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John A Persing - One of the best experts on this subject based on the ideXlab platform.
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what is the skull structure influence of squamosal suture Synostosis in nonsyndromic and syndromic crouzon cranioSynostosis
Journal of Craniofacial Surgery, 2019Co-Authors: Xiaona Lu, Guocheng Chen, Antonio J Forte, Raysa Cabrejo, Anusha Singh, Gabrick Kyle, Derek M Steinbacher, Michael Alperovich, Nivaldo Alonso, John A PersingAbstract:BACKGROUND The morphologic effect of squamosal Synostosis has not been clarified, due to its low occurrence rate, and its inclination to be combined with premature closure of other major sutures, especially in syndromic Synostosis. This study's goal is to explore the morphological influence of squamosal Synostosis in both syndromic and nonsyndromic cranioSynostosis patients. METHODS Twenty-five computed tomography (CT) scans from nonsyndromic squamosal Synostosis (NSS), Crouzon syndrome with squamosal Synostosis (CSS), Crouzon syndrome without squamosal Synostosis, and normal controls were included. Three-dimensional (3D) cephalometry, entire cranial volume and cranial fossa volume were measured using Materialise software. RESULTS The entire cranial volume of all groups was similar. The NSS developed a gradual reduction in the severity of reduced segmental volume from the anterior cranial fossa, to the middle cranial fossa, and to the unaffected volume of posterior cranial fossa. The CSS developed the most severe volume reduction of middle cranial fossa (12608.30 ± 2408.61 mm vs 26077.96 ± 4465.74 mm, 52%), with proportionate volume reduction of the anterior (7312.21 ± 2435.97 mm vs 10520.63 ± 2400.43 mm, 30%) and posterior cranial fossa (33487.29 ± 5598.93 mm vs 48325.04 ± 14700.44 mm, 31%). The NSS developed a narrower anterior cranial fossa, a clockwise rotated Frankfort horizontal plane, and a retracted chin (pogonion). CONCLUSION Squamosal Synostosis may alter the development of cranial fossa volume, especially the volume of middle cranial fossa. However, the development of cranial base, and related facial features are determined largely by major vault sutures and associated syndromic conditions, rather than the squamosal suture alone. LEVEL OF EVIDENCE II.
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classification of subtypes of apert syndrome based on the type of vault suture Synostosis
Plastic and reconstructive surgery. Global open, 2019Co-Authors: Xiaona Lu, Antonio J Forte, Raysa Cabrejo, Derek M Steinbacher, Michael Alperovich, Nivaldo Alonso, Rajendra Sawhmartinez, Robin T Wu, Alexander Wilson, John A PersingAbstract:Background: Apert syndrome patients are different in clinical pathology, including obstructive sleep apnea, cleft palate, and mental deficiency. These functional deficiencies may be due to anatomic deformities, which may be caused by different forms of associated suture fusion. Therefore, a classification system of Apert syndrome based on the type of cranioSynostosis pattern might be helpful in determining treatment choices. Methods: CT scans of 31 unoperated Apert syndrome and 51 controls were included and subgrouped as: class I. Bilateral coronal Synostosis; class II. PanSynostosis; and class III. Perpendicular combination Synostosis: a. unilateral coronal and metopic Synostosis; b. sagittal with bilateral/unilateral lambdoid Synostosis; and c. others. Results: Class I is the most common (55%) subtype. The cranial base angulation of class I was normal; however, the cranial base angulation on the cranium side of the skull in class II increased 12.16 degrees (P = 0.006), whereas the facial side cranial base angle of class IIIa decreased 4.31 degrees (P = 0.035) over time. The external cranial base linear measurements of class I showed more evident reduction in anterior craniofacial structures than posterior, whereas other subtypes developed more severe shortening in the posterior aspects. Conclusions: Bicoronal Synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined panSynostosis patients have flatter cranial base, whereas the combined unilateral coronal Synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.
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classification of trigonocephaly in metopic Synostosis
Plastic and Reconstructive Surgery, 2012Co-Authors: Joel S Beckett, John A Persing, Priyanka Chadha, Derek M SteinbacherAbstract:Background:The orbitofrontal deformity in metopic Synostosis is recognized clinically but has not been quantitatively defined in a large patient population. The authors' purpose was to document the dysmorphology in metopic Synostosis and define subtype gradations.Methods:Demographic and computed tom
David W Johnson - One of the best experts on this subject based on the ideXlab platform.
