Publications by authors named "John A Persing"

128 Publications

Cephalocranial Disproportionate Fossa Volume and Normal Skull Base Angle in Pfeiffer Syndrome.

J Craniofac Surg 2021 Mar-Apr 01;32(2):581-586

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

Background: Pfeiffer syndrome is a rare syndromic craniosynostosis disorder, with a wide range of clinical manifestations. This study aims to investigate the structural abnormalities of cranial fossa and skull base development in Pfeiffer patients, to provide an anatomic basis for surgical interventions.

Method: Thirty preoperative CT scans of Pfeiffer syndrome patients were compared to 35 normal controls. Subgroup comparisons, related to differing suture synostosis, were performed.

Results: Overall, the volume of anterior and middle cranial fossae in Pfeiffer patients were increased by 31% (P < 0.001) and 19% (P = 0.004), versus controls. Volume of the posterior fossa in Pfeiffer patients was reduced by 14% (P = 0.026). When only associated with bicoronal synostosis, Pfeiffer syndrome patients developed enlarged anterior (68%, P = 0.001) and middle (40%, P = 0.031) fossae. However, sagittal synostosis cases only developed an enlarged anterior fossa (47%, P < 0.001). The patients with solely bilateral squamosal synostosis, developed simultaneous reduced anterior, middle and posterior cranial fossae volume (all P ≤ 0.002). The overall skull base angulation, measured on both intracranial and subcranial surfaces, grew normally.

Conclusion: Enlarged anterior cranial fossae in Pfeiffer syndrome children is evident, except for the squamosal synostosis cases which developed reduced volume in all fossae. Volume of the middle cranial fossa is influenced by associated cranial vault suture synostosis, specifically, sagittal synostosis cases develop normal middle fossa volume, while the bicoronal cases develop increased middle fossa volume. Posterior cranial fossa development is restricted by shortened posterior cranial base length. Surgical intervention in Pfeiffer syndrome patients optimally should be indexed to different suture synostosis.
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http://dx.doi.org/10.1097/SCS.0000000000007203DOI Listing
March 2021

Relative Importance of Facial Thirds in Facial Feminization Surgery.

Aesthet Surg J 2021 Mar 1. Epub 2021 Mar 1.

Yong Loo Lin School of Medicine, National University of Singapore, Singapore.

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http://dx.doi.org/10.1093/asj/sjab108DOI Listing
March 2021

Long-Term Neurocognitive Outcomes of Spring-Assisted Surgery versus Cranial Vault Remodeling for Sagittal Synostosis.

Plast Reconstr Surg 2021 Mar;147(3):661-671

From the Department of Surgery, Section of Plastic and Reconstructive Surgery, Yale School of Medicine; and the Departments of Plastic Surgery and Neurosurgery, Wake Forest School of Medicine.

Background: A long-term neurocognitive comparison of patients with sagittal synostosis who underwent spring-assisted surgery or cranial vault remodeling has not been performed.

Methods: Patients with sagittal synostosis who underwent spring-assisted surgery or cranial vault remodeling were recruited from Wake Forest School of Medicine and Yale School of Medicine, respectively. Cognitive tests administered included an abbreviated intelligence quotient, academic achievement, and visuomotor integration. An analysis of covariance model compared cohorts controlling for demographic variables.

Results: Thirty-nine spring-assisted surgery and 36 cranial vault remodeling patients were included in the study. No significant differences between cohorts were found with respect to age at surgery, sex, race, birth weight, family income, or parental education. The cranial vault cohort had significantly older parental age (p < 0.001), and mean age at testing for the spring cohort was significantly higher (p = 0.001). After adjusting for covariates, the cranial vault cohort had significantly higher verbal intelligence quotient (116.5 versus 104.3; p = 0.0024), performance intelligence quotient (109.2 versus 101.5; p = 0.041), and full-scale intelligence quotient (114.3 versus 103.2; p = 0.0032). When included patients were limited to intelligence quotients from 80 to 120, the cranial vault cohort maintained higher verbal (108.0 versus 100.4; p = 0.036), performance (104.5 versus 97.7; p = 0.016), and full-scale (107.6 versus 101.5; p = 0.038) intelligence quotients. The cranial vault cohort had higher visuomotor integration scores than the surgery group (111.1 versus 98.1; p < 0.001). There were no significant differences in academic achievement.

Conclusions: Sagittal synostosis patients who underwent cranial vault remodeling had higher intelligence quotient and visuomotor integration scores. There were no differences in academic achievement. Both cohorts had intelligence quotient scores at or above the normal range. Further studies are warranted to identify factors that may contribute to cognitive outcome differences.

Clinical Question/level Of Evidence: Therapeutic, II.
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http://dx.doi.org/10.1097/PRS.0000000000007640DOI Listing
March 2021

Airway Growth in Preoperative Patients with Crouzon Syndrome.

Facial Plast Surg Aesthet Med 2021 Feb 12. Epub 2021 Feb 12.

Division of Plastic and Reconstructive Surgery, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, USA.

Obstructive sleep apnea is common in patients with Crouzon syndrome, yet it may be caused by multiple factors. This study aims to investigate the natural history of airway development in preoperative Crouzon patients, from infants to adults. Preoperative computed tomography (CT) scans (Crouzon syndrome,  = 73; control,  = 87) were divided into five age subgroups. CT scans were measured using Materialise software. Before 6 months of age, nasal airway volume in patients with Crouzon syndrome was smaller than normal by 37% ( = 0.002), and the cross-sectional area at the choana reduced by 45% ( < 0.001). The reduction of nasal airway volume and cross-sectional area reached their nadir at 2 years of age, with shortening of 44% and 63% (both  < 0.001), respectively. They gradually caught up to normal dimensions after 6 years of age. Between 2 and 6 years, the pharyngeal airway in patients with Crouzon syndrome reduced 44% ( = 0.011) compared with controls. However, the airway cross-sectional area at condylion and gonion levels was less than normal, before 6 months (35%,  = 0.024) and (44%,  = 0.006) after 2 years of age, respectively. This reduction remains into adulthood. Nasal airway volume is more limited in children with Crouzon syndrome who are younger than 2 years of age. Whereas after 2 years of age, the pharyngeal airway develops significant volume restriction, leading to timing and specific treatment area foci based on the site of temporal maximal constriction.
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http://dx.doi.org/10.1089/fpsam.2020.0441DOI Listing
February 2021

Comments on "TCF12".

Authors:
John A Persing

J Craniofac Surg 2021 Feb 2. Epub 2021 Feb 2.

