[Unusual presentation of rib malformation]. / Présentation inhabituelle d'une malformation costale.

Rib malformation and anatomical variations are not well known and are still often underdiagnosed. Usually, rib malformations are fortuitously discovered. We describe here the case of a girl, 4 years and 4 months old, who presented at the emergency unit for fever and an anterior tumefaction of the ribcage, without any other symptoms. She was eupneic with a normal pulmonary auscultation and viral tonsillitis with a negative streptococcus test. The thoracic tumefaction was parasternal, painless, and fixed and measured approximately 2.5 × 2cm. Ultrasound findings consisted of a duplicated and hypoechogenic hypertrophy of the sterno-costal cartilage of the 4th left rib. Magnetic resonance imaging (MRI) confirmed the diagnosis of chondral bifidity of the sterno-costal junction of the 4th left rib. Fever, due to the viral tonsillitis, disappeared after 4 days. Rib malformations are rare, often anterior, unilateral, and preferentially located on the 3rd or the 4th rib. The main malformative rib lesions are bifid ribs, rib spurs, and widened ribs. Very rarely, they can be associated with Gorlin-Goltz syndrome or with other malformations such as VATER complex. The main differential diagnoses of these rib malformations are traumatic, tumoral, and infectious etiologies. In case of tumoral diseases, the topography of the lesion focuses the etiologic diagnosis: whereas an anterior and cartilaginous lesion is always benign, a lateral or posterior lesion can be an Ewing sarcoma. Rib malformation investigation consists in meticulous questioning, a complete clinical examination looking for any associated anomaly, completed by basic imaging explorations such as plain thoracic radiography focused on the ribcage and ultrasound. Finally, complementary computerized tomography or preferably MRI, depending on the anatomic location of the lesion, confirms the final diagnosis, as presented in our case report, and removes any uncertainty.

Bone erosion of the sternocostal joint in a patient with Behcet's disease.

Behcet's disease (BD) is a polysymptomatic and recurrent systemic vasculitis with a chronic course and unknown cause. Erosive arthropathy is extremely rare. We report a 52-year-old female patient with BD demonstrating bone erosion of the sternocostal joint.

Imaging of sternocostoclavicular joint in spondyloarthropaties and other rheumatic conditions.

OBJECTIVE: To retrospectively evaluate the role of the various imaging techniques in the study of the sternocostoclavicular joint, in patients with spondyloarthropathies and other rheumatic conditions and to assess potential pitfalls in the radiological diagnosis. SUBJECTS AND METHODS: Thirty patients, 11 male and 19 female, mean age 45 years, with involvement of the sternocostoclavicular joint as part of rheumatologic disorders (psoriatic arthritis, ankylosing spondylitis, Tietze syndrome, SAPHO syndrome, and condensing osteitis of the clavicle) were studied. Conventional radiography, CT, MRI and bone scintigraphy were performed. The following imaging findings were evaluated: soft tissue swelling, bone sclerosis, cortical bone erosions, joint space narrowing, subchondral sclerosis, periosteal new bone formation, synovial reaction and intrarticular effusion. All the images were independently reviewed by two musculoskeletal radiologists. RESULTS: Conventional radiography demonstrated only sclerosis of the clavicula in 8 pts (26%) and the sternum in 3 pts (10%), cortical bone erosions in 7 pts (23%), joint space narrowing in 6 pts (23%) and periosteal bone formation in 2 pts (10%). At the CT examination sclerosis of the clavicula and the sternum was observed in 13 pts (44%), cortical bone erosions in 22 pts (76%), joint space narrowing in 10 pts (34%), ligament ossification in 12 pts (41%), subchondral sclerosis in 9 pts (34%) and periosteal bone formation in 10 pts (34%). The MRI was the most sensitive technique in the evaluation of the soft tissue swelling in 9 pts (56%), intrarticular effusion in 13 pts (81%) and synovial reaction in 13 pts (81%). Finally, bone scintigraphy showed an increased uptake at the sterno-costoclavicular joint in all patients who underwent the examination. CONCLUSION: The radiological evaluation of the anterior chest wall in patients with different rheumatic disorders represents a problem of difficult diagnostic evaluation both for the anatomic region complexity and for the variability of the radiographic findings. The integrated use of X-ray, CT, MRI and nuclear medicine is suggested to avoid misdiagnosis.

Magnetic resonance imaging in Tietze's syndrome.

