Cranial bone thickness and density anomalies quantified from CT images can identify chronic increased intracranial pressure.

PURPOSE: The diagnosis of chronic increased intracranial pressure (IIP)is often based on subjective evaluation or clinical metrics with low predictive value. We aimed to quantify cranial bone changes associated with pediatric IIP using CT images and to identify patients at risk. METHODS: We retrospectively quantified local cranial bone thickness and mineral density from the CT images of children with chronic IIP and compared their statistical differences to normative children without IIP adjusting for age, sex and image resolution. Subsequently, we developed a classifier to identify IIP based on these measurements. Finally, we demonstrated our methods to explore signs of IIP in patients with non-syndromic sagittal craniosynostosis (NSSC). RESULTS: We quantified a significant decrease of bone density in 48 patients with IIP compared to 1,018 normative subjects (P < .001), but no differences in bone thickness (P = .56 and P = .89 for age groups 0-2 and 2-10 years, respectively). Our classifier demonstrated 83.33% (95% CI: 69.24%, 92.03%) sensitivity and 87.13% (95% CI: 84.88%, 89.10%) specificity in identifying patients with IIP. Compared to normative subjects, 242 patients with NSSC presented significantly lower cranial bone density (P < .001), but no differences were found compared to patients with IIP (P = .57). Of patients with NSSC, 36.78% (95% CI: 30.76%, 43.22%) presented signs of IIP. CONCLUSION: Cranial bone changes associated with pediatric IIP can be quantified from CT images to support earlier diagnoses of IIP, and to study the presence of IIP secondary to cranial pathology such as non-syndromic sagittal craniosynostosis.

What to do with an incidental finding of a fused sagittal suture: a modified Delphi study.

OBJECTIVE: As many as 5% of normocephalic children may have a prematurely fused sagittal suture, yet the clinical significance and best course of management of this finding remain unclear. Providers in the Synostosis Research Group were surveyed to create a multicenter consensus on an optimal treatment and monitoring algorithm for this condition. METHODS: A four-round modified Delphi method was utilized. The first two rounds consisted of anonymous surveys distributed to 10 neurosurgeons and 9 plastic surgeons with expertise in craniosynostosis across 9 institutions, and presented 3 patients (aged 3 years, 2 years, and 2 months) with incidentally discovered fused sagittal sutures, normal cephalic indices, and no parietal dysmorphology. Surgeons were queried about their preferred term for this entity and how best to manage these patients. Results were synthesized to create a treatment algorithm. The third and fourth feedback rounds consisted of open discussion of the algorithm until no further concerns arose. RESULTS: Most surgeons preferred the term "premature fusion of the sagittal suture" (93%). At the conclusion of the final round, all surgeons agreed to not operate on the 3- and 2-year-old patients unless symptoms of intracranial hypertension or papilledema were present. In contrast, 50% preferred to operate on the 2-month-old. However, all agreed to utilize shared decision-making, taking into account any concerns about future head shape and neurodevelopment. Panelists agreed that patients over 18 months of age without signs or symptoms suggesting elevated intracranial pressure (ICP) should not undergo surgical treatment. CONCLUSIONS: Through the Delphi method, a consensus regarding management of premature fusion of the sagittal suture was obtained from a panel of North American craniofacial surgeons. Without signs or symptoms of ICP elevation, surgery is not recommended in patients over 18 months of age. However, for children younger than 18 months, surgery should be discussed with caregivers using a shared decision-making process.

Intraoperative Anaphylaxis to Gelatin during Alveolar Bone Grafting for Cleft Palate.

Intraoperative anaphylaxis can be life threatening. Anaphylaxis to gelatin-based topical hemostatic agents is an underrecognized hypersensitivity. To date, only 21 cases of intraoperative anaphylaxis have been reported for gelatin-based hemostatic agents. In this article, we report the case of a 10-year-old male patient who sustained anaphylaxis after the use of Gelfoam during harvest of a bone graft. Rapid diagnosis and treatment of intraoperative anaphylaxis is imperative to prevent adverse outcomes. Referral to an allergist for identification of the allergen and appropriate notation in the medical record are paramount to avoid future anaphylactic events. Surgeons should avoid gelatin-based hemostatic agents, such as Gelfoam, in patients with reported intolerance of gelatin-based foods and medicines.

