Atlas of MRI findings of sacroiliitis in pediatric sacroiliac joints to accompany the updated preliminary OMERACT pediatric JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system: Part I: Active lesions.

Magnetic resonance imaging (MRI) is an increasingly important tool for identifying involvement of the sacroiliac joints (SIJ) in juvenile idiopathic arthritis (JIA). The key feature for diagnosing active sacroiliitis is bone marrow edema (BME), but other features of active arthritis such as joint space inflammation, inflammation in an erosion cavity, capsulitis and enthesitis can be seen as well. Structural changes may also be seen. Systematic MRI assessment of inflammation and structural damage may aid in monitoring the disease course, choice of therapeutics and evaluating treatment response. In this pictorial essay, we illustrate normal MRI findings and growth-related changes of the SIJ in the pediatric population, as well as the different MRI features of SIJ inflammation. This atlas demonstrates fundamental MRI disease features of active inflammation in a format that can serve as a reference for assessing SIJ arthritis according to the updated preliminary JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system proposed by the MRI in JIA working group of Outcome Measures in Rheumatology and Clinical Trials (OMERACT). The atlas is intended to be read in conjunction with its companion Part 2, Structural Lesions.

Atlas of MRI findings of sacroiliitis in pediatric sacroiliac joints to accompany the updated preliminary OMERACT pediatric JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system: Part II: Structural damage lesions.

Magnetic resonance imaging (MRI) is the imaging modality of choice for identifying sacroiliitis in juvenile idiopathic arthritis (JIA). Besides active lesions of sacroiliitis, of which bone marrow edema (BME) is the key feature, structural damage lesions can also be detected. Structural changes include erosion, sclerosis, fat lesion, backfill and ankylosis, and are more common at later stages. Systematic MRI assessment of inflammation and structural damage may aid in monitoring the course of the disease and evaluating treatment options. In this pictorial essay, we illustrate normal MRI findings and growth-related changes of the SIJ in the pediatric population, as well as the different MRI features of structural damage of sacroiliitis. This atlas can serve as a reference for assessing structural lesions of SIJ arthritis according to the updated preliminary JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system proposed by the MRI in JIA working group of Outcome Measures in Rheumatology and Clinical Trials (OMERACT). The atlas is intended to be read in conjunction with its companion Part 1, Active Lesions.

Common incidental findings on sacroiliac joint MRI in children clinically suspected of juvenile spondyloarthritis.

PURPOSE: To determine the prevalence of incidental findings on sacroiliac (SI) joint MRI in children clinically suspected of Juvenile Spondyloarthritis (JSpA). METHODS: In this retrospective multi-center study of 540 children clinically suspected of JSpA who underwent MRI of SI joints from February 2012 to May 2018, the prevalence of sacroiliitis and other incidental findings was recorded. RESULTS: In 106/540 (20 %) children MRI features of sacroiliitis were present. In 228 (42 %) patients MRI showed at least one incidental finding other than sacroiliitis. A total of 271 abnormal findings were reported. The most frequent incidental findings were at lumbosacral spine (158 patients, 29 %) and hip (43 patients, 8 %). The most common incidental finding was axial degenerative changes, seen in 94 patients (17 %). Other less frequent pathologies were: simple (bone) cyst in 15 (2,8 %) patients; enthesitis/tendinitis in 16 (3 %) patients; non-specific focal bone marrow edema (BME) away from SI joints in 10 (1,9 %) patients; ovarian cysts in 7 (1,3 %) patients; BME in the course of chronic recurrent multifocal osteomyelitis (CRMO) in 4 (0,7 %) patients; muscle pathology in 4 (0,7%) patients; benign tumors in 3 (0,6 %) patients; (old) fractures in 3 (0,6 %) patients; bony apophyseal avulsion in 2 (0,4 %) patients and malignant tumors in 2 (0,4 %) patients. CONCLUSION: Incidental findings are common on MRI of the SI joints in children clinically suspected of JSpA, particularly at the lumbar spine and hips. They are seen even more frequently than sacroiliitis and can be relevant, as some will have clinical significance or require treatment.

Massive pneumomediastinum with subcutaneous emphysema after elective adenotonsillectomy in children: Involvement of the Boyle-Davis mouth gag.

