NeuroBehcet's-related intracranial hypertension without cerebral venous thrombosis: case report and review of literature.

BACKGROUND: We present a rare case of NeuroBehcet's-related intracranial hypertension without cerebral venous thrombosis (NBrIHwCVT), occurring as the first presentation of NeuroBehcet's. In addition, we describe the novel use of subcutaneous tocilizumab for this indication. This is followed by a review of the literature on this topic. CASE: The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet's disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of > 40cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity. LITERATURE REVIEW: We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later. CONCLUSION: As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.

Long-Term Control of Nasal Tip Position: Quantitative Assessment of Caudal Septal Extension Graft.

BACKGROUND: Control of nasal tip position is critical to final rhinoplasty outcomes. Two frequent methods of exerting tip control are columellar strut and caudal septal extension graft (SEG). Past work has demonstrated that SEG are better able to preserve tip position. However, there is no quantitative data describing long-term projection and rotation. The purpose of this study was to analyze long-term maintenance of tip projection and rotation following SEG. METHODS: A retrospective study of adult patients undergoing open rhinoplasty was conducted. Three-dimensional photogrammetric evaluation of nasal tip position was performed. Anthropometric points were analyzed preoperatively and postoperatively. Outcome variables were tip projection, tip rotation, and nasal length. RESULTS: Twenty patients were included with an average follow-up time of 3.3 years (2.0 - 6.6 years). From postoperative week 1 to 6, there was a statistically significant decrease in rotation (-4.3%, p<0.01). There were no statistically significant decreases in projection, rotation, or nasal length from 6 weeks postoperative to 2 years postoperative, or from 6 weeks postoperative to final follow-up (2.0 - 6.6 years). CONCLUSIONS: Nasal tip projection and rotation appear to decrease from the immediate postoperative position, likely due to resolving edema. In this study, patients that underwent open rhinoplasty with SEG experienced modest loss of projection and rotation until 6 weeks postoperative, but projection and rotation were maintained from 6 weeks postoperative to 2 years and beyond. This study provides evidence that SEG maintains long-term changes in tip projection and rotation. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Concomitant Cervical Spine Injuries in Pediatric Maxillofacial Trauma: An 11 Year Review of the National Trauma Data Bank.

PURPOSE: Craniofacial trauma with concomitant cervical fractures (CCFs) is responsible for significant morbidity and mortality in the pediatric population. We aim to characterize its incidence, injury patterns, outcomes, and risk factors, along with identifying any association between mandible fractures and cervical injuries via the National Trauma Databank. METHODS: A retrospective cohort study was performed using National Trauma Databank records between 2007 and 2017 to identify patients equal or under the age of 18 years hospitalized for maxillofacial trauma and with recorded cervical injury. Variables of interest include age, gender, race/ethnicity, trauma type (blunt vs penetrating), Injury Severity Score, area involved, mechanism of injury, comorbid conditions, inpatient complications, and discharge disposition. Retrospective cohorts were separated by CCF status. Univariate, bivariate, and multivariable regression analysis was utilized, with P-value <.05 considered statistically significant. RESULTS: A total of 32,952 patients were included in the study, with the majority being White (60.8%), male (68.2%), and between the ages of 13 and 18 years (65%). Of these, 8.2% experienced CCF. Most common mechanisms of injury were motor vehicle trauma (32.6%), interpersonal violence (18.8%), and falls (13.5%). Univariate analysis revealed patients with CCF were significantly older (15.2 vs 12.9; P < .001), more likely to be motor vehicle occupants (46.6 vs 31.9%; P < .001), and suffer polyfacial fractures (62.6 vs 60.7%; P < .001). Longer length of stay (9.4 vs 3.6 days; P < .001) and significantly higher inpatient complications such as deep vein thrombosis, pulmonary embolism, unplanned intubation, severe sepsis, pressure ulcer, ventilator-associated pneumonia, and unplanned return to operating room were observed in the CCF cohort. Female gender (1.5 [1.37 to 1.64; 95% confidence interval {CI}] P < .001) and higher Injury Severity Score (1.12 [1.11 to 1.11; 95% CI] P < .001) were associated with significantly higher odds on multivariable analysis. The presence of a mandible fracture was not associated with increased CCF on multivariate analysis (1.06 [0.92 to 1.22; 95% CI] P = .36). CONCLUSIONS: There are statistically significant differences in demographics, outcomes, and injury patterns in maxillofacial patients with CCF that may help guide treatment. No association between mandible fractures and cervical trauma was identified.