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

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http://dx.doi.org/10.1097/SCS.0000000000007520DOI Listing
February 2021

Functional Network Development in Sagittal Craniosynostosis Treated With Whole Vault Cranioplasty.

J Craniofac Surg 2021 Jan 29. Epub 2021 Jan 29.

Department of Surgery, Division of Plastic Surgery, Yale Medical School Department of Diagnostic Radiology, Yale University School of Medicine, PO Box 208042 Department of Neurosurgery, Yale Medical School Yale Child Study Center, Yale School of Medicine, New Haven, CT George Washington University, Washington, DC.

Introduction: In this study, the authors seek to clarify the neurological changes before and after whole vault cranioplasty (WVC) in patients born with sagittal craniosynostosis.

Methods: A case control study design was performed that included thirty functional MRI scans, from 25 individual patients. Functional MRI and diffusion tension imaging data were analyzed with BioImageSuite (Yale University, USA). 9 functional brain networks were analyzed, with appropriate correlated functional regions of the brain and utilized for analysis.

Results: Comparing functional MRI the infants after WVC versus infants before WVC group, the after WVC group demonstrated an increased connectivity in the left frontoparietal, secondary (V2), and third (V3) visual networks (P < 0.001). The right frontoparietal (RFPN) had decreased connectivity (P < 0.001). There is also a decrease and increase in anisotropy in the cingulum and precuneus despite surgery, respectively (P < 0.05). Adolescents treated with WVC compared to controls, demonstrated an increased connectivity in the salience and decreased connectivity in the RFPN relative to adolescent controls.

Conclusions: Patients born with sagittal craniosynostosis have different connections in infancy in most of the defined cerebral networks compared to controls. After surgery, there are specific connectivity changes that occur in the RFPN, left frontoparietal, V2, and V3 networks, which are areas associated with executive function and emotional control. Changes identified in white matter tract microstructure connections could be influential in changes in functional connectivity. Although, as a child with sagittal craniosynostosis develops, much of the abnormal network connections, seen in infancy preoperatively, corrects to some degree after surgery. However, some aberrancies in the salience and RFPN networks remain potentially affecting executive functioning.
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http://dx.doi.org/10.1097/SCS.0000000000007505DOI Listing
January 2021

Long-Term Neurocognitive Outcomes in Sagittal Synostosis: The Impact of Reoperation.

J Craniofac Surg 2021 Jan-Feb 01;32(1):58-61

Section of Plastic Surgery, Yale University School of Medicine.

Introduction: Optimal age at surgery in nonsyndromic sagittal craniosynostosis continues to be debated. Previous reports suggest that earlier age at whole vault cranioplasty more frequently requires reoperation. It is unknown, however, whether reoperation affects neurocognitive outcome. This study examined the impact of reoperation on neurocognitive outcome in children with nonsyndromic sagittal craniosynostosis using comprehensive neurocognitive testing.

Methods: Forty-seven school-age children (age 5-16 years) with nonsyndromic sagittal craniosynostosis who underwent whole-vault cranioplasty were included in this analysis. Participants were administered a battery of standardized neuropsychological testing to measure neurocognitive outcomes.

Results: Thirteen of the 47 participants underwent reoperation (27.7%); 11 out of the 13 reoperations were minor revisions while 2 reoperations were cranioplasties. Reoperation rate was not statistically different between patients who had earlier surgery (at age ≤6 months) versus later surgery (at age >6 months) (P > 0.05). Nonreoperated patients who had only one later-in-life surgery did not perform statistically better than reoperated patients on any outcome measure of neurocognitive function, including IQ, academic achievement, visuomotor integration, executive function, and behavior. Comparing reoperated earlier surgery patients with nonreoperated later surgery patients, reoperated earlier surgery patients had higher full-scale and verbal IQ (P < 0.05), scored higher on word reading, reading comprehension, spelling, numerical operations, and visuomotor integration (P < 0.05), and had fewer indicators of suspected learning disabilities (P < 0.01) compared to nonreoperated later surgery patients.

Conclusion: Reoperation rate after whole vault cranioplasty was 27.7%, with few cases of repeat cranioplasty (4.2% of all patients). Reoperation was not associated with worse neurocognitive outcome. Reoperated earlier surgery patients in fact performed better in IQ, academic achievement and visuomotor integration when compared to nonreoperated later surgery patients.
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http://dx.doi.org/10.1097/SCS.0000000000006909DOI Listing
January 2021

A Tribute to Ian Jackson: Current Controversies in Craniosynostosis Research.

J Craniofac Surg 2020 Dec 9. Epub 2020 Dec 9.

Section of Plastic and Reconstructive Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT.

As a noted clinician innovator in the field of craniofacial surgery, Dr. Ian Jackson investigated key issues pertinent to his time, and many of these reviews remain relevant today. He endeavored to develop a deeper understanding of answers to controversial problems throughout his career, which was often reflected by his published research. In recent years, notable controversial topics within craniosynostosis research have included the optimal timing and method of surgical intervention, the clinical criteria for diagnosis of different patterns of craniosynostosis, and the consequences of varying severities of deformity on neurocognitive development. Though having a distinct etiology, deformational plagiocephaly results in similar cranial deformation. Thus, the understanding of the etiology, diagnosis, and management of craniosynostosis and deformational plagiocephaly is at the frontier of craniofacial research, and is best addressed with this same attitude of genuine curiosity that Dr. Ian Jackson exhibited. This review aims to provide background and illustrate possible future directions of some specific areas within craniosynostosis research that are under active investigation.
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http://dx.doi.org/10.1097/SCS.0000000000007331DOI Listing
December 2020

Neurological Functional Connectivity in Unilateral Coronal Synostosis: A Side-Based Comparison.

J Craniofac Surg 2020 Nov 25. Epub 2020 Nov 25.

Section of Plastic Surgery, Department of Surgery, Yale School of Medicine.

Purpose: Unicoronal synostosis (UCS) has been associated with reading, language, and social dysfunction. Limited brain function connectivity studies exist for UCS with none devoted to comparing outcomes by side of synostosis (left versus right-sided UCS).

Methods: Twelve patients with surgically treated UCS, 7 right-sided and 5 left-sided, were age matched to healthy controls. Resting state functional MRI was acquired in a 3T Siemens TIM Trio scanner (Erlangen, Germany). Data was collected with intrinsic connectivity distribution and seed-connectivity analysis using BioImage Suite (Yale School of Medicine). Region of interest analysis was performed based on Brodmann areas related to emotional, executive, language, motor, and visuo-spatial function. Significance was set at P < 0.05.