Adenotonsillectomy, a very common surgical procedure in otorhinolaryngology, is considered easy and safe surgery. However, clinicians should be aware of some less common but potentially life-threatening complications. This report discusses subcutaneous emphysema with pneumomediastinum following elective adenotonsillar surgery in children. The Boyle-Davis mouth gag seemed to play a part in the pathogenic mechanism of this rare complication in this case. Better insights in the mechanism of this severe complication of adenotonsillectomy may contribute to the prevention of this complication.

MRI of the sacroiliac joints in spondyloarthritis: the added value of intra-articular signal changes for a 'positive MRI'.

OBJECTIVE: To determine if intra-articular signal changes at the sacroiliac joint space on MRI have added diagnostic value for spondyloarthritis, when compared to bone marrow edema (BME). MATERIALS AND METHODS: A retrospective study was performed on the MRIs of sacroiliac joints of 363 patients, aged 16-45 years, clinically suspected of sacroiliitis. BME of the sacroiliac joints was correlated to intra-articular sacroiliac joint MR signal changes: high T1 signal, fluid signal, ankylosis and vacuum phenomenon (VP). These MRI findings were correlated with final clinical diagnosis. Sensitivity (SN), specificity (SP), likelihood ratios (LR), predictive values and post-test probabilities were calculated. RESULTS: BME had SN of 68.9%, SP of 74.0% and LR+ of 2.6 for diagnosis of spondyloarthritis. BME in absence of intra-articular signal changes had a lower SN and LR+ for spondyloarthritis (SN = 20.5%, LR+ 1.4). Concomitant BME and high T1 signal (SP = 97.2%, LR + = 10.5), BME and fluid signal (SP = 98.6%, LR + = 10.3) or BME and ankylosis (SP = 100%) had higher SP and LR+ for spondyloarthritis. Concomitant BME and VP had low LR+ for spondyloarthritis (SP = 91%, LR + =0.9). When BME was absent, intra-articular signal changes were less prevalent, but remained highly specific for spondyloarthritis. CONCLUSION: Our results suggest that both periarticular and intra-articular MR signal of the sacroiliac joint should be examined to determine whether an MRI is 'positive' or 'not positive' for sacroiliitis associated with spondyloarthritis.

MR signal in the sacroiliac joint space in spondyloarthritis: a new sign.

OBJECTIVES: To determine the diagnostic value of MR signal within the sacroiliac (SI) joint space in spondyloarthritis (SpA). METHODS: A retrospective analysis of MRIs of SI joints was performed in 363 patients, aged 16-45 years, clinically suspected of sacroiliitis. Intra-articular SI joint MR signals were categorized as normal, high T1 signal, fluid signal, ankylosis or vacuum phenomenon (VP). These MRI findings were correlated with the final diagnosis, according to the ASAS criteria. Sensitivity, specificity, and positive and negative likelihood ratios (LR) and predictive values were calculated. RESULTS: Presence of intra-articular high T1 signal, fluid signal and ankylosis had a specificity of 95.8 %, 95.3 % and 99.5 % for SpA. High T1 signal, fluid signal and ankylosis were present in 38.4 %, 19.2 % and 17.9 % of SpA patients and in 4.2 %, 4.7 % and 0.5 % of patients without SpA, resulting in LR+ of 9.0, 4.1 and 37.9, respectively. VP was present in 13.2 % of SpA patients and in 20.8 % of patients without SpA, resulting in an LR+ of 0.6. CONCLUSIONS: Presence of high T1 signal, fluid signal and ankylosis within the SI joint on MRI have high specificity for SpA. High T1 signal is the most sensitive MRI feature within the SI joint for SpA. KEY POINTS: • MRI of the SI joints is typically obtained for diagnosis of spondyloarthritis. • The MR signal within the SI joint itself reflects features of spondyloarthritis. • Intra-articular high T1 signal, fluid signal and ankylosis are seen in spondyloarthritis. • The vacuum phenomenon makes spondyloarthritis less likely.

Diagnositic value of pelvic enthesitis on MRI of the sacroiliac joints in enthesitis related arthritis.

