Skin fragility and wound management in Ehlers- Danlos Syndrome: a report by the Ehlers Danlos Syndrome society skin working group.

The Ehlers-Danlos Syndromes (EDS) are a heterogenous group of heritable connective tissue disorders, characterised by joint hypermobility, skin hyperextensibility and generalised tissue fragility. In all types of EDS skin wound healing is impaired to a variable degree. Additional support through wound management plans may help to improve these outcomes, however, there is paucity of evidence regarding clinical management of skin fragility and wounds in EDS. This paper aims to review current evidence and provide recommendations for management of skin wounds in EDS types. Preventative measures to avoid skin injury are strongly recommended, including avoidance of high impact sport and use of appropriate protection such as shin guards. Bruising is common and some types of EDS are associated with haematoma formation with management including compression bandages and consideration of pharmacological therapy. Skin fragility and tears should be managed with a focus on protection of remaining tissue, avoidance of wound tension and low adherence dressings to avoid further injury. This paper provides clear recommendations to address skin management for this group of patients. It highlights the lack of good quality published data to support treatment decisions.

The Severity of Initial Unilateral Cleft Lip and Nasal Deformity Predicts Long-Term Postoperative Esthetic Outcome.

BACKGROUND: Infantile cleft lip and nasal severity influence the final esthetic result of the repair. Although various authors have described methods of cleft lip and nasal repair, there is a paucity of data that correlates cleft severity with esthetic outcomes. The aim of this study was to examine the correlation between presurgical severity of unilateral cleft deformity and long-term postoperative esthetic outcomes. METHODS: This retrospective study, based at a single institution, investigated patients with complete unilateral cleft lip, with or without cleft palate, who underwent repair by a single surgeon, had preoperative infantile facial casts, and had postoperative facial photographs at 6 to 11 years of age (N=31). Preoperative nostril width ratio and columellar angle measurements were taken from facial casts. Postoperative, long-term nasolabial appearance was rated by 5 blinded observers used a modified Kuijpers-Jagtman scale. Linear regression was used to determine the relationship between preoperative cleft severity and postoperative ratings. RESULTS: Preoperative nostril width ratio directly correlated with postoperative nasal form score (r=0.40; P=0.026); likewise, preoperative columellar angle predicted postoperative nasal form score (r=0.37; P=0.040). Preoperative cleft severity was not significantly correlated with vermillion border appearance. Cronbach α values of 0.91 (nasal form) and 0.79 (vermillion border) indicated good inter-rater reliability. Kappa values of 0.87 (nasal form) and 0.70 (vermillion border) indicated good intrarater reliability. CONCLUSIONS: Preoperative unilateral cleft nose severity directly correlates with long-term postoperative nasal appearance in childhood. Outcome studies should present and control for preoperative severity to allow more accurate assessment of repair techniques.

Strength in Numbers: A SWOT Analysis of Plastic Surgery.

Background: Plastic surgeons comprise the minority of practicing surgeons, with an even smaller minority practicing in an academic setting. As the practice of medicine and the systems in which we operate continue to evolve, it is essential that plastic surgeons have a say in the changing landscape. This study conducted a strengths, weaknesses, opportunities, and threats (SWOT) analysis of plastic surgery to identify unifying strengths and common threats. Methods: An electronic survey was distributed to American Council of Academic Plastic Surgeons' Winter Meeting attendees on three separate occasions preceding the meeting. Respondents were asked to provide demographic information and to identify the top three strengths, weaknesses, opportunities, and threats (SWOT analysis) for the specialty. Subgroup analyses were performed based on demographic characteristics. Results: A total of 187 responses were received from meeting attendees, representing an 89.0% response rate. Most respondents were non-Hispanic (78.6%), White (66.8%), women (59.5%), and faculty/independent physicians (65.8%). The most identified strength in plastic surgery was our problem-solving abilities (62.0%). The most identified weakness was poor public perception of plastic surgery (54.0%). The most identified opportunity was demonstration of value to health systems (67.9%), and the most identified threat was scope of practice creep by other specialties (78.1%). The SWOT analysis identified lack of surgeon diversity as a key weakness, improvement of surgeon diversity as a key opportunity, and lack of diversity among plastic surgeons as a key threat to the specialty. Conclusion: Only through a diverse but united front can we effectively use our strengths to face our threats and employ opportunities to overcome our weaknesses.

Topical vanadate improves tensile strength and alters collagen organisation of excisional wounds in a mouse model.

