Keratocystic odontogenic tumors as first clinical manifestation of nevoid basal cell carcinoma syndrome in pediatric age: our microinvasive surgical approach.

Keratocystic odontogenic tumors as first clinical manifestation of nevoid basal cell carcinoma syndrome in pediatric age: our microinvasive surgical approach. Aim. Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is a rare genetic condition involving multiple organs; Keratocystic Odontogenic Tumors (KCOTs) are often the first clinical manifestation in pediatric age. The aim of this study was to describe the clinical and histopathological features of KCOTs as first clinical sign of NBCCS in pediatric patients allowing an early diagnosis, and their treatment with conservative microinvasive piezosurgery. Methods. Twenty pediatric patients affected by NBCCS showing 60 KCOTs came to our attention at the Complex Operating Unit of Odontostomatology, Policlinic of Bari, from 1996 to 2014. After clinical examination, OPT and computed tomography analysis, all patients underwent conservative micro-invasive surgical treatment under general anesthesia, consisting in enucleation of KCOTs, cavity ostectomy and osteoplasty with conventional rotative instruments and piezoelectric tools in order to remove damaged bone, epithelial remnants and satellite cysts with maximal teeth preservation in consideration of the young age of the patients, and to possibly minimize the recurrence risk. A sterile gel formulation of sodium hyaluronate and amino acids (Gly-Pro-Leu-Lys) was put into the bone defect, allowing for faster bone regeneration and healing of the surgical site. All the surgical specimens were sent for histopathological examination. The patients underwent clinical and radiological (OPT) follow-up after 7, 15 and 30 days, 2, 3, 6 and 12 months, and, then, once a year. The followup time ranged from 18 months to 8 years. Results. There were 10 males and 10 females, with an average age of 10,6 years. At the initial evaluation, 13 patients showed swelling, teeth agenesis, and dental inclusions or dislocations as first clinical manifestation of their disease; in the remaining cases, KCOTs were asymptomatic. Among the 20 patients, 11 had been previously diagnosed with NBCCS because of familiarity or presence of characteristic features of the syndrome, while KCOTs were the first clinical sign of NBCCS for the remaining 9 patients without familial history of the syndrome; subsequently, the genetic analysis showing PTCH1 mutations confirmed the diagnosis. 60 KCOTs were totally identified: 39 lesions were located in the mandible and 21 lesions were located on the upper jaw. The histopathological analyses highlighted thin connective tissue walls covered by para- and orthokeratotic stratified squamous epithelium, usually about 5-8 cell layers thick, around cystic lumens filled with desquamated keratin, thus confirming the diagnosis of KCOT. The epithelial lining included a well-defined, palisading basal layer of cuboidal to small columnar cells and a superficial layer with corrugated appearance. Satellite cysts could also be seen due to tissue budding of the basal cell layers into the adjacent connective tissue. The clinico-radiological follow-up of 53 lesions showed progressively decreased radiolucent areas up to complete healing within 12 months from the surgical treatment. Differently, OPT disclosed lack of decreasing radiolucency of 7 lesions after 12 months suggesting KCOTs recurrence, that is more frequent in syndromic KCOTs than in sporadic ones due to their higher dimensions and to the involvement of nobile structures such as teeth which should be preserved in these young patients as much as possible. Conclusion. KCOTs in pediatric patients require conservative approaches for permanent teeth preservation. While conventional enucleation leads up to 60% recurrence rates, cavity ostectomy with piezoelectric tools significantly reduced the recurrence risk allowing the preservation of permanent teeth

3D Cephalometric Normality Range: Auto Contractive Maps (ACM) Analysis in Selected Caucasian Skeletal Class I Age Groups

The objective of this paper is to define normal values of a novel 3D cephalometric analysis and to define the links through an artificial neural network (ANN)

Tissuue Engineering in Maxillar Sinus Lifting: A Comparation of Differents Grafts and Confocal Laser Scanning Microscopic Evaluation

Bone is a specialized connective tissue, most prominently characterized by its mineralized organic matrix that imparts the physical properties that allow bone tissue to resist load, to support functional organs, and to protect highly sensitive body parts. Bone loss and bone damage may occur as a result of genetic conditions, infectious diseases, tumours, and trauma. Bone healing and repair, involves integrative activity of native tissues and living cells, and lends itself to the incorporation of naturally derived or biocompatible synthetic scaffolds, aimed at replacing missing or damaged osseous tissues. There are several modalities of bone regeneration including tissue engineering, guided bone regeneration, distraction ontogenesis, and bone grafting. This book concentrates on such procedures that may well be counted among the recent outstanding breakthroughs in bone regenerative therapy