Results: Compared to controls, all UCS patients demonstrated decreased connectivity in areas of the parietal and temporal cortices responsible for visuo-motor coordination and language function. Right UCS patients demonstrated decreased intrinsic connectivity in regions related to complex motor movement and proprioception relative to control subjects. Left UCS patients demonstrated decreased seed connectivity between regions of the parietal lobe and occipital lobe related to motor coordination, visual function, and language compared to right UCS patients.

Conclusion: Unicoronal synostosis had decreased functional connectivity in regions associated with memory, visual information processing, and motor function. Moreover, left-sided UCS had decreased connectivity in circuits for motor coordination and language when compared to right-sided UCS. This study provides data suggestive of long-term sequelae of UCS that varies by sidedness, which may be responsible for neurocognitive impairments found in previous cognitive analyses.
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http://dx.doi.org/10.1097/SCS.0000000000007274DOI Listing
November 2020

Distinguishing Craniomorphometric Characteristics of Unilateral Lambdoid Craniosynostosis.

J Craniofac Surg 2021 Jan-Feb 01;32(1):125-129

Section of Plastic and Reconstructive Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT.

Background: Unilateral lambdoid synostosis (ULS) represents the rarest form of single suture nonsyndromic craniosynostosis. Differentiating between posterior deformational plagiocephaly (DP) and ULS has been difficult due to overlapping clinical findings. Past analyses have been limited by sample size. This study was undertaken to clarify anatomical features of ULS.

Methods: A multiinstitution study was undertaken, analyzing CT imaging from patients with documented ULS to determine clinical and pathological characteristics. Similar analyses were performed on DP patients to differentiate the 2 conditions.

Results: Twenty-seven ULS patient scans and 10 DP scans were included. For ULS patients mean age was 6.6 months, majority male (75%), and majority left-sided ULS (71%). The synostosed side ear was anteriorly displaced in 100% of ULS patients, mean difference of 9.6° (P < 0.001), and inferiorly in 96.3% of patients, mean difference of 4.4 mm, relative to the nonsynostosed side. The posterior fossa deflection (PFD) was deviated 5.9° toward the synostosed side relative to the anterior midline. In DP, there was no significant difference between sides in EAC measurements. The PFD and EAC displacements were significantly smaller in DP relative to ULS (P < 0.001 for each). An ipsilateral mastoid bulge was found in 100% of ULS and 0% of DP on CT imaging.

Conclusion: Contrary to some previously published findings, the ear is more anteriorly displaced ipsilateral to the fused lambdoid suture in 100% of ULS patients. Ear position alone is not a reliable indicator to differentiate between DP and ULS. A mastoid bulge is a more reliable indicator of ULS.
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http://dx.doi.org/10.1097/SCS.0000000000007098DOI Listing
November 2020

Racial Disparity Between Asian and Caucasian Crouzon Syndrome in Skull Morphology.

J Craniofac Surg 2020 Nov/Dec;31(8):2182-2187

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

Background: Racial disparity in pathological consequences in skull growth may impact the treatment plan for different patient populations. This study attempts to explore the differences between Asian and Caucasian cranial morphology in Crouzon syndrome.

Method: Ninety-one computed tomographic scans were included (Asian Crouzon syndrome, n = 12; Asian controls, n = 22; Caucasian Crouzon syndrome, n = 16; Caucasian controls, n = 41), and measured using Mimics and 3-matics software.

Results: The entire cranial base length was reduced 11.92 mm (P = 0.004) in Asian Crouzon patients, and 14.58 mm (P < 0.001) in Caucasian Crouzon patients, compared to respective controls. The cranial base angle on the facial side of basicranium was more narrowed in Crouzon syndrome in both races, with similar changes of degrees (9.61°, P = 0.002, in Asian Crouzon; 9.20°, P = 0.019, in Caucasian Crouzon). However, the intracranial side was statistically more narrowed only in the Asian group (9.86°, P = 0.003). Both Asian and Caucasian Crouzon patients developed reduced posterior fossa volume, by 15% (P = 0.034) and 17% (P = 0.004), respectively. However, Caucasian Crouzon patients developed a more shortened anterior and middle cranial base, than that of Asian patients. The separation of lateral pterygoids was only significantly increased in Caucasian patients (5.49°, P < 0.001).

Conclusion: Crouzon syndrome causes a shortened, widened, and kyphotic cranial base across both races. It also restricts the development of the posterior cranial fossa. However, the skull malformation is not the same between populations: Asian Crouzon patients developed more narrowed cranial base angulation on the intracranial side of basicranium, while Caucasian Crouzon patients developed more widened lateral pterygoids.
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http://dx.doi.org/10.1097/SCS.0000000000006741DOI Listing
March 2021

Neurologic Characterization of Craniosynostosis: Can Direct Brain Recordings Predict Language Development?

J Craniofac Surg 2021 Jan-Feb 01;32(1):78-82

Yale School of Medicine.

Purpose: Nonsyndromic craniosynostosis (NSC) is associated with language deficits. Conventional tests, such as the Bayley Scales of Infant Development (BSID), may not reflect accurate long-term cognition. Alternatively, mismatch negativity (MMN) waves recorded via electroencephalogram (EEG) measure neural responses to speech and may objectively predict language development. This study aimed to (1) correlate infant MMN to future language achievement and (2) compare MMN among subtypes of NSC.

Methods: Pre and postoperatively (mean operative age 9.5 months), NSC participants received the BSID and EEG phoneme-discrimination paradigm(80 dB,250 Hz). The MMN was the largest negative amplitude in the difference wave 80 to 300 ms after stimuli. To measure cognitive outcome, patients completed a neurodevelopmental battery (Wechsler-Abbreviated Scale of Intelligence and Wechsler-Fundamentals) at >6 years of age.

Results: Eleven NSC patients with EEG testing in infancy were neurocognitively tested (average age 8.0 years; 27% female; 55% sagittal, 27% metopic, 9% unicoronal, 9% sagittal/metopic). The left frontal cluster MMN strongly correlated with word-reading (r = 0.713, P = 0.031), reading-comprehension (r = 0.745, P = 0.021), and language-composites (r = 0.0771, P = 0.015). Conversely, BSID scores did not yield significant predictive value (r < 0.5, P > 0.05). Follow-up event related potentials (ERP) comparison included 39 normal control, 18 sagittal, 17 metopic, 6 unilateral-coronal infants. Preoperatively, sagittal (P = 0.003) and metopic (P = 0.003) patients had attenuated left frontal MMN compared to controls. Postoperatively, the sagittal cohort was normalized to controls while metopic patients retained attenuations (P = 0.041).

