Enfermedad de Caffey: reporte de un caso clínico / Caffey's disease: report of a clinical case / Doença de Caffey: relato de um caso clínico

Introducción: La enfermedad de Caffey es una patología ósea inflamatoria, rara, autolimitada, casi exclusiva de lactantes. Objetivos: Jerarquizar el abordaje diagnóstico de una patología poco frecuente. Caso clínico: 4 meses 22 días, varón, consulta por irritabilidad y edema de miembro inferior izquierdo de 4 días de evolución. Sin traumatismos ni fiebre. Examen físico: edema indurado en tercio medio e inferior de pierna izquierda, no rubor ni calor local. Dolor a la palpación de cara anterior y lateral de tibia y peroné. Limitación funcional, no resaltos óseos. Radiografía: engrosamiento del periostio en tibia y peroné a nivel diafisario. Hemograma: Glóbulos blancos 15.380 KU/L, Hemoglobina 10,8 g/dL, Plaquetas 816.400 10/ul, proteína C reactiva 13,90 mg/dl. Con planteo de probable infección osteoarticular se inicia clindamicina ( gentamicina e ingresa a cuidados moderados. Dada la persistencia de edema e irritabilidad, al quinto día se solicita resonancia magnética: hallazgos sugerentes de un probable proceso inflamatorio- infeccioso de partes blandas con compromiso óseo. Completa 14 días de clindamicina y 7 días de gentamicina intravenosa, hemocultivo negativo. Persiste con edema, irritabilidad y dolor. A los 21 días, se revalora la presentación clínica-imagenológica, se plantea enfermedad de Caffey. Se inicia anti-inflamatorio con buena evolución. Conclusiones: La enfermedad de Caffey es una colagenopatía rara, que afecta lactantes. El diagnóstico es clínico - radiológico (irritabilidad, tumefacción de partes blandas y alteraciones radiológicas). El pronóstico a largo plazo suele ser favorable. Es importante considerar el diagnóstico en lactantes que se presentan con esta sintomatología para evitar retrasos diagnósticos e instauración de tratamientos innecesarios.

Introduction: Caffey's disease is a rare disease that is reported almost exclusively in infants. Objective: Describe the case of a rare pathology, prioritizing the diagnostic approach. Clinical case: 4 month -old, healthy male. Consultation due to irritability and edema of the left lower limb for 4 days. No trauma or fever. Physical examination: indurated edema in the left leg, no redness or local heat. Pain on palpation of the anterior and lateral aspect of the tibia and fibula. Functional limitation, no bony protusions. Leg x-ray: thickening of the periosteum in the tibia and fibula at the diaphyseal level. Hemogram: White Blood Cells 15,380 KU/L Hemoglobin: 10.8 g/dL. Platelets: 816,400 10/ul, C-reactive protein: 13.90 mg/dl. He was admitted with a suggestion of probable osteoarticular infection. Clindamycin ( gentamicin is started. Given the persistence of edema and irritability despite treatment, on the fifth day an MRI was requested: findings suggestive of a probable inflammatory-infectious process of soft tissues with bone involvement. Completed 14 days of clindamycin and 7 days of intravenous gentamicin, blood culture negative. It persists with edema, irritability and pain. After 21 days, the clinical-imaging presentation was reassessed and Caffey's disease was considered. Anti-inflammatory begins with good evolution. Conclusions: Caffey's disease is a rare collagenopathy, that affects infants. The diagnosis is clinical - radiological (irritability, soft tissue swelling and radiological alterations). The long-term prognosis is usually favorable. It is important to consider the diagnosis in infants who present with these symptoms to avoid diagnostic delays and initiation of unnecessary treatments.

Introdução: A doença de Caffey é uma patologia óssea inflamatória rara, autolimitada, quase exclusiva de lactentes. Objetivos: Priorizar a abordagem diagnóstica de uma patologia rara. Caso clínico: 4 meses 22 dias, sexo masculino, consulta por irritabilidade e edema do membro inferior esquerdo de 4 dias de evolução. Sem trauma ou febre. Exame físico: edema endurecido em terço médio e inferior da perna esquerda, sem vermelhidão ou calor local. Dor à palpação das faces anterior e lateral da tíbia e fíbula. Limitação funcional, sem saliências ósseas. Radiografia: espessamento do periósteo na tíbia e fíbula ao nível diafisário. Hemograma: Glóbulos brancos 15.380 KU/L, Hemoglobina 10,8 g/dL, Plaquetas 816.400 10/ul, Proteína C reativa 13,90 mg/dl. Com sugestão de provável infecção osteoarticular, foi iniciada clindamicina + gentamicina e internado em cuidados moderados. Dada a persistência do edema e da irritabilidade, no quinto dia foi solicitada ressonância magnética: achados sugestivos de provável processo inflamatório-infeccioso de partes moles com envolvimento ósseo. Completou 14 dias de clindamicina e 7 dias de gentamicina intravenosa, hemocultura negativa. Persiste com edema, irritabilidade e dor. Após 21 dias, o quadro clínico-imagem foi reavaliado e considerada doença de Caffey. O antiinflamatório começa com uma boa evolução. Conclusões: A doença de Caffey é uma colagenopatia rara que afeta lactentes. O diagnóstico é clínico-radiológico (irritabilidade, edema de partes moles e alterações radiológicas). O prognóstico a longo prazo é geralmente favorável. É importante considerar o diagnóstico em lactentes que apresentam esses sintomas para evitar atrasos no diagnóstico e início de tratamentos desnecessários.

Progress in the treatment of diffuse idiopathic skeletal hyperostosis combined with thoracolumbar fracture / 国际外科学杂志

Diffuse idiopathic skeletal hyperostosis(DISH) is a kind of ankylosing spinal disease, which usually occurs in the middle-aged and elderly. It is mainly manifested as ossification and calcification of ligaments and tendon attachment points. It is a bone disease that mainly involves the spine but is not accompanied by severe intervertebral disc degeneration or sacroiliac joint and intervertebral facet joint ankylosis. DISH combined with thoracolumbar fracture has a high rate of delayed diagnosis and neurological deterioration, so this kind of fracture should be diagnosed and treated in time. However, there are different conclusions on the choice of treatment methods and postoperative efficacy, and there are still disputes in some academic aspects.The author consulted the relevant literature and reviewed the treatment methods of DISH combined with thoracolumbar fracture in order to provide reference for the clinical treatment of this kind of fracture.

