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Guido Ciprandi,1,2 Serena Crucianelli,2 Francesca Grussu,2 Giorgio Spuntarelli,2 Simone Faustino Maria Marino,2 Urbano Urbani,2 Paola Bernaschi,3 Annamaria Sisto,3 Maria Ida Rizzo,2 Mario Zama4 1Chief Pediatric Wound Care High Specialization, Plastic and Maxillofacial Surgery Unit, Bambino Gesu’ Children’s Hospital, Research Institute, Rome, Italy; 2Consultant, Plastic and Maxillofacial Surgery Unit, Bambino Gesù Children’s Hospital, Research Institute, Rome, Italy; 3Microbiology Unit, Bambino Gesu’ Children’s Hospital, Research Institute, Rome, Italy; 4Head, Plastic and Maxillofacial Surgery Unit, Bambino Gesù Children’s Hospital, Research Institute, Rome, ItalyCorrespondence: Guido Ciprandi, via Arturo Colautti, 5, Rome, 00152, Italy, Tel +39.3389400595, Fax +39.06.69592115, Email guido.ciprandi@opbg.net; guidociprandi@gmail.comAbstract: Although similar in structure, pediatric skin is more delicate and vulnerable compared with adult skin and, as a result, is more prone to wounding. The immune response in pediatric skin is underdeveloped because of immature inflammatory cells and lower number of bone marrow progenitor cells. Therefore, pediatric patients, particularly newborns, have weak responses to microorganisms. The use of antimicrobial agents (eg, antibiotics, antimicrobial wound dressings, etc.) to aid in the prevention and/or treatment of wounds prone to or which are infected is one treatment option. Antimicrobial wound dressings using DACC technology physically bind bacteria and reduce the need for chemically active antimicrobial agents. This perspective is intended to highlight the benefit of DACC technology wound dressings for the prevention and treatment of pediatric wounds related to wound infection. We have found that DACC technology dressings are of benefit in the treatment of pediatric wounds and offer a significant resource for the treatment of pediatric wounds.Keywords: pediatric wounds, infection, DACC technology

Abstract Background Vascular Smooth Muscle Cells (VSMCs), due to their plasticity and ability to shift from a physiological contractile-quiescent phenotype to a pathological proliferating-activated status, play a central role in the onset and progression of atherosclerosis and cardiovascular diseases. PDGF-BB, among a series of cytokines and growth factors, has been identified as the critical factor in this phenotypic switch. In order to obtain new insights on the molecular effects triggered by PDGF-BB, a hammerhead ribozyme targeting the membrane receptor PDGFR-β was applied to inhibit PDGF pathway in porcine VSMCs. Findings Ribozymes, loaded on a cationic polymer-based vehicle, were delivered into cultured VSMCs. A significant impairment of the activation mechanisms triggered by PDGF-BB was demonstrated since cell migration decreased after treatments. In order to functionally validate the effects of PDGFR-β partial knock down we focused on the phosphorylation status of two proteins, protein disulfide isomerase-A3 (PDI-A3) and heat shock protein-60 (HSP-60), previously identified as indicative of VSMC phenotypic switch after PDGF-BB stimulation. Interestingly, while PDI-A3 phosphorylation was counteracted by the ribozyme administration indicating that PDI-A3 is a factor downstream the receptor signalling cascade, the HSP-60 phosphorylation status was greatly increased by the ribozyme administration. Conclusion These contradictory observations suggested that PDGF-BB might trigger different parallel pathways that could be modulated by alternative isoforms of the receptors for the growth factor. In conclusion the knock down strategy here described enables to discriminate between two tightly intermingled pathways. Moreover it opens new attractive perspectives in functional investigations where combined gene knock down and proteomic technologies would allow the identification of key factors and pathways involved in VSMC-linked pathological disorders.

Abstract Background Defining the optimal treatment for patients with inoperable non small cell lung cancer (NSCLC), presenting with metastatic mediastinal lymph nodes, is challenging. Nevertheless, preoperative chemotherapy or radiotherapy might offer a chance for these patients for radical surgical resection and, possibly, complete recovery. Case Presentation A 62-year old man with IIIA-N2 inoperable NSCLC was treated with first-line single agent docetaxel. A platinum-based treatment, though considered more active, was ruled out because of renal impairment. The patient tolerated the treatment very well and, although his initial response was not impressive, after 14 cycles he obtained a complete clinical response, which was confirmed pathologically after he underwent surgical lobectomy. Conclusion In non-operable NSCLC patients not eligible for a platinum-based treatment, single-agent docetaxel can provide complete pathologic responses. Failure to obtain a response after the first few cycles should not automatically discourage to continue treatment.