Repair of an Occipital Meningocele and Scalp Soft-tissue Reconstruction in a Newborn Patient.

The differential diagnosis of large congenital scalp defects includes aplasia cutis and encephalocele, among others. Treatment includes conservative management with dressings or operative management with dermal substitutes, skin grafting, local flaps, and free flaps. This case report discusses the technical considerations and reconstructive strategies for repair of a meningocele in a newborn with a large 5.5-cm scalp defect. The key strategies include preemptive cerebrospinal fluid (CSF) diversion with external ventricular drain to reduce the risk of CSF leak and mitigate wound-healing complications; careful identification and avoidance of key anatomic structures, such as the superior sagittal sinus, as anatomy may be significantly distorted due to the presence of a meningocele and after CSF diversion; and careful, thoughtful design of the local scalp flaps to maximize blood supply and to avoid tension on the final reconstruction.

Data-driven cranial suture growth model enables predicting phenotypes of craniosynostosis.

We present the first data-driven pediatric model that explains cranial sutural growth in the pediatric population. We segmented the cranial bones in the neurocranium from the cross-sectional CT images of 2068 normative subjects (age 0-10 years), and we used a 2D manifold-based cranial representation to establish local anatomical correspondences between subjects guided by the location of the cranial sutures. We designed a diffeomorphic spatiotemporal model of cranial bone development as a function of local sutural growth rates, and we inferred its parameters statistically from our cross-sectional dataset. We used the constructed model to predict growth for 51 independent normative patients who had longitudinal images. Moreover, we used our model to simulate the phenotypes of single suture craniosynostosis, which we compared to the observations from 212 patients. We also evaluated the accuracy predicting personalized cranial growth for 10 patients with craniosynostosis who had pre-surgical longitudinal images. Unlike existing statistical and simulation methods, our model was inferred from real image observations, explains cranial bone expansion and displacement as a consequence of sutural growth and it can simulate craniosynostosis. This pediatric cranial suture growth model constitutes a necessary tool to study abnormal development in the presence of cranial suture pathology.

Geometric learning and statistical modeling for surgical outcomes evaluation in craniosynostosis using 3D photogrammetry.

BACKGROUND AND OBJECTIVE: Accurate and repeatable detection of craniofacial landmarks is crucial for automated quantitative evaluation of head development anomalies. Since traditional imaging modalities are discouraged in pediatric patients, 3D photogrammetry has emerged as a popular and safe imaging alternative to evaluate craniofacial anomalies. However, traditional image analysis methods are not designed to operate on unstructured image data representations such as 3D photogrammetry. METHODS: We present a fully automated pipeline to identify craniofacial landmarks in real time, and we use it to assess the head shape of patients with craniosynostosis using 3D photogrammetry. To detect craniofacial landmarks, we propose a novel geometric convolutional neural network based on Chebyshev polynomials to exploit the point connectivity information in 3D photogrammetry and quantify multi-resolution spatial features. We propose a landmark-specific trainable scheme that aggregates the multi-resolution geometric and texture features quantified at every vertex of a 3D photogram. Then, we embed a new probabilistic distance regressor module that leverages the integrated features at every point to predict landmark locations without assuming correspondences with specific vertices in the original 3D photogram. Finally, we use the detected landmarks to segment the calvaria from the 3D photograms of children with craniosynostosis, and we derive a new statistical index of head shape anomaly to quantify head shape improvements after surgical treatment. RESULTS: We achieved an average error of 2.74 ± 2.70 mm identifying Bookstein Type I craniofacial landmarks, which is a significant improvement compared to other state-of-the-art methods. Our experiments also demonstrated a high robustness to spatial resolution variability in the 3D photograms. Finally, our head shape anomaly index quantified a significant reduction of head shape anomalies as a consequence of surgical treatment. CONCLUSION: Our fully automated framework provides real-time craniofacial landmark detection from 3D photogrammetry with state-of-the-art accuracy. In addition, our new head shape anomaly index can quantify significant head phenotype changes and can be used to quantitatively evaluate surgical treatment in patients with craniosynostosis.

Joint Cranial Bone Labeling and Landmark Detection in Pediatric CT Images Using Context Encoding.

