Skeletal expansion via craniofacial distraction osteogenesis technique in syndromic craniosynostosis: impact on ophthalmic parameters.

BACKGROUND: This study aims to compare the changes in ophthalmic parameters among syndromic craniosynostosis patients who underwent craniofacial skeletal expansion procedures via distraction osteogenesis (DO). METHOD: A retrospective study was conducted involving syndromic craniosynostosis patients who underwent surgical expansion via the DO technique from the year 2012 to March 2022. Changes in six parameters which consist of visual acuity, refractive error, optic disc health, intraocular pressure, degree of proptosis and orbital volume were measured objectively pre and post-surgery. For categorical parameters, the Chi-square cross-tab test was done. Paired sample T-test was used for normally distributed variables. Wilcoxon signed-rank test was used for non-normally distributed data. RESULTS: Visual impairment was present in 21.4% of eyes before surgery and increased to 28.5% post-surgery. Three patients had changes of refractive error post-surgery with one developed hypermetropia, another developed anisometropia and the last had improvement to no refractive error. Two patients had optic disc swelling which was resolved post-surgery. Intraocular pressure changes were inconsistent post-surgery. All patients achieved a significant reduction in the degree of proptosis post-surgery. Orbital volume calculation using computed tomography (CT) scans shows a significant increase in volume post-surgery for all patients. CONCLUSION: Our study shows a significant increase in orbital volume post-surgery with a reduction in the degree of proptosis. Optic disc and nerve health improved after the surgery. Changes in terms of visual acuity, refractive error and IOP were inconsistent after the surgical intervention.

Automated three-dimensional analysis of facial asymmetry in patients with syndromic coronal synostosis: A retrospective study.

Craniosynostosis, characterized by premature fusion of one or more cranial sutures, results in a distorted skull shape. Only three studies have assessed facial asymmetry manually in unicoronal synostosis patients. It is therefore important to understand how uni- and bicoronal synostosis affect facial asymmetry with a minimum risk of human bias. An automated algorithm was developed to quantify facial asymmetry from three-dimensional images, generating a mean facial asymmetry (MFA) value in millimeters to reflect the degree of asymmetry. The framework was applied to analyze postoperative 3D images of syndromic patients (N = 35) diagnosed with Muenke syndrome, Saethre-Chotzen syndrome, and TCF12-related craniosynostosis with respect to MFA values from a healthy control group (N = 89). Patients demonstrated substantially higher MFA values than controls: Muenke syndrome (unicoronal 1.74 ± 0.40 mm, bicoronal 0.77 ± 0.21 mm), Saethre-Chotzen syndrome (unicoronal 1.15 ± 0.20 mm, bicoronal 0.69 ± 0.16 mm), and TCF12-related craniosynostosis (unicoronal 1.40 ± 0.51 mm, bicoronal 0.66 ± 0.05 mm), compared with controls (0.49 ± 0.12 mm). Longitudinal analysis identified an increasing MFA trend in unicoronal synostosis patients. Our study revealed higher MFA in syndromic patients with uni- and bicoronal synostosis compared with controls, with the most pronounced MFA in Muenke syndrome patients with unilateral synostosis. Bicoronal synostosis patients demonstrated higher facial asymmetry than expected given the condition's symmetrical presentation.

Clomiphene Citrate and Enclomiphene Hydrochloride Exposure Is Associated With Interfrontal Suture Fusion in Zebrafish.

Background: The last several decades have witnessed an increase in metopic craniosynostosis incidence. Population-based studies suggest that pharmacological exposure in utero may be responsible. This study examined effects of the fertility drug clomiphene citrate (CC) on calvarial development in an established model for craniofacial development, the zebrafish Danio rerio. Results: Zebrafish larvae were exposed to clomiphene citrate or its isomer enclomiphene for five days at key points during calvarial development. Larvae were then raised to adulthood in normal rearing water. Zebrafish were analyzed using whole-mount skeletal staining. We observed differential effects on survivability, growth and suture formation depending on the treatment. Treatments with CC or enclomiphene at 5.5 mm SL led to increased fusion of the interfrontal suture (p < .01) compared to controls. Conclusions: Exposure to fertility drugs appears to affect development of the cranial vault, specifically the interfrontal suture, in zebrafish. Further research is required to identify the signaling mechanisms at play. This work suggests that fertility drug treatment may contribute to the increased incidence of metopic craniosynostosis observed globally.

