Multicenter comparison of head shape outcomes for three minimally invasive strip craniectomy techniques for sagittal craniosynostosis.

OBJECTIVE: A literature gap exists comparing whole head shape outcome following correction of sagittal craniosynostosis. The objective of this multicenter study was to provide an analysis of long-term results following three different endoscopic strip craniectomy techniques for correction of sagittal craniosynostosis: 1) spring-assisted strip craniectomy, 2) wide-strip craniectomy with biparietal and bitemporal barrel-stave wedge osteotomies plus helmet orthosis, and 3) narrow-strip craniectomy plus orthosis without barrel staves. METHODS: Pre- and postoperative 3D stereophotogrammetric images were collected from patients who underwent craniosynostosis surgery. Procedures were divided among institutions as follows: spring-assisted strip craniectomies were performed at Atrium Health Wake Forest Baptist Hospital; narrow-strip craniectomies were performed at St. Louis Children's Hospital by one craniofacial surgeon; and wide-vertex craniectomies were performed at St. Louis Children's Hospital prior to 2010, and then continued at Children's Medical Center Dallas. Pre- and postoperative 3D whole-head composite images were generated for each procedure to visually represent outcomes at final follow-up and compared with age-matched normal controls. RESULTS: Patients in the spring-assisted strip craniectomy group showed normalization of frontal bossing and skull height compared with age-matched controls, whereas patients undergoing wide-strip craniectomy showed greater correction of occipital protrusion. Patients in the narrow-strip craniectomy cohort had intermediate results between these outcomes. Nested aggregate head shapes showed good correction of head shapes from all techniques. CONCLUSIONS: This large, retrospective, multicenter study illustrated whole head shape outcomes from three different craniectomy procedures. Although each procedure showed some differences in loci of primary correction, all three surgical methods demonstrated good correction of primary scaphocephalic deformity.

3D imaging of vascular anomalies using raster-scanning optoacoustic mesoscopy.

OBJECTIVES: Vascular anomalies such as capillary malformations (CMs) and infantile hemangiomas (IHs) are common pediatric vascular disorders that are treated with therapeutic laser. The treatment method, however, relies on subjective evaluation of clinical findings and can have unpredictable results. Raster-scanning optoacoustic mesoscopy (RSOM) is an innovative imaging technology using pulsed-light laser to excite hemoglobin, generating ultrasound waves that are converted into three-dimensional images of tissues. RSOM can provide objective information about superficial structures such as the microvasculature of vascular anomalies. MATERIALS AND METHODS: In this study, we explore the clinical potential of RSOM to study vascular anomalies before and after laser treatment. We scanned nine patients with CM (n = 6) and IH (n = 3) who underwent laser treatment and calculated the blood vessel volume. RESULTS: Overall, there was a posttreatment volume increase in CM, and a decrease in IH. CONCLUSION: These findings support the possibility that RSOM may have a role in developing an objective method of evaluating these lesions, leading to a tailored treatment approach and avoidance of adverse outcomes.

Reduction Glossectomy for Macroglossia in Beckwith-Wiedemann Syndrome: Is Post-Op Intubation Necessary?

OBJECTIVE: Macroglossia is a characteristic feature of Beckwith-Wiedemann syndrome (BWS), commonly treated with reduction glossectomy to restore form and function. There exists no consensus on the perioperative management of these patients undergoing tongue reduction surgery, including anecdotal information regarding how long postoperative intubation should be maintained. The aim of this study is to evaluate the necessity of prolonged postoperative intubation in patients receiving tongue reduction surgery via the surgical and anesthetic management methods at our center. DESIGN: Retrospective case series. SETTING: Institutional care at Level I Children's Hospital. PARTICIPANTS: All children less than 18 years old with BWS and congenital macroglossia who underwent tongue reduction surgery over 5 consecutive years at our center (N = 24). INTERVENTIONS: Tongue reduction surgery via the "W" technique. MAIN OUTCOME MEASURES: Success of immediate postoperative extubation and related surgical complications. RESULTS: Immediate, uncomplicated postoperative extubation was successfully performed in all patients who received tongue reduction surgery for congenital macroglossia. CONCLUSIONS: Prolonged postoperative intubation for tongue reduction surgery may not be necessary as immediate, uncomplicated postoperative extubation was achieved in 100% of patients who received tongue reduction surgery at our center.

