Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis / Asociación de hiperostosis esquelética idiopática difusa y esclerosis valvular aórtica

The principal objective of this investigation was to analyze the association between diffuse idiopathic skeletal hyperostosis (DISH) and the presence of aortic valve sclerosis (AVS). For this study we used results from 1000 consecutive outpatients (473 males), older than 50 years of age (average 67.6 years), that had been examined with Doppler echocardiogram and anterior and lateral chest radiographs. Overall, 195 patients (19.5%) were diagnosed with DISH and 283 (28.3%) with AVS. DISH was more prevalent than AVS in males (66.7% vs. 42.6%, p< 0.0001) and in older patients (73.6 ± 9 years vs. 66.1 ± 9 years, p < 0.0001). Furthermore, 55.4% of patients with dorsal DISH presented aortic sclerosis calcification vs. 21.7% of patients free of DISH (OR = 4.47; 95% CI = 3.22-6.21). The adjusted odds ratio (OR) was calculated by sex and age resulting in 3.04 (95% CI = 2.12-4.36; p < .0001). A statistically significant association was found between DISH and AVS in accordance to age and sex. The biological plausibility of this association is based on similar risk factors, pathogenic mechanisms and vascular complications.

El objetivo principal fue analizar la asociación entre la hiperostosis esquelética idiopática difusa (DISH) y la presencia de esclerosis valvular aórtica (AVS). Se evaluaron los resultados de 1000 pacientes ambulatorios consecutivos (473 varones), mayores de 50 años (promedio, 67.6 años), que habían sido examinados con un ecocardiograma Doppler y radiología torácica anterior y lateral. Globalmente, 195 pacientes (19.5%) tuvieron diagnóstico de DISH y 283 (28.3%) de AVS. DISH fue más prevalente que AVS en varones (66.7% vs. 42.6%, p < 0.0001) y en pacientes de mayor edad (73.6 ± 9 años vs. 66.1 ± 9 años, p < 0.0001). Además, 55.4% de los pacientes con DISH dorsal presentaron AVS vs. 21.7% de los pacientes sin DISH (OR = 4.47; 95% CI = 3.22-6.21). El odds ratio (OR) ajustado por sexo y edad fue 3.04 (95% CI = 2.12-4.36; p < 0.0001). Se encontró una asociación estadísticamente significativa entre DISH y AVS, que se mantuvo después de ajustar por sexo y edad. La plausibilidad biológica de esta asociación se basa en los factores de riesgo, mecanismos patogénicos y complicaciones vasculares compartidos.

Doença de Forestier e suas implicações otorrinolaringológicas: revisão de literatura / Forestier's disease and its implications in otolaryngology: literature review

Introdução: A Doença de Forestier afeta a coluna vertebral, preferencialmente em homens Coluna vertebral; idosos. Não é rara, mas é frequentemente não reconhecida e pode levar a relevante morbimortalidade. Quando acomete a coluna cervical, pode gerar importantes manifestações otorrino-Transtornos de laringológicas. Objetivo: Destacar os sintomas faringolaríngeos da doença de Forestier. Método: Revisão da literatura nas bases de dados Web of Knowledge, PubMed e SciELO e entre os dez periódicos de maior número de citações na área de otorrinolaringologia e também busca manual por publicações nas listas de referências dos artigos selecionados, principalmente os de cunho histórico. Resultados: A doença não tem etiologia clara. Os sintomas das complicações são mais exuberantes que os da doença propriamente dita. A disfagia é o sintoma cervical mais conhecido. Outros sintomas discutidos são a apneia do sono, globus faríngeo, tosse, disfonia, dispneia, otalgia reflexa e sintomas medulares compressivos. O diagnóstico é efetuado com apropriado estudo radiológico. O tratamento é baseado em estratégia conservadora. Pacientes com disfagia refratária e comprometimento respiratório podem ser submetidos a tratamento cirúrgico. Conclusão: A doença de Forestier deve ser suspeitada em pacientes idosos com os principais sintomas das complicações, os quais são comuns na prática otorrinolaringológica, para o início precoce de acompanhamento multidisciplinar. .

Introduction: Forestier's disease affects the spinal column of primarily elderly men. It is not rare, but it is often undiagnosed and can lead to significant morbidity and mortality. When it affects the cervical spine, it can result in important otorhinolaryngological manifestations. Objective: To analyze the pharyngeal and laryngeal symptoms of the Forestier's disease. Methods: Literature review of the Web of Knowledge, PubMed, and SciELO databases and of the ten most frequently cited journals in the field of otorhinolaryngology. Additionally, a manual search was performed for publications in the reference lists of selected articles, mostly those of a historical nature. Results: The etiology of the disease is still unclear. Symptoms of complications are more significant than the disease itself. Dysphagia is the most common cervical symptom and has several involved mechanisms. Other symptoms are sleep apnea, pharyngeal globus, coughing, dysphonia, dyspnea, otalgia, and medullary compression. The diagnosis is verified by appropriate radiological study. Treatment is based on a conservative strategy. Patients with refractory dysphagia and respiratory impairment can be surgically treated. Conclusion: Forestier's disease should be suspected in elderly patients with the major symptoms of complications, which are common in otorhinolaryngology practice and when identified, a multidisciplinary approach should be instituted as soon as possible. .