Temporomandibular Disorders in Psoriasis Patients with and without Psoriatic Arthritis: An Observational Study

Psoriasis is a chronic, remitting and relapsing inflammatory disorder, involving the skin, nails, scalp and mucous membranes, that impairs patients' quality of life to varying degrees. Psoriatic arthritis is a chronic seronegative, inflammatory arthritis, usually preceded by psoriasis. Temporomandibular disorders is a generic term referred to clinical conditions involving the jaw muscles and temporomandibular joint. The aim of this study was to assess symptoms and signs of temporomandibular disorders in psoriasis patients with and without psoriatic arthritis

Peripheral Giant Cell Granuloma of the Jaws as First Sign of Primary Hyperparathyroidism: A Case Series

Peripheral giant cell granulomas (PGCG) associated with hyperparathyroidism (HPT) are rare clinical entities. The aim of this study is to report on 21 PGCGs of the oral cavity as the first clinical sign of unknown primary HPT (PHPT) referred to the Complex Operating Unit of Odontostomatology of Aldo Moro University of Bari from 2009 to 2019. Surgical treatment consisted in conservative enucleation of the lesion, if possible, with contextual bone rim osteoplasty with piezosurgical tools and following histological examination. After histological diagnosis of PGCG, PHPT screening was performed dosing parathyroid hormone and serum calcium. In all the patients haematological investigation demonstrated elevated values of parathyroid hormone and serum calcium ruling out an unknown PHPT. Specifically, after endocrinological evaluation, patients showed PHPT related to: parathyroid adenoma (13), parathyroid hyperplasia (two, one of which occurred in a intra-thyroidal parathyroid), and parathyroid carcinoma (1) and were scheduled for surgical treatment. Considering that PGCGs could represent the first clinical sign of an undiagnosed PHPT and the screening of PHPT is a non-invasive and cheap exam, in case of histological diagnosis of a giant cell lesion, both central and peripheral, especially in patients with synchronous or history of methacronous giant cell lesions, parathyroidal screening should be mandatory

New Dimensional Staging of Bisphosphonate-Related Osteonecrosis of the Jaw Allowing a Guided Surgical Treatment Protocol: Long-Term Follow-Up of 266 Lesions in Neoplastic and Osteoporotic Patients from the University of Bari

Bisphosphonate-related osteonecrosis of the jaw (BRONJ) is the most serious side effect in patients receiving bisphosphonates (BPs) for neoplastic disease and osteoporosis. The aim of this study is to propose a new dimensional stage classification, guiding the surgical treatment of BRONJ patients, and to evaluate the success rate of this new management. From 2004 to 2013, 203 neoplastic and osteoporotic patients with 266 BRONJ lesions were referred to the Odontostomatology Unit of the University of Bari. All patients underwent surgery after suspension of BPs therapy and antibiotic treatment. The surgical procedure was complemented by piezosurgery and followed by the application of hyaluronate and amino acids. The new dimensional staging suggests the choice of the surgical approach, and allows the prediction of postoperative complications and soft and hard tissues healing time, guiding the surgical treatment protocol. This protocol could be a successful management strategy for BRONJ, considering the low recurrences rate and the good stabilisation of the surgical sites observed after a long-term follow-up

Fibrolipoma of the lip treated by diode laser surgery: A case report

<p>Abstract</p> <p>Introduction</p> <p>Several neoplasms of the adipose tissue can involve the soft tissues of the head and neck region. These neoplasms are mainly treated surgically and an accurate histological examination is mandatory for a precise diagnosis.</p> <p>Case presentation</p> <p>We report a case of fibrolipoma involving the lower lip of a 43-year-old man, which was successfully treated by diode laser surgery. This approach allowed adequate resection of the neoplasm with minimal damage to the adjacent tissues, thus reducing post-surgical scarring.</p> <p>Conclusion</p> <p>Diode laser surgery for the treatment of benign lesions of the oral mucosa appears to be a convenient alternative to conventional blade surgery and has proved to be effective for the excision of fibrolipoma of the lip. The possibility of avoiding direct suture after excision is surely helpful when aesthetic areas, such as the lip, are surgically treated. For these reasons, and also considering the lower histological alteration of the specimen obtained with diode laser surgery if adequately used, the diode laser is undoubtedly a good alternative to conventional surgery.</p

