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Cranial synostosis

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https://www.readbyqxmd.com/read/29438206/surgical-outcome-after-less-rigid-fixation-in-open-cranial-vault-remodeling-for-craniosynostosis
#1
Khai Luen Koh, Ali Zain
: Open cranial vault remodeling techniques require rigid fixation with hardware such as plates and screws; however, complications can occur. The purpose of this study was to assess the surgical outcome after open reconstruction for craniosynostosis with less rigid fixation using nonabsorbable suture. METHODS: Retrospective review of patients who underwent open craniofacial reconstruction for craniosynostosis at the Hospital Kuala Lumpur between January 2011 and December 2016 were performed...
February 12, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29437519/molecular-analysis-of-gli3-ihh-rab23-and-jag1-in-a-rabbit-model-of-craniosynostosis-likely-exclusion-as-the-loci-of-origin
#2
James R Gilbert, Gwen M Taylor, Joseph E Losee, Mark P Mooney, Gregory M Cooper
OBJECTIVE: Craniosynostosis (CS) involves the premature fusion of one or more cranial sutures. The etiology of CS is complex and mutations in more than 50 distinct genes have been causally linked to the disorder. Many of the genes that have been associated with CS in humans play an essential role in tissue patterning and early craniofacial development. Among these genes are members of the Hedgehog (HH) and Notch signal transduction pathways, including the GLI family member Gli3, Indian Hedgehog ( Ihh), the RAS oncogene family member Rab23, and the Notch ligand JAGGED1 ( Jag1)...
March 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29408824/craniofacial-distraction-in-the-management-of-anterior-plagiocephaly-a-novel-idea-and-a-systematic-review-of-the-literature
#3
Tarek Elbanoby
BACKGROUND: The objective of this work is to present a review of all reports involving the management of unicoronal synostosis with distraction osteogenesis (DO). Also, we have innovated a new method in the treatment of cases of anterior plagiocephaly at a young age using DO. METHODS: We present a case in which anterior plagiocephaly was treated by DO of both metopic and hemicoronal sutures in a 4-month-old female patient. A comprehensive systematic literature review was completed using the search terms "distraction osteogenesis," "unicoronal synostosis," "anterior plagiocephaly," and "craniosynostosis...
February 7, 2018: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29354335/adult-chiari-type-1-malformation-with-holocord-syringomyelia-associated-with-sagittal-synostosis
#4
Masakazu Sano, Junichi Yoshimura, Yukihiko Fujii
Craniosynostosis associated with Chiari malformation (CM) is usually found in infants with an underdeveloped posterior fossa. We here present a case of adult craniosynostosis, CM, and symptomatic syringomyelia caused by the protrusion of the posterior rim of the foramen magnum without a tight posterior fossa. A 22-year-old woman with an abnormal head shape and forearm hypesthesia was given a diagnosis of sagittal suture synostosis with CM and syringomyelia caused by foramen magnum stenosis. She underwent foramen magnum decompression with a C1 laminectomy without cranial vault expansion or duraplasty...
January 2018: NMC Case Report Journal
https://www.readbyqxmd.com/read/29329488/genetic-and-molecular-insights-into-genotype-phenotype-relationships-in-osteopathia-striata-with-cranial-sclerosis-oscs-through-the-analysis-of-novel-mouse-wtx-mutant-alleles
#5
Glenda Comai, Agnès Boutet, Kristina Tanneberger, Filippo Massa, Ana-Sofia Rocha, Aurelie Charlet, Clara Panzolini, Fariba Jian Motamedi, Robert Brommage, Wolfgang Hans, Thomas Funck-Brentano, Martin Hrabe de Angelis, Christine Hartmann, Martine Cohen-Solal, Jürgen Behrens, Andreas Schedl
The X-linked WTX/AMER1 protein forms an important component of the β-catenin destruction complex that can both enhance and suppress canonical β-catenin signalling. Somatic mutations in WTX/AMER1 have been found in a proportion of the pediatric kidney cancer Wilms' tumour. By contrast, germline mutations cause the severe sclerosing bone dysplasia osteopathia striata congenita with cranial sclerosis (OSCS), a condition usually associated with fetal or perinatal lethality in male patients. Here we addressed the developmental and molecular function of WTX by generating two novel mouse alleles...
