Head growth in patients with myelomeningocele treated with prenatal and postnatal surgery.

OBJECTIVE: The need for permanent CSF diversion is lower in patients who have undergone prenatal surgery for myelomeningocele (MMC) than in those who have undergone postnatal closure. Differences in brain development and head growth between treatment groups are not known, particularly for those who do not require surgical treatment for hydrocephalus. The objective of this study was to determine differences in head growth and to generate MMC-specific head circumference (HC) growth curves for patients who underwent either prenatal or postnatal surgery. METHODS: The authors retrospectively identified patients from St. Louis Children's Hospital who were treated for MMC between 2016 and 2021. HC data were obtained from birth until the most recent follow-up or hydrocephalus treatment. Nonlinear least-squares regression analysis was performed to fit the data into four models: two-term power, Gompertz, West ontogenetic, and Weibull. Subsequently, the curves were assessed for their utility in predicting hydrocephalus treatment. RESULTS: Sixty-one patients (29 females [47.5%], 25 [41%] underwent prenatal surgery, mean gestational age at birth 36.6 weeks) were included in the study. The Weibull model best fit the HC data (prenatal adjusted R2 = 0.95, postnatal adjusted R2 = 0.95), while the Gompertz model had the worst fit (prenatal adjusted R2 = 0.56, postnatal adjusted R2 = 0.39) across both cohorts. Prenatal MMC repair patients had significantly larger HC measurements than their postnatal repair counterparts. The 50th percentile of the Weibull curve was determined as a useful threshold for hydrocephalus treatment: children with HC measurements that crossed and remained above this threshold were significantly more likely to have hydrocephalus treatment regardless of time of MMC repair (prenatal relative risk [RR] 10.0 [95% CI 1.424-70.220], sensitivity 85.7% [95% CI 0.499-0.984], and specificity 82.4% [95% CI 0.600-0.948]; postnatal RR 4.750 [95% CI 1.341-16.822], sensitivity 90.5% [95% CI 0.728-0.980], and specificity 75.0% [95% CI 0.471-0.924]). The HC growth curves of the MMC patients treated prenatally were significantly larger than the WHO HC curves (p < 0.001). CONCLUSIONS: The Weibull model was identified as the HC growth curve with the best fit for MMC patients and serves as a useful predictor of hydrocephalus treatment. For MMC patients with hydrocephalus, prenatal repair patients fit the model well but postnatal repair patients did not, potentially indicating different mechanisms of hydrocephalus development. Those treated prenatally had significantly larger HC measurements compared with both the general population and those treated postnatally. Further study is needed to understand the long-term cognitive outcomes and optimal management of clinically asymptomatic patients with large HC measurements who were treated prenatally for MMC.

Predictors and timing of hydrocephalus treatment in patients undergoing prenatal versus postnatal surgery for myelomeningocele.