Historique : L'incidence de craniosynostoses métopiques a augmenté ces dernières décennies. D'après les études en population, l'exposition aux médicaments in utero pourrait en être responsable. La présente étude traite des effets d'un agent ovulatoire, le citrate de clomifène (CC), sur le développement de la voûte crânienne d'un modèle établi de développement crâniofacial, le poisson-zèbre Danio rerio. Résultats : Les chercheurs ont exposé des larves de poisson-zèbre au citrate de clomifène ou à son isomère, l'enclomiphène, sur une période de cinq jours à des moments clés du développement de la voûte crânienne. Ils les ont ensuite élevés jusqu'à l'âge adulte dans de l'eau normale. Ils ont analysé les poissons-zèbres par coloration du squelette entier et observé des effets différentiels sur la capacité de survie, la croissance et la formation des sutures en fonction du traitement. Les traitements au CC ou à l'enclomiphène d'une longueur standard de 5,5 mm ont entraîné une fusion accrue de la suture métopique (p < 0,01) par rapport aux sujets témoins. Conclusions : L'exposition aux agents ovulatoires semble influer sur le développement de la voûte crânienne, notamment la suture métopique, chez le poisson-zèbre. D'autres recherches devront être réalisées pour déterminer les mécanismes de signalisation en jeu. Selon la présente étude, les agents ovulatoires peuvent contribuer à l'incidence accrue de craniosynostose métopique sur la scène mondiale.

The Use of Artificial Intelligence for the Classification of Craniofacial Deformities.

Positional cranial deformities are a common finding in toddlers, yet differentiation from craniosynostosis can be challenging. The aim of this study was to train convolutional neural networks (CNNs) to classify craniofacial deformities based on 2D images generated using photogrammetry as a radiation-free imaging technique. A total of 487 patients with photogrammetry scans were included in this retrospective cohort study: children with craniosynostosis (n = 227), positional deformities (n = 206), and healthy children (n = 54). Three two-dimensional images were extracted from each photogrammetry scan. The datasets were divided into training, validation, and test sets. During the training, fine-tuned ResNet-152s were utilized. The performance was quantified using tenfold cross-validation. For the detection of craniosynostosis, sensitivity was at 0.94 with a specificity of 0.85. Regarding the differentiation of the five existing classes (trigonocephaly, scaphocephaly, positional plagiocephaly left, positional plagiocephaly right, and healthy), sensitivity ranged from 0.45 (positional plagiocephaly left) to 0.95 (scaphocephaly) and specificity ranged from 0.87 (positional plagiocephaly right) to 0.97 (scaphocephaly). We present a CNN-based approach to classify craniofacial deformities on two-dimensional images with promising results. A larger dataset would be required to identify rarer forms of craniosynostosis as well. The chosen 2D approach enables future applications for digital cameras or smartphones.

Radiomorphologic profiles of nonsyndromic sagittal craniosynostosis.