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the significance of squamosal suture Synostosis
Journal of Craniofacial Surgery, 2016Co-Authors: Karen A Eley, Gregory P L Thomas, Fintan Sheerin, Deirdre Cilliers, Steven A Wall, David W JohnsonAbstract:: The squamosal suture is one of the lateral minor skull sutures, separating the parietal and squamous temporal bones. While the phenotypic appearances and sequelae of Synostosis of the major cranial vault sutures are well documented, little is reported concerning Synostosis of the squamosal suture (SQS). The aim of this study was to determine the frequency of squamosal suture Synostosis, and to document the significance of this entity.A retrospective review of the diagnostic imaging for all new pediatric patients (aged ≤16 years) referred to the Oxford Craniofacial Unit between January 2008 and February 2013 was completed to identify patients with SQS. Computed tomography (CT) imaging was available in 422 patients and the axial and three-dimensional reconstructed images reviewed.Squamosal suture Synostosis was confirmed in 38 patients (9%). It was present in conjunction with major suture Synostosis in 33 patients and in isolation in 5. The incidence increased with age. It was more common in patients with syndromic cranioSynostosis (18%) and associated syndromic conditions (36%) than in those with isolated major suture Synostosis (6%). It was found to occur with coronal, lambdoid, and sagittal Synostosis, but was most frequent with multisuture fusion patterns. Squamosal suture Synostosis was not associated with a consistent calvarial deformity either in isolation or when associated with a major suture fusion. No patient underwent surgery specifically to correct SQS.In conclusion, contrary to previous reports, squamosal suture Synostosis is a relatively frequent finding in the general case mix of a typical craniofacial unit, but is of limited clinical significance.
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management of isolated sagittal Synostosis in the absence of scaphocephaly a series of eight cases
Plastic and Reconstructive Surgery, 2010Co-Authors: Daniel G Morritt, Steve A Wall, Peter G Richards, Jayaratnam Jayamohan, David W JohnsonAbstract:BACKGROUND: The diagnosis of isolated sagittal Synostosis is usually made at birth, with a boat-shaped deformity of the skull (scaphocephaly). The Oxford Craniofacial Unit has successfully managed eight cases of isolated sagittal Synostosis without scaphocephaly. To the authors' knowledge, there have been no reports of this in the literature. METHODS: The Oxford Craniofacial Unit database (set up in 2004) was searched. This identified 193 cases of isolated sagittal Synostosis, eight of which did not have a scaphocephalic head shape (i.e., a cephalic index <76). RESULTS: Patients with isolated sagittal Synostosis in the absence of scaphocephaly represent 4.1 percent of all isolated sagittal Synostosis patients in the authors' database. Intracranial pressure was measured in six of the eight cases and found to be high in four of these (67 percent). CONCLUSIONS: It is unclear why the secondary features of sagittal Synostosis did not develop in these cases. Possible explanations include fusion of the suture late after the majority of skull growth has taken place and a patent metopic suture allowing growth to occur anteriorly; other sutures appeared patent radiologically but may not have been functioning normally. Although difficult to recognize, patients with isolated sagittal Synostosis in the absence of scaphocephaly are important to identify because they are at high risk of developing raised intracranial pressure. A new management algorithm for treatment of these cases is described that has been used successfully in the Oxford Craniofacial Unit.
Nathan R Selden - One of the best experts on this subject based on the ideXlab platform.
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delayed intracranial hypertension after cranial vault remodeling for nonsyndromic single suture Synostosis
Journal of Neurosurgery, 2013Co-Authors: Justin S Cetas, Morad Nasseri, Targol Saedi, Anna A Kuang, Nathan R SeldenAbstract:Object Delayed intracranial hypertension may occur after cranial vault remodeling for Synostosis and may result in visual loss and developmental delay. Delayed intracranial hypertension is relatively common in children with syndromic, multisuture Synostosis, but the incidence is poorly defined in children with single-suture nonsyndromic Synostosis. This study evaluates the frequency of reoperation for delayed intracranial hypertension after single-suture Synostosis repair. Methods Patients who had undergone cranial vault remodeling for nonsyndromic single-suture Synostosis and were treated at a single tertiary pediatric hospital between July 2000 and December 2010 were analyzed for the occurrence of delayed intracranial hypertension and reoperation for cranial vault remodeling. Results Eighty-one patients with clinical follow-up of at least 3 years were analyzed from a total of 156 consecutive patients. The average patient age at the initial operation was 9.1 months. Five (6.2%) of 81 patients presented w...
