Hospital For Special Surgery

 Inversion Recovery Coronal (A) and Axial (B, C). Please note B is slightly cranial to C in position.

 CT through the lung bases using a lung window

 CT through the upper mediastinum using a soft tissue window

 MRI of the lower extremities demonstrates high signal on the IR sequence indicating an edema pattern within the gluteus medius (left more than right) and then in both sets of adductor musculature.

CT of the lung bases demonstrates interstitial lung disease with reticulonodular opacities at the periphery and mild honeycombing. CT of the upper mediastinum demonstrates a patulous esophagus with fluid and air seen within the distended lumen.

Second case, same diagnosis: A 62-year-old woman with palpable mass of left elbow.

: Sagittal Proton Density MRI through the left elbow

 AP and lateral radiographs of the left elbow

Sagittal MRI demonstrates low signal masses in the posterior soft tissue that on radiographs corresponds with large, confluent, tumorlike calcification in the posterior soft tissue

Connective tissue disorder more prevalent in women than men that produces symptomatology generally in the second to third decade and continues to progress. Underlying pathology is a fibrosis that affects multiple organ systems. Classic findings include myositis of the proximal musculature that later precipitates fatty atrophy and large, conglomerate tumorlike or sheet like calcification of the soft tissue. It is frequently associated with other syndromes and produces an interstitial lung disease, preferentially at the lung bases, and esophageal dysmotility or a patulous esophagus.


• Resnick, D. Kranskdorf, M. Bone and Joint Imaging. 3rd Edition. 2005
• 

http://www.medicinenet.com/scleroderma/article.htm

http://www.emedicine.com/med/topic2076.htm


• Bassett LW, Blocka KL, Furst DE, Clements PJ, Gold RH. Skeletal findings in progressive systemic sclerosis (scleroderma). AJR Am J Roentgenol. 1981 Jun;136(6):1121-6

. ©2013 Hospital for Special Surgery.
Click here for more 

Articles

Scleroderma

Image 1: Inversion recovery midline sagittal; T2 midline sagittal; T1 midline sagittal

Image 2: T2 axial at the 4 level; T2 coronal

 Sagittal sequences demonstrate syndesmophyte formation along the anterior vertebral column. There is also increased signal in the lower intervertebral discs on the T1 and T2 sequences which in this situation corresponds to calcification of the disc. Coronal sequence demonstrates fusion of the sacroiliac joints and the axial sequence demonstrates dural ectasia and thecal diverticula.

Image 3: T2 midline sagittal; T1 midline sagittal

Image 4: T2 axial at the 4 level; T2 coronal

 Seronegative arthropathy that produces syndesmophytes (ossification of the annulus fibrosus) that start at the thoracolumbar junction and extend cranially and caudally, sacroilitis with eventual fusion, and enthesopathic change. In cases of marked disease and later in the time course dural ectasia may become manifest with associated cauda equina syndrome.

Image 5: Supplemental radiographs radiographs demonstrate syndesmophyte formation of the cervical spine as well as osseous bridging of the facet joints. Single view of the left knee demonstrates enthesopathic change along the course of the MCL.


• Resnick and Kransdorf. Bone and Joint Imaging. 2005.
• 

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17332972&itool=iconabstr&query_hl=3&itool=pubmed_docsum

http://www.rheumatology.org/public/factsheets/as.asp

Case History:  A 52-year-old woman with low back pain

Ankylosing Spondylitis

Surface coil high resolution, proton density, sagittal sequence

 Surface coil high resolution, proton density, axial sequence

 Three hour delayed spot images, Tc99 mDP bone scan

 Radiographs demonstrate a subtle loss of the normal femoral head neck offset but no focal lesion. MRI demonstrates marrow edema of the right femoral neck and a joint effusion. Subtle low signal focus is seen on the axial image and a labral tear is present on the sagittal sequence. CT demonstrates a subtle lucent focus of the anterior femoral cortex and there is increased activity focally at this location on the bone scan.

 Bony lesion of osteoid and immature bone that incites an adjacent reactive bony/inflammatory response secondary to prostoglandin release. Typically in younger patients (5-25 ) and with a classic clinical pattern of night time pain alleviated with aspirin. Classified as cortical, cancellous, and subperiosteal. This case presents the rarest type, a subperiosteal lesion. Current standard of care is radiofrequency ablation if possible or resection.

http://bonetumor.org/tumors/pages/page13.html

Lee, EH. Osteoid osteoma: A current review.

Osteoid Osteoma

 An 82-year-old man with longstanding bilateral hip pain. Patient has prior cervical spine surgery and no history of previous trauma.

Sagittal reformations of CT study about the midsagittal line

 Radiographs of the pelvis demonstrate markedly abnormal/dysplastic femoral heads with severe arthrosis of both hips, remodelled acetabulae, and foreshortened femoral necks with coxa vara deformity. Cervical spine radiographs demonstrate previous occipito-cervical fusion with poor delineation of the upper cervical spine architecture.

However, noted is a marked widening of the atlantodental interval when comparing the neutral to the flexion views. Cross sectional imaging of the cervical spine demonstrates decreased height of the vertebral bodies with increased AP diameter and prominent end plate irregularities. There are multiple non-fused ossification centers seen at the tip of the dens and with an overall decreased amount of bone at the dens than typically seen. Severe stenosis is seen at the C1/C2 junction with severe compression of the cord and high signal within the cord representing edema/myelomalacia. Underlying developmental spinal stenosis is also seen.

Deformed/Dysplastic femoral heads with severe arthrosis; Foreshortened necks with coxa vara

: Marked widening of atlantodens interval with flexicon; Previous posterior fusion; Decreased height, increased AP diameter adn irregular endplates of vertebrae

: Non-fused multipartite tip of the dens with overall hypoplasia of tip of the dens; Abnormal vertebrae as described previously

: Kinging or severe stenosis with compression of the cord at C1/C2

Severe stenosis of central canal with severe compression of the cord

 SED is an inherited dysplasia that involves the ends of the bones or epiphyses and the spine. It comes in two variants, congenita (present at birth) and tarda which has a normal appearance at birth and then develops at 4 years of age and older. Given the underlying dysplasia there is premature osteoarthritis which in this patient may have been neglected. In the spine, there is typically a hypoplastic dens which leads to spinal instability and as in this patient leads to fusion to help prevent a catastrophic event. The presence of an os odontoideum or non fused tip of the dens may be seen but is not as typically present.

The vertebral bodies are decreased in height and at times may be completely flat yielding platyspondyly. Ovoid or trapezoidal bodies in the pediatric patient typically than yield vertebrae in the adult with decreased height, increased AP diameter, and end plate irregularities as seen here. Severe stenosis or C1/C2 kinking may be found as compared to the typical cervicomedullary kinking found in achondroplasia. In this patient, no myelopathic symptoms were present, astonishingly so. Imaging of the other appendicular structures would have shown mutliple areas of epiphyseal dysplasia and advanced arthrosis.

Resources: Resnick. Diagnosis of Bone and Joint Disorders. 4th Ed. 2002


http://emedicine.medscape.com/article/1260836-overview

Eric Bogner, MD
Assistant Attending Radiologist, Hospital for Special Surgery
Assistant Professor of Radiology, Weill Cornell Medical College

Hospital For Special Surgery

Articles

Scleroderma

Ankylosing Spondylitis

Osteoid Osteoma

Spondyloepiphyseal Dysplasia (SED)