Juvenile Recurrent Parotitis: Video-Documented Sialendoscopy.

Juvenile recurrent parotitis (JRP) is characterised by recurrent episodes of painful parotid swelling in children. JRP is the second most common cause of parotitis in childhood, behind only paramyxovirus. The prevention of recurrent attacks represents the most dramatic and serious aspect of this pathology. Since 2004, different authors have evaluated sialendoscopy for the diagnostic and therapeutic management of JRP. In this paper, we share our clinical experience of the use of sialendoscopy for the treatment of JRP. We document with video sialendoscopy the glandular pathology in four children with a mean age of 11.5 years, who had suffered from 3-6 episodes/year of inflammation prior to treatment. The use of sialendoscopy in our patients was effective in preventing recurrences. For the first time, the videosialendoscopy of a series of children diagnosed with JRP is documented in the literature.

Regeneration of mandibular osteoradionecrosis with autologous cross-linked serum albumin scaffold.

Osteoradionecrosis is one of the most severe complications of radiotherapy administered for head and neck tumors. We present the first two cases of advanced and refractory mandibular osteoradionecrosis treated by application of a novel autologous cross-linked 3D serum matrix. Patients were followed clinically and radiographically up to 24 months. Complete wound healing and intact mucosal cover were achieved in both cases. At 12 months, the radiographic values showed an almost complete regeneration of the bone defect, which continued a favourable progression increased to the maximum by 24 months after surgery. The use of an autologous serum-derived scaffold proved to be a quick, predictable, cost-effective and safe adjunct to the conservative surgical treatment of this pathology.

Influence of the teaching program on the learning in knowledge and practice of osteonecrosis of the jaws produced by antireasorptives in dental students of the Principality of Asturias (Spain).

BACKGROUND: This study aims to evaluate the influence of changes in the teaching contents on medication-related osteonecrosis of the jaw may have on the knowledge and the capacity for practical case resolution about this pathology. MATERIAL AND METHODS: A cross-sectional descriptive study was conducted through a survey divided into four sections: degree of means of knowledge acquisition, habitual practice and ability to solve clinical cases. The total number of respondents (n = 225) was divided into two groups: Group A (Year 2015-2016) and Group B (Year 2016-2017). The students in Group B received more teaching content on the subject than group A. RESULTS: A total of 175 survey responses were collected. The internet was the preferred tool for continuing education in both groups. The best known bisphosphonates (BPs) were Alendronate (Fosamax®: 56.9% Group A, 67.5% Group B) and Zoledronic Acid (Zometa®: 56.9% Group A, 51.8% Group B). A low percentage of students (37.9% Group A, 43.4% Group B) acknowledged the existence of other drugs that could also cause osteonecrosis of the jaws. Regarding the correct resolution of practical cases, the respondents of Group B reached a significantly higher score (5.67) than the score observed in Group A (4.04). CONCLUSIONS: Training on medication-related osteonecrosis among dental students is susceptible to improvement. Introducing minor changes in the teachings allows this goal to be successfully achieved. Key words:Osteonecrosis of the jaw (ONJ), bisphosphonate-related osteonecrosis of the jaws (BRONJ), medication-related osteonecrosis of the jaw (MRONJ), dental education.

Revisión sistemática del schwannoma localizado en el suelo oral. A propósito de un caso / Oral floor schwannoma. Systematic review and case report