OBJECTIVE: To evaluate the usefulness of magnetic resonance imaging (MRI) in Tietze's syndrome which, to our knowledge, has not previously been reported in the literature. METHODS: Twelve consecutive outpatients with clinical features of Tietze's syndrome underwent evaluation, including the anamnesis, clinical general examination, clinical evaluation of costosternal and sternoclavicular joints (SCJ) and biochemical and instrumental investigations. Twenty normal subjects age- and sex-matched to the patients' group were examined in a similar manner. MRI of costosternal and SCJ was performed using a 1.5 Tesla unit (Gyroscan NT 1.5 Philips, The Netherlands and GE Signa Excite HD, GE Healthcare, Milwaukee, Wis., USA). RESULTS: The MRI pattern of primary Tietze's syndrome was characterized as follows: enlargement and thickening of cartilage at the site of complaint (12/12 patients); focal or widespread increased signal intensities of affected cartilage on both TSE T2-weighted and STIR or FAT SAT images (10/12 patients); bone marrow oedema in the subcondral bone (5/12 patients); vivid gadolinium uptake in the areas of thickened cartilage, in the subcondral bone marrow and/or in capsule and ligaments (10/12, 4/12 and 7/12 patients respectively). CONCLUSION: Magnetic resonance is an excellent technique to evidence both the cartilage and bone abnormalities, therefore it represents the elective method in the investigation of primary Tietze's syndrome, due to its high sensitivity, diagnostic reliability and biological advantages thanks to the lack of ionizing radiation.

Imaging of the seronegative anterior chest wall (ACW) syndromes.

The sternocostoclavicular (SCC) region is subject to the same diseases that occur in joints, with congenital and developmental anomalies, inflammatory and infectious diseases, soft tissue and bone tumors as well as the seronegative spondyloarthropathies, including ankylosing spondylitis, reactive arthritis, enteropathic arthritis, psoriatic arthritis, pustulosis palmoplantaris and other syndromes. Most of these conditions present with swelling of the joint, which may be associated with pain and/or tenderness. These disorders are characterised by onset usually before the age of 40 years, absence of serum autoantibodies and sometimes the association with antigen human leukocyte antigen B27. Traditional X-ray study is indicated in the initial evaluation of SCC joint disorders, but other imaging modalities typically are often necessary to clarify the pathology. Computed axial tomography scans are indicated for disease processes in which bony destruction or ossification may occur. Magnetic resonance imaging provides more detailed and useful information when evaluating suspected pathology involving inflammation or soft tissue mass. Bone scintigraphy can help to correlate active inflammation of the SCC joint with symptoms of pain. The purpose of this study is to introduce the clinical and radiological aspects of the seronegative anterior chest wall diseases, particularly the contribution of the different imaging techniques.

Tophaceous gout of the first costochondral junction in a heart transplant patient.

We report the case of a 49-year-old man with a 10-year history of gout, who presented with a painful left first costochondral junction mass. A computed tomography (CT)-guided biopsy of the mass revealed foreign body giant cell reaction and crystalline deposition consistent with tophaceous gout.

First sternocostal degenerative arthritis with intrarticular fluid collection. A case report.

A rare case with clinical condition of first sternocostal degenerative arthritis with intra-articular fluid collection that developed after long-lasting intense exercise (weight-lifting) for twenty years is reported. Imaging findings and differential diagnoses of the case are presented.

[SAPHO syndrome: clinico-rheumatologic and radiologic differentiation and classification of a patient sample of 86 cases]. / Das SAPHO-Syndrom: Klinisch-rheumatologische und radiologische Differenzierung und Klassifizierung eines Krankengutes von 86 Fällen.

Synovitis (inflammatory arthritis), acne (pustulosa), pustulosis (psoriasis, palmoplantar pustulosis), hyperostosis (acquired), and ostitis (bland osteomyelitis) are symptoms forming the acronym SAPHO, which is a syndrome of nosologic heterogeneity. All entities forming the SAPHO syndrome are connected by a non-obligate dermatoskeletal association with an aseptic pustulous character. 86 cases were analyzed clinically, radiologically and by histology/histopathology. 31 adult patients showed the typical triad of pustulosis palmo-plantaris (psoriatica, PPP), sterno-costo-clavicular hyperostosis (SCCH), and "productive" spondylopathy, which we define as entity I. spondarthritis hyperostotica pustulopsoriatica (Spond.hyp.pp). Twelve adolescent and 13 adult patients showed entity no. II: chronic recurrent multifocal osteomyelitis (CRMO), being characterized by non-purulent osteomyelitis of plasma-cell sclerotic type, potentially being a reactive inflammatory process. 50% of the adult patients with CRMO showed PPP. Differentiation between these two entities is possible by detection of ossifying enthesiopathy in cases of Spond. hyp.pp and primarily chronic osteomyelitis in cases of CRMO. Two more entities or abortive forms of group I and II are III: the inflammatory syndrome of the anterior chest-wall (ACW syndrome) and IV: the more productive form of isolated sterno-costoclavicular hyperostosis (SCCH). Both are connected quite frequently to HLA-B-27-independent forms of spondarthritis and to pustulous dermatosis. More rarely we find osteo-articular symptoms in cases of acne pustulosa, which form group V: acne-associated spondarthritis and CRMO in the case of acne. Adult forms of CRMO with different forms of appearance (lumosacro-iliac hyperostosis with retroperitobeal fibrosis, pelvic type with affection of the hip-joint) are described. The immunologic theory of a "reactive osteomyelitis" potentially triggered by saprophytes is described. The inverse acne triad is brought in a context of skin symptoms. A case of intercurrent postpartum symptoms together with ulcerative colitis is described. Three cases of patients with Crohn's disease are described. Clinical features, radiological findings, and histopathological elements are brought together to determine the connections between the different entities and the possibilities of differentiation. With these elements together with bone-scan, it is often not necessary to obtain a bone specimen. Therapeutical possibilities, especially concerning CRMO, are discussed. "SAPHO syndrome" is more a sign-post on the way to a more subtle diagnosis when it comes to hyperostotic, skin-associated diseases, and it needs interdisciplinary work to clear the situation.

