Juvenile dermatomyositis and polymyositis: a follow-up study of long-term sequelae.

BACKGROUND: Juvenile dermatomyositis/polymyositis (DMS/PM) are childhood inflammatory myopathies that normally affect children between 2 and 15 years of age. The disease is manifested as severe proximal muscle weakness and, in JDMS, as a characteristic cutaneous eruption. It is difficult to predict the course of the disease. This study is aimed at objectively assessing the long-term morbidity of dermatomyositis/polymyositis in children. METHODS: Twelve cases of JDMS/PM identified through chart review were evaluated by physical and dermatologic examination, muscle testing, ultrasonography, and magnetic resonance imaging (MRI). RESULTS: The patient population included 6 male and 6 female patients with a mean age of 18.8 years (standard deviation 12.4). The mean age at onset of JDMS/PM was 5.8 years (SD 2.8), and the mean time from onset of symptoms to beginning of the study was 10.1 years (SD 9.3). The mean duration of corticosteroid therapy was 53.1 months (SD 40.5), with patients reporting an average of 1.7 relapses (SD 1.5). Patients compared their own activity level with that of healthy control subjects, and on a scale of 1 to 5 with 5 being normal, their mean score was 3.9 (SD 0.9). Fifty-eight percent of the patients had at least one residual finding on physical examination, and 78% of those with JDMS had residual dermatologic sequelae. Despite these findings, the mean grade for the group was 4.6 (SD 0.6) for muscle strength, using a scale of 0 to 5 with 0 being no muscle resistance and 5 being normal. In 60% of patients examined by ultrasonography, increased echogenicity indicated possible residual fibrotic change in at least one muscle group. Although MRI showed no active disease process in four patients, scattered intramuscular calcification was found in one patient. CONCLUSIONS: Multiple physical and dermatologic sequelae are commonly present in patients with JDMS/PM on long-term follow-up, but these do not preclude a productive life. Ultrasonography and MRI provide additional noninvasive means of analysis, but because of the current high cost, MRI is not routinely recommended.

Intraosseous pneumatocyst of the iliac bone.

Intraosseous pneumatocyst of the iliac bone is usually an innocuous incidental finding with characteristic radiographic features. Because this entity is uncommon and has not been described in the orthopaedic literature, many orthopaedic surgeons are unfamiliar with the entity. Three cases of intraosseous pneumatocyst of the iliac bone are presented to illustrate some of the imaging features to assist in recognizing it for what it is: an innocuous incidental finding that should not prompt invasive procedures such as a biopsy.

Magnetic resonance imaging of the musculoskeletal system. Part 4. The knee.

Since its introduction to musculoskeletal imaging in the early 1980s, magnetic resonance imaging has proven to be an excellent technique for evaluating patients with knee problems. Studies have shown it to be accurate in the identification of abnormalities of the menisci, ligaments, patellofemoral joints, and other soft tissue and osseous structures in the knee. The main advantages of magnetic resonance imaging are its noninvasive nature and its high accuracy and negative predictive value in evaluating the menisci and anterior cruciate ligament. Magnetic resonance imaging has been shown to be useful in the detection and diagnosis of various traumatic and nontraumatic knee abnormalities. It has also proved useful in the diagnosis of occult or unsuspected bone lesions. Magnetic resonance imaging can therefore help in the selection of those patients who need therapeutic arthroscopy. There is evidence that magnetic resonance imaging of the knee is a cost effective screening technique when used in conjunction with the clinical findings in patients who are candidates for arthroscopy. Magnetic resonance imaging of the knee is still a relatively expensive modality.

Synovial osteochondromatosis.

Primary or idiopathic synovial osteochondromatosis is an uncommon condition probably caused by synovial metaplasia and typically resulting in multiple calcified periarticular bodies. Synovial osteochondromatosis also may occur secondary to other joint disorders. This article reviews the pathogenesis, pathologic features, clinical features, and radiologic imaging of synovial osteochondromatosis. The differentiation of synovial osteochondromatosis from other conditions associated with periarticular calcified bodies is discussed.

Low-grade chondrosarcoma in a 12-year-old boy.