Objetivos. Revisar de manera sistemática el schwannoma localizado en el suelo oral. Presentar un caso tratado en nuestro departamento. Material y método. Mediante la utilización de motores de búsqueda se identificaron artículos sobre schwannoma localizado en el suelo oral. Los criterios de inclusión fueron: a) diagnóstico definitivo de schwannoma localizado en el suelo oral; b) casos publicados en inglés, español o alemán. Se analizaron variables relacionadas con las características clínicas, diagnósticas y de tratamiento. Resultados. Se identificaron 19 artículos que cumplían los criterios de inclusión. Edad media: 44,3 años (rango 17-77); 42,1% mujeres y 57,8% hombres; tiempo de evolución de 8,7 meses (rango 1-60); tumoración localizada en el lado izquierdo del suelo oral 52,6%, en el lado derecho 42,1%; diámetro mayor medio 38,3 mm (rango 10-70); afectación del nervio hipogloso 15,7%, nervio lingual 15,7%, nervio milohioideo 5,2%, no se identificó nervio de origen en el 21,1% de los casos; punción aspiración con aguja fina no diagnóstica en el 31,5% e identificación de tumoración benigna en el 21%. La exéresis quirúrgica se realizó en el 100% de los casos con un tiempo de seguimiento de 34,3 meses (rango 1-120). No se documentaron recurrencias. Conclusiones. Los schwannomas localizados en el suelo de la boca son infrecuentes. Se presentan en torno a los 40 años, con leve predominancia por el sexo masculino y del lado izquierdo del suelo oral. El tiempo de evolución se encuentra próximo a los 9 meses. Usualmente asintomático. Los nervios hipogloso y lingual se afectan por igual. En proporción similar, el nervio de origen no puede ser identificado. La punción aspiración con aguja fina no es efectiva. El tratamiento de elección consiste en la enucleación del tumor una vez diagnosticado. No se documentaron recidivas (AU)

Objective. Systematically review the oral floor schwannoma. Report of a case treated in our department. Material and method. Published articles about oral floor schwannoma were identified. The inclusion criteria were: a) Final diagnosis of schwannoma located in the oral floor. b) Articles published in English, Spanish or German. The variables were analysed regarding clinical features, diagnosis and treatment. Results. Nineteen articles that met the inclusion criteria. Average age: 44.3 years (range 17-77); 42.1% were females and 57.8% males; time of lesion development was 8.7 months (range 1-60); side of the oral floor location: left side 52.6%, right side 42.1%; average diameter: 38.3 mm (range 10-70); hypoglossal nerve involvement: 15.7%, lingual nerve: 15.7%, mylohyoid nerve: 5.2%, nerve not identified in 21.1% of cases; fine needle aspiration biopsy: non diagnostic in 31.5%, benign tumor identified 21%. Surgical excision was performed in 100% of the cases with a follow up of 34.3 months (rank 1-120). No recurrences were reported. Conclusions. Schwannomas located on the floor of the mouth are uncommon. Environment are presented at age 40 with a slight predominance for males and mainly the left. The time evolution is close to 9 months. The hypoglossal and lingual nerves are affected equally. In similar proportion the nerve of origin cannot be identified. Fine needle aspiration biopsy is not efficient. The treatment of choice is enucleation of the tumor 11 diagnosed. No recurrences were observed (AU)

Sentinel node biopsy versus elective neck dissection. Which is more cost-effective? A prospective observational study.

OBJECTIVE: The aim of this prospective not randomized observational study was to determine the costs and outcomes of sentinel lymph node biopsy (SNB) vs elective neck dissection (END) among patients with early oral squamous cell carcinoma (OSCC). MATERIALS AND METHODS: Seventy-three consecutive patients were divided according to neck staging method. Patients took the decision themselves after receiving detailed information of both suggested treatment tools. False negative (FN) and negative predictive value (NPV) were assessed. Log-rank test was used to compare disease-free survival (DFS) and overall survival (OS). Only direct costs were analysed. Cost information derived from volumes for hospital stay, surgery and neck outcome were obtained from an internal database of tertiary health care center. RESULTS: Thirty-two patients underwent SNB and 41 underwent an END (levels I-III). Average follow-up time was 48.2 months (range 7-80). Five neck recurrences were recorded in the SNB group (range 11-21). Seven neck recurrences occurred in the END group (range 9-16). No significant differences were found in DFS or OS. True negative patients in SNB group incurred in 42% less costs than END group. FN regardless of radiotherapy, was also lower in the SNB group. However, pN+ patients generated 23% more costs in the SNB group. CONCLUSION: In this not randomized observational study with an average follow-up period of 48.2 months, SNB appear to confer less cost than END, with similar prognosis.

