Cleft lip and palate surgery simulator: Open source simulation model.

Objective: Cleft lip and palate is the most common craniofacial birth anomaly and requires surgery in the first year of life. However, craniofacial surgery training opportunities are limited. The aim of this study was to present and evaluate an open-source cleft lip and palate hybrid (casting and three-dimensional (3D) printing) simulation model which can be replicated at low cost to facilitate the teaching and training of cleft surgery anatomy and techniques. Design: The soft tissue component of the cleft surgery training model was casted using a 3D printed 5-component mold and silicone. The bony structure was designed to simulate the facial anatomy and to hold the silicone soft tissue. Setting: Two groups, one group of trainees and one group of expert surgeons, at University Hospital Basel in Switzerland and Pontifical Catholic University of Chile in Santiago, Chile, tested the cleft lip and palate simulation model. Participants completed a Likert-based face and content validity questionnaire to assess the realism of the model and its usefulness in surgical training. Results: More than 70 % of the participants agreed that the model accurately simulated human tissues found in patients with unilateral cleft lip and palate. Over 60 % of the participants also agreed that the model realistically replicated surgical procedures. In addition, 80-90 % of the participants found the model to be a useful and appropriate tool for teaching the anatomy and surgical techniques involved in performing unilateral cleft lip and palate repair. Conclusion: This open-source protocol provides a cost-effective solution for surgeons to introduce the cleft morphology and surgical techniques to trainees on a regular basis. It addresses the current financial barrier that limits access to commercially available models during the early stages of surgeon training prior to specialization in the field.

Perfil epidemiológico de neoplasias epiteliales de glándulas salivales / Epithelial tumors of salivary glands: review of 286 pathology reports

Background:: Epithelial tumors of the salivary glands, including benign tumors and aggressive malignancies with different prognoses, are uncommon. Aim: To describe the frequency and distribution of salivary gland tumors according to age, gender and anatomical location. Material and Methods: Review of pathological reports of salivary gland tumors of a Pathology laboratory at a clinical hospital from 2006 to 2016. Results: Five hundred ninety salivary gland biopsies were reviewed. Of these, 286 (49%) were primary epithelial tumors of the salivary glands. Two hundred thirty (80%) were benign and 56 (20%) were malignant tumors. Regarding location, 274 (96%) were in the major salivary glands, and 12 (4%) in the minor salivary glands. The most common histological types were pleomorphic adenoma for benign tumors in 172 cases, followed by papillary cystadenoma lymphomatosum in 33 cases. Mucoepidermoid carcinoma was the most common malignant tumor in 14 cases. Conclusions: These results are similar to reports from abroad, however more studies are necessary to be able to establish a more representative and updated analysis.

Oral self-mutilation in Lesch-Nyhan Syndrome. Case Report. / Síndrome de Lesch-Nyhan y automutilación oral. Reporte de un caso.

INTRODUCTION: Lesch-Nyhan syndrome (LNS) is an inherited recessive X-related disorder caused by the deficiency of the enzyme hypoxanthin-guanine phosphorribosyl transferase (HPRT). Compul sive self-mutilation and dystonia occurs before the first year of age and is expressed by persistent bites on the oral mucosa, lips, tongue, fingers, and shoulders. The dental intervention performed on most of these patients is multiple tooth extraction to prevent serious secondary lesions. OBJECTIVE: To present a clinical case of LNS and describe pediatric dentistry management in patients with self-mutilating behavior. CLINICAL CASE: Male patient, 7 years old, LNS carrier. He was referred to the Dental Unit from the Department of Pediatric Neurology for evaluation and management of self-inflicted wounds on fingers, lips and cheeks associated with weight loss and decreased food intake. The surgical procedure consisted of multiple extractions, surgical remodeling of the residual alveolar ridges under general anesthesia. In the second postoperative month, the patient was discharged definitively, with an adequate nutritional status and no signs of self-mutilation in hands or oral cavity. CONCLUSIONS: Although LNS is rare, it is essential to know how to proceed in order to provide the best quality of life for patients and their families. Early tooth extractions, as an initial phase in severe cases, seem to be the most useful alternative to minimize damage.