OBJECTIVE: Although hydrocephalus rates have decreased with intrauterine surgery for myelomeningocele (MMC), 40%-85% of children with MMC still go on to develop hydrocephalus. Prenatal ventricle size is known to be associated with later development of hydrocephalus; however, it is not known how prediction measures or timing of hydrocephalus treatment differ between pre- and postnatal surgery for MMC. The goal of this study was to determine anatomical, clinical, and radiological characteristics that are associated with the need for and timing of hydrocephalus treatment in patients with MMC. METHODS: The authors retrospectively identified patients from Barnes Jewish Hospital or St. Louis Children's Hospital between 2016 and 2021 who were diagnosed with MMC prenatally and underwent either pre- or postnatal repair. Imaging, clinical, and demographic data were examined longitudinally between treatment groups and hydrocephalus outcomes. RESULTS: Fifty-eight patients were included (27 females, 46.6%), with a mean gestational age at birth of 36.8 weeks. Twenty-three patients (39.7%) underwent prenatal surgery. For the overall cohort, the ventricle size at prenatal ultrasound (HR 1.175, 95% CI 1.071-1.290), frontal-occipital horn ratio (FOHR) at birth > 0.50 (HR 3.603, 95% CI 1.488-8.720), and mean rate of change in head circumference (HC) in the first 90 days after birth (> 0.10 cm/day: HR 12.973, 95% CI 4.262-39.486) were identified as predictors of hydrocephalus treatment. The factors associated with hydrocephalus in the prenatal cohort were FOHR at birth > 0.50 (HR 27.828, 95% CI 2.980-259.846) and the rate of change in HC (> 0.10 cm/day: HR 39.414, 95% CI 2.035-763.262). The factors associated with hydrocephalus in the postnatal cohort were prenatal ventricle size (HR 1.126, 95% CI 1.017-1.246) and the mean rate of change in HC (> 0.10 cm/day: HR 24.202, 95% CI 5.119-114.431). FOHR (r = -0.499, p = 0.008) and birth HC (-0.409, p = 0.028) were correlated with time to hydrocephalus across both cohorts. For patients who underwent treatment for hydrocephalus, those in the prenatal surgery group were significantly more likely to develop hydrocephalus after 3 months than those treated with postnatal surgery, although the overall rate of hydrocephalus was significantly higher in the postnatal surgery group (p = 0.018). CONCLUSIONS: Clinical and imaging factors associated with hydrocephalus treatment differ between those receiving pre- versus postnatal MMC repair, and while the overall rate of hydrocephalus is lower, those undergoing prenatal repair are more likely to develop hydrocephalus after 3 months of age. This has implications for clinical follow-up timing for patients treated prenatally, who may live at a distance from the treatment site.

Impact of Neighborhood Deprivation and Social Vulnerability on Outcomes and Interventions in Patients with Cleft Palate.

OBJECTIVE: To examine whether neighborhood disadvantage impacts length of follow-up, interventions, and outcomes for patients with cleft palate. DESIGN: Retrospective cohort. SETTING: Cleft Palate Craniofacial Institute Database at St. Louis Children's Hospital. PATIENTS/PARTICIPANTS: Patients with cleft palate following in St. Louis Children's Hospital Cleft Palate Multidisciplinary Team Clinic. INTERVENTIONS: Primary palatoplasty between 2012-2017. Patients were divided into quartiles across area deprivation index (ADI) and social vulnerability index (SVI), two validated, composite metrics of neighborhood disadvantage, to examine whether living in neighborhoods from different deprivation quartiles impacts outcomes of interest. MAIN OUTCOME MEASURE: Follow-up through age 5, surgeries and surgical complications, speech, developmental, and behavioral outcomes. RESULTS: 205 patients were included. 39% of patients belonged to the most deprived ADI quartile, while 15% belonged to the most vulnerable SVI quartile. There were no differences between ADI or SVI quartiles in number of operations received (p ≥ 0.40). Patients in the most deprived ADI quartile were significantly more likely to have speech/language concerns (OR 2.32, 95% CI [1.20-4.89], p = 0.01). Being in a more vulnerable SVI quartile was associated with developmental delay (OR 2.29, 95% CI [1.04-5.15], p = 0.04). ADI and SVI quartile did not impact risk of loss to follow-up in the isolated and combined cleft lip and palate subgroups (p ≥ 0.21). CONCLUSIONS: Neighborhood disadvantage impacts speech and developmental outcomes in patients with cleft palate despite comparable length of follow-up in multidisciplinary team clinic.

Association between Bioimpedance Spectroscopy and Magnetic Resonance Lymphangiography in the Diagnosis and Assessment of Lymphedema.