Anterior "W" Tongue Reduction for Macroglossia in Beckwith-Wiedemann Syndrome.

INTRODUCTION: Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by surgeons. The purpose of this study is to evaluate the postoperative outcomes of the anterior "W" tongue reduction technique in patients with BWS. METHODS: A retrospective review was conducted of all patients diagnosed with BWS that underwent an anterior "W" tongue reduction for macroglossia in the past 7 years, performed by 2 surgeons. Demographics, procedural characteristics, perioperative outcomes, and complications were assessed. RESULTS: A total of 19 patients met inclusion criteria consisting of 8 male and 11 female patients. The mean age at the time of surgery was 405 days, mean surgeon operating time was 1.06 h, and mean length of follow-up was 467 days. Postoperative oral competence was observed in 100% of patients. There was no reported history of sleep apnea or airway compromise. Speech delay was seen in 4 patients pre- and postoperatively. Feeding issues decreased from 7 patients preoperatively to 1 patient postoperatively. Preoperative prevalence of class III malocclusion (53%) and isolated anterior open bite (26%) decreased postoperatively to 37% and 16%, respectively. The only reported complications were superficial tip wound dehiscence in 3 patients treated with nystatin antifungal therapy. None of the patients required revisional surgery. CONCLUSION: Patients treated with the anterior "W" tongue reduction technique had low rates of perioperative complications and significant improvements in oral competence. Anterior "W" tongue reduction is safe and effective for the correction of macroglossia in patients with BWS.

Correction of Metopic Craniosynostosis Using Limited Incision Strip Craniectomy Versus Open Fronto-Orbital Reconstruction: An Assessment of Aesthetic Outcomes.

BACKGROUND: Metopic craniosynostosis is traditionally repaired with fronto-orbital advancement (FOA) or, alternatively, limited short scar strip craniectomy (LSSSC) followed by helmet therapy. There is controversy among surgeons regarding resultant head shape outcomes between the 2 methods. This study aims to assess how surgeons perceive the postoperative aesthetic results of the 2 metopic craniosynostosis repair methods. METHODS: A retrospective analysis was performed on 13 (n = 6 LSSSC; n = 7 FOA) patients who presented for surgical correction of isolated metopic craniosynostosis via either LSSSC (followed by helmet therapy) or FOA. Clinical photographs at 1 year postop were shown to 10 craniofacial surgeons who rated the aesthetic outcomes on a Likert scale of 1 (poor) to 5 (excellent) and guessed which surgical method was performed. RESULTS: Mean age at the time of the procedure was younger in LSSSC than FOA (3.1 ±â€Š1.0 versus 17.5 ±â€Š8.5 months; P < 0.001). Mean blood loss was significantly lower with LSSSC versus FOA (202.0 ±â€Š361.2 versus 371.43 ±â€Š122.9 mL; P < 0.001), as was mean blood transfusion requirement (92.5 ±â€Š49.9 versus 151.3 ±â€Š51.2 mL; P < 0.001) and mean duration of the operation (3:06 ±â€Š0:24 versus 7:53 ±â€Š0:31 hours; P < 0.001). Mean surgeon scores of aesthetic outcomes were similar between groups: LSSSC, 3.27 ±â€Š1.09; FOA, 3.51 ±â€Š0.95 (P = 0.171). When asked to identify which procedure patients had received, only 63.8% of responses were correct. CONCLUSIONS: Limited short scar strip craniectomy offers an important alternative to traditional open FOA and should be considered as an option for children diagnosed with metopic craniosynostosis.

