Cranial synostosis

PURPOSE: The purpose of this study was to evaluate perinatal features and the rate of cesarean section in children with non-syndromic sagittal synostosis and to compare these with the official statistics. METHODS: The birth data of 36 consecutive children (25 boys) operated on using cranial vault remodeling because of primary sagittal synostosis were analyzed retrospectively from hospital records. The children were born between 2007 and 2011, and the surgery was performed before the age of 1 year...

OBJECTIVE Cranial vault expansion is performed in pediatric patients with craniosynostosis to improve head shape. Another argument for performing total cranial vault reconstruction is the potential reduction in the harmful effects of elevated intracranial pressure (ICP) that are associated with craniosynostosis. Alternatively, molding helmets have been shown to improve the cranial index (CI) in patients with sagittal synostosis without surgery. However, it is unknown if the use of molding helmets without surgery contributes to adverse changes in ICP...

Fibroblast growth factor receptor 2 (FGFR2) signaling is critical for proper craniofacial development. A gain-of-function mutation in the 2c splice variant of the receptor's gene is associated with Crouzon syndrome, which is characterized by craniosynostosis, the premature fusion of one or more of the cranial vault sutures, leading to craniofacial maldevelopment. Insight into the molecular mechanism of craniosynostosis has identified the ERK-MAPK signaling cascade as a critical regulator of suture patency. The aim of this study is to investigate the role of FGFR2c-induced ERK-MAPK activation in the regulation of coronal suture development...

BACKGROUND: A relationship between lambdoid craniosynostosis and Chiari deformations has been suggested, but the true extent of this association remains uncertain. The authors reviewed a single center's experience treating lambdoid synostosis to further elucidate this relationship, examine surgical outcomes, and identify associations that might impact future treatments. METHODS: A retrospective chart review was performed of all patients treated for lambdoid craniosynostosis, excluding the syndromic craniosynostoses...

STUDY DESIGN: Retrospective case series. PURPOSE: To assess the effect of non-kyphotic aligned congenital C3-4 synostosis on the adjacent segment in 10 patients. OVERVIEW OF LITERATURE: In the cervical spine, fusion disease at the adjacent motion segments may be a risk factor for potential neurological compromise and death. METHODS: Radiograms of 10 patients 13 to 69 years of age presenting with neck/shoulder discomfort or pain with or without trauma history were examined...

BACKGROUND: The metopic suture is unlike other cranial sutures in that it normally closes in infancy. Consequently, the diagnosis of metopic synostosis depends primarily on a subjective assessment of cranial shape. The purpose of this study was to create a simple, reproducible radiographic method to quantify forehead shape and distinguish trigonocephaly from normal cranial shape variation. METHODS: Computed tomography scans were acquired for 92 control patients (mean age, 4...

The frontonasal dysplasias are a group of craniofacial phenotypes characterized by hypertelorism, nasal clefting, frontal bossing, and abnormal hairline. These conditions are caused by recessive mutations in members of the aristaless gene family, resulting in abnormal cranial neural crest migration and differentiation. We report a family with a dominantly inherited craniofacial phenotype comprised of frontal bossing with high hairline, ptosis, hypertelorism, broad nasal tip, large anterior fontanelle, cranial base anomalies, and sagittal synostosis...

A unique single suture craniosynostosis case from the 9(th) century is presented in this paper. Although craniosynostosis is a fairly common pediatric anomaly nowadays, its occurrence in archaeological collections is an uncommon precedent. Since the diagnosis and treatment of premature cranial suture closure usually happens at an early age, evaluation of the whole developmental process is a rare opportunity. The right-sided coronal suture synostosis of this 30-35 years old woman gives an interesting opportunity to observe the effect of this phenomenon in adulthood...

BACKGROUND: Craniosynostosis is a condition characterized by the premature closure of one or more of the cranial vault sutures. It can occur alone or in association with other congenital defects and may be part of a syndrome. The sagittal suture is most commonly affected, comprising 40-60% of cases. Premature fusion of the sagittal suture can cause scaphocephaly due to compensatory anterior-posterior growth of the skull. This is morphologically considered as a narrow elongated skull with a decreased cephalic index, and is diagnosed clinically and/or radiologically...

Human ZIC1 (zinc finger protein of cerebellum 1), one of five homologs of the Drosophila pair-rule gene odd-paired, encodes a transcription factor previously implicated in vertebrate brain development. Heterozygous deletions of ZIC1 and its nearby paralog ZIC4 on chromosome 3q25.1 are associated with Dandy-Walker malformation of the cerebellum, and loss of the orthologous Zic1 gene in the mouse causes cerebellar hypoplasia and vertebral defects. We describe individuals from five families with heterozygous mutations located in the final (third) exon of ZIC1 (encoding four nonsense and one missense change) who have a distinct phenotype in which severe craniosynostosis, specifically involving the coronal sutures, and variable learning disability are the most characteristic features...

