Does Magnetic Resonance Imaging Distinguish Juvenile Idiopathic Arthritis From Other Causes of Progressive Temporomandibular Joint Destruction?

PURPOSE: Similarities in initial presentations of temporomandibular joint (TMJ) involvement from juvenile idiopathic arthritis (JIA), idiopathic condylar resorption, and other forms of progressive TMJ destruction in children create diagnostic confusion. Treatment pathways, however, depend on determination of etiology. The purpose of this study was to compare TMJ magnetic resonance images (MRIs) of patients with joint degeneration localized to the TMJs to those with JIA and TMJ involvement. STUDY DESIGN, SETTING, SAMPLE: This is a retrospective cross-sectional study including subjects younger than 18 years that presented from February 2008 to October 2019 with clinical TMJ degeneration, a gadolinium-enhanced TMJ MRI and a negative pediatric rheumatologic workup (non-JIA group), and a series of age and sex-matched subjects with TMJ degeneration on gadolinium-enhanced MRI and JIA (JIA group). MRIs were evaluated in a blinded fashion by 1 pediatric radiologist. The primary outcome variable was the radiologist's accuracy in predicting study grouping, assessed in 1 randomly-selected joint per patient. Secondary outcome variables included MRI characteristics of inflammation, osseous damage and articular disc morphology. Independent samples t-tests, sensitivity/specificity, Fisher's exact and Mann-Whitney tests were computed as applicable, and P < .05 was considered significant. RESULTS: The sample included 34 subjects: 16 non-JIA (75% female, age 13.9 ± 2.8 years) and 18 JIA (77% female, age 13.6 ± 2.8 years) (P ≥ .738). The radiologist correctly classified 64.7% of subjects as non-JIA or JIA (P = .078, sensitivity = 94.4%, specificity = 31.3%). Inflammatory and osseous findings were similar between groups (P ≥ .073). The disc was anteriorly displaced in 9 non-JIA and 0 JIA joints (P < .001, sensitivity = 100%, specificity = 100%) and flattened in 3 non-JIA and 14 JIA joints (P = .006, sensitivity = 38.9%, specificity = 90.6%). CONCLUSION AND RELEVANCE: Inflammatory and osseous findings on gadolinium-enhanced TMJ MRIs are insufficient to determine the etiology of progressive TMJ destruction. Disc characteristics, however, significantly differ between JIA and non-JIA etiologies and may be important in differentiating these conditions.

Management of Orofacial Manifestations of Juvenile Idiopathic Arthritis: Interdisciplinary Consensus-Based Recommendations.

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. We undertook this study to develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA, and to create a future research agenda related to management of TMJ arthritis in children with JIA. Recommendations were developed using online surveying of relevant stakeholders, systematic literature review, evidence-informed generation of recommendations during 2 consensus meetings, and Delphi study iterations involving external experts. The process included disciplines involved in the care of orofacial manifestations of JIA: pediatric rheumatology, radiology, orthodontics, oral and maxillofacial surgery, orofacial pain specialists, and pediatric dentistry. Recommendations were accepted if agreement was >80% during a final Delphi study. Three overarching management principles and 12 recommendations for interdisciplinary management of orofacial manifestations of JIA were outlined. The 12 recommendations pertained to diagnosis (n = 4), treatment of TMJ arthritis (active TMJ inflammation) (n = 2), treatment of TMJ dysfunction and symptoms (n = 3), treatment of arthritis-related dentofacial deformity (n = 2), and other aspects related to JIA (n = 1). Additionally, a future interdisciplinary research agenda was developed. These are the first interdisciplinary recommendations to guide clinical management of TMJ JIA. The 3 overarching principles and 12 recommendations fill an important gap in current clinical practice. They emphasize the importance of an interdisciplinary approach to diagnosis and management of orofacial manifestations of JIA.

Three-dimensional magnetic resonance imaging ultrashort echo-time cones for assessing lung density in pediatric patients.