Repair of segmental mandibular bone defects in sheep using bone marrow stromal cells and autologous serum scaffold: a pilot study.

AIM: The aim of this study was to evaluate effective bone regeneration using an autologous serum scaffold (alone or seeded with autologous bone marrow-mesenchymal stem cells, BM-MSCs), when implanted in a 30 mm length segmental mandibular defect in sheep. MATERIALS AND METHODS: The bone defect was filled either with serum scaffold alone (control group; n = 5) or combined with BM-MSCs (experimental group; n = 10). Bone regeneration was determined at 12 (T12; 2 control sheep and 4 experimental sheep) and 32 weeks (T32; 3 control and 6 experimental sheep), as measured by computed and microcomputed tomography and histological examination. RESULTS: Two sheep of the Experimental group died after surgery. While complete bone union in the control group was only observed at T32, it was observed both at T12 (1/4 sheep) and T32 (3/4 sheep) in the experimental group. When properties/characteristics of new bone where compared, a better bone quality, similar to native bone, was observed in the scaffold combined with BM-MSCs. CONCLUSIONS: Based on these results, we conclude that the serum scaffold can promote efficient repair of large bone defects, but the combination with BM-MSCs accelerates this process, increasing significantly the amount and quality of bone formed.

Epidemiología, farmacología y caracterización clínica de la osteonecrosis de los maxilares. Un estudio retrospectivo de 70 casos / Epidemiology, pharmacology and clinical characterization of bisphosphonate-related osteonecrosis of the jaw. A retrospective study of 70 cases

Introducción y objetivos: Los bisfosfonatos son fármacos con un amplio espectro de indicaciones cuya principal capacidad es la inhibición de la función osteoclástica. En el año 2003 se ha descrito una complicación asociada a su empleo, la osteonecrosis de los maxilares por bisfosfonatos (ONMB). Los objetivos del presente estudio son identificar los casos recogidos de ONMB en un hospital de tercer nivel durante 8 años, evaluando las principales variables en relación con la enfermedad, el bisfosfonato empleado y los factores de riesgo locales o generales que pudieran actuar como desencadenante en la patogénesis de la ONMB. Material y método: Se procedió a la selección los pacientes diagnosticados de ONMB en un centro de referencia para una población de 1.100.000 habitantes. Las variables analizadas se dividieron en 3 grupos: pacientes, fármaco (incluyendo el análisis de la dosis aplicada y la ponderación dosis/potencia) y osteonecrosis. Resultados: Se recogieron 70 casos (44 mujeres y 26 varones), con una media de 66,8 años. Dieciocho pacientes habían recibido un aminobisfosfonato oral y 52 por vía intravenosa. El tiempo medio de administración fue de 26,53 meses. En el 67,1% de los pacientes se pudo identificar un factor local desencadenante, siendo el más frecuente la exodoncia (48,6%). Aunque la exposición ósea estaba presente en el 75,7% de los casos, 8 enfermos padecieron una osteonecrosis sin exposición, manifestando la presencia de dolor y/o fístula crónica. El 58,6% experimentaron una resolución completa con un tiempo medio de control de 16,28 meses. Conclusiones: El 25% de las ONMB en nuestra serie se relacionaron con la administración de un bisfosfonato oral, especialmente el alendronato. El ácido zoledrónico es el agente que menos miligramos precisa para desarrollar la enfermedad. La exposición ósea solitaria fue el dato clínico más habitual, afectando especialmente a sectores posteriores mandibulares en pacientes con enfermedad metastásica (AU)