Objective: This study evaluates long-term outcomes in adults with Unilateral and Bilateral Cleft Lip and Palate (UCLP/BCLP) treated during the period 1992 to 1995 with tibial periosteal graft in primary repair., Design: Retrospective study., Setting: Department of Plastic and Maxillofacial Surgery, Children's Hospital Bambino Gesù (Italy)., Patients: The study included 52 patients with non-syndromic BCLP/UCLP who met the inclusion criteria., Interventions: All patients underwent a standardized surgical protocol using a tibial periosteal graft as primary repair of the hard palate., Main Outcome Measure(s): Long-term outcomes on maxillary growth, residual oronasal fistula, and leg length discrepancy., Results: About <2% of patients showed oral-nasal communication. Mean value of maxillary depth was 86° ± 4.5°. The lower value for maxillary retrusion was 76.8° in relation to the Frankfurt plane. At the x-ray control, 12.2% of patients showed leg discrepancy with a difference of always <2 cm., Conclusions: The rate of maxillary retrusion obtained was the same if compared to other techniques. Tibial periosteal graft reduces the risk of fistula and the need for reintervention after secondary bone graft. The study did not observe negative impacts on leg growth after 25 years., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The muscular sculpture (MS) is a new technique of male body contouring to achieve: tight skin that very well reveals muscular bulk, a male muscular and athletic body, minimal observable scars and even patients who are not, nor have been in. With this study, we describe the new technique for sculpting of lower extremities. In literature are not described techniques to accomplish a sound aesthetic result in sculpting surgery about lower extremities., Methods: For this surgical procedure, we illustrate the technique that was obtained from 5 male patients (principles outlined in the Declaration of Helsinki have been followed). We describe this surgery with six steps: defatting step; MS; internal scar removal; irregularity removal; dermal grasping; last residues of fat removing., Results: We obtain a high satisfaction rate as high as 100% for short and long term. Compared to the complications more described in literature (infection, hematoma, seroma, wound dehiscence, hypertrophic scars, sensory nerve injury and recurrent skin laxity, Monarca and Rizzo in Aesthetic Plast Surg 39(2):199-202, 2015), we have not had complications. We had no major complications. We observed, with our other studies, a direct relationship between complications and smoking habit. Successful body-contouring surgery requires a patient to embrace positive lifestyle habits: exercise, a proper diet and other positive lifestyle changes (Monarca et al. in Plast Reconstr Surg 123(5):1637-1638, 2009), and a high compliance and to avoid all the excesses., Conclusions: We obtain amazing aesthetic results with total patient satisfaction without complications. There are not enough studies in the literature about body sculpting, and is necessary to deepen this technique in order to obtain increasingly encouraging results., Level of Evidence V: Sculpting surgery, Body contouring, 41 leg surgery, adipose tissue. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 ., (© 2022. Springer Science+Business Media, LLC, part of Springer Nature and International Society of Aesthetic Plastic Surgery.)

Velopharyngeal dysfunction (VPD) diagnosis and speech surgery outcomes are currently based solely on subjective evaluation criteria consisting of perceptual speech assessment and functional imaging. This study describes an objective and comparable method in VPD assessment and investigates the concurrence between the objective and subjective evaluations. The present study included 20 paediatric patients presenting with VPD after primary repair (intravelar veloplasty) of cleft palate. Our protocol was based on computerized analysis of voice parameters by means of an objective tool, spectrography integrated with Multi-Dimensional Voice Program (MDVP). The protocol also included perceptual evaluation by speech therapist and phoniatrician (consensus listening), and parents. This is a single surgeon, single centre experience and all patients underwent a secondary Furlow's palatoplasty. Assessments were performed pre- and postoperatively and upon completion of speech therapy. Results were compared using the two-tailed t student test for paired data. Statistical significance was set for p-values <0.05. Data analysis confirmed an improvement in velopharyngeal closure after surgery and speech therapy consistently with the results of perceptual evaluations. The results of the study confirmed the availability and reliability of an objective method for VPD evaluation based on the analysis of voice parameters with investigations that are simple and easily available in a hospital setting., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Mucormycosis is an opportunistic and aggressive fungal infection that mainly affects immunocompromised patients who generally suffer from diabetes mellitus, immune impairment, hematological disease. It is a life-threatening infection and the management is not standardized. The literature proposes aggressive and early surgical approach, even at the expense of mutilation. We report a case of rhino-orbital mucormycosis in a child with myeloblastic leukemia and the successful treatment using the instill negative pressure wound therapy combined with reconstructive surgery in order to reduce mortality and to avoid disfigurement. KEY WORDS: Amphotericin B, Apex syndrome, Forehead flap, Instill NPWT, Myeloaplasia Mucormycosis.

Cleft lip and palate (CL/P) is the most prevalent craniofacial birth defect in humans. None of the surgical procedures currently used for CL/P repair lead to definitive correction of hard palate bone interruption. Advances in tissue engineering and regenerative medicine aim to develop new strategies to restore palatal bone interruption by using tissue or organ-decellularized bioscaffolds seeded with host cells. Aim of this study was to set up a new natural scaffold deriving from a decellularized porcine mucoperiosteum, engineered by an innovative micro-perforation procedure based on Quantum Molecular Resonance (QMR) and then subjected to in vitro recellularization with human bone marrow-derived mesenchymal stem cells (hBM-MSCs). Our results demonstrated the efficiency of decellularization treatment gaining a natural, non-immunogenic scaffold with preserved collagen microenvironment that displays a favorable support to hMSC engraftment, spreading and differentiation. Ultrastructural analysis showed that the micro-perforation procedure preserved the collagen mesh, increasing the osteoinductive potential for mesenchymal precursor cells. In conclusion, we developed a novel tissue engineering protocol to obtain a non-immunogenic mucoperiosteal scaffold suitable for allogenic transplantation and CL/P repair. The innovative micro-perforation procedure improving hMSC osteogenic differentiation potentially impacts for enhanced palatal bone regeneration leading to future clinical applications in humans., (© 2021. The Author(s).)