Síndrome de Lesch-Nyhan y automutilación oral. Reporte de un caso / Oral self-mutilation in Lesch-Nyhan Syndrome. Case Report

Resumen: Introducción: El síndrome de Lesch-Nyhan (SLN) es un trastorno hereditario recesivo relacionado con el cromosoma X, causado por la deficiencia de la enzima hipoxantina-guanina fosforribosil transferasa (HPRT). La automutilación compulsiva y distonía ocurre antes del año de edad y se expresa con mordeduras persistentes en la mucosa oral, labios, lengua, dedos y hombros. La intervención odontológica realizada en la mayoría de estos pacientes es la extracción dental múltiple para prevenir lesiones graves secundarias. Objetivo: presentar un caso clínico de SLN y describir el manejo odonto-pediátrico en pacientes con conducta automutilatoria. Caso clínico: Paciente varón, 7 años de edad, portador de SLN. Fue referido a la Unidad de Odontología desde el Departamento de Neurología Pediátrica para la evaluación y manejo de heridas autoinfligidas en dedos, labios y mejillas asociadas a una pérdida de peso y disminución de la ingesta de alimentos. El procedimiento quirúrgico consistió en extracciones dentales múltiples, y remodelación quirúrgica de las crestas alveolares residuales, bajo anestesia general. Al segundo mes posquirúrgico el paciente fue dado de alta definitivamente, con un adecuado estado nutricional y sin signos de automutilación en manos ni en cavidad oral. Conclusio nes: A pesar, que el SLN es infrecuente, es esencial saber cómo proceder para dar la mejor calidad de vida a los pacientes y sus familias. Las extracciones tempranas del diente, como fase inicial en casos severos, parecen ser la alternativa más útil para minimizar el daño y el dolor por la automutilación.

Abstract: Introduction: Lesch-Nyhan syndrome (LNS) is an inherited recessive X-related disorder caused by the deficiency of the enzyme hypoxanthin-guanine phosphorribosyl transferase (HPRT). Compul sive self-mutilation and dystonia occurs before the first year of age and is expressed by persistent bites on the oral mucosa, lips, tongue, fingers, and shoulders. The dental intervention performed on most of these patients is multiple tooth extraction to prevent serious secondary lesions. Objective: To present a clinical case of LNS and describe pediatric dentistry management in patients with self-mutilating behavior. Clinical case: Male patient, 7 years old, LNS carrier. He was referred to the Dental Unit from the Department of Pediatric Neurology for evaluation and management of self-inflicted wounds on fingers, lips and cheeks associated with weight loss and decreased food intake. The surgical procedure consisted of multiple extractions, surgical remodeling of the residual alveolar ridges under general anesthesia. In the second postoperative month, the patient was discharged definitively, with an adequate nutritional status and no signs of self-mutilation in hands or oral cavity. Conclusions: Although LNS is rare, it is essential to know how to proceed in order to provide the best quality of life for patients and their families. Early tooth extractions, as an initial phase in severe cases, seem to be the most useful alternative to minimize damage.

[Epithelial tumors of salivary glands. Review of 286 pathology reports]. / Perfil epidemiológico de neoplasias epiteliales de glándulas salivales.

BACKGROUND: Epithelial tumors of the salivary glands, including benign tumors and aggressive malignancies with different prognoses, are uncommon. AIM: To describe the frequency and distribution of salivary gland tumors according to age, gender and anatomical location. MATERIAL AND METHODS: Review of pathological reports of salivary gland tumors of a Pathology laboratory at a clinical hospital from 2006 to 2016. RESULTS: Five hundred ninety salivary gland biopsies were reviewed. Of these, 286 (49%) were primary epithelial tumors of the salivary glands. Two hundred thirty (80%) were benign and 56 (20%) were malignant tumors. Regarding location, 274 (96%) were in the major salivary glands, and 12 (4%) in the minor salivary glands. The most common histological types were pleomorphic adenoma for benign tumors in 172 cases, followed by papillary cystadenoma lymphomatosum in 33 cases. Mucoepidermoid carcinoma was the most common malignant tumor in 14 cases. CONCLUSIONS: These results are similar to reports from abroad, however more studies are necessary to be able to establish a more representative and updated analysis.