Purpose: Craniosynostosis (CS) treatment is complex and varies widely. Large-scale outcome studie... more Purpose: Craniosynostosis (CS) treatment is complex and varies widely. Large-scale outcome studies are difficult given the practice variation, low incidence of disease, and long time between intervention and final outcome. Established in 2016, the Synostosis Research Group (SynRG) is the largest multi-center consortium focused on prospectively evaluating the diagnosis and management of patients with CS. Here we present a preliminary analysis of these data.
Supplemental Digital Content is available in the text Introduction: In this study, the authors se... more Supplemental Digital Content is available in the text 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.
Plastic and Reconstructive Surgery - Global Open, 2020
cancer (n=2, 4%), implant rupture (n=2, 4%), and postoperative infections (n=2, 4%). Single cases... more cancer (n=2, 4%), implant rupture (n=2, 4%), and postoperative infections (n=2, 4%). Single cases (n=1, 2% each) of leukemia, small bowel cancer, lung disease, pneumonia, autoimmune and joint disease, amyotrophic lateral sclerosis, liver failure, and sudden death, as well as 2 cases (4%) of newborn deaths, to mothers with breast implants, were also identified. A literature review demonstrated that 54% of alleged implant-related deaths were not truly associated with breast implant use: the majority of these reports (82%) originated from the public and third-party sources, rather than evidence-based reports by healthcare professionals and journal articles.
Background: Little is known about the detailed growth of the cranial fossae, even though they pro... more Background: Little is known about the detailed growth of the cranial fossae, even though they provide an important structural connection between the cranial vault and the facial skeleton. This study details the morphologic development of isolated cranial vault synostosis and associated syndromes on cranial fossa development. Methods: A total of 125 computed tomographic scans were included (nonsyndromic bicoronal synostosis, n = 36; Apert syndrome associated with bicoronal synostosis, n = 24; Crouzon syndrome associated with bicoronal synostosis, n = 11; and controls, n = 54). Three-dimensional analyses were produced using Materialise software. Results: The regional anterior and middle cranial fossae volumes of nonsyndromic bicoronal synostosis are characterized by significant increases of 43 percent (p < 0.001) and 60 percent (p < 0.001), respectively, and normal posterior cranial fossa volume. The cranial fossae depths of nonsyndromic bicoronal synostosis were increased, by 37, 42, and 21 percent (all p < 0.001) for anterior, middle, and posterior cranial fossae, respectively, accompanying the shortened cranial fossae lengths. The volume and morphology of all cranial fossae in Apert syndrome nearly paralleled nonsyndromic bicoronal synostosis. However, Crouzon syndrome had reduced depths of cranial fossae, and more restricted fossa volumes than both Apert syndrome and nonsyndromic bicoronal synostosis. Conclusions: Cranial vault suture synostosis is likely to be more influential on cranial fossae development than other associated influences (genetic, morphologic) in Apert and Crouzon syndromes. Isolated Apert syndrome pathogenesis is associated with an elongation of the anterior cranial fossa length in infants, whereas in Crouzon syndrome, there is a tendency to reduce cranial fossa depth, suggesting individual adaptability in cranial fossae development related to vault synostosis.
Surgical treatment for tumors of the skull base remains gross total resection with microscopic ne... more Surgical treatment for tumors of the skull base remains gross total resection with microscopic negative margins. Sufficient surgical access is paramount to adequate treatment but must be balanced with patient morbidity and protection of vital neurovascular structures. While endoscopic surgery has made transfacial access less common, there are still indications for open transfacial exposure including tumors that involve facial soft tissues, the palate, anterolateral frontal sinus, dural involvement lateral to the mid-pupillary line, tumor recurrence/repeat resection, and/or lack of access to endoscopic equipment or expertise. The authors present a ‘‘line-of-sight’’ algorithm for selection of approach, discuss pre-operative planning, review selected clinical From the Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT. Received December 28, 2020. Accepted for publication January 2, 2021. Address correspondence and reprint requests to John A. Persing,...