Technique for Cephalometric Analysis of the Craniofacial Skeleton Following Facial Feminization Surgery.

Facial feminization surgery (FFS) is characterized by a series of relatively diverse procedures aimed at aligning skeletal and soft tissue facial appearance with one's experienced feminine gender. Although there are several well-described outcomes from surgical techniques, there is no standardized methodology to provide reliable analyses of postoperative FFS outcomes. This paper describes the first reliable and reproducible technique to accurately and consistently measure post-FFS changes to guide surgical planning to optimize patient outcomes.

Ear Molding Therapy of Congenital Ear Anomalies: Long-Term Aesthetic Outcomes and Caretaker Satisfaction.

INTRODUCTION: Ear molding is a safe, non-surgical approach to treat newborns with congenital ear anomalies. In this study, we aimed to investigate long-term aesthetic outcomes and caretaker satisfaction from ear molding therapy. METHODS: A retrospective chart review from 2018 to 2020 was conducted for infants who underwent ear molding treatment at our institution. Patient demographics and treatment related variables were collected. Caretakers were surveyed regarding their experience, expectations, and aesthetic outcome at 1 year (short-term) and 3 years (long-term) from treatment. Independent physicians evaluated treatment efficacy. Responses were converted to a Likert scale (1-5), with 5 representing most desirable. RESULTS: Overall, 38 of 42 patients participated in our long-term study (90%) for a total of 62 ears. Average follow-up was 3.31 ± 0.50 years after completion of treatment. Mean age at treatment was 23.2 ± 19.7 days with a mean treatment duration of 21.7 ± 7.7 days. Caretakers' satisfaction regarding auricular appearance remained high (short-term: 4.18 vs. long-term: 4.17, p = 0.54) and anticipated social distress decreased over time. Physician aesthetic evaluations were favorable between "somewhat effective" and "very effective" and remained consistent over time (short term: 3.46 vs. long-term: 3.31, p = 0.31). Furthermore, physician evaluations were higher for deformations than malformations (p = 0.04) and in children who began treatment by 30 days old (p = 0.04). CONCLUSION: Caretaker satisfaction from ear molding therapy remained high after long-term follow up, and social distress from the perception of their child's ears decreased with time. Physician aesthetic ratings confirmed efficacy, with better outcomes seen in deformations than in malformations. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Gender Disparities Among Craniofacial Surgeons.

To delineate career differences between genders of practicing plastic surgeons who have completed craniofacial fellowship given the known disparities in surgeons' professional and personal lives and an already lengthy residency training, there is concern that less women may commit to further fellowship training.Craniofacial fellowship programs were contacted to identify graduates and an internet search was attempted where information was not available. Surgeon profiles and literature databases were used to obtain practice demographics and publications.Accredited fellowships were identified through the American Society of Craniofacial Surgeons directory.Program responses along with internet searches identified 201 graduates from 26 programs, of which 132 (66%) were men and 69 (34%) were women. On average, male graduates had 7.1 years in practice versus 6.6 years for females graduates (P = .176). There were significant differences between average number of publications (24.7 publications for males vs 14.1 for females, P = .009) and academic practice (46% males vs 64% females, P = .018). A similar percentage of males and females held leadership positions (13% males vs 16%, P = .552).Despite similar years in practice, men had significantly higher publications while women were significantly more likely to practice in an academic setting. Females are increasing their representation in academia and leadership within the craniofacial community. However, efforts must still be directed at increasing exposure to craniofacial surgery and supporting research and leadership pursuits earlier on during their careers.