BACKGROUND: MRI of lung parenchyma is challenging because of the rapid decay of signal by susceptibility effects of aerated lung on routine fast spin-echo sequences. OBJECTIVE: To assess lung signal intensity in children on ultrashort echo-time sequences in comparison to a fast spin-echo technique. MATERIALS AND METHODS: We conducted a retrospective study of lung MRI obtained in 30 patients (median age 5 years, range 2 months to 18 years) including 15 with normal lungs and 15 with cystic fibrosis. On a fast spin-echo sequence with radial readout and an ultrashort echo-time sequence, both lungs were segmented and signal intensities were extracted. We compared lung-to-background signal ratios and histogram analysis between the two patient cohorts using non-parametric tests and correlation analysis. RESULTS: On ultrashort echo-time the lung-to-background ratio was age-dependent, ranging from 3.15 to 1.33 with high negative correlation (Rs = -0.86). Signal in posterior dependent portions of the lung was 18% and 11% higher than that of the anterior lung for age groups 0-2 and 2-18 years, respectively. The fast spin-echo sequence showed no variation of signal ratios by age or location, with a median of 0.99 (0.98-1.02). Histograms of ultrashort echo-time slices between controls and children with aggravated cystic fibrosis with mucus plugging and wall thickening exhibited significant discrepancies that differentiated between normal and pathological lungs. CONCLUSION: Signal intensity of lung on ultrashort echo-time is higher than that on fast spin-echo sequences, is age-dependent and shows a gravity-dependent anterior to posterior gradient. This signal variation appears similar to lung density described on CT.

Right ventricular dilatation in patients with pulmonary regurgitation after repair of tetralogy of Fallot: How fast does it progress?

OBJECTIVE: Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction. METHODS: Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatation. RESULTS: There was no significant increase in RV end-diastolic volumes (RVEDVi) indexed body surface area (BSA) (median 150 [81-249] vs. 150 [82-260] mL/m2) and end-systolic volumes indexed for BSA (RVESVi) (75 [20-186] vs. 76 [39-189] mL/m2) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDVi 78 [56-137] vs. 81 [57-128] mL/m2 and LV end-systolic volume index 34 [23-68] vs. 35 [18-61] mL/m2). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥20 mL/m2) in twenty patients (24%) from 154 mL/m2 (87-237) to 184 mL/m2 (128-260, P < 0.001). LV dimensions showed a similar trend with LVEDVi increase from 80 ml/m2 (57-98) to 85 ml/m2 (72-105, P = 0.002). Shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV dilatation. CONCLUSION: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair.

Structural and perfusion magnetic resonance imaging of congenital lung malformations.

BACKGROUND: A radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children. OBJECTIVE: To describe magnetic resonance imaging (MRI) findings of congenital bronchopulmonary foregut malformations and investigate the ability of lung MRI for their classification. MATERIALS AND METHODS: This is a retrospective analysis of consecutive MRI examinations performed for suspected congenital lung anomalies in 39 children (median age: 3.8 months, range: 2 days-15 years). Morphological and perfusion findings were characterised on respiratory-gated fast spin echo and dynamic contrast-enhanced sequences obtained at 1.5 tesla. Abnormalities were classified independently by two readers and compared to an expert diagnosis based on pathology, surgery and/or other imaging. RESULTS: Main diagnoses included bronchopulmonary lesions in 33 patients, scimitar syndrome in 4 patients, pulmonary arteriovenous malformation and oesophageal duplication cyst in one patient each. Of 46 observed abnormalities, 44 (96%) were classified correctly with very good interobserver agreement (96% concordance rate). The 39 detected lung lesions included isolated overinflation (17/39, 44%), cystic pulmonary airway malformation (8/39, 21%), bronchopulmonary sequestration (7/39, 18%), bronchogenic cyst (4/39, 10%) and hybrid lesion (3/39, 8%). All lung lesions presented as perfusion defect at peak pulmonary enhancement. Non-cystic lesions showed a delayed peak (median delay: 2.8 s, interquartile range: 0.5 to 4.0 s) in relation to normal lung parenchyma. CONCLUSION: A dedicated lung MRI protocol including respiratory compensated sequences, dynamic angiography and perfusion is able to reliably delineate parenchymal and vascular components of congenital bronchopulmonary foregut malformations. Therefore, MRI may be considered for comprehensive postnatal evaluation of congenital thoracic malformations.

Structural and perfusion magnetic resonance imaging of the lung in cystic fibrosis.

