Cranial synostosis

AIM: To review the cases of craniosynostosis secondary to ventricular shunting procedure. MATERIAL AND METHODS: We retrospectively evaluated the medical records of all pediatric patients with hydrocephalus who were treated with ventriculoperitoneal shunt procedure between the years 2017 and 2021 at the Selcuk University, Ankara University, and Bursa Uludag University. RESULTS: Twenty-one patients were included in the study. The median age at the time of insertion of ventriculoperitoneal shunt for hydrocephalus was 8...

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...

UNLABELLED: Craniosynostosis is characterized by congenital absence or premature closure of skull sutures. The most common form of craniosynostosis is synostosis of sagittal suture followed by scaphocephaly. There are some head deformities similar to scaphocephaly such as positional and constitutional dolichocephaly, etc. These patients have no sagittal suture synostosis. However, there are difficulties in differential diagnosis between these deformities and scaphocephaly. OBJECTIVE: To develop differential diagnostic criteria between dolichocephalic head deformities and true scaphocephaly following sagittal synostosis...

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...

BACKGROUND: Although craniosynostoses involving the major sutures have been well described, the frequency of isolated minor suture craniosynostoses is much lower. Squamosal craniosynostosis (SQS) is a rare form of cranial synostosis, and the paucity of literature has made the creation of a standardized treatment plan difficult. We present a systematic review of the literature on isolated SQS to identify disease characteristics that lead to a need for operative intervention and to delineate patterns in surgical management...

OBJECTIVE: To analyze the clinical and genetic characteristics of an infant with craniosynostosis. METHODS: An infant who was admitted to Wuhan Children's Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology in April 2021 due to widening of the lateral ventricles for over a month was selected as the study subject. Clinical data of the patient was collected. Peripheral blood samples were collected from the infant and her parents for chromosomal karyotyping and whole exome sequencing...

To assess whether 3-dimensional (3D) volumetrics can be used to track and evaluate postoperative course of patients treated with endoscopic suturectomy for nonsyndromic sagittal synostosis, we compared changes in 2-dimensional (2D) measurements along with 3D volumetric correlates throughout the period of helmet therapy. Forty-six patients treated at our institution with endoscopic suturectomy for sagittal synostosis were retrospectively reviewed. Head circumference (HC), cephalic index (CI), and total cranial volumes (TCVs) were measured at 3 timepoints following surgery using optical surface scans obtained for helmet orthotics...

BACKGROUND: Various surgical techniques for cranial reconstruction of patients with bicoronal synostosis have been suggested. The outcome is, however, still often suboptimal. METHODS: In a 5-month-old child with apert syndrome, following a craniotomy incision, lambdoid suturotomy was done bilaterally. Two springs were implanted bilaterally over the lambdoid sutures. Cephalic index was obtained from three-dimensional computed tomography scans, and photographs were analyzed for aesthetic evaluation...

BACKGROUND: Primary craniosynostosis is a congenital craniofacial disorder in which cranial sutures prematurely close. Iatrogenic secondary stenosis is abnormal cranial suture closure caused by surgical manipulation of the suture. In contrast, idiopathic secondary stenosis develops in a suture that did not undergo surgical manipulation. The objective of this systematic review was to consolidate and characterize the incidence, classification, and management of idiopathic secondary stenosis in the literature...

The current study aimed to evaluate the early efficacy in infants with isolated non-syndromic sagittal synostosis who underwent minimally invasive endoscopic-assisted surgery. The clinical data of infants with isolated non-syndromic sagittal synostosis who were admitted to the Department of Neurosurgery of the Children's Hospital of Nanjing Medical University and underwent endoscopic-assisted surgery from October 2018 to December 2021 were retrospectively analyzed. All the infants underwent minimally invasive endoscopic-assisted surgery, and were treated with supine sleeping position after surgery...

BACKGROUND: Sagittal synostosis is the most common type of premature suture closure, and many surgical techniques are used to correct scaphocephalic skull shape. Given the rarity of direct comparisons of different surgical techniques for correcting craniosynostosis, this study compared outcomes of craniotomy combined with springs and H-craniectomy for non-syndromic sagittal synostosis. METHODS: Comparisons were performed using available pre- and postoperative imaging and follow-up data from the two craniofacial national referral centers in Sweden, which perform two different surgical techniques: craniotomy combined with springs (Gothenburg) and H-craniectomy (Renier's technique; Uppsala)...