Wound dehiscence, oftentimes a result of the poor tensile strength of early healing wounds, is a significant threat to the post-operative patient, potentially causing life-threatening complications. Vanadate, a protein tyrosine phosphatase inhibitor, has been shown to alter the organisation of deposited collagen in healing wounds and significantly improve the tensile strength of incisional wounds in rats. In this study, we sought to explore the effects of locally administered vanadate on tensile strength and collagen organisation in both the early and remodelling phases of excisional wound healing in a murine model. Wild-type mice underwent stented excisional wounding on their dorsal skin and were divided equally into three treatment conditions: vanadate injection, saline injection control and an untreated control. Tensile strength testing, in vivo suction Cutometer analysis, gross wound measurements and histologic analysis were performed during healing, immediately upon wound closure, and after 4 weeks of remodelling. We found that vanadate treatment significantly increased the tensile strength of wounds and their stiffness relative to control wounds, both immediately upon healing and into the remodelling phase. Histologic analysis revealed that these biomechanical changes were likely the result of increased collagen deposition and an altered collagen organisation composed of thicker and distinctly organised collagen bundles. Given the risk that dehiscence poses to all operative patients, vanadate presents an interesting therapeutic avenue to improve the strength of post-operative wounds and unstable chronic wounds to reduce the risk of dehiscence.

Successful application of the innovation process to a case of Floyd Type I tracheal agenesis.

Background: Innovation is broadly defined as the act of introducing a new product, idea, or process. The field of surgery is built upon innovation, revolutionizing technology, science, and tools to improve patient care. While most innovative solutions are aimed at problems with a significant patient population, the process can also be used on orphan pathologies without obvious solutions. We present a case of tracheal agenesis, a rare congenital anomaly with an overwhelming mortality and few good treatment options, that benefited from the innovation process and achieved survival with no ventilator dependence at three years of age. Methods: Utilizing the framework of the innovation process akin to the Stanford Biodesign Program, 1) the parameters of the clinical problem were identified, 2) previous solutions and existing technologies were analyzed, newly invented solutions were brainstormed, and value analysis of the possible solutions were carried out using crowd wisdom, and 3) the selected solution was prototyped and tested using 3D modeling, iterative testing on 3D prints of actual-sized patient parts, and eventual implementation in the patient after regulatory clearance. Results: A 3D-printed external bioresorbable splint was chosen as the solution. Our patient underwent airway reconstruction with "trachealization of the esophagus": esophageotracheal fistula resection, esophagotracheoplasty, and placement of a 3D-printed polycaprolactone (PCL) stent for external esophageal airway support at five months of age. Conclusions: The innovation process provided our team with the guidance and imperative steps necessary to develop an innovative device for the successful management of an infant survivor with Floyd Type I tracheal agenesis. Article summary: We present a case of tracheal agenesis, a rare congenital anomaly with an overwhelming mortality and few good treatment options, that benefited from the innovation process and achieved survival with no ventilator dependence at three years of age.The importance of this report is to reveal how the innovation process, which is typically used for problems with significant patient population, can also be used on orphan pathologies without obvious solutions.

Interfacility Transfer Guidelines for Isolated Facial Trauma: A Multidisciplinary Expert Consensus.

BACKGROUND: The objective of this study was to develop guidelines for the transfer of patients with isolated craniomaxillofacial trauma. METHODS: A national, multidisciplinary expert panel was assembled from leadership in national organizations and contributors to published literature on facial reconstruction. The final panel consisted of five plastic surgeons, four otolaryngologist-head and neck surgeons, and four oral and maxillofacial surgeons. The expert panelists' opinions on transfer guidelines were collected using the modified Delphi process. Consensus was predefined as 90 percent or greater agreement per statement. RESULTS: After four Delphi consensus building rounds, 13 transfer guidelines were established, including statements on fractures of the frontal sinus, orbit, midface, and mandible, as well as soft-tissue injuries. Twelve guidelines reached consensus. CONCLUSIONS: The decision to transfer a patient with craniomaxillofacial trauma to another facility is complex and multifactorial. While a percentage of overtriage is acceptable to promote safe disposition of trauma patients, unnecessarily high rates of secondary overtriage divert emergency medical services, increase costs, delay care, overload tertiary trauma centers, and result in tertiary hospital staff providing primary emergency coverage for referring hospitals. These craniomaxillofacial transfer guidelines were designed to serve as a tool to improve and streamline the care of facial trauma patients. Such efforts may decrease the additional health care expenditures associated with secondary overtriage while decompressing emergency medical systems and tertiary emergency departments.