BACKGROUND: Because of its absence of ionising radiation and possibility for obtaining functional information, MRI is promising for assessing lung disease in children who require repetitive imaging for long-term follow-up. OBJECTIVE: To describe MRI findings in children with cystic fibrosis and evaluate semi-quantitative dynamic contrast-enhanced lung perfusion. MATERIALS AND METHODS: We retrospectively compared lung MRI in 25 children and young adults with cystic fibrosis (median age 3.7 years) to 12 children (median age 2 years) imaged for other pathologies. MRI at 1.5 T included respiratory-gated sequences and contrast-enhanced lung perfusion imaging. We described and graded any morphologic change. Signal enhancement and time to peak values of perfusion abnormalities were compared to those of normally enhancing lung parenchyma. RESULTS: Frequent findings in patients with cystic fibrosis were bronchial wall thickening (24/25, 96%), areas of consolidation (22/25, 88%), enlarged lymph nodes (20/25, 80%), bronchiectasis (5/25, 20%) and mucus plugging (3/25, 12%). Compared to normally enhancing lung, perfusion defects (21/25, 84%), characterised by decreased enhancement, showed prolonged time to peak. Areas of consolidation showed increased enhancement. While time to peak of procedure-related atelectasis was not significantly different from that of normal lung, disease-related consolidation showed prolonged time to peak (P=0.01). CONCLUSION: Lung MRI demonstrates structural and perfusion abnormalities in children and young people with cystic fibrosis. Semi-quantitative assessment of dynamic contrast-enhanced perfusion imaging might allow differentiation between procedure-related atelectasis and disease-related consolidation.

Toward Establishing a Standardized Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis.

OBJECTIVE: The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJ pathology on magnetic resonance imaging (MRI). Consensus-driven development and validation of an MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis and serving as an outcome measure. We report on a multi-institutional collaboration toward developing a TMJ MRI scoring system for JIA. METHODS: Seven readers independently assessed MRI scans from 21 patients (42 TMJs, from patients ages 6-16 years) using 3 existing MRI scoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions, and items were discussed among 10 JIA experts through 2 rounds of Delphi surveys, nominal group voting, and subsequent consensus meetings to create a novel TMJ MRI scoring system. RESULTS: Average-measure absolute agreement intraclass correlation coefficients (avICCs) for the total scores of all 3 scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed preferable for assessing minor joint changes over time. Eight items were considered sufficiently reliable and/or important for integration into the consensus scoring system: bone marrow edema and enhancement (avICC 0.57-0.61, smallest detectable difference [SDD] ± 45-63% prior to redefining), condylar flattening (avICC 0.95-0.96, SDD ± 23-28%), effusions (avICC 0.85-0.88, SDD ± 25-26%), erosions (avICC 0.94, SDD ± 20%), synovial enhancement and thickening (previously combined, avICC 0.90-0.91, SDD ± 33%), and disk abnormalities (avICC 0.90, SDD ± 19%). CONCLUSION: A novel TMJ MRI scoring system was developed by consensus. Further iterative refinements and reliability testing are warranted in upcoming studies.

Method of visualisation influences accuracy of measurements in cone-beam computed tomography.

This study evaluated the potential impact of different visualisation methods of cone-beam computed tomography (CBCT) on the accuracy of linear measurements of calcified structures, and assessed their interchangeability. High resolution (0.125 mm voxel) CBCT scans were obtained from eight cadaveric heads. The distance between the alveolar bone ridge and the incisal edge was determined for all mandibular incisors and canines, both anatomically and with measurements based on the following five CBCT visualisation methods: isosurface, direct volume rendering, multiplanar reformatting (MPR), maximum intensity projection of the volume of interest (VOIMIP), and average intensity projection of the volume of interest (VOIAvIP). All radiological methods were tested for repeatability and compared with anatomical results for accuracy, and limits of agreement were established. Interchangeability was evaluated by reviewing disparities between the methods and disclosing deterministic differences. Fine intra- and inter-observer repeatability was asserted for all visualisation methods (intraclass correlation coefficient ≤0.81). Measurements were most accurate when performed on MPR images and performed most disappointingly on isosurface-based images. Direct volume rendering, VOIMIP and VOIAvIP achieved acceptable results. It can be concluded that visualisation methods influence the accuracy of CBCT measurements. The isosurface viewing method is not recommended, and multiplanar reformatted images should be favoured for linear measurements of calcified structures.