Background and objectives: Bisphosphonates are widely prescribed drugs whose principal capacity is inhibiting the osteoclast function. In 2003 a complication related to their administration, bisphosphonate-related osteonecrosis of the jaw (BRONJ), was described. The objectives of this study were to identify diagnosed cases of BRONJ in a third-level hospital over 8 years, evaluating the main features in relation to the disease, the bisphosphonate and the presence of local or general risk factors that could trigger the BRONJ. Material and method: Patients diagnosed with BRONJ in a centre of reference for a population of 1,100,000 inhabitants were selected. Variables analysed were classified into 3 groups: patients, bisphosphonate (focusing on dose and weighting dose/potency) and osteonecrosis. Results: Seventy cases were studied —44 women and 26 men—, with a mean age of 66.8 years. Eighteen patients received bisphosphonates orally and 52, intravenously. The mean time of administration was 26.53 months. In 67.1% of the patients it was possible to identify a local trigger, with the most common being tooth extraction (48.6%). Bone exposure was present in 89.2% of the cases, while 12 patients developed BRONJ without exposed bone, with only pain and/or chronic sinus tracts. Complete resolution was achieved in 58.6% of the patients, with a mean time of control of 16.28 months. Conclusions: 25% of the BRONJ cases were related to the administration of oral bisphosphonates, especially alendronate. Zoledronic acid was the bisphosphonate that required the fewest milligrams to induce osteonecrosis. Single bone exposure was the most common clinical finding, especially in the molar mandibular region in patients with metastatic disease (AU)

Epidemiology, pharmacology and clinical characterization of bisphosphonate-related osteonecrosis of the jaw. A retrospective study of 70 cases.

BACKGROUND AND OBJECTIVES: Bisphosphonates are widely prescribed drugs whose principal capacity is inhibiting the osteoclast function. In 2003 a complication related to their administration, bisphosphonate-related osteonecrosis of the jaw (BRONJ), was described. The objectives of this study were to identify diagnosed cases of BRONJ in a third-level hospital over 8 years, evaluating the main features in relation to the disease, the bisphosphonate and the presence of local or general risk factors that could trigger the BRONJ. METHODS: Patients diagnosed with BRONJ in a centre of reference for a population of 1,100,000 inhabitants were selected. Variables analysed were classified into 3 groups: patients, bisphosphonate (focusing on dose and weighting dose/potency) and osteonecrosis. RESULTS: Seventy cases were studied -44 women and 26 men-, with a mean age of 66.8 years. Eighteen patients received bisphosphonates orally and 52, intravenously. The mean time of administration was 26.53 months. In 67.1% of the patients it was possible to identify a local trigger, with the most common being tooth extraction (48.6%). Bone exposure was present in 89.2% of the cases, while 12 patients developed BRONJ without exposed bone, with only pain and/or chronic sinus tracts. Complete resolution was achieved in 58.6% of the patients, with a mean time of control of 16.28 months. CONCLUSIONS: 25% of the BRONJ cases were related to the administration of oral bisphosphonates, especially alendronate. Zoledronic acid was the bisphosphonate that required the fewest milligrams to induce osteonecrosis. Single bone exposure was the most common clinical finding, especially in the molar mandibular region in patients with metastatic disease.

Sinus mucosal thickening in bisphosphonate-related osteonecrosis of the jaws: a case-control study.

Osteonecrosis of the jaws is a clinically significant complication of bisphosphonate (BP) medications. Otherwise, the effects of BPs on oral soft tissue or cells remain unknown. The main objective of the present study was to determine whether the presence of sinus mucosal thickening was significantly related to BP-related osteonecrosis of the jaw (BRONJ). A case-control study was conducted on 32 patients who underwent treatment of BRONJ with conventional radiological investigations (panoramic radiographs) and computed tomography. The results indicated that patients with BRONJ had a 5.57-fold greater probability of presenting sinus mucosal thickening than controls. Although the existence of this thickening was more common in patients with advanced-stage disease or low levels of C-telopeptide-cross-linked type I collagen, no significant difference was observed between cases and controls. While considering the limitations inherent in the design and number of cases analyzed in our study, patients with osteonecrosis of the jaw were found to have a 5.57-fold greater probability of presenting sinus mucosal thickening (>3 mm) than healthy subjects.