Image segmentation, labeling, and landmark detection are essential tasks for pediatric craniofacial evaluation. Although deep neural networks have been recently adopted to segment cranial bones and locate cranial landmarks from computed tomography (CT) or magnetic resonance (MR) images, they may be hard to train and provide suboptimal results in some applications. First, they seldom leverage global contextual information that can improve object detection performance. Second, most methods rely on multi-stage algorithm designs that are inefficient and prone to error accumulation. Third, existing methods often target simple segmentation tasks and have shown low reliability in more challenging scenarios such as multiple cranial bone labeling in highly variable pediatric datasets. In this paper, we present a novel end-to-end neural network architecture based on DenseNet that incorporates context regularization to jointly label cranial bone plates and detect cranial base landmarks from CT images. Specifically, we designed a context-encoding module that encodes global context information as landmark displacement vector maps and uses it to guide feature learning for both bone labeling and landmark identification. We evaluated our model on a highly diverse pediatric CT image dataset of 274 normative subjects and 239 patients with craniosynostosis (age 0.63 ± 0.54 years, range 0-2 years). Our experiments demonstrate improved performance compared to state-of-the-art approaches.

Fusion of Lateral Calvarial Sutures on Volume-Rendered Computed Tomography Reconstructions in Patients With Known Craniosynostosis.

INTRODUCTION: After treating a child with familial sagittal craniosynostosis, clinocephaly, and bilateral parietomastoid/posterior squamosal suture fusion, the authors wondered if major-suture synostosis and clinocephaly were associated with abnormal fusion of minor lateral calvarial sutures. METHODS: The authors reviewed all preoperative volume-rendered head computed tomography reconstructions performed for craniosynostosis at their institution from 2010 through 2014 and determined whether the sphenoparietal, squamosal, and parietomastoid sutures were open, partially fused, or fused. The authors determined whether any sutures were abnormally fused based upon a previous study from their center, in which abnormal fusion was defined as either 1 of 3 abnormal fusion patterns or abnormally-early fusion. The authors then determined the rate of abnormal fusion of these sutures and whether abnormal fusion was associated with (1) major-suture craniosynostosis, (2) type of craniosynostosis (sutures involved; single-suture versus multisuture; syndromic versus nonsyndromic), and (3) clinocephaly. RESULTS: In 97 included children, minor lateral sutures were abnormally fused in 8, or 8.2%, which was significantly higher than in children without craniosynostosis from our earlier study. Abnormal minor lateral suture fusion was not associated with the type of single-suture synostosis or with multisuture synostosis but was associated with syndromic synostosis. Four of 8 children with abnormal minor lateral suture fusion had multisuture synostosis and 6 had syndromic synostosis. Lateral sutures were abnormally fused in 1 of 4 subjects with clinocephaly, which was not significant. CONCLUSION: Abnormal minor lateral calvarial suture fusion is significantly associated with major-suture craniosynostosis, especially syndromic synostosis.

Data-driven Normative Reference of Pediatric Cranial Bone Development.

Available normative references of cranial bone development and suture fusion are incomplete or based on simplified assumptions due to the lack of large datasets. We present a fully data-driven normative model that represents the age- and sex-specific variability of bone shape, thickness, and density between birth and 10 years of age at every location of the calvaria. Methods: The model was built using a cross-sectional and multi-institutional pediatric computed tomography image dataset with 2068 subjects without cranial pathology (age 0-10 years). We combined principal component analysis and temporal regression to build a statistical model of cranial bone development at every location of the calvaria. We studied the influences of sex on cranial bone growth, and our bone density model allowed quantifying for the first time suture fusion as a continuous temporal process. We evaluated the predictive accuracy of our model using an independent longitudinal image dataset of 51 subjects. Results: Our model achieved temporal predictive errors of 2.98 ± 0.69 mm, 0.27 ± 0.29 mm, and 76.72 ± 91.50 HU in cranial bone shape, thickness, and mineral density changes, respectively. Significant sex differences were found in intracranial volume and bone surface areas (P < 0.01). No significant differences were found in cephalic index, bone thickness, mineral density, or suture fusion. Conclusions: We presented the first pediatric age- and sex-specific statistical reference for local cranial bone shape, thickness, and mineral density changes. We showed its predictive accuracy using an independent longitudinal dataset, we studied developmental differences associated with sex, and we quantified suture fusion as a continuous process.