[Front chest pain and paresis of recurrent nerve: sapho syndrome]. / Dolor torácico anterior y paresia del nervio recurrente: síndrome sapho.

The AA. present a rarely encountered case combining a left recurrent palsy due to an sclerosis and hyperostosis of the first costoesternal joint, which improved after an antiinflammatory treatment.

Anterior chest wall malignancies. A review of ten cases.

OBJECTIVES: To report the features of malignancies responsible for a chest wall mass and involving the sternum, the sternocostal and/or sternoclavicular joints, the chondrocostal junction and/or the adjacent soft tissues. METHODS: The medical records of patients with a chest wall mass due to malignant disease were reviewed retrospectively. The following data were abstracted from each record: characteristics of the pain and mass, constitutional symptoms, physical findings, laboratory test results, findings from imaging studies (plain radiographs, computed tomography and magnetic resonance imaging of the chest, radionuclide bone scan), histologic features of the biopsy specimen from the chest wall mass and origin of the mass. RESULTS: Seven men and three women with a mean age of 53.1 years were included in the study. A single patient had a history of malignant disease (lymphoma); in the remaining nine patients the chest wall mass was the first manifestation of the malignancy. All ten patients had pain with a mixed time pattern. The mass was located on the sternum in half the patients and in a parasternal location in the other half. Erythrocyte sedimentation rate elevation was found in seven patients, an increased serum level of lactate dehydrogenase in one and a monoclonal immunoglobulin in three. Sternal lesions were visible on plain radiographs in four patients. Computed tomography of the chest consistently disclosed sternal or sternocostal lytic lesions with spread to the adjacent soft tissues; in five cases, enlarged lymph nodes were visible in the anterior part of the mediastinum. Magnetic resonance imaging of the chest did not add to the information provided by computed tomography. Radionuclide uptake on the bone scan was increased, decreased, or normal at the site of the lesion. The cause was Hodgkin's disease in two cases, non-Hodgkin's lymphoma in three, metastatic bone disease in two (from an adenocarcinoma of the lung and a hepatocarcinoma, respectively), multiple myeloma in one, and solitary plasmacytoma in two. CONCLUSION: A chest wall mass can be caused by a known or as yet undiagnosed malignancy. Chest wall involvement due to malignant disease in rare, however. The specific features of sternal metastases, lymphomas involving the sternum, and sternal plasmacytomas are discussed. Nonmalignant chest wall lesions that can manifest as a bulging or swelling of the chest wall are reviewed.

[Degenerative chondroarthropathy of the sternocostal joint following heart surgery]. / Degeneratív chondroarthropathia a sternocostalis ízületeken szívmütétek után.

Involvement of the sternocostal joints was investigated in a series of 46 males and 18 females following median sternotomy annually in a 5-year period and compared to 62 age- and sex matched control subjects after one year solely. Both groups had a mean age of 49.2 years. The degenerative chondroarthropathy of sternocostal joints was 1.69-times more frequent in heart operated upon patients as compared to control persons. Based on radiographic findings the degenerative chondroarthropathies were classed in 0 to 3 severity groups. Were seen articular space narrowing in 95.4%, osteophytes of the margin of the articular surface in 88%, subchondral bony eburnation in 79% and cystic radiolucencies in 48.9% of sternocostal joints on poststernotomy standard plain film tomograms. Not occurred intraarticular gas phenomenon and bony ankylosis. The development of arthropathies is traced back to mechanical stress-related predisposing factors and stressed the importance of oculoneutral dehiscences that simulated normal roentgenanatomic projections and caused a masked insufficiency in sternocostal junctions.

The acquired hyperostosis syndrome: a little known skeletal disorder with distinctive radiological and clinical features.

