Radiological Evolution Research Articles

Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: A single center study of a cohort of 71 subjects

To assess the basic features and outcomes of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. We identified all patients seen in our unit between 1990 and 2008 diagnosed according to the proposed inclusion criteria with SAPHO syndrome, who had a followup of at least 2 years. Seventy-one patients (48 women, 23 men) with SAPHO syndrome were identified. The median disease duration at the end of followup was 10 years (interquartile range [IQR] 7-15 years), and the median followup duration was 11 years (IQR 6-11.5 years). Six patients were diagnosed with Crohn's disease. Fourteen patients had never had cutaneous involvement, but 8 patients presented >1 skin manifestation. Nine patients (13%) presented a limited (<6 months) monophasic disease course, 25 cases (35%) had a relapsing-remitting course, and 37 patients (52%) had an acute painful phase with a prolonged course lasting >6 months. A total of 4% of the patients were HLA-B27 positive. Female sex (odds ratio [OR] 7.2, 95% confidence interval [95% CI] 2.2-22.9) and the presence at onset of anterior chest wall (ACW) involvement (OR 5.7, 95% CI 1.8-18.1), peripheral synovitis (P = 0.0036), skin involvement (OR 10.3, 95% CI 3.4-31.1), and high values of acute-phase reactants (OR 7.7, 95% CI 2.7-22) were correlated with a chronic disease course and involvement of new osteoarticular sites. A chronic course is the more common evolution of SAPHO syndrome. Female sex, elevated erythrocyte sedimentation rate and C-reactive protein values, ACW involvement, peripheral synovitis, and skin involvement at the onset seem to be associated with a chronic course.

Exacerbação aguda da fibrose pulmonar idiopática

Some patients with Idiopathic Pulmonary Fibrosis (IPF) have disease accelerated deterioration without identifiable cause referred as “acute exacerbation” or “accelerated stage”. It is characterized by severe worsening of dyspnea, hypoxemia and new or progressive opacities on imaging studies. The typical histological findings are diffuse alveolar damage in addition to the features of usual interstitial pneumonia pattern. Mortality in this clinical entity is very high and no efficacious therapeutic have been described.The authors describe the clinical, functional and radiological features, treatment and evolution of five patients with IPF acute exacerbation. A discussion will be carry out concerning the IPF acute exacerbation usual features comparing with the alterations noticed in those patients.Rev Port Pneumol 2009; XV (2): 305-312

Differential patterns of evolution in acute middle cerebral artery infarction with perfusion–diffusion mismatch: Atherosclerotic vs. cardioembolic occlusion

Background An acute perfusion–diffusion mismatch is known to be the strongest predictor of infarct growth. However, the differential patterns of clinical and radiological evolution according to stroke mechanism are unknown. Methods The study retrospectively reviewed consecutive patients who had 1) acute middle cerebral artery (MCA) territory infarction, 2) diffusion- and perfusion-weighted imaging (DWI and PWI) and MR angiography within 24 h of onset, and follow-up DWI 5 days later, 3) stenosis (≥ 50%) or occlusion of MCA on baseline imaging, 4) a baseline PWI–DWI mismatch > 20%, and 5) either atherosclerotic MCA disease (MCAD) or cardioembolism (CE). National Institutes of Health Stroke Scale (NIHSS) scores and infarct volume at baseline and 5 days were obtained. Results Of 90 patients, 52 had MCAD and 38 had CE. At baseline, CE group had more severe stroke (median NIHSS, 9 vs. 5; p = 0.001) and larger infarct volume (median 8.32 cc vs. 3.0 cc; p = 0.034) than MCAD group. During the 1-week period, CE group had larger infarct volume growth (median 12.85 cc vs. 3.02 cc; p = 0.004) than MCAD group, although clinical improvement based on NIHSS (baseline minus 5-day) tended to be higher for CE than MCAD group (median 3 vs. 1; p = 0.08). The correlation between infarct volume and NIHSS score was stronger in CE ( r = 0.841) compared to MCAD ( r = 0.582) group at 5-day. Conclusions Substantial differences in the clinico-radiological evolution of acute ischemic stroke exist according to stroke mechanism. These data emphasize the importance of the stroke mechanism in the design of MRI-based acute stroke trials.