Diagnóstico de un síndrome de Morgagni-Stewart-Morel en una paciente con ictus isquémico. Reporte de un caso

RESUMEN El síndrome de Morgagni-Stewart-Morel es una rara enfermedad que se caracteriza por hiperostosis frontal interna bilateral asociada a alteraciones metabólicas, psiquiátricas, hipertensión arterial y disfunción de pares craneales de etiología no definida. Se presenta el caso de una paciente de 77 años con historia de diabetes mellitus tipo 2, hipertensión arterial sistémica y trastorno psiquiátrico, que ingresó en el Servicio de Neurología del Hospital General Universitario Dr. Gustavo Aldereguía Lima de Cienfuegos con cuadro de disartria y hemiparesia derecha. Se realizaron estudios de diagnóstico por imagen de cráneo donde se observó la presencia de hiperostosis frontal interna extensa, atrofia cortical y un infarto talámico izquierdo. Durante su estancia hospitalaria se documentó la presencia de obesidad grado I, hirsutismo e hipercolesterolemia. La paciente cumplió con los criterios del síndrome de Morgagni-Stewart-Morel al manifestar la presencia de hiperostosis frontal interna con manifestaciones, endocrinológicas y neuropsiquiátricas. Se presenta el caso con el objetivo de evidenciar el diagnóstico de una enfermedad de baja incidencia en pacientes cerebrovasculares isquémicos con la presencia de una sintomatología que incluye varios factores de riesgo vasculares bien documentados.

ABSTRACT Morgagni-Stewart-Morel syndrome is a rare disease characterized by bilateral internal frontal hyperostosis associated with metabolic and psychiatric disorders, arterial hypertension, and dysfunction of the cranial nerves of undefined etiology. The case of a 77-year-old patient with a history of type 2 diabetes mellitus, systemic arterial hypertension and psychiatric disorder, who was admitted to the Neurology Service of the Dr. Gustavo Aldereguía Lima University Hospital in Cienfuegos with dysarthria and right hemiparesis is presented. Diagnostic skull imaging studies were performed where the presence of extensive internal frontal hyperostosis, cortical atrophy and a left thalamic infarction was observed. During his hospital stay, the presence of grade I obesity, hirsutism and hypercholesterolemia was documented. The patient met the criteria for Morgagni-Stewart-Morel syndrome by manifesting the presence of internal frontal hyperostosis with endocrinological and neuropsychiatric manifestations. The case is presented in order to demonstrate the diagnosis of a low incidence disease in ischemic cerebrovascular patients with the presence of symptoms that include several well-documented vascular risk factors.

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.

Estudio anatómico de la hiperostosis porótica en órbitas oculares: cribra orbitalia en la población arqueológica de Tutuquén, región del Maule, Chile / Anatomical study of porotic hyperostosis in the eye orbits: cribra orbitalia in the archaeological population of Tutuquén, Maule region, Chile

RESUMEN: La cribra orbitalia o hiperostosis porótica del techo orbitario es considerada una manifestación ósea microperforativa de condiciones patológicas hematológicas, especialmente la anemia ferropénica. Este hallazgo, se enmarca de manera casi exclusiva a estudios en poblaciones arqueológicas. El propósito de este estudio fue describir la expresión de cribra orbitalia en una población arqueológica de Chile central. Restos óseos de 32 individuos fueron analizados, los cuales fueron obtenidos de la población del Monumento Arqueológico Cementerio Tutuquén, los que están depositados en el Museo Regional de Rancagua. En cada una de la muestras incluidas, se evaluaron períodos de datación, sexo, rango etáreo, presencia v/s ausencia de cribra orbitalia y en los casos en que ésta estuvo presente, se determinó su intensidad mediante visión directa con lente de aumento. Un 59,38 % de los individuos presentó cribra orbitalia. De éstos, 26,32 % fueron de sexo femenino, 31,58 % de sexo masculino y 42,10 % indeterminado. De los individuos del período 10.000 AP, 33,33 % presentó cribra orbitalia; de los individuos del período 7.000 AP un 50 % y de los individuos pertenecientes al período 1.000 AP, un 68,4 %. Al clasificar la severidad de la cribra orbitalia se observó que 31,57 % de los individuos presentaron Cribra orbitalia tipo I; 36,84 % tipo II; 10,52 % tipo III; 10,52 % tipo IV y 10,52 % tipo V. Los datos aportados complementarán el conocimiento morfopatológico de la órbita ocular humana desde el estudio de poblaciones arqueológicas.

SUMMARY: The cribra orbitalia or porotic hyperostosis of the orbital roof is considered a microperforative bone manifestation of hematological pathological conditions, especially iron deficiency anemia. This finding is almost exclusively part of studies in archaeological populations. The purpose of this study was to describe the expression of cribra orbitalia in an archaeological population of central Chile. Bone remains of 32 individuals were analyzed, which were obtained from the population of the Tutuquén Cemetery Archaeological Monument, which are deposited in the Regional Museum of Rancagua. In each of the included samples, dating periods, sex, age range, presence v / s absence of cribra orbitalia were evaluated and in the cases in which it was present, its intensity was determined by direct vision with a magnifying lens. The 59.38 % of the individuals presented cribra orbitalia. Of these, 26.32 % were female, 31.58 % male, and 42.10 % undetermined. Of the individuals in the period 10,000 BP, 33.33 % presented cribra orbitalia; of the individuals of the period 7,000 AP, 50% and of the individuals belonging to the period 1,000 AP, 68.4 %. When classifying the severity of the cribra orbitalia, it was observed that 31.57 % of the individuals had type I; 36.84 % type II; 10.52 % type III; 10.52 % type IV and 10.52 % type V. The data provided will complement the morpho-pathological knowledge of the human eye orbit from the study of archaeological populations.