PURPOSE: 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 of cranial dysmorphology. The goal of this study was to depict the most common combinations of radiomorphologic characteristics of NSC and to separate groups where the patients were morphologically similar to one another and at the same time significantly different from others. METHODS: The study was conducted on anonymized thin-cut CT scans of 131 children with NSC aged 1-12 months (mean age 5.42 months). The type of cranial dysmorphology was assessed using four criteria: skull shape, pattern of sagittal suture fusion, morphologic features and cerebrospinal fluid (CSF) spaces alterations. After assigning the categories, an unsupervised k-modes clustering algorithm was applied to identify distinct patients clusters representing radiomorphologic profiles determined by investigated characteristics. RESULTS: Cluster analysis revealed three distinct radiomorphologic profiles including the most common combinations of features. The profiles were not influenced by sex nor age but were significantly determined by skull shape (V = 0.58, P < 0.0001), morphologic features (V = 0.50, P < 0.0001) and pattern of sagittal suture fusion (V = 0.47, P < 0.0001). CSF alterations did not significantly correlate with the profiles (P = 0.3585). CONCLUSION: NSC is a mosaic of radiologic and morphologic features. The internal diversity of NSC results in dissimilar groups of patients defined by unique combinations of radiomorphologic characteristics, from which the skull shape is the most differentiating factor. Radiomorphologic profiles support the idea of clinical trials targeted at more selective outcomes assessment.

Metopic ridge presenting to pediatric dermatology and vascular anomalies clinics.

Metopic ridge (MeR) is a midline osseous forehead prominence resulting from physiologic closure of the underlying metopic suture. This mass-like ridge can be mistaken for serious conditions such as a craniosynostosis or vascular anomaly, prompting concern and workup. We reviewed patients presenting for a forehead mass to Vascular Anomalies and Dermatology clinics and diagnosed with MeR to increase familiarity with this finding and to encourage MeR in the differential diagnosis of pediatric midline forehead masses.

Caracterización de pacientes operados de craneosinostosis no sindrómica. Villa Clara, 2018-2022

Fundamento la craneosinostosis es una de las patologías neuroquirúrgicas más frecuentes en la edad pediátrica. Los conocimientos sobre su manejo son de vital importancia para intervenir con el tratamiento de manera oportuna. Objetivo caracterizar los pacientes operados de craneosinostosis no sindrómica. Métodos se realizó un estudio descriptivo y de corte transversal, en el período enero/2018- diciembre/2022, con la totalidad de pacientes intervenidos de craneosinostosis en el Hospital Pediátrico José Luis Miranda, de Villa Clara (N=28). Las variables analizadas fueron: sexo, color de la piel, clasificación de la malformación según sutura afectada, síntomas y signos asociados a la malformación, edad al realizar la intervención quirúrgica, técnica quirúrgica y complicaciones. Resultados se observó predominio del sexo masculino y de pacientes con color blanco de la piel. La forma de presentación más frecuente fue la escafocefalia, y se asoció a síntomas como el retardo en el desarrollo psicomotor y estrabismo. Las técnicas quirúrgicas realizadas fueron de remodelado total de la bóveda craneal, que en la mayoría de los niños se realizó antes de los 23 meses. La complicación más frecuente fue la infección de la herida quirúrgica. Conclusiones la craneosinostosis es una patología neuroquirúrgica frecuente en el sexo masculino. La forma de presentación más frecuente es la escafocefalia y se puede asociar a síntomas y signos neurológicos. Las técnicas quirúrgicas realizadas para remodelado de la bóveda craneal se deben realizar antes del primer año de vida, con vistas a disminuir el índice de complicaciones.

Background craniosynostosis is one of the most frequent neurosurgical pathologies in the pediatric age. Knowledge about its management is of vital importance to intervene with treatment in a timely manner. Objective to characterize the patients operated on for non-syndromic craniosynostosis. Methods a descriptive and cross-sectional study was carried out, from January/2018 to December/2022, with all the patients operated on for craniosynostosis at the José Luis Miranda Pediatric Hospital, in Villa Clara (N=28). The analyzed variables were: sex, skin color, classification of the malformation according to the affected suture, symptoms and signs associated with the malformation, age at surgery, surgical technique, and complications. Results a predominance of the male sex and patients with white skin color was observed. The most frequent form of presentation was scaphocephaly, and it was associated with symptoms such as delayed psychomotor development and strabismus. The surgical techniques performed were total remodeling of the cranial vault, which in most children was performed before 23 months old. The most frequent complication was infection of the surgical wound. Conclusions craniosynostosis is a frequent neurosurgical pathology in the male sex. The most frequent form of presentation is scaphocephaly and it can be associated with neurological symptoms and signs. Surgical techniques performed for remodeling of the cranial vault must be performed before the first year of life, in order to reduce the complications rate.