Conclusion: ERP assessment in NSC had significantly better predictive value for future neurocognition than the BSID. Preoperatively, sagittal and metopic patients had attenuated neural response to language; postoperatively, sagittal patients had improved responses in comparison to metopic patients. Use of ERP assessment may help tailor treatment for language deficits earlier in development.
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http://dx.doi.org/10.1097/SCS.0000000000007004DOI Listing
September 2020

Spring-Assisted Strip Craniectomy Versus Cranial Vault Remodeling: Long-Term Psychological, Behavioral, and Executive Function Outcomes.

J Craniofac Surg 2020 Oct;31(7):2101-2105

Department of Surgery, Division of Plastic Surgery, Yale School of Medicine.

Background: Controversy exists regarding the optimal surgical approach for non-syndromic sagittal synostosis. This study provides the first comparative analysis of the long-term behavioral, psychological, and executive function outcomes for patients who underwent either cranial vault remodeling (CVR) or spring-assisted strip craniectomy (SAS).

Methods: Thirty-six CVR patients and 39 SAS patients were evaluated. Parents and caregivers completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Behavior Assessment System for Children, Second Edition (BASC-2) to evaluate behavioral, emotional, social, adaptive, and executive functioning skills.

Results: There were no statistically significant differences between the CVR and the SAS groups (P > 0.05) in any of the BRIEF areas of function. Furthermore, the BASC-2 battery illustrated no significant differences in all areas analyzed except one. Among the 2 groups, the CVR group was rated as having fewer social withdrawal symptoms on the BASC-2 (47.00 ± 10.27) compared to the SAS cohort (54.64 ± 10.96), F = 6.79, P = 0.012, Cohen d = 0.688. However, both means were still within the normal range.

Conclusions: Children undergoing SAS and CVR procedures for isolated sagittal synostosis were not rated as having clinically significant behavioral, emotional, social, adaptive, or executive functioning problems on parental forms.
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http://dx.doi.org/10.1097/SCS.0000000000006806DOI Listing
October 2020

Sphenoid Bone Structure and Its Influence on the Cranium in Syndromic Versus Nonsyndromic Craniosynostosis.

J Craniofac Surg 2021 Jan-Feb 01;32(1):67-72

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

Background: Little is known about the detailed growth of the sphenoidal and temporal bones, even though they contribute significantly to the cranial base and cranial fossa skeletons. They also serve to connect the cranial vault with facial structure. This study details their morphologic development in isolated bicoronal synostosis and associated syndromes.

Methods: Eighty-one CT scans were included (nonsyndromic bicoronal synostosis, n = 28; Apert syndrome associated with bicoronal synostosis, n = 19; Crouzon syndrome associated with bicoronal synostosis, n = 8; and controls, n = 26), and measured using Materialize software.

Results: Sphenoidal and temporal bone volumes in nonsyndromic bicoronal synostosis are reduced 23% (P = 0.005) and 24%(P = 0.003) at 6 months of age, compared to controls. Apert and Crouzon syndrome patients developed similar reduced volumes. The greater wing of the sphenoid and pterygoid processes in nonsyndromic bicoronal synostosis are initially inferiorly rotated at 2 months of age, by 9.60° (P = 0.002) and 4.33° (P = 0.023), respectively. In Apert syndrome, these rotations were reduced by 4.82° (P = 0.003) and 12.60° (P < 0.001), (like Crouzon syndrome). However, in Apert syndrome, the length of pterygoid processes is shortened by 11% (P = 0.018) compared to nonsyndromic bicoronal synostosis. Crouzon syndrome skulls did not develop a statistically significant shortening relative to nonsyndromic bicoronal synostosis. Mediolateral expansion of the sphenoid in nonsyndromic bicoronal synostosis was less than normal (P = 0.023), and it was further reduced in syndromic skulls.

Conclusion: Isolated bicoronal synostosis tends to reduce the volume of sphenoidal and temporal bones, and inferior and posterior rotation of the entire sphenoid. Syndromic conditions restrict sphenoidal rotation, and limit the expansion of sphenoidal greater wing and pterygoid plate.
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http://dx.doi.org/10.1097/SCS.0000000000006914DOI Listing
September 2020

Comparison of Neurocognitive Outcomes in Postoperative Adolescents with Unilateral Coronal Synostosis.

Plast Reconstr Surg 2020 09;146(3):614-619

From the Section of Plastic and Reconstructive Surgery, Department of Surgery, and the Yale Child Study Center, Yale University School of Medicine; and the Division of Plastic Surgery, Department of Surgery, Perelman School of Medicine at the University of Pennsylvania.

Unilateral coronal nonsyndromic craniosynostosis is associated with asymmetric skull growth, which may influence cerebral long-term function. Twenty affected adolescents who underwent cranial vault remodeling at a mean age of 8.2 months (12 from the Yale Craniofacial Clinic and eight from the Children's Hospital of Philadelphia) all completed a double-blinded neurodevelopmental assessment at an average age of 12.1 years. The study cohort included 55 percent female and 50 percent right-sided craniosynostosis. Mean verbal intelligence quotient was highest at 117.3, and mean performance intelligence quotient was 106.4, for a mean full-scale intelligence quotient of 112.5. Patients performed above the national average on all academic achievements except for numerical operations, which was significantly lower than word reading (p = 0.022). Patients performed below average on all Beery-Buktenica visual motor tests; motor-coordination was poorer than both visual motor integration and visual perception (p = 0.027 and p = 0.005). Significant positive correlations existed between paternal education/visual perception (r = 0.450; p = 0.046) and household income/verbal intelligence quotient (r = 0.628; p = 0.004). Patients with right unilateral coronal nonsyndromic craniosynostosis had improved spelling compared with left-sided patients on multivariate regression (p = 0.033). Female patients had higher motor coordination (p = 0.024). Breast-fed patients had better performance intelligence quotient (p = 0.024), visual motor integration (p = 0.014), and visual perception (p = 0.031). Adolescents who underwent cranial vault remodeling at two institutions had above average intelligence quotient scores, but worse mathematical and visual motor achievement compared with control subjects. Left-side craniosynostosis patients performed worse in spelling than right-side patients. Breast-feeding was an independent predictor for improved performance intelligence quotient, visual motor achievement, and visual perception performance. Study findings are limited by the cohort size. A larger population study is required, which could validate or modify the study conclusions.
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http://dx.doi.org/10.1097/PRS.0000000000007067DOI Listing
September 2020

What Is the Difference in Cranial Base Morphology in Isolated and Syndromic Bicoronal Synostosis?