Modified Chondrocutaneous Flaps for Middle Helical Rim Defects.

This article describes a unique approach for reconstruction of large helical rim defects. By raising bilateral chondrocutaneous flaps of the helical rim while including a semicircular chondrocutaneous excision of the adjacent scapha and antihelix, large defects can be repaired with minimal loss to the overall external ear circumference. This is a technically simple and reliable method that has resulted in excellent cosmetic outcomes and minimal morbidity in our practice.

A Role for Artificial Intelligence in the Classification of Craniofacial Anomalies.

ABSTRACT: Development of an objective algorithm to diagnose and assess craniofacial conditions has the potential to facilitate early diagnosis, especially for care providers with limited craniofacial expertise. Deep learning, a branch of artificial intelligence, can automatically analyze and categorize disease without human assistance. Convolutional neural networks (CNN) have excelled in utilizing medical images to automatically classify disease. In this study, the authors developed CNN models to detect and classify non-syndromic craniosynostosis (CS) using 2D images. The authors created an annotated data set of labeled CS (normal, metopic, sagittal, and unicoronal) conditions using standard clinical photography from the image repository at our center. The authors extended this dataset set by adding photographic images of children with craniofacial conditions from the internet. A total of 1076 images were used in this study. The authors developed a CNN model using a pre-trained ResNet-50 model to classify the data as metopic, sagittal, and unicoronal. The testing accuracy for the CS ResNet50 model achieved an overall testing accuracy of 90.6%. The sensitivity and precision were: 100% and 100% for metopic, 93.3% and 100% for sagittal, and 66.7% and 100% for unicoronal, respectively. The CNN model performed with promising accuracy. These results support the idea that deep learning has a role in diagnosis of craniofacial conditions. Using standard 2D clinical photography, such systems can provide automated screening and detection of these conditions. In the future, ML may be applied to prediction and assessment of surgical outcomes, or as an open-source remote diagnostic resource.

Comparison of Head Shape Outcomes in Metopic Synostosis Using Limited Strip Craniectomy and Open Vault Reconstruction Techniques.

AIM: Metopic craniosynostosis (MCS), with its trigonocephalic head shape, is often treated with either limited incision strip craniectomy (LISC) followed by helmet orthotic treatment, or open cranial vault reconstruction techniques (OCVR). There is controversy regarding resultant shape outcomes among craniofacial surgeons. Those adverse to LISC claim normal head shape is never attained, while proponents believe there is gradual correction to an equivalent outcome. This study aims to quantitate, over time, the three-dimensional (3D) head shapes in patients who have undergone LISC or OCVR intervention for MCS. METHODS: Sixty-three 3D images of 26 patients with MCS were analyzed retrospectively. Head shape analyses were performed at: (1) preoperative, (2) 1-month postoperative, (3) 10 to 14 months postoperative (1 year), and (4) 2 years postoperative. Composite 3D head shapes of patients were compared at each time point. Two-dimensional (2D) standardized cross sections of the forehead were also compared. RESULTS: Composite head shapes for both groups were nested, to allow visual comparison as the child's forehead grows and expands. The difference between LISC and OCVR 2D cross sections was calculated; 108.26 mm preoperatively, 127.18 mm after 1-month postoperative, 51.05 mm after 10 to 14 months postoperative, and 27.03 mm after 2 years postoperative. CONCLUSIONS: This study found excellent head shape outcomes for both the LISC and OCVR techniques at 2 years of age. It also corroborates the slow and progressive improvement in head shape with the LISC technique. This study highlights the advantages of 3D photography for measurement of contour outcomes, utilizing both 2D vector and 3D whole head analytical techniques.

Progressive Postnatal Pansynostosis in Crouzon Syndrome.