Radiological evolution in IMAGe association: A case report

IMAGe association is a recently recognized multi-system disorder of unknown etiology. IMAGe is a mnemonic acronym that stands for Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies (OMIM 300290). Suspicion for the disorder is readily raised by the distinctive clinical and endocrinological constellation, and radiological identification of metaphyseal dysplasia is crucial for the diagnosis. However, knowledge of the onset, evolution, severity, and variation of the metaphyseal dysplasia is currently limited. We illustrate the radiological evolution of an affected girl from her premature birth to early childhood. Her initial skeletal changes included thin ribs, delayed ossification of the juxtatruncal bones, and delayed epiphyseal ossification. The former two became less conspicuous during infancy. Metaphyseal dysplasia was not discerned at birth. However, mild metaphyseal cupping, sclerosis and longitudinal striations became manifest in late infancy, and then progressed with age. It is thought that the skeletal alterations in IMAGe association encompass retarded endochondral ossification normalized later on and mild metaphyseal dysplasia of postnatal onset.

Chondrocalcinosis and Hypomagnesemia: Clinical and Radiological Progression

Hypomagnesemia is a rare secundary metabolic disorder associated with calcium pyrophosphate dihydrate cristal deposition disease in joint structures and may cause asymptomatic chondrocalcinosis (linear calcification of cartilage), pseudogout, and chronic arthropathy. We report 2 young men with relapsing acute knee monoarthritis with chondrocalcinosis and hypomagnesemia. After follow-up clinical and radiological events al least for 5 years and treatment with magnesium lactate, these patients have not shown new pseudogout attacks. We discuss knee radiological evolution in both patients, outstanding major knee radiological deterioration in the patient with early symptoms and a familial chondrocalcinosis association, in spite of clinical asymptomatic status.

Pathological femur fracture secondary to radiation therapy for soft tissue sarcoma

Bone fracture is a well known possible late complication of radiation treatment. Little has been written about fractures of long bones after irradiation. We present a case of femur bone necrosis secondary to postoperative radiation for a soft tissue sarcoma of the thigh 20 years earlier. Fixation of the diaphyseal fracture and radiological evolution are described.

Treatment of Non-septic Femoral Head Necrosis with a Vascularized Iliac Crest Graft

We present our experience of the treatment of aseptic femoral head necrosis with a vascularized iliac crest graft. This was a retrospective study of 25 hips (22 patients) operated in our hospital between June 1993 and September 2002. There was a majority of men, 20 cases; and the left side was most frequently affected. The main predisposing factor was alcoholism, 8 cases. No predisposing factor was found in 5 cases. All the patients were assessed clinically according to the Harris scale and by X-rays according to the modified Ficat-Arlet staging system to determine necrosis progression. The mean follow-up was 4 years. Mean functional assessment was 87 points. Results were: excellent and good, 76%; fair and poor, 24%. Some degree of deterioration was detected on X-ray in 40% of the hips with a mean of 5.2 years. According to radiological assessment criteria satisfactory evolution was seen in 66%. Our results are comparable to those seen in the literature. No progression of necrosis was seen on X-rays in 60% of the patients operated. Presentamos nuestra experiencia en el tratamiento de la necrosis aséptica de la cabeza femoral con el injerto vascularizado de cresta ilíaca mediante el análisis retrospectivo de 25 caderas (22 pacientes) intervenidas en nuestro hospital entre junio de 1993 y septiembre de 2002. Predominio de hombres con 20 casos y del lado izquierdo. El factor predisponente más numeroso fue el abuso del alcohol en 8 casos. No se encontró en 5 casos. Todos los pacientes fueron evaluados clínicamente según la escala de Harris y radiológicamente se valoró la progresión de la necrosis según la clasificación de Ficat y Arlet modificada por la ARCO con un seguimiento medio de 4 años. La valoración funcional media fue de 87 puntos con un 76% de excelentes y buenos resultados y un 24% de regulares y malos resultados. El 40% de las caderas sufrió algún tipo de deterioro radiológico con una media de 5,2 años. Según los criterios de valoración radiológica, el 66% evolucionó satisfactoriamente. Nuestros resultados están en la línea de los publicados en la literatura sin evidenciarse la progresión radiológica de la necrosis en el 60% de los pacientes intervenidos.