Evaluation of skin care concerns and patient's perception of the effect of NanoSilk Cream on facial skin.

BACKGROUND: Skin aging is an inevitable process with one of the key features of aging being dryness or flakiness of the skin. Previous in vivo and in vitro testing has highlighted that a silk-based product may be effective in improving moisture retention in skin. METHODS: We evaluated the safety and efficacy of our silk-based product through a combination of objective- including scanning electron microscopy (SEM) and EpiDerm Skin Irritation tests - and subjective tests - including direct evaluation of patient's own perception of their skin. RESULTS: In alignment with previous studies, patients reported significant concerns about aging, wrinkling, or saggy skin. We found that our silk-based product was safe and effective in improving hydration and resilience of facial skin and a majority of participants stated they would continue to use this product, when commercially available. CONCLUSION: Our novel silk-based product, NanoSilk Cosmo, is safe for use on human facial skin and it improves skin resiliency and hydration.

Vascularized Pericranial Flap as a Method to Prevent Persistent Skull Defects After Craniectomy for Sagittal Synostosis.

ABSTRACT: Some cranial defects resulting from sagittal craniectomy for craniosynostosis never completely close and require cranioplasty. This study evaluates the results of 2 methods to minimize such defects: (1) trapezoidal craniectomy that is narrower posteriorly (2) vascularized pericranial flap that is sewn to the dura under a rectangular craniectomy.Children who underwent primary open sagittal craniectomy with biparietal morcellation (with/without frontal cranioplasty) for single-suture nonsyndromic sagittal synostosis from 2013 through 2018 were included. Children were excluded if there was a dural tear, if they had no 1-year follow-up, or if they had unmeasured and/or uncounted skull defects. Surgeries were divided into (1) standard craniectomy, (2) trapezoidal craniectomy, or (3) craniectomy with pericranial flap. Differences in percentage of children with defects and mean total defect area 1 year postsurgery were compared between the 3 groups.We reviewed 148 cases. After exclusions, 34 of 53 children (64%) who underwent standard craniectomy, 6 of 17 children (35%) who had pericranial flaps, and 5 of 11 children (46%) who underwent trapezoidal craniectomy had defects 1 year postsurgery. The percentage of children with defects (P = 0.0364) but not the defect area was significantly higher in the standard craniectomy than in the pericranial flap group. The percentage of subjects with defects was not significantly different between the standard and the trapezoidal craniectomy groups.Sewing a vascularized pericranial flap to the dura at the craniectomy site may protect against persistent bony defects after sagittal craniectomy for craniosynostosis. Longer follow-up is needed to determine if this technique leads to lower rates of cranioplasty.

Evolution of a Virtual Multidisciplinary Cleft and Craniofacial Team Clinic During the COVID-19 Pandemic: Children's Hospital Colorado Experience.

Multidisciplinary care in the era of COVID mitigation presented a unique opportunity to evolve a multidisciplinary Telehealth experience at the Children's Hospital Colorado. We describe our experience in developing unique programming to remain in compliance with an experience as recommended by the Parameters of Care while integrating information technology accessible via the electronic health record, multimedia adjuncts, and the integration of multiple institutional participants in creating a platform to offer care via Telehealth. Visit structure, planning, implementation, and advantages and disadvantages of the programming are discussed.

Reconstruction of Complex Scalp Defect After Cirsoid Aneurysm Resection: A Multidisciplinary Approach.