BACKGROUND: This study assesses associations between bioimpedance spectroscopy (BIS) and magnetic resonance lymphangiography (MRL) in the staging and assessment of lymphedema. METHODS: Adults who received MRL and BIS between 2020 and 2022 were included. We collected fluid, fat, and lymphedema severity ratings, and measured fluid stripe thickness, subcutaneous fat width, and lymphatic diameter on MRL. BIS lymphedema index (L-Dex) scores were collected from patient charts. We assessed sensitivity and specificity of L-Dex scores to detect MRL-identified lymphedema, and examined associations between L-Dex scores and MRL imaging measures. RESULTS: Forty-eight limbs across 40 patients were included. L-Dex scores had 72.5% sensitivity and 87.5% specificity for detecting MRL-defined lymphedema, with a 96.7% estimated positive predictive value and 38.9% negative predictive value. L-Dex scores were associated with MRL fluid and fat content scores (p ≤ 0.05), and lymphedema severity (p = 0.01), with better discrimination between fluid than fat content levels on pairwise analysis, and poor discrimination between adjacent severity levels. L-Dex scores were correlated with distal and proximal limb fluid stripe thickness (distal: rho = 0.57, p < 0.01; proximal: rho = 0.58, p < 0.01), partially correlated with distal subcutaneous fat thickness when accounting for body mass index (rho = 0.34, p = 0.02), and were not correlated with lymphatic diameter (p = 0.25). CONCLUSION: L-Dex scores have high sensitivity, specificity, and positive predictive value for the identification of MRL-detected lymphedema. L-Dex has difficulty distinguishing between adjacent severity levels of lymphedema and a high false negative rate, explained in part by reduced discrimination between levels of fat accumulation.

Investigating Weekend Effect in the Management of Upper and Lower Extremity Degloving Injuries.

Background: Weekend presentation has been associated with adverse outcomes in emergent conditions, including stroke, myocardial infarction, and critical limb ischemia. We examine whether a weekend effect exists in the management of and outcomes after extremity degloving injuries. Methods: The cohort included adults presenting with open extremity degloving injuries to a tertiary level one trauma center between June 2018 and May 2022. We collected demographics, comorbidities, injury information, interventions, and complications. Propensity score weighting was used to minimize confounding differences between those presenting on weekends (Sat-Sun) versus weekdays (Mon-Fri). Weighted regressions were used to examine differences in interventions by day of presentation. Multivariable weighted regressions accounting for differences in interventions received were used to examine whether weekend presentation was associated with amputation risk, complications, or functional deficits. Results: Ninety-five patients with 100 open extremity degloving injuries were included. In total, 39% of injuries were weekend-presenting. There was a higher rate of noninsulin-dependent diabetes among patients presenting on weekends (P = 0.03). Weekend-presenting injuries had higher median Injury Severity Scores (P = 0.04). Propensity-weighted regression analysis revealed differences in interventions received on weekends, including lower rates of pedicled and free flaps and bone graft, and increased rates of negative-pressure wound therapy (P ≤ 0.02). Multivariable regression analysis revealed weekend presentation was a significant independent risk factor for amputation of the affected extremity [odds ratio 2.27, 95% CI (1.01-5.33), P = 0.05]. Conclusion: Weekend presentation may impact interventions received and amputation risk in patients presenting with open extremity degloving injuries.

From Augmented to Virtual Reality in Plastic Surgery: Blazing the Trail to a New Frontier.

BACKGROUND: Augmented reality (AR) and virtual reality (VR)-termed mixed reality-have shown promise in the care of operative patients. Currently, AR and VR have well-known applications for craniofacial surgery, specifically in preoperative planning. However, the application of AR/VR technology to other reconstructive challenges has not been widely adopted. Thus, the purpose of this investigation is to outline the current applications of AR and VR in the operative setting. METHODS: The literature pertaining to the use of AR/VR technology in the operative setting was examined. Emphasis was placed on the use of mixed reality technology in surgical subspecialities, including plastic surgery, oral and maxillofacial surgery, colorectal surgery, neurosurgery, otolaryngology, neurosurgery, and orthopaedic surgery. RESULTS: Presently, mixed reality is widely used in the care of patients requiring complex reconstruction of the craniomaxillofacial skeleton for pre- and intraoperative planning. For upper extremity amputees, there is evidence that VR may be efficacious in the treatment of phantom limb pain. Furthermore, VR has untapped potential as a cost-effective tool for microsurgical education and for training residents on techniques in surgical and nonsurgical aesthetic treatment. There is utility for mixed reality in breast reconstruction for preoperative planning, mapping perforators, and decreasing operative time. VR has well- documented applications in the planning of deep inferior epigastric perforator flaps by creating three-dimensional immersive simulations based on a patient's preoperative computed tomography angiogram. CONCLUSION: The benefits of AR and VR are numerous for both patients and surgeons. VR has been shown to increase surgical precision and decrease operative time. Furthermore, it is effective for patient-specific rehearsal which uses the patient's exact anatomical data to rehearse the procedure before performing it on the actual patient. Taken together, AR/VR technology can improve patient outcomes, decrease operative times, and lower the burden of care on both patients and health care institutions.