BACKGROUND: Open middle and posterior cranial vault expansion (OPVE) or endoscopic (ES) strip craniectomy are two surgical techniques for normalization of head shape in isolated sagittal synostosis. This study aims to compare two-year cranial morphometrics after these two approaches. METHODS: We performed morphometric analysis on preoperative (t0), immediately post-operative (t1) and 2-year (t2) postoperative CT scans of patients who underwent OPVE or ES prior to 4 months of age...

OBJECTIVE: Unilateral lambdoid synostosis (ULS) is characterized by occipital flattening, mastoid bulging, and contralateral parietal bossing. Anterior craniofacial features are less well-defined. This study utilizes volumetric, craniometric, and composite heat maps of three-dimensional (3D) rendered CT scans to analyze anterior craniofacial asymmetry in ULS and compared to controls. DESIGN: A retrospective review of three-dimensional CT scans. SETTING: Tertiary care pediatric institution...

PURPOSE: To assess the role of FGFR2 mutations and sutural synostotic patterns on facial skeleton dysmorphology in children with syndromic craniosynostosis. METHODS: Preoperative high-resolution CT images in 39 infants with syndromic craniosynostosis were evaluated. Patients were divided into infants with and without FGFR2 mutations; each group was split according to synostotic involvement of minor sutures/synchondroses: isolated or combined involvement of middle (MCF) and posterior cranial fossae (PCF)...

ETS2 repressor factor ( ERF ) insufficiency causes craniosynostosis (CRS4) in humans and mice. ERF is an ETS domain transcriptional repressor regulated by Erk1/2 phosphorylation via nucleo-cytoplasmic shuttling. Here, we analyze the onset and development of the craniosynostosis phenotype in an Erf-insufficient mouse model and evaluate the potential of the residual Erf activity augmented by pharmacological compounds to ameliorate the disease. Erf insufficiency appears to cause an initially compromised frontal bone formation and subsequent multisuture synostosis, reflecting distinct roles of Erf on the cells that give rise to skull and facial bones...

PURPOSE: Studies previously implicated PRRX1 in craniofacial development, including demonstration of murine Prrx1 expression in the pre-osteogenic cells of the cranial sutures. We investigated the role of heterozygous missense and loss-of-function variants in PRRX1 associated with craniosynostosis. METHODS: Trio-based genome, exome or targeted sequencing were used to screen PRRX1 in patients with craniosynostosis; immunofluorescence analyses were used to assess nuclear localization of wild-type and mutant proteins...

OBJECTIVES: Unilateral coronal synostosis (UCS) results in a surgically demanding deformation, as the deformity is asymmetric in the calvarium but also presents with facial scoliosis and orbital dystopia. Traditional cranioplasties correct the forehead but have little effect on the face and orbits. Here, we describe a consecutive series of patients operated for UCS with osteotomy of the fused suture combined with distraction osteogenesis (FOD). METHODS: Fourteen patients [mean age: 8...

OBJECTIVE: Posterior cranial distraction (PCD) is a surgical technique to address craniosynostosis, especially in syndromic patients. The technique has the ability to significantly expand the cranium, while requiring minimal dural dissection, compared to cranial remodeling. Our goals were to determine the patient characteristics and surgical outcomes of PCD. The two questions that we sought to answer were: 1) What is the average published complication rate and the most common complications of PCD? and 2) How much intracranial volume expansion can one expect with PCD? DESIGN: A PubMed database search of articles on PCD was performed...

OBJECTIVE: The aim of this study is to compare three surgical interventions to correct sagittal synostosis: frontobiparietal remodeling (FBR), extended strip craniotomy (ESC), and spring assisted correction (SAC), based on 3D photogrammetry and operation characteristics. METHODS: All patients diagnosed with non-syndromic sagittal synostosis, born between 1991 and 2019, who underwent FBR, ESC or SAC, and had at least one postoperative 3D photogrammetry image taken during one of six follow-up moments until the age of six, were considered for this study...

INTRODUCTION: The diagnosis and management of metopic craniosynostosis involves subjective decision-making at the point of care. The purpose of this work is 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) and an unsupervised model used for cranial morphology in general (Cranial Morphology Deviation)...