FGF9-Associated Multiple Synostoses Syndrome Type 3 in a Multigenerational Family.

Multiple synostoses syndrome (OMIM: #186500, #610017, #612961, #617898) is a genetically heterogeneous group of autosomal dominant diseases characterized by abnormal bone unions. The joint fusions frequently involve the hands, feet, elbows or vertebrae. Pathogenic variants in FGF9 have been associated with multiple synostoses syndrome type 3 (SYNS3). So far, only five different missense variants in FGF9 that cause SYNS3 have been reported in 18 affected individuals. Unlike other multiple synostoses syndromes, conductive hearing loss has not been reported in SYNS3. In this report, we describe the clinical and selected radiological findings in a large multigenerational family with a novel missense variant in FGF9: c.430T>C, p.(Trp144Arg). We extend the phenotypic spectrum of SYNS3 by suggesting that cleft palate and conductive hearing loss are part of the syndrome and highlight the high degree of intrafamilial phenotypic variability. These findings should be considered when counseling affected individuals.

Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature.

BACKGROUND: Progressive familial intrahepatic cholestasis is a heterogeneous group of disorders, leading to intrahepatic cholestasis, with the possibility of chronic liver failure and biliary cirrhosis. Oligodontia is either the manifestation of a specific syndrome or is non-syndromic. To the best of our knowledge, this is the first case report of type 3 progressive familial intrahepatic cholestasis and concurrent oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition in the medical and dental literature. CASE PRESENTATION: We present the dental and medical histories and comprehensive dental management of a girl with type 3 progressive familial intrahepatic cholestasis and several dental anomalies, who was referred to a dental clinic due to severe dental caries and pain. CONCLUSION: Our findings suggest that PFIC with manifestations as oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition, might indicate an unknown syndrome; otherwise, the craniofacial anomalies are the manifestations of an independent disease coinciding with PFIC. Moreover, our case is a good example of the importance of timely medical and dental care in confining further health-related complications. The patient was able to ingest without any pain or discomfort after receiving proper dental management.

"Validation of Artificial Intelligence Severity Assessment in Metopic Craniosynostosis".

OBJECTIVE: Several severity metrics have been developed for metopic craniosynostosis, including a recent machine learning-derived algorithm. This study assessed the diagnostic concordance between machine learning and previously published severity indices. DESIGN: Preoperative computed tomography (CT) scans of patients who underwent surgical correction of metopic craniosynostosis were quantitatively analyzed for severity. Each scan was manually measured to derive manual severity scores and also received a scaled metopic severity score (MSS) assigned by the machine learning algorithm. Regression analysis was used to correlate manually captured measurements to MSS. ROC analysis was performed for each severity metric and were compared to how accurately they distinguished cases of metopic synostosis from controls. RESULTS: In total, 194 CT scans were analyzed, 167 with metopic synostosis and 27 controls. The mean scaled MSS for the patients with metopic was 6.18 ± 2.53 compared to 0.60 ± 1.25 for controls. Multivariable regression analyses yielded an R-square of 0.66, with significant manual measurements of endocranial bifrontal angle (EBA) (P = 0.023), posterior angle of the anterior cranial fossa (p < 0.001), temporal depression angle (P = 0.042), age (P < 0.001), biparietal distance (P < 0.001), interdacryon distance (P = 0.033), and orbital width (P < 0.001). ROC analysis demonstrated a high diagnostic value of the MSS (AUC = 0.96, P < 0.001), which was comparable to other validated indices including the adjusted EBA (AUC = 0.98), EBA (AUC = 0.97), and biparietal/bitemporal ratio (AUC = 0.95). CONCLUSIONS: The machine learning algorithm offers an objective assessment of morphologic severity that provides a reliable composite impression of severity. The generated score is comparable to other severity indices in ability to distinguish cases of metopic synostosis from controls.

