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

Minimally invasive (flapless) crown lengthening by erbium:YAG laser in aesthetic zone.

Crown lengthening is a surgical procedure aimed at exposure of a larger tooth surface by gingivectomy alone or with cortical bone remodelling for aesthetic purposes in the anterior zone of the maxilla or for reconstruction of teeth affected by subgingival caries. We report two cases of crown lengthening in the anterior maxilla for aesthetic purposes by gingival and bone re-contouring performed by erbium-doped yttrium aluminium garnet (erbium:YAG) laser. As highlighted in this report, the erbium:YAG laser-assisted crown lengthening is less invasive and also leads to faster clinical outcomes in contrast to the conventional surgical technique by scalpel incision, flap elevation and osteoplastic

Minimally invasive (flapless) crown lengthening by erbium:YAG laser in aesthetic zone.

Crown lengthening is a surgical procedure aimed at exposure of a larger tooth surface by gingivectomy alone or with cortical bone remodelling for aesthetic purposes in the anterior zone of the maxilla or for reconstruction of teeth affected by subgingival caries. We report two cases of crown lengthening in the anterior maxilla for aesthetic purposes by gingival and bone re-contouring performed by erbium-doped yttrium aluminium garnet (erbium:YAG) laser. As highlighted in this report, the erbium:YAG laser-assisted crown lengthening is less invasive and also leads to faster clinical outcomes in contrast to the conventional surgical technique by scalpel incision, flap elevation and osteoplastic

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

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

Covid-19 Symptomatic Patients with Oral Lesions: Clinical and Histopathological Study on 123 Cases of the University Hospital Policlinic of Bari with a Purpose of a New Classification

The aim of this study is to report on the oral lesions detected in 123 patients diagnosed at the University Hospital of Bari from October 2020 to December 2020, focusing on the correlation of clinical and pathological features in order to purpose a new classification. Methods. General and specialistic anamnesis were achieved and oral examination was performed. The following data were collected: age/gender, general symptoms and form of Covid-19, presence and features of taste disorders, day of appearance of the oral lesions, type and features of oral lesions and day of beginning of therapies. If ulcerative lesions did not heal, biopsy was performed. Results. Many types of oral lesions were found and classified into four groups considering the timing of appearance and the start of the therapies. Early lesions in the initial stages of Covid-19 before the start of therapies was observed in 65.9% of the patients. In the histopathological analysis of four early lesions, thrombosis of small and middle size vessels was always noticed with necrosis of superficial tissues. Conclusion. The presence of oral lesions in early stages of Covid-19 could represent an initial sign of peripheral thrombosis, a warning sign of possible evolution to severe illness. This suggests that anticoagulant therapies should start as soon as possible

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

Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to the onset of different types of lymphoma in such patients. Specifically, patients affected by Sjögren syndrome may develop lymphomas many years after the original diagnosis. Several epidemiologic, hematologic, and histological features may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly extranodal marginal zone lymphomas and, less often, diffuse large B-cell, is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma, and predictive factors, still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57-year-old patient. The diagnostic work-up, including radiograms, core needle biopsy, and histological examination, is discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patient

Oral lichen planus in children: An Italian case series

Oral lichen planus usually occurs in adults; there are no clear data regarding the incidence and the clinical features of oral lichen planus in children. This paper reports clinical findings, treatments, and outcomes of 13 Italian patients with oral lichen planus in childhood diagnosed between 2001 and 2021. The most common finding was keratotic lesions with reticular or papular/plaque-like patterns, confined to the tongue in seven patients. Although oral lichen planus in childhood is rare and the malignant transformation index is unknown, specialists must be aware of its characteristics and oral mucosal lesions must be correctly diagnosed and managed

Multimodality Imaging in Cardiomyopathies with Hypertrophic Phenotypes

Multimodality imaging is a comprehensive strategy to investigate left ventricular hypertrophy (LVH), providing morphologic, functional, and often clinical information to clinicians. Hypertrophic cardiomyopathy (HCM) is defined by an increased LV wall thickness not only explainable by abnormal loading conditions. In the context of HCM, multimodality imaging, by different imaging techniques, such as echocardiography, cardiac magnetic resonance, cardiac computer tomography, and cardiac nuclear imaging, provides essential information for diagnosis, sudden cardiac death stratification, and management. Furthermore, it is essential to uncover the specific cause of HCM, such as Fabry disease and cardiac amyloidosis, which can benefit of specific treatments. This review aims to elucidate the current role of multimodality imaging in adult patients with HCM