniosynostosis. Ophthalmologic records were reviewed for diagnoses of amblyopia, strabismus, and refractive error. RESULTS: Of the 91 children, 19 (20.9%) had astigmatism, eight (8.8%) had amblyopia, eight (8.8%) had strabismus, five had myopia (5.5%), five had hyperopia (5.5%), and five had anisometropia (5.5%). The incidence of amblyopia and its risk factors found in our study are higher than the rate found in the clinically normal pediatric population. CONCLUSIONS: In our patient population, children with isolated metopic craniosynostosis demonstrate an increased rate of amblyopia and its risk factors. Amblyopia is best treated early in life to achieve a successful outcome. A referral to a pediatric ophthalmologist for a formal eye exam and potential treatment is therefore recommended for children with isolated metopic craniosynostosis.

Incidence of Amblyopia and Its Risk Factors in Children With Isolated Metopic Craniosynostosis.

OBJECTIVE: Ophthalmic abnormalities in children with syndromic craniosynostosis have been reported previously, and referral of these children to a pediatric ophthalmologist is recommended. However, it is not as clear whether a child with nonsyndromic synostosis needs to be referred to a pediatric ophthalmologist. The aim of this study is to report the incidence of amblyopia and its risk factors in children with isolated metopic craniosynostosis. DESIGN: An institutional review board-approved, retrospective review was performed on 91 children diagnosed with isolated metopic craniosynostosis. Ophthalmologic records were reviewed for diagnoses of amblyopia, strabismus, and refractive error. RESULTS: Of the 91 children, 19 (20.9%) had astigmatism, eight (8.8 %) had amblyopia, eight (8.8%) had strabismus, five had myopia (5.5%), five had hyperopia (5.5%), and five had anisometropia (5.5%). The incidence of amblyopia and its risk factors found in our study are higher than the rate found in the clinically normal pediatric population. CONCLUSIONS: In our patient population, children with isolated metopic craniosynostosis demonstrate an increased rate of amblyopia and its risk factors. Amblyopia is best treated early in life to achieve a successful outcome. A referral to a pediatric ophthalmologist for a formal eye exam and potential treatment is therefore recommended for children with isolated metopic craniosynostosis.

A critical eye on metopic craniosynostosis: A retrospective study assessing orbital dysmorphology as a marker of disease.

This investigation sought to ascertain whether orbital morphology could predict genuine metopic craniosynostosis (MCS). The study retrospectively analyzed preoperative three-dimensional computed tomography (3D-CT) scans of patients who underwent surgical correction for MCS. MCS severity was evaluated using the interfrontal angle (IFA). Orbital dysmorphology was assessed based on multiple angles, including supraorbital notches and nasion (SNS), infraorbital foramina and nasion (INI), zygomaticofrontal suture-supraorbital notch-dacryon (ZSD), and orbital long axis (OLA). Results were juxtaposed against age/gender-matched controls and individuals with non-synostotic metopic ridge (MR). The study included 177 patients: 68 MCS, 35 MR, and 74 control subjects. All orbital measurements exhibited significant differences across groups. IFA demonstrated a strong association with all orbital measurements, particularly SNS (B = 0.79, p < 0.001). SNS showed the highest area under the curve among orbital measurements (0.89). Using a 95% sensitivity threshold, the optimal diagnostic angle for SNS was 129.23° (specificity 54%, sensitivity 96%). These findings suggest a correlation between orbital dysmorphology and trigonocephaly severity. The observed dysmorphology manifested in a superomedially accentuated rotational pattern. Importantly, SNS angle predicted MCS, with an angle greater than 130° indicating <5% likelihood of MCS diagnosis. The simplicity of measuring SNS angle on any 3D-CT scan highlights its practical use for assisting with MCS diagnosis.

Metopic craniosynostosis: Dynamic cranioplasty for trigonocephaly versus fronto-orbital remodeling and advancement - A retrospective study.

The aim of this study was to compare a traditional fronto-orbital remodeling and advancement (FORA) with the dynamic cranioplasty for trigonocephaly (DCT). The authors analyzed patients who underwent surgery for trigonocephaly. Perioperative data were compared. Parents were asked to use a visual analog scale to evaluate the pre- and postoperative distance between the eyes, the forehead shape, and the global appearance of the face. A panel of observers was asked to grade pre- and postoperative photographs using a similar visual analog scale. Pre- and postoperative anthropometric data were collected and analyzed in a subset of the study population aged 9 years or older. The total sample size was 51 patients (DCT n = 39; FORA n = 12). Durations of surgery and anesthesia were shorter in the DCT group (115 vs 194 min, p = 0.001; 226 vs 289 min, p = 0.001). Patients in the DCT group received similar preoperative ratings to those in the FORA group, but significantly higher postoperative ratings by parents for all three questions. There were no significant differences in postoperative ratings by the panel or postoperative anthropometric data. DCT is safe and effective. It is preferred over FORA because it is associated with shorter durations of surgery and anesthesia, while providing higher degrees of parental satisfaction and similar aesthetic and anthropometric outcomes.