BACKGROUND: To determine the prevalence and diagnostic value of pelvic enthesitis on MRI of the sacroiliac (SI) joints in enthesitis related arthritis (ERA). METHODS: We retrospectively studied 143 patients aged 6-18 years old who underwent MRI of the SI joints for clinically suspected sacroiliitis between 2006-2014. Patients were diagnosed with ERA according to the International League of Associations for Rheumatology (ILAR) criteria. All MRI studies were reassessed for the presence of pelvic enthesitis, which was correlated to the presence of sacroiliitis on MRI and to the final clinical diagnosis. The added value for detection of pelvic enthesitis and fulfilment of criteria for the diagnosis of ERA was studied. RESULTS: Pelvic enthesitis was seen in 23 of 143 (16 %) patients. The most commonly affected sites were the entheses around the hip (35 % of affected entheses) and the retroarticular interosseous ligaments (32 % of affected entheses). MRI showed pelvic enthesitis in 21 % of patients with ERA and in 13 % of patients without ERA. Pelvic enthesitis was seen on MRI in 7/51 (14 %) patients with clinically evident enthesitis, and 16/92 (17 %) patients without clinically evident enthesitis. In 7 of 11 ERA-negative patients without clinical enthesitis but with pelvic enthesitis on MRI, the ILAR criteria could have been fulfilled, if pelvic enthesitis on MRI was included in the criteria. There is a high correlation between pelvic enthesitis and sacroiliitis, with sacroiliitis present in 17/23 (74 %) patients with pelvic enthesitis. CONCLUSIONS: Pelvic enthesitis may be present in children with or without clinically evident peripheral enthesitis. There is a high correlation between pelvic enthesitis and sacroiliitis on MRI of the sacroiliac joints in children. As pelvic enthesitis indicates active inflammation, it may play a role in assessment of the inflammatory status. Therefore, it should be carefully sought and noted by radiologists examining MRI of the sacroiliac joints in children.

Diagnostic value of MRI features of sacroiliitis in juvenile spondyloarthritis.

AIM: To determine the diagnostic utility of magnetic resonance imaging (MRI) features of sacroiliitis in juvenile spondyloarthritis (JSpA). MATERIALS AND METHODS: This was a prospective study of 80 paediatric patients who underwent MRI of the sacroiliac joints that were clinically suspected to have sacroiliitis. The prevalence of MRI features of active and structural lesions of sacroiliitis was recorded. Patients were classified according to the International League of Association for Rheumatology criteria. The MRI findings were compared to the final clinical diagnosis. RESULTS: Sacroiliitis was seen in 25/80 (31%) patients. MRI showed active inflammation in 23 patients (29%): synovial enhancement (28%), high short tau inversion recovery (STIR)-signal in the joint space (29%), bone marrow oedema (BMO; 20%), and capsulitis (8%). Structural changes were present in 14 patients (18%): erosion (14%), fat infiltration (13%), sclerosis (8%), and ankylosis (1%). Of all MRI features, ankylosis (100%), capsulitis (98%), BMO (96%), and erosion (96%) had the highest specificity for JSpA; global diagnostic impression (55%) and synovial enhancement (52%) were the MRI features with the highest sensitivity. The likelihood ratios (LR+) for diagnosis of JSpA were high for BMO (10.5), capsulitis (7.5), global diagnostic impression (6.9), and erosions (6.75), but greater for BMO concomitant with synovial enhancement (LR+ 19.5) and for erosion concomitant with BMO (LR+ 12) or synovial enhancement (LR+ 13.5). CONCLUSION: There are multiple features of active inflammation and structural damage visible at MRI of the sacroiliac joints that can provide a specific diagnosis of JSpA when present in children with suspected sacroiliitis. Synovial enhancement is the MRI feature with the highest sensitivity for JSpA. If BMO is seen concomitant with synovial enhancement or erosion, the diagnosis of JSpA is very likely. Ankylosis, capsulitis, bone marrow oedema, and erosion all have a high specificity for JSpA. Absence of MRI findings of sacroiliitis does not exclude the diagnosis of JSpA.

Limited role of gadolinium to detect active sacroiliitis on MRI in juvenile spondyloarthritis.