Background: Little is known about the detailed growth of the sphenoidal and temporal bones, even ... more 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 ro...
Background: Based on an established classification system of Crouzon syndrome subtypes, detailed ... more 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),...
Background From infancy to adulthood, the mandible develops increased ramus height, prominence of... more Background From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. Methods One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analy...
Craniosynostosis is one of the most common craniofacial conditions treated by neurologic and plas... more Craniosynostosis is one of the most common craniofacial conditions treated by neurologic and plastic surgeons. In addition to disfigurement, children with craniosynostosis experience significant cognitive dysfunction later in life. Surgery is performed in infancy to correct skull deformity; however, the field is at a crossroads regarding the best approach for correction. Since the cause of brain dysfunction in these patients has remained uncertain, the role and type of surgery might have in attenuating the later-observed cognitive deficits through impact on the brain has been unclear. Recently, however, advances in imaging such as event-related potentials, diffusion tensor imaging, and functional MRI, in conjunction with more robust clinical studies, are providing important insight into the potential etiologies of brain dysfunction in syndromic and nonsyndromic craniosynostosis patients. This review aims to outline the cause(s) of such brain dysfunction including the role extrinsic ...
A report published by the Institute of Medicine in 1999 indicated that medical error kills betwee... more A report published by the Institute of Medicine in 1999 indicated that medical error kills between 44,000 and 98,000 inpatients each year. 1 A significant portion of these deaths relate to human error; however, it is not clear from the study how many of these human errors relate to ...
Journal of Vascular and Interventional Radiology, 2000
To assess the long-term efficacy of embolotherapy in combination with surgery for management of s... more To assess the long-term efficacy of embolotherapy in combination with surgery for management of symptomatic high-flow arteriovenous malformations (HFAVMs) of the lower and upper extremities. Twenty consecutive patients with symptomatic high-flow lower extremity AVMs (LE-AVMs; n = 9) and upper extremity AVMs (UE-AVMs; n = 11) were treated from 1982 to 1999. All nine patients with LE-AVM had pain and seven had ulceration of the skin. All 11 patients with UE-AVM had debilitating pain, seven had weakness of the affected hand, and two had bony erosion. Embolization of the nidus beneath the site of maximum pain or ulceration was performed percutaneously from the femoral artery through coaxially placed microcatheters (n = 18) or surgical cutdown (n = 2). Cyanoacrylate (isobutyl or n-butyl) diluted with iophendylate or ethiodized oil was used in 19 of 20 patients. Follow-up was completed in eight of nine patients with LE-AVM (mean, 8.6 y) and nine of 11 patients with UE-AVM (mean, 7.4 y) after treatment. One patient with localized LE-AVM was functioning well 13 years after embolotherapy and another was functioning well 16 years after undergoing three embolotherapy procedures and two skin grafts. Five of nine patients with LE-AVM required below-the-knee (n = 4) or above-the-knee (n = 1) amputation 1-6 years after technically and clinically successful embolotherapy. All three trifurcation arteries were diffusely involved in HFAVM in patients requiring amputation. Healing of the two amputation sites, involved by AVM at the knee, was excellent after preoperative geniculate artery embolotherapy. All 11 patients with UE-AVM experienced marked symptomatic improvement; seven after embolotherapy alone and the other four after resection of AVM. One complication of digital spasm was reversed by administration of nerve blocks. LE-AVM with diffuse involvement of all three trifurcation arteries ultimately required amputation because of recurrence of symptoms after technically and clinically successful embolotherapy. Cyanoacrylate embolotherapy alone or in combination with surgical resection of the AVM provided excellent long-term palliation in patients with UE-AVM.
Depression of profile in the temporal region is commonly seen after orbital rim advancement proce... more Depression of profile in the temporal region is commonly seen after orbital rim advancement procedures. A newly developed temporalis musculoosseous flap has been designed with the intent to prevent this postoperative occurrence.