The presence of circulating tumor cells (CTCs) in the blood of patients with metastatic breast, colorectal and prostate cancer have been widely investigated; however, few studies have examined CTCs in patients with laryngeal cancer. The present pilot study aimed to detect pre- and postoperative CTCs in the blood of patients with laryngeal cancer and evaluate the association with prognosis. Eight patients with laryngeal squamous cell carcinoma (LSCC) at stage III were included in the present study and underwent total or subtotal laryngectomy and radical bilateral neck lymph node dissection. Blood samples were collected from all patients before and after surgery at different time-points. The following processing steps were followed; preoperative blood sampling, surgery, postoperative blood sampling at 3, 6 and 12 month follow-ups, and prognostic association analysis. CTCs were retained on ScreenCell filters for cytological characterization. The presence of CTCs was associated with a less favorable prognosis, whereas a decrease of CTCs in the postoperative sampling was observed in patients who exhibited an improved therapeutic response. The results of the present pilot study revealed a possible association between the presence of CTCs and a less favorable prognosis in patients with LSCC; therefore, these preliminary findings may encourage further research into the incorporation of a liquid biopsy in the management of LSCC, as this may help identify patients with occult metastatic disease earlier and in a non-invasive manner. In addition, this approach may represent novel independent prognostic factor for use in the clinical evaluation of patients with LSCC., (Copyright © 2020, Spandidos Publications.)

The loss or deficiency of a tissue or organ is a serious health problem and severely affects the patients' quality of life. In the near future, an option for solving this problem will be the development of bio-derived extracellular matrices (ECM) from huPlastic Romaman or animal tissues and their usage for in vitro or in vivo cellular reseeding. Many researchers are working on this development. Nowadays, different decellularization protocols have been developed to manufacture ECMs but there is not yet a consensus about the most efficient procedures. This review aims to describe the state of the art in the field of decellularization of complex mucosal tissues, analizing and comparing the most recent and most valiant articles published about this procedure.

Background and Objectives: Previous literature has disclosed that facial attractiveness affects the esthetic evaluation of nose and lip deformity on frontal and lateral photographs. However, it has never been debated if the removal of the external facial features on three-dimensional (3D) models ("cropped assessment bias") could provide a considerable usefulness in the interpretation and comparison of the results. Additionally, it has been assumed on two-dimensional (2D) studies that esthetic assessment biases with respect to observer gender, and it is not acknowledged if and to the extent that "gender assessment bias" may be influenced by a three-dimensional layout. The aim of this study is to investigate if facial traits and observers' gender may affect the esthetic ratings of unilateral cleft lip and palate (UCLP) patients after soft tissue reconstruction. Materials and Methods: Three-dimensional images of ten UCLP patients' images were acquired before the intervention (T0), one-month (T1) and six-months (T2) postoperative. Geomagic ® software (version 2014; 3D Systems, Rock Hill, SC, USA) was used to remove the external facial features of 3D surface models. Five-point scale developed by Asher-McDade et al. was used to rate both nasolabial attractiveness and impairment for full-face (FF) and cropped-face (CF) 3D images. Forty-three judges (21 males, 22 females) were enrolled for the esthetic evaluation. Intraclass correlation coefficient (ICC) was used to test intra- and inter-examiner reliability; a value of 0.7 was set as the minimum acceptable level of reliability. Results: When comparing the 2 sets of observations (FF and CF), the ICC ranged from 0.654 to 0.823. Concerning gender assessment bias, the ICC ranged from 0.438 to 0.686 and from 0.722 to 0.788 for males and females, respectively. Concerning inter-examiner reliability, ICC for questions 2-7 ranged from 0.448 to 0.644 and from 0.659 to 0.817 at T0 and T2, respectively. Conclusions: The removal of external facial features provides subtle differences on the esthetic assessment of UCLP patients. Moreover, based on our data, examiners' gender differences may affect esthetic assessment of UCLP patients. Despite the subjectivity of esthetic judgments, a reliable, validated and reproducible scoring protocol should consider the influence of gender differences on 3D esthetic assessment of UCLP patients.

The processing techniques of the adipose tissue represents one of the most debated topics. There are different processing techniques. As of today, the standard considered procedure is the centri1Romafugaton according to Coleman. In addition, other procedures include decantation/sedimentation, filtration and water jet force, which offer the specialist valid alternatives. In the present review, the clinical evidence of the techniques of centrifugation and decantation will be evaluated by studying histological data; maintenance of tropism and the maintenance of mesenchymal cells. These two aspects are different in the centrifugation and decantation techniques.

Purpose: To quantify and compare pre-operative and post-operative volumetric adjustments of the upper lip tissues in patients with cleft lip., Methods: The authors performed an anthropometric study and a quantitative analysis of the differences based on three-dimensional morphology of the nasolabial area. Twenty facial images using the three-dimensional stereophotogrammetry were taken from ten selected subjects on two separate occasions, sitting in natural head position. Facial landmarks were marked and measurements recorded, in order to calculate the volumetric adjustments in the soft tissues of the upper lip, comparing the preoperative and postoperative results. Student test and p-Value were performed for statistical analysis., Results: The analysis of the 3D images showed variability with the pre- and postoperative volumes of the nasolabial area with: an increase of upper lip volume all patients; a complete view of the severity in the preoperative; and an improvement of the appearance in the postoperative. The amount of increase of the upper lip volume was established about 29,7%. For all measurements, the variability between pre- and post-operative was significant (p < 0.01)., Conclusion: The 3D stereophotogrammetry technique allows a detailed preoperative evaluation and an accurate assessment of the surgical outcomes. The study provides a value of volumetric variation of the upper lip in individuals with cleft lip., Key Words: Cleft lip, Nasolabial area, 3D Morphological Analysis, 3D Stereophotogrammetry, Upper lip volume.

