Rehabilitación bucal después de la retirada de un fibroma cemento-osificante central / Oral rehabilitation after removal of a central cemento-ossifying fibroma

ABSTRACT Introduction: The cemento-ossifying fibroma is a benign bone neoplasm that affects mainly the female sex during the third or fourth decades of life. This lesion has a slow growth and the treatment is the surgical removal of the tumor, because radiotherapy is not indicated. Objective: to describe and discuss a case of central cemento-ossifying fibroma. Case report: A 41-year-old female patient sought dental treatment due to tooth mobility in the anterior region of the mandible. In this region of the mandible, an increase in volume was noted with a firm consistency on palpation, covered by normal mucosa, and displacement of teeth. Radiographically, the presence of an extensive lesion in this region was observed. An incisional biopsy was performed which led to the final diagnosis of central cemento-ossifying fibroma. Subsequently the tumor was completely removed. An autogenous bone graft was performed and four osseointegrated implants were installed to rehabilitate the patient. Implant-supported prostheses (lower arch) and a complete denture (upper arch) were installed to restore esthetics and function of the patient's oral cavity. Conclusion: Despite being a benign tumor, the central cemento-ossifying fibroma caused functional and aesthetic damage to the patient and required a complex rehabilitation treatment. After approximately 5 years of tumor removal, there was no recurrence of the lesion or signs of peri-implant or periodontal diseases, evidencing the success of the treatments(AU)

RESUMEN Introducción: El fibroma cemento-osificante es una neoplasia ósea benigna que afecta principalmente al sexo femenino durante la tercera o cuarta décadas de la vida. Esta lesión tiene un crecimiento lento y el tratamiento es la extirpación quirúrgica del tumor, porque la radioterapia no está indicada. Objetivo: Describir y analizar un caso de un fibroma cemento-osificante central. Presentación caso: Paciente de 41 años de edad buscó tratamiento dental debido a la movilidad dental en la región anterior de la mandíbula. En esta región de la mandíbula se observó un aumento de volumen con una consistencia firme a la palpación, cubierta por la mucosa normal y desplazamiento de los dientes. Radiográficamente, se observó la presencia de una lesión extensa en esta región. Se realizó una biopsia incisional, que indicó el diagnóstico final del fibroma cemento-osificante central. Después el tumor fue completamente extirpado. Se realizó un injerto óseo autógeno y, posteriormente, se instalaron cuatro implantes osteointegrados para rehabilitar al paciente. Se instalaron prótesis soportadas por implantes (arco inferior) y una dentadura (arco superior) para restablecer la estética y la función de la cavidad bucal de la paciente. Conclusiones: A pesar de ser un tumor benigno, el fibroma cemento-osificante central causó daños funcionales y estéticos al paciente y requirió un complejo tratamiento de rehabilitación. Después de 5 años de la extirpación del tumor, no hubo recurrencia de la lesión. Además, no hubo signos de enfermedades periimplantarias y/o periodontales, lo que demuestra el éxito del tratamiento(AU)

Delayed Diagnosis of Gorlin-Goltz Syndrome: The Importance of the Multidisciplinary Approach.

Gorlin-Goltz syndrome (GGS), also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder with high penetrance and variable expressivity. The classic triad originally described by Gorlin and Goltz in 1960 is composed of multiple nevoid basal cell carcinomas (NBCCs), odontogenic keratocysts (OKCs) in the jaws and bifid ribs. in 1977, this triad was modified by Rayner et al, and to GGS diagnosis, the OKCs had to appear in combination with calcification of the cerebellar falx or palmar and plantar pits. It may occur that although GGS syndrome is a well-known condition, only the specific symptom could be observed by different specialists. Therefore, the patient cannot be placed in an always complex clinical panel. The authors introduce an example in this report. In the present case, the patient had NBCCs, OKCs, and probably other signs of GGS since 1998, and has been treated for this conditions separated, without a diagnosis of a syndromic condition. A 54-year-old white woman was referred to the oral medicine service due to cyst located in the right mandibular body. She had history of skin cancer and undergone surgeries and radiotherapies for the lesions treatment, scars on the skin face due to the lesions removed, and a new ulcerated lesion on the back of was diagnosed. In addition, the patient presented frontal and parietal bossing leading to increased cranial circumference, hypertelorism, strabismus, broad base, and mandibular prognathism. To the image examination, skull radiography revealed calcification of the falx cerebri; on chest X-ray bifid rib was observed and spine radiography showed vertebral osteophytes. Panoramic radiograph showed a well-defined bilocular radiolucent image located in posterior and anterior mandibular region. The whole elements induced us to investigate the patient's past medical history, which revealed that since 1998 had the diagnosis of NBCC and OKC. A multidisciplinary approach becomes necessary for the diagnosis and follow-up of patients with GGS, considering the complexity of the clinical manifestations. Therefore, it is of primary importance for dental surgeons and dermatologists to know the signs and symptoms of GGS to perform early diagnosis and to avoid progression of the oral cysts or metastasis of the skin lesions.