Progressive vacuolating glycine leukoencephalopathy with pulmonary hypertension

To report two unrelated patients with a new phenotype of nonketotic hyperglycinemia associated with idiopathic pulmonary hypertension. Clinical findings included rapidly progressive neurological deterioration with onset in the first year of life characterized by developmental regression without seizures or electroencephalogram abnormalities during follow-up. Both patients died before the age of 18 months. Glycine cleavage system deficiency was confirmed by enzymatic studies in frozen liver. Molecular analysis in the related genes showed no pathogenic mutation. Radiological and pathological findings were consistent with progressive vacuolating encephalopathy. Our patients with biochemical and enzymatic parameters consistent with atypical nonketotic hyperglycinemia. The clinical and radiological evolution, as progressive vacuolating leukoencephalopathy and the association with pulmonary hypertension constitute a previously unrecognized variant.

Neumonectomía en atelectasia masiva post infección por adenovirus

We described the clinical and radiological evolution and pulmonary function tests in three patients aging 8 to 14 years old at present time. All of them suffered severe pneumonia caused by adenovirus infection during the infant period. During the acute viral disease, massive lung atelectasis refractory to treatment, determined the indication of pneumonectomy. All of the removed lungs presented bronchiectasis, atelectasis and fibrosis. In two children the medical condition improved after surgery, allowing to discontinue oxygen therapy. Wotwith standing, both of them had severe restrictive and obstructive limitation and signs of chronic damage at CTscan obstructive exacerbations decreased and the patients have been to carry out a normal life. The third patient has evolved with progressive clinical deterioration and pulmonary hypertension. The is under continuous oxygen therapy, because of extensive damage in the remaining lung. Being the pneumonectomy an exceptional procedure in children, in these patients the indication was due to medical treatment failure and progressive deterioration. Notwithstanding two children have shown, up to now, a satisfactory adaptation to this condition

Congenital achalasia: facts and fantasies

Achalasia, a poorly relaxing lower esophageal sphincter, produces a functional obstruction and the expected symptoms of dysphagia, regurgitation and eventually weight loss. The cause of achalasia remains largely unknown in Western countries, Chagas' disease being the most frequent etiology in Brazil. We report on two sets of monozygotic male twins with typical manifestations of achalasia. The majority of authors attribute a limited contribution unless achalasia is related to a multisystem disorder, like the triple-A or Allgrove's syndrome, an autosomal recessive disease characterized by the triad of adrenocorticotropic hormone (ACTH) resistant adrenal insufficiency, achalasia and alacrima. The four cases reported demonstrated the genetic influence of achalasia in patients without multisystem disorders. We believe that idiopathic achalasia is a syndrome with similar clinical, pathological, radiological and manometric evolution, but with a great variety of etiological agents, one of them being the congenital form.

Spontaneous collapse of the tibial plateau: radiological staging

This paper proposes a radiological staging system for necrosis of the tibial plateau, similar to those already developed for the hip and the medial femoral condyle. We retrospectively studied the clinical case histories and radiographic findings of 14 patients (15 affected knees) with histologically proven osteonecrosis of the tibial plateau. Stage I was marked by normal radiograph, but increased uptake in bone scan and subchondral areas of abnormal marrow signal intensity in magnetic resonance imaging (MRI), as reported in other osteonecrosis sites. Stage II was characterised by cystic and sclerotic changes, and stage III fracture of the medial rim of the medial tibial plateau and tibial plateau collapse were present. Stage IV was marked by joint narrowing. These changes appeared earlier and were more pronounced when there was genu varum/valgum or involvement of the lateral tibial plateau. The radiological evolution of the disease appears to follow a four-stage course over a period of roughly one year from the onset of symptoms.

Neumonía cavitada por Rhodococcus equi en paciente inmunocomprometido no infectado por virus de inmunodeficiencia humana: Caso clínico y revisión

Rhodococcus equi, is a grampositive intracellular bacillus, that causes infection mainly in immunocompromised hosts. We report the case of a 52 years old woman, with a systemic lupus erythematosus and a progressive 10 months evolution with cough, dyspnea, mucous-purulent sputum, occasionally hemoptysis, intermittent fever, and weight loss of 10%. With partial response to antibiotic treatment, radiological evolution of thoracic scanners evidenced the development of multiple bilateral areas of consolidation, some of them nodular. Percutaneous thoracic biopsy showed characteristic histology and microbiological analysis yielded the identification of Rhodococcus equi in both bronchoalveolar lavage and pulmonary biopsy. She received prolonged bi-associated antibiotic treatment with good clinical and radiological response. This agent must be considered in the study of immunocompromised patients that present with a prolonged evolution of pneumonia.