Interfacility Transfers for Isolated Craniomaxillofacial Trauma: Perspectives of the Facial Trauma Surgeon.

STUDY DESIGN: Secondary overtriage is a burden to the medical system. Unnecessary transfers overload trauma centers, occupy emergency transfer resources, and delay definitive patient care. Craniomaxillofacial (CMF) trauma, especially in isolation, is a frequent culprit. OBJECTIVE: The aim of this study is to assess the perspectives of facial trauma surgeons regarding the interfacility transfer of patients with isolated CMF trauma. METHODS: A 31-item survey was developed using Likert-type scale and open-ended response systems. Internal consistency testing among facial trauma surgeons yielded a Cronbach's α calculation of .75. The survey was distributed anonymously to the American Society of Maxillofacial Surgeons, the North American Division of AO Craniomaxillofacial, and the American Academy of Facial Plastic and Reconstructive Surgery. Statistical significance in response plurality was determined by nonoverlapping 99.9% confidence intervals (P < .001). Sum totals were reported as means with standard deviations and z scores with P values of less than .05 considered significant. RESULTS: The survey yielded 196 responses. Seventy-seven percent of respondents did not believe that most isolated CMF transfers required emergency surgery and roughly half (49%) thought that most emergency transfers were unnecessary. Fifty-four percent of respondents agreed that most patients transferred could have been referred for outpatient management and 87% thought that transfer guidelines could help decrease unnecessary transfers. Twenty-seven percent of respondents had no pre-transfer communication with the referring facility. Perspectives on the transfer of specific fracture patterns and their presentations were also collected. CONCLUSION: Most facial trauma surgeons in this study believe that emergent transfer for isolated CMF trauma is frequently unnecessary. Such injuries rarely require emergent surgery and can frequently be managed in the outpatient setting without activating emergency transfer services. The fracture-specific data collected are a representation of the national, multidisciplinary opinion of facial trauma surgeons and correlate with previously published data on which specific types of facial fractures are most often transferred unnecessarily. The results of this study can serve as the foundation for interfacility transfer guidelines, which may provide a valuable resource in triaging transfers and decreasing associated health-care costs.

Computer-Aided Design and Manufacture of Intraoral Splints: A Potential Role in Cleft Care.

BACKGROUND: Nasoalveolar molding is a nonsurgical modality for the treatment of cleft lip and palate that uses an intraoral splint to align the palatal shelves. Repeated impressions are needed for splint modification, each carrying risk of airway obstruction. Computer-aided design and manufacturing (CAD/CAM) has the ability to simplify the process. As a precursor to CAD/CAM splint fabrication, a proof-of-concept study was conducted to compare three-dimensional splints printed from alginate impressions versus digital scans. We hypothesized that intraoral digital scanning would compare favorably to alginate impressions for palate registration and subsequent splint manufacture, with decreased production times. METHODS: Alginate and digital impressions were taken from 25 healthy teenage volunteers. Digital impressions were performed with a commercially available intraoral scanner. Plaster casts made from alginate impressions were converted to Standard Triangle Language files. Patient-specific matched scans were evaluated for total surface area with the concordance correlation coefficient. Acrylic palatal splints were three-dimensionally printed from inverse digital molds. Subjective appliance fit was assessed using a five-point scale. RESULTS: A total of 23 participants were included. Most subjects preferred digital impression acquisition. Impression methods showed moderate agreement (concordance correlation coefficient 0.93). Subjects rated splints from digital impressions as having a more precise fit (4.4 versus 3.9). The digital approach decreased impression phase time by over 10-fold and overall production time by 28%. CONCLUSIONS: CAD/CAM has evolved extensively over the past two decades and is now commonplace in medicine. However, its utility in cleft patients has not been fully realized. This pilot study demonstrated that CAD/CAM technologies may prove useful in patients requiring intraoral splints.

Ethnic and Gender Diversity Comparison between Surgical Patients and Caring Surgeons.

BACKGROUND: Diversity within the medical workforce remains a topic of discussion in academia, particularly when it comes to the underrepresentation of certain ethnic groups and gender in the surgical specialties. In this article, we look at how the gender and ethnicity of surgeons at a large academic institution in a rural setting compare with those of the population it serves. METHODS: We looked at demographic data from 2008 to 2018 and compared population trends among surgeons and patients. RESULTS: We found that while whites represent the large majority in both the surgeon and patient populations, absolute number and percentage of whites in the patient population seem to be trending downward from 2008 to 2018, but trending upward among surgeons (attendings and residents). In addition, we found that while Asians make up only 1% of the patient population, they represent the second largest group (17%) among surgeons, with more than twice the proportion percentage of the second largest group within the patient population, composed of Hispanics (6%). Finally, we found a significant gender difference between the 2 populations with almost two-thirds of the surgeons being men, compared with the nearly even split of men and women within the patient population. CONCLUSIONS: Ultimately, understanding how gender and ethnic diversity in the surgical workforce compares with that of the patient population being served may aid in designing training programs to address cultural competency and awareness as well as in impacting administrative decisions and hiring.