OBJECTIVE: The aim of this study is to determine the added diagnostic value of contrast-enhanced (CE) magnetic resonance imaging (MRI) compared to routine non contrast-enhanced MRI to detect active sacroiliitis in clinically juvenile spondyloarthritis (JSpA). MATERIALS AND METHODS: A total of 80 children clinically suspected for sacroiliitis prospectively underwent MRI of the sacroiliac (SI) joints. Axial and coronal T1-weighted (T1), Short-tau inversion recovery (STIR) and fat-saturated T1-weighted gadolinium-DTPA (Gd) contrast-enhanced (T1/Gd) sequences were obtained. The presence of bone marrow edema (BME), capsulitis, enthesitis, high intra-articular STIR signal, synovial enhancement and a global diagnostic impression of the MRI for diagnosis of sacroiliitis was recorded. RESULTS: STIR and T1/Gd sequences had 100% agreement for depiction of BME, capsulitis and enthesitis. High intra-articular STIR signal was seen in 18/80 (22.5%) patients, 15 (83%) of whom also showed synovial enhancement in the T1/Gd sequence. Sensitivity (SN) and specificity (SP) for a clinical diagnosis of JSpA were similar for high STIR signal (SN = 33%, SP = 85%) and T1/Gd synovial enhancement (SN = 36%, SP = 92%). Positive likelihood ratio (LR+) for JSpA was twice as high for synovial enhancement than high STIR signal (4.5 compared to 2.2). Global diagnostic impression was similar (STIR: SN = 55%, SP = 87%, LR + =4 .2; T1/Gd: SN = 55%, SP = 92%, LR + = 6.9). CONCLUSION: MRI without contrast administration is sufficient to identify bone marrow edema, capsulitis and retroarticular enthesitis as features of active sacroiliitis in juvenile spondyloarthritis. In selected cases when high STIR signal in the joint is the only finding, gadolinium-enhanced images may help to confirm the presence of synovitis.

Sex steroids in relation to sexual and skeletal maturation in obese male adolescents.

BACKGROUND: Childhood obesity is associated with an accelerated skeletal maturation. However, data concerning pubertal development and sex steroid levels in obese adolescents are scarce and contrasting. OBJECTIVES: To study sex steroids in relation to sexual and skeletal maturation and to serum prostate specific antigen (PSA), as a marker of androgen activity, in obese boys from early to late adolescence. METHODS: Ninety obese boys (aged 10-19 y) at the start of a residential obesity treatment program and 90 age-matched controls were studied cross-sectionally. Pubertal status was assessed according to the Tanner method. Skeletal age was determined by an x-ray of the left hand. Morning concentrations of total testosterone (TT) and estradiol (E2) were measured by liquid chromatography-tandem mass spectrometry, free T (FT) was measured by equilibrium dialysis, and LH, FSH, SHBG, and PSA were measured by immunoassays. RESULTS: Genital staging was comparable between the obese and nonobese groups, whereas skeletal bone advancement (mean, 1 y) was present in early and midadolescence in the obese males. Although both median SHBG and TT concentrations were significantly (P < .001) lower in obese subjects during mid and late puberty, median FT, LH, FSH, and PSA levels were comparable to those of controls. In contrast, serum E2 concentrations were significantly (P < .001) higher in the obese group at all pubertal stages. CONCLUSION: Obese boys have lower circulating SHBG and TT, but similar FT concentrations during mid and late puberty in parallel with a normal pubertal progression and serum PSA levels. Our data indicate that in obese boys, serum FT concentration is a better marker of androgen activity than TT. On the other hand, skeletal maturation and E2 were increased from the beginning of puberty, suggesting a significant contribution of hyperestrogenemia in the advancement of skeletal maturation in obese boys.

Anal canal duplication in an 11-year-old-child.

Anal canal duplication (ACD) is the least frequent digestive duplication. Symptoms are often absent but tend to increase with age. Recognition is, however, important as almost half of the patients with ACD have concomitant malformations. We present the clinical history of an eleven-year-old girl with ACD followed by a review of symptoms, diagnosis, treatment, and prognosis based on all the reported cases in English literature.

Colon transit time and anorectal manometry in children and young adults with spina bifida.

PURPOSE: This study evaluates colon transit time (CTT) and anorectal manometry (ARM) in spina bifida (SB) patients in relation to the level of lesion, mobility, constipation, and continence status. METHODS: SB patients between 6 and 19 years, who are not using antegrade continence enemas and followed at the SB Reference Centre UZ Ghent, were asked to participate. Medical history was retrospectively retrieved from the medical file. Stool habits were prospectively collected using standardized questionnaires. CTT was measured using the 6-day pellet abdominal X-ray method. ARM was performed in non-sedated children with a water-perfused, latex-free catheter. RESULTS: Forty out of 52 eligible patients consented to perform CTT, of which 19 also performed the ARM. Fifteen (37 %) SB patients were constipated despite treatment. Twenty-six (65 %) were (pseudo) continent. The total CTT was significantly prolonged in SB patients (median CTT 86.4 vs. 36 h controls). The CTT was significantly prolonged in constipated SB patients compared to non-constipated SB patients (122.4 vs. 52.8 h). Spontaneously continent patients had a normal CTT (33.6 h) as well as a significantly higher resting pressure compared to the pseudo-continent and incontinent SB patients (resting pressure 56.5 vs. 32.5 mmHg). An abnormal CTT was associated with a treatment necessity to achieve pseudo-continence (p = 0.006). CONCLUSION: CTT in SB patients was significantly prolonged, indicating a neurogenic involvement of the bowel and slow transit constipation. SB patients with a normal CTT and a normal ARM spontaneously achieved fecal continence. CTT can help tailor the continence therapy in SB patients.

