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https://www.readbyqxmd.com/read/29771828/results-following-adoption-of-a-modified-melbourne-technique-of-total-scaphocephaly-correction
#1
Julia D Sharma, Justine L O'Hara, Alessandro Borghi, Naiara Florez-Rodriguez, William Breakey, Juling Ong, Nu Owase Jeelani, David J Dunaway, Greg James
The Melbourne technique was described in 2008 as a novel method for complete correction of scaphocephaly. Since 2015, it has become our operation of choice for children with sagittal synostosis who are too old at presentation for minimally invasive techniques. Our modifications were 2-position (initially supine then prone) technique and undertaking a formal fronto-orbital remodeling to correct forehead contour. Retrospective chart review was used to record demographics, blood transfusion frequency and volumes, operating time, length of stay, clinical outcome, and complications...
May 15, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29762326/temporal-progression-of-craniofacial-dysmorphology-in-unilateral-coronal-synostosis-a-mechanistic-hypothesis
#2
Kirun Baweja, Alexander H Sun, Rajendra Sawh-Martinez, John A Persing
AIM: This study chronicles skull base and face development in nonsyndromic unilateral coronal synostosis (UCS) during infancy, to characterize the mechanistic progression of facial dysmorphology. METHODS: Computed tomography scans from 51 subjects were reviewed (26 UCS, 25 controls) and data were reconstructed. Patients were stratified into 5 age groups. A series of measurements were taken from the reconstructions. RESULTS: All patients had a unilaterally fused coronal suture at the time of analysis...
May 14, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29761256/quantitative-analysis-of-cranial-orbital-changes-in-infants-with-anterior-synostotic-plagiocephaly
#3
Rosalinda Calandrelli, Fabio Pilato, Luca Massimi, Marco Panfili, Concezio Di Rocco, Cesare Colosimo
PURPOSE: The effects of premature fusion of one coronal suture cause skull and orbital alterations in term of side-to-side asymmetry. This study aimed to quantify the cranio-orbital complex changes related to the severity of skull base dysmorphology in patients with unicoronal synostosis. METHODS: Twenty-four infants affected by unicoronal synostosis were subdivided in three subgroups according to the severity of skull base deformity and their high-resolution CT images were quantitatively analyzed (groups IIa, IIb, III)...
May 14, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29742364/surgical-correction-of-metopic-craniosynostosis-a-3-d-photogrammetric-analysis-of-cranial-vault-outcomes
#4
Olivia E Linden, Vanessa M Baratta, Jose A Gonzalez, Margaret E Byrne, Petra M Klinge, Stephen R Sullivan, Helena O Taylor
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...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29675757/fetal-methotrexate-syndrome-and-antley-bixler-syndrome-should-not-be-confused
#5
REVIEW
C Richards, Christine M Hall, D Johnson, Amaka C Offiah
Exposure to methotrexate in utero can result in fetal methotrexate syndrome - a condition characterised by prenatal-onset growth retardation, craniosynostosis, dysmorphic facies and multiple limb abnormalities. A literature review was prompted by the findings in a girl who is presented here with full consent from her guardian. She is the third child of unrelated parents and was 4 years old at the time of this report. Her mother took 15 mg methotrexate on two occasions during early pregnancy for juvenile idiopathic arthritis, unaware that she was pregnant...
April 19, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29672313/delayed-chronic-subdural-hematoma-after-total-cranial-vault-reconstruction-for-sagittal-synostosis
#6
Christopher R Pasarikovski, Taylor J Abel, Chris R Forrest, Peter B Dirks, George M Ibrahim
When diagnosed and treated after the first year of life, the surgical management of sagittal synostosis typically necessitates total cranial vault reconstruction. The safety of total cranial vault reshaping has improved greatly over the past 3 decades. We report on the first published case of an asymptomatic 5-year-old patient who was found to have a large left sided chronic subdural hematoma 4 months after total cranial vault reshaping requiring surgery, detected on routine imaging. We hypothesize that augmentation of venous blood egression through the emissary veins during surgery may have led to venous hypertension and subsequently the development of the chronic subdural hematoma...
2018: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29662726/chronic-debilitating-headache-in-adults-caused-by-craniocerebral-disproportion-treatment-by-cranial-vault-expansion
#7
Ken R Winston, Brooke French, Jason Bunn
Craniocerebral disproportion is rarely considered as a cause for chronic, debilitating headache in adults. Children reported with this disorder typically suffer from headaches and lethargy for many years and have multisutural synostosis. The terms craniocerebral disproportion, craniostenosis, and slit-ventricle syndrome are used inconsistently as diagnostic designations. Three adults with craniocerebral disproportion who had been treated in infancy for two different pathologies are reported. All benefited greatly from cranial vault expansion...
February 13, 2018: Curēus
https://www.readbyqxmd.com/read/29653011/-advance-in-clinical-research-on-antley-bixler-syndrome
#8
Min Xie, Hongying Wang, Linqi Chen, Haibo Li, Hong Li
Antley-Bixler syndrome (ABS) is a rare childhood disorder affecting skeletal development. Some patients may also have genital anomalies and impaired steroidogenesis. Diagnostic criteria for ABS has not been fully established, though craniosynostosis, midface hypoplasia and elbow synostosis are minimum requirements. The etiology of ABS is complex, which included autosomal dominant form caused by FGFR2 gene mutations, autosomal recessive form caused by POR gene mutations, and high oral dose of fluconazole during pregnancy...