Multidisciplinary Optimal Outcomes Reporting and Team Clinic Retention in Isolated Nonsyndromic Cleft Palate.

OBJECTIVE: Optimal Outcomes Reporting was recently introduced to categorize outcomes after cleft palate repair. We seek to propose an expanded version of Optimal Outcomes Reporting and to determine if correlation exists between the expanded outcomes and persistence with team care follow-up through age 9. DESIGN: Retrospective cohort study. SETTING: Cleft team at large pediatric hospital. PATIENTS: Patients with isolated nonsyndromic cleft palate (n = 83) born from 2001-2012. MAIN OUTCOME MEASURES: Patients who continued to present at age 5 or greater were assessed for optimal outcomes. Optimal outcomes were: surgery - no fistula or velopharyngeal insufficiency; otolaryngology - no obstructive sleep apnea or signs of chronic middle ear disease; audiology - no hearing loss; speech-language pathology - no assessed need for speech therapy. RESULTS: Of the 83 patients identified, 41 were assessed for optimal outcomes. Optimal outcome in any discipline was not associated with follow-up through age 9 (0.112 ≤ p ≤ 0.999). For all disciplines, the group with suboptimal outcomes had a higher proportion of patients from geographic areas in the most disadvantaged quartile of social vulnerability index, with the strongest association in the group with suboptimal speech outcome (OR 6.75, 95% CI 0.841-81.1). CONCLUSIONS: Optimal outcomes and retention in team clinic were not statistically significantly associated, but clinically relevant associations were found between patients in the most disadvantaged quartile of social vulnerability and their outcomes. A patient-centered approach, including caregiver education about long-term care for patients with cleft palate, would allow for enhanced resource utilization to improve retention for patients of concern.

Family Experiences with Diagnosis of Craniosynostosis: Thematic Analysis of Online Discussion Boards.

OBJECTIVE: Apply thematic analysis of online discussion boards to characterize families' experiences and concerns regarding craniosynostosis diagnoses to aid physicians in tailoring care to families. DESIGN: Grounded theory-based qualitative analysis. SETTING: Discussion boards related to craniosynostosis identified via Google and Yahoo. PATIENTS/PARTICIPANTS: Posts about craniosynostosis between 2017-2022. INTERVENTIONS: Thematic analysis was performed using three rounds of coding. Post features including author type and use of technical language were examined. MAIN OUTCOME MEASURE: Overarching themes emerging from analysis of posts, with forums analyzed until sufficient thematic repetition was observed. RESULTS: 366 posts from 4 websites by 290 unique users were included. Parents of patients with craniosynostosis wrote 59% of posts while patients wrote 4%. Five selective codes were identified: 1) Building Community, 2) Diagnosis/Evaluation, 3) Treatment, 4) Outcomes, and 5) Emotional Concerns. Building Community was the most assigned code (85% of posts). 71% of parents' posts expressing emotional concerns expressed negative emotions, commonly regarding anxiety about diagnosis (71%), frustration about doctors' responses (21%), or negative reactions to online search results (17%). 88% of patients' posts expressed positive emotions, discussing positive long-term outcomes. Concerns that may guide physicians included anxiety about delayed diagnosis, difficulty distinguishing postpartum head shape changes from craniosynostosis, and difficulty finding a care team. CONCLUSIONS: Online discussion boards allow families of patients with craniosynostosis to share experiences and find community. Improving communication between surgeons, pediatricians, and families about timing of evaluation and revising online information about this condition may ameliorate some anxiety associated with this diagnosis.