Muscle magnetic resonance imaging of the lower limbs: valuable diagnostic tool in the investigation of childhood neuromuscular disorders.

Children presenting with neuromuscular symptoms are subject to exhaustive investigations. As it is noninvasive, muscle magnetic resonance imaging (MRI) is an important diagnostic tool in children, yet its impact has so far been mainly studied in small groups of genetically defined diseases, where specific MRI patterns are known. To assess the contribution of muscle MRI of the lower limbs in a diverse cohort of patients, we reviewed the diagnostic findings in 39 patients with a suspected neuromuscular disorder that underwent muscle MRI (28/39), biopsy (26/39), or both (18/39). MRI was performed without sedation in 26 of 28 patients at a mean age of 10 years (range, 1-27 years). In 10 of 28 cases (35%), MRI significantly contributed to the final diagnosis, and in 7 of 28 cases (25%), muscle MRI directly instructed genetic testing. These cases included Bethlem myopathy, laminopathy, calpainopathy, and RYR1-related myopathies. Muscle MRI serves as a valuable additional tool to guide diagnosis in suspected neuromuscular disorders in children, especially in cases with nonspecific biopsy findings.

Port-a-cath-related thrombosis and postthrombotic syndrome in pediatric oncology patients.

OBJECTIVE: To investigate Port-A-Cath (PAC)-related thrombosis and postthrombotic syndrome (PTS) in children with cancer. STUDY DESIGN: The study population was a consecutive cohort of children diagnosed with cancer and a PAC implanted at diagnosis. Children were evaluated for the presence of PAC-related thrombosis by magnetic resonance venography and the presence of congenital prothrombotic risk factors and PTS. RESULTS: A total of 114 children (median age, 6.04 years) were included. Of these children, 48 (42%) were treated for solid tumors and 66 (58%) were treated for hematopoietic tumors, including 38 for acute lymphoblastic leukemia. At the time of magnetic resonance venography, 42 children (37%) had the PAC still in place, and 72 (63%) had the PAC removed. Overall, PACs were in place for a total of 324.92 PAC-years. PAC-related thrombosis was detected in 45 children (39.5%) with a current or previous PAC. Of these, 21 (47%) had a solid tumor, 14 (31%) had acute lymphoblastic leukemia, and 10 (22%) had another hematopoietic tumor. Younger age at diagnosis, female sex, duration of PAC use, and left-side PAC placement were independently associated with an increased risk of thrombosis, whereas asparaginase therapy and the presence of inherited prothrombotic risk factors were not. Mild PTS (ie, presence of prominent collateral vessels in the skin) was present in 5.6% of the children. CONCLUSION: PAC-related thrombosis is common in pediatric oncology patients. In some children, thrombotic complications can lead to the development of PTS.

Arterial spin labelling MRI for assessment of cerebral perfusion in children with moyamoya disease: comparison with dynamic susceptibility contrast MRI.

INTRODUCTION: This study seeks to evaluate the diagnostic accuracy of cerebral perfusion imaging with arterial spin labelling (ASL) MR imaging in children with moyamoya disease compared to dynamic susceptibility contrast (DSC) imaging. METHODS: Ten children (7 females; age, 9.2 ± 5.4 years) with moyamoya disease underwent cerebral perfusion imaging with ASL and DSC on a 3-T MRI scanner in the same session. Cerebral perfusion images were acquired with ASL (pulsed continuous 3D ASL sequence, 32 axial slices, TR = 5.5 s, TE = 25 ms, FOV = 24 cm, matrix = 128 × 128) and DSC (gradient echo EPI sequence, 35 volumes of 28 axial slices, TR = 2,000 ms, TE = 36 ms, FOV = 24 cm, matrix = 96 × 96, 0.2 ml/kg Gd-DOTA). Cerebral blood flow maps were generated. ASL and DSC images were qualitatively assessed regarding perfusion of left and right ACA, MCA, and PCA territories by two independent readers using a 3-point-Likert scale and quantitative relative cerebral blood flow (rCBF) was calculated. Correlation between ASL and DSC for qualitative and quantitative assessment and the accuracy of ASL for the detection of reduced perfusion per territory with DSC serving as the standard of reference were calculated. RESULTS: With a good interreader agreement (κ = 0.62) qualitative perfusion assessment with ASL and DSC showed a strong and significant correlation (ρ = 0.77; p < 0.001), as did quantitative rCBF (r = 0.79; p < 0.001). ASL showed a sensitivity, specificity and accuracy of 94 %, 93 %, and 93 % for the detection of reduced perfusion per territory. CONCLUSION: In children with moyamoya disease, unenhanced ASL enables the detection of reduced perfusion per vascular territory with a good accuracy compared to contrast-enhanced DSC.