Presentación atípica de síndrome de CamuratiEngelmann en una paciente femenina colombiana. Reporte de caso / Atypical presentation of Camurati-Engelmann syndrome in a colombian female patient. Case report

Resumen El síndrome de Camurati-Engelmann, también conocido como displasia diafisaria progresiva, es una enfermedad rara, autosómica dominante y con una prevalencia de uno por cada millón de habitantes. Genera mutaciones del factor de crecimiento transformante beta, que participa en la proliferación ósea. Son frecuentes las manifestaciones osteomusculares y neurológicas, con escasas expresiones de laboratorio. El diagnóstico se basa en la clínica, los hallazgos radiológicos y la confirmación genética; el tratamiento se dirige al control sintomático y el pronóstico es incierto. La presente publicación tiene como objetivo compartir con la comunidad médica el tercer caso de síndrome de Camurati-Engelmann conocido en Colombia. Se trata de una paciente femenina de 33 años con cuadro clínico de distonías intensas y signos y síntomas característicos de este síndrome, cuyo diagnóstico fue confirmado por prueba molecular, encontrando la presencia de la variante patogénica p.Arg156Cys en el gen TGF-β1, con presentación de novo. MÉD.UIS.2021;34(1): 119-27.

Abstract Camurati-Engelmann syndrome, also known as progressive diaphyseal dysplasia, is a rare, autosomal dominant disease with a prevalence of one per million inhabitants. It generates mutations of the transforming growth factor beta, which participates in bone proliferation. Osteomuscular and neurological manifestations are frequent, with few laboratory expressions. The diagnosis is based on the clinic, radiological findings, and genetic confirmation, treatment is aimed at symptom control and prognosis is uncertain. The objective of this publication is to share with the medical community the third case of Camurati-Engelmann syndrome known in Colombia. This is a 33-year- old female patient with a clinical picture of intense dystonia and characteristic signs and symptoms of this syndrome, whose diagnosis was confirmed by molecular testing, finding the presence of the pathogenic variant p.Arg156Cys in the TGF-β1 gene, with de novo presentation. MÉD.UIS.2021;34(1): 119-27.

Organ histopathological associations with hyperostosis frontalis interna in humans / Asociaciones histopatológicas de órganos con hiperostosis frontal interna en humanos

SUMMARY: An increased thickening of the frontal bone by irregular laminar additions on the inner surface just deep to the dura mater have been known in the archaeological and medical record as hyperostosis frontalis interna (HFI). The distribution of this is idiosyncratically restricted to the frontal and has no known etiology. The prevalence among post-menopausal females and rarity in males suggests that it is hormonally driven. Here we report histopathological findings of particularly hormonally active organs (pituitaries, gonads and liver) from a geriatric cadaveric sample in which HFI is assessed. HFI was present in 50 % of males (7/14) and 95 % (21/22) of females. All males with HFI had testicular atrophy or had testes absent. Males with HFI category C or D had moderate to severe testicular atrophy. Decreased numbers of interstitial cells (Leydig cells) were present in 83.3 % of males with HFI. All but one female (21/22) from this study exhibited evidence of HFI, and ovarian pathologies were unevenly distributed (fibromas in two) and most exhibited signs of being healthy and post-menopausal. Liver pathologies had opposite patterns between the sexes, with more liver pathologies occurring among males without HFI (particularly passive congestion and bile stasis). The only exceptions were that the one case of liver neoplasia was found in a male with HFI and steotosis was found in two cases with HFI and one case without HFI. In females all cases of liver pathologies (steotosis, hepatitis, passive congestion, fibrosis, and bile stasis) were associated with HFI. It appears that gonadal pathology is most closely associated with HFI in males but not females, suggesting that the role of estradiol in this unusual growth of bone in geriatric humans may be worth investigating further.

RESUMEN: Un aumento del engrosamiento del hueso frontal, por adiciones laminares irregulares en la superficie interna, justo en la profundidad de la duramadre, se conoce en los registros arqueológicos y médicos como hiperostosis frontal interna (HFI). La distribución de ésta, se restringe idiosincráticamente al hueso frontal y no tiene etiología conocida. La prevalencia entre las mujeres posmenopáusicas y la rareza en los hombres sugiere que se debe a las hormonas. Aquí informamos los hallazgos histopatológicos de órganos hormonalmente activos (hipófisis, gónadas e hí- gado) de una muestra de cadáveres geriátricos en la que se evaluó HFI. La HFI estuvo presente en el 50 % de los hombres (7/14) y el 95 % (21/22) de las mujeres. Todos los hombres con HFI tenían atrofia testicular o no tenían testículos. En los hombres con HFI categoría C o D se observó atrofia testicular de moderada a grave. Hubo una disminución en el número de células intersticiales (células de Leydig) en el 83,3 % de los hombres con HFI. En 21de 22 mujeres se observó evidencia de HFI, y las patologías ováricas se distribuyeron de manera desigual (fibromas en dos) y la mayoría exhibió signos de estar sana y posmenopáusica. Las patologías hepáticas tenían patrones opuestos entre los sexos, con más patologías hepáticas entre los hombres sin HFI (particularmente congestión pasiva y estasis biliar). Las excepciones fueron que el único caso de neoplasia hepática se encontró en un varón con HFI y se presentó esteatosis en dos casos con HFI y un caso sin HFI. En las mujeres, todos los casos de patologías hepáticas (esteatosis, hepatitis, congestión pasiva, fibrosis y estasis biliar) se asociaron con HFI. Al parecer la patología gonadal está más estrechamente asociada con la HFI en los hombres que en las mujeres, lo que sugiere un rol del estradiol en este crecimiento inusual de hueso en hombres de avanzada edad. Sería importante realizar investigaciones más detalladas precisas respecto a la hiperostosis frontal interna.

Clinical outcomes of transforaminal lumbar interbody fusion for patients with lumbar spinal stenosis and diffuse idiopathic skeletal hyperostosis / 中华骨科杂志