Glaucomatous optic neuropathy is the most commonly acquired optic neuropathy encountered in clinical practice. It is the second leading cause of blindness globally, after cataracts, but it presents a greater public health challenge than cataracts, because the blindness it causes is irreversible. It has pathogenesis still largely unknown and no established cure. Alterations in serum antibody profiles, upregulation, and downregulation have been described, but it still remains elusive if the autoantibodies seen in glaucoma are an epiphenomenon or causative. Hypertension, diabetes, and hearing disorders also are associated. This review is a glaucoma update with focus about the recent advances in the last 15 years.

Hypertrophic pachymeningitis (HP) is a rare disorder that causes thickening of the dura mater. Inflammatory lesions may be located in the cerebral or spinal dura mater or, less frequently, in both locations simultaneously. Numerous clinico-pathological entities cause thickening of the pachymeninges. Indeed, HP is a potential manifestation of many different diseases, but the diagnosis often remains uncertain. Cases in which the pachymeningitis has no known aetiology are termed "idiopathic" HP (IHP). Recently, it has been suggested that IgG4-related disease represents a subset of cases previously diagnosed as idiopathic hypertrophic pachymeningitis. Little is known regarding the pathogenic events of IHP. In a general theory, the inflammatory infiltrate, mainly consisting of B and T lymphocytes, activates fibroblasts and induces collagen deposition, leading to tissue hypertrophy and increased dural thickness. Clinical manifestations of IHP depend upon the location of the inflammatory lesions and compression of the adjacent nervous structures. Three central pathological features are lymphoplasmacytic infiltration, obliterative phlebitis, and storiform fibrosis. MRI is the examination of choice for the preliminary diagnosis of IHP. Histopathological examination of a biopsy specimen of the dura mater would finally confirm the diagnosis. The differential diagnosis for HP is broad and includes infections, autoimmune disorders, and neoplasia. Currently, there is no consensus about treatment for patients with IHP. There is a preference for glucocorticoid treatment on diagnosis followed by the addition of other immunosuppressive agents in the event of a recurrence. Rituximab is used in patients who did not respond to glucocorticoids or to conventional steroid-sparing agents.

Purpose: To report a surgical algorithm for the treatment of bilateral cleft lip stigmata., Methods: The investigators designs a retrospective study composed of patients with bilateral cleft lip stigmata. The surgical approach is on the basis of the severity of the deformity and of course the age of the patient. It consists in Simple scar revision with orbicularis muscle synthesis; Two dermal flaps tunneled in the central vermilion; Abbe flap. The investigators analyzes early and late complications, and performs the evaluation of the cosmetic appearance by a parent or patient himself/herself, a surgeon and a blinded third party observer., Results: The sample was composed of 351 patients. At short-term follow-up, viability of the flaps, function and morphology are good. At long-term follow-up, we observed significant improvement of the characteristics and profile of the patient's face and a normal function of the lips. We recorded a good scarring and a high satisfaction rate by evaluation of patients/ surgeon/ blinded third party observer., Conclusion: The results of this study suggest that a surgical algorithm on the basis of the severity of the deformity and of course the age of the patient can represent an option of choice for most patients with bilateral cleft lip stigmata., Key Words: Abbe flap, Bilateral cleft lip stigmata, Cleft lip, Dermal flaps, Orbicularis muscles.

Cancer stem cells (CSCs) are a group of cells with eternal life or infinite self-renewal ability, which have high migrating, infiltrative, and metastatic abilities. Though CSCs only account for a small proportion in tumors, the high resistance to traditional therapy exempts them from therapy killing and thus they can reconstruct tumors. Previous studies found that heterogeneity of cancer cells extensively exists in head and neck cancers. Our current knowledge, about CSCs in the laryngeal cancer (LC), largely depends on head and neck studies. Given the lack of systematic data about CSCs in LC, we propose a review of the literature, reporting the evidences of CSCs in tumorigenesis of LC, with the purpose to provide new insights into the prevention and/or treatment of laryngeal carcinoma.