Colon transit time in healthy children and adolescents.

PURPOSE: The aims of this study are to describe normal colon transit time (CTT) in healthy children, correlate results with age, the Bristol stool scale, and stool frequency, and to evaluate intra- and interobserver variability. METHODS: Inclusion criteria were as follows: healthy children between 3 and 18 years old with a normal defecation pattern, no history of abdominal surgery, and no medication use. Total and segmental CTT is measured by taking ten polythene radiopaque markers during six consecutive days followed by a single abdominal X-ray on day 7. Total and segmental CTT are calculated by multiplying the number of markers by 2.4 (Abrahamsson et al. Scand J Gastroenterol 32:72-80, 1988). RESULTS: Fifty-four children and adolescents have participated: 30 boys and 24 girls (median age 10 years (3-18 years)). Median total CTT is 36 h (<2.4-86.4 h). There is no significant difference for age category (toddlers 31.2 h (<2.4-74.4 h), elementary school 36 h (2.4-79.2 h), and adolescents 43.2 h (14.4-86.4 h)). Segmental CTT reveals a median right colon CTT of 4.8 h (0-28.8 h); a median left colon CTT of 2.4 h (0-31.2 h); and a median rectosigmoidal CTT of 24 h (0-64.8 h). The Bristol stool scale correlates with total CTT (p = 0.031). The intra- and interobserver variability displays an ICC of 0.999 for the total CTT. CONCLUSION: The CTT of normal healthy children is not sex- or age-related (above the age of 3 years). The Abrahamson method for CTT measurement by using bony landmarks for the determination of colon segments is easy to perform and well tolerated with a virtual inexistent rating difference between different observers.

Bone size and bone strength are increased in obese male adolescents.

CONTEXT: Controversy exists on the effect of obesity on bone development during puberty. OBJECTIVE: Our objective was to determine differences in volumetric bone mineral density (vBMD) and bone geometry in male obese adolescents (ObAs) in overlap with changes in bone maturation, muscle mass and force development, and circulating sex steroids and IGF-I. We hypothesized that changes in bone parameters are more evident at the weight-bearing site and that changes in serum estradiol are most prominent. DESIGN, SETTING, AND PARTICIPANTS: We recruited 51 male ObAs (10-19 years) at the entry of a residential weight-loss program and 51 healthy age-matched and 51 bone-age-matched controls. MAIN OUTCOME MEASURES: vBMD and geometric bone parameters, as well as muscle and fat area were studied at the forearm and lower leg by peripheral quantitative computed tomography. Muscle force was studied by jumping mechanography. RESULTS: In addition to an advanced bone maturation, differences in trabecular bone parameters (higher vBMD and larger trabecular area) and cortical bone geometry (larger cortical area and periosteal and endosteal circumference) were observed in ObAs both at the radius and tibia at different pubertal stages. After matching for bone age, all differences at the tibia, but only the difference in trabecular vBMD at the radius, remained significant. Larger muscle area and higher maximal force were found in ObAs compared with controls, as well as higher circulating free estrogen, but similar free testosterone and IGF-I levels. CONCLUSIONS: ObAs have larger and stronger bones at both the forearm and lower leg. The observed differences in bone parameters can be explained by a combination of advanced bone maturation, higher estrogen exposure, and greater mechanical loading resulting from a higher muscle mass and strength.

Fibromuscular dysplasia of the carotid artery.

We describe the ultrasound-Doppler and angio-CT findings of the internal carotid string-of-beads sign in a patient with fibromuscular dysplasia (FMD). FMD is a disease of unknown etiology typically affecting the medium and larger arteries of young and middle-aged women. The most commonly affected arteries are the renal arteries followed by the internal carotid arteries. The lumbar, mesenteric, celiac, hepatic and iliac arteries are less commonly affected. Patients typically present with renovascular hypertension in case of renal artery involvement, Transient ischemic attack or stroke can be one of the presenting symptoms in carotid artery involvement.