Objective:To evaluate the outcomes of transforaminal lumbar interbody fusion (TLIF) for patients with lumbar spinal stenosis (LSS) and diffuse idiopathic skeletal hyperostosis (DISH).Methods:This study recruited 33 patients (15 male and 18 female) with LSS and DISH who underwent TLIF surgery from January 2010 to July 2018. The mean age was 65.1±11.2 years old and the instrumented segments averaged 1.45±0.42 levels. Another group of LSS patients without DISH were well matched to the DISH group at a 1∶1 ratio in terms of age, sex, and instrumented levels. Lumbar lordosis, local lordosis as well as lowerlumbarlordosis were measured on X-ray taken before and after surgery. According to Pfirrmann's classification, the degree of preoperative disc degeneration was evaluated on preoperative MRI, and the incidence of postoperative fusion cage subsidence was recorded during follow-up. Oswestry disability index (ODI) was used to evaluate the lumbar function and visual analogue scale(VAS) scores for low back pain and leg pain were used to evaluate the quality of life.Results:There were no significant difference between two groups in terms of age, bone mineral density, operative time, postoperative bleeding volume, fusion levels and postoperative follow-up time. Compared with the non-DISH group (3.20±0.95), the DISH group had higher degeneration disc according to Pfirrmann's classification (3.82±0.64) ( t=3.109, P=0.002), lower lumbar lordosis(37.4°±8.5° vs. 45.2°±12.2°, t=3.013, P=0.003), and lower lower lumbar lordosis (18.3°±3.9° vs. 21.9°±5.4°, t=3.104, P=0.002). After TLIF surgery, lumbar lordosis was significantly improved in both groups. During follow-up, notable correction loss was noted in DISH group in terms of lumbar lordosis (43.6°±9.7° vs. 50.1°±10.2°, t=2.652, P=0.010), lower lumbar lordosis (19.1°±4.7° vs. 22.9°±5.2°, t=2.540, P=0.013) as well as local lumbar lordosis (17.4°±6.5° vs. 22.7°±7.2°, t=3.138, P=0.002). Moreover, these above value in the DISH group were significantly lower than those in the non-DISH group. At the latest follow-up, 12 patients in the DISH group were identified with cage subsidence, which were significantly higher than in the non-DISH group (36.3% vs. 12.1%, χ2=5.280, P=0.022). Till the latest follow-up, both groups had considerable improvement of the ODI score, back pain and leg pain VAS score. However, the back pain VAS scores in the DISH group were significantly higher than that in the non-DISH group ( t=2.862, P=0.005). Conclusion:Compared with LSS patients without DISH, LSS patients with DISH are more likely to have cage subsidence and loss of correction of lumbar lordosis angle after TLIF surgery. Moreover, the VAS score of low back pain in LSS patients with DISH was lower than those without DISH.

The diffuse idiopathic skeletal hyperostosis classification based on CT and the correlation of ossification and sagittal spinal morphology / 中华骨科杂志

Objective:To investigate CT classification of diffuse idiopathic skeletal hyperostosis (DISH), and to analyze the correlation between the position of ossification in the anterolateral spine and the sagittal configuration of the spine.Methods:The medical records of 109 patients (70 male and 39 female) who underwent whole spine computerized tomography (CT) from October 2018 to October 2020 were retrospectively analyzed. The average age was 68.4±6.9 years old, ranging from 60 to 88 years old. High resolution CT volume rendering technique images were used to assess the degree of anterolateral spinal ossification in each vertebral space, and a CT grading system was established. Sagittal parameters such as thoracic kyphosis (TK), lumbar lordosis(LL), cervical lordosis (CL), sacral slope (SS), and thoracolumbar junction angle (TLJ) of the patients were measured. The sagittal morphology of the spine was divided into four types using the modified Abelin-Genevois (AG) sagittal classification. In AG type 1 patients, the kyphotic vertex was located in the middle of the thoracic spine (T 4-T 11). In AG type 2 patients, there was no significant kyphotic vertex. In AG type 3 patients, the kyphotic vertex was located in the thoracolumbar segment (T 12-L 2). In AG type 4 patients, the kyphotic vertex was located in the upper thoracic segment (T 1-T 3). Inter-observer and intra-observer reliability were calculated by intra-group correlation coefficient ( ICC). Statistical analysis was conducted to investigate the correlation between different AG types and ossification location and severity. Results:The new DISH grading system classifies the severity of anterolateral spinal ossification in each intervertebral space into grades 0 to 3 with an intra-observer ICC value of 0.871 and inter-observer ICC value of 0.874. Combined with Resnick's DISH diagnostic criteria, 97 patients (89.0%) in this study had four consecutive intervertebral spaces with ossification grade 1 or above. For these patients, in T 4-T 11, the standardized ossification grade of AG type 1 was 1.24±0.69, greater than that of AG type 2 (0.84±0.71) and AG type 3 (1.00±0.70), and the differences were statistically significant ( F=23.101, P<0.001). In T 12-L 2, the standardized ossification grade of AG type 3 was 1.44±0.87, which was higher than AG type 1 (1.06±0.84) and AG type 2 (0.72±0.63), the differences were statistically significant ( F=14.008, P<0.001). In this study, no patients with kyphosis apex in the cervicothoracic region (AG type4) were found. In T 1-T 3, there was no statistical difference between the three groups ( F=0.303, P=0.738); in the whole thoracic and lumbar spine (T 1-L 5), there was statistically significant difference in the total ossification grade ( F=14.374, P<0.001), there was no statistical difference between AG type 1 and AG type 3 ( P=0.254), both of which were higher than AG type 2 ( P<0.001). Conclusion:The new DISH ossification grading system proposed in this study has high credibility, which can be used in DISH's study. This study confirmed that the region where the apex of kyphosis is located is prone to anterolateral ossification of the spine.

Prevalence and distribution of diffuse idiopathic skeletal hyperostosis in patients with thoracic ossification of the ligamentum flavum / 中华骨科杂志

Objective:To analyze the prevalence and distribution characteristics of diffuse idiopathic skeletal hyperostosis (DISH) in hospitalized patients with thoracic ossification of the ligamentum flavum (TOLF).Methods:The clinical records of 132 consecutive TOLF patients from January 2018 to June 2019 were retrospectively studied. DISH was identified by the preoperative X-ray and CT and its prevalence was calculated. The prevalence of patients with different genders, different age groups and different ossification types was compared. The segmental distribution of DISH and the distribution in the upper (T 1-T 4), middle (T 5-T 8), and lower thoracic spine (T 9-T 12) were analyzed. Ossification degree of DISH was evaluated based on the Meta scoring system. The demographic characteristics (age, gender, BMI, etc.) were compared between DISH and non-DISH group. Results:Forty-nine patients was diagnosed as DISH with the prevalence of 37.1% in all included cases. The prevalence was about twice as high in male (46.3%) than in female (23.1%) ( χ2=8.806, P=0.003). The prevalence in the age groups of <40, 40-49, 50-59, 60-69 and ≥70 years was 20.0%, 28.0%, 34.4%, 44.0%, and 66.7%, respectively. The prevalence in long-segment TOLF patients (45.1%) was significantly higher than that in short-segment TOLF patients (24.0%) ( χ2=5.937, P=0.015). DISH most frequently affected T 8,9 levels (91.8%). The total number and mean number of ossified segments were 365 and 7.4, respectively. Ossification lesions in the upper, middle, lower thoracic spine accounted for 26.03%, 40.54%, and 33.15%, respectively. Grade I, grade II, and grade III ossification accounted for 21.4%, 28.5% and 50.1%, respectively. The mean age of the DISH group was older than the non-DISH group ( t=2.024, P=0.045). The proportion of male patients in the DISH group was significantly higher than that in the non-DISH group ( χ2=8.806, P=0.003). The average height and weight in the DISH group were significantly greater than those in the non-DISH group ( t=2.564, P=0.012; t=2.191, P=0.030), whereas no significant differences in BMI and constituent ratio of concurrent diabetes, cardiac disease, hypertension between two groups were observed. Conclusion:The prevalence of DISH in patients with TOLF is 37.1%. Male, elderly and long-segment TOLF patients are associated with higher prevalence. DISH frequently occurs in the middle and lower thoracic spine, and T 8,9 is the common affected segment. Ossification lesions may develop with age. Demographic characteristics of DISH group differ, to some extent, from those of non-DISH group.