Fronto-orbital advancement and remodeling (FOAR) remains the most widely practiced treatment of unicoronal craniosynostosis (UCS) despite recent studies of ocular dysfunction and aesthetic shortcomings in the long-term. The aim of the study was to compare perioperative morbidity and short-term outcomes of a recently developed, nondevascularizing, distraction-based treatment of UCS with conventional FOAR. To do so, the authors compared the first 6 patients who were treated with a new osteotomy/distraction approach to the last 6 patients who underwent traditional FOAR for the treatment of UCS with regards to demographics, operative details, perioperative morbidity, and short-term outcomes...

Scaphocephaly is the most common single suture craniosynostosis. Surgical technique has evolved from simple strip craniectomy over π-procedures and vertex craniectomies to extensive cranial remodeling which is preferred procedure nowadays. The purpose of this paper is to present our modification of Renier's standard "H" technique and its preliminary results in detail. Eleven patients with scaphocephaly were surgically treated from January 2011 until January 2014. Only children with isolated sagittal synostosis were included in the study...

BACKGROUND: Historically, surgical treatment of children with a delayed presentation of cranial synostosis required complex cranial vault reconstruction. Recently, less invasive options for surgical correction, such as internal distraction osteogenesis, have been explored. In this study, we describe the successful management of delayed presentation of sagittal synostosis using distraction osteogenesis. METHODS: A bicoronal incision was made and 2 large rectangular osteotomies were performed bilaterally, involving the frontal, parietal, temporal and occipital bones...

PURPOSE: The characteristic features of prematurely fused craniosynostosis in plain radiographs have already been described in literature, but there is no clinical trial investigating the individual features of every single form of craniosynostosis. We described suture-specific characteristics as well as its frequency of appearance in plain radiographs in every different form of craniosynostosis. Intraoperative findings served as control to confirm the diagnosis. METHODS: One hundred twenty-seven children with prematurely fused cranial sutures who underwent a skull X-ray from 2008 to 2012 were investigated in the present study...

AIM: To fabricate a cost effective, indigenous and simple orthotics helmet for post-operative cranial molding in patients with craniosynostosis surgery. METHODS: We present a case of 15 month old infant with secondary cranial vault deformity. Cranial vault remodeling surgery involving the posterior skull was planned and executed to increase the posterior gap, so that brain growth would be facilitated towards this empty space. Materials such as thermoplastic sponge, thermoplastic ionomer resin sheet, soft sponge and Velcro straps are used to fabricate a cranial orthotics helmet...

PURPOSE: Distraction osteogenesis (DO) is a less daunting procedure than extensive cranial vault remodeling and has been used to correct sagittal craniosynostosis. The purposes of this study are to describe DO in combination with expansion and compression procedures and to report analytic results based on the cranial index (CI), volumetric measurement, and neurodevelopmental tests. METHODS: Between June 2002 and May 2013, 32 patients with non-syndromic sagittal synostosis who had undergone antero-posterior compression with bitemporal expansion were recruited...

PURPOSE: Premature unilateral coronal craniosynostosis results in distinctive cranial and facial abnormalities of varying severity, including orbital dystopia and an abnormal head shape. As the face is affected, these children may encounter stigmatization. To avoid this scenario, many parents elect for their child to undergo surgical correction. Laypeople's perception of children with either untreated or treated unilateral coronal craniosynostosis (UCS) has not yet been objectively evaluated...

BACKGROUND: Premature cranial suture fusion may prevent neonatal skull malleability during birth, increasing the risk of unplanned cesarean delivery and neonatal birth trauma caused by cephalopelvic disproportion. We sought to determine the incidence of perinatal maternofetal complications in cases of craniosynostosis. METHODS: Records of children presenting with nonsyndromic craniosynostosis to a tertiary pediatric hospital from 1996 to 2012 were reviewed retrospectively with focus on birth history and birth-related complications...

Craniosynostosis, a condition that includes the premature fusion of one or multiple cranial sutures, is a relatively common birth defect in humans and the second most common craniofacial anomaly after orofacial clefts. There is a significant clinical variation among different sutural synostoses as well as significant variation within any given single-suture synostosis. Craniosynostosis can be isolated (i.e., nonsyndromic) or occurs as part of a genetic syndrome (e.g., Crouzon, Pfeiffer, Apert, Muenke, and Saethre-Chotzen syndromes)...

The authors present a new and unique pattern of sutural fusion "peace sign synostosis" (PSS) characterized by synostosis of the metopic, bicoronal, and sagittal sutures and associated with abnormalities of the TWIST1 gene known to be associated with Saethre-Chotzen syndrome (SCS). To do so, we performed a retrospective review of patients with bicoronal, metopic, and at least partial anterior sagittal synostoses at the Children's Hospital of Philadelphia and Seattle Children's Hospital. Patients' demographics, genetic analysis, perioperative and clinic notes were reviewed...