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Periventricular nodular heterotopia (PNH) is a neuronal migration defect characterized by the presence of ectopic grey matter nodules adjacent to the walls of the lateral ventricles. The main genetic etiology of PNH are variants in the Filamin A gene (FLNA, MIM #300049), located in the X chromosome. It affects mostly females (embryonic lethality in males), with about 50% of cases inherited from healthy mothers or with a mild phenotype. It is associated with epilepsy (75%-90%), cardiovascular (65%) and pulmonary pathologies (25%).A 28-year-old primigravida was referred for prenatal care in obstetrics department because of personal history of obliterative bronchiolitis. She has a family history of asthma (mother and sister) and adulthood-onset epilepsy (father). The pregnancy was uneventful up to 20 weeks and 3 days when bilateral periventricular irregularities and mega cisterna magna were identified on ultrasound in a female fetus. Neurosonography was performed, which led to the hypothesis of diffuse PNH, supported by MRI. The hypothesis of PNH associated to the FLNA gene was made. Brain MRI on the pregnant woman was requested, which confirmed a similar pattern of PNH. The arrayCGH (PerkinElmer, Prenatal filter 37K) was normal, and whole exome sequencing identified the likely pathogenic c.1554del p.(Val519fs*) variant in the FLNA gene.We present a case of X-linked hereditary PNH that highlights the value of fetal neurosonography in making a putative diagnosis. The diagnosis was supported by MRI in both fetus and mother. The investigation was supplemented by genetic studies, which confirmed the diagnosis.

Background: In 2022, the American Association for Thoracic Surgery (AATS) and the European Society of Thoracic Surgeons (ESTS) published joint guidelines regarding the timing, duration, and choice of agent for perioperative venous thromboembolism prophylaxis for thoracic cancer patients. Now, 1 year after their release, we looked to assess practices and general adherence to these recommendations. Methods: We conducted a survey among board-certified/board-eligible thoracic surgeons in the United States, between July and October 2023. Results: A total of 103 board-certified thoracic surgeons responded to the survey. Over half of the surgeons reported using preoperative chemical thromboprophylaxis routinely for lobectomy/sublobar resections (56.3%), pneumonectomy/extended lung resections (64.1%), and esophagectomy (67%). Over two thirds of thoracic surgeons limited the duration of postoperative chemical thromboprophylaxis to the patient’s length of hospital stay and never administered chemoprophylaxis post-discharge. Among surgeons who always continued chemical thromboprophylaxis post-discharge, low-molecular-weight heparin (LMWH) was the most commonly used agent (>70%), followed by direct oral anticoagulants (13.8%-16.7%). Only 33.3% of surgeons prescribing post-discharge chemical thromboprophylaxis after lobectomy/sublobar resections continued prophylaxis up to 4 weeks postoperatively. Conclusions: Contrary to the 2022 joint AATS/ESTS guidelines, the majority of surveyed thoracic surgeons in the United States do not routinely prescribe postoperative thromboprophylaxis after lung and esophageal cancer resections. The dogma of routine extended thromboprophylaxis must be reevaluated as modern minimally invasive thoracic surgery allows for very earlier ambulation and enhanced recovery. There is a need for randomized controlled trials exploring the utility of extended thromboprophylaxis and newer agents such as direct oral anticoagulants.

Aortic coarctation is a rare cause of secondary hypertension (

Communication is a core component of a clinician’s role; however, when clinicians conduct research, communicating the emerging findings and recommendations to different types of stakeholders can be unfamiliar territory. Communicating research to advocate for change can be even more challenging. Clinician researchers seeking to be agents for change need to conceive and craft specific, evidence-based messages and communicate these effectively to different stakeholders to negotiate action. As part of a global health research program, we developed and tested a novel game-based model to strengthen the communication skills of clinician researchers, from 4 countries, for improving services for chronic obstructive pulmonary disease. This model focused on communication with 3 key stakeholder groups for knowledge translation: Patients/carers, healthcare providers and policy makers/healthcare managers. Delivered through a series of facilitated, online meetings, this model consisted of 2 parts: developing and rehearsing advocacy messages with coaching support, and then testing them with a panel of 3 representative stakeholders, and an audience of fellow researchers. All the country teams reported increased confidence in crafting advocacy messages for specific stakeholders and have applied lessons learned from the model. Delivering this model within a global health research program requires mentoring, time, commitment, resources and translation support to address language barriers. It offers an exemplar to build the communication skills of clinician and non-clinician researchers so that they can go beyond dissemination toward translation of evidence into policy and practice.