The effect of treatment timing on clinical and psychological outcomes with Twin Block therapy: A multicentre two-arm parallel randomised controlled trial.

OBJECTIVE: To evaluate whether delaying treatment with a twin block appliance affects treatment outcomes, in terms of skeletal and dental change and psychological disadvantage. DESIGN: Multicentre, two-arm parallel randomised controlled trial. SETTING: Three UK hospital orthodontic units. METHODS: A total of 75 participants were randomly allocated into an Immediate Treatment Group (ITG) (n= 41) and an 18-month delayed Later Treatment Group (LTG) (n=34). OUTCOMES: Dentofacial changes were recorded from lateral cephalograms and study models, psychological impact was measured using a child perception questionnaire (CPQ11-14) and an Oral Aesthetic Subjective Impact Score (OASIS) at registration (DC1), 18 months (DC2) and 3 years (DC3). RESULTS: The ITG initial effect was a mean ANB reduction was 1.6° (P<0.001) (95% confidence interval [CI] = 0.89-2.29), and an overjet reduction of 5.06 mm (P<0.001) (95% CI = 3.96-6.16) (boys: aged 12 years 8 months; girls: aged 11 years 8 months). There were no statistically significant differences between the ITG and LTG for twin block treatment outcomes, but the ITG active treatment time was longer (P=0.004) (ITG: 13.1 months; LTG: 9.8 months). There was insufficient evidence to state that patients were psychologically disadvantaged by waiting 18 months for active twin block treatment (P>0.05). CONCLUSION: Participants achieved similar clinical and psychological outcomes irrespective of whether they started their twin block at the age of referral (ITG) or 18 months later (LTG). This suggests that boys aged 12 years 8 months and girls aged 11 years 8 months, on average, are not disadvantaged by waiting 18 months for active twin block treatment.

An age-related algorithm for management of micro-orbitism from anophthalmia: a systematic review with supplemental case reports.

Management of pediatric anophthalmia and resultant micro-orbitism is challenging. The efficacy and safety of treatment methods vary with age as bony changes grow recalcitrant to implants in those at skeletal maturity and osteotomies become technically challenging following frontal sinus pneumatization. This study aims to review methods for managing micro-orbitism and develop an age-based treatment approach. A systematic literature review was conducted. Data were screened and extracted by two investigators and relevant English-language primary-literature was analyzed. Information on sample-size, number of orbits, intervention, age, complications, and prosthetic retention was obtained. Representative case reports are presented, in addition. Nineteen studies met inclusion: 294 orbits in 266 patients were treated. Two studies reported distraction-osteogenesis. Two studies utilized bone grafting. Osteotomies were performed in 41 patients from three studies. Use of solid implants was detailed in two studies. Three studies described osmotic implant. Four studies described inflatable implants. Other techniques were described by three of the included studies, two of which utilized dermis-fat grafting. All but one study were observational case reports or case series. Across all studies regardless of surgical technique, risk of bias and heterogeneity was high due to attrition bias and selective outcomes-reporting. Selection of therapy should be tailored to skeletal-age to optimize outcomes; those 0-4 yrs are managed with dermis-fat grafts, 5-7 yrs managed with implants, and 8+ yrs managed with osteotomies. For those 8+ yrs with aerated frontal sinuses or insufficient bone stock, we propose onlay camouflage prosthetics which improve projection, increase orbital volume, and avoid risk for frontal sinus injury.

Expanding the Utilization of Low-Dose Computed Tomography in Plastic and Reconstructive Surgery Based on Validated Practices Among Surgical Specialties.