Disfagia cervical espondilótica por hiperostosis esquelética difusa idiopática en un paciente joven / Diffuse idiopathic skeletal hyperostosis causing dysphagia in a young patient

Diffuse idiopathic skeletal hyperostosis (DISH) is an under-diagnosed condition producing flowing ossification of the antero-lateral ligaments of the spine. Affecting predominantly males over 65years old, it is an unusual cause of dysphagia and dysphonia. We report a 45-year-old mole with a three years history of dysphonia and three months of dysphagia. The initial diagnosis was gastroesophageal reflux, and an endoscopy ruled out esophageal luminal pathology. Cervical spine radiographs showed ossification of the cervical anterior longitudinal ligament with large, prominent osteophytes from C3 to C6, producing esophageal and upper airway compression; these images were compatible with DISH. Cervical osteophyte resection resulted in complete resolution of symptoms. DISH should be considered in the differential diagnosis of dysphagia and dysphonia.

Hiperostosis esquelética idiopática difusa (DISH), respecto de dos casos / Diffuse idiopathic skeletal hiperostosis, respect of two cases

La hiperostosis esquelética idiopática difusa (DISH) o enfermedad de Forestier es una patología que presenta como principal manifestación la osificación del ligamento longitudinal anterior (LLA) y la formación de puentes óseos intervertebrales. Las repercusiones otorrinolaringológicas con poca frecuencia son descritas, siendo la disfagia, la disfonía y la disnea los síntomas más comunes. Presentamos dos casos clínicos de DISH en pacientes de sexo masculino de setenta y cuatro años y otro de setenta y un años respectivamente, con disfagia de varias semanas de evolución asociado a disfonía y disnea en un caso. Realizamos la discusión de ambos casos y de las manifestaciones otorrinolaringológicas de esta patología.

Diffuse idiopathic skeletal hiperostosis (DISH) or Forester's disease is a pathology characterized by the ossification of the anterior longitudinal ligament (ALL) and the formation of intervertebral osseous bridges. The otolaryngology repercussions are rarely described, dysphagia, hoarseness and dyspnea being the most common symptoms. We present a clinical case of two patients with DISH in a 74 year-old male patient and another of 71 year-old respectively, suffering from several weeks of dysphagia associated with dysphonia and dyspnea in one case. We are going to discuss both cases together with the otolaryngological manifestations of this pathology.

Diffuse interstitial skeletal hyperostosis (DISH) in type 2 diabetes.

A 58 years type 2 diabetic woman, school teacher by profession, presented with backache, neck pain and generalised weakness since last few months. Pain was mild with stiffness and neck pain was particularly associated with extension of the neck towards back. There was no focal neurological deficit on central nervous system examination. X-ray of lumbo- sacral spine showed prolific osteophytes and new bone formation in the body of lumbar vertebrae. Cervical X-ray showed 'Melting candle-wax' appearance at the anterior to the cervical vertebrae. In view of clinical and radiological association the case was diagnosed as DISH syndrome. It is being presented for its rarity.

Quadriplegia complicating ossification of the posterior longitudinal ligament.

We report a case of quadriplegia complicating ossification of posterior longitudinal ligament (OPLL) in a patient who was also found to have diffuse idiopathic skeletal hyperostosis (DISH). She also had osteomalacia (Vit. D deficiency) with secondary hyperparathyroidism. There could be a cause and effect relationship between the abnormal biochemistry and OPLL.

Spinal cord and cauda equina compression in 'DISH'.

Diffuse idiopathic skeletal hyperostosis (DISH) has long been regarded as a benign asymptomatic clinical entity with an innocuous clinical course. Precise information is lacking in the world literature. Authors report the results of a retrospective analysis of 74 cases of DISH. Eleven patients presented with progressive spinal cord or cauda equina compression. In nine cases ossified posterior longitudinal ligament (OPLL) and in two cases ossified ligamentum flavum (OLF) were primarily responsible. Surgically treated patients (eight) had far better outcome as compared to the patients managed conservatively, as they had refused surgery. 'DISH' is neither a benign condition, nor it always runs a innocuous clinical course. In fact, in about 15% of the cases, serious neurological manifestations occur, which may require a major neurosurgical intervention.

Disfagia por enfermedad de Forestier. Algunas consideraciones a propósito de un caso / Dysphagia caused by Forestier disease: apropos of a case

Un osteofito cervical puede producir sintomatología por compresión faríngea, semejante a la exploración de un tumor de hipofaringe. Presentamos un caso de disfagia por enfermedad de Forestier (hiperostosis de columna con osteofitosis anterior). El tránsito baritado del esófago demostró estenosis faríngea producida por el osteofito. El estudio radiológico de la columna cervical nos brindó diagnóstico

Hiperostosis perióstica idiopática transitoria con disproteinemia, síndrome de Goldbloom / Transient idiopathic periosteal hyperostosis with dysproteinemia, Goldbloom's syndrome

Se presenta el caso de una niña de 13 años, que luego de una infección respiratoria alta aguda evolucionó con dolor intenso en las extremidades y compromiso importante de la nutrición. El estudio radiológico reveló reacción perióstica difusa, más intensa a nivel de los huesos largos. En el laboratorio destacaba anemia normocítica normocrómica discreta, eritrosedimentación elevada, plasmocitosis medular y disproteinemia, con gran aumento de las gammaglobulinas. Tanto el cuadro clínico como los hallazgos radiológicos y de laboratorio regresaron sin mediar tratamiento alguno, observándose normalización completa a los 11 meses desde el episodio agudo. El caso clínico presentado corresponde al síndrome de Goldbloom, descrito originalmente en 2 niños en 1966, existiendo sólo otros 3 casos publicados en la literatura