Is endoscope-assisted strip craniectomy the future of metopic suture craniosynostosis treatment? An 11-year experience with 62 patients.

OBJECTIVE: Endoscopic mini-invasive treatment for sporadic trigonocephaly is becoming a widely accepted surgical treatment. In most centers this treatment is performed in association with postoperative helmeting. The aim of the present study was to review and report the authors' 11-year experience of endoscope-assisted metopic suturectomy for treatment of 62 trigonocephaly patients without helmet use. METHODS: For this retrospective study, clinical data of 62 consecutive pediatric patients (age 3-8 months) were obtained from the data bank of the "Anna Meyer" Children Hospital. These patients had been diagnosed with trigonocephaly (type II and III) and undergone surgery performed with a mini-invasive endoscopic technique during the period from January 2011 to January 2022. No helmet was used postoperatively in these patients, and they were evaluated through craniometric measurements, pre-/postoperative photographs, and parents' impressions, as well as thorough clinical examinations during follow-up appointments. RESULTS: The mean patient follow-up period was 6 ± 1.3 years. The female/male ratio was 1:2; 52% of the patients presented with type II trigonocephaly and the remaining patients with type III. The mean age at surgery was 153 ± 44 days (5 ± 1.5 months, range 3-8 months). In 92% of the patients the surgical outcome was defined as good to excellent. However, 4 patients presented with an unsatisfactory outcome, including 1 patient with a CSF collection requiring surgical repair 2 months after the first surgery and 1 patient who developed infection of the surgical wound and needed a second surgery. In the latter patient the outcome was evaluated as satisfactory, and no sequelae regarding the infection were encountered during follow-up. CONCLUSIONS: According to the authors' experience, endoscopic metopic suturectomy alone, without the use of a helmet, is a valid surgical option for trigonocephaly treatment, and its application can be considered in patients of older age groups (up to 8 months). Thus, in the right patient selection context, this technique represents the treatment of choice.

Impact of age at endoscopic metopic synostosis repair on anthropometric outcomes.

OBJECTIVE: Endoscopic strip craniectomy for metopic craniosynostosis relies on rapid growth and postoperative helmeting for correction. Endoscopic repair is generally performed before patients reach 4 months of age, and outcomes in older patients have yet to be quantified. Here, the authors examined a cohort of patients treated with endoscopic repair before or after 4 months of age to determine aesthetic outcomes of delayed repairs. METHODS: Data from eligible patients were retrospectively assessed and aggregated in a dedicated metopic synostosis database. Inclusion criteria were radiographically confirmed metopic synostosis and endoscopic treatment. Patients were dichotomized into two groups: those younger than 4 months and those 4 months or older at the time of repair. The frontal width and interfrontal divergence angle (IFDA) were measured on reconstructed CT images. These measurements, alongside operative time, estimated blood loss, and transfusion rates, were compared between groups using the Student t-test or chi-square test. RESULTS: The study population comprised 28 patients treated before 4 months of age and 8 patients treated at 4-6 months of age. Patient sex and perioperative complications did not differ by age group. Older age at repair was not significantly associated with 1-year postoperative IFDA (140° ± 4.2° vs 142° ± 5.0°, p = 0.28) or frontal width (84 ± 5.2 vs 83 ± 4.4 mm, p = 0.47). CONCLUSIONS: One-year postoperative IFDA and frontal width do not differ significantly between patients treated before and after 4 months of age. Further study with longer follow-up is necessary to confirm the longevity of these results at skeletal maturity.

A new method for quantification of frontal retrusion and complex skull shape in metopic craniosynostosis: a pilot study of a new outcome measure for endoscopic strip craniectomy.

OBJECTIVE: The objective of this study was to propose a new skull outline-based method to objectively quantify complex 3D skull shapes and frontal and supraorbital retrusion in metopic craniosynostosis using 3D photogrammetry. METHODS: A standard section from 3D photogrammetry, which represents the trigonocephalic shape, was used in this study. From the midpoint of the area of this section, half diagonals were calculated to the skull outline at 5° increments in the anterior half of the head. These half diagonals were used to create a sinusoidal curve, and the area under the sinusoidal curve (AUC) was used to represent the mathematical expression of the trigonocephalic head shape. The AUC from 0° to 180° (90° from the midline to each side) was calculated and is referred to as AUC0→180. The AUC from 60° to 120° (30° from the midline to each side) was also calculated and is referred to as AUC60→120. A total of 24 patients who underwent endoscopic strip craniectomy and 13 age- and sex-matched controls were included in the study. The AUC values obtained in patients at different time points and controls were analyzed. RESULTS: The mean preoperative AUC60→120 and AUC0→180 in the patients were significantly lower than those in control individuals. The increase in both AUC60→120 and AUC0→180 values is statistically significant at the discontinuation of helmet therapy and at final follow-up. Receiver operating characteristic curve analysis indicated that AUC60→120 is a more accurate classifier than AUC0→180. CONCLUSIONS: The proposed method objectively quantifies complex head shape and frontal retrusion in patients with metopic craniosynostosis and provides a quantitative measure for follow-up after surgical treatment. It avoids ionizing radiation exposure.