April 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/29649371/subsegmental-osteotomy-technique-with-distraction-osteogenesis-for-natural-frontal-contouring-in-metopic-synostosis
#9
Kihwan Han, Woonhyeok Jeong, Jinhan Kim, Sangho Oh
Vectoring issues occur due to the dynamics of unidirectional procedure that is selected. To circumvent such difficulties, we have devised subsegmental osteotomy with distraction. In our technique, frontal bone flap was divided into 4 segments that were linked to each other by suturing wires in an 8-year-old patient with trigonocephaly. Subsegmental osteotomy and wiring of bone flap enabled us to slide bone in a more curvilinear manner and achieve natural contour. Distraction was initiated on postoperative day 7 at the rate of 1 mm/d for 20-mm gain...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29621073/normalization-of-speech-processing-after-whole-vault-cranioplasty-in-sagittal-synostosis
#10
Carolyn Chuang, Max Rolison, Jenny F Yang, Eric D Brooks, Peter W Hashim, Roberto Travieso, Jordan Terner, Derek M Steinbacher, Nicole Landi, Katherine K M Stavropoulos, Linda C Mayes, John A Persing, James C McPartland
BACKGROUND: Neurocognitive studies have found impairments in language-related abilities in nonsyndromic craniosynostosis, highlighting clinical importance of early language processing. In this study, neural response to speech sounds in infants with nonsyndromic sagittal craniosynostosis (NSC) is compared, preoperatively and postoperatively, using event-related potentials (ERPs) to objectively characterize development in language processing. METHODS: Electroencephalogram was recorded while 39 infants (12 NSC and 27 controls; ages 73-283 days) listened to the Hindi dental /da/ and retroflex /da/ phonemes (non-native phonemic discrimination task)...
April 4, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29618135/outcomes-of-40-nonsyndromic-sagittal-craniosynostosis-patients-as-adults-a-case-control-study-with-26-years-of-postoperative-follow-up
#11
Niina Salokorpi, Tuula Savolainen, Juha-Jaakko Sinikumpu, Leena Ylikontiola, George K Sándor, Pertti Pirttiniemi, Willy Serlo
BACKGROUND: While sagittal synostosis is the most common craniosynostosis, long-term follow-up of these patients is lacking. OBJECTIVE: To evaluate the results of surgical management of those patients with sagittal synostosis who attain adulthood. METHODS: An outcome study of surgically treated isolated sagittal synostosis patients operated between 1977 and 1998 was conducted at the Craniofacial Center of Oulu University Hospital, Oulu, Finland with an average follow-up time of 26...
April 2, 2018: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29601967/tarsal-coalition-in-paediatric-patients
#12
REVIEW
P-L Docquier, P Maldaque, M Bouchard
Tarsal coalition is due to failure of segmentation between two or more foot bones during embryological development at a site where the joint cleft fails to develop. Depending on the nature of the tissue connecting the bones, the abnormality is a syndesmosis, synchondrosis, or synostosis. Although the coalition exists at birth, synostosis usually develops only late during growth. Talo-calcaneal and calcaneo-navicular coalitions account for over 90% of all cases of tarsal coalition. The joint at the site of the coalition is stiff...
March 27, 2018: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/29594536/use-of-black-bone-mri-in-the-diagnosis-of-the-patients-with-posterior-plagiocephaly
#13
Linda Kuusela, Ada Hukki, Nina Brandstack, Taina Autti, Junnu Leikola, Anne Saarikko
PURPOSE: Ionising radiation exposure is especially harmful to brain development. The purpose of this study was to evaluate whether black-bone (BB) magnetic resonance imaging (MRI), a non-ionising imaging method, offers an alternative to ionising imaging methods such as computed tomography (CT) in the examination of cranial deformities. METHODS: From 2012 to 2014, a total of 408 children were referred to the Craniofacial Centre at the Helsinki University Hospital for further examination due to flatness of the posterior skull...
March 29, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29579020/-quality-of-life-in-adults-with-non-syndromic-craniosynostosis
#14
Daniel M Mazzaferro, Sanjay Naran, Ari M Wes, Leanne Magee, Jesse A Taylor, Scott P Bartlett
BACKGROUND: While studies have analyzed quality of life (QOL) in children with non-syndromic craniosynostosis (NSC), to date nobody has investigated long-term QOL in adults with NSC. The purpose of this study is to compare QOL in adult NSC patients with a cohort of unaffected controls. METHODS: We queried our institution's prospectively maintained craniofacial registry for NSC patients 18 years and older, and administered the validated World Health Organization Quality of Life (WHOQOL-BREF) questionnaire...