Plast Reconstr Surg 2020 09;146(3):599-610

From the Division of Plastic and Reconstructive Surgery, Yale School of Medicine; the Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; the Department of Plastic and Reconstructive Surgery, Erasmus Medical Center; the Division of Plastic and Reconstructive Surgery, New York Medical College; the Division of Plastic Surgery, University of Connecticut School of Medicine; and the Department of Plastic Surgery, University of São Paulo.

Background: The association of isolated craniosynostosis and the influence of syndromic forms confound the understanding of craniofacial morphologic development. This study attempts to clarify the individual influences of isolated bicoronal synostosis, Apert syndrome, and Crouzon syndromes on skull base morphology.

Methods: One hundred seventeen computed tomographic scans were included (nonsyndromic bicoronal synostosis, n = 36; Apert syndrome with bicoronal synostosis, n = 25; Crouzon syndrome with bicoronal synostosis, n = 11; controls, n = 45). Cephalometric measurements were analyzed using Materialise software.

Results: Nonsyndromic bicoronal synostosis patients developed a shortened cranial base length, with a significantly shortened distance between nasion and sella (p = 0.005). The cranial base angles of nonsyndromic bicoronal synostosis in both the cranial side (N-S-BA) and facial side (N-SO-BA) increased significantly, by 17.04 degrees (p < 0.001) and 11.75 degrees (p < 0.001), respectively. However, both the N-S-BA and N-SO-BA angles of Apert syndrome and Crouzon syndrome were narrowed more than that of nonsyndromic bicoronal synostosis [by 12.11 degrees (p < 0.001) and 12.44 degrees (p < 0.001), respectively, in Apert syndrome; and by 11.66 degrees (p = 0.007) and 13.71 degrees (p = 0.007), respectively, in Crouzon syndrome]. However, there is no statistically significant difference of these two angles between Apert syndrome and Crouzon syndrome, when they were only associated with bicoronal synostosis. Contrary to the relatively normal subcranial space of nonsyndromic bicoronal synostosis, both Apert and Crouzon syndromes developed a reduced subcranial space.

Conclusions: Isolated bicoronal synostosis resulted in a flattened cranial base, whereas Apert syndrome and Crouzon syndrome developed a normal cranial base angle when only associated with bicoronal synostosis. The syndromic skulls had additional significantly reduced subcranial space.
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http://dx.doi.org/10.1097/PRS.0000000000007068DOI Listing
September 2020

Surgical management of lymphedema: a review of current literature.

Gland Surg 2020 Apr;9(2):503-511

Division of Plastic and Reconstructive Surgery, Yale University School of Medicine, New Haven, CT, USA.

Lymphedema may be characterized by a progressive clinical course and limitations in improvement despite multi-modality treatment. In westernized countries, it most commonly presents as an undesirable complication of cancer treatment, particularly breast cancer. In the past several decades, surgical treatments for lymphedema have advanced, alongside developments in microsurgery. Lymphovenous anastomosis (LVA) and lymph node transplantation are physiological therapies that may reduce lymphedema through addressing its route cause. Ablative techniques such as liposuction and subcutaneous excision aid in resolving the accumulation of proteinaceous adipose and fibrotic tissue seen in advanced lymphedema. The goal of this review is to examine the outcomes and limitations of current surgical techniques used in lymphedema management.
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http://dx.doi.org/10.21037/gs.2020.03.14DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225501PMC
April 2020

Radiographic Severity of Metopic Craniosynostosis Correlates with Long-Term Neurocognitive Outcomes.

Plast Reconstr Surg 2020 05;145(5):1241-1248

From the Department of Surgery, Section of Plastic and Reconstructive Surgery, Yale School of Medicine.

Background: Reports of neurodevelopmental delays in adolescents with metopic craniosynostosis have ranged from 15 to 61 percent. Previously, event-related potentials have correlated preoperative radiographic severity with language deficiencies in infancy. This study sought to characterize neurocognitive testing at cranial maturity and correlate outcomes to preoperative radiographic severity.

Methods: Patients diagnosed with metopic craniosynostosis who underwent surgical correction in infancy completed a neurodevelopmental battery evaluating age-normalized intelligence quotient, academic achievement, and visuomotor integration. Data were stratified by preoperative endocranial bifrontal angle (moderate, >124 degrees; severe, <124 degrees). Multiple variable regression was used to control measured intelligence and achievement for age at surgery, age at testing, parental education, and income. Significance was set at p < 0.05.

Results: Twenty patients completed neurodevelopmental testing. Mean intelligence quotient was 111.7 ± 13 and academic achievement was similar to national averages (word reading, 53.4 percent; reading comprehension, 53.4 percent; reading composite, 53.5 percent; spelling, 44 percent; and math, 52.9 percent). Radiographic measurements revealed 36 percent of patients with moderate phenotype and 64 percent with severe. Patients with severe phenotypes had lower intelligence quotient measures and scored more poorly in every academic measure tested. Word reading (113 versus 95; p = 0.035) and reading composite (109 versus 98; p = 0.014) reached significance.

Conclusions: Overall, cranial mature patients with metopic craniosynostosis had above average intelligence quotient and academic achievement near the national mean. Long-term neurocognitive function was correlated to preoperative radiographic severity in metopic craniosynostosis, with more severe cases performing worse.

Clinical Question/level Of Evidence: Risk, II.
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http://dx.doi.org/10.1097/PRS.0000000000006746DOI Listing
May 2020

Cranial Fossa Volume and Morphology Development in Apert Syndrome.

Plast Reconstr Surg 2020 04;145(4):790e-802e

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Background: Apert syndrome causes normal or enlarged intracranial volume overall as patients grow. This study aimed to trace the segmental anterior, middle, and posterior cranial fossae volume and structural morphology in these patients, to help discern a more focused and individualized surgical treatment plan for patients with Apert syndrome.

Methods: This study included 82 preoperative computed tomographic scans (Apert, n = 32; control, n = 50) divided into five age-related subgroups. The scans were measured using image processing and three-dimensional modeling software.

Results: The middle cranial fossa volume was increased and was the earliest change noted. It was increased by 45 percent (p = 0.023) compared with controls before 6 months of age and remained increased into adulthood (161 percent, p = 0.016), with gradually increasing severity. The anterior and posterior cranial fossae volumes also increased, by 35 percent (p = 0.032) and 39 percent (p = 0.007), respectively. Increased depth of cranial fossae contributed most to the increase in volumes of patients with Apert syndrome, with correlation coefficients of 0.799, 0.908, and 0.888 for anterior, middle, and posterior cranial fossa, respectively. The intracranial volume was increased 12 percent (p = 0.098) across the entire test age range (0 to 26 years old), but only had statistical significance during the age range of 6 to 18 years (22 percent, p = 0.001).