Alternative treatments for oral bisphosphonate-related osteonecrosis of the jaws: A pilot study comparing fibrin rich in growth factors and teriparatide

OBJECTIVES: The aim of this study is to describe and compare the evolution of recurrent bisphosphonate-related osteonecrosis of the jaws (BRONJ) in patients treated with plasma rich in growth factors or teriparatide. MATERIAL AND METHODS: Two different types of treatments were applied in patients diagnosed of recurrent BRONJ in a referral hospital for 1.100.000 inhabitants. In the group A, plasma rich in growth factors was applied during the surgery. In the group B, the treatment consisted in the subcutaneous administration of teriparatide. All the cases of BRONJ should meet the following conditions: recurrent BRONJ, impossibility of surgery in stage 3 Ruggiero classification and absence of diagnosed neoplastic disease. Clinical and radiographic evolution of the patients from both groups was observed. RESULTS: Nine patients were included, 5 in group A and 4 in group B. All the patients were women on oral bisphosphonate therapy for primary osteoporosis (5 patients) or osteoporosis-related to the use of corticosteroids (4 patients). Alendronate was the most common oral bisphosphonate associated with BRONJ in our study (four patients in group A and two in group B). The mean age was 72,8 years in the group A and 73,5 years in the group B. All the patients from group A showed a complete resolution of their BRONJ. Only one patient in the group B showed the same evolution. CONCLUSIONS: In our series, the plasma rich in growth factors showed better results than the teriparatide in the treatment of recurrent BRONJ

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Bisphosphonate-related osteonecrosis associated with dental implants: a microcomputed tomography study.

PURPOSE: The aims of this study were to quantify bone mineral density (BMD) and trabecular architecture of sequestra in patients with bisphosphonate-related osteonecrosis of the jaw (BRONJ) associated with dental implants and to assess if there are differences between trabecular bone in "implant loading-triggered" and "implant surgery-triggered" BRONJ. MATERIALS AND METHODS: Bone sequestra of 2 patients diagnosed with BRONJ associated with dental implants were scanned using high-resolution microcomputed tomography (microCT). Images were obtained at a voltage of 50 kV and 800 µA, and the specimens were scanned at 180 degrees with a single rotation step of 0.3, 1-mm aluminum filter, and a pixel size of 12 µm. The morphometric parameters examined were: BMD, ratio of bone volume/tissue volume (BV/TV), trabecular thickness (Tb.Th), trabecular number (Tb.N), trabecular separation (Tb.Sp), connectivity density (Conn.D, mm), degree of anisotropy, and the structural model index. RESULTS: BMD and BV/TV were higher in bone sequestration than in healthy bone. Tb.Sp was lower and Tb.N and Tb.Th were higher in the BRONJ group. Conn.D and Tb.N values were significantly high in implant surgery-triggered sequestrum but substantially low in sequestra caused by loading as compared with those of the control sample. CONCLUSIONS: MicroCT is useful for assessing bone sequestration of BRONJ associated to dental implants. The necrotic bone is similar to that described in conventional BRONJ.

Alternative treatments for oral bisphosphonate-related osteonecrosis of the jaws: a pilot study comparing fibrin rich in growth factors and teriparatide.

OBJECTIVE: The aim of this study is to describe and compare the evolution of recurrent bisphosphonate-related osteonecrosis of the jaws (BRONJ) in patients treated with plasma rich in growth factors or teriparatide. MATERIAL AND METHODS: Two different types of treatments were applied in patients diagnosed of recurrent BRONJ in a referral hospital for 1.100.000 inhabitants. In the group A, plasma rich in growth factors was applied during the surgery. In the group B, the treatment consisted in the subcutaneous administration of teriparatide. All the cases of BRONJ should meet the following conditions: recurrent BRONJ, impossibility of surgery in stage 3 Ruggiero classification and absence of diagnosed neoplastic disease. Clinical and radiographic evolution of the patients from both groups was observed. RESULTS: Nine patients were included, 5 in group A and 4 in group B. All the patients were women on oral bisphosphonate therapy for primary osteoporosis (5 patients) or osteoporosis-related to the use of corticosteroids (4 patients). Alendronate was the most common oral bisphosphonate associated with BRONJ in our study (four patients in group A and two in group B). The mean age was 72,8 years in the group A and 73,5 years in the group B. All the patients from group A showed a complete resolution of their BRONJ. Only one patient in the group B showed the same evolution. CONCLUSION: In our series, the plasma rich in growth factors showed better results than the teriparatide in the treatment of recurrent BRONJ.

