Síndrome de Forester, hiperostosis esquelética idiopática difusa: reporte de un caso de disfagia orofaringea con tratamiento conservador y revisión de literatura / Forestier 's Syndrome, diffuse idiopathic skeletal hiperostosis: report of a case of oropharyngeal dysphagia with conservative treatment and literature review

El síndrome de Forestier es una enfermedad reumatológica, de origen desconocido, que consiste en la calcificación de diferentes zonas del cuerpo, predominantemente a nivel del ligamento vertebral común anterior, donde se forman osteofitos capaces de producir sintomatología variada y de intensidad variable. Normalmente asintomática, aunque, cuando afecta la columna cervical, el síntoma más común es la disfagia. Se presenta un caso de Síndrome de Forestier que consultó por disfagia en la Unidad de Cirugía de Cabeza y Cuello del Servicio de Otorrinolaringología del Instituto de Previsión Social de Asunción, Paraguay. La semiología permitió observar un abombamiento submucoso en la pared posterior de la orofaringe de 1,5 cm de diámetro, que pudo ser evaluado y confirmado por tomografía. Debido a poca intensidad de la sintomatología y escasa repercusión en el estado general se decidió realizar un tratamiento conservador con buenos resultados, tras dos años de seguimiento clínico. En estos casos la cirugía ocupa un lugar secundario, ya sea ante el fracaso de esta conducta conservadora o ante la progresión de los síntomas

Forestier Syndrome is a rheumatological disease of unknown origin, which consist in calcification of different areas of the body, predominantly at the level of the anterior common vertebral ligament, where osteophytes capable of producing varied symptoms of variable intensity are formed. Normally asymptomatic, although, when it affects the cervical spine, the most common symptom is dysphagia.A case of Forestier syndrome is presented who consulted for dysphagia in the Head and Neck Surgery Unit of the Otolaryngology Service of the Institute of Social Prevision, Asuncion, Paraguay. The semiology allowed to observe a submucosal bulge in the posterior wall of the oropharynx of 1,5 cm in diameter, which could be evaluated and confirmed by tomography.Due to the low intensity of the symptoms and little impact on the general state, it was decided to carry out a conservative treatment with good results, after two years of clinical follow-up. In these cases, surgery occupies a secondary place, either before the failure of this conservative behavior or the progression of the symptoms.

Enfermedad de Forestier en paciente mayor de 65 años: reporte de caso con seguimiento a 6 años de manejo conservador / Diffuse idiopathic skeletal hyperostosis (DISH): report of one case

Forestier Disease, or Diffuse Idiopathic Skeletal Hyperostosis (DISH), causes a considerable ossification of the anterior longitudinal ligament of the spine. When it involves cervical segments, it can be accompanied by dysphagia, dysphonia and/or dyspnea. This entity usually has a benign course. Surgical treatment is required for progressive cases. We report a 72-year-old male with a history of chronic cervical pain and slight neck stiffness. On the imaging studies, there was an exuberant ossification of the anterior longitudinal ligament, at the cervical spine level, between C2 and C7. He was managed conservatively without clinical deterioration in a six-year follow-up.

[Diffuse idiopathic skeletal hyperostosis (DISH). Report of one case]. / Enfermedad de Forestier en paciente mayor de 65 años: reporte de caso con seguimiento a 6 años de manejo conservador.

Forestier Disease, or Diffuse Idiopathic Skeletal Hyperostosis (DISH), causes a considerable ossification of the anterior longitudinal ligament of the spine. When it involves cervical segments, it can be accompanied by dysphagia, dysphonia and/or dyspnea. This entity usually has a benign course. Surgical treatment is required for progressive cases. We report a 72-year-old male with a history of chronic cervical pain and slight neck stiffness. On the imaging studies, there was an exuberant ossification of the anterior longitudinal ligament, at the cervical spine level, between C2 and C7. He was managed conservatively without clinical deterioration in a six-year follow-up.