Conclusions: Malformation of the middle cranial fossa is an early, perhaps the initial, pivotal cranial morphologic change in Apert syndrome. Increased cranial fossae depth is an inherent characteristic of the maldevelopment. Normalization of cranial volume and circumference overall may not achieve a normal skull structure, as it does not correct regional craniocerebral disproportion.
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April 2020

Preferences of Transgender and Gender-Nonconforming Persons in Gender-Confirming Surgical Care: A Cross-Sectional Study.

Ann Plast Surg 2021 01;86(1):82-88

Yale University School of Medicine, New Haven, CT.

Introduction: Increased awareness for transgender and gender-nonconforming individuals may lead to increased demand for surgical interventions in gender-confirming care. However, limited literature exists regarding transgender and gender-nonconforming preferences and experiences with medical or surgical care. The authors aim to characterize the medical and surgical care sought by this population, as well as their surgical preferences, motivations, and barriers to care.

Methods: An online questionnaire about opinions and personal experiences with medical and surgical care during gender transition was publicized via regional online social networking forums in Connecticut and surrounding areas catering to transgender communities.

Results: Responses were received from 313 participants. Participants were 97% male gender at birth and 92% white with an average (SD) age of 51.6 (13.5) years. Fifty-nine percent identified as male-to-female transgender and 20% as gender nonconforming. Respondents were aware of their gender identity at a mean (SD) age of 9.6 (9.0) years, but did not begin transitioning until a mean (SD) age of 38.9 (20.8) years, with gender-nonconforming respondents choosing to transition at a significantly younger age as compared with transgender respondents (29.8 vs 41.4 years; P = 0.0061, unpaired t test). Only 42% of all respondents, with a significantly greater number of transgender as opposed to gender-nonconforming individuals, had previously met with a physician to discuss transitioning (49% vs 21%, P = 0.002, χ test). Eight percent of the study population had undergone gender confirmation surgery (GCS), 52% were interested in GCS, and 40% were not interested in GCS. Primary motivation for GCS included discomfort in one's current body (28%), and barriers to GCS included cost (40%) and reactions of family (40%), partners (32%), and friends (25%).

Conclusions: Transgender and gender-nonconforming individuals lack medical support for gender transition, with fewer than half of survey respondents reporting a prior meeting with a physician to discuss transitioning. The reported perspectives offer important insight into transgender preferences that should act as the basis of future efforts to improve the efficacy of gender-confirming care.
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January 2021

Assessing Facial Asymmetry in Postoperative Patients With Unilateral Coronal Craniosynostosis.

J Craniofac Surg 2020 Jun;31(4):1000-1005

Yale School of Medicine, Section of Plastic and Reconstructive Surgery, New Haven, CT.

Background: Despite surgical correction of unilateral craniosynostosis (ULC), complex cranial base angulation can result in partial reversion to preoperative deformity with growth and time. Using 3-dimensional imaging, dysmorphic facial features of ULC in school-age patients were quantified and related to how they contribute to overall facial asymmetry and patient-reported outcomes.

Methods: Children who underwent surgical correction of ULC were recruited from Yale University and Children's Hospital of Philadelphia. The 3D photographs were analyzed utilizing a Procrustes analysis of shape. Pearson's correlation was used to determine dysmorphic features' impact on overall asymmetry. Patients were stratified into "moderate" and "severe" asymmetry. Finally, asymmetry was correlated to patient-reported outcome scores. Statistical analysis was performed with SPSS-25 with P < 0.05 as statistically significant.

Results: Twenty-one patients were included with average age at analysis of 12.3 years. Fifty-seven percent of patients had right-sided fusion. The overall Procrustes analysis indicated a root mean square difference of 2.21 mm. Pearson's correlation indicated that the facial middle 3rd (P ≤ 0.001), orbital dystopia (P < 0.001), chin point deviation (P = 0.011), and nasal root angulation (P = 0.019) contributed most to overall asymmetry. Patients in the severe asymmetry cohort had greater facial middle-third asymmetry (P < 0.001) and orbital dystopia (P < 0.001). Asymmetry did not correlate with patient-reported outcomes.

Conclusion: Patients with ULC have persistent facial asymmetry at school-age with the greatest levels of asymmetry in the facial middle-third, orbit, and nasal root. Beyond the cranial dysmorphology, initial skull base angulation in unilateral coronal craniosynostosis manifests in long-term mid and lower-third facial asymmetry.
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http://dx.doi.org/10.1097/SCS.0000000000006355DOI Listing
June 2020

Reply: Airway Analysis in Apert Syndrome.

Plast Reconstr Surg 2020 03;145(3):661e-662e

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, Conn.

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March 2020

Classification of Subtypes of Crouzon Syndrome Based on the Type of Vault Suture Synostosis.

J Craniofac Surg 2020 May/Jun;31(3):678-684

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

Background: Patients with Crouzon syndrome develop various types of anatomic deformities due to different forms of craniosynostosis, yet they have similar craniofacial characteristics. However, exact homology is not evident. Different pathology then may be best treated by different forms of surgical technique. Therefore, precise classification of Crouzon syndrome, based on individual patterns of cranial suture involvement is needed.

Methods: Ninety-five computed tomography (CT) scans (Crouzon, n = 33; control, n = 62) were included in this study. All the CT scans are divided into 4 types based on premature closure of sutures: class I = coronal and lambdoidal synostosis; class II = sagittal synostosis; class III = pansynostosis; and class IV = "Others." The CT scan anatomy was measured by Materialise software.

Results: The class III, pansynostosis, is the most prevalent (63.6%). The classes I, III, and IV of Crouzon have significantly shortened entire anteroposterior cranial base length, with the shortest base length in class III. The external cranial measurements in class I show primarily a decreased posterior facial skeleton, while the class III presented with holistic facial skeleton reduction. Class II has the least severe craniofacial malformations, while class III had the most severe.

Conclusion: The morphology of patients with Crouzon syndrome is not identical in both cranial base and facial characteristics, especially when they associated with different subtypes of cranial suture synostosis. The classification of Crouzon syndrome proposed in this study, summarizes the differences among each subgroup of craniosynostosis suture involvement, which, theoretically, may ultimately influence both the timing and type of surgical intervention.
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September 2020

Intracranial hypertension and cortical thickness in syndromic craniosynostosis.

Dev Med Child Neurol 2020 07 14;62(7):799-805. Epub 2020 Feb 14.

Department of Plastic and Reconstructive Surgery, Erasmus Medical Center, Rotterdam, the Netherlands.