Indoor Dust/Air Partitioning: Evidence for Kinetic Delay in Equilibration for Low-Volatility SVOCs.

Effective modeling of semivolatile organic chemical (SVOC) partitioning between air and indoor dust is investigated by calculating partition ratios for selected SVOCs between air and n-octanol as well as 8 other oligomers similar in chemical structure to common components of household dust. COSMO-RS solvation theory was used to calculate air-oligomer partition ratios, which were converted to estimates for KOA (octanol-air) and Kdust-air. The results are compared with reported monitored partition ratios with good agreement for the more volatile SVOCs of vapor pressure (VP) exceeding 10-5 Pa and corresponding calculated log Kdust-air (m3 g-1) of <5.5 or unitless log KOA of <11.5. For less volatile SVOCs, reported values of KDA are significantly lower than predicted, with the deviation increasing with decrease in VP. This effect is attributed to a kinetic delay in which characteristic times for equilibration exceed the dust-air contact time, and equilibrium is not achieved. It is hoped that the approach of computing partition ratios of SVOCs using oligomers selected on the basis of likely dust sources and compositions may improve the estimation of indoor human exposure to SVOCs present in air and dust and assist in exploiting dust samples to monitor and evaluate the fate and exposure of organic chemicals used indoors.

A perspective on the role of fugacity and activity for evaluating the PBT properties of organic chemicals and providing a multi-media synoptic indicator of environmental contamination.

The hazard and risk from organic chemicals present in the environment are routinely evaluated using P (persistence), B (bioaccumulation) and T (toxicity) criteria. We present a perspective on how models based on the equilibrium criteria of fugacity and chemical activity can contribute to all three evaluations, thus providing a consistent and seamless assessment process. Persistence and its closely related, but fundamentally different property, chemical residence time can be determined from degradation half-lives (typically obtained from monitoring data) and multi-media mass balance models describing chemical fate and transport in real or evaluative environments. Bioaccumulation is driven by equilibrium partitioning processes that can be estimated from fugacity models treating uptake by respiration and diet in single organisms and in food webs, most commonly for aquatic systems for which confirmatory monitoring data can be obtained. Biomagnification is readily evaluated both experimentally and as a fugacity (or activity) increase from prey to predator. Toxicokinetic evaluations of chemical uptake and disposition in a variety of organisms, including humans, can be obtained using fugacity- and physiologically-based pharmacokinetic models. Toxicodynamic processes of chemical interactions with organisms leading to adverse toxic outcomes are less obviously amenable to fugacity assessment with the notable exception of baseline toxicity (narcosis), the most common mode of action of commercial chemicals. It is shown that a range of fugacities can be defined and correlated for specific species thus enabling direct comparison of fugacities estimated or monitored in the environment with fugacities resulting in baseline toxicity. Finally, it is shown that fugacity and activity can serve as multi-media metrics of chemical contamination in ecosystems, thus enabling identification of specific regions and species at greatest risk in a pictorial format that is readily understandable to scientific and regulatory communities and to the general public.

A Prospective Approach to Inform and Treat 1340 Patients at Risk for BIA-ALCL.

BACKGROUND: The increasing incidence and associated mortality of breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) has become alarming. However, many patients remain unaware of their risk for BIA-ALCL and may overlook early warning signs of the cancer. The authors aim to contact all breast implant patients at a single institution to educate them on the disease and provide screening and treatment as indicated. METHODS: All patients who had breast implants placed at Penn State Hershey Medical Center from 1979 to November of 2017 were mailed a letter to describe BIA-ALCL and to encourage a follow-up visit. Patient information regarding demographics, implant type, the number of calls and follow-up visits, physical examination findings, and patient decisions after being informed of the disease were recorded prospectively. RESULTS: One thousand two hundred eighty-four letters were mailed to 1020 patients (79.4 percent) with smooth implants and 264 patients (20.6 percent) with textured implants. Seventy-six calls were received and 100 patients (84 smooth and 16 textured) were evaluated within the first 2 months. Of the 16 patients with textured implants, nine are undergoing secondary surgery to remove or replace their textured device. CONCLUSIONS: Informing patients at risk for BIA-ALCL is an important endeavor. Patients educated on the disease will likely be diagnosed and treated earlier, which can prevent the need for adjuvant chemotherapy and/or radiation therapy and decrease mortality. The authors provide a method, supporting documents, and preliminary data to help other institutions contact their breast implant patients at risk for BIA-ALCL.