Following a Surgical Paradigm Shift Through the Adoption of Nerve Transfers Among Board-Eligible and Practicing Plastic Surgeons.

BACKGROUND: Nerve transfers represent a new paradigm in the treatment of nerve injuries. Their current level of adoption among surgeons is unknown. This study evaluates the incidence of nerve transfers on case logs of board-eligible plastic surgeons over the past 14 years and surveys practicing nerve surgeons regarding their use of this technique. METHODS: We queried the American Board of Plastic Surgery case log database for all nerve reconstruction Current Procedural Terminology codes from 2008 to 2021 and assessed trends and relationships between geographic region, examination year, and nerve transfer use. We surveyed nerve surgery professional societies to assess trends in practice, compared with a 2017 survey. RESULTS: A total of 1959 nerve reconstruction cases were logged by 738 candidates from 2008 to 2021. Twelve percent of cases included nerve transfers. The proportion of nerve transfer codes (Z = -11.57; P < .0001) and the proportion of candidates performing nerve transfers (Z = -9.21, P < .0001) increased over the study period. Nerve transfers were associated with geographic region (χ2 = 25.826, P = .0002), with most cases performed in the Midwest (26.4%). A higher proportion of practicing nerve surgeons reported performing nerve transfers in this survey than in our 2017 survey (χ2 = 16.7, P < .001). CONCLUSIONS: There has been an increase in nerve transfers logged in the past 14 years by board-eligible plastic surgeons, as well as increased use among currently practicing nerve surgeons. Although nerve transfer use is increasing among both plastic and orthopedic surgeons, a greater proportion of nerve reconstructions include nerve transfers in the plastic surgery cohort.

"Calvarial Vault Remodeling Technique for Lambdoid Craniosynostosis."

SUMMARY: Premature fusion of the lambdoid suture is the most uncommon single suture synostosis. It presents with a classic "windswept" appearance, with a trapezoid-shaped head and significant skull asymmetry notable for an ipsilateral mastoid bulge and contralateral frontal bossing. Due to the rarity of lambdoid synostosis, little is known about optimal techniques for its treatment. In particular, the proximity of the lambdoid suture to critical intracranial structures such as the superior sagittal and transverse sinuses represents a potential for significant intraoperative bleeding. Prior work has shown that parietal asymmetry persists after repair in these cases. Here, we present a technique for the treatment of unilateral lambdoid craniosynostosis along with two representative cases.This calvarial vault remodeling technique requires the removal of both ipsilateral and contralateral parietal bones. These are moved across hemispheres and re-inset on opposite sides to help correct the parietal asymmetry. Obliquely orientated barrel stave osteotomies are performed to provide a safe mechanism for correction of occipital flattening. Our early results show improvement in correction of volume asymmetry one year post-operatively relative to patients treated with prior calvarial vault remodeling techniques. We believe the technique presented here corrects the windswept appearance in patients with lambdoid craniosynostosis while also reducing the potential for complications. Further work will be necessary to confirm this technique's long-term efficacy in a larger cohort.

Impact of neighborhood deprivation and social vulnerability on long-term outcomes and desire for revision in patients with craniosynostosis.