Analysis of serum levels of bone turnover biochemical markers in patients with SAPHO syndrome / 中华风湿病学杂志

Objective:To evaluate the changes and significance of bone turnover makers in patients with SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.Methods:Thirty-two patients with SAPHO syndrome who were treated in the department of rheumatology and immunology of Beijing Jishuitan Hospital were collected as the study group, and 28 healthy subjects were taken as the control group. The clinical data and bone turnover markers were compared between the two groups, and the correlation between the bone turnover markers and disease-related indicators were analyzed. Two independent samples were comp-ared by using t test (in line with normal distribution) and rank sum test (not in line with normal distribution). The results of more than two independent samples were compared by one-way analysis of variance, and the correlation between two variables was analyzed by Spearman. Results:Compared with the clinical data of the two groups, hemoglobin (Hb) of the study group [128(122, 140) g/L] was significantly lower than that of the control group [139(125, 154) g/L]( U=306.5, P<0.05), but alkaline phosphatase (ALP) [75.00(66.00, 85.75) mmol/L] was significantly higher than that of the control group [56.00(48.50, 63.25) mmol/L] ( U=153, P<0.01). The comparison of bone turnover markers showed that serum total procollagen type 1 amino-terminal propeptide (tP1NP)[52.51(41.72, 86.11) ng/ml], Beta C-terminal cross-linked telopeptides of type Ⅰ collagen (β-CTX) [0.57(0.39, 0.72) ng/ml] and OC [(20±8) ng/ml] levels in the study group were significantly higher than those in the control group [34.91(28.97, 42.80) ng/ml, 0.34(0.27, 0.49) ng/ml, (15±4) ng/ml] ( U=183, P<0.01; U=223, P<0.01; t=3.180, P<0.01). There was no significant difference in 25-(OH)VD 3 between the two groups [14.73(12.25, 19.23) ng/ml, 16.72(11.74, 20.92) ng/ml] ( P>0.05). The serum biochemical markers of bone turnover were not related to spine, bone and joints involvement. The grouping results of the number of joints involved showed that there were significant differences in serum osteocalcin (OC) levels of patients ( F=3.684, P<0.05), and the more the number of joints involved, the lower the OC value. Correlation analysis showed that serum tP1NP ( r=0.805), β-CTX ( r=0.460) and OC levels were positively correlated with each other(all P<0.01). Levels of tP1NP, β-CTX, OC, and 25-(OH)VD 3 in the study group were not related to age, course of disease, and neutrophil granulocyte (all P>0.05). However, β-CTX was positively correlated with C-reactive protein (CRP) ( r=0.392, P<0.05), and OC was positively correlated with erythrocyte sedimentation rate (ESR) ( r=0.475, P<0.05). Conclusion:The levels of tP1NP, β-CTX and OC in patients with SAPHO syndrome are significantly increased. Levels of β-CTX and OC can reflect the severity of patients' inflammation. The level of OC is related to the number of joint involvement of the patient.

Hiperostosis esquelética difusa idiopática, una causa infrecuente de disfagia y disfonía

Resumen La hiperostosis esquelética difusa idiopática (DISH) es una enfermedad sistémica caracterizada por la osificación del ligamento longitudinal anterior de la columna. Los pacientes suelen ser asintomáticos o presentar dolor leve o rigidez, sin embargo, cuando afecta la región cervical puede ocasionar disfagia, disfonía o disnea. Presentamos el caso de un paciente de 63 años con disfonía y disfagia en quien los estudios demostraron desplazamiento del aritenoides y colapso del seno piriforme debido a un osteofito a nivel de C4. El paciente presentó mejoría con tratamiento conservador. Realizamos una discusión del caso y una revisión de la literatura sobre diagnóstico y tratamiento de esta patología.

Abstract Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic disease characterized by ossi- fication of the anterior longitudinal ligament of the spine. Patients are usually asympto- matic, or present mild pain or stiffness, however cervical compromise can cause dysphagia, dyspnea and dysphonia. We present the case of a 63-year-old patient with hoarseness and dysphagia. Studies revealed anterior displacement of the arytenoid cartilage and collapse of the pyriform sinus secondary to an osteophyte at C4 level. The patient showed improvement with conservative management. We present a discussion about this case and the available scientific evidence on the diagnosis and treatment of this pathology.

Características clínico-radiológicas de la hiperostosis esquelética idiopática difusa en 2 centros médicos de Cali, Colombia: reporte de 24 casos / Clinical and radiological characteristics of diffuse idiopathic skeletal hyperostosis in two medical centers in Cali, Colombia: Report of 24 cases