January 12, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29307033/centile-charts-for-cranial-sutures-in-children-younger-than-1-year-based-on-ultrasound-measurements
#6
Katya Rozovsky, Nicholas James Barrowman, Elka Miller
BACKGROUND: Cranial US allows for the evaluation of premature closure (synostosis) or abnormal widening of the cranial sutures. An understanding of the normal anatomy is required to help define the presence or absence of abnormality. OBJECTIVE: To provide reference for normal ultrasound measurements of cranial sutures during the child's first year. MATERIALS AND METHODS: We included children ages 0 to 12 months who were referred to the hospital during 2011-2013 for radiographic evaluation of cranial sutures...
January 6, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29227405/osseous-convexity-at-the-anterior-fontanelle-a-presentation-of-metopic-fusion
#7
Alexander Haosi Sun, John A Persing
BACKGROUND: Craniosynostosis, or a premature fusion of 1 or more cranial vault sutures, results in characteristic head shape deformities. In previous reports, an osseous prominence at the anterior fontanelle has been suggestive of adjacent suture fusion and local elevation in intracranial pressure (ICP). This prominence has been termed the "volcano" sign, and has been described in the anterior fusion of the sagittal suture and serves as an indication for surgery. METHODS: Two patients presented for head shape evaluation with mild metopic ridging and anterior fontanellar osseous convexities consistent with the volcano sign...
December 7, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29184744/multidirectional-cranial-distraction-osteogenesis-with-simplified-modifications-for-treating-sagittal-synostosis
#8
Ataru Sunaga, Yasushi Sugawara, Hideaki Kamochi, Akira Gomi, Daekwan Chi, Rintaro Asahi, Masanori Mori, Shunji Sarukawa, Hirokazu Uda, Kotaro Yoshimura
Background: Multidirectional cranial distraction osteogenesis (MCDO) is a procedure of ours developed earlier for treating craniosynostosis. However, the numerous bone flaps led to prolonged operative time and occasional bone detachment from dura. We have since simplified the osteotomy design. In treating sagittal synostosis, required bone flaps have been reduced to 11 (from ~20). Methods: In a 2-year period (2014-2015), 5 boys with sagittal synostosis underwent MCDO using our simplified and fixed-form osteotomy...
October 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/29176416/is-postoperative-intensive-care-unit-care-necessary-following-cranial-vault-remodeling-for-sagittal-synostosis
#9
Erik M Wolfswinkel, Lori K Howell, Artur Fahradyan, Beina Azadgoli, J Gordon McComb, Mark M Urata
BACKGROUND: Of U.S. craniofacial and neurosurgeons, 94 percent routinely admit patients to the intensive care unit following cranial vault remodeling for correction of sagittal synostosis. This study aims to examine the outcomes and cost of direct ward admission following primary cranial vault remodeling for sagittal synostosis. METHODS: An institutional review board-approved retrospective review was undertaken of the records of all patients who underwent primary cranial vault remodeling for isolated sagittal craniosynostosis from 2009 to 2015 at a single pediatric hospital...
December 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29068968/isolated-intraorbital-frontosphenoidal-synostosis
#10
Paul A Mittermiller, Kristen W Yeom, Robert M Menard
Unilateral anterior plagiocephaly is most commonly the result of deformational plagiocephaly or unilateral coronal synostosis, a premature fusion of the frontoparietal suture. However, other sutures within the coronal ring have been implicated in producing anterior cranial asymmetries. These fusions can occur in isolation or in concert with adjacent sutures. The frontosphenoidal suture is one such suture within the coronal ring that has been involved both concomitantly with and independently of frontoparietal suture fusion...