OBJECTIVE: The authors utilized the area deprivation index (ADI), a validated composite measure of socioeconomic disadvantage, and the social vulnerability index (SVI) to examine whether differences in neighborhood deprivation impact interventions and outcomes among patients with craniosynostosis. METHODS: Patients who underwent craniosynostosis repair between 2012 and 2017 were included. The authors collected data about demographic characteristics, comorbidities, follow-up visits, interventions, complications, desire for revision, and speech, developmental, and behavioral outcomes. National percentiles for ADI and SVI were determined using zip and Federal Information Processing Standard (FIPS) codes. ADI and SVI were analyzed by tertile. Firth logistic regressions and Spearman correlations were used to assess associations between ADI/SVI tertile and outcomes/interventions that differed on univariate analysis. Subgroup analysis was performed to examine these associations in patients with nonsyndromic craniosynostosis. Differences in length of follow-up among the nonsyndromic patients in the different deprivation groups were assessed with multivariate Cox regressions. RESULTS: In total, 195 patients were included, with 37% of patients in the most disadvantaged ADI tertile and 20% of patients in the most vulnerable SVI tertile. Patients in more disadvantaged ADI tertiles were less likely to have physician-reported desire (OR 0.17, 95% CI 0.04-0.61, p < 0.01) or parent-reported desire (OR 0.16, 95% CI 0.04-0.52, p < 0.01) for revision, independent of sex and insurance status. In the nonsyndromic subgroup, inclusion in a more disadvantaged ADI tertile was associated with increased odds of speech/language concerns (OR 4.42, 95% CI 1.41-22.62, p < 0.01). There were no differences in interventions received or outcomes among SVI tertiles (p ≥ 0.24). Neither ADI nor SVI tertile was associated with risk of loss to follow-up among nonsyndromic patients (p ≥ 0.38). CONCLUSIONS: Patients from the most disadvantaged neighborhoods may be at risk for poor speech outcomes and different standards of assessment for revision. Neighborhood measures of disadvantage represent a valuable tool to improve patient-centered care by allowing for modification of treatment protocols to meet the unique needs of patients and their families.

The Association between Breast Cancer Related Lymphedema and Area Deprivation Index.

BACKGROUND: Lymphedema affects up to 34% of patients after breast cancer treatment but remains underdiagnosed and undertreated. Here, we use area deprivation index (ADI), a measure of neighborhood socioeconomic disadvantage, to determine how socioeconomic status may affect risk for and diagnosis of breast cancer-related lymphedema. METHODS: Records of patients who underwent surgical treatment of breast cancer between 2017-2020 were examined. Patients' nine-digit ZIP codes were utilized to determine their deprivation level as a national ADI percentile, and those fitting into the most and least deprived quartiles were compared with evaluate lymphedema risk factors and incidence. RESULTS: A total of 1,333 breast cancer patients were included, 812 (61%) of whom resided within the most disadvantaged ADI quartile nationally, and 521 within the least disadvantaged quartile. The most deprived group had higher rates of diabetes, obesity, and regional breast cancer, and received more extensive surgeries (7.5% modified radical mastectomy vs 1.9%, p < 0.001) and chemotherapy compared with the least disadvantaged quartile. The most disadvantaged cohort were more often at extreme risk of lymphedema utilizing the Risk Assessment Tool Evaluating Lymphedema Risk (9.1% versus 2.5%, p < 0.001); however, the incidence of lymphedema diagnoses was not significantly higher (13% vs 12%, p > 0.9). Logistic regression showed that the most deprived ADI quartile had 44% lower odds of a lymphedema diagnosis in comparison to the least deprived quartile. CONCLUSION: Residing in more socioeconomically disadvantaged neighborhoods is associated with lower odds of a lymphedema diagnosis, despite higher rates of risk factors for lymphedema, suggesting significant underdiagnosis in this population.

Predictors and Consequences of Intraoperative Anastomotic Failure in DIEP Flaps.