March 19, 2018: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29570518/surgical-treatment-of-nonsyndromic-unicoronal-craniosynostosis
#15
Jake Alford, Christopher A Derderian, James M Smartt
PURPOSE: Unicoronal synostosis (UCS) remains one of the most difficult craniofacial conditions to treat. This review attempts to consolidate all existing literature from the past 25 years that has investigated surgical treatment of nonsyndromic UCS. Additional attention is paid to specific areas of controversy regarding surgical management and evaluation of UCS: outcomes of fronto-orbital advancement (FOA) versus endoscopic strip craniectomy (ESC), emergence of spring-associated cranioplasty for craniosynostosis, and morphologic assessment metrics...
March 22, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29566257/seven-additional-families-with-spondylocarpotarsal-synostosis-syndrome-with-novel-biallelic-deleterious-variants-in-flnb
#16
S Salian, A Shukla, H Shah, S N Bhat, V R Bhat, S Nampoothiri, R Shenoy, S R Phadke, S V Hariharan, K M Girisha
The location and/or type of variants in FLNB result in a spectrum of osteochondrodysplasias ranging from mild forms, like spondylocarpotarsal synostosis syndrome and Larsen syndrome, to severe perinatal lethal forms, such as atelosteogenesis I and III and Boomerang dysplasia. Spondylocarpotarsal synostosis syndrome is characterized by disproportionate short stature, vertebral anomalies and fusion of carpal and tarsal bones. Biallelic loss-of-function variants in FLNB are known to cause spondylocarpotarsal synostosis syndrome and 9 families and 9 pathogenic variants have been reported so far...
March 22, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29561489/isolated-post-shunt-metopic-synostosis-and-neural-tube-defects
#17
William Abouhassan, John Kuang Chao, Ananth S Murthy
BACKGROUND: Craniosynostosis is an uncommon complication after shunting procedures for congenital hydrocephalus. We report a case of a child with myelomeningocele and normocephaly at the time of birth. She underwent ventricular shunting for Chiari malformation and hydrocephalus at 3 days of age. An immediate postoperative CT scan confirmed all sutures were open. Serial CT scans document an open metopic suture at 2 months, closed metopic suture at 5 months, and trigonocephaly at 11 months with concomitant slit ventricle syndrome, and collapsed lateral and third ventricles...
March 20, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29540340/mecom-associated-syndrome-a-heterogeneous-inherited-bone-marrow-failure-syndrome-with-amegakaryocytic-thrombocytopenia
#18
Manuela Germeshausen, Phil Ancliff, Jaime Estrada, Markus Metzler, Eva Ponstingl, Horst Rütschle, Dirk Schwabe, Richard H Scott, Sule Unal, Angela Wawer, Bernward Zeller, Matthias Ballmaier
Heterozygous mutations in MECOM (MDS1 and EVI1 complex locus) have been reported to be causative of a rare association of congenital amegakaryocytic thrombocytopenia and radioulnar synostosis. Here we report on 12 patients with congenital hypomegakaryocytic thrombocytopenia caused by MECOM mutations (including 10 novel mutations). The mutations affected different functional domains of the EVI1 protein. The spectrum of phenotypes was much broader than initially reported for the first 3 patients; we found familial as well as sporadic cases, and the clinical spectrum ranged from isolated radioulnar synostosis with no or mild hematological involvement to severe bone marrow failure without obvious skeletal abnormality...
March 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29520643/quantitative-evaluation-of-facial-hypoplasia-and-airway-obstruction-in-infants-with-syndromic-craniosynostosis-relationship-with-skull-base-and-splanchnocranium-sutural-pattern
#19
Rosalinda Calandrelli, Fabio Pilato, Luca Massimi, Marco Panfili, Gabriella D'Apolito, Simona Gaudino, Cesare Colosimo
PURPOSE: Craniosynostostic syndromes are due to multisuture synostoses and affect the entire craniofacial skeleton. This study analyzed the facial complex and airways to quantify the relationship between insufficient facial growth, airways obstruction, and the sutural pattern of the splanchnocranium and cranial fossae. METHODS: Preoperative high-resolution CT images in 19 infants with syndromic craniosynostosis were quantitatively analyzed. Because all children showed involvement of minor sutures/synchondroses coursing in the posterior cranial fossa, they were divided into three groups according to the synostotic involvement of "minor" sutures/synchondroses coursing in anterior (ACF) and middle (MCF) cranial fossae: group 1 (ACF), group 2 (MCF), and group 3 (ACF-MCF)...
May 2018: Neuroradiology
https://www.readbyqxmd.com/read/29520493/the-reverse-sauv%C3%A3-kapandji-procedure-for-the-treatment-of-posttraumatic-proximal-radioulnar-synostosis
#20
Isidro Jiménez, Pedro J Delgado
Proximal radioulnar synostosis is a rare but highly disabling posttraumatic complication in periarticular elbow injuries. Surgical treatment is an option for functionally limiting proximal radioulnar synostosis; however, the approach can endanger local neurovascular structures, especially if the synostosis affects the level of the bicipital tuberosity. We report two cases of proximal radioulnar synostosis with a preoperative prono-supination range of motion of 0° and 15° treated by a reverse Sauvé-Kapandji procedure resecting a 1-cm section of the radial shaft distal to the bicipital tuberosity and leaving the synostosis in place...
March 8, 2018: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
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