Inflammatory myofibroblastic tumor of the lung and the maxillary region: a benign lesion with aggressive behavior.

Inflammatory myofibroblastic tumor (IMT) is a rare mass-forming lesion characterized by fibroblastic or myofibroblastic spindle cell proliferation with varying degrees of inflammatory cell infiltration. Although it has been reported in virtually every organ in the body, the lung is the most common site of involvement. Extrapulmonary IMTs, although rare, have been reported and are characterized by different, more aggressive behavior. We report an extremely rare case of maxillary metastases of pulmonary IMT. Lung IMT was initially misdiagnosed, and oral lesion mimicked clinically and radiologically a radicular cyst. On histologic examination, cells exhibited diffuse and intense immunoreactivity for α -smooth muscle actin and vimentin whereas both pulmonary and oral IMTs presented absence of cellular atypia and lack of expressivity of oncogenic determinants. Distant metastases of lung IMT are extremely unusual, and this is the first report to our knowledge with this particular clinical course. Despite the possibility that the present case could also represent a metachronous multifocal IMT, with pulmonary and extrapulmonary lesions, similar histopathological and immunohistochemical patterns in lung and maxillary region suggest a metastatic course.

Merkel cell carcinoma: Our experience in this rare pathology

Merkel cell carcinoma (MCC) was first described in 1972 by Toker, who described five cases of the so-called“trabecular carcinoma of the skin”. MCC is a rare, aggressive skin cancer that affects mainly the elderly. Sun exposedareas are mainly affected, specially the head and neck. Immunohistochemical analysis is essential to reacha correct diagnosis. According to the origin of MCC, the tumor expresses both epithelial and neuroendocrinemarkers. MCC has a propensity for recurrence, regional and distant metastases. Several treatment options areavailable, such as surgical excision or Moh’s surgery, accompanied by neck dissection or radio and chemotherapyin advanced cases. The present study aims to evaluate the clinical behaviour and the evolution of five cases of thisdisease that were treated at our institution (reference to 1100000 inhabitants) in the last five years. It is stressed onthe bad prognosis of this pathology, which presents high rates of locoregional recurrence, even though the recentadvances in radio and chemotherapy (AU)

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Oncocytic carcinoma of the parotid gland with late cervical lymph node metastases: a case report.

INTRODUCTION: Oncocytic carcinoma is a rare proliferation of cytomorphologically malignant oncocytes mainly found in glandular tissue, accounting for 0.5% of all epithelial salivary gland malignancies and 0.18% of all epithelial salivary gland tumors. CASE PRESENTATION: We report a case of oncocytic carcinoma arising in the parotid gland of a 65-year-old Caucasian man. Our patient initially underwent left superficial parotidectomy, including the removal of the mass. A close follow-up was made, and four years after first surgery cervical lymph node metastases were confirmed. Therefore, a complete parotidectomy and radical neck dissections were performed. There were no complications and no sign of recurrence after six months of follow-up. CONCLUSION: Oncocytic carcinoma is an extremely rare malignancy in the salivary glands. Prophylactic neck dissection may be indicated for tumors larger than 2 cm in diameter (our patient's tumor was 2.5 cm at its greatest diameter). The clinical course of our patient, with the appearance of cervical lymph node metastases after four years of follow-up, supports this approach. Further investigation of the prognosis and correct treatment of patients with oncocytic carcinoma are required as more cases are reported.

Merkel cell carcinoma: our experience in this rare pathology.