A Simple and Descriptive Assessment of Morphology Based on the Horizontal Plane of the Pediatric Head and Creation of a Normative Database in Japanese Children 6 Years Old and under: Horizontal Vector Analysis.

We herein introduce horizontal vector analysis, a simple method for assessing cranial morphology based on measurement of the head's horizontal plane, and use this method to establish normal cranial morphology in Japanese children Computed tomography scans taken in 2010-2019 in healthy Japanese children aged ≤ 6 years. The two measurement planes were parallel to the orbitomeatal plane: namely, a plane passing through the dorsum sellae (DS) and the plane superior to that with the maximal area (Max plane). A protractor was used to circumferentially measure the lengths from the central point to the outer surface of the skull. A total of 487 images were extracted. The distances between the DS and Max planes were consistently almost 30 mm for each age group, so we fixed the Max plane as the plane 30 mm superior to the DS plane. Finally, we established datasets of normal values for each age group and sex. Using these norms, perioperative evaluation of various cranial deformities could be performed more easily and circumstantially.

Multisuture and Syndromic Craniosynostoses: Simplifying the Complex.

Most complex craniosynostoses are managed the same way as syndromic craniosynostoses (SCs), as these patients often experience similar problems regarding cognition and increased intracranial pressure (ICP). The evaluation and treatment plan for craniosynostoses is complex, and this, additionally, is complicated by the age at presentation. In this article, the authors review the complexity of SCs in the presentation and management. An algorithm is necessary for such multifaceted and multidimensional pathology as craniosynostoses. In most algorithms, posterior calvarial distraction is a consistent early option for complex craniosynostoses presenting early with raised ICP. Addressing the airway early is critical when significant airway issues are there. All other surgical interventions are tailored on the basis of presentation and age.

The foramen magnum in scaphocephaly.

PURPOSE: The foramen magnum (FM) presents various alterations in craniosynostoses, such as brachycephaly or Crouzon syndrome. However, to date, no study has been devoted to its morphology and morphometry in scaphocephaly, which is the most common of cranial deformities resulting from premature fusion of cranial sutures. METHODS: We assessed the morphology and morphometry of FM using preoperative thin-cut CT scans of 107 children with non-syndromic sagittal craniosynostosis aged 1-12 months (mean age 5.38 months). A series of sagittal and transverse dimensions were taken and the FM area was calculated in each case. Obtained data were compared to the age-matched control group of 101 normocephalic children. RESULTS: Dolichotrematous type of FM was dominant in the scaphocephaly group and observed in 63/107 cases (58.9%). The mean FM area in the scaphocephaly group was 519.64 mm2 and was significantly smaller compared to the control group (p = 0.0011). The transverse diameter and anterior sagittal diameter were also significantly smaller (p = 0.0112 and p = 0.0003, respectively). CONCLUSION: The area of FM in scaphocephaly is smaller compared to normal individuals. This is associated with a significant reduction of the width of FM in children with sagittal craniosynostosis. FM in scaphocephaly is larger than in other reported series of children with brachycephaly or Crouzon syndrome.

Accelerated cranial distraction protocol for one-piece fronto-orbital distraction osteogenesis without bandeau in patients with craniosynostosis.