Purpose: Craniosynostosis (CS) treatment is complex and varies widely. Large-scale outcome studie... more Purpose: Craniosynostosis (CS) treatment is complex and varies widely. Large-scale outcome studies are difficult given the practice variation, low incidence of disease, and long time between intervention and final outcome. Established in 2016, the Synostosis Research Group (SynRG) is the largest multi-center consortium focused on prospectively evaluating the diagnosis and management of patients with CS. Here we present a preliminary analysis of these data.
Supplemental Digital Content is available in the text Introduction: In this study, the authors se... more Supplemental Digital Content is available in the text 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.
Plastic and Reconstructive Surgery - Global Open, 2020
cancer (n=2, 4%), implant rupture (n=2, 4%), and postoperative infections (n=2, 4%). Single cases... more cancer (n=2, 4%), implant rupture (n=2, 4%), and postoperative infections (n=2, 4%). Single cases (n=1, 2% each) of leukemia, small bowel cancer, lung disease, pneumonia, autoimmune and joint disease, amyotrophic lateral sclerosis, liver failure, and sudden death, as well as 2 cases (4%) of newborn deaths, to mothers with breast implants, were also identified. A literature review demonstrated that 54% of alleged implant-related deaths were not truly associated with breast implant use: the majority of these reports (82%) originated from the public and third-party sources, rather than evidence-based reports by healthcare professionals and journal articles.
Background: Little is known about the detailed growth of the cranial fossae, even though they pro... more Background: Little is known about the detailed growth of the cranial fossae, even though they provide an important structural connection between the cranial vault and the facial skeleton. This study details the morphologic development of isolated cranial vault synostosis and associated syndromes on cranial fossa development. Methods: A total of 125 computed tomographic scans were included (nonsyndromic bicoronal synostosis, n = 36; Apert syndrome associated with bicoronal synostosis, n = 24; Crouzon syndrome associated with bicoronal synostosis, n = 11; and controls, n = 54). Three-dimensional analyses were produced using Materialise software. Results: The regional anterior and middle cranial fossae volumes of nonsyndromic bicoronal synostosis are characterized by significant increases of 43 percent (p < 0.001) and 60 percent (p < 0.001), respectively, and normal posterior cranial fossa volume. The cranial fossae depths of nonsyndromic bicoronal synostosis were increased, by 37, 42, and 21 percent (all p < 0.001) for anterior, middle, and posterior cranial fossae, respectively, accompanying the shortened cranial fossae lengths. The volume and morphology of all cranial fossae in Apert syndrome nearly paralleled nonsyndromic bicoronal synostosis. However, Crouzon syndrome had reduced depths of cranial fossae, and more restricted fossa volumes than both Apert syndrome and nonsyndromic bicoronal synostosis. Conclusions: Cranial vault suture synostosis is likely to be more influential on cranial fossae development than other associated influences (genetic, morphologic) in Apert and Crouzon syndromes. Isolated Apert syndrome pathogenesis is associated with an elongation of the anterior cranial fossa length in infants, whereas in Crouzon syndrome, there is a tendency to reduce cranial fossa depth, suggesting individual adaptability in cranial fossae development related to vault synostosis.
Surgical treatment for tumors of the skull base remains gross total resection with microscopic ne... more Surgical treatment for tumors of the skull base remains gross total resection with microscopic negative margins. Sufficient surgical access is paramount to adequate treatment but must be balanced with patient morbidity and protection of vital neurovascular structures. While endoscopic surgery has made transfacial access less common, there are still indications for open transfacial exposure including tumors that involve facial soft tissues, the palate, anterolateral frontal sinus, dural involvement lateral to the mid-pupillary line, tumor recurrence/repeat resection, and/or lack of access to endoscopic equipment or expertise. The authors present a ‘‘line-of-sight’’ algorithm for selection of approach, discuss pre-operative planning, review selected clinical From the Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT. Received December 28, 2020. Accepted for publication January 2, 2021. Address correspondence and reprint requests to John A. Persing,...