The acquired hyperostosis syndrome (AHS) is a chronic inflammatory disorder of bone of unknown etiology. It is accompanied by circumscribed hyperostosis which can be associated with ossifying lesions at sites of tendinous and ligamentary insertions and erosive or non-erosive arthritis. The predominant location of lesions is the sternocostoclavicular region (approximately 80% of patients), less frequent are involvement of the spine, pelvis, and appendicular skeleton. In 20%-60% of cases AHS is associated with palmoplantar pustulosis, psoriasis, or severe acne (acne fulminans or conglobata). The X-ray appearance of AHS is a more or less homogeneous increase in density with blurred margins, which on scintiscan with labeled phosphate compounds is associated with intense accretion of tracer. These features are associated with a variable increase in the acute phase reactants and a conspicuously low increase, if any, in serum alkaline phosphatase. The therapeutic modalities which have been used so far are entirely symptomatic. Long-lasting improvement has been reported following percutaneous anti-inflammatory radiation therapy.

Acquired hyperostosis syndrome: spectrum of manifestations at the sternocostoclavicular region. Radiologic evaluation of 34 cases.

Thirty-four patients with chest wall hyperostosis, a condition which has been designated by various terms in the literature were evaluated radiologically. We prefer the name acquired hyperostosis syndrome (AHS), which we categorize into the complete, incomplete and possible form. In complete AHS, sternocostoclavicular hyperostosis is associated with axial and/or peripheral (endosteal, periosteal, enthesopathic, metaplastic) hyperostosis and with psoriasiform or acneform dermatosis. In addition, these three manifestations are accompanied by erosive or non-erosive peripheral and/or axial arthritis to a variable degree. Sometimes, concomitant findings which are consistent with ankylosing spondylitis are also to be found in the axial skeleton. AHS is manifested at 11 different sites on the anterior chest wall. Ossification forms of the costal cartilage, inflammatory enthesopathies (three different insertions) and focal hyperostoses as well as processes of remodelling of the ribs, clavicles and sternum which are described in detail have particular diagnostic significance. AHS can start simultaneously at one, two or several sites on the anterior chest wall. Conventional tomography (possibly supplemented by CT) is necessary for early diagnosis and for analysis of the various findings on the anterior chest wall.

Sternocostal joints. Anatomic, radiographic and pathologic features in adult cadavers.

Using radiographic-pathologic correlation, we studied the sternocostal joints derived from 27 consecutive cadavers and one additional cadaver with rheumatoid arthritis. Radiographic findings that were tabulated included joint space narrowing, sternal or costal osteophytes, articular calcification, vacuum phenomena, and the degree of ossification of the costal cartilages. The first sternocostal joint could be classified as either a synchondrosis or synostosis in every instance; however, a joint cavity lateral to the first sternocostal joint represented a normal variation and was seen radiographically in 10 specimens. The second sternocostal joint was synovial in type and intimately related to the manubriosternal joint; cavitation within this joint was present bilaterally in 36% of our specimens. Degenerative changes in the sternocostal articulations were characterized much more frequently by sternal osteophytes than by costal osteophytes or joint space narrowing. Calcification compatible with chondrocalcinosis was observed in two cadavers. Radiographic and pathologic evidence of synovial inflammation was evident in the sternocostal joints of the rheumatoid specimen.

Symptomatic manubriosternal joint involvement in rheumatoid arthritis.

The manubriosternal joint is commonly involved in rheumatoid arthritis but rarely gives rise to symptoms. A patient is reported with seropositive, erosive rheumatoid arthritis, who developed symptoms resembling pleuritic pain, arising from synovitis of the manubriosternal joint. Treatment with intra-articular steroid injection resolved these symptoms rapidly.

Pathogenesis of discovertebral and manubriosternal joint abnormalities in rheumatoid arthritis: a cadaveric study.

The precise pathogenesis of discovertebral lesions in rheumatoid arthritis has long been debated with the emergence of 2 conflicting theories: discovertebral extension of synovial inflammation from a neighboring articulation (Luschka, costovertebral joint) and repetitive discal trauma with Schmorl's nodes. In order to address this controversy, we performed a radiographic-pathologic correlation of a rheumatoid cadaver and compared the alterations seen in the cervical and lumbar discovertebral junction; we further included an evaluation of the manubriosternal joint, another cartilaginous articulation. Our data confirmed that advanced discovertebral lesions in the cervical and lumbar spine are related primarily to endplate failure that itself is caused by apophyseal joint destruction and segmental spinal instability, although inflammatory rheumatoid lesions of the cervical intervertebral discs probably arising from the Luschka joints are also evident. Conversely, the lesions of the manubriosternal joint were produced by inflammatory changes related to the development of a synovial cavity in the articulation.