Craniometric Measurements and Surgical Outcomes in Trigonocephaly Patients Who Underwent Surgical Treatment.

Objective The aim of this study was to discuss the results of craniometric measurements and surgical treatments in patients operated for isolated trigonocephaly (TC) in light of the current literature. Methods A total of 18 cases who underwent surgery for isolated TC were included in the study. Age, gender, family history, follow-up time, complications, duration of surgery, surgical blood loss, and amount of blood replacement in these patients were recorded. Craniometric measurements such as metopic angle (MA), cephalic index (CI), interparietal distance (IPD), intercoronal distance (ICD), and their ratio to each other were evaluated according to pre-and postoperative parameters. Photographs of the patients were taken before and after the operation. The Whitaker classification and Kampf "aesthetic outcome staging" were used in the evaluations. Results The mean MA values after the operation increased to reach above 147 degrees in all cases. The average CI did not change. ICD measurement averages increased significantly. The average IPD/ICD ratio decreased due to the increase in ICD and the enlargement of the anterior fossa after the operation. According to the Whitaker classification and Kampf "aesthetic outcome staging" scale, 17 of our cases were at stage I, rated as perfect, and one was at stage II, rated as good. Conclusion Surgery performed at the appropriate time for TC yields cosmetically satisfactory results. Since potential neurological and cognitive morbidities occur mostly in school-age patients, long-term follow-up of the cases is required. Performing craniometric measurements enables patients to be evaluated with objective and measurable numerical data.

[Neuropsychological consequences of craniosynostosis: Non-syndromic scaphocephaly]. / Le retentissement neuropsychologique de la craniosténose : cas de la scaphocéphalie non syndromique.

BACKGROUND: Scaphocephaly increases the rate of some modifications of cognitive and mood profile in a manner that remains to be elucidated. OBJECTIVE: We aimed to describe the impact of scaphocephaly on neuropsychological profile and more particularly on the executive functions. PATIENTS AND METHODS: An experimental group of 19 children older than 5 years, operated on for scaphocephaly, was compared with a control group of 10 children operated on for trigonocephaly, using IQ tasks, attention tasks and mood scales. A group of 6 children from 2 to 4 years old, operated on for scaphocephaly, and a group of 6 children with non-operated scaphocephaly are also described. RESULTS: Both the experimental group and the control group showed unchanged IQ, whereas attention deficit and anxiety disorder were more frequent in the experimental group. Cognitive profiles differed between groups, with a higher rate of impaired inhibitory control of visual processing in the scaphocephaly group, contrasting with a higher rate of impaired auditory verbal working memory in the trigonocephaly group. Comparable profiles were also found in groups of younger or non-operated children with scaphocephaly. CONCLUSIONS: Many children with scaphocephaly must cope with a specific neuropsychological profile throughout development. This study suggests the interest for these children and their families of specific follow-up in reference centers.

Current Controversies in Metopic Suture Craniosynostosis.

Metopic craniosynostosis is being reported with an increasing incidence and is now the second most common type of isolated suture craniosynostosis. Numerous areas of controversy exist in the work-up and management, including defining the diagnosis in the less severe phenotype, the association with neurodevelopmental delay, the impact of surgical treatment, and the applicability of various techniques and their timing on outcomes.

Metopic "ridge" vs. "craniosynostosis": Quantifying severity with 3D curvature analysis.

BACKGROUND: There has been an increased incidence of surgical treatment for metopic craniosynostosis (MCS) over the past decade. MCS presents as a wide spectrum, ranging from severe trigonocephaly on one end to an isolated metopic ridge on the other. Current surgical diagnosis relies upon subjective clinical assessment of patients' cranial shape, which is often combined with impressions from radiologic imaging. In our study, we have developed a semi-automated methodology using three-dimensional curvature analysis to rigorously separate the phenotypes along the spectrum. METHODS: Three clinically distinct groups of patients with CT images were obtained: 1) Normal subjects without any deformity; 2) "Benign" metopic ridge (BMR) without classic trigonocephaly; 3) Severe trigonocephaly with MCS. CT scans were converted into three-dimensional skull surface images, and curvature analysis was performed using two user-defined regions of interest (mid-forehead and lateral orbit). RESULTS: Three-dimensional curvature analysis of mid-forehead strip, and right/left lateral orbital areas was performed in thirty patients. The difference in average mean curvature between true MCS and BMR was 10.5 m(-1) and -18.6 m(-1) for mid-forehead strip and right/left lateral orbital rim, respectively. The average mean curvatures of mid-forehead strip and right/left lateral orbit areas among the three groups were significantly different (p < 0.0001). K-means clustering reliably classified patients into different severity groups based on average mean curvature of the two regions of interest. CONCLUSIONS: The described methods are effective in classifying severity of orbitofrontal deformity in the spectrum of MCS, which can aid providers in identifying an appropriate threshold for surgical treatments of MCS.