ABSTRACT: Crouzon syndrome is a known craniosynostosis syndrome with a heterogenous presentation. Suture closure can rapidly develop postnatally and involve multiple sutures, requiring vigilant clinical monitoring to prevent delay in diagnosis and developing intracranial hypertension. The authors present the case of a male patient with Crouzon syndrome who developed postnatal pansynostosis at 18 months of age when several missed appointments led to diagnosis after complete closure of all sutures and signs of elevated intracranial pressure. Posterior cranial vault distraction was performed soon after, and the patient improved clinically. There is a need for improved monitoring and management of these patients to prevent delay in diagnosis and potential permanent sequelae as well as minimizing radiation exposure through watchful CT scanning.

Congenital Inverted Conchal Bowl Deformity: Proposed Technique.

Congenital inverted conchal bowl is a rare deformity with unknown etiology. Its occurrence poses both functional and aesthetic difficulties for patients. We report 2 cases of congenital inverted conchal bowl deformity in an 8-year-old male and 17-year-old female. Both patients were first evaluated in a delayed fashion with concern for difficulty hearing, poor hygiene, and aesthetic concerns. Patient presentation, surgical management, and 6-month follow-up results are presented. Both patients had improved functional and aesthetic outcome after surgical correction.

The Use of Eye Tracking to Discern the Threshold at Which Metopic Orbitofrontal Deformity Attracts Attention.

INTRODUCTION AND OBJECTIVES: Surgical treatment for trigonocephaly aims to eliminate a stigmatizing deformity, yet the severity that captures unwanted attention is unknown. Surgeons intervene at different points of severity, eliciting controversy. This study used eye tracking to investigate when deformity is perceived. MATERIAL AND METHODS: Three-dimensional photogrammetric images of a normal child and a child with trigonocephaly were mathematically deformed, in 10% increments, to create a spectrum of 11 images. These images were shown to participants using an eye tracker. Participants' gaze patterns were analyzed, and participants were asked if each image looked "normal" or "abnormal." RESULTS: Sixty-six graduate students were recruited. Average dwell time toward pathologic areas of interest (AOIs) increased proportionally, from 0.77 ± 0.33 seconds at 0% deformity to 1.08 ± 0.75 seconds at 100% deformity (P < .0001). A majority of participants did not agree an image looked "abnormal" until 90% deformity from any angle. CONCLUSION: Eye tracking can be used as a proxy for attention threshold toward orbitofrontal deformity. The amount of attention toward orbitofrontal AOIs increased proportionally with severity. Participants did not generally agree there was "abnormality" until deformity was severe. This study supports the assertion that surgical intervention may be best reserved for more severe deformity.

Prevalence and Risk Factors for Anemia in a Population With Hidradenitis Suppurativa.

BACKGROUND:  Hidradenitis suppurativa (HS) is an inflammatory disease presenting as nodules evolving into scarred plaques. HS is associated with many co-morbidities, including anemia of chronic disease, though few studies report on this association. OBJECTIVES: This study evaluated the prevalence of anemia among the HS patient population and potential associations between risk factors for HS and anemia development. METHODS: Records diagnosed HS patients in one private practice (BR) were reviewed by investigators. The 92-patient cohort was evaluated for multiple data-points and responses grouped based on age, gender, ethnicity, body mass index (BMI), smoking status, and comorbidities. Data were analyzed using STATA to perform descriptive analysis and bivariate analysis. RESULTS: The prevalence of anemia in this cohort was 41.3%. Of anemic patients, the majority were men (65.2%), African American (60.6%), and never/former smokers (48.6%). There was a significant increase in the odds of developing anemia in HS patients that are men (odds ratio (OR) 3.8) and African American (OR 3.5). CONCLUSIONS: We show that the prevalence of anemia in an HS patient population greatly surpasses that of the U.S. population (~5%). It is clear that anemia is a significant complication for HS patients. We hope that physicians can recognize the importance of screening patients with HS for anemia to medically optimize treatment for their patients.