Biliary injury secondary to trauma is frequently associated with long-term complications. Liver transplantation is rarely indicated but might be the best therapeutic option in severe or intractable cases. We report the case of a 19-year-old male referred for liver transplantation due to biliary injury after abdominal trauma. A Roux-en-Y hepaticojejunostomy was initially performed without immediate complications. Anastomotic stricture developed requiring several trials of biliary dilatation and stenting through a percutaneous approach. The presence of liver cirrhosis and the intractability of this complication culminated in the decision of liver transplantation. The authors present clinical course, complications and interventional procedures that were used in a judicious step-up approach.

OBJECTIVES:. Delayed cerebral ischemia (DCI) is a major driver of morbidity after aneurysmal subarachnoid hemorrhage (aSAH). Quantitative pupillometry has been shown to be of prognostic value after acute neurological injury. However, the evidence for the use of pupillometric features for the detection of DCI has been conflicting. The aim of this study was to investigate the prognostic value of frequent pupillometric monitoring for DCI detection. DESIGN:. Observational cohort study from a prospective aSAH registry. SETTING:. Tertiary referral center. PATIENTS:. Adult patients with confirmed aSAH admitted to the ICU between March 2019 and December 2023. INTERVENTIONS:. None. MEASUREMENTS AND MAIN RESULTS:. One hundred fourteen patients were included, of which 31 (27.2%) suffered from DCI. All patients underwent frequent pupillometry (every 3 hr). We determined the absolute value of the neurological pupil index (NPi) and constriction velocity (CV), and their value normalized to the maximal recorded value between the admission and the pupillometry measure to account for personalized baselines. The association between pupillometry values and the occurrence of DCI within 6–24 hours was investigated. Normalized CV had the best discriminative performance to identify DCI within 8 hours, with an area under the receiver operating characteristic curve of 0.82 (95% CI, 0.69–0.91). NPi, as well as non-normalized metrics, were not significantly associated with DCI. CONCLUSIONS:. Normalized CV has a clinically and statistically significant association with the occurrence of DCI after aSAH. Frequent quantitative pupillometry could improve the multimodal monitoring of patients after aSAH with the goal of improving the identification of patients likely to benefit from therapeutic interventions.

Background. There is no consensus on the ideal strategy to treat posttransplant focal segmental glomerulosclerosis. The multiple-target therapy, which consisted of high-dose intravenous cyclosporine, prednisone, and plasmapheresis, showed favorable results. Methods. This single-center, prospective study sought to evaluate the multiple-target therapy in an independent cohort of patients. Results. Thirteen patients with posttransplant focal segmental glomerulosclerosis received multiple-target therapy. Complete remission was achieved in 2 patients (15.4%), and partial remission in another 2 patients (15.4%). Four patients (30.7%) did not show remission, and 5 patients (38%) lost the graft because of posttransplant focal segmental glomerulosclerosis during the 12-mo follow-up. Premature discontinuation of treatment occurred in 10 patients (77%), all associated with infectious adverse events. Cytomegalovirus was the most common complication, and preemptive therapy was used instead of prophylaxis. Conclusions. In this cohort of patients, the efficacy of the multiple-target therapy was poor and limited by the high incidence of infectious adverse events.

Objective: Anastomotic stenosis caused by hypertrophic granulation tissue often develops in response to orthotopically implanted bioengineered tracheal grafts. To determine mechanisms responsible for the development and persistence of this granulation tissue, we looked for changes in gene expression from tissue specimens from the graft-native interface. Methods: RNA was isolated from paraffin-embedded tissue samples of the anastomotic sites of orthotopically implanted bioengineered tracheal grafts of 9 animals. Tissue samples were binned into 3 groups based on degree of stenosis: no stenosis (

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.

Background:. Augmentation mastopexy remains a challenging surgery and has been frequently associated with suboptimal outcomes and remarkable reoperation rates, and one of the greatest challenges in mastopexy surgery is areolar lift, especially when implants are simultaneously used. Through the authors’ experience, this study is aimed to show a modification of the vertical approach with greater safety of the areolar pedicle. Methods:. The study included all patients who underwent augmentation mastopexy surgery performed by the authors between 2019 and 2022, whether primary or nonprimary, and performed a retrospective chart review of all patients who underwent this procedure. Results:. The length of the areolar lift ranged from 0 cm to 14 cm. Among the 17.4% of nonprimary mastopexies, the longest areolar lift was 11 cm. No cases of nipple–areola complex ischemia/necrosis were observed. With this technique, there were 6.2% complications (n = 31), none of which were considered serious. Conclusions:. This surgical sequence is a safe option for areolar lift in augmentation mastopexy. The vertical approach also has the advantage of producing considerably shorter horizontal scars. It is also reproducible, keeping the implant stable, which results in consistent long-term results.