The vacuum cleft sign: an uncommon radiological sign

The intravertebral vacuum cleft sign (VCS) is an uncommon radiological sign, characterized by a radiolucent zone in the vertebral body. It is composed of 95% nitrogen and small amounts of oxygen and carbon dioxide. Post-traumatic ischemic necrosis could be its physiopathological mechanism, along with other pathologies like osteoporosis, corticosteroid therapy, diabetes, arteriosclerosis, alcoholism, multiple myeloma, bone metastasis and osteomyelitis. The broad diagnosis is made by antero-posterior X-ray, but computed tomography scan (CT scan) and magnetic resonance imaging (MRI) may help with the differential diagnosis. The aims of this paper are, on one hand, to communicate the clinical case of a 73-year-old osteoporotic woman with traumatic vertebral fractures who developed this sign in her radiological survey. On the other hand, its secondary aims are to review the medical literature about this sign and to show the clinical and radiological evolution after a percutaneous vertebroplasty.

Intralesional infiltration of corticosteroids in the treatment of localised langerhans’ cell histiocytosis of the mandible: Report of known cases and three new cases

Langerhans’ cell histiocytosis (LCH) can involve virtually any site and organ of the body, either as an isolated lesion or as a widespread systemic disease. Bone can be involved as a part of generalised disease or as a separate entity, formerly known as eosinophilic granuloma. The clinical cause of localised LCH (LLCH) to bone is generally benign. Solitary bone lesions may resolve spontaneously after diagnostic biopsy in a period of months to years. If treatment is indicated, as in larger lesions with pain, swelling and risk for spontanous fracture, the disease can be controlled by chemotherapy or local measures such as surgical curettage, low-dose radiotherapy and intralesional injection of corticosteroids. There are no controlled studies in the literature comparing outcomes of these treatment modalities because of low incidence of the disease and general tendency to regress. Three patients (female, 28 months; male, 9 years; male, 15 years) with LLCH of the mandible were treated in an one stage procedure with intralesional injection of 80, 40 and 80 mg methylpredisolone succinate, respectively, as the primary form of treatment. Patients were seen for clinical and radiological evolution 1, 3, 6, 9 and 12 months after treatment, and yearly thereafter. The overall outcome was excellent. The lesions showed clinically and radiologically complete remission approximately 6 months after treatment. There were no complications nor morbidity of the treatment. After a follow-up period of 12 months, the patients are well with no evidence of residual disease on radiographs. Local injection of corticosteroids should be the initial choice of treatment of LLCH of the mandible, because of the favourable treatment outcome in this disease and possible complications and lasting effects of surgery, radiotherapy and chemotherapy.

Malformación de Chiari tipo I: evolución postoperatoria a dos años. Análisis de 10 casos

OBJECTIVE To describe the clinical and radiological evolution of ten patients with Chiari I malformation (CIM), treated surgically. Ten patients with Chiari I malformation underwent suboccipital craniectomy, laminectomy of C1 and dural patch grafting. All of them were followed for at least two years. A magnetic resonance imaging was performed 6 months after surgery. Clinical preoperative evolution and time of installation, results of MRI and clinical postoperative evolution were correlated. In postoperative clinical evolution, we noted an important improvement in sensitive function, decrease of the syringohydromielia and partial improvement of cerebellar functions. Improvement of the motor alteration was less marked. During the two years after treatment the muscular atrophy did not change. Chiari I malformation is a congenital disease with many clinical manifestations which usually increase with time. A suitable diagnostic studies as well as an appropriate treatment are needed to improve the neurological results.

Enfisema pulmonar e cirurgia de redução de volume – a propósito de um caso clínico

Lung volume reduction surgery (LVRS) has been proposed as a palliative therapy in severe emphysema, with good results in lung function improvement, exercise capacity and quality of life. The authors present a case report of a 34 years old man with severe, heterogeneous lung emphysema submit to bilateral LVRS in 2000 January. Pre-surgical conditions, clinical and radiological evolution after surgery and for a 30 months follow up were analysed. Based on a literature revision, some technical aspects of LVRS, selection criteria and comparative study of medical and surgical therapy were discussed.