MRI of the temporo-mandibular joint: which sequence is best suited to assess the cortical bone of the mandibular condyle? A cadaveric study using micro-CT as the standard of reference.

OBJECTIVE: To determine the best suited sagittal MRI sequence out of a standard temporo-mandibular joint (TMJ) imaging protocol for the assessment of the cortical bone of the mandibular condyles of cadaveric specimens using micro-CT as the standard of reference. METHODS: Sixteen TMJs in 8 human cadaveric heads (mean age, 81 years) were examined by MRI. Upon all sagittal sequences, two observers measured the cortical bone thickness (CBT) of the anterior, superior and posterior portions of the mandibular condyles (i.e. objective analysis), and assessed for the presence of cortical bone thinning, erosions or surface irregularities as well as subcortical bone cysts and anterior osteophytes (i.e. subjective analysis). Micro-CT of the condyles was performed to serve as the standard of reference for statistical analysis. RESULTS: Inter-observer agreements for objective (r = 0.83-0.99, P < 0.01) and subjective (κ = 0.67-0.88) analyses were very good. Mean CBT measurements were most accurate, and cortical bone thinning, erosions, surface irregularities and subcortical bone cysts were best depicted on the 3D fast spoiled gradient echo recalled sequence (3D FSPGR). CONCLUSION: The most reliable MRI sequence to assess the cortical bone of the mandibular condyles on sagittal imaging planes is the 3D FSPGR sequence. KEY POINTS: MRI may be used to assess the cortical bone of the TMJ. • Depiction of cortical bone is best on 3D FSPGR sequences. • MRI can assess treatment response in patients with TMJ abnormalities.

Incidental findings of mass lesions on neuroimages in children.

Increasing use of neuroimaging in children has led to more incidental findings of CNS mass lesions, the management of which is uncertain. The authors' aims in this study are to describe these mass lesions and their evolution, as well as to discuss the management options and determine the prevalence of incidental CNS mass lesions at their pediatric clinic. A retrospective study was undertaken in children with primary CNS tumors who were younger than 18 years old and were admitted to the University Children's Hospital of Zurich, Switzerland, between January 1995 and December 2010. In 19 (5.7%) of 335 patients with newly diagnosed CNS tumors, the diagnosis of a CNS mass lesion was an incidental finding. Reasons for obtaining neuroimages in these 19 patients were head trauma (in 6 patients); research protocols (in 3); nasal/orbital malformations (in 2); endocrinological and psychiatric evaluations (in 2); and vertebral bone anomaly without neurological signs, absence seizures, congenital ataxia, recurrent vomiting, developmental delay, and "check-up" at the explicit request of the parents (in 1 patient each). Seven patients underwent immediate surgery for low-grade glioma (4 patients) and craniopharyngioma, ependymoma, and choroid plexus papilloma (1 patient each); and 12 were treated conservatively or were observed. Ten of 12 conservatively treated patients remained stable (median follow-up time 1.8 years) and the other 2 underwent delayed surgery because of tumor progression (medulloblastoma in one patient and fibrillary astrocytoma in the other). Clinicians are increasingly challenged by the discovery of incidental CNS mass lesions. A subgroup of such lesions (with typical imaging patterns such as tectal glioma and dysembryoplastic neuroepithelial tumor) can be monitored conservatively, clinically, and radiographically. Future prospective studies are needed to define optimal management strategies based on larger collections of natural histories, as well as to assess the true prevalence of incidental CNS mass lesions.

Increased cerebellar volume in the early stage of fucosidosis: a case control study.