Parkinson's disease is a neurodegenerative disease that causes the death of dopaminergic neurons in the substantia nigra. The resulting dopamine deficiency in the basal ganglia leads to a movement disorder that is characterized by classical parkinsonian motor symptoms. Parkinson's disease is recognized as the most common neurodegenerative disorder after Alzheimer's disease. PD ethiopathogenesis remains to be elucidated and has been connected to genetic, environmental and immunologic conditions. The past decade has provided evidence for a significant role of the immune system in PD pathogenesis, either through inflammation or an autoimmune response. Several autoantibodies directed at antigens associated with PD pathogenesis have been identified in PD patients. This immune activation may be the cause of, rather than a response to, the observed neuronal loss. Parkinsonian motor symptoms include bradykinesia, muscular rigidity and resting tremor. The non-motor features include olfactory dysfunction, cognitive impairment, psychiatric symptoms and autonomic dysfunction. Microscopically, the specific degeneration of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies, which are brain deposits containing a substantial amount of α-synuclein, have been recognized. The progression of Parkinson's disease is characterized by a worsening of motor features; however, as the disease progresses, there is an emergence of complications related to long-term symptomatic treatment. The available therapies for Parkinson's disease only treat the symptoms of the disease. A major goal of Parkinson's disease research is the development of disease-modifying drugs that slow or stop the neurodegenerative process. Drugs that enhance the intracerebral dopamine concentrations or stimulate dopamine receptors remain the mainstay treatment for motor symptoms. Immunomodulatory therapeutic strategies aiming to attenuate PD neurodegeneration have become an attractive option and warrant further investigation., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Glaucoma is the most commonly acquired optic neuropathy. It represents a public health challenge because it causes an irreversible blindness. Emerging evidence indicates that the pathogenesis of glaucoma depends on several interacting pathogenetic mechanisms, which include mechanical effects by an increased intraocular pressure, decreased neutrophine-supply, hypoxia, excitotoxicity, oxidative stress, and the involvement of autoimmune processes. In particular, alterations in serum antibody profiles have been described. However, it is still unclear whether the autoantibodies seen in glaucoma are an epiphenomenon or causative. Oxidative stress appears to be a critical factor in the neurodestructive consequences of mitochondrial dysfunction, glial activation response, and uncontrolled activity of the immune system during glaucomatous neurodegeneration. In addition, hearing loss has been identified in association with glaucoma. A higher prevalence of antiphosphatidylserine antibodies of the immunoglobulin G class was seen in normal-tension glaucoma patients with hearing loss in comparison with normal-tension glaucoma patients with normacusis. This finding suggests a similar pathological pathway as a sign for generalized disease., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: Epithelial-to-mesenchymal transition (EMT) consists of a rapid and reversible change in the cellular phenotype toward the mesenchymal cell phenotype that facilitates cell migration and invasion of the tumor into surrounding tissues followed by metastasis. In the present study, we sought to determine the clinical significance of E-cadherin, N-cadherin, β-catenin, α-catenin, γ-catenin, caveolin-1, and vimentin in a cohort of patients with stage I to IVA laryngeal squamous cell carcinoma (LSCC) treated with surgery with or without adjuvant therapy using immunohistochemical analyses., Study Design: Individual retrospective cohort study., Methods: E-cadherin, N-cadherin, β-catenin, α-catenin, γ-catenin, caveolin-1, and vimentin immunohistochemical expression were evaluated in a cohort of 82 patients with stages I to IVA LSCC. The Fisher exact test was used for categorical variables, and the Mann-Whitney test where appropriate for continuous variables. Survival comparisons was performed using the log-rank test. A multivariate analysis using the Cox proportional hazards model was performed and considered all EMT markers., Results: In multivariate analysis, T stage was an independent risk factor for adverse disease-specific survival (DSS) and overall survival (OS) (P = .025 and .019, respectively). Cytoplasmic β-catenin overexpression was independently associated with a longer DSS (P = .0007), and E-cadherin overexpression was found to be an independent risk factor for poor OS (P = .030)., Conclusions: E-cadherin and β-catenin pathways could represent future therapeutic targets in the treatment of LSCC. However, validation of our results in prospective cohorts of patients with LSCCs is required before their clinical implementation., Level of Evidence: NA., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Despite the fact that epilepsy is the third most common chronic brain disorder, relatively little is known about the processes leading to the generation of seizures. Accumulating data support an autoimmune basis in patients with antiepileptic drug-resistant seizures. Besides, recent studies show that epilepsy and autoimmune disease frequently co-occur. Autoimmune epilepsy is increasingly recognized in the spectrum of neurological disorders characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy. An autoimmune cause is suspected based on frequent or medically intractable seizures and the presence of at least one neural antibody, inflammatory changes indicated in serum or spinal fluid or on MRI, or a personal or family history of autoimmunity. It is essential that an autoimmune etiology be considered in the initial differential diagnosis of new onset epilepsy, because early immunotherapy assures an optimal outcome for the patient., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. The disease predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). Microscopic polyangiitis was considered to be a disease entity by Savage et al. in 1985. Microscopic polyangiitis has a reported low incidence and a slight male predominance. The aetiology of MPA remains unknown. There is, however, increased evidence that MPA is an autoimmune disease in which ANCAs, particularly those reacting with MPO, are pathogenic. MPA belongs to the systemic vasculitides, indicating that multiple organs can be affected. The major organs involved in MPA are the kidneys and the lungs. As expected for an illness that affects multiple organ systems, patients with MPA can present with a myriad of different symptoms. Ear, nose and throat (ENT) manifestations are not considered to be clinical symptoms of MPA, but in the majority of populations described, ENT involvement was found in surprisingly high percentages. MPA is part of the ANCA-associated vasculitides, which are characterized by necrotizing vasculitis of small vessels. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Kawasaki disease (KD) is a self-limited childhood systemic vasculitis that exhibits a specific predilection for the coronary arteries. KD predominantly affects young children between the ages of 6months and 4years. Incidence rates in Asians are up to 20 times higher than Caucasians. The aetiology of KD is not known. One reasonable open hypothesis is that KD is caused by an infectious agent that produces an autoimmune disease only in genetically predisposed individuals. The typical presentation of KD is a young child who has exhibited a high swinging fever for five or more days that persists despite antibiotic and/or antipyretic treatment. The lips are dry and cracked. There is a characteristic strawberry tongue, and a diffuse erythema of oropharyngeal mucosal surfaces. Lymphadenopathy is usually unilateral and confined to the anterior cervical triangle. Coronary aneurysms generally appear during the convalescence phase (beginning during the second week). The absence of any laboratory tests for KD means that the diagnosis is made by the presence of a constellation of clinical features. The aim of echocardiography is to assess the presence of coronary artery dilatation or aneurysm formation. Effective therapies exist for most patients with acute KD, but the exact mechanisms of action are not clear. Treatment with aspirin and intravenous immunoglobulins (IVIG) are first-line therapies. However, options are plentiful for the children who fail this treatment, but these treatments are not as beneficial. Some centres attempt to salvage resistant patients using intravenous pulsed doses of methylprednisolone. Other centres use infliximab or combinations of these approaches., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Unlabelled: Optimal male body contouring considers the entire body and drapes tight skin over the muscles. Males have pride in the appearance of their muscular development. There should be a deliberate effort to achieve an athletic body contour that reveals superficial musculature and selectively highlights adherences. We describe a new technique of male body contouring to achieve tight skin that very well reveals muscular bulk and accentuates adherences; minimal observable scars; a male muscular and athletic body, even for patients who are not, nor have been in the past, bodybuilders. Normal-weight and overweight patients need one operative step, in which the fat is removed and the muscles are sculpted at the same time by using selective liposuction, and if necessary, the fat is repositioned through lipofilling. The ex-obese patients need two operative steps: in the first, we perform abdominoplasty with inguinal scar (to remove the excess abdominal tissue and to lift the pubis) and periareolar scar (to breast lift and reduction); in the second, we sculpt the muscles and the adherences by selective liposuction. In all male patients, the authors strive to achieve a trapezoid chest and sculpted pectoralis major muscle, pronounced alba and semilunar lines, evident muscular bulk, and adherences in both the abdomen and extremities., Level of Evidence V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS≥1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Goodpasture's syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening. It was first described as a distinctive syndrome by Pasture in 1919. Autoimmune Inner Ear Disease (AIED) may be associated. The etiology of GS is unknown. Researchers hypothesized a genetic predisposition HLA-associated. Complex immunological mechanisms are in the pathogenesis. The disease is caused by autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen. The limited presence of this molecule in the body explains the interest confined to specific target organs, such as the lung and kidney. It occurs when the immune system attacks the walls of the lungs and the tiny filtering units in the kidneys. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Purpose: Retromolar trigone (RMT) tumours are rare and aggressive malignancies, which require an aggressive surgical approach. The reconstruction oral cavity defects represent a challenge because of the critical role of this area both aesthetically and functionally. Free radial forearm (RF) or anterolateral thigh (ALT) flap are the first choice for the repair of intraoral defects. In reviewing the literature, there is lack of evidence pertaining to the differences between RF and ALT flaps in the reconstruction of patients with RMT tumours. This study evaluates the better microvascular reconstruction after RMT cancer resection., Methods: Thirty patients with RMT cancer underwent oropharingectomy and microvascular reconstruction using the free RF flap (RF group) and the ALT perforator flap (ALT group). The two groups were homogeneous for sex, age, anatomic area, body mass index, and clinicopathologic profile. Viability, complications, scarring, cosmetic appearance, disorder of sensations, ROM, disease-specific items and satisfaction rate were analyzed, and statistical analysis was performed., Study Design: Prospective study., Results: There were differences between the RF and ALT groups in the morphofunctional outcomes, both short-term and long-term follow-up. These differences were statistically significant (p < 0.05) for donor site complications, cosmetic appearance, and scar evaluations. Manual dexterity was slower on the operated donor side than on the nonoperated side in the 33.3 % in the RF group., Conclusions: The study showed that the free ALT perforator flap provides better results in appearance and scarring than the RF flap for intraoral reconstruction after RMT cancer resection.