This study aimed to compare the bony relapse ratios of standard and accelerated distraction protocols (ADP) in one-piece fronto-orbital distraction osteogenesis (DO). Patients with unilateral or bilateral coronal synostoses who underwent one-piece fronto-orbital DO were included. The accelerated cranial distraction protocol included a 3-day latency period and a distraction rate of 1-2 mm/day, followed by a 4-week consolidation period. Intracranial volume was measured using computed tomography (CT) before the surgery, at the end of the consolidation period, and 1 year after the removal of distractors. The intracranial volume changes and relapse ratios were calculated. This study included 32 patients; of these, 16 were included in each of the ADP and standard protocol (SP) groups. The mean ages were 1.4 years and 1.6 years in the ADP and SP groups, respectively (p = 0.895). In the ADP and SP groups, the expanded volumes were 270.9 ± 90.3 cm3 and 284.6 ± 149.7 cm3 (p = 0.91) and the growth-corrected expanded volumes were 162.1 ± 67.5 cm3 and 177.1 ± 105.2 cm3, respectively (p = 0.867). The relapse and growth-corrected relapse ratios showed no significant differences between the two groups, suggesting similar stability between the two protocols. The relapse ratios were 7.1 ± 4.8% and 7.3 ± 5.0% (p = 0.91) and the growth-corrected relapse ratios were -3.0 ± 3.3% and -2.4 ± 2.7%, respectively (p = 0.498). Within the limitations of the study, it seems that the ADP can shorten the distraction period without compromising stability. This may contribute to resolving the disadvantages and highlighting the advantages of DO.

Técnica de clonación 3D aplicada en planificación quirúrgica para craneosinostosis sagital en Latinoamérica-Perú: reporte de caso

La craneosinostosis sagital es el cierre prematuro de la sutura sagital, ocasionando alteraciones funcionales y estructurales. El tratamiento es quirúrgico, y actualmente se cuenta con diversas técnicas, las cuales requieren de una planificación y entrenamiento para lograr óptimos resultados. Se presenta el caso de un varón de 1 año presenta crecimiento anteroposterior anormal del cráneo, indicándose tomografía cerebral sin contraste evidenciando una sinostosis sagital. Se realiza la planificación quirúrgica de la técnica a desarrollar mediante modelo 3D personalizado a escala real. Paciente cursa con buena evolución y es dado de alta. Finalmente, la tecnología de clonación 3D esencial para la educación y desarrollo neuroquirúrgico permitiendo acceder a modelos táctiles de alta precisión y bajo costo que mejoran la calidad del manejo de craneosinostosis.

Sagittal craniosynostosis is the premature closure of the sagittal suture, causing functional and structural alterations. The treatment is surgical, and there are currently various techniques, which require planning and training to achieve optimal results. We present the case of a 1-year-old male with abnormal anteroposterior growth of the skull, indicating brain tomography without contrast, showing sagittal synostosis. Surgical planning of the technique to be developed is carried out using a real-scale personalized 3D model. The patient progresses well and is discharged. Finally, essential 3D cloning technology for neurosurgical education and development allows access to high-precision, low-cost tactile models that improve the quality of craniosynostosis management.

Three-dimensional printing models improve long-term retention in medical education of pathoanatomy: A randomized controlled study.

Craniosynostosis is a rare and complex pathology, and visuospatial skills are necessary for a good understanding of the condition. While the use of three-dimensional (3D) models has improved the understanding of complex craniofacial anatomy, no study has evaluated the impact of this teaching support on long-term retention. Our randomized controlled trial was designed to compare the long-term retention of information with 3D-printed models of four types of craniosynostosis versus classic 3D reconstructions displayed in two-dimensional (2D) among undergraduate students. All students benefited from the same standardized course followed by the manipulation of the learning tool associated with the group for 15 min. Long-term retention was assessed by the capability to properly recognize different types of craniosynostosis 3 weeks after the course. Eighty-five students were enrolled. Previous educational achievements and baseline visuospatial skills were similar between the groups. The bivariate analysis showed the mean score in the 3D and 2D groups were 11.32 (2.89) and 8.08 (2.81), respectively (p < 0.0001). 3D-printed models of structures with spatial complexity such as various craniosynostosis patterns improve significantly medical students' long-term retention, indicating their educational efficacy.

Zygomatic repositioning and Le Fort II distraction with intraoral devices in Apert syndrome: A case report.