Preliminary data on Pemphigus vulgaris treatment by a proteomics-defined peptide: a case report

BACKGROUND: Although described by Hippocrates in 400 B.C., pemphigus disease still needs a safe therapeutical approach, given that the currently used therapies (i.e. corticosteroids and immunosuppressive drugs) often provoke collateral effects. Here we present preliminary data on the possible use of a proteomics derived desmoglein peptide which appears promising in halting disease progression without adverse effects. METHODS: The low-similarity Dsg3(49–60)REWVKFAKPCRE peptide was topically applied for 1 wk onto a lesion in a patient with a late-stage Pemphigus vulgaris (PV) complicated by diabetes and cataract disease. The peptide was applied as an adjuvant in combination with the standard corticosteroid-based immunosuppressive treatment. RESULTS: After 1 wk, the treated PV eroded lesion appeared dimensionally reduced and with an increased rate of re-epithelization when compared to adjacent non-treated lesions. Short-term benefits were: decrease of anti-Dsg antibody titer and reduction of the corticosteroid dosage. Long-term benefits: after two years following the unique 1-wk topical treatment, the decrease of anti-Dsg antibody titer persists. The patient is still at the low cortisone dosage. Adverse effects: no adverse effect could be monitored. CONCLUSION: With the limits inherent to any preliminary study, this case report indicates that topical treatment with Dsg3(49–60)REWVKFAKPCRE peptide may represent a feasible first step in the search for a simple, effective and safe treatment of PV

Primary Oro-Facial Manifestations of Langerhans Cell Histiocytosis in Pediatric Age: A Bi-Institutional Retrospective Study on 45 Cases

Aims: Langerhans Cell Histiocytosis is a rare hematologic disorder usually affecting children and most commonly involving the head and neck region. Primary oro-facial manifestations are rare, and their diagnosis is often challenging as they are numerous and often resemble common pathologies, refractory to conventional medical and/or instrumental treatments. For such reasons, the diagnosis is frequently delayed, as is the following staging and therapy onset. We retrospectively studied 45 pediatric patients affected by Langerhans Cell Histiocytosis with onset in the head and neck, to examine their clinical and radiological features at the early stage. Materials and Methods: The study was a retrospective bi-institutional analysis (Department of Pediatric Dentistry and Pediatric Oncology of “Sapienza” University of Rome, Department of Interdisciplinary Medicine of the University of Bari “Aldo Moro”), which enrolled 45 patients (age range 0–18 year-old) affected by Langerhans Cell Histiocytosis with oro-facial onset. Data regarding clinical appearance, number, site, synchronous or metachronous occurrence, involved tissues/organs, radiographic features and clinical outcomes were collected, listed and overall differentiated by two age ranges (0–10-year-olds and 10–18-year-olds). Results: Patients were 26 males and 19 females, with an average age at the time of diagnosis of 4.8 ± 3.8 years (median = 3.9 years). The most common findings were inflamed, hyperplastic, painful and often ulcerated gingival lesions (22 cases), associated with deciduous tooth mobility and/or dislocation with bone loss in 18 cases, followed by nine single eosinophilic granulomas of the mandible and two of the maxilla. Lesions of the palatal mucosa were observed in six patients; nine patients showed on radiograms the characteristic “floating teeth” appearance in the mandible with synchronous lesions of the maxilla in six. Paresthesia was relatively un-frequent (three cases) and the pathological fracture of the mandible occurred in six. Head/neck lymph nodes involvement was associated with oral lesions in 12 cases and skull lesions in 14. Otitis (media or externa) was detected in four instances, exophthalmia in two, cutaneous rush in nine, contextual presence or subsequent onset of insipidus diabetes in eight. As for therapy, single or multiple small jaw lesions were all surgically removed; chemotherapy with vinblastine alone or associated with corticosteroids was the principal treatment in almost the 80% of cases; more than 50% of patients received corticosteroids, while only three patients received adjunctive radiotherapy. The overall mortality account for less than 9% (four of 45 cases) and recurrence observed in eight patients after therapy. Conclusions: Langerhans Cell Histiocytosis may mimic several oro-facial inflammatory and neoplastic diseases. Considering the potential disabling sequela following head and neck localization of Langerhans Cell Histiocytosis in children, especially at the periodontal tissues with teeth and alveolar bone loss, lesion recognition along with the histological examination of suspicious tissues is mandatory to achieve an early diagnosis and to prevent further organ involvement