Hiperostose esquelética idiopática difusa canina - relato de caso / Canine diffuse idiopathic skeletal hyperostosis - case report / Hiperostosis esquelética idiopática difusa canina - relato de caso

A hiperosteose esquelética idiopática difusa é uma doença de ocorrência rara, caracterizada pela ossificação maciça progressiva dos tecidos moles que envolvem os esqueletos axial e apendicular, acometendo prevalentemente o ligamento longitudinal ventral. Os aspectos etiológicos e terapêuticos da enfermidade ainda não foram completamente esclarecidos, havendo poucas informações disponíveis. Este trabalho relata o primeiro caso dessa doença em cães na literatura nacional, e tem por objetivo descrever os aspectos clínicos e radiográficos da enfermidade, em uma cadela de sete anos de idade, que apresentou como queixa principal dificuldade deambulatória e alteração postural da coluna toracolombar associada a lombalgia. O diagnóstico foi obtido por meio de radiografia convencional, e o tratamento inicial utilizando um anti-inflamatório COX-2 seletivo demonstrou bons resultados no alívio da dor e no controle dos sinais clínicos em curto prazo.

Diffuse idiopathic skeletal hyperostosis is a rare disease characterized by massive and progressive ossification of soft tissues that surround the axial and appendicular skeletons, affecting more frequently the ventral longitudinal ligament. The etiological and therapeutic aspects of the disease have not yet been completely clarified and there is little information available on the occurrence of the disease in the canine species. This work reports the first case of this disease in the national literature and aims to describe the clinical and radiographic aspects of the condition in a seven-year-old bitch presenting gait abnormalities and thoracolumbar postural alterations associated with lower back pain. Diagnosis was made by conventional radiography and initial treatment with a selective COX-2 anti-inflammatory drug has shown good results in pain relief and short-term control of clinical signs.

La hiperostosis esquelética idiopática difusa es una enfermedad rara, que se caracteriza por la osificación masiva y progresiva de los tejidos blandos que rodean a los esqueletos axial y apendicular, particularmente el ligamento longitudinal ventral. Aun no se han aclarado ciertos aspectos etiológicos y terapéuticos, existiendo poca información relacionada con la enfermedad. Este trabajo relata el primer caso de esta enfermedad en Brasil, y tiene como objetivo describir las características clínicas y radiográficas en una perra de siete anos, que presentaba dificultad para caminar, alteración postural de la columna toracolumbar y lumbalgia. el diagnóstico se obtuvo mediante exámenes radiográficos convencionales, y el tratamiento inicial fue con un antiinflamatorio CO-2 selectivo, con buenos resultados en relación al dolor y control de los signos clínicos en el corto plazo.

Hiperostose esquelética idiopática difusa canina - relato de caso / Canine diffuse idiopathic skeletal hyperostosis - case report / Hiperostosis esquelética idiopática difusa canina - relato de caso

A hiperosteose esquelética idiopática difusa é uma doença de ocorrência rara, caracterizada pela ossificação maciça progressiva dos tecidos moles que envolvem os esqueletos axial e apendicular, acometendo prevalentemente o ligamento longitudinal ventral. Os aspectos etiológicos e terapêuticos da enfermidade ainda não foram completamente esclarecidos, havendo poucas informações disponíveis. Este trabalho relata o primeiro caso dessa doença em cães na literatura nacional, e tem por objetivo descrever os aspectos clínicos e radiográficos da enfermidade, em uma cadela de sete anos de idade, que apresentou como queixa principal dificuldade deambulatória e alteração postural da coluna toracolombar associada a lombalgia. O diagnóstico foi obtido por meio de radiografia convencional, e o tratamento inicial utilizando um anti-inflamatório COX-2 seletivo demonstrou bons resultados no alívio da dor e no controle dos sinais clínicos em curto prazo.(AU)