Erdheim-Chester disease is a rare and multisystemic entity. It results from the infiltration of tissues by foamy histiocytes. The etiology is unknown, but there are mutations in the MAPK pathway in over 80% of patients, more frequently BRAF mutation. The most commonly affected organs and systems are the skeleton, central nervous system, cardiovascular system, kidney, lungs, and skin. The most common clinical manifestations are bone pain, usually in the lower limbs, and diabetes insipidus. The diagnosis is challenging. It requires a combination of clinical, radiological, histopathological, and molecular findings. We present the case of a patient with typical clinical and radiological manifestations: bone pain and diabetes insipidus at presentation, bilateral long bone cortical sclerosis, hairy kidney appearance, coated aorta, right atrium pseudotumor, and periorbital masses.

Background: Oral food challenge (OFC) is useful for diagnosing food allergies and assessing tolerance, but severe reactions may occur during the procedure. Objective: To characterize the frequency and severity of reactions during cow's milk (CM) OFCs. Methods: A cross-sectional study was conducted to analyze the outcome of cow's milk oral food challenges (CMOFCs) performed to confirm IgE-mediated CM allergy or to assess food tolerance. CM was given first as baked milk (BM), followed by whole CM if there was no prior reaction to BM. An OFC was considered positive if IgE-mediated symptoms developed up to 2 h after ingestion. Symptoms were described and variables including age at OFC, prior anaphylaxis, other atopic diseases, and skin test results were compared according to the OFC outcomes. Results: A total of 266 CMOFCs were performed, including 159 patients with a median age of 6.3 years old. One hundred thirty-six tests were positive and 62 resulted in anaphylaxis. Thirty-nine anaphylactic reactions were observed up to 30 min after the first dose. Severe anaphylaxis (cardiovascular and/or neurological involvement) was reported in 5 tests. A second dose of epinephrine was required in 3 tests, and 1 presented a biphasic response. Younger patients had a higher risk of anaphylaxis during baked milk oral food challenge (BMOFC) (p = 0.009). The frequency of anaphylaxis was higher in patients submitted to BM (p = 0.009). Conclusions: Anaphylaxis is a known complication of CMOFCs even when there is no prior anaphylaxis or when conducted with baked products. This study reinforces the importance of conducting OFC in appropriate settings with a well-trained team.

Background: A radial-to-femoral pressure gradient (RFPG) can occur in roughly one-third of cardiac surgical patients. Such a gradient has been associated with smaller stature and potentially smaller radial artery diameter. We hypothesized that preoperative radial artery diameter could be a predictor of RFPG. We also investigated the clinical impact of using a femoral versus a radial arterial catheter in terms of vasoactive support. Methods: Using ultrasound, we measured the bilateral radial artery diameters of 160 cardiac surgical patients. All arterial pressure values were continuously recorded. Significant RFPG was defined as ≥25 mm Hg in systolic and/or ≥10 mm Hg in mean arterial pressure. One hundred and forty-nine additional patients were used to validate the impact of our observations. Results: Using 78,013 pressure datapoints in 129 patients, 34.8% of patients had an RFPG with a mean duration of 54 ± 48 minutes. Patients with a radial artery diameter

Objectives: The majority of available scores to assess mortality risk of coronavirus disease 2019 (COVID-19) patients in the emergency department have high risk of bias. Therefore, this cohort aimed to develop and validate a score at hospital admission for predicting in-hospital mortality in COVID-19 patients and to compare this score with other existing ones. Methods: Consecutive patients (≥ 18 years) with confirmed COVID-19 admitted to the participating hospitals were included. Logistic regression analysis was performed to develop a prediction model for in-hospital mortality, based on the 3978 patients admitted between March–July, 2020. The model was validated in the 1054 patients admitted during August–September, as well as in an external cohort of 474 Spanish patients. Results: Median (25–75th percentile) age of the model-derivation cohort was 60 (48–72) years, and in-hospital mortality was 20.3%. The validation cohorts had similar age distribution and in-hospital mortality. Seven significant variables were included in the risk score: age, blood urea nitrogen, number of comorbidities, C-reactive protein, SpO2/FiO2 ratio, platelet count, and heart rate. The model had high discriminatory value (AUROC 0.844, 95% CI 0.829–0.859), which was confirmed in the Brazilian (0.859 [95% CI 0.833–0.885]) and Spanish (0.894 [95% CI 0.870–0.919]) validation cohorts, and displayed better discrimination ability than other existing scores. It is implemented in a freely available online risk calculator (https://abc2sph.com/). Conclusions: An easy-to-use rapid scoring system based on characteristics of COVID-19 patients commonly available at hospital presentation was designed and validated for early stratification of in-hospital mortality risk of patients with COVID-19.