October 24, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29067505/length-of-synostosis-and-segmented-intracranial-volume-correlate-with-age-in-patients-with-non-syndromic-sagittal-synostosis
#11
Arja Heliövaara, Junnu Leikola, Virve Koljonen, Pia Vuola, Mika Koivikko
PURPOSE: The aim of this study is to compare the length of synostosis and segmented intracranial volume (SIV) with age in children with non-syndromic sagittal synostosis. METHODS: Thirty-three consecutive patients (22 boys) who had been operated by cranial vault remodeling because of sagittal synostosis were compared retrospectively from 3D-CT imaging data sets obtained from volumetric CT. The mean age of the patients at preoperative CT imaging was 0.49 (range 0...
October 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29040146/the-role-of-preoperative-imaging-in-the-management-of-nonsyndromic-lambdoid-craniosynostosis
#12
Kavitha Ranganathan, Antonio Rampazzo, Asra Hashmi, Karin Muraszko, Jennifer Strahle, Christian J Vercler, Steven R Buchman
BACKGROUND: The necessity of imaging for patients with craniosynostosis is controversial. Lambdoid synostosis is known to be associated with additional anomalies, but the role of imaging in this setting has not been established. The purpose of this study was to evaluate the impact of preoperative imaging on intraoperative and postoperative management among patients undergoing operative intervention for lambdoid craniosynostosis. METHODS: A retrospective review of patients undergoing cranial vault remodeling for lambdoid craniosynostosis between January 2006 and 2014 was conducted...
October 16, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28968328/the-directive-growth-approach-for-nonsyndromic-unicoronal-craniosynostosis-patient-and-clinical-outcomes
#13
Robert J Mann, Matthew P Fahrenkopf, Michael Burton, John Girotto, John Polley
Deformities of the cranium in patients with nonsyndromic single-suture synostosis occur because of growth restriction at fused sutures and growth over compensation at normal sutures. Traditional surgery includes ostectomies of the synostotic suture to release these restricted areas and osteotomies to enable immediate cranial remodeling. In the process of reshaping the cranium, traditional approaches usually involve obliteration of both the normal functioning suture and the pathologic suture. The directive growth approach (DGA) is a new, simpler, more natural way to repair deformities caused by single-suture cranial synostosis...
November 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28962092/twenty-year-review-of-a-single-surgeon-s-experience-using-a-unique-surgical-technique-to-correct-lambdoidal-synostosis
#14
Charles Rodriguez-Feo, Julian Winocour, Roberto Ramirez, Marcia Spear, Kevin Kelly
BACKGROUND: Numerous techniques for the surgical management of lambdoidal synostosis have been previously described; however, no best practice technique currently exists. Surgical procedures range from complete posterior calvarial reconstruction to distraction osteogenesis techniques. Our primary purpose is to describe a novel approach to correct unilateral or bilateral lambdoidal synostosis. METHODS: A retrospective review was performed on a single surgeon's experience with craniosynostosis (1994-2014)...
October 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28922245/a-craniometric-analysis-of-cranial-base-and-cranial-vault-differences-in-patients-with-metopic-craniosynostosis
#15
Sanjay Naran, Daniel Mazzaferro, Ari Wes, Arastoo Vossough, Scott P Bartlett, Jesse A Taylor
PURPOSE: The effect of synostosis is not confined to the immediate vicinity of the suture. The authors hypothesized metopic craniosynostosis results in migration of lateral structures from midline, and differences in segmental volume of the cranial fossa. METHODS: A retrospective case-controlled cohort analysis of patients with nonsyndromic metopic craniosynostosis was performed. Craniometric angles, distances to landmarks from midline, cephalic index, and segmented volume ratios were calculated...