RESUMEN Introducción: La hiperostosis esquelética idiopática difusa (DISH, por sus siglas en inglés) es una afección caracterizada por la calcificación y la osificación progresiva de los ligamentos y las entesis. La mayoría de los pacientes permanecen asintomáticos hasta etapas avanzadas de la enfermedad, donde la limitación y el dolor son característicos. Objetivo: Describir las características demográficas, clínicas y radiológicas de los pacientes con DISH evaluados en el Centro Médico Imbanaco de Cali y en la Clínica de Artritis Temprana, en Cali, Colombia. Materiales y métodos: Es un estudio descriptivo, de corte transversal. Se revisaron los registros de pacientes diagnosticados con DISH, seguidos entre enero de 2000 y octubre de 2018. El diagnóstico se confirmó según los criterios de Resnick-Niwayama. Se encontraron 24 pacientes, todos se incluyeron para el análisis final. Resultados y discusión: En esta serie se encontraron 20 varones y 4 mujeres. La mediana de edad al diagnóstico fue de 70,5 arios (RIQ: 61,3-73,8 arios), siendo menor en las mujeres (71,5 versus 60 años; p = 0,04). La mediana de tiempo de evolución de los síntomas fue de 5 años (RIQ: 3-10 años), la duración fue menor en el grupo de las mujeres (5 versus 4 años; p = 0,20). El 54,2% tenían sobrepeso y el 20,8% eran diabéticos. El síntoma principal fue la limitación cervical. Los segmentos vertebrales (C: cervical; T: torácico; L: lumbar) más afectados por la osificación del ligamento longitudinal anterior (LLA) fueron C5-C6, T8-T10 y L1-L3. La afección periférica predominó en las crestas ilíacas. Todos los pacientes realizaron terapia física y 3 fueron sometidos a cirugía cervical. El grado de limitación funcional fue valorado en 19 pacientes a través de los cuestionarios modified Health Assessment Questionnaire (mHAQ) y Bath Ankylosing Spondylitis Functional Index (BASFI). La mediana del puntaje de ambos cuestionarios fue 2 veces más alta en las mujeres. Conclusión: La DISH fue más frecuente en varones mayores de 65 años y se asoció con enfermedades metabólicas como la obesidad y la diabetes. Aunque las diferencias no fueron significativas, los resultados sugieren que las mujeres presentan un fenotipo grave de la enfermedad explicado por el inicio temprano y curso progresivo de los síntomas, así como mayor limitación funcional medida por mHAQ y BASFI.

ABSTRACT Introduction: Diffuse idiopathic skeletal hyperostosis (DISH) is a condition characterised by calcification and progressive ossification of ligaments and entheses. Most patients remain asymptomatic until advanced stages of the disease, where limitation and pain are characteristic. Objective: To describe the demographic, clinical, and radiological characteristics of patients with DISH evaluated in the Centro Médico Imbanaco and Clínica de Artritis Temprana, in Cali, Colombia. Materials and methods: A descriptive, cross-sectional study was conducted by reviewing the records of patients diagnosed with DISH between January 2000 and October 2018. The diagnosis was confirmed according to the Resnick-Niwayama criteria. A total of 24 patients were found, and all were included for the final analysis. Results and discussion: The series included 20 men and 4 women, with a median age at diagnosis of 70.5 years (IQR 61.3-73.8 years), beinglower in women (71.5 versus 60 years; P=.04). The median time of onset of the symptoms was 5 years (IQR 3-10 years), and the duration was shorter in women (5 versus 4 years; P=.20). It was observed that 54% were overweight and 20% were diabetic. The main symptom was cervical limitation. The most affected vertebral segments due to the ossification of the anterior longitudinal ligament (ALL) were C5-C6, T8-T10 and L1-L3 (cervical C; thoracic T; lumbar L). The peripheral involvement was mainly in the iliac crests. All patients received physiotherapy, and three of them underwent cervical surgery. The degree of functional limitation was assessed in 19 patients using the mHAQ (Modified Health Assessment Questionnaire) and BASFI (Functional Ankylosing Spondylitis Functional (BASFI) questionnaires. The median score of both questionnaires was 2 times higher in women Conclusion: Diffuse idiopathic skeletal hyperostosis was more frequent in men over 65 years of age, and was associated with metabolic conditions such as obesity and diabetes. Although the differences were not significant, the results suggest that women have a more severe phenotype of the disease, explained by the early onset and progressive course of symptoms, as well as greater functional limitation measured by mHAQ and BASFI.

Surgical treatment of fractures of the ankylosed spine / Tratamento cirúrgico de fraturas da coluna anquilosada / Tratamiento quirúrgico de las fracturas en la columna anquilosada

ABSTRACT Objective We aim to identify retrospectively surgically treated patients with an ankylosed spine who sustained a vertebral fracture. Our goal is to evaluate the main outcomes and complications. Methods We selected patients through the database of surgical interventions in the setting of fractures of an ankylosed spine segment between January 1st 2008 and June 30th 2018. We collected data from digital medical records. The parameters analyzed include hospital length of stay, Intensive Care Unit (ICU) admission, perioperative and postoperative complications as well as neurological evolution. Results Fractures occurred in 14 patients with ankylosing spondylitis (82%) and 3 patients with diffuse idiopathic skeletal hyperostosis (18%). All patients were male and the mean age was 69 years. Fourteen fractures occurred after minor trauma (83%), of which 11 were due to falls from standing height or lower (65%). The cervical spine represents the majority of the levels involved (65%). Seven patients were admitted to the ICU (41%) and 11 suffered neurological damage. There was improvement of neurological status in less than 50% and there were high percentages of post-operative complications. Conclusion Patients with ankylosed spine diseases are at higher risk for vertebral fracture, even after minor trauma, and these are located predominantly in the cervical spine. The surgical treatment of these conditions is effective as it allows improvement of the patient's neurological status. However, they still present higher morbidity and mortality, as well as increased post-op complications. Prevention of falls may drastically change patients' outcome, neurological function and independence in activities of daily living. Level of evidence IV; A case series therapeutic study.

RESUMO Objetivo Identificar retrospetivamente casos de tratamento cirúrgico de fraturas vertebrais em pacientes com coluna anquilosada. O nosso propósito consiste em avaliar os principais desfechos e respectivas complicações. Métodos Selecionamos pacientes através do banco de dados de intervenções cirúrgicas no quadro de fraturas de um segmento da coluna anquilosada entre 1 de janeiro de 2008 a 30 de junho de 2018. Coletamos os dados a partir dos prontuários médicos digitais. Os parâmetros analisados incluem período de internação hospitalar, admissão na Unidade de Tratamento Intensivo (UTI), complicações pré- e pós-operatórias, assim como evolução neurológica. Resultados As fraturas ocorreram em 14 pacientes com espondilite snquilosante (82%) e em 3 pacientes com hiperostose esquelética difusa idiopática (18%). Todos os pacientes eram do sexo masculino e a idade média era de 69 anos. Quatorze fraturas ocorreram devido a trauma menor (83%), das quais 11 eram devido a quedas da própria altura ou inferiores (65%). A coluna cervical representa a maioria dos níveis envolvidos (65%). Sete pacientes foram admitidos na UTI (41%) e 11 sofreram lesão neurológica. Houve melhoria do estado neurológico em menos de metade dos pacientes e altas porcentagens de complicações pós-operatórias. Conclusão Os pacientes com doenças da coluna anquilosada têm maior risco de fraturas vertebrais, mesmo após trauma menor, localizando-se predominantemente na coluna cervical. O tratamento cirúrgico dessas condições é eficaz, uma vez que permite melhora do estado neurológico do paciente. Entretanto, ainda apresentam altos índices de morbilidade e mortalidade, assim como maior incidência de complicações pós-operatórias. A prevenção de quedas pode alterar drasticamente o desfecho, função neurológica e independência nas atividades diárias do paciente. Nível de evidência IV; Estudo terapêutico de série de casos.