Purpose: We sought to examine the prognostic value of fluorodeoxyglucose-positron emission tomography (PET) imaging during chemoradiation for unresectable non-small cell lung cancer for survival and hypothesized that tumor PET response is correlated with peripheral T-cell function. Methods and Materials: Forty-five patients with American Joint Committee on Cancer version 7 stage IIB-IIIB non-small cell lung cancer enrolled in a phase II trial and received platinum-doublet chemotherapy concurrent with 6 weeks of radiation (NCT02773238). Fluorodeoxyglucose-PET was performed before treatment start and after 24 Gy of radiation (week 3). PET response status was prospectively defined by multifactorial radiologic interpretation. PET responders received 60 Gy in 30 fractions, while nonresponders received concomitant boosts to 74 Gy in 30 fractions. Peripheral blood was drawn synchronously with PET imaging, from which germline DNA sequencing, T-cell receptor sequencing, and plasma cytokine analysis were performed. Results: Median follow-up was 18.8 months, 1-year overall survival (OS) 82%, 1-year progression-free survival 53%, and 1-year locoregional control 88%. Higher midtreatment PET total lesion glycolysis was detrimental to OS (1 year 87% vs 63%, P < .001), progression-free survival (1 year 60% vs 26%, P = .044), and locoregional control (1 year 94% vs 65%, P = .012), even after adjustment for clinical/treatment factors. Twenty-nine of 45 patients (64%) were classified as PET responders based on a priori definition. Higher tumor programmed death-ligand 1 expression was correlated with response on PET (P = .017). Higher T-cell receptor richness and clone distribution slope were associated with improved OS (P = .018-0.035); clone distribution slope was correlated with PET response (P = .031). Conclusions: Midchemoradiation PET imaging is prognostic for survival; PET response may be linked to tumor and peripheral T-cell biomarkers.

Postpartum papillary muscle rupture (PMR) is extremely uncommon and tolerated poorly with limited management options other than emergency surgical intervention. This case demonstrates the challenges of postpartum PMR in a young woman with unrecognized vascular Ehlers-Danlos syndrome and highlights the importance of preconception screening of cardiovascular disease. (Level of Difficulty: Beginner.)

Transient cortical blindness (TCB) is an uncommon complication following coronary angiography. This report describes a case of TCB after coronary angiography in a patient admitted for acute myocardial infarction. The patient’s vision recovered completely within 16 h, and cardiac magnetic resonance imaging performed 2 months later revealed no significant abnormalities. (Level of Difficulty: Beginner.)

Introduction: Human T-lymphotropic virus (HTLV)-1–associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a neurological disorder that mostly affects women. This disease is characterized by a progressive loss of motor function and disruptions in sensory function in the lower limbs. HTLV-1 is also associated with isolated neurologic dysfunctions, overactive bladder, and erectile dysfunction. The occurrence of sexual dysfunction in HTLV-1–infected women remain unclear. Aim: To investigate associations between HTLV-1 infection and sexual dysfunction in both asymptomatic infected women and those diagnosed with HAM/TSP compared with uninfected women. Methods: HTLV-1–infected and uninfected women were assessed for sexual dysfunction using the Female Sexual Function Index instrument. Sexual dysfunction was considered if global Female Sexual Function Index scores were