Susac's syndrome is a rare disease that is characterised by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. It was first described as a distinctive syndrome by Susac in 1979. There have been 304 reported individual patients with Susac's syndrome. Etiopathogenesis is not clear, although it is now thought that it is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear. Antiendothelial cell antibodies (AECAs) play an important role in mediating the endothelial cell injury with consequent deposition of thrombotic material in the lumen of the small vessel. In biopsies of the brain, microinfarcts with atrophy of the white and grey matter could be detected. These microinfarcts are caused by a microangiopathic process with arteriolar wall proliferation, lymphocytic infiltration and basal lamina thickening. At clinical onset, the most common manifestation was central nervous system symptoms, followed by visual symptoms and hearing disturbances. Diagnosis is based on Magnetic Resonance Imaging (MRI), retinal fluorescein angiography, and audiometry; these are considered crucial tests to enable diagnosis. Antiendothelial cell antibodies (AECAs) are also of diagnostic relevance. Based on the hypothesis of being an autoimmune disease, treatment has to be immunosuppressive. In addition, anticoagulation measures, antiplatelet agents and antivasospastic agents should be considered. The majority of patients did not initially present with the complete triad of symptoms. An appropriate approach would be to perform a search for absent components of the triad if the clinical presentation is suggestive of Susac's syndrome. Improved understanding of the presentation of Susac's syndrome will prevent misdiagnosis and ensure that patients receive the best possible care., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Aim: This article reports a case of GCRG seen in a young man with a rare double localization in the same finger and illustrates the degree of diagnostic difficulty and treatment., Material of Study: A 16-year-old man presented with dull pain and swelling of the left middle finger. Plain radiography of the hand showed expansive and lucent lytic lesions with circumferential cortical destruction in the small bones of the proximal and middle phalanges. He underwent en-bloc resection and reconstruction., Results: Histologic findings were consistent with the radiologic diagnosis of GCRG, although several of the features were considered atypical, including the appearance of the giant cells and the areas of the stroma that more closely resembled a giant cell tumor (GCT). At 6 months postoperatively, the morphologic recovery was complete and he had returned to unrestricted activities. His range of motion at the involved joint was the almost completely recovered, and his grip strength on the third setting of the Jamar scale was 48 and 42 kg for the right and left hands respectively., Conclusions: GCRG is a rare intraosseous lesion that must be considered in the differential diagnosis of hand and foot lesions, such us giant cell tumor, brown tumor, giant-cell-rich osteosarcoma.

Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare and distinct variant of low grade fibrosarcoma, found mainly in deep soft tissue of adult extremities. We report a case of Sclerosing Epithelioid Fibrosarcoma of soft tissue, which developed in a 69-year-old woman who presented a tumor involving the fourth finger of her right foot and which has not progressed much in size during the three months prior the surgical excision. Our patient is the hundredth case of Sclerosing Epithelioid Fibrosarcoma reported in literature since 1995, thus confirming the rarity of the tumor. Our experience showed that it is important to make an early diagnosis, in consideration of the clinical aggressiveness of this cancer. Another important aspect concerns the postoperative follow-up. The monitoring of PET-CT technique , although not standardized, could become part of proceedings of therapy and follow-up of tumor, thus allowing oncological radicality and avoid large amputations. To date, 24 months after diagnosis of SEF, our patient feels well, attends our outpatient clinic regularly and shows no evidence of relapse and/or metastasis.

Aim: Circumcision is the most common procedure for phimosis. In recent years, the value of foreskin properties as well as aesthetic reasons determined to other operations preserving the foreskin. We report 5-years experience with a technique that preserve the physical foreskin appearance intact., Patients and Methods: Fifty-two patients, eligible to undergo phimosis surgery, underwent prepuce-sparing plasty and simple running suture. Evaluation of results was made with photos comparative and verified by using presence/absence of recurrence, scarring evaluation, and VAS for patient satisfaction., Results: Forty-eight patients reported no complications. There were no cases of bleeding, infection, pathological scarring, phimosis recurrence. The scar showed a good pliability and a thin thickness. Patient satisfaction was high., Conclusions: The association of prepuce-sparing plasty and simple running suture highlighted an effective and easy method for the correction of acquired phimosis in adult patients, with excellent functional and cosmetic results.

The loss of skin envelope is a frequent and costly problem in health care. This article provides an overview on the state of the art in scalp reconstruction with dermal substitutes, as well as our personal experience of ten critical patients with non-melanoma skin cancer of the scalp. These patients were treated in a two-stage procedure by wide tumor excision, apposition of a dermal induction template (Hyalomatrix®) and successive skin grafting. Four patients underwent subgaleal tumor excision with preservation of the periosteum and six patients en bloc tumor excision together with the external cortical bone. A 10×10 cm template was used in all patients. Two weeks after demolition surgery, we observed neodermis formation. Results were documented by comparative photography, visual analogue scale for patient satisfaction, and Vancouver scar scale for evaluation of final graft characteristics. Patients were tumor-free during follow-up. The procedure achieved good scalp reshaping and graft scarring evolution. Patient satisfaction was high. Hyalomatrix® was effective for oncological scalp reconstruction in critical patients. It prepared the wound bed for graft take while awaiting histological diagnosis and confirmation of margin clearance. Further studies on dermal substitutes are needed to improve benefit in patients.

Basal cell carcinoma (BCC) is the most common type of cancer in Europe, Australia and the U.S.A. After nasal skin cancer excision, especially if it is less than 1-2 cm in diameter, several surgical techniques can be used to reconstruct the lack of substance.In the management of our two patients we have chosen to utilize, instead, a well-known plastic surgery technique, Z-plasty, which is both easy to perform that effective to achieve excellent cosmetic results. As shown by our two clinical cases, the use of Z-plasty for lack of substance of the nose tip and bridge can be extremely effective in terms of cosmetic results. In comparison with the other reconstructive techniques, in fact, it is very easy to perform and allows to avoid or minimize the distortion of the nasal tip and wing, to orient the incision along the skin tension lines and, finally, to replace the lack with very similar structures regarding color, thickness and tissue architecture to those removed.