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Benign radiographic coronal Synostosis after sagittal Synostosis repair.
Journal of Craniofacial Surgery, 2013Co-Authors: Anna A Kuang, Tina Jenq, Ryne A. Didier, Lauren Moneta, Dianna M.e. Bardo, Nathan R SeldenAbstract:: Whether cranial vault remodeling surgery for nonsyndromic, isolated sagittal suture Synostosis affects the patency of initially normal, unaffected sutures is unknown. The influence of coronal and lambdoidal suture patency after cranial vault remodeling on the trajectory of subsequent cranial growth is also unknown. Disruption of normal sutural anatomy during cranial vault reconstruction could influence the incidence of secondary cranioSynostosis and need for reoperation in a small proportion of these patients.We performed a retrospective review of patients younger than 1 year with nonsyndromic sagittal Synostosis treated at a single tertiary referral pediatric hospital from September 2005 to January 2010 by an interdisciplinary team. Computed tomographic images obtained preoperatively, immediately postoperatively, and 2 years postoperatively were evaluated for the occurrence of secondary Synostosis of initially nonsynostotic sutures. Craniofacial disorders clinic and ophthalmologic follow-up records were also analyzed for the occurrence of radiographic cranial restenosis, clinical or ophthalmologic signs of intracranial hypertension (ICH), and reoperation.Fifty-one patients younger than 1 year underwent primary surgical repair of isolated, nonsyndromic sagittal suture Synostosis during the study period. Thirty-seven of these patients (71%) had completed 2-year clinical and radiographic follow-up by the time of analysis, constituting the study population. The average age at surgery was 5.4 months (range, 3.1-11.5 months). Thirty-three (89%) of the 37 study patients showed radiographic evidence of bilateral secondary coronal Synostosis (SCS). Five patients (15%) additionally showed partial lambdoid Synostosis. One patient with radiographic SCS (3%) required reoperation for radiographic cranial restenosis, clinical signs and symptoms of ICH, and papilledema first noted 1 year after primary cranial vault reconstruction.There is a high incidence of secondary coronal suture Synostosis following cranial vault remodeling for isolated, nonsyndromic sagittal Synostosis. Postoperative SCS was only rarely associated with secondary radiographic cranial stenosis, clinical or ophthalmologic signs of ICH, and the need for reoperation.
John B Mulliken - One of the best experts on this subject based on the ideXlab platform.
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increased frequency of left handedness in patients with unilateral coronal Synostosis
The Cleft Palate-Craniofacial Journal, 2009Co-Authors: Albert K Oh, John B Mulliken, Richard A Labrie, Gary F RogersAbstract:OBJECTIVE: Left-handedness reportedly has been more common in persons with neurological afflictions (e.g., stroke) and malformations (e.g., cleft lip with or without cleft palate) that demonstrate marked unilateral involvement. Coronal Synostosis is also more frequently unilateral, affecting the right side more commonly than the left. We sought to compare left-handedness in patients with unilateral coronal Synostosis versus healthy controls. SUBJECTS: All patients aged 3 years or older with nonsyndromic unilateral coronal Synostosis and healthy controls recruited by pediatricians blinded to the study. MAIN OUTCOME MEASURES: Prospective data obtained for all participants included age, gender, and handedness. In patients with unilateral coronal Synostosis, the side of Synostosis and age at surgery were documented. Left-handedness in the study and control groups was compared using chi-square analysis. Left-handedness also was analyzed in the study group according to side of fusion. RESULTS: Eighty-six patients with nonsyndromic unilateral coronal Synostosis comprised the study group; there were 96 controls. The mean ages of the study (8.8 years) and control groups (9.8 years) were not statistically different (p > .05). There were more girls in the study group (67%) than in the control group (56%), but this difference was not statistically significant (p > .05). Left-handedness was documented in 30.2% of the study group and 11.4% of the control group (p < .005). Left-handedness was twice as common in patients with left versus right unilateral coronal Synostosis (44.4% versus 20.4%; p < .05). CONCLUSIONS: Left-handedness is nearly three times more common in patients with unilateral coronal Synostosis than in controls and four times more likely in patients with left-sided fusion.