Diffuse idiopathic skeletal hyperostosis is a rare disease characterized by massive and progressive ossification of soft tissues that surround the axial and appendicular skeletons, affecting more frequently the ventral longitudinal ligament. The etiological and therapeutic aspects of the disease have not yet been completely clarified and there is little information available on the occurrence of the disease in the canine species. This work reports the first case of this disease in the national literature and aims to describe the clinical and radiographic aspects of the condition in a seven-year-old bitch presenting gait abnormalities and thoracolumbar postural alterations associated with lower back pain. Diagnosis was made by conventional radiography and initial treatment with a selective COX-2 anti-inflammatory drug has shown good results in pain relief and short-term control of clinical signs.(AU)

La hiperostosis esquelética idiopática difusa es una enfermedad rara, que se caracteriza por la osificación masiva y progresiva de los tejidos blandos que rodean a los esqueletos axial y apendicular, particularmente el ligamento longitudinal ventral. Aun no se han aclarado ciertos aspectos etiológicos y terapéuticos, existiendo poca información relacionada con la enfermedad. Este trabajo relata el primer caso de esta enfermedad en Brasil, y tiene como objetivo describir las características clínicas y radiográficas en una perra de siete anos, que presentaba dificultad para caminar, alteración postural de la columna toracolumbar y lumbalgia. el diagnóstico se obtuvo mediante exámenes radiográficos convencionales, y el tratamiento inicial fue con un antiinflamatorio CO-2 selectivo, con buenos resultados en relación al dolor y control de los signos clínicos en el corto plazo.(AU)

Diffuse idiopathic skeletal hyperostosis association with thoracic spine kyphosis: a cross-sectional study for the Health Aging and Body Composition Study.

STUDY DESIGN: A descriptive study of the association between diffuse idiopathic skeletal hyperostosis (DISH) and kyphosis. OBJECTIVE: To investigate the association of DISH with Cobb angle of kyphosis in a large cohort of older subjects from the Health Aging and Body Composition Study. SUMMARY OF BACKGROUND DATA: DISH and thoracic kyphosis are well-defined radiographical findings in spines of older individuals. Characteristics of DISH (ossifications between vertebral segments) reflect changes of spine anatomy and physiology that may be associated with Cobb angle of kyphosis. METHODS: Using data from 1172 subjects aged 70 to 79 years, we measured DISH and Cobb angle of kyphosis from computed tomographic lateral scout scans. Characteristics of participants with and without DISH were assessed using the χ² and t tests. Association between DISH and Cobb angle was analyzed using linear regression. Cobb angle and DISH relationship was assessed at different spine levels (thoracic and lumbar). RESULTS: DISH was identified on computed tomographic scout scan in 152 subjects with 101 cases in only the thoracic spine and 51 in both thoracic and lumbar spine segments. The mean Cobb angle of kyphosis in the analytic sample was 31.3° (standard deviation = 11.2). The presence of DISH was associated with a greater Cobb angle of 9.1° and 95% confidence interval (95% CI) (5.6-12.6) among African Americans and a Cobb angle of 2.9° and 95% CI (0.5-5.2) among Caucasians compared with those with no DISH. DISH in the thoracic spine alone was associated with a greater Cobb angle of 10.6° and 95% CI (6.5-14.7) in African Americans and a Cobb angle of 3.8° and 95% CI (1.0-6.5) in Caucasians compared with those with no DISH. CONCLUSION: DISH is associated with greater Cobb angle of kyphosis, especially when present in the thoracic spine alone. The association of DISH with Cobb angle is stronger within the African American population.

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.

Long-term results of surgical treatment of dysphagia secondary to cervical diffuse idiopathic skeletal hyperostosis.