RESUMEN Objetivo Identificar retrospectivamente casos de tratamiento quirúrgico de fracturas vertebrales en pacientes con columna anquilosada. Nuestro propósito consiste en evaluar sus principales resultados y respectivas complicaciones. Métodos Seleccionamos pacientes mediante banco de datos de intervenciones quirúrgicas en el cuadro de fracturas de un segmento de la columna anquilosada entre el 1 de enero de 2008 al 30 de junio de 2018. Recolectamos los datos a partir de los prontuarios médicos digitales. Los parámetros analizados incluyen período de internación hospitalaria, admisión en la Unidad de Tratamiento Intensivo (UTI), complicaciones pre y postoperatorias, así como evolución neurológica. Resultados Las fracturas ocurrieron en 14 pacientes con espondilitis anquilosante (82%) y en 3 pacientes con hiperostosis esquelética difusa idiopática (18%). Todos los pacientes eran del sexo masculino y la edad promedio era de 69 años. Catorce fracturas ocurrieron debido a trauma menor (83%), de las cuales 11 eran debido a caídas de la propia altura o inferiores (65%). La columna cervical representa la mayoría de los niveles implicados (65%). Siete pacientes fueron admitidos en la UTI (41%) y 11 sufrieron lesión neurológica. Hubo mejora del estado neurológico en menos de la mitad de los pacientes y altos porcentajes de complicaciones postoperatorias. Conclusión Los pacientes con enfermedades de la columna anquilosada tienen mayor riesgo de fracturas vertebrales, incluso después de trauma menor, localizándose predominantemente en la columna cervical. El tratamiento quirúrgico de esas condiciones es eficaz, ya que permite mejora del estado neurológico del paciente. Entretanto, aún presentan altos índices de morbilidad y mortalidad, así como mayor incidencia de complicaciones postoperatorias. La prevención de caídas puede alterar drásticamente los resultados, función neurológica e independencia en las actividades diarias del paciente. Nivel de evidencia IV; Estudio terapéutico de serie de casos.

Surgical Outcomes of Dysphagia Provoked by Diffuse Idiopathic Skeletal Hyperostosis in the Cervical Spine

PURPOSE: This study aimed to predict the surgical outcomes of diffuse idiopathic skeletal hyperostosis (DISH)-related dysphagia (DISH-phagia) and to evaluate the importance of prevertebral soft tissue thickness (PVST).MATERIALS AND METHODS: In total, 21 surgeries (anterior osteophytectomy or anterior cervical decompression and fixation) were included in this study for DISH-phagia from 2003 to 2019. Clinical outcomes were assessed using the Dysphagia Outcome and Severity Scale (DOSS) preoperatively, at 1 month postoperatively, and last follow up (mean 29.5 months). PVST was measured using lateral plain radiographs. Paired t-test and Spearman's correlation test was used to identify relationships between various PVST indices and DOSS.RESULTS: Comparisons were made from 17 patients out of 21, in which the record had all of three measurements. The narrowest PVST preoperatively was 2.55±0.90 mm, with a DOSS score of 4.47±1.61, and that at 1 month after surgery was 5.02±2.33 mm, with a DOSS score of 6.12±1.32. At last follow up, PVST and DOSS values were 3.78±0.92 mm and 5.82±1.34, and three patients experienced symptom relapse. Significant relationships were found between PVST and DOSS at all time points: before surgery (R=0.702, p<0.001), 1 month after surgery (R=0.539, p=0.012), and last follow up (R=0.566, p=0.020).CONCLUSION: Surgical removal of anterior osteophytes is an effective treatment option for DISH-phagia, and PVST is a useful parameter in DISH-phagia. The goal of DISH surgery should be to remove DISH as much as possible to ensure sufficient PVST postoperatively.

Uncommon bilateral maxillary exostosis: case report / Exostose maxilar bilateral incomum: relato de caso

ABSTRACT Exostoses or hyperostoses are benign bony outgrowths originating from the cortical bone and depend on their location for a more precise designation. The most common types found in the oral cavity are the torus palatinus and the torus mandibularis. Buccal and palatal exostoses are located along the buccal aspect of the maxilla and/or the mandible (commonly in the premolar and molar areas) and on the palatal aspect of the maxilla (usually in the tuberosity area), respectively. The etiology of exostoses still hasn't been enlightened but an interaction between environmental and genetic factors is accredited. They are usually asymptomatic, unless the mucosa becomes ulcerated. The frequency of exostoses increases with age, having their biggest prevalence from 60 years old, being more common in men and suffering ethnic influences. A thorough evaluation is important for the correct diagnosis since other lesions have similar clinical characteristics to the exostoses such as osteomas. The majority of exostoses are diagnosed clinically along with radiographic interpretations, making the biopsy dispensable and the treatment is usually unnecessary. The aim of this article was to describe a case report of bilateral maxillary exostosis, unusual, in a female patient. If an excessive amount of bone is present the exostoses may exhibit a relative radiopacity on dental radiographs. Initially, periapical and panoramic radiographs were performed to evaluate the alterations. Due to the size of the exostoses a concomitant Cone Beam Computed Tomography was performed to confirm the diagnosis. The patient is in follow-up.