We present a case of a rare central chondrosarcoma in a 12-year-old boy and discuss its radiographic and magnetic resonance appearance.

Three-dimensional MRI of the glenoid labrum.

The objective of this study was to assess the accuracy of three-dimensional (3D) magnetic resonance imaging (MRI) reformation in the evaluation of tears of the glenoid labrum complex (GLC). Fifty-five shoulders were evaluated by MRI using standard spin-echo sequences. Gradient-refocused-echo axial projections were used to assess the GLC on the two-dimensional (2D) studies. Three-dimensional Fourier transform multiplanar gradient-recalled imaging with a resolution of 0.7 mm was also performed in all patients. Independent analyses of the anterior and posterior labra were performed in a blinded manner for both the 2D and 3D studies by three experienced musculoskeletal radiologists. Observations of the imaging studies were compared with the videoarthroscopic findings. The appearance of the GLC was rated on a scale of 0 to 4 (0-2 = normal, 3, 4 = abnormal or torn). The diagnostic confidence was averaged from the three reader's scores. Anterior labral tears were effectively detected with sensitivities of 89% and 96% and specificities of 96% and 100% (P < 0.0001) for the 2D and 3D studies, respectively. For posterior labral tears, the sensitivity and specificity of the 2D method were 47% and 98%, respectively. The sensitivity and specificity of the 3D volume sequence were 53% and 98%, respectively. The lower sensitivity of both imaging methods for detecting posterior labral tears may be influenced by the smaller number (n = 5) of arthroscopically confirmed cases in our study and reflects the difficulty of visualizing the posteroinferior borders of the GLC with present MRI techniques.

Type 4 acromion: a new classification.

The three types of acromial processes previously described in the literature include type 1, in which the undersurface is straight, and types 2 and 3, in which the undersurface is concave. In this report, a fourth type of acromion is described in which the undersurface is convex near its distal end. The frequency of a type 4 acromion in the normal population is discussed based on a retrospective review of the MR examinations of 30 patients that originally were read as normal.

MR diagnosis of superior labral anterior posterior (SLAP) injuries of the glenoid labrum: value of routine imaging without intraarticular injection of contrast material.

OBJECTIVE: Superior labral anterior posterior (SLAP) lesions are peculiar types of tears that involve the superior and anterior aspects of the glenoid labrum and extend posteriorly. The biceps tendon may also be involved in the injury. There have been conflicting reports in the literature about the value of MR imaging in depicting these injuries. Some authors have advocated imaging in special planes and, more recently, the use of intraarticular injection of contrast material to facilitate the diagnosis of SLAP injuries. Symptoms of SLAP injury of the glenoid labrum are nonspecific, and patients are often imaged according to routine imaging protocols that include images in axial, oblique coronal, and oblique sagittal planes using spin-echo pulse sequences. Intraarticular injection of contrast material is not part of routine MR imaging of the shoulder. We sought to determine the value of routine MR imaging without intraarticular contrast enhancement in the diagnosis of SLAP injuries of the glenoid labrum. MATERIALS AND METHODS: The MR images of eight patients with arthroscopically proved SLAP lesions were selected for this study. The MR imaging appearance of the glenoid labrum, biceps tendon attachment, and rotator cuff tendons were retrospectively evaluated in the axial, oblique coronal, and oblique sagittal planes in each case. The findings were correlated with the surgical observations. RESULTS: All eight patients showed an abnormal labrum on the coronal MR images, on 88% of the axial images, and on 50% of the sagittal images. An oblique or horizontal linear focus of increased signal was seen to traverse the labrum in 50% of the cases on sagittal images. Fifty percent of the cases also showed an intraarticular body, presumably representing the displaced fragment of a bucket-handle tear, on all imaging planes (the MR "Cheerio" sign). CONCLUSION: Routine MR imaging of the shoulder without intraarticular injection of contrast material is useful in the diagnosis of SLAP injuries of the glenoid labrum.

Comparison of axial T1 spin-echo and T1 fat-saturation magnetic resonance imaging techniques in the diagnosis of chondromalacia patellae.