Category: Ankle Arthritis Introduction/Purpose: Brazil experiences a late participation in total ankle arthroplasty, which could have positive and negative aspects. The positive view argues about the modern implants that Brazil has received in the past years, skipping the early TAR generation who present more complications and low survival rate in the literature. The negative aspects are related to gap of experience, Brazilian surgeon could not participate in the development of the technique and implants designs during all these years. This paper present the aspects of the Brazilian experience with total ankle replacement since the earliest procedures performed. Methods: Data since the first series of TARs in Brazil were colect from university institution, personal data base from surgeons and previous publication, survivalship, complications, number of each implant and implant availability were recorded. Results: In Brazil, indications for TAR are not so different from around the world, the data we had access demonstrate 65% of post-traumatic arthritis, 26 % of inflammatory arthritis, 2% post-infectious arthritis and 7 % of primary arthritis. In fortheen years 263 surgeries were performed, in different parts of the country, but only one surgeon performed 43 cases (27,3% of the total). Table 1 demonstrate the number of procedures performed per year. Survivor rate of each implant available in the country in the first year were 94%, 86,19% in the second year, 82,84% in the third year, 81,62% in the fourth year and 71,47% in the fifth year. Conclusion: In Brazil there are limited and different ankle arthroplasty systems available for use. The procedure itself continues to be technically demanding and require surgical sophistication and expertise. A national registry to justify the procedure indication; report the outcomes and survivorship has not been developed. This procedure is growing around the county, as well as the surgeon experience, but we should remember that TAR is not for every patient and that the appropriate indication, based on the evidence available, is fundamental to obtaining durable and predictable outcomes.

Background:. Appropriate management of soft tissue injury associated with orthopedic trauma is challenging in low- and middle-income countries (LMICs) due to the lack of available reconstructive surgeons. The Surgical Management and Reconstructive Training (SMART) course teaches orthopedic surgeons reconstructive techniques aimed at improving soft tissue management. This study aims to identify additional barriers to implementing these techniques for surgeons in LMICs who have attended SMART courses. Methods:. This is a mixed-methods study including a Likert-scale-based survey administered to 150 surgeons from LMICs attending the 2018 SMART courses in Tanzania and San Francisco and key informant interviews with 20 surgeons who perform soft tissue coverage procedures. Results:. In surveys, respondents reported inadequate local plastic surgeon availability for lower extremity fracture requiring muscle flaps (88%). Surgeons agreed that flap surgeries are important for patients with significant soft tissue injury following open fractures (97%). They reported inadequate access to instruments, such as dermatomes (59%) and Humby knives (32%), and senior-level support (31%). Fewer than half of surgeons with flap experience (n = 85) felt confident in training peers (45%). In interviews, delays in returning patients to operating rooms were frequently cited as a barrier (90%). Conclusions:. Our study demonstrates that soft tissue procedures are perceived as a high priority among orthopedic surgeons, but there are multiple barriers, including a lack of plastic surgeons, and many modifiable barriers including a lack of surgical equipment, peer training, and senior colleague support.

Atrial fibrillation (AF) usually starts as paroxysmal but can evolve relentlessly to the persistent and permanent forms. However, the mechanisms governing such a transition are unknown. The authors show that intracardiac serum levels of galectin (Gal)-3 are greater in patients with persistent than paroxysmal AF and that Gal-3 independently predicts atrial tachyarrhythmia recurrences after a single ablation procedure. Using a sheep model of persistent AF the authors further demonstrate that upstream therapy targeting Gal-3 diminishes both electrical remodeling and fibrosis by impairing transforming growth factor beta–mediated signaling and reducing myofibroblast activation. Accordingly, Gal-3 inhibition therapy increases the probability of AF termination and reduces the overall burden of AF. Therefore the authors postulate that Gal-3 inhibition is a potential new upstream therapy to prevent AF progression.

Background. It remains controversial if arterial perfusion in addition to portal vein perfusion during machine preservation improves liver graft quality. Comparative studies using both techniques are lacking. We studied the impact of using single or dual machine perfusion of donation after circulatory death rat livers. In addition, we analyzed the effect of pulsatile versus continuous arterial flow. Methods. Donation after circulatory death rat livers (n = 18) were preserved by 6 hours cold storage, followed by 1 hour subnormothermic machine perfusion (20°C, pressure of 40/5 mm Hg) and 2 hours ex vivo warm reperfusion (37°C, pressure of 80/11 mm Hg, 9% whole blood). Machine preservation was either through single portal vein perfusion (SP), dual pulsatile (DPP), or dual continuous perfusion (DCP) of the portal vein and hepatic artery. Hydrodynamics, liver function tests, histopathology, and expression of endothelial specific genes were assessed during 2 hours warm reperfusion. Results. At the end of reperfusion, arterial flow in DPP livers tended to be higher compared to DCP and SP grafts. However, this difference was not significant nor was better flow associated with better outcome. No differences in bile production or alanine aminotransferase levels were observed. SP livers had significantly lower lactate compared to DCP, but not DPP livers. Levels of Caspase-3 and tumor necrosis factor-α were similar between the groups. Expression of endothelial genes Krüppel-like-factor 2 and endothelial nitric oxide synthase tended to be higher in dual perfused livers, but no histological evidence of better preservation of the biliary endothelium or vasculature of the hepatic artery was observed. Conclusions. This study shows comparable outcomes after using a dual or single perfusion approach during end-ischemic subnormothermic liver machine preservation.