Aim: To evaluate the impact of risk factors for intracranial hypertension (ICH) on cerebral cortex thickness in syndromic craniosynostosis.

Method: ICH risk factors including papilloedema, hydrocephalus, obstructive sleep apnea (OSA), cerebellar tonsillar position, occipitofrontal circumference (OFC) curve deflection, age, and sex were collected from the records of patients with syndromic craniosynostosis (Apert, Crouzon, Pfeiffer, Muenke, Saethre-Chotzen syndromes) and imaging. Magnetic resonance images were analysed and exported for statistical analysis. A linear mixed model was developed to determine correlations with cerebral cortex thickness changes.

Results: In total, 171 scans from 107 patients (83 males, 88 females [including repeated scans], mean age 8y 10mo, range 1y 1mo-34y, SD 5y 9mo) were evaluated. Mean cortical thickness in this cohort was 2.78mm (SD 0.17). Previous findings of papilloedema (p=0.036) and of hydrocephalus (p=0.007) were independently associated with cortical thinning. Cortical thickness did not vary significantly by sex (p=0.534), syndrome (p=0.896), OSA (p=0.464), OFC (p=0.375), or tonsillar position (p=0.682).

Interpretation: Detection of papilloedema or hydrocephalus in syndromic craniosynostosis is associated with significant changes in cortical thickness, supporting the need for preventative rather than reactive treatment strategies.

What This Paper Adds: Papilloedema is associated with thinning of the cerebral cortex in syndromic craniosynostosis, independently of hydrocephalus.
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July 2020

Cranial Fossa Development in Differing Subtypes of Crouzon Syndrome.

J Craniofac Surg 2020 May/Jun;31(3):673-677

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

Background: Based on an established classification system of Crouzon syndrome subtypes, detailed regional morphology and volume analysis may be useful, to clarify Crouzon cranial structure characteristics, and the interaction between suture fusion and gene regulated overall growth of the calvarium and basicranium.

Methods: CT scans of 36 unoperated Crouzon syndrome patients and 56 controls were included and subgrouped as: type I. Bilateral coronal synostosis; type II. Sagittal synostosis; type III. Pansynostosis; type IV. Perpendicular combination synostosis.

Results: Type I of Crouzon syndrome patients developed a slightly smaller posterior fossa (22%), and increased superior cranial volume (13%), which is the only subtype that develops a greater superior cranial volume. The effect of competing increased and decreased segmental volume is associated with a 24% enlargement of overall cranial volume (P = 0.321). In class III, the anterior fossa volume was increased by 31% (P = 0.007), while the volume of posterior fossa was decreased by 19% (P < 0.001). These resulted in a 7% (P = 0.046) reduction in the overall intracranial volume. Type II and type IV patients developed a trend toward anterior, middle, and posterior fossae, and entire cranial volume reduction.

Conclusions: Pansynostosis is the most often form of associated craniosynostoses of Crouzon syndrome, however bilateral coronal synostosis may not dominate this form of Crouzon syndrome. The anterior, middle and posterior cranial fossae may have simultaneously reduced volume if the midline suture synostosis is involved. Individualized treatment planning for Crouzon syndrome patient, theoretically should include the patient's age and temporal associated maldevelopment suture sequence.
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http://dx.doi.org/10.1097/SCS.0000000000006181DOI Listing
September 2020

Frustration and Emotional Regulation in Nonsyndromic Craniosynostosis: A Functional Magnetic Resonance Imaging Study.

Plast Reconstr Surg 2019 12;144(6):1371-1383

From the Department of Surgery, Section of Plastic Surgery, the Department of Radiology, the Magnetic Resonance Research Center, and Yale Child Study Center, Yale School of Medicine.

Background: Nonsyndromic craniosynostosis may manifest with complex behavioral, attentional, and emotional sequelae. The authors characterized higher level brain connectivity in adolescent nonsyndromic craniosynostosis patients in response to emotional frustration.

Methods: Surgically corrected patients older than 9 years with nonsyndromic craniosynostosis were age/sex/handedness matched to controls. Patients participated in a "go/no-go" task, structured as win/lose/recovery paradigms. BioImage Suite was used to analyze whole-brain intrinsic connectivity between tasks with cluster-corrected group-level t maps. A value of p < 0.05 was significant.

Results: Seven unilateral coronal (average age, 12.2 years), six metopic (average age, 11.5 years), and controls were included. Unilateral coronal had worse emotional regulation scores on the Behavior Rating Inventory of Executive Function survey (p = 0.065) and performed poorly on the go/no-go task (p < 0.001). Metopic had four regions of interest, with the majority having decreased activity compared with controls, and few differences between tasks. Unilateral coronal patients had 11 regions of interest; the majority decreased during the win and lose conditions, but all increased during the recovery condition. Metopic patients had decreased blood oxygenation level- dependent signal in the posterior cingulate (p = 0.017) and middle temporal gyrus (p = 0.042). Unilateral coronal had decreased signal in the posterior cingulate (p = 0.023), middle temporal gyrus (p = 0.027), and thalamus (p = 0.033), but increased signal in the cuneus (p = 0.009) and cerebellum (p = 0.009). Right unilateral coronal, but not metopic/controls, had increased right brain activity in the caudate (p = 0.030), thalamus (p = 0.011), temporal lobe (p = 0.012), and cerebellum (p = 0.029).

Conclusions: Unilateral coronal patients may have emotional dysregulation in response to frustration, whereas metopic patients may have attenuated emotional reactions. Evidence of right unilateral coronal brain laterality suggests that the area of suture fusion may contribute to the mechanism of dysfunction.

Clinical Question/level Of Evidence: Risk, II.
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December 2019

Cranial Fossa Volume in Differing Subtypes of Apert Syndrome.

J Craniofac Surg 2019 Nov-Dec;30(8):2345-2349

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT.

Background: Based on an established classification system of Apert syndrome subtypes, detailed regional morphology and volume analysis may be useful to provide additional clarification to individual Apert cranial structure characteristics, and treatment planning.

Methods: Computed tomography scans of 32 unoperated Apert syndrome and 50 controls were included and subgrouped as: type I, bilateral coronal synostosis; type II, pansynostosis; type III, perpendicular combination synostosis. Three-dimensional analysis of craniometric points was used to define structural components using Materialise Mimics and 3-Matics software.

Results: Occipitofrontal circumference of all subtypes of Apert syndrome patients is normal. Intracranial volumes of types I and II were normal, but type III was 20% greater than controls. Middle cranial fossa volume was increased in all 3 types, with the greatest increase in type II (86%). Type II developed a 69% increase in anterior cranial fossa volume, whereas type III had 39% greater posterior cranial fossa volume. Increased cranial fossa depth contributed most to above increased volume. The anteroposterior lengths of middle and posterior cranial fossae were reduced in type I (15% and 17%, respectively). However, only the anterior cranial fossa was significantly shortened in type III.