Actualización en el tratamiento de la neurocisticercosis

Neurocysticercosis is the most frequent central nervous system parasitosis, although quite infrequent in our country. Its rising incidence can be explained by immigration from regions of the world where this disease is endemic. This paper reviews treatment options for this condition. PATIENTS AND METHODS. Eight patients have been diagnosed with neurocysticercosis in the last three years in our hospital. Patient age, sex, origin, symptoms, CT and MR images, diagnostic tests, treatment and duration of medical treatment and clinical and neuroradiological evolution are examined. Patient age ranged from 25 to 33 years, all eight came from South America and the most frequent initial symptom was an epileptic crisis. Two patients had a single lesion, six had multiple lesions and all eight showed the lesion in the parenchyma. Only 50% showed a positive serum ELISA test for Taenia solium. Initial treatment was surgical in only one patient and the other seven received albendazol. In one of the latter the drug was ineffective and surgery was undertaken. A ten month follow up period has found a favorable evolution in all the patients, who are all also asymptomatic at the present time. Neurocysticercosis is more common among immigrants than the local population in Spain. The initial treatment should be medical, with surgery as an option for non responders. We believe that duration of the medical treatment will depend on the patient and the clinico radiological evolution of his/her disease.

Familial Infantile Bilateral Striatal Necrosis

The clinical and radiological evolution of familial infantile bilateral striatal necrosis (IBSN) was evaluated in 11 of 15 affected children born to consanguineous Israeli Bedouin parents and reported from the Schneider Children’s Medical Center, Petah Tikva and Sackler School of Medicine, Tel Aviv University, and other centers in Israel.

Reversible posterior leukoencephalopathy secondary to indinavir-induced hypertensive crisis: A case report

Reversible posterior leukoencephalopathy syndrome (RPLS) is an uncommon entity related to multiple and different pathologies, the most common being hypertensive crisis. It is believed to be secondary to the breakdown on the blood-brain barrier. At the beginning, it is undistinguishable from other leukoencephalopathies. However, the disappearance of brain lesions after removal of the potential cause, establish the differential diagnosis with other leukoencephalopathies. We present the case of an HIV-infected patient with a RPLS related to a hypertensive crisis short after the initiation of indinavir-containing highly active antiretroviral therapy. Once blood pressure was controlled and indinavir replaced by nelfinavir, white matter lesions at magnetic resonance imaging disappeared. The clinical and radiologic evolution excludes other diagnosis as progressive multifocal leukoencephalopathy and points indinavir as a potential hypertension-inducing agent in HIV-infected predisposed subjects.

Overview of the radiology, histology, and bone morphogenetic protein expression during distraction osteogenesis of the mandible.

Distraction osteogenesis (DO) is a form of in vivo tissue engineering during which an osteotomy and controlled distraction are used to lengthen bone. The molecular signals that govern distraction-induced bone formation have not been fully elucidated. Specifically, the role of bone morphogenetic proteins (BMPs) in DO of the mandible remains unclear. To characterize the radiologic and histologic evolution of newly formed bone during DO of the mandible and to relate these changes to the expression of BMPs. Fourteen skeletally mature male rabbits were used. A distractor device was surgically applied to one side of the mandible following osteotomy. After 1 week (latency period), distraction was started at a rate of 0.25 mm every 12 hours for 3 weeks (distraction period) and was followed by a 3-week consolidation period. Two animals were sacrificed each week after surgery (weeks 1 to 7). The mandible was resected and the new bone assessed by radiography and histology. The expression of BMPs was also analyzed using immunohistochemistry. There was radiographic and histologic evidence of bone formation during the distraction period. By week 6, there was mature woven bone within the distraction zone. Bone morphogenetic proteins 2 and 4 were strongly expressed in osteoblasts during distraction and in chondrocytes during consolidation. The expression of BMP-7 was relatively minor. The temporal and spatial pattern of BMP expression suggests that these proteins are important mediators of mandibular DO. Understanding the expression of BMPs may facilitate the use of recombinant proteins to enhance the rate and quality of bone generation during craniofacial DO.