Background: Little is known about the detailed growth of the sphenoidal and temporal bones, even ... more 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 ro...
Background: Based on an established classification system of Crouzon syndrome subtypes, detailed ... more 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),...
Background From infancy to adulthood, the mandible develops increased ramus height, prominence of... more Background From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. Methods One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analy...
Craniosynostosis is one of the most common craniofacial conditions treated by neurologic and plas... more Craniosynostosis is one of the most common craniofacial conditions treated by neurologic and plastic surgeons. In addition to disfigurement, children with craniosynostosis experience significant cognitive dysfunction later in life. Surgery is performed in infancy to correct skull deformity; however, the field is at a crossroads regarding the best approach for correction. Since the cause of brain dysfunction in these patients has remained uncertain, the role and type of surgery might have in attenuating the later-observed cognitive deficits through impact on the brain has been unclear. Recently, however, advances in imaging such as event-related potentials, diffusion tensor imaging, and functional MRI, in conjunction with more robust clinical studies, are providing important insight into the potential etiologies of brain dysfunction in syndromic and nonsyndromic craniosynostosis patients. This review aims to outline the cause(s) of such brain dysfunction including the role extrinsic ...
A report published by the Institute of Medicine in 1999 indicated that medical error kills betwee... more A report published by the Institute of Medicine in 1999 indicated that medical error kills between 44,000 and 98,000 inpatients each year. 1 A significant portion of these deaths relate to human error; however, it is not clear from the study how many of these human errors relate to ...
Journal of Vascular and Interventional Radiology, 2000
To assess the long-term efficacy of embolotherapy in combination with surgery for management of s... more To assess the long-term efficacy of embolotherapy in combination with surgery for management of symptomatic high-flow arteriovenous malformations (HFAVMs) of the lower and upper extremities. Twenty consecutive patients with symptomatic high-flow lower extremity AVMs (LE-AVMs; n = 9) and upper extremity AVMs (UE-AVMs; n = 11) were treated from 1982 to 1999. All nine patients with LE-AVM had pain and seven had ulceration of the skin. All 11 patients with UE-AVM had debilitating pain, seven had weakness of the affected hand, and two had bony erosion. Embolization of the nidus beneath the site of maximum pain or ulceration was performed percutaneously from the femoral artery through coaxially placed microcatheters (n = 18) or surgical cutdown (n = 2). Cyanoacrylate (isobutyl or n-butyl) diluted with iophendylate or ethiodized oil was used in 19 of 20 patients. Follow-up was completed in eight of nine patients with LE-AVM (mean, 8.6 y) and nine of 11 patients with UE-AVM (mean, 7.4 y) after treatment. One patient with localized LE-AVM was functioning well 13 years after embolotherapy and another was functioning well 16 years after undergoing three embolotherapy procedures and two skin grafts. Five of nine patients with LE-AVM required below-the-knee (n = 4) or above-the-knee (n = 1) amputation 1-6 years after technically and clinically successful embolotherapy. All three trifurcation arteries were diffusely involved in HFAVM in patients requiring amputation. Healing of the two amputation sites, involved by AVM at the knee, was excellent after preoperative geniculate artery embolotherapy. All 11 patients with UE-AVM experienced marked symptomatic improvement; seven after embolotherapy alone and the other four after resection of AVM. One complication of digital spasm was reversed by administration of nerve blocks. LE-AVM with diffuse involvement of all three trifurcation arteries ultimately required amputation because of recurrence of symptoms after technically and clinically successful embolotherapy. Cyanoacrylate embolotherapy alone or in combination with surgical resection of the AVM provided excellent long-term palliation in patients with UE-AVM.
Depression of profile in the temporal region is commonly seen after orbital rim advancement proce... more Depression of profile in the temporal region is commonly seen after orbital rim advancement procedures. A newly developed temporalis musculoosseous flap has been designed with the intent to prevent this postoperative occurrence.
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