In Portugal, tetrahydrobiopterin (BH4)-responsive patients with phenylketonuria (PKU) are identified using a loading test (LT). Phenylalanine/natural protein (Phe/NP) intake is increased to elevate blood Phe prior to the LT. In a longitudinal retrospective study, the impact of Phe/NP titration post-LT in 58 patients (19.6 ± 8.2 years) with PKU during 4 study periods (SPs) was examined. In SP1 (2010-2013), patients were diet treated only; in SP2 (2014), the Phe/NP titration was followed by the LT in SP3 (2015). In SP4 (2016), patients received diet treatment only (n = 49) or BH4 + diet (n = 9). The median percentage blood Phe within the target range was higher in SP1 versus SP4 (64 [28-85] vs 45 [0-66]; P < .001). Our results suggest that transient Phe/NP titration, associated with a deliberate increase in NP, may adversely affect metabolic control. Controlled studies are necessary to examine the longer term impact of temporary increased NP with BH4 LT in non-BH4-responsive patients.

Background:. Limited data exist on plastic surgery practices in Sub-Saharan Africa. The aim of this study was to characterize the spectrum of disease and operative procedures at a teaching hospital in Maputo, Mozambique to help understand the challenges of providing care for the local providers and to provide contextual relevance for training through partnerships. Methods:. A mixed-methods approach was utilized to perform an ongoing needs assessment. A retrospective review was performed of plastic surgery operative records, ward admissions records, and death records in a tertiary-care hospital in Maputo, Mozambique for the period January 2015 to December 2015. Results:. Limited resources (equipment, block-time, personnel, and perioperative services) were observed. The most common diagnoses for the 455 patients evaluated were burns (44%) and neoplasms (17%). Congenital abnormalities accounted for only 1% of the patient diagnoses. Of the 408 procedures performed, the majority were skin grafts (43%) and skin excisions (31%). Sepsis from burns accounted for 70% of documented deaths (14/20). The mean number of days to skin grafting for inpatients was 53 days. Conclusion:. We observed a large burden of burns and skin graft procedures at a public referral teaching hospital in Mozambique. Our findings provide contextual relevance to help focus public health efforts and improve plastic surgery training and practices.

Luciano Henrique de Jesus, Laura de Campos Hildebrand, Manoela Domingues Martins, Francinne Miranda da Rosa, Chris Krebs Danilevicz, Manoel Sant'Ana Filho Department of Oral Pathology, School of Dentistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil Abstract: Polymethylmethacrylate (PMMA) has been widely used in the correction of wrinkles because of its long-lasting cosmetic improvements. However, side effects and complications may occur, and its clinical appearance on the oral mucosa can be similar to that of inflammatory or neoplastic disease. The aim of this research was to compare the clinical and histopathologic responses to PMMA injected by two different methods. Twenty-two rats received an injection of PMMA using the tunneling technique (gold standard), with subcutaneous deposition of the filler in the face, or a variation of the technique with transcutaneous submucosal deposition of the filler in the cheek. The tissue reaction was analyzed clinically every 24 hours during the first week, then once a week for the following 3 months. Histologic evaluation was based on the local inflammatory response to the filler. No clinical changes were observed during the initial evaluation period (0–14 days). After 14 days, only the submucosal group showed extra-oral enlargement (n=4, 18.2%). Histopathologic analysis revealed nodule formation in four animals (18.2%) in the submucosal group, with no nodules observed in the subcutaneous group. The data obtained in this study demonstrate that the technique used to deliver the filler may influence the risk of adverse reactions. Keywords: dermal filler, polymethylmethacrylate, adverse reactions