BACKGROUND: Scalp arteriovenous malformations, also known as cirsoid aneurysms, are complex collections of directly communicating arteries and veins. As a cirsoid aneurysm grows, it can recruit a blood supply from multiple intracranial and extracranial vessels and involve both scalp and facial tissue. Depending on their size and complexity, a variety of strategies can be used to treat them. CASE DESCRIPTION: We have presented the case of a giant cirsoid aneurysm treated with endovascular embolization, resection, and reconstruction using multiple expanded scalp and facial flaps. A 15-year-old boy had presented with a pulsatile left temporal scalp mass that had slowly grown to involve most of his left scalp and extend into the ipsilateral face. At his next presentation, at 19 years old, he had recently developed episodic lateral visual field loss, photophobia, headaches, and vertigo. Catheter angiography demonstrated an extensive arteriovenous malformation supplied primarily by the left superficial temporal, posterior auricular, and occipital arteries, as well as by the ophthalmic artery, The angiogram also showed a dural arteriovenous fistula. Initially, tissue expanders were placed in the vertex, occipital, and left lower facial regions. The patient then underwent endovascular embolization, followed by resection and reconstruction of the tissue defect using multiple expanded scalp and facial flaps. The patient recovered well without neurological deficits and had complete resolution of his symptoms. Our surgical collaboration resulted in overall preservation of his hairline and facial symmetry. CONCLUSION: Large cirsoid aneurysms can require multidisciplinary treatment combining embolization, resection, and plastic surgical techniques to close the tissue defects.

Automated syndrome diagnosis by three-dimensional facial imaging.

PURPOSE: Deep phenotyping is an emerging trend in precision medicine for genetic disease. The shape of the face is affected in 30-40% of known genetic syndromes. Here, we determine whether syndromes can be diagnosed from 3D images of human faces. METHODS: We analyzed variation in three-dimensional (3D) facial images of 7057 subjects: 3327 with 396 different syndromes, 727 of their relatives, and 3003 unrelated, unaffected subjects. We developed and tested machine learning and parametric approaches to automated syndrome diagnosis using 3D facial images. RESULTS: Unrelated, unaffected subjects were correctly classified with 96% accuracy. Considering both syndromic and unrelated, unaffected subjects together, balanced accuracy was 73% and mean sensitivity 49%. Excluding unrelated, unaffected subjects substantially improved both balanced accuracy (78.1%) and sensitivity (56.9%) of syndrome diagnosis. The best predictors of classification accuracy were phenotypic severity and facial distinctiveness of syndromes. Surprisingly, unaffected relatives of syndromic subjects were frequently classified as syndromic, often to the syndrome of their affected relative. CONCLUSION: Deep phenotyping by quantitative 3D facial imaging has considerable potential to facilitate syndrome diagnosis. Furthermore, 3D facial imaging of "unaffected" relatives may identify unrecognized cases or may reveal novel examples of semidominant inheritance.

Fusion patterns of major calvarial sutures on volume-rendered CT reconstructions.

OBJECTIVE: Recently, the authors investigated the normal course of fusion of minor lateral calvarial sutures on "3D" volume-rendered head CT reconstructions in pediatric trauma patients. While evaluating these reconstructions, they found many more fused sagittal sutures than expected given the currently accepted prevalence of sagittal craniosynostosis. In the present study, using the same set of head CT reconstructions, they investigated the course of fusion of the sagittal as well as the lambdoid, coronal, and metopic sutures. METHODS: They reviewed all volume-rendered head CT reconstructions performed in the period from 2010 through mid-2012 at Children's Hospital Colorado for trauma patients aged 0-21 years. Each sagittal, lambdoid, coronal, or metopic suture was graded as open, partially fused, or fused. The cephalic index (CI) was calculated for subjects with fused and partially fused sagittal sutures. RESULTS: After exclusions, 331 scans were reviewed. Twenty-one subjects (6%) had fusion or partial fusion of the sagittal suture. Four of the 21 also had fusion of the medial lambdoid and/or coronal sutures. In the 17 subjects (5%) with sagittal suture fusion and no medial fusion of adjacent sutures, the mean CI was 77.6. None of the 21 subjects had been previously diagnosed with craniosynostosis. Other than in the 21 subjects already mentioned, no other sagittal or lambdoid sutures were fused at all. Nor were other coronal sutures fused medially. Coronal sutures were commonly fused inferiorly early during the 2nd decade of life, and fusion progressed superiorly and medially as subjects became older; none were completely fused by 18 years of age. Fusion of the metopic suture was first seen at 3 months of life; fusion was often not complete until after 2 years. CONCLUSIONS: The sagittal and lambdoid sutures do not usually begin to fuse before 18 years of age. However, more sagittal sutures are fused before age 18 than expected given the currently accepted prevalence of craniosynostosis. This finding is of unknown significance, but likely many of them do not need surgery. The coronal suture often begins to fuse inferiorly early in the 2nd decade of life but does not usually complete fusion before 18 years of age. The metopic suture often starts to fuse by 3 months of age, but it may not completely fuse until after 2 years of age.