INTRODUCTION: As computed tomography (CT) usage increases, so have concerns over radiation-induced malignancy. To mitigate these risks, low-dose CT (LDCT) has emerged as a versatile alternative by other specialties, although its use in plastic surgery remains sparse. This study aimed to investigate validated uses of LDCT across surgical specialties and extrapolate these insights to expand its application for plastic surgeons. METHODS: A systematic review of the literature was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using search terms "low dose CT" OR "low dose computed tomography" AND "surgery," where the name of each surgical specialty was substituted for word "surgery" and each specialty term was searched separately in combination with the 2 CT terms. Data on radiation dose, outcomes, and level of evidence were collected. Validated surgical applications were correlated with similar procedures and diagnostic tests performed routinely by plastic surgeons to extrapolate potential applications for plastic surgeons. RESULTS: A total of 3505 articles were identified across surgical specialties, with 27 ultimately included. Depending on the application, use of LDCT led to a 25% to 97% reduction in radiation dose and all studies reported noninferior image quality and diagnostic capability compared with standard-dose CT. Potential identified uses included the following: evaluation of soft tissue infections, preoperative and postoperative management of facial and hand fractures, flap design, 3D modeling, and surgical planning. DISCUSSION: Low-dose CT is a valid imaging alternative to standard-dose CT. Expanded utilization in plastic surgery should be considered to minimize the iatrogenic effects of radiation and to promote patient safety without compromising outcomes.

Ergonomics Among Craniofacial Surgeons: A Survey of Work-Related Musculoskeletal Discomfort and Injury.

INTRODUCTION: Surgical procedures with loupe magnification, headlights, and microscopes expose craniofacial surgeons to mechanical stress that can increase risk of long-term musculoskeletal pain and injury. Identifying the prevalence and cause of work-related musculoskeletal discomfort may guide preventative strategies to prolong well-being, job satisfaction, and greater duration of surgical careers. METHODS: A 29-question online survey was distributed to the surgeon members of the American Cleft Palate-Craniofacial Association. Eight hundred seventy-three surveys were distributed, and the anonymous responses were recorded using Google forms. RESULTS: One hundred ninety-six unique responses were recorded (22.5% response rate). A total of 64.2% reported experiencing musculoskeletal symptoms during their career, with neck, lower back, and shoulders being the most common problem areas. Multivariate analysis demonstrated surgical loupes (odds ratio 2.36, P = 0.03) and length of surgical practice >15 years (odds ratio 1.95, P = 0.04) were independently associated with greater odds of developing symptoms. Headlights (median pain = 3, P < 0.001), loupes (median pain = 3.5, P < 0.001), and operative microscope use (median pain = 2, P = 0.02) were all associated with higher pain while operating. A total of 52.5% respondents sought medical treatments, 50.5% were concerned musculoskeletal discomfort would affect their careers, 56.6% reported a colleague that required an operation, and 30.2% reported a colleague on temporary or permanent disability. CONCLUSIONS: Craniofacial surgery often involves long procedures, use of surgical adjuncts, and ergonomically straining postures, which can lead to musculoskeletal discomfort and injury. This under-reported and important phenomenon merits candid conversation and active preventative strategies to prolong surgical careers, improve professional satisfaction, and maximize patient safety.

A study of smile aesthetic perception among dental professionals, patients and parents towards impacted maxillary canine treatment options.

OBJECTIVE: To evaluate the smile aesthetics of the different treatment options for impacted maxillary canines as perceived by orthodontists, dentists, patients and parents. DESIGN: Cross-sectional survey. SETTING: Four rater groups were selected to complete the questionnaire consisting of an orthodontist group, a general dental practitioner group, a patient group consisting of patients aged 11-18 years who were considered to be 'Gillick competent' and a parent group consisting of parents/guardians who accompanied the patients to their orthodontic appointments. METHOD: Four digitally manipulated images were created to imitate different treatment options available for the treatment of ectopic maxillary canines including: alignment of the impacted maxillary canine; substituted premolars; retained deciduous canines; or gaps present. The images were embedded into piloted questionnaires. Each participant completing the questionnaire was asked to mark the visual analogue scale (VAS) beneath each image according to their perception of attractiveness of each image. Quantitative scoring of the perceived attractiveness of the smile was assessed by one calibrated assessor measuring the distance from the start of the scale to the marked cross placed on the VAS. A two-way ANOVA (mixed between-within subject's ANOVA) was used to compare perception of differences in smile aesthetics. RESULTS: There were significant differences found in the VAS between the groups (P = 0.002) and between the treatment options (P < 0.001) There was no statistically significant difference found between the aligned canines and substituted premolars images by the patient (P = 0.2) or parent group (P = 0.5). All groups most disliked the treatment option where gaps were present at the end of treatment. CONCLUSION: The patient and parent groups showed similar perception in smile aesthetics for aligned canines and substituted premolars and all groups showed a strong dislike to the presence of gaps. The orthodontist group showed the most critical aesthetic perceptions.