RESUMO Exostoses ou hiperostoses são crescimentos ósseos benignos originados da cortical óssea e dependem de sua localização para uma denominação mais precisa. Os tipos mais comuns encontrados na cavidade oral são o Tórus Palatino e o Tórus Mandibular. Exostoses bucais e palatais acometem a face vestibular do processo alveolar na maxila e/ou na mandíbula (comumente na região de molares e pré-molares) e a superfície do processo alveolar posterior (usualmente na área da tuberosidade maxilar), respectivamente. A etiologia das exostoses ainda não foi esclarecida, mas acredita-se em uma interação entre fatores ambientais e genéticos. São usualmente assintomáticas, a não ser que a mucosa se torne ulcerada. A frequência aumenta com a idade, tendo sua maior prevalência a partir de 60 anos de idade, sendo mais comuns em homens e sofrendo influências étnicas. É importante uma avaliação minuciosa para o correto diagnóstico, visto que outras lesões, como os osteomas, possuem características clínicas semelhantes às das exostoses. A maior parte das exostoses é diagnosticada clinicamente em conjunto com interpretações radiográficas, tornando a biópsia dispensável e o tratamento é usualmente desnecessário. O objetivo deste artigo foi relatar um caso clínico de exostose maxilar bilateral, incomum, em uma paciente do sexo feminino. Se uma quantidade excessiva de osso está presente, as exostoses podem mostrar uma relativa radiopacidade nas radiografias dentárias. Inicialmente foram realizadas radiografias periapicais e radiografia panorâmica para avaliar as alterações. Devido ao tamanho das exostoses, realizou-se uma Tomografia Computadorizada de Feixe Cônico, para confirmação do diagnóstico. A paciente encontra-se em acompanhamento.

Síndrome de Klippel y Trénaunay / Klippel-Trenaunay syndrome

RESUMEN Introducción: el síndrome de Klippel y Trénaunay es el resultado de un trastorno de desarrollo embrionario de los tejidos mesodérmicos que afectan la angiogénesis en diferentes etapas, posiblemente después de una lesión intrauterina, por lo que aparece con muy poca frecuencia en la adultez. Presentación del caso: se trata de una mujer de 31 años de edad que acude al cuerpo de guardia por presentar sangramiento rectal y edema en miembro inferior izquierdo que abarca el muslo. Al examen físico se constata nevo vascular ubicado en la extremidad inferior izquierda y venas varicosas que refiere la paciente tener desde edades muy tempranas de su vida. Los exámenes de imágenes confirmaron el diagnóstico de un síndrome Klippel y Trénaunay. Recibió tratamiento según protocolo logrando mejoría clínica evidente. Conclusiones: el adecuado diagnóstico de este síndrome permitirá un seguimiento y tratamiento médico adecuados por el equipo multidisciplinario responsable logrando así una mejor calidad de vida del paciente.

ABSTRACT Introduction: Klippel-Trenaunay syndrome (KTS) is the result of an embryonic developmental disorder of mesodermal tissues affecting angiogenesis at different stages, possibly after intrauterine injury, so it occurs very rarely in adulthood. Case report: a 31-year-old woman who comes to the on-call body for rectal bleeding and edema in the left lower limb encompasses the thigh. Physical examination shows vascular nevus located in the lower left extremity and varicose veins that the patient refers to have from very early ages of life. Imaging confirmed the diagnosis of Klippel-Trenaunay syndrome (KTS). The patient underwent treatment according to the protocols established, achieving evident clinical improvement. Conclusions: a proper diagnosis of this syndrome will allow adequate follow-up and medical treatment by the multidisciplinary team in charge; as a result a better quality of life for the patient was achieved.

Síndrome Sapho: reporte de caso / SAPHO syndrome: report of a case

El síndrome SAPHO es una enfermedad inflamatoria de origen multifactorial que incluye una asociación entre manifestaciones dermatológicas y osteoarticulares. La presencia de osteítis e hiperostosis definen la enfermedad. No existe un tratamiento estándar, pero se usan AINES como primera línea. Se presenta el caso de un varón de 18 años con acné severo en dorso, pecho, barbilla y zona retroauricular, asociado a fiebre alta y placas eritematosas en ambas piernas. La gammagrafía presenta hipercaptación en tibia derecha, con relación a osteítis no infecciosa. Tuvo evolución favorable con antibióticos y corticoide oral.

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a multifactorial inflammatory condition that includes an association between dermatological and bone and joint manifestations. The presence of osteitis and hyperostosis define this disease. There is no standard therapy for SAPHO syndrome, but NSAIDs are used as first line. We present the case of an 18-year-old male subject with severe acne on his back, chest, chin, and the retroauricular area, being this associated to high fever and erythematous plaques in both legs. The bone scan showed right leg hyper-uptake, related to non-infectious osteitis. The patient did well with antibiotics and oral steroids.

Uremic lion face syndrome / Face Leonina na Síndrome Urêmica

Abstract Mineral bone disorder is a common feature of chronic kidney disease. Lion face syndrome is rare complication of severe hyperparathyroidism in end-stage renal disease patients, which has been less commonly reported due to dialysis and medical treatment advances in the last decade. The early recognition of the characteristic facial deformity is crucial to prompt management and prevent severe disfigurement. The authors present a rare case of severe hyperparathyroidism presenting with lion face syndrome and bone fractures.

Resumo O distúrbio mineral e ósseo é uma característica comum da doença renal crônica. A síndrome da face leonina é uma complicação rara do hiperparatireoidismo grave em pacientes com doença renal terminal, que tem sido menos relatada devido aos avanços na diálise e tratamento médico na última década. O reconhecimento precoce da deformidade facial característica é crucial para estimular o tratamento precoce e prevenir a desfiguração severa. Os autores apresentam um caso raro de hiperparatireoidismo grave, apresentando síndrome da face leonina e fraturas ósseas.

Ossification Of The Anterior Longitudinal Ligament: Clinical And Embryological Aspect

Normal anatomy of vertebral column is extremely vital for weight transmission as well as normal day to dayactivities. Osteogenic outgrowths and ossifications are commonly reported in literature; however, extensiveossification of the anterior longitudinal ligament (ALL) is a rare finding. During routine undergraduate teachingin the Department of Anatomy, Hamdard Institute of Medical Sciences, New Delhi, we came across ossification inthe ALL of an elderly male skeleton, extending from T9 to L2, with complete bridging between vertebrae T11 -T12as well as L1- L2 and broken (possibly due to bone processing) in region between T9-T10, T10- T11, T12-L1.Ossification was extensive on the right side as compared to the left. Knowledge of such ossification will aid asurgeon during lateral transpsoas approach of vertebral column and prevent complications while performingsurgeries in this region such as lordiosis restoration with ALL release and in placement of hyperlordotic cage etc.