Background:. In craniofacial microsomia, microtia and canal atresia pose formidable reconstructive challenges. We review our institutional experience in treating microtia and atresia to identify variables associated with 4 outcomes measures: complications, surgical revisions, aesthetic outcomes, and psychosocial function. Methods:. Craniofacial microsomia patients treated at the University of California Los Angeles Craniofacial Clinic between 2008 and 2014 greater than 13 years of age (n = 68) were reviewed for microtia and atresia treatment and outcomes. Results:. In total, 91.2% of patients diagnosed with craniofacial microsomia presented with microtia, affecting 75 ears. Both a male and right-sided predominance were observed. Fifty-six patients (90.3%) underwent autologous external ear reconstruction at an average age of 8.5 years. Age, type of incision, and size of cartilage framework did not predict total number of surgeries or complications. Severity of ear anomalies correlated with increased number of surgeries (P < 0.001) and decreased aesthetic outcomes (P < 0.001) but not complications. In total, 87.1% of patients with microtia had documented hearing loss, of which the majority were conductive and 18.5% were mixed sensorineural and conductive. Hearing deficits were addressed in 70.4% of patients with external hearing aids, bone anchored hearing aids, or canaloplasty. Of all variables, improvement of psychosocial function was correlated only to hearing loss treatment of any type (P = 0.01). Conclusions:. On evaluation of surgical and patient characteristics, severity of microtia predicted the total number of surgical revisions performed and aesthetic ratings. In addition, we found that the only factor that correlated with improved patient and parent-reported psychosocial outcomes was treatment of hearing loss.

Background. Both prolonged cold ischemia time (CIT) and donor history of diabetes mellitus (DM) are associated with reduced graft survival after liver transplantation. However, it is unknown whether the adverse effect of prolonged CIT on posttransplant graft survival is more pronounced after transplant with DM versus non-DM donor grafts. Methods. The study sample included 58 226 liver transplant recipients (2002-2015) from the Scientific Registry of Transplant Recipients. Multivariable Cox survival regression with interaction analysis was used to quantify the extent to which history of donor DM (n = 6478) potentiates the adverse effect of prolonged (≥8 hours) CIT (n = 18 287) on graft survival. Results. Donor DM and CIT 8 hours or longer were each associated with increased risk of graft failure (GF) (adjusted hazard ratio [aHR], 1.19; 95% confidence interval [CI], 1.06-1.35 and aHR, 1.42; 95% CI, 1.32-1.53, respectively) compared with transplanted grafts without either risk factor. However, the combination of DM and CIT 8 hours or longer was associated with a higher risk of GF than either factor alone (aHR, 1.79; 95% CI, 1.55-2.06) and had a synergy index of 1.30. The interaction was significant on a multiplicative scale in the later postoperative period, days 31 to 365 (P = 0.047). Conclusions. These results suggest that liver grafts from DM donors are more susceptible to the adverse effects of prolonged CIT than livers from non-DM donors. We need to be cognizant that they are more susceptible to ischemic injury, and this may be considered during the allocation process.

Fabry disease, caused by deficient alpha-galactosidase A lysosomal enzyme activity, remains challenging to health-care professionals. Laboratory diagnosis in males is carried out by determination of alpha-galactosidase A activity; for females, enzymatic activity determination fails to detect the disease in about two-thirds of the patients, and only the identification of a pathogenic mutation in the GLA gene allows for a definite diagnosis. The hurdle to be overcome in this field is to determine whether a mutation that has never been described determines a “classic” or “nonclassic” phenotype, because this will have an impact on the decision-making for treatment initiation. Besides the enzymatic determination and GLA gene mutation determination, researchers are still searching for a good biomarker, and it seems that plasma lyso-Gb3 is a useful tool that correlates to the degree of substrate storage in organs. The ideal time for treatment initiation for children and nonclassic phenotype remains unclear.

Subdural hematoma (SH) is a neurosurgical emergency, usually caused by head trauma. Non-traumatic causes include aneurysm or arterial–venous malformation rupture, coagulopathy and others. We report the case of a 66 year-old man who developed apparently unprovoked signs of increased intracranial pressure. Brain computed tomography scan showed an acute spontaneous SH, surgically treated. Throughout surgery, a ruptured cortical artery with intensive bleeding appeared and was cauterized. After surgery, patient remained comatose and a new CT demonstrated Duret hemorrhage at the brainstem. Acute spontaneous SH of arterial origin is rare and highly lethal, in which a good prognosis relies on early diagnosis and treatment.