Merkel cell carcinoma (MCC) was first described in 1972 by Toker, who described five cases of the so-called "trabecular carcinoma of the skin ". MCC is a rare, aggressive skin cancer that affects mainly the elderly. Sun exposed areas are mainly affected, specially the head and neck. Immunohistochemical analysis is essential to reach a correct diagnosis. According to the origin of MCC, the tumor expresses both epithelial and neuroendocrine markers. MCC has a propensity for recurrence, regional and distant metastases. Several treatment options are available, such as surgical excision or Moh's surgery, accompanied by neck dissection or radio and chemotherapy in advanced cases. The present study aims to evaluate the clinical behaviour and the evolution of five cases of this disease that were treated at our institution (reference to 1100000 inhabitants) in the last five years. It is stressed on the bad prognosis of this pathology, which presents high rates of locoregional recurrence, even though the recent advances in radio and chemotherapy.

Unilateral absence of submandibular gland secondary to stones. Aplasia versus early atrophy

Major salivary gland absence is a rare disorder. The cause of congenital absence of the salivary glands has not been determined, but it may be associated with ectodermal defects of the first and second branchial arches. Isolated absence of a unilateral submandibular gland is an unusual entity with less than ten cases reported in the literature.The etiopathogenesis of isolated absence of a major salivary gland without other developmental anomaliesis still unclear. The formation of a sialolith within the remaining Wharton’s duct, associated with isolated aplasia(versus atrophy) of a unilateral submandibular gland has been recently reported. We describe in this work two cases of sialolithiasis within the ipsilateral remaining Wharton´s duct in patients with isolated absence of a unilateral submandibular gland. In the cases reported, absence of the submandibular gland may have been the result ofthe complete acinar atrophy secondary to an early obstruction of Wharton´s duct (AU)

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Unilateral absence of submandibular gland secondary to stones. Aplasia versus early atrophy.

Major salivary gland absence is a rare disorder. The cause of congenital absence of the salivary glands has not been determined, but it may be associated with ectodermal defects of the first and second branchial arches. Isolated absence of a unilateral submandibular gland is an unusual entity with less than ten cases reported in the literature. The etiopathogenesis of isolated absence of a major salivary gland without other developmental anomalies is still unclear. The formation of a sialolith within the remaining Wharton's duct, associated with isolated aplasia (versus atrophy) of a unilateral submandibular gland has been recently reported. We describe in this work two cases of sialolithiasis within the ipsilateral remaining Wharton's duct in patients with isolated absence of a unilateral submandibular gland. In the cases reported, absence of the submandibular gland may have been the result of the complete acinar atrophy secondary to an early obstruction of Wharton's duct.

Autosomal dominant osteopetrosis and maxillomandibular osteomyelitis.

Osteopetroses represent a heterogeneous group of rare, hereditary bony dysplasias. They range from a devastating neurometabolic disease (including severe malignant infantile osteopetrosis) to 2 more benign conditions principally affecting adults: autosomal dominant osteopetrosis (ADO) type I and type II. The present study describes the maxillofacial manifestations associated with the 2 subgroups of ADO. In this paper, we present the oldest patient described in the literature with ADO type I (76 years old). We also document the first ADO type II patient described in the literature with synchronic osteomyelitis of the mandible and the maxilla.

Complete spontaneous regression of Merkel cell carcinoma.

Merkel cell carcinoma (MCC) is a very aggressive primary cutaneous neoplasm most often occurring on the head and neck of the elderly. Spontaneous regression of MCC was first described in 1986. A 79-year-old woman with MCC on the right cheek underwent spontaneous regression of the malignancy, documented by photographic follow-up, computed tomography, and histologic studies. A review of the literature is presented. Complete clinical and histologic regression of MCC was observed in the present case. Although the literature documents 11 similar cases, only 6 can be regarded as complete spontaneous regressions following exclusive performance of a biopsy (primary complete spontaneous regression). Primary complete spontaneous regression of MCC is infrequent, and most case reports describe this phenomenon in women with MCC on the cheek. The reasons underlying regression are unknown.