The aim of this case report is to describe the surgical technique and outcome using internal intraoral distraction devices in LeFort II distraction with zygomatic repositioning (LF2ZR). In Apert syndrome the midface is characterized by a complex hypoplasia, with the central part being more affected than the lateral orbito-zygomatic complex. In LF2ZR, the zygomas are repositioned and internally fixated, and the central midface is further advanced through a LeFort II distraction. In previous publications, the distraction has been performed using external halo-based devices. It seems that the LF2ZR procedure can be planned and performed with adequate accuracy using virtual surgical planning tools. Knowledge about the possibility of using internal intraoral distraction devices in LF2ZR is important, as the inconspicuous placement of intraoral devices can be advantageous for some patients.

Computed tomography findings of Crouzon syndrome: A case report.

Crouzon syndrome is a genetic condition characterized by a premature fusion of skull sutures resulting in head and facial deformities. Crouzon syndrome is usually suspected at birth through physical examination or in the antenatal period via ultrasonographic assessment. Once Crouzon syndrome is suspected, advanced imaging methods such as three-dimensional computed tomography must be requested, showing early signs of cranial sutures fusion. In this paper, we present a case of a six-year-old girl who was taken to a pediatrician control appointment due to abnormal facies. During the physical examination, a suspicion of Crouzon syndrome was raised. Therefore, a head computed tomography was requested, showing asymmetrical calvarium thickening, diffuse indentation of the inner table of the skull, and moderate hydrocephalus with a big cyst in the posterior fossa. Due to these findings, the patient was remitted to maxillofacial surgery for further evaluation; however, the medical appointment could not be achieved as a consequence of the poor medical insurance of the girl. This paper aims to describe and discuss the computed tomography findings of Crouzon syndrome.

Hallermann-Streiff Syndrome and Psychosis: A Case Report.

Description Hallermann-Streiff syndrome is a rare genetic congenital disorder, with fewer than 200 cases reported to date, that is characterized by brachycephaly with frontal bossing, micrognathia, a "bird-like" beaked nose, microphthalmia with congenital cataracts, dental abnormalities, hypotrichosis, skin atrophy, and short stature. There is limited data on psychosis in individuals with Hallermann-Streiff syndrome; the information available depicts mania rather than psychosis. This case report reviews the presentation and treatment of psychosis in a 32-year-old male with Hallermann-Streiff syndrome who was involuntarily admitted to an inpatient psychiatric unit for persecutory delusions and hallucinations. The patient's psychosis responded well to risperidone with a resolution of psychosis and suicidal ideation in 6 days. Psychosis in individuals with Hallermann-Streiff syndrome appears to respond to a high-potency second-generation antipsychotic. Given this patient's early onset of psychosis and limited social/occupational impairment, it is unclear if psychosis is a component of Hallermann-Streiff syndrome or if he has comorbid schizophrenia or schizoaffective disorder, bipolar type.

One-piece fronto-orbital distraction osteogenesis without bandeau in patients with coronal craniosynostosis: A five-year follow-up retrospective study of 45 consecutive patients.

This study aimed to review the surgical outcomes, complications, and long-term relapses in patients with unilateral or bilateral coronal craniosynostosis, who underwent one-piece fronto-orbital distraction osteogenesis (FODO) without bandeau. The cephalic index, frontal angle, and supraorbital symmetry ratio were measured on the initial and follow-up computed tomography images. Esthetic outcomes were evaluated using the Whitaker classification. 45 patients were included in this study. The average follow-up interval was 5.4 ± 1.1 years (range 2.5-8.5 years). In patients with bilateral coronal craniosynostosis, the frontal angle decreased with a relapse ratio of -2.8 ± 4.3% during long-term follow-up compared with that during short-term follow-up (p = 0.028). In patients with unilateral coronal craniosynostosis, the supraorbital symmetry ratio decreased slightly, with a relapse ratio of -3.8 ± 2.6% during long-term follow-up (p = 0.017). Complications included dural tears during osteotomy (n = 2), early distractor removal (n = 2), and wound problems (n = 3). Within the limitations of the study it seems that one-piece fronto-orbital distraction osteogenesis (FODO) generates successful and sustainable results even in the long term. Therefore, this treatment option should be considered for patients with unilateral and bilateral coronal craniosynostoses whenever appropriate.