E. Michel Azouz

Synovial hemangioma: imaging features in eight histologically proven cases, review of the literature, and differential diagnosis

ObjectiveThis study was undertaken to describe the imaging characteristics of synovial hemangioma, with the goal of improving the disappointing rate (22%) of clinical diagnosis of this condition. A review of the literature and the differential diagnosis of intra-articular lesions, including synovial osteochondromatosis and pigmented villonodular synovitis, are also presented.PatientsThe subjects of the study were 8 patients (4 males, 4 females; age range: 5–47 years; mean age: 19 years) with histologically confirmed synovial hemangioma involving the knee (n=7) or wrist (n=1). We retrospectively examined the imaging studies performed in these patients, including plain radiography (n=8), magnetic resonance imaging (MRI; n=4), angiography (n=3), arthrography (n=2), and contrast-enhanced computed tomography (CT; n=2).ResultsPlain radiographs showed a soft tissue density suggesting either joint effusion or a mass in all patients. Phleboliths and bone erosions on plain films in four patients with extra-articular soft tissue involvement pointed to the correct diagnosis. Angiography, showing fine-caliber, smooth-walled vessels, contrast pooling in dilated vascular spaces, and early visualization of venous structures, was diagnostic in two patients. Neither arthrography nor CT yielded specific enough findings. MRI was consistently effective in allowing the correct diagnosis to be made preoperatively, showing an intra-articular or juxta-articular mass of intermediate signal intensity on T1-weighted images and of high signal intensity on T2or T2*-weighted images with low-signal channels or septa within it. A fluid-fluid level was found in two patients with a cavernous-type lesion.ConclusionDespite the limited nature of this study, it shows clearly that MRI is the procedure of choice whenever an intra-articular vascular lesion such as synovial hemangioma is suspected. Nonetheless, phleboliths and evidence of extra-articular extension on plain radiographs point to angiography as an effective procedure of first resort because it can be combined with embolotherapy.

Juvenile spondyloarthropathies: clinical manifestations and medical imaging

The spondyloarthropathies comprise four distinct entities — ankylosing spondylitis, psoriatic arthritis, the arthritis associated with inflammatory bowel disease, and Reiters syndrome and other related forms of reactive arthritis. Although these are distinct diseases, they have a number of clinical, radiologie, and genetic characteristics in common which permit them to be classified under the unifying term “spondyloarthropathy”. They are diseases of young adults, and when they present in patients under 16 years of age we refer to them as the “juvenile” spondyloarthropathies. They must be distinguished from juvenile rheumatoid arthritis, which is a totally separate entity; however the distinction may not always be obvious. Involvement of peripheral and sacroiliac joints commonly occurs in the juvenile spondyloarthropathies. The peripheral arthritis may be erosive and associated with bone apposition at the joint margins. Axial involvement is usually a late finding. Dactylitis and tenosynovitis are frequently present early on. Enthesitis, a highly specific feature, occurs much more often in the juvenile spondyloarthropathies than in the adult forms and it may be the only presenting feature. The plain radiograph is the primary and most important imaging modality for the assessment of these diseases. However, an expanding role of magnetic resonance imaging is evident.The spondyloarthropathies comprise four distinct entities — ankylosing spondylitis, psoriatic arthritis, the arthritis associated with inflammatory bowel disease, and Reiters syndrome and other related forms of reactive arthritis. Although these are distinct diseases, they have a number of clinical, radiologie, and genetic characteristics in common which permit them to be classified under the unifying term “spondyloarthropathy”. They are diseases of young adults, and when they present in patients under 16 years of age we refer to them as the “juvenile” spondyloarthropathies. They must be distinguished from juvenile rheumatoid arthritis, which is a totally separate entity; however the distinction may not always be obvious. Involvement of peripheral and sacroiliac joints commonly occurs in the juvenile spondyloarthropathies. The peripheral arthritis may be erosive and associated with bone apposition at the joint margins. Axial involvement is usually a late finding. Dactylitis and tenosynovitis are frequently present early on. Enthesitis, a highly specific feature, occurs much more often in the juvenile spondyloarthropathies than in the adult forms and it may be the only presenting feature. The plain radiograph is the primary and most important imaging modality for the assessment of these diseases. However, an expanding role of magnetic resonance imaging is evident.

Keutel syndrome: Further characterization and review

Keutel syndrome is a rare autosomal recessive disorder characterized by diffuse cartilage calcification, characteristic physiognomy, brachytelephalangism, peripheral pulmonary stenosis, hearing loss, and borderline to mild mental retardation. We report on an Arab boy with Keutel syndrome and cerebral calcifications identified at 15 years while investigating a seizure disorder. The parents are phenotypically normal first cousins. Thirteen cases in 9 families (including this case) have been published. Six families were consanguineous, two had multiple affected sibs (males and females) and 4 families originated from the Middle East.

