RESUMEN
PATIENTS: Singleton-Merten syndrome is an extremely rare autosomal dominant condition with less than 10 reported cases in the literature. It is characterized by abnormal aortic calcifications and dental abnormalities. The goal of this case report is to discuss the abnormal oral clinical features and the modified treatment protocol that was used in order to achieve osseointegration of dental implants in a patient having abnormal bone density and bone turnover associated with Singleton-Merten Syndrome. DISCUSSION: Following extraction of the remaining teeth, titanium implants (Friadent GmbH, Mannheim, Germany and Straumann(®), Basel, Switzerland) were placed in the upper and lower jaw of the patient. The upper jaw which was treated with dental implants, received a bar supported implant retained prosthesis and the lower jaw an implant retained telescopic prosthesis. The patient was regularly followed up for the past 13 years during which, clinical and radiological evaluation of osseointegration was undertaken. All the loaded implants showed clinical and radiographic evidence of osseointegration. With a follow up of 13 years after insertion of the first implant, the patient reported functioning well with no complications. CONCLUSION: The treatment with dental implants in the extremely rare Singleton-Merten syndrome patients is a reasonable treatment option to rehabilitate maxillofacial aesthetics and establish normal function of the jaws.
Asunto(s)
Enfermedades de la Aorta/cirugía , Hipoplasia del Esmalte Dental/cirugía , Implantación Dental Endoósea/métodos , Metacarpo/anomalías , Enfermedades Musculares/cirugía , Odontodisplasia/cirugía , Osteoporosis/cirugía , Calcificación Vascular/cirugía , Adolescente , Enfermedades de la Aorta/metabolismo , Enfermedades de la Aorta/fisiopatología , Enfermedades de la Aorta/rehabilitación , Densidad Ósea , Remodelación Ósea , Hipoplasia del Esmalte Dental/metabolismo , Hipoplasia del Esmalte Dental/fisiopatología , Hipoplasia del Esmalte Dental/rehabilitación , Estética Dental , Estudios de Seguimiento , Humanos , Masculino , Metacarpo/metabolismo , Metacarpo/fisiopatología , Metacarpo/cirugía , Enfermedades Musculares/metabolismo , Enfermedades Musculares/fisiopatología , Enfermedades Musculares/rehabilitación , Odontodisplasia/metabolismo , Odontodisplasia/fisiopatología , Odontodisplasia/rehabilitación , Procedimientos Quirúrgicos Ortognáticos , Oseointegración , Osteoporosis/metabolismo , Osteoporosis/fisiopatología , Osteoporosis/rehabilitación , Titanio , Calcificación Vascular/metabolismo , Calcificación Vascular/fisiopatología , Calcificación Vascular/rehabilitaciónAsunto(s)
Enfermedades de la Aorta/cirugía , Ingestión de Alimentos/fisiología , Fístula Esofágica/cirugía , Migración de Cuerpo Extraño/cirugía , Anciano , Enfermedades de la Aorta/diagnóstico por imagen , Enfermedades de la Aorta/etiología , Enfermedades de la Aorta/rehabilitación , Huesos , Urgencias Médicas , Endoscopía del Sistema Digestivo , Fístula Esofágica/diagnóstico por imagen , Fístula Esofágica/etiología , Fístula Esofágica/rehabilitación , Femenino , Migración de Cuerpo Extraño/complicaciones , Migración de Cuerpo Extraño/diagnóstico por imagen , Humanos , Radiografía , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVES: Endovascular repair of the descending thoracic aorta is a very promising technique in elective and, particularly, emergency situations. This study assessed the impact of urgency of the procedure on outcome and mid-term quality of life in surviving patients. METHODS: Post hoc analysis of prospectively collected data of 58 consecutive patients (January 2001-December 2005) with surgical pathologies of the descending thoracic aorta treated by endovascular means. Six patients were excluded due to recent operations on the ascending aorta before thoracic endovascular repair. The remaining patients (n=52) were 69+/-10 years old, and 43 were men (83%). Twenty-seven had been treated electively, and 25 for emergency indications. Reasons for emergency were acute type B aortic dissections with or without malperfusion syndrome in 14, and aortic ruptures in 11 cases. Follow-up was 29+/-16 months. Endpoints were perioperative and late morbidity and mortality rates and long-term quality of life as assessed by the short form health survey (SF-36) and Hospital Anxiety and Depression Scale questionnaires. RESULTS: Cohorts were comparable regarding age, sex, cardiovascular risk factors, and comorbidities. Perioperative mortality was somewhat higher in emergency cases (12% vs 4%, p=0.34). Paraplegia occurred in one patient in each cohort (4%). Overall quality of life after two and a half years was similar in both treatment cohorts: 72 (58-124) after emergency, and 85 (61-105) after elective endovascular aortic repair (p=0.98). Normal scores range from 85 to 115. Anxiety and depression scores were in the normal range and comparable. CONCLUSIONS: Thoracic endovascular aortic repair is an excellent and safe treatment option for the diseased descending aorta, particularly in emergency situations. Early morbidity and mortality rates can be kept very low. Mid-term quality of life was not affected by the urgency of the procedure. Similarly, mid-term anxiety and depression scores were not increased after emergency situations.