Background: The purpose of this study was to evaluate the effect of the dissection technique on outcomes and complications after a full abdominoplasty, comparing 2 different techniques used to raise the abdominal flap: the steel scalpel and the diathermocoagulation device on coagulation mode. Methods: A prospective study was performed at a single center from January 2009 to December 2011 of patients submitted to abdominoplasty with umbilical transposition. Two groups were identified: group A, abdominoplasty performed with steel scalpel/knife; and group B, abdominoplasty performed with diathermocoagulation on coagulation mode. Several variables were determined: general characteristics, time until drain removal, daily and total volume of drain output, length of hospital stay, operative time, readmission, reoperation, emergency department visits, and local and systemic complications. Results: A total of 119 full abdominoplasties were performed in women (group A, 39 patients; group B, 80 patients). There were no statistically significant differences between groups with respect to general characteristics, except for body mass index, comorbidities, and weight of the surgical specimen; there were no differences for operative time, systemic complications, hematoma, and necrosis incidence. The scalpel group had a highly significant reduction of 54.56% on total drain output, and a 2.65 day reduction on time to drain removal and no reported cases of seroma or healing problems (difference of 81.25% and 90.00%, respectively, between the 2 groups). Conclusions: Performing abdominal dissection with scalpel had a beneficial effect on patient recovery, as it reduced time requested for drain removal, total drain output, and incidence of seroma and wound healing problems.

The aim of this study was to compare retrospectively the effect of three different treatments on the healing outcome of bisphosphonate-related osteonecrosis of the jaws (BRONJ) in cancer patients. Twenty-two cancer patients were treated for BRONJ with one of the following protocols: clinical (pharmacological therapy), surgical (pharmacological plus surgical therapy), or PRP plus LPT (pharmacological plus surgical plus platelet rich plasma (PRP) plus laser phototherapy (LPT). The laser treatment was applied with a continuous diode laser (InGaAlP, 660nm) using punctual and contact mode, 40mW, spot size 0.042 cm(2), 6J/cm(2) (6s) and total energy of 0.24J per point. The irradiations were performed on the exposed bone and surrounding soft tissue. The analysis of demographic data and risk factors was performed by gathering the following information: age, gender, primary tumor, bisphosphonate (BP) used, duration of BP intake, history of chemotherapy, use of steroids, and medical history of diabetes. The association between the current state of BRONJ (with or without bone exposure) and other qualitative variables was determined using the chi-square or Fisher's exact test. In all tests, the significance level adopted was 5%. Most BRONJ lesions occurred in the mandible (77%) after tooth extraction (55%) and in women (72%). A significantly higher percentage of patients reached the current state of BRONJ without bone exposure (86%) in the PPR plus LPT group than in the pharmacological (0%) and surgical (40%) groups after 1-month follow-up assessment. These results suggest that the association of pharmacological therapy and surgical therapy with PRP plus LPT significantly improves BRONJ healing in oncologic patients. Although prospective studies with larger sample sizes are still needed, this preliminary study may be used to inform a better-designed future study. [ABSTRACT FROM AUTHOR]

Background: Oral mucositis is a common complication in the treatment of cancer. Its management and prevention are seen as high priority in cancer patient care. The aim of the present study was to investigate the effect of topical chamomile in the treatment of oral mucositis induced by 5-fluoracil (5-FU) in hamsters.Materials and Methods: One hundred five hamsters were randomly separated into three groups (35 animals each): group I--without treatment (control); group II--treatment with chamomile (Ad-Muc®); and group III--treatment with corticoid (betamethasone elixir--Celestone®). The animals received an intraperitoneal injection of 5-FU on days 0 and 2. On days 3 and 4, the buccal mucosa was scratched and therapy was initiated on day 5. Three animals were sacrificed on days 0, 2, 5, 8, 10, 12, 14, and 16, weighed, and the buccal mucosa removed for clinical and histopathological analysis.Results: The animals that developed mucositis and were treated with chamomile or the corticoid agent weighed significantly less than those in the control group. The group treated with the corticoid agent exhibited a more severe clinical condition, whereas the group treated with chamomile exhibited mild mucositis throughout the experiment. The group treated with chamomile had a 12-fold greater chance of scoring zero (absence of mucositis) than the control group. Analysis of the histopathological results demonstrated that the group treated with chamomile exhibited a lesser degree of mucositis throughout the evaluation period in comparison to the control and corticoid groups.Conclusion: Chamomile proved effective in the treatment of oral mucositis in a hamster model. However, well-designed clinical studies are needed to confirm the clinical efficacy of this medicine in humans. [ABSTRACT FROM AUTHOR]