Diagnostic accuracy of the Spanish version of the Pediatric Sleep Questionnaire for screening of obstructive sleep apnea in habitually snoring children.

OBJECTIVES: We aimed to determine the diagnostic test accuracy of the Spanish version of the respiratory symptoms scale of the Pediatric Sleep Questionnaire (PSQ) in habitually snoring children for identifying obstructive sleep apnea (OSA). METHODS: Habitually snoring children referred for polysomnography (PSG) were recruited. Parents answered the PSQ prior to PSG. Based on an apnea-hypopnea index (AHI) >1.0 in PSG, children were divided into OSA and primary snorers. Correlations to PSG indices and diagnostic test accuracy measures were calculated. RESULTS: Of the 83 (n = 53 males, mean age 9.5 ± 3.6 years) habitually snoring children included, 35 had OSA. The previously validated PSQ cutoff value of 0.33 showed a specificity of 0.72 and sensitivity of 0.78. The PSQ score correlated significantly with the AHI rs = 0.313 (p-value = 0.004). Six items of the PSQ were significantly different between cases and controls. A subscale constructed on these six PSQ items concerning respiratory symptoms showed a good sensitivity (0.886) and an excellent negative likelihood ratio (0.261). PSQ was able to identify 89% of the children with OSA correctly. CONCLUSIONS: This version of the PSQ was able to identify children with OSA, separating them from those with primary snoring. The use of this simple, standardized questionnaire tool seems to be helpful and may improve clinical decision making in habitually snoring children.

Perfil clínico de patología neuromuscular hereditaria en el Instituto de Rehabilitación Infantil Teletón, Valparaíso, Chile / Clinical profile of patients with neuromoscular hereditary diseases in Child Rehabilitation Institute Teletón, Valparaiso, Chile

Introducción: Las enfermedades neuromusculares (ENM) son una causa importante de discapacidad progresiva en el niño. Objetivo: Describir el perfil clínico de las consultas por ENM hereditarias, atendidas actualmente en Instituto de Rehabilitación Infantil Teletón (IRI), Valparaíso. Pacientes y Método: estudio descriptivo, retrospectivo. Selección y análisis de pacientes con ENM en control activo, del registro estadístico de IRI Valparaíso. Resultados: Total 115 pacientes, hombres 70 por ciento. Edad promedio 14,9 años (rango: 1-28 a). Motivo de consulta más frecuente: trastorno de la marcha (49,5 por ciento). Las etiologías encontradas fueron: muscular (67 por ciento), neuropatías (21 por ciento) y enfermedad de motoneurona (10 por ciento). Los diagnósticos más frecuentes fueron: Distrofinopatías 30 por ciento, Charcot Marie Tooth 21,7 por ciento, Miopatías Congénitas 15,6 por ciento, Atrofia Muscular Espinal 10 por ciento, Distrofia Miotónica 7,8 por ciento. Discusión: El sexo masculino fue más prevalente lo que puede atribuirse a la mayor frecuencia de Distrofinopatías dentro de las ENM. La latencia para el diagnóstico es variable según la patología, siendo en promedio 3,2 años. Las frecuencias de diagnósticos encontrados coinciden parcialmente con la epidemiología descrita.

Introduction: Neuromuscular diseases (NMD) are a major cause of progressive disability in children. Objective: To describe the clinical profile of hereditary NMD consultations, currently being attended in IRI Valparaíso. Patients and Method: Selection and analysis of actually attending NMD patients from the IRI statistical registration. Results: 115 patients were identified, 70 percent men. Mean age 14.9 years (1-28). The most frequent cause for consultation was gait disorder (49.5 percent. Etiologies were: muscular (67 percent), neuropathy (21 percent) and motor neuron disease (10 percent). The most common diagnoses were: dystrophinopathies (30 percent), Charcot Marie Tooth 21.7 percent, Congenital Myopathy (15.6 percent), Spinal Muscular Atrophy (10 percent), Myotonic Dystrophy (7.8 percent). Discussion: Prevalence was higher for males, which is attributed to the higher frequency of dystrophinopathies. Time for diagnosis was variable depending on the disease, with a mean of 3,2 years. The frequency of NMD were partially coincidental with previously reported epidemiologic data.