Solitary lucent epiphyseal lesions in children

We evaluated retrospectively the varying radiographic appearances of 15 solitary lucent epiphyseal lesions occurring in children. Imaging modalities used included plain films, conventional tomography, nuclear scintigraphy, and computed tomography. Forty percent of the lesions (6) were due to osteomyelitis. The remaining lesions included tuberculosis (1), foreign body granuloma (1), chondroblastoma (2), chondromyxoid fibroma (1), enchondroma (1), osteoid osteoma (2), and eosinophilic granuloma (1). Although the radiographic appearances of such lesions may be particularly characteristic, pathologic correlation is frequently necessary. The high incidence of osteomyelitis in our cases emphasizes its importance as a cause for a lucent epiphyseal lesion.

Metastases to bone in medulloblastoma

Medulloblastoma is a relatively common intracranial neoplasm in childhood. Its extraneural spread was, until recently, thought to be a rare occurrence. Metastases are most commonly to bone. Five patients with medulloblastoma metastatic to bone are presented, and findings are compared to those of previous reports. Two of the five cases showed patchy extensive osteosclerosis of the pelvis and/or proximal femora. One case had concurrent lymph node involvement. In patients with past or present medulloblastoma and bone pain, metastases to bone should be excluded. Medulloblastoma metastatic to bone is a rare cause of extensive osteosclerosis.

Subperiosteal osteoid osteoma of the talus.

We report three patients with subperiosteal osteoid osteoma of the talus. All showed an erosion of the dorsal surface of the talus with medullary bone sclerosis. Adjacent paraosseous soft tissue calcification was seen in two lesions. Computed tomography demonstrated the nidus of the osteoid osteoma in two cases.

Arthrogryposis multiplex congenita, craniofacial, and ophthalmological abnormalities and normal intelligence: A new syndrome?

We report on an 8-year-old boy with clinical manifestations suggestive of a new arthrogryposis syndrome. These included characteristic craniofacial abnormalities, cleft palate, arthrogryposis multiplex congenita, pulmonary hypoplasia, cryptorchidism, and unusual ophthalmological findings. There was no intrauterine growth retardation or decreased fetal movements. Despite the poor prognosis expected in early life, the patient presented with normal mental capability on follow-up. Family data showed that a maternal first cousin of the mother (mothers brothers son) had similar findings and died in infancy. Differential diagnosis included Pena-Shokeir syndrome or phenotype, Gordon syndrome, Marden-Walker syndrome, and the syndrome of arthrogryposis with ophthalmoplegia and retinopathy. The possibility of autosomal dominant inheritance with reduced penetrance is suggested for this apparently new syndrome.

Inflammatory arthritis in children with osteochondrodysplasias.

Osteochondrodysplasias are a heterogeneous group of genetic skeletal dysplasias. Patients with these diseases commonly develop an early degenerative arthritis or osteoarthritis. Occasional observations of inflammatory arthritis have been made in this population but such observations are based on clinical grounds alone without confirmatory imaging studies. Four patients followed up in a paediatric rheumatology clinic with three different skeletal dysplasias, who had both clinical and radiological evidence of an inflammatory arthritis and coexistent degenerative arthritis, are described.

Imaging for bone and joint infection in children and adults

Bone and joint infection is certainly not a new disease. Despite our armamentarium of sophisticated antibiotics, the problem is not completely solved and permanent damage to bone or articular cartilage does occur, particularly in the growing child.‘,2 Clinical awareness, laboratory confirmation and accurate imaging tailored to the needs of individual patients are required to diagnose osteomyelitis and septic arthritis, establish the exact anatomic location(s), isolate the organism(s), and stage the disease. Once the location of the affected area is confirmed, aspiration and culture are the most useful tests to diagnose septic arthritis, as well as acute, and perhaps subacute, osteomyelitis.’ Aspiration may be performed blind or under fluoroscopic, ultrasonic, or computed tomographic guidance. In the acute phase, early clinical diagnosis, and immediate appropriate antibiotic treatment or intervention are crucial if one aims at eliminating the morbidity associated with bone and joint sepsis. Osteomyelitis or septic arthritis should be carefully looked for in the patient with diabetes, sepsis, sickle cell disease, intravenous drug abuse or after an open fracture, wound or the placement of an orthopaedic device. It may be haematogenous, e.g. in the patient with sepsis; or secondary to an open wound, fracture or surgery; or from a contiguous focus of soft tissue

Computed tomography in interstitial lung disease

The number and scope of published articles dealing with computed tomography of interstitial lung disease are limited. We present seven cases in which computed tomography detected the presence or extent of interstitial lung disease better than conventional radiography: two patients with histiocytosis X, one with bronchopulmonary dysplasia, one with bleomycin lung toxicity, and three with radiation-induced lung injury. The computed tomography appearance of histiocytosis X and bronchopulmonary dysplasia have not been previously described. Transverse computed tomography images provide information regarding stage of activity and nature of interstitial lung processes not available with standard imaging techniques. We advocate the use of computed tomography in the initial investigation and follow-up of patients with histiocytosis X. Post-radiation pneumonitis and fibrosis can be detected earlier with computed tomography as well.