25-year Follow-up of Primary Tibial Periosteal Graft for Hard Palate Repair in Cleft Lip and Palate: Outcomes, Concerns and Controversies.

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.

Male Lower Extremity Sculpting.

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 .

Optimal care for rhino-orbital mucormycosis in child with myeloblastic leukemia. Case report and literature review.

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.

Detection of circulating tumor cells in patients with laryngeal cancer using ScreenCell: Comparative pre- and post-operative analysis and association with prognosis.

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.

Lay People Esthetic Evaluation of Primary Surgical Repair on Three-Dimensional Images of Cleft Lip and Palate Patients.

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.

How to eliminate bilateral cleft lip stigmata 30 year experience.

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.

Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease.

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.

Cancer stem cells in laryngeal cancer: what we know.

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.

The prognostic role of E-cadherin and ß-catenin overexpression in laryngeal squamous cell carcinoma.

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, ß-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, ß-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 ß-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 ß-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.

Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches.

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.

Churg-Strauss syndrome.

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.

Retromolar trigone reconstructive surgery: prospective comparative analysis between free flaps.

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.

Double localization of giant cell reparative granuloma of the bone in the same finger. Differential diagnosis and treatment.

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.

The hundredth case of sclerosing epithelioid fibrosarcoma (SEF).

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.

Scalp reconstruction using dermal induction template: state of the art and personal experience.

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.

Use of the Z-plasty technique in nasal skin cancers. Two clinical cases.

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.