BACKGROUND CONTEXT: Large, prominent osteophytes along the anterior aspect of the cervical spine have been reported as a cause of dysphagia. Improvement of swallowing after surgical resection has been reported in a few case reports with short-term follow-up. The current report describes outcomes of a series of five patients with surgical treatment for this rare disorder, with a long-term follow-up. PURPOSE: To study the clinical and radiographic outcomes of a case series of patients surgically treated for dysphagia secondary to cervical diffuse idiopathic skeletal hyperostosis (DISH). STUDY DESIGN: Retrospective review of a case series. PATIENT SAMPLE: Five cases from a University Hospital. OUTCOME MEASURES: Clinical and imagenological follow-up. METHODS: The records of five patients with dysphagia who had undergone anterior surgical resection of prominent osteophytes secondary to DISH were reviewed. Extrinsic esophageal compression secondary to anterior cervical osteophytes was radiographically confirmed via preoperative barium esophagogram swallowing study. All patients underwent anterior cervical osteophytes resection without fusion. Postoperatively, patients were followed-up clinically and radiographically with routine lateral cervical radiographs. RESULTS: Preoperative esophagogram showed that the esophageal obstruction was present at one level in three cases and two levels in two cases. The C3-C4 level was involved in three cases, C4-C5 in three cases, and C5-C6 in one case. There were no postoperative complications, including recurrent laryngeal nerve palsy, wound infection, or hematomas. All patients had resolution of dyphagia soon after surgery (within 2 weeks). Postoperative radiographs demonstrated complete removal of osteophytes. At final follow-up, ranging from 1 to 9 years (average 59.8 months, median 53 months), no patients reported recurrence of dysphagia. Final radiographic examination demonstrated minimal regrowth of the osteophytes. CONCLUSIONS: Although rarely indicated, surgical resection of anterior cervical osteophytes from DISH causing dyphagia produces good clinical and radiographical outcomes. After thorough evaluation to rule out other intrinsic or extrinsic causes of swallowing difficulty, surgical treatment of this uncommon condition might be considered.

Hiperostosis cortical idiopática con disproteinemia (síndrome de Goldbloom) / Idiopathic periosteal hypeostosis with dysproteinemia (Goldblooms syndrome)

La hiperostosis cortical idiopática con disproteinemia o síndrome de Goldbloom (SG) es un síndrome de escasa frecuencia y de etiología desconocida. Suele comenzar después de la primera infancia como una virosis inespecífica, seguido luego por fiebre, hiperostosis y trastornos en el proteinograma. Comentamos la situación de un niño de 5 años de edad con SG que se presentó como un síndrome febril prolongado (SFP) de 9 meses de evolución y dolores óseos. Al cabo del 5º mes de evolución aparecieron induraciones calientes y profundas en las extremidades proximales de ambas tibias. Presentó hipergamaglobulinemia. Las radiografías mostraron hiperostosis cortical y la centellografía un aumento de la captación del material r

Coexistence of diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis.

To the best of our knowledge, only two patients with concurrent diffuse idiopathic skeletal hyperostosis (DISH) and ankylosing spondylitis (AS) have been reported so far. Here we present 3 patients in whom clinical and radiological findings indicative of DISH and AS coexisted. Two of these cases exhibited HLA B27. Although the presence of sacroiliitis would appear to exclude DISH, calcification and ossification of the anterior common vertebral ligament (ACVL) confirmed diagnosis of the latter disease.

[Transient idiopathic periosteal hyperostosis with hyperproteinemia (Goldbloom syndrome)]. / Hiperostosis perióstica idiopática transitoria con disproteinemia (síndrome de Goldbloom).

The case of a 13 year old girl is presented, who developed intense pain in extremities and experienced marked weight loss after an acute upper respiratory tract infection. Roentgenograms revealed a diffuse periosteal reaction, most intensely affecting long bones. Principal laboratory findings were a slight normocytic, normochromic anemia; high erythrocyte sedimentation rate; medullar plasmocytosis and dysproteinemia, with a large increase in plasma gammaglobulin concentration. Clinical recovery and normalization of roentgenograms and laboratory parameters were observed eleven months after the acute episode, with no intervening therapeutic measures. This case matches Goldbloom's syndrome, described originally in two children in 1966. Only 3 other cases have been reported in the literature.