BACKGROUND: Successful intraoperative microvascular anastomoses are essential for deep inferior epigastric perforator (DIEP) flap survival. This study identifies factors associated with anastomotic failure during DIEP flap reconstruction and analyzes the impact of these anastomotic failures on postoperative patient outcomes and surgical costs. METHODS: A retrospective cohort study was conducted of patients undergoing DIEP flap reconstruction at two high-volume tertiary care centers from January 2017 to December 2020. Patient demographics, intraoperative management, anastomotic technique, and postoperative outcomes were collected. Data were analyzed using Student's t-tests, Chi-square analysis, and multivariate logistic regression. RESULTS: Of the 270 patients included in our study (mean age 52, majority Caucasian [74.5%]), intraoperative anastomotic failure occurred in 26 (9.6%) patients. Increased number of circulating nurses increased risk of anastomotic failure (odds ratio [OR] 1.02, 95% confidence Interval [CI] 1.00-1.03, p <0.05). Presence of a junior resident also increased risk of anastomotic failure (OR 2.42, 95% CI 1.01-6.34, p <0.05). Increased surgeon years in practice was associated with decreased failures (OR 0.12, CI 0.02-0.60, p <0.05). Intraoperative anastomotic failure increased the odds of postoperative hematoma (OR 8.85, CI 1.35-59.1, p <0.05) and was associated with longer operating room times (bilateral DIEP: 2.25 hours longer, p <0.05), longer hospital stays (2.2 days longer, p <0.05), and higher total operating room cost ($28,529.50 vs. $37,272.80, p <0.05). CONCLUSION: Intraoperative anastomotic failures during DIEP flap reconstruction are associated with longer, more expensive cases and increased rates of postoperative complications. Presence of increased numbers of circulators and junior residents was associated with increased risk of anastomotic failure. Future research is necessary to develop practice guidelines for optimizing patient and surgical factors for intraoperative anastomotic success.

Porous Polyethylene Versus Autologous Costochondral Reconstruction for Microtia: Incidence and Analysis of Secondary Procedures.

OBJECTIVE: To examine the frequency of autologous and alloplastic ear reconstructions for patients with microtia in the United States, and describe post-index procedure rates associated with each method. DESIGN: Retrospective cohort study. SETTING: Claims data from 500 + hospitals from IBM® MarketScan® Commercial and Multi-State Medicaid databases. PATIENTS/PARTICIPANTS: A total of 649 patients aged 1 to 17 years with International Classification of Diseases, ninth/tenth revision (ICD-9/10) diagnoses for microtia, congenital absence of the ear, or hemifacial microsomia. INTERVENTIONS: Alloplastic or autologous ear reconstruction between 2006 and 2018. MAIN OUTCOME MEASURE: Post-index procedures performed within 1 year following the index repair, analyzed across the study period and separately for each half of the study period (2006-2012, 2012-2018). RESULTS: A total of 486 (75%) qualifying patients received autologous and 163 (25%) received alloplastic reconstruction. Secondary procedure rates were significantly higher in the autologous group at 90 days (P = .034), 180 days (P < .001), and at 365 days (P < .001). Alloplastic reconstruction accounted for 23.2% of reconstructions in the first half of the study period compared with 26.7% in the second half (P = .319). One-year secondary procedure rates in the autologous group were not significantly different between both halves of the study period (69.7% vs 67.1%, P = .558), but were significantly lower in the second half for the alloplastic group (44.9% vs 20.2%, P = .001). CONCLUSIONS: In these databases, autologous reconstruction is more common than alloplastic reconstruction. Autologous reconstruction is staged, with most undergoing a secondary procedure between 3 months and 1 year postoperatively. Secondary procedure rates decreased over time in patients undergoing alloplastic reconstruction.

School-age outcomes in patients with unilateral coronal synostosis: comparison of fronto-orbital advancement and endoscopic strip craniectomy.