Radiotherapy represents a major problem in facial surgery. Orbital and periorbital radiation therapy causes a contraction of the soft tissues. Scarring with ectropion is the most severe complication, with shrinking of the anterior lamella, skin dystrophy, muscle atrophy, and alteration of the remaining soft tissues. Goals for reconstruction include correction of distorted orbitofacial tissues and the restoration of orbital structures. The management of these patients is not standardized. We suggest systematically using a combined approach of surgery and lipofilling to restore the orbital deformity and dystrophy, respectively. For this purpose, we present the case of a 65-year-old woman with asymmetry of the orbital regions and severe lower eyelid cicatricial ectropion due to multiple radiation treatments in childhood for an extensive cavernous hemangioma of the right side of the face. We performed a reconstructive procedure using a tarsal strip technique in association with contralateral upper eyelid graft to correct the extensive retraction of the right lower eyelid and lid asymmetry. Subsequently, the patient underwent lipofilling to correct the post-radiotherapy dystrophy. Skin texture, softness, and elasticity greatly improved with further symmetrization. The combined treatment with surgery and lipofilling can significantly improve the functional and cosmetic outcome of shortened and dystrophic eyelids with a successful result with regard to post-radiotherapy retraction., (Copyright © 2012 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Reconstruction of face deformities resulting from skin cancer includes reconstructing the area with similar tissue. This prospective study aimed to compare the functional and aesthetic outcomes between two types of local flaps (the island pedicle flap vs the bilobed flap) used in reconstruction of the ala and back nose., Methods: In this study, 120 patients with skin cancer of the nose underwent ala and back reconstruction: 60 patients using the island flap (IF group) and 60 patients using the bilobed flap (BF group). The two groups were homogeneous for sex, age, and anatomic area. Complications, scarring according to the scale of Vancouver, cosmetic appearance, and disorders of sensations were analyzed, and statistical analysis was performed using Chi-square and analysis of variance (ANOVA)., Results: Early complications were more common in the BF group than in the IF group (p < 0.005). The Vancouver Scar Scale scores were significantly better for the IF patients (p < 0,005), who also showed better results in the analysis of cosmetic outcomes (p < 0.005)., Conclusion: The study showed that the island flap used for ala and back nose reconstruction provides better functional and cosmetic results than the bilobed flap, from both functional and aesthetic points of view., Level of Evidence Iii: This journal requires that authors assign a level of evidence to each article.

Giant cell-rich osteolytic lesions may have overlapping clinical, radiologic, and histopathologic features, with an important degree of difficulty of diagnosis and treatment. We report a case of double osteolytic lesion at the middle-finger in a young man without previous history of hand trauma. He underwent en-bloc resection of the bone lesions and reconstruction by graft of hydroxyapatite, resulting in a good morpho-functional result. Histological diagnosis was giant cell reparative granuloma (GCRG), although several features were considered atypical, including the appearance of the giant cells and the areas of the stroma that more closely resembled a giant cell tumor. GCRG is a benign rare intraosseous lesion and the true nature is controversial and unknown. The theories are that it could be a reactive lesion, a developmental anomaly or a benign neoplasm. It appears as an osteolytic lesion that must be considered in the differential diagnosis of other "critical" bone lesions similar in clinical, as well as radiologic and pathological appearance. Further characterization studies are helpful and necessary for the proper management.

The goals of reconstructing deformities of the face secondary to skin cancer include reconstructing the area with similar tissue. The comparison between different types of local flaps for reconstruction of nasal ala is presented in a prospective, randomized study. The objective of this study was to compare the functional and esthetic outcomes between the nasolabial and island flaps. A total of 60 patients with skin cancer of the nose underwent ala reconstruction: 30 cases by nasolabial flap (group A) and 30 by island flap (group B). The two groups were homogeneous for sex, age, and anatomical area. Complications, scarring according to the scale of Vancouver, cosmetic appearance, and disorders of sensations were analyzed and statistical analysis was performed. In group A, early complications were more common than in group B. The scars of group B patients were more like those of undamaged skin. A total of 10% patients of group A evaluated the cosmetic appearance of their nose as very good, while in group B, 30% patients gave such an evaluation. Local flaps provide better reconstruction and the island flap appears to have more efficacy when compared with nasolabial flap for nasal ala reconstruction.

Introduction: Dog bites present a complex problem. Extensive facial trauma is a challenging problem to treat with priority for functional outcome. This paper describes the conservative treatment in a very difficult case of facial trauma with unusual infections due to the bites., Case Report: A 45 year-old woman was admitted in hypovolaemic shock with amputation of nose, upper and lower lips, left cheek and chin caused by dog bites. After vital parameters and volaemy were stabilized, wound toilet was performed, followed by skin and mucosal rotation flaps and anterior nasal tamponade; the lesion has then been covered with a collagen/oxidized regenerated cellulose dressing and sterile gauzes. Culture test highlighted coagulase-negative Staphylococcus and Candida albicans. However after few days, the patient developed septic-undulant hyperpyrexia, retinitis, renal candidiasis, folliculitis. Systemic Candida infection resistant to fluconazole was diagnosed. Amphotericin B was given to the patient and the facial wound was managed conservatively with an active medication because of inoperability conditions. The outcome of the use of active medications was an immediate response with excessive granulation tissue followed by a rapid re-epithelization., Conclusion: As our case has shown, conservative treatment can be a valid alternative therapy in the treatment of large wounds with invasive candidosis and candidaemia or other major contraindications to surgery. In fact, in cases where surgical reconstruction is not a feasible option, conservative treatment can allow a rapid repair of the skin barrier.

Antiphospholipid syndrome (APS) is an autoimmune condition characterized by vascular thromboses and/or pregnancy morbidity in the presence of antiphospholipid antibodies. The variant "catastrophic" (CAPS) is defined as a potential life-threatening disease, characterized by multiple small vessel thromboses that can lead to multiple organ failure. Surgery is between precipitating factors in CAPS International Registry, but it's still unclear the relationship between surgery and syndrome. The present study reports two surgical procedures, performed on two patients with APS admitted with diagnosis of loss of substance of the lower limb needing of reconstructive treatment. We compared and analyzed aggressive and minimally invasive surgery.