INTRODUCTION: Yet unreported in this lysosomal storage disease, we aimed to quantify our observation that patients with fucosidosis may show abnormally increased cerebellar volumes during early childhood. METHODS: Five normocephalic fucosidosis patients (age range 2-25 months, three males) were included in this retrospective case control study. The control cohort consisted of 25 children (age range 0-36 months, 15 males). Image postprocessing was performed independently by two radiologists. Using validated software, manual tracing of contours on contiguous sagittal magnetic resonance images was allowed for cerebellar volumetry. We tested the null hypothesis that mean cerebellar volumes of four fucosidosis patients (age 16, 20, 21, and 25 months) and of an age-matched control cohort (n = 8, age range 13-26 months) were equal based on a two-tailed unpaired t-test. RESULTS: Interobserver agreement was excellent (R = 1, p < 0.01). A rough trajectory of normal cerebellar development appeared to flatten around the age of 1 year. With mean volumes of 121.36 and 102.30 ml, respectively, cerebellar volumes of fucosidosis patients with a mean age of 21 months were significantly increased compared to age-matched controls (p < 0.05). In a single patient, longer-term follow-up with MRI at the age of 47 months was available and showed cerebellar atrophy. CONCLUSION: Increased cerebellar volume was shown to be an additional feature in the early stage of fucosidosis. The combination with a confirmed tendency toward atrophy of the cerebellum during later course of the disease is probably unique in the context of metabolic disorders of the brain.

Transsphenoidal extension of heterotopic glioneuronal tissue: pathoanatomic considerations in symptomatic neonates.

PURPOSE: In this clinical investigation, we aimed (1) to re-evaluate the nature of glioneuronal tissue with transsphenoidal extension and how it fits into the nomenclature of midline malformations and mass lesions; (2) to find out if our imaging findings support current pathoanatomic concepts of clefts and canals in the sphenoid body of newborns. METHODS: In two neonates with respiratory distress due to nasopharyngeal masses, 3T MRI was performed, and CT in one of them. Imaging features were analyzed in consensus by two pediatric neuroradiologists with histological reports being available. An interdisciplinary panel compared the findings to those of case publications and differential entities from our institutional case collection. RESULTS: Referring to our rare case of transsphenoidal cerebral heterotopia and unique case of hypothalamic hamartoma with transsphenoidal herniation, glioneuronal heterotopia may definitely extend through the sphenoid bone. Consequently, there is reason for brain heterotopias to be labeled as such also in case of an intracranial component. Connection between heterotopic glioneuronal tissue in the nasopharynx and a hypothalamic hamartoma may go along with indistinct margins to normal brain. Neither extension through a transsphenoidal cleft nor association with a cleft palate are specific for cerebral heterotopia. Our findings support the hypothesis that transsphenoidal cerebral heterotopias do not or at least not invariably follow the route of Rathke's pouch, known as the craniopharyngeal canal. CONCLUSION: Transsphenoidal glioneuronal heterotopia should be the top differential diagnosis in MR imaging if a non-enhancing nasopharyngeal mass of an infant extends through a craniopharyngeal cleft within the intersphenoid synchondrosis.

Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine.

BACKGROUND: To explore the role of contrast-enhanced magnetic resonance angiography (CE-MRA) in clinical routine for evaluating neonates with pulmonary atresia (PA) and to describe their pulmonary artery morphology and blood supply.CE-MRA studies of 15 neonates with PA (12 female; median weight: 2900 g) were retrospectively evaluated by two radiologists in consensus. Each study was judged to be either diagnostic or non-diagnostic depending on the potential to evaluate pulmonary artery morphology and pulmonary blood supply. In those cases where surgery or conventional angiocardiography was performed results were compared. RESULTS: CE-MRA was considered diagnostic in 87%. Pulmonary artery morphology was classified as "confluent with (n = 1) and without (n = 1) main pulmonary artery", "non-confluent" (n = 6) or "absent" (n = 7). Source of pulmonary blood supply was "a persistent arterial duct" (n = 12), "a direct" (n = 22) or "indirect (n = 9) aortopulmonary collateral artery (APCA)" or "an APCA from the ascending aorta" (n = 2). In no patient were there any additional findings at surgery or conventional angiocardiography which would have changed the therapeutic or surgical approach. CONCLUSIONS: CE-MRA is a useful diagnostic tool for the preoperative evaluation of the morphology of pulmonary arteries and blood supply in neonates with PA. In most cases diagnostic cardiac catheterization can be avoided.