November 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28921242/use-of-computer-assisted-design-and-manufacturing-to-localize-dural-venous-sinuses-during-reconstructive-surgery-for-craniosynostosis
#16
Rajiv R Iyer, Adela Wu, Alexandra Macmillan, Leila Musavi, Regina Cho, Joseph Lopez, George I Jallo, Amir H Dorafshar, Edward S Ahn
OBJECTIVE: Cranial vault remodeling surgery for craniosynostosis carries the potential risk of dural venous sinus injury given the extensive bony exposure. Identification of the dural venous sinuses can be challenging in patients with craniosynostosis given the lack of accurate surface-localizing landmarks. Computer-aided design and manufacturing (CAD/CAM) has allowed surgeons to pre-operatively plan these complex procedures in an effort to increase reconstructive efficiency. An added benefit of this technology is the ability to intraoperatively map the dural venous sinuses based on pre-operative imaging...
September 18, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28914870/-shunt-induced-craniosynostosis-topicality-of-the-problem-choice-of-the-approach-and-features-of-surgical-treatment
#17
S A Kim, G V Letyagin, V E Danilin, A A Sysoeva
RATIONALE: Shunt-induced craniosynostosis is one of the late complications of CSF shunting surgery, which affects the patient's condition, clinical picture, and treatment approach. OBJECTIVE: to evaluate the prevalence rate and clinical significance of this disease, define the indications for surgery, and choose the optimal surgical approach. MATERIAL AND METHODS: The study included 59 children with shunt system dysfunction, aged 1 to 14 years, who were treated at the Department in the period from 2014 to 2016...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28914635/clinical-genetics-of-craniosynostosis
#18
Andrew O M Wilkie, David Johnson, Steven A Wall
PURPOSE OF REVIEW: When providing accurate clinical diagnosis and genetic counseling in craniosynostosis, the challenge is heightened by knowledge that etiology in any individual case may be entirely genetic, entirely environmental, or anything in between. This review will scope out how recent genetic discoveries from next-generation sequencing have impacted on the clinical genetic evaluation of craniosynostosis. RECENT FINDINGS: Survey of a 13-year birth cohort of patients treated at a single craniofacial unit demonstrates that a genetic cause of craniosynostosis can be identified in one quarter of cases...
December 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28885444/surgical-treatment-of-nonsyndromic-craniosynostosis
#19
Kristen A Klement, Karri A Adamson, Narges L Horriat, Arlen D Denny
Studies on the treatment of nonsyndromic craniosynostosis demonstrate decreasing morbidity and mortality; however, complication rates still range from 5% to 14%. Here, the authors present their last 10 years' experience treatment of nonsyndromic single suture craniosynostosis. A retrospective review was performed on patients who underwent open surgical treatment of nonsyndromic craniosynostosis over 10 years. Patient characteristics and clinical outcomes were reviewed. Radiological analysis of intracranial volumes was performed using Amira software...
October 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28819680/quantitative-approach-to-the-posterior-cranial-fossa-and-craniocervical-junction-in-asymptomatic-children-with-achondroplasia
#20
Rosalinda Calandrelli, Marco Panfili, Gabriella D'Apolito, Giuseppe Zampino, Alessandro Pedicelli, Fabio Pilato, Cesare Colosimo
PURPOSE: We propose an magnetic resonance imaging (MRI)-based quantitative morphovolumetric approach to the posterior cranial fossa (PCF) and craniocervical junction (CCJ) changes in achondroplastic patients investigating possible associations with ventriculomegaly and medullary compression. METHODS: We analyzed MRI of 13 achondroplastic children not treated by surgery. 3D FSPGR T1-weighted images were used to analyze (1) PCF synchondroses; (2) PCF volume (PCFV), PCF brain volume (PCFBV), PCFV/PCFBV ratio, cerebellar volume, cerebrospinal fluid (CSF) spaces volume, and IV ventricle volume; (3) PCF (clivus, supraocciput, exocciput lengths, tentorial angle) and CCJ (AP and LL diameters of foramen magnum (FM)) morphometry; (4) measurements of FM and jugular foramina (JF) areas; and (5) supratentorial ventricular volume...
October 2017: Neuroradiology
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