Sinus pericranii in the upper eyelid: diagnosis and management guidelines.

Sinus pericranii (SP) is a rare malformation involving aberrant connections between the dural venous sinuses and extracranial veins. Classically considered congenital pathology, there are reported instances of post-traumatic SP in adults. Very rarely, the upper eyelid is involved and the resulting ptosis can obstruct the visual axis. In these cases, surgical removal may be warranted. Because of the high risk of massive intraoperative hemorrhage, careful preoperative identification and diagnostic workup is essential. The authors report a patient with new onset, asymmetric eyelid ptosis referred for evaluation, and subsequently diagnosed with SP. Through a multi-disciplinary assessment, deferring surgical intervention was determined to be appropriate management. The lesion was monitored clinically, and at 6 months follow-up, there were no noted adverse outcomes.

Health-related quality of life in paediatric haemophilia B patients treated with rIX-FP.

INTRODUCTION: Frequent infusions and bleeds can impact on the health-related quality of life (HRQoL) of paediatric haemophilia B patients. rIX-FP (IDELVION® ) is a fusion protein linking recombinant factor IX with recombinant albumin, and is associated with low bleeding rates with a weekly regimen, which could improve HRQoL. AIMS: To measure the effect of rIX-FP prophylaxis on the HRQoL of paediatric patients and treatment satisfaction in their caregivers using the Haemo-QoL and Hemo-SATP questionnaires, respectively. METHODS: At baseline and end-of-study (EOS), patients 4-11 years old participating in the PROLONG-9FP program answered the Haemo-QoL questionnaire and gave information on their socio-demographic data and physical activity. Caregivers completed the Hemo-SatP . Minimal important differences (MID) (|Cohen's d| > 0.5) between baseline and EOS and the number of responders (patients with meaningful subject-level improvements over time) at EOS were calculated. RESULTS: Twenty patients (age group I: 4-7 years old [n = 12]; age group II: 8-12 years old [n = 8]) completed the Haemo-QoL questionnaire at baseline. MIDs were found in age group I representing improvement for "physical health" (d = -0.547) domain; 60% of patients were responders for "physical health." In age group II, MIDs were seen in most domains; 71.4% patients were responders in "total score." In caregivers, improvements were seen for most domains of the Hemo-SatP with a small effect size. Fewer patients missed school when treated with rIX-FP and 94.1% patients maintained their physical activity level. CONCLUSION: Prophylaxis with rIX-FP led to substantial improvements in HRQoL in paediatric patients and treatment satisfaction in caregivers.

Comparative Incidence of Patellofemoral Complications Between 2 Total Knee Arthroplasty Systems in a Multicenter, Prospective Clinical Study.

BACKGROUND: Cumulative incidence rates (CIRs) of patellofemoral complications (PCCs) and patellofemoral symptomatic crepitus (SC) using a new knee system-total knee arthroplasty (NEW-TKA) were compared with those of a currently available product-total knee arthroplasty (CA-TKA). METHODS: Twenty-two investigators prospectively enrolled 704 patients into a study using CA-TKA; 364 received a posterior-stabilized configuration. Twenty-three investigators (19 from the CA-TKA study) enrolled 1138 patients with NEW-TKA; 584 received a posterior-stabilized configuration. CIRs were estimated with Kaplan-Meier methods. RESULTS: CA-TKA had 32 PCCs (6.15% CIR at 1 year and 8.26% at 2 years). NEW-TKA had 19 PCCs (3.15% CIR at 1 year and 4.11% at 2 years). CA-TKA had 15 SCs, 12 before 2 years (CIR = 3.67%); NEW-TKA had 5 SCs before 2 years (CIR = 1.21%). NEW-TKA had a statistically lower CIR of overall PCC and SC (log-rank P values = .018 for PCC and .017 for SC). CONCLUSION: Interim 1- and 2-year CIRs of PCC and SC in NEW-TKA were less than half of those for CA-TKA, which is a promising trend for this new implant.