Epilepsia asociada a tumores del sistema nervioso central en niños / Epilepsy associated with central nervous system tumors in children

Introducción: La prevalencia de epilepsia en pacientes con tumores del sistema nervioso central (SNC), producto del tumor per se o secundaria al tratamiento, es mayor que en la población general. El objetivo de este estudio es analizar la frecuencia y características de la epilepsia en pacientes pediátricos con tumores del SNC. Método: Estudio descriptivo retrospectivo, realizado a través de la revisión de fichas médicas de los pacientes pediátricos con tumores de SNC entre los años 2001- 2010 en Hospital Carlos Van Buren de Valparaíso. Resultados: Revisados 97 casos pediátricos de tumores del SNC, dieciocho (18,5 por ciento eran portadores de epilepsia, 2/3 sexo masculino, promedio de edad al diagnóstico del tumor fue 7 años y de primera crisis epiléptica 6 años 7 meses. Un 61 por ciento debutó con crisis epilépticas previo al diagnóstico de tumor. Dieciseis de 18 tumores (88 por ciento) fueron supratentoriales, comprometiendo principalmente el lóbulo temporal (9 de 16). Un 83 por ciento fueron neuroepiteliales, los más frecuentes fueron astrocitomas (50 por ciento). Dieciseis casos (88 por ciento) fueron sometidos a intervención quirúrgica. En relación a la epilepsia, 73 por ciento presentó crisis parciales complejas y 38 por ciento (6 casos de 16) evolucionó con epilepsia refractaria durante el seguimiento. Hubo 3 casos que fallecieron. Conclusión: Las crisis epilépticas, sobre todo las crisis focales fueron una manifestación frecuente en este grupo de pacientes pediátricos con tumores del SNC, especialmente en aquellos de localización supratentorial, ya sea como manifestación inicial y clave para el diagnóstico de tumor o durante su evolución. Un 38 por ciento evolucionó como epilepsia refractaria. El número de intervenciones quirúrgicas y la localización tumoral incidieron en la evolución de la epilepsia. Se enfatiza la importancia de una evaluación acuciosa y búsqueda etiológica, en niños que debutan con crisis epilépticas.

Introduction: Patients with brain tumors, show a higher prevalence of epilepsy than the general population, because of the tumor itself or as a consequence of treatment. The aim of this study is to analyze the incidence and characteristics of epilepsy in patients with brain tumors. Method: Retrospective descriptive study, medical records of pediatric patients with brain tumors between the years 2001-2010 from Hospital Carlos Van Buren were reviewed. Results: From 97 patients with brain tumors, 18 (18.5 percent) presented with epilepsy. Two thirds were males. Mean age for brain tumor diagnosis was 7 years, and for first epileptic seizure 6 years 7 months. In 61 percent epileptic seizures started previous to the tumor diagnosis. Sixteen out of 18 patients (88 percent) had supratentorial, mainly temporal tumors (9/16). 83 percent were neuroepithelial, from which astrocytomas were the most frequent (50 percent). Sixteen patients had surgical treatment (88 percent). Epileptic seizures were complex partial in 73 percent. 38 percent evolved to refractory epilepsy in an average of 5 year follow-up. Discussion: Epileptic seizures, mainly complex partial seizures, were a frequent manifestation of patients with brain tumors, specially supratentorial, as the initial event or in follow up. Thirty eight per cent evolved to refractory epilepsy. Number of surgical interventions and localization of the tumor affected the evolution of epileptic seizures. The relevance of searching etiology in children who have a first epileptic seizure is emphazised.

Pronóstico del cáncer cérvico uterino estadios IIB-IIIB tratados con radio quimioterapia: experiencia Hospital Van Buren, Valparaíso, Chile / Prognosis of stage IIB-IIIB cervical cancer treated with radiochemotherapy: experience from the Van Buren Hospital, Valparaíso, Chile

Objetivo: analizar retrospectivamente resultados y factores pronósticos de pacientes con cáncer cérvico uterino estadios IIB-IIIB tratados con radioquimioterapia concomitante.Materiales y métodos: desde septiembre 1999 a diciembre del 2002, 164 pacientes ingresaron con el diagnóstico de cáncer de cérvix, seleccionándose 83 en estadios IIB-IIIB, las que fueron tratadas con radioterapia y cisplatino semanal por cinco semanas.Resultados: el 89,15 por ciento completó cinco ciclos de quimioterapia. La mediana de sobrevida global fue de 41 meses, con una sobrevida global (SG), sobrevida libre de enfermedad (SLE) y sobreviva libre de recurrencia loco-regional (SLRLR) a tres años de 68,8 por ciento, 66,3 por ciento y 83,1 por ciento, respectivamente. Factores pronósticos de sobrevida fueron etapa IIIB, diámetro tumoral clínico ≥ 7 cm, no realización de braquiterapia y enfermedad biparametrial.Conclusiones: el tratamiento con radioterapia y quimioterapia concomitante con cisplatino en pacientes con cáncer cervicouterino estadios IIB-IIIB, es posible de realizar en el Hospital Carlos Van Buren perteneciente al sistema de salud público chileno, con buena tolerancia, resultados y factores pronósticos similares a los de la literatura internacional.