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increased frequency of left handedness in patients with unilateral coronal Synostosis
Annual Meeting Plastic Surgery Research Council, 2009Co-Authors: Albert K Oh, John B Mulliken, Richard A Labrie, Gary F RogersAbstract:Objective: Left-handedness reportedly has been more common in persons with neurological afflictions (e.g., stroke) and malformations (e.g., cleft lip with or without cleft palate) that demonstrate marked unilateral involvement. Coronal Synostosis is also more frequently unilateral, affecting the right side more commonly than the left. We sought to compare left-handedness in patients with unilateral coronal Synostosis versus healthy controls. Subjects: All patients aged 3 years or older with nonsyndromic unilateral coronal Synostosis and healthy controls recruited by pediatricians blinded to the study. Main Outcome Measures: Prospective data obtained for all participants included age, gender, and handedness. In patients with unilateral coronal Synostosis, the side of Synostosis and age at surgery were documented. Left-handedness in the study and control groups was compared using chi-square analysis. Left-handedness also was analyzed in the study group according to side of fusion. Results: Eighty-six patients with nonsyndromic unilateral coronal Synostosis comprised the study group; there were 96 controls. The mean ages of the study (8.8 years) and control groups (9.8 years) were not statistically different (p > .05). There were more girls in the study group (67%) than in the control group (56%), but this difference was not statistically significant (p > .05). Left-handedness was documented in 30.2% of the study group and 11.4% of the control group (p < .005). Left-handedness was twice as common in patients with left versus right unilateral coronal Synostosis (44.4% versus 20.4%; p < .05). Conclusions: Left-handedness is nearly three times more common in patients with unilateral coronal Synostosis than in controls and four times more likely in patients with left-sided fusion.
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astigmatism in unilateral coronal Synostosis incidence and laterality
Journal of Aapos, 2007Co-Authors: Richard Levy, John B Mulliken, Gary F Rogers, Mark R Proctor, Linda R DagiAbstract:Purpose Unilateral coronal Synostosis alters orbital development and can be associated with strabismus, oblique muscle dysfunction, astigmatism, and amblyopia. While the ocular effects are most dramatic on the side of the fusion, the shape of the contralateral orbit is also abnormal and the effect of these changes on bilateral visual development has not been fully described. The purpose of this study was to analyze the incidence and laterality of astigmatism in children with unilateral coronal Synostosis. Methods We retrospectively reviewed the charts of 39 consecutive patients with unilateral coronal Synostosis. Recorded data included the following: patient demographics, visual acuity, cycloplegic refraction, ocular motility, procedures for craniofacial and strabismus correction, and presence and treatment of amblyopia at each visit. Results Twenty-one of 39 patients (54%) had 1.00 D or more astigmatism in at least one eye at their last recorded refraction. Sixteen of the 21 (76%) had aniso-astigmatism of 1.00 D or more. Of the 16, 14 (88%) had the greater degree of astigmatism in the contralateral eye. Fifteen of the 39 studied (38%) had amblyopia and, in 12 of these 15 patients (80%), the amblyopic eye was contralateral to the Synostosis. Nineteen of 39 patients (49%) had greater ipsilateral fundus excyclotorsion, whereas only 1 of 39 (3%) had greater contralateral excyclotorsion. Conclusions Patients with unilateral coronal Synostosis are at risk for developing a greater degree of astigmatism in the eye contralateral to the Synostosis. Ocular disturbances in the contralateral eye, especially anisometropic amblyopia, must be considered when treating patients with this type of cranioSynostosis.
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Concordant contralateral lambdoidal Synostosis in dizygotic twins.
Journal of Craniofacial Surgery, 2005Co-Authors: Gary F Rogers, Paul D. Edwards, Carolyn D Robson, John B MullikenAbstract:: Twin studies have been widely used to investigate genetic versus environmental causality of malformations. While there are numerous reports of concordant sutural fusions in syndromic twins, there are few cases in siblings with nonsyndromic single suture Synostosis. Lambdoidal Synostosis has no clear genetic etiology. Discordant Synostosis has been reported in one monozygotic twin; there is also an unsubstantiated report of concordance in dizygotic twins. We describe dizygotic twins concordant for contralateral lambdoidal Synostosis. Mutational analysis for FGFR 1,2,3 was negative. Given the low incidence, absence of reported inheritability, and lack of documented concordance in monozygotic twins, the pathogenesis of isolated lambdoidal fusion can only be ascribed to stochastic influences.
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molecular analysis of patients with synostotic frontal plagiocephaly unilateral coronal Synostosis
Plastic and Reconstructive Surgery, 2004Co-Authors: John B Mulliken, Karen W Gripp, Catherine A Stolle, D Steinberger, Ulrich MullerAbstract:Mutations in genes known to be responsible for most of the recognizable syndromes associated with bilateral coronal Synostosis can be detected by molecular testing. The genetic alterations that could cause unilateral coronal Synostosis are more elusive. It is recognized that FGFR and TWIST mutations
Derek M Steinbacher - One of the best experts on this subject based on the ideXlab platform.