WITHDRAWN: Porous Metal Augments Show Excellent Mid-to-Long Term Survival in Complex Acetabular Reconstruction.

This article has been withdrawn at the request of the author(s) and/or editor. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.

Opioid prescribing patterns for distal radius fractures in the ambulatory setting: A 10-year retrospective study.

OBJECTIVE: Distal radius fractures (DRFs) are one of the most common orthopedic injuries, with most managed in the nonoperative ambulatory setting. The objectives of this study are to examine National Health Center Statistics (NHCS) data for DRF treated in the nonoperative ambulatory setting to identify opioid and nonopioid analgesic prescribing patterns and to determine demographic risk factors for prescription of these medications. Design, setting, patients, and measures: This study is a retrospective analysis of data collected by the NHCS from 2007 to 2016. Utilizing International Classification of Diseases codes, all visits to emergency departments and doctors' offices for DRFs were identified. Variables of interest included demographic data, expected payment source, and prescription of opioid or nonopioid analgesics. RESULTS: During the study timeframe, 15,572,531 total visits for DRFs were recorded. DRF visits requiring opioid and nonopioid analgesic prescriptions increased over time. Patients aged 45-64 years were significantly more likely to receive an opioid prescription than any other age group (p < 0.05). Opioid prescription was positively correlated with the use of workers' compensation and negatively correlated with patients receiving services under charity care (p < 0.05). CONCLUSIONS: Prescriptions of both opioid and nonopioid analgesic medications for DRF have been steadily increasing over time in the nonoperative ambulatory setting, with middle-aged adults most likely to receive an opioid prescription. Opioid prescription rates differ significantly between patients utilizing workers' compensation and patients receiving services under charity care, suggesting that socioeconomic factors play a role in prescribing patterns.

The role of genetics on behavioral outcomes in nonsyndromic sagittal synostosis.

OBJECTIVE: Previous work identified an association between genetics and neurodevelopmental delays in patients with nonsyndromic craniosynostosis. The authors investigated the role of genetic mutations on behavioral outcomes of patients with treated sagittal synostosis. METHODS: Parents of children aged 6-18 years with surgically corrected sagittal synostosis were recruited to complete the Child Behavioral Checklist (overall behavioral problems), Conners 3rd Edition-Parent (attention-deficit/hyperactivity disorder), Social Responsiveness Scale 2nd Edition (autism spectrum disorder [ASD]), and Behavior Rating Inventory of Executive Function 2nd Edition (executive function). Genomic analysis was completed, and patients were identified if they had mutations in high probability of loss of function intolerant (pLI) genes (high pLI vs nonhigh pLI). Genetic burden was assessed relative to controls. Multivariate linear regression determined the association of mutations in high pLI genes with behavioral scores, while controlling for sociodemographic factors, age at surgery, surgery type, and IQ. RESULTS: Sixteen of 45 patients were in the high pLI group. There were no differences between the groups in terms of sociodemographic factors. A greater proportion of children in the high pLI group scored at or above borderline clinical levels for aggression (18.8% vs 0.0%, p = 0.05) and externalizing problems (31.3% vs 3.7%, p = 0.02). Among children in the nonhigh pLI group, older age at surgery was associated with worse scores on the rule-breaking, aggression, and externalizing problems domains and four out of five ASD domains. CONCLUSIONS: Children with treated nonsyndromic sagittal synostosis and mutations in high pLI genes had worse behavioral problems in externalizing behaviors and aggression, whereas older age at surgery was a significant predictor of worse behavioral outcomes in patients without mutations in high pLI genes.