Multilayered and Flap Closure Technique for Pediatric Spinal Deformity Surgery.

Wound complications after pediatric spinal deformity surgery are a relatively common and challenging complication that results in emotional, physical, and monetary costs to the patients, families, and healthcare providers. The role of wound closure techniques for the prevention of wound complications is being increasingly explored. Interdisciplinary care by spinal surgeons and plastic surgeons has been recently found to decrease wound complication rates in patients with nonidiopathic scoliosis.

Resolution of obstructive sleep apnea after mandibular distraction osteogenesis in setting of delayed tongue-lip adhesion takedown: A case report.

RATIONALE: There is a high prevalence of obstructive sleep apnea (OSA) in patients with Pierre Robin sequence (PRS), and treatment approaches are highly variable. One approach is a temporary tongue-lip adhesion (TLA) that acts as a temporizing measure while the mandible continues to grow and is usually taken down at 1 year of age. PATIENT CONCERNS: Side effects of prolonged tongue-lip adhesion and optimal workup and treatment of persistent OSA in the setting of a tongue-lip adhesion. DIAGNOSES: Pierre Robin sequence (PRS), persistent obstructive sleep apnea (OSA), and tongue-lip adhesion (TLA). INTERVENTIONS: Mandibular distraction osteogenesis (MDO), adenotonsillectomy, and tongue-lip adhesion takedown. OUTCOMES: Resolution of OSA. LESSONS: This case puts into question the efficacy of isolated TLA in infants with Pierre Robin sequence and OSA, and places emphasis on the importance of considering an earlier workup of other potential causes of obstruction and the potential need for MDO as a primary or adjunctive approach to treatment.

Chronic Debilitating Headache in Adults Caused by Craniocerebral Disproportion: Treatment by Cranial Vault Expansion.

Craniocerebral disproportion is rarely considered as a cause for chronic, debilitating headache in adults. Children reported with this disorder typically suffer from headaches and lethargy for many years and have multisutural synostosis. The terms craniocerebral disproportion, craniostenosis, and slit-ventricle syndrome are used inconsistently as diagnostic designations. Three adults with craniocerebral disproportion who had been treated in infancy for two different pathologies are reported. All benefited greatly from cranial vault expansion.

Surgical management of a nasal AVM in a pediatric patient: A case report.

Arteriovenous malformations (AVMs) are a type of high-flow vascular malformation that are characterized by abnormal capillary communications between the arterial and venous systems. While they are most commonly located in the head and neck region, their appearance in the nose is considerably rare, resulting in a paucity of literature regarding the surgical management of these lesions. We present the case of a 13-year-old male with a 6.5 × 6 cm AVM of the nose with a history of frequent nosebleeds since early childhood, often requiring aggressive measures, such as silver nitrate cauterization for control. Use of nasal decongestants and aminocaproic acid provided only transient improvement. After determination of arterial supply, AVM was approached with a combination of preoperative selective embolization and surgical excision with subsequent forehead flap defect coverage. Due to the size and complexity of this AVM, extra precautions were taken to avoid severe intraoperative bleeding, and femoral sheaths were placed prior to excision. The patient tolerated the procedure well, and with subsequent debulking surgery and Laser Hair Removal achieved an acceptable cosmetic outcome.

Designing a Fibular Flow-Through Flap with a Proximal Peroneal Perforator-Free Flap for Maxillary Reconstruction.

Reconstruction of a composite maxillary defect is frequently performed with a fibular osteocutaneous free flap to address both the bony and mucosal defect. If during the harvest of the fibula a distal skin perforator is not present due to vascular variations, reconstruction can potentially be done using the soleus muscle for filling of the palatal mucosal defect. An additional challenge arises when the accompanying skin paddle that has been harvested is not perfused, but the fibula remains viable. This case report describes salvage following loss of the skin paddle in an osteocutaneous fibular free flap by designing a fibular flow-through flap using a proximal peroneal perforator free flap. The use of this second free flap allows a skin paddle to be positioned on the distal fibular segment, provides a surgical backup, and limits the donor sites to the same extremity.