OBJECTIVE: Outcomes research on unilateral coronal synostosis is mostly limited to the early postoperative period. This study examines facial asymmetry, desire for revision, and patient-reported outcomes at school age in children who received either endoscopic strip craniectomy with helmet therapy or fronto-orbital advancement (open repair). METHODS: Patients with repaired unilateral coronal synostosis born between 2000 and 2017, with 3D photographs taken when they were between 3.5 and 8 years of age, were eligible for study inclusion. Three pairs of bilateral linear measurements and two angular measurements were taken. Parent- and physician-reported desire for revision and patient-reported outcomes (Patient-Reported Outcomes Measurement Information System cognitive function and Quality of Life in Neurological Disorders stigma scores) were collected from patient charts. RESULTS: Thirty-five patients qualified, including 25 with open repair and 10 with endoscopic repair. The median patient ages at repair were 3 months and 8 months in the endoscopic and open groups, respectively. The average ages at final 3D photography were 5.8 years and 5.5 years in the endoscopic and open groups, respectively. Digital anthropometry revealed no significant differences in measures of facial asymmetry between the repair groups (p ≥ 0.211). Midface depth (tragion to subnasale) was significantly less symmetric at school age than other linear measures (F(2,102) = 9.14, p < 0.001). Forehead asymmetry was significantly associated with parent- and physician-reported desire for revision (p ≤ 0.006). No significant associations were found between physical asymmetry and patient-reported stigma or cognitive function (p > 0.046, Holm-Bonferroni correction). CONCLUSIONS: Children who underwent open or endoscopic repair for unilateral coronal synostosis have comparable facial symmetry at school age, but midface depth remains highly asymmetrical in both groups. Forehead asymmetry at school age correlates with parent- and physician-reported desire for revision.

Syndromic Hydrocephalus.

Hydrocephalus, the abnormal accumulation and impaired circulation/clearance of cerebrospinal fluid, occurs as a common phenotypic feature of a diverse group of genetic syndromes. In this review, we outline the genetic mutations, pathogenesis, and accompanying symptoms underlying syndromic hydrocephalus in the context of: L1 syndrome, syndromic craniosynostoses, achondroplasia, NF 1/2, Down's syndrome, tuberous sclerosis, Walker-Warburg syndrome, primary ciliary dyskinesia, and osteogenesis imperfecta. Further, we discuss emerging genetic variants associated with syndromic hydrocephalus.

Clinical Staging to Estimate the Probability of Severe Postoperative Complications in Patients With Vestibular Schwannoma.

Importance: Vestibular schwannomas have long been treated as a homogeneous entity. Clinical symptoms at presentation may help elucidate the underlaying pathophysiologic characteristics of tumor subtypes. Describing the heterogeneity of these benign tumors may assist in predicting clinical outcomes associated with their treatment. Objective: To create a tumor staging system that incorporates symptoms at presentation and tumor size to predict severe surgical complications. Design, Setting, and Participants: A retrospective cohort of patients at a single-center tertiary referral center from January 1, 1998, to October 13, 2020, was studied. Patients diagnosed with sporadic vestibular schwannoma surgically treated at Washington University in St Louis, Missouri, were included. Main Outcomes and Measures: Severe surgical complications within 30 days of surgery as determined by the Clavien-Dindo classification system. Patients experiencing a complication of grade 3 or above were determined to have a severe complication. Results: Of 185 patients evaluated, 40 (22%) had severe postoperative complications. Twenty of the 40 patients (50%) were women; mean (SD) age was 46 (13) years. Patients with severe complications were more likely to have large tumors (>2.5 cm in largest diameter), vestibular symptoms, and recent hearing loss at presentation. Using conjunctive consolidation, a 4-stage clinical severity staging system that incorporates clinical symptoms and tumor size at presentation was created to predict severe complications. The clinical severity staging system demonstrated an improvement in the ability to discriminate severe complications (C index, 0.754; 95% CI, 0.67-0.84) from a model of tumor size alone (C index, 0.706; 95% CI 0.62-0.79). Conclusions and Relevance: This cohort study found that, among patients with vestibular schwannoma, symptoms present at initial evaluation, in addition to tumor size, served as predictors of severe postoperative complications. A new clinical severity staging system incorporating symptoms at presentation can be helpful for clinicians to identify patients at high risk for severe postoperative complications.