To test the diagnostic value of T1 spin-echo and T1 fat-saturated magnetic resonance images (MRIs), we reviewed axial T1-weighted images with and without fat saturation in 20 patients with clinically suspected chondromalacia of the patella. All scans were obtained on 1.5-MR units. The scans were randomly ordered and reviewed independently at different times by two radiologists without knowledge of the arthroscopy results. The sensitivity of the individual techniques for detecting grade 3 or 4 chondromalacia patellae was 92% for fat-saturated axial T1-weighted images alone, and 67% for axial T1-weighted images without fat saturation. The sensitivity of the combined techniques was 100% for grades 3 and 4 and 90% for all grades (0 to 4). Chondromalacia patellae is diagnosed more accurately by using T1 fat saturation than by using T1 spin-echo images. With a combination of the two techniques, accuracy is 90% to 100%.

Isolated subacromial bursal fluid on MRI of the shoulder in symptomatic patients: correlation with arthroscopic findings.

Fluid in the subacromial bursa (SAB) is a common finding on magnetic resonance (MR) images of the shoulder, and the implications of this finding have not been clarified. We retrospectively reviewed and correlated the MR features with arthroscopic findings in 21 symptomatic patients who had fluid in the SAB on MR imaging without demonstrable rotator cuff tear. Rotator cuff impingement was the most frequent surgical finding (42.9%). Other frequent surgical observations were glenoid labrum abnormality (28.6%), bursitis (19%), and supraspinatus tendinitis (14.3%). Distribution of acromial types was similar to that reported by Bigliani et al., and impingement was evenly distributed among acromial types in our study population. We conclude that in our patient population group the MR finding of isolated SAB fluid in symptomatic patients is highly likely to be associated with the finding of other abnormalities in the shoulder joint at surgery.

Pattern of childhood pleural disease in Benin City, Nigeria.

Pleural disease is a frequent finding in childhood, occurring with a frequency of 12.8% in all childhood radiographs or 1.48% of all pediatric hospital admissions in the University of Benin Teaching Hospital in Nigeria. Pleural disease was found more frequently in males with a male:female ratio of 1.9:1. Pleural disease also occurred twice as commonly in the right hemithorax. Pleural calcification is uncommon in childhood. There was a seasonal variation in the occurrence of pleural disease with the highest numbers being seen in the month of July, which is the wettest month of the year in Benin. Pyogenic pneumonia and measles-related pneumonia remain the more frequent causes of pleural disease in childhood.

Appendiceal abscess--an unusual cause of acute urinary retention: a case report.

Acute urinary retention can result as a complication of gastrointestinal diseases such as diverticulitis and granulomatous enterocolitis. Appendiceal abscess has been reported as a rare cause of acute urinary retention in children but not in adults. A young adult male who had appendiceal abscess and presented in acute urinary retention forms the subject of this report.

Case report 789: Dorsal defect of the left patella.

Dorsal defect of the patella is an uncommon but well known abnormality. It is believed to be an incidental finding that should not merit further intervention. However, the association of patellofemoral symptoms and a dorsal defect of the patella suggests abnormality of the cartilage overlying the defect. Resection of this area of abnormal cartilage has resulted in relief of symptoms in some patients. A patient who had a dorsal defect of the patella and persistent symptoms had MR imaging. The MR scans showed signal inhomogeneity with streaks of high signal in the cartilage overlying the lesion on both T1-weighted and T2-weighted images. Drilling of the defect relieved his pain.

Diffuse abdominal wall cellulitis in ascending omphalitis--a lethal association in neonatal necrotizing fasciitis.

Necrotizing fasciitis is a grave complication of ascending omphalitis in the neonate, but because it is rare some of the signs are not well documented. Rapidly progressive anterior abdominal wall edema and cellulitis were noted in 16 patients who were clinically diagnosed with necrotizing fasciitis. Fifteen of these patients died soon after emergence of anterior abdominal wall edema and cellulitis. The appearance of anterior abdominal edema and cellulitis in a patient with ascending omphalitis has serious implications and demands intensive care if the patient is to survive.