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what is the skull structure influence of squamosal suture Synostosis in nonsyndromic and syndromic crouzon cranioSynostosis
Journal of Craniofacial Surgery, 2019Co-Authors: Xiaona Lu, Guocheng Chen, Antonio J Forte, Raysa Cabrejo, Anusha Singh, Gabrick Kyle, Derek M Steinbacher, Michael Alperovich, Nivaldo Alonso, John A PersingAbstract:BACKGROUND The morphologic effect of squamosal Synostosis has not been clarified, due to its low occurrence rate, and its inclination to be combined with premature closure of other major sutures, especially in syndromic Synostosis. This study's goal is to explore the morphological influence of squamosal Synostosis in both syndromic and nonsyndromic cranioSynostosis patients. METHODS Twenty-five computed tomography (CT) scans from nonsyndromic squamosal Synostosis (NSS), Crouzon syndrome with squamosal Synostosis (CSS), Crouzon syndrome without squamosal Synostosis, and normal controls were included. Three-dimensional (3D) cephalometry, entire cranial volume and cranial fossa volume were measured using Materialise software. RESULTS The entire cranial volume of all groups was similar. The NSS developed a gradual reduction in the severity of reduced segmental volume from the anterior cranial fossa, to the middle cranial fossa, and to the unaffected volume of posterior cranial fossa. The CSS developed the most severe volume reduction of middle cranial fossa (12608.30 ± 2408.61 mm vs 26077.96 ± 4465.74 mm, 52%), with proportionate volume reduction of the anterior (7312.21 ± 2435.97 mm vs 10520.63 ± 2400.43 mm, 30%) and posterior cranial fossa (33487.29 ± 5598.93 mm vs 48325.04 ± 14700.44 mm, 31%). The NSS developed a narrower anterior cranial fossa, a clockwise rotated Frankfort horizontal plane, and a retracted chin (pogonion). CONCLUSION Squamosal Synostosis may alter the development of cranial fossa volume, especially the volume of middle cranial fossa. However, the development of cranial base, and related facial features are determined largely by major vault sutures and associated syndromic conditions, rather than the squamosal suture alone. LEVEL OF EVIDENCE II.
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classification of subtypes of apert syndrome based on the type of vault suture Synostosis
Plastic and reconstructive surgery. Global open, 2019Co-Authors: Xiaona Lu, Antonio J Forte, Raysa Cabrejo, Derek M Steinbacher, Michael Alperovich, Nivaldo Alonso, Rajendra Sawhmartinez, Robin T Wu, Alexander Wilson, John A PersingAbstract:Background: Apert syndrome patients are different in clinical pathology, including obstructive sleep apnea, cleft palate, and mental deficiency. These functional deficiencies may be due to anatomic deformities, which may be caused by different forms of associated suture fusion. Therefore, a classification system of Apert syndrome based on the type of cranioSynostosis pattern might be helpful in determining treatment choices. Methods: CT scans of 31 unoperated Apert syndrome and 51 controls were included and subgrouped as: class I. Bilateral coronal Synostosis; class II. PanSynostosis; and class III. Perpendicular combination Synostosis: a. unilateral coronal and metopic Synostosis; b. sagittal with bilateral/unilateral lambdoid Synostosis; and c. others. Results: Class I is the most common (55%) subtype. The cranial base angulation of class I was normal; however, the cranial base angulation on the cranium side of the skull in class II increased 12.16 degrees (P = 0.006), whereas the facial side cranial base angle of class IIIa decreased 4.31 degrees (P = 0.035) over time. The external cranial base linear measurements of class I showed more evident reduction in anterior craniofacial structures than posterior, whereas other subtypes developed more severe shortening in the posterior aspects. Conclusions: Bicoronal Synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined panSynostosis patients have flatter cranial base, whereas the combined unilateral coronal Synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.
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classification of trigonocephaly in metopic Synostosis
Plastic and Reconstructive Surgery, 2012Co-Authors: Joel S Beckett, John A Persing, Priyanka Chadha, Derek M SteinbacherAbstract:Background:The orbitofrontal deformity in metopic Synostosis is recognized clinically but has not been quantitatively defined in a large patient population. The authors' purpose was to document the dysmorphology in metopic Synostosis and define subtype gradations.Methods:Demographic and computed tom