Conclusions: Occipitofrontal circumference and overall intracranial volume is not always consistent in individual subunits of Apert syndrome. Detailed and segmental anterior, middle, and posterior cranial fossae volumes and morphology should be analyzed to see what impact this may have related to surgical planning.
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January 2020

Secondary Cleft Rhinoplasty in 1720 Patients: Are National Practices Consistent With Guidelines?

Cleft Palate Craniofac J 2020 04 8;57(4):438-443. Epub 2019 Oct 8.

Section of Plastic and Reconstructive Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT, USA.

Objective: To assess the timing, type, and associated adjunct procedures for secondary cleft rhinoplasty nationally.

Design: Data were extracted from a national database of all secondary cleft rhinoplasty procedures (Current Procedural Terminology [CPT] codes 30460 and 30462). Frequency statistics were utilized to analyze demographics, comorbidities, surgical procedures, and timing. Chi-squared analysis and Fisher exact test were used for analysis.

Setting: National Surgical Quality Improvement Program-Pediatric Database.

Participants: A total of 1720 patients met inclusion criteria for secondary cleft rhinoplasty repair.

Interventions: No relevant intervention.

Main Outcomes And Measures: Age, demographics, comorbidities, and associated procedures.

Results: Over 5 consecutive years, 1720 patients underwent secondary cleft lip rhinoplasty nationally. Mean patient age was 9.3 ± 5.3 years. Unilateral cleft rhinoplasty patients were older (9.0 years) than bilateral patients (7.8 years; = .001). Rib grafting was performed in 6.3% of patients at a mean age of 10.6 years with a higher proportion of Asian and female patients. Auricular grafts were more commonly performed by otolaryngology than plastic surgery. The most common adjunct procedures included secondary cleft lip revision (33.1%) and tympanostomy tube placement (10.2%). When subdividing by type of cleft rhinoplasty, tip rhinoplasty was performed at a mean age of 7.3 years compared to rhinoplasty with osteotomies and a major septal component at 12.1 years ( < .001).

Conclusions: This study reveals that a large proportion of cleft rhinoplasties are performed in skeletally immature patients. Although patients undergoing rib grafting, nasal osteotomies, and a major septal component were older, these procedures are still performed in a large proportion of patients who are younger than expected.
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April 2020

SMAD6 Genotype Predicts Neurodevelopment in Nonsyndromic Craniosynostosis.

Plast Reconstr Surg 2020 01;145(1):117e-125e

From the Department of Surgery, Section of Plastic Surgery, Yale School of Medicine.

Background: De novo or rare transmitted mutations in the SMAD6 gene affect 7 percent of midline nonsyndromic synostosis patients. This study aimed to determine the neurocognitive sequelae of SMAD6 synostosis.

Methods: Nonsyndromic synostosis patients 6 years or older with SMAD6 mutations and non-SMAD6 nonsyndromic synostosis controls were recruited. All patients completed a double-blinded neurodevelopmental battery (i.e., Wechsler Fundamentals, Wechsler Abbreviated Scale of Intelligence, Beery-Buktenica Developmental test), and parents/guardians completed behavioral surveys (Behavior Rating Inventory of Executive Function and Behavior Rating System for Children).

Results: Twenty-eight patients participated: 10 known SMAD6 patients (average age, 10 years; 1 female; eight metopic and two sagittal; nine treated with cranial vault remodeling and one treated with strip craniectomy) and 18 non-SMAD6 controls (age, 9.5 years; three female; 12 metopic and six sagittal; 17 treated with cranial vault remodeling and one treated with strip craniectomy). There were no differences between any demographics. Testing age, surgical age, parental education, and household income correlated with cognition (p < 0.05). After controlling for these factors, SMAD6 patients performed worse on numerical operations (p = 0.046), performance intelligence quotient (p = 0.018), full-scale intelligence quotient (p = 0.010), and motor coordination (p = 0.043) compared to age/race/gender/synostosis/operation-matched controls. On behavioral surveys, SMAD6 patients scored worse on 14 assessments, including aggression, communication, and behavior.

Conclusions: This prospective double-blinded study revealed that neuropsychiatric development of nonsyndromic synostosis may be under genetic control. SMAD6 mutations led to poorer mathematics, performance intelligence quotient, full-scale intelligence quotient, and motor coordination, even after controlling for exogenous factors. Genetic testing may be critical for advocating early adjunctive neurodevelopmental therapy.

Clinical Question/level Of Evidence: Risk, II.
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January 2020

Airway Analysis in Apert Syndrome.

Plast Reconstr Surg 2019 09;144(3):704-709

From the Division of Plastic Surgery, Mayo Clinic; the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; the Division of Plastic and Reconstructive Surgery, Yale School of Medicine; and the Department of Plastic Surgery, University of São Paulo.

Background: Apert syndrome is frequently combined with respiratory insufficiency, because of the midfacial deformity which, in turn, is influenced by the malformation of the skull base. Respiratory impairment resulting from Apert syndrome is caused by multilevel limitations in airway space. Therefore, this study evaluated the segmented nasopharyngeal and laryngopharyngeal anatomy to clarify subcranial anatomy in children with Apert syndrome and its relevance to clinical management.

Methods: Twenty-seven patients (Apert syndrome, n = 10; control, n = 17) were included. All of the computed tomographic scans were obtained from the patients preoperatively, and no patient had confounding disease comorbidity. Computed tomographic scans were analyzed using Surgicase CMF. Craniometric data relating to the midface, airway, and subcranial structures were collected. Statistical significance was determined using t test analysis.

Results: Although all of the nasal measurements were consistent with those of the controls, the nasion-to-posterior nasal spine, sphenethmoid-to-posterior nasal spine, sella-to-posterior nasal spine, and basion-to-posterior nasal spine distances were decreased 20 (p < 0.001), 23 (p = 0.001), 29 (p < 0.001), and 22 percent (p < 0.001), respectively. The distance between bilateral gonions and condylions was decreased 17 (p = 0.017) and 18 percent (p = 0.004), respectively. The pharyngeal airway volume was reduced by 40 percent (p = 0.01).

Conclusion: The airway compromise seen in patients with Apert syndrome is attributable more to the pharyngeal region than to the nasal cavity, with a gradually worsening trend from the anterior to the posterior airway, resulting in a significantly reduced volume in the hypopharynx.
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September 2019