Autologous Fat Grafting in Cleft Lip and Palate.

Structural fat grafting is a technically simple procedure that has revolutionized the field of plastic surgery, with applications ranging from tissue regeneration to scar modulation to volume restoration. This technique has found applications in the treatment of cleft lip and palate, where it can be used to fill deficient midfacial volume and treat velopharyngeal insufficiency. In this article, the authors summarize the current literature supporting the use of fat grafting in patients with clefts. Evidence as to the safety, efficacy, longevity, and potential complications of the technique are discussed.

QSPRs for Molecular Diffusion Coefficients in Polymeric Passive Samplers: A Comparison of Simple Molecular and Quantum-mechanical Sigma-moment Descriptors.

Linear quantitative structure-property relationships (QSPRs) for the prediction of diffusion coefficients (log Dp ) were developed for organic contaminants in two common passive sampler materials, polydimethylsiloxane (PDMS) and low-density polyethylene (LDPE). Literature data was compiled for both PDMS and LDPE resulting in final data sets of 196 and 79 compounds, respectively. Data sets contained compounds with log Dp values that ranged over about 5 log units and 3 log units for PDMS and LDPE, respectively. The quality of log Dp prediction using either simple molecular descriptors or quantum-chemical based COSMO-RS sigma moment descriptors was compared for both materials. For PDMS, the sigma moment descriptor QSPR had the best predictivity with a correlation coefficient of R2 =0.85 and root mean square error (RMSE) of 0.36 for log Dp . The molecular descriptor QSPR resulted in a correlation coefficient of R2 =0.78 and RMSE of 0.45 for log Dp . For LDPE, the molecular descriptor QSPR had the best predictivity, with the final correlation coefficient of R2 =0.86 and RMSE of 0.21 for log Dp . The sigma moment descriptor QSPR resulted in a correlation coefficient of R2 =0.66 and RMSE of 0.33 for log Dp . The purely electronic structure-based sigma moments are therefore shown to be a viable option for descriptors compared to the more commonly used molecular descriptors for organic contaminants in PDMS. The significance of the descriptors in each QSPR is discussed.

Relations between emotion, memory encoding, and time perception.

This study examined duration judgments for taboo and neutral words in prospective and retrospective timing tasks. In the prospective task, participants attended to time from the beginning and generated shorter duration estimates for taboo than neutral words and for words that they subsequently recalled in a surprise free recall task. These findings suggested that memory encoding took priority over estimating durations, directing attention away from time and causing better recall but shorter perceived durations for taboo than neutral words. However, in the retrospective task, participants only judged durations in a surprise test at the end, and their duration estimates were longer for taboo than neutral words. Present findings therefore suggest that the same emotion-linked memory encoding processes can cause underestimation of durations in prospective tasks but overestimation in retrospective tasks, as if emotion enhances recall of ongoing events but causes overestimation of the durations of those events in retrospect.

Kinetic Delay in Partitioning and Parallel Particle Pathways: Underappreciated Aspects of Environmental Transport.

We outline the general conditions under which chemicals of high hydrophobicity or, more generally, high partition ratios (HPRs) in water or air, may experience unexpectedly long kinetic delays in approaching equilibrium conditions with organic media. Such a "hydrophobic delay" in the case of K OW or an "aerophobic" delay in the case of KOA may be misinterpreted as being caused by a change in partitioning behavior or mechanism, resulting in development of nonlinear regression models describing intermedia partitioning. In fact, the partitioning is fundamentally linear but is distorted by a kinetic delay in partitioning. To illustrate this concept, we first compile the fundamental equations describing the diffusive equilibration processes, including a complementary transport mechanism termed a parallel particle pathway (PPP). Such a mechanism may occur simultaneously, shortening the HPR delay and complicating interpretation. Second, we describe five examples in which the HPR delay explains the observed and occasionally difficult-to-interpret environmental behavior of chemicals, namely studies of air-aerosol partitioning, chemical accumulation in indoor dust and surfaces, air-vegetation partitioning, internal transport in organisms, and fish bioaccumulation and toxicity. We believe that the general HPR delay and PPP issues deserve exposure as a commonly occurring and often underappreciated process.