Autoclaved autologous bone graft for orbital reconstruction for recurrent temporal bone orbital osteosarcoma.

Post-tumor excision and reconstruction of the craniofacial region is a complex and technically demanding process due to its proximity to numerous vital structures and irregularly shaped bony tissue. As such, novel methods are needed when reconstruction of irregularly shaped structures is necessary. Autoclaving of autologous bone grafts is an established practice in orthopedic and neurosurgical practice, but has only been described twice previously for orbital reconstruction. We performed grafting of an autoclaved autologous bone segment as part of surgery on a 30-year-old man to treat his recurrent temporal osteosarcoma with orbital involvement, which is rare. In addition, we went on to highlight key differences between bone autoclaving and pasteurization, an alternative heat treatment technique, for orbital reconstruction post-tumor excision. Although he suffered a second recurrence 8 months later, there was no evidence of recurrence in the autoclaved bone. To treat his second recurrence, he subsequently underwent a modified eyelid-conjunctiva sparing orbital exenteration, also an uncommonly performed procedure. Also, we subsequently examined the novel technique of a lid-sparing and conjunctiva-sparing orbital exenteration and its benefits. He continues to remain under follow-up.

Telemedicine screening for cytomegalovirus retinitis using digital fundus photography.

BACKGROUND: Screening for cytomegalovirus retinitis (CMVR) is important in patients with acquired immune deficiency syndrome and low CD4(+) counts. However, many human immunodeficiency virus (HIV) patients lack access to specialist ophthalmological care. Telemedicine screening is a cost-effective method for screening these patients. We aimed to report the use of composite nine-field digital fundus photography (DFP) images for CMVR screening. We report its sensitivity and specificity in detecting CMVR and the level of agreement with gold-standard binocular indirect ophthalmoscopy. MATERIALS AND METHODS: An audit was performed on our national CMVR screening program that screened all HIV patients referred to the Ophthalmology Department at Tan Tock Seng Hospital, Singapore. All patients underwent retinal screening with DFP. Images were categorized as CMVR-positive, CMVR-negative, suspicious, or unreadable by blinded retinal specialists. Patients subsequently underwent dilated gold-standard indirect ophthalmoscopy by a different retinal specialist. Diagnoses were categorized as CMVR-positive, CMVR-negative, or unreadable. Sensitivity and specificity of retinal findings on DFP and kappa values for level of agreement between the two screening methods were calculated. RESULTS: Three hundred seventy screenings on 188 patients were performed. Twenty-three eyes diagnosed with CMVR on indirect ophthalmoscopy were also identified on DFP (100% sensitivity). A 99.9% specificity was achieved. The fundus photograph of one eye without CMVR was read as CMVR-positive because of an artifact, accounting for a false-positive. Kappa values ranged from 0.739 to 0.987. CONCLUSIONS: DFP is a sensitive and specific method of screening HIV patients for CMVR and has a high level of agreement with indirect ophthalmoscopy.

Robotic surgery in gynecology: program initiation and early outcomes at a community hospital.

In 2005, the U.S. Food and Drug Administration (FDA) approved the use of the da Vinci robotic surgical system. This resulted in significant changes in gynecologic surgery. The da Vinci system has improved patient care by reducing the risk of complications associated with surgical intervention and decreasing the length of hospital stays. This study is a retrospective review of the first 215 cases in which the da Vinci surgical technology was implemented in a community hospital. The majority of procedures performed were total abdominal hysterectomies with or without salpingo-oophrectomy. The results were profoundly supportive of the benefits of using the da Vinci system for gynecologic surgery. Of the 215 procedures, only five converted to open, yielding a conversion rate of 2.33%; zero mortalities were incurred. Over three-quarters of the case group experienced an average hospital stay of one day, with an overall complication rate of 7.44%. Early postoperative complications included UTI, urinary retention, vaginal bleeding, small bowel obstruction and port site dehiscence. Overall, the case group results suggest that robotic surgery improved patient care, and potentially allowed surgeons to have better visualization and tissue manipulation during surgery.