Minimizing neurovascular complications during image-guided percutaneous cryoablation of a cervical spinal aneurysmal bone cyst using protective doxycycline sclerotherapy: a case report.

Background: Aneurysmal bone cysts (ABC) are rare, locally aggressive bone tumors primarily observed in pediatric patients. Surgical curettage is the treatment of choice. Image-guided percutaneous cryoablation (CYOA) is a recently implemented alternative technique in cases not amenable to surgery. CYOA may be limited if the lesion is close to critical neurovascular structures. In this case report, a cervical spinal ABC was successfully treated using CYOA in combination with complementary and protective image-guided percutaneous doxycycline sclerotherapy (DS) to dissect and treat the portion of the lesion in contact with critical structures. Case Description: A 4-year-old male presented with a symptomatic ABC within the C5 vertebral body, which encompassed the right vertebral artery and contacted the right C5-C6 spinal cord and nerve roots. After ruling out surgery due to the proximity of critical neurovascular structures, treatment with CYOA was performed. However, subsequent follow-up showed recurrence in the part of the lesion contacting critical structures. A second approach was then decided, using DS to dissect and treat the most vulnerable portion and CYOA to treat the remaining tumor. Follow-up showed almost complete sclerosis of the lesion and total resolution of symptoms 3 years after treatment. Conclusions: Using DS to dissect and treat the portion of the lesion in contact with critical neurovascular structures during CYOA treatment of a cervical spinal ABC allowed for a safe and effective approach in our case.

Bacterial osteomyelitis in pediatric patients: a comprehensive review.

Bacterial osteomyelitis, an inflammatory response in the bone caused by microorganisms, typically affects the metaphysis in the skeletally immature. Bacterial osteomyelitis possesses a significant diagnostic challenge in pediatric patients due to its nonspecific clinical presentation. Because the metaphysis is the primary focus of infection in skeletally immature patients, understanding the normal physiologic, maturation process of bones throughout childhood allows to understand the pathophysiology of osteomyelitis. Timely and accurate diagnosis is crucial to initiate appropriate treatment, and prevent long-term sequelae and efforts must be made to isolate the causative organism. The potential causative organism changes according to the age of the patient and underlying medical conditions. Staphylococcus Aureus is the most common isolated bacteria in pediatric pyogenic osteomyelitis whereas Kingella Kingae is the most common causative agent in children aged 6 months to 4 years. Imaging plays a pivotal role in the diagnosis, characterization, evaluation of complications, and follow up of bacterial osteomyelitis. Imaging also plays a pivotal role in the evaluation of potential neoplastic and non-neoplastic mimickers of osteomyelitis. In children, MRI is currently the gold standard imaging modality when suspecting bacterial osteomyelitis, whereas surgical intervention may be required in order to isolate the microorganism, treat complications, and exclude mimickers.

Radiologic and Clinical Features of Infantile Hemangioma: Potential Pitfalls and Differential Diagnosis.

Infantile hemangioma (IH) is the most common neoplasm in children, but it may mimic other types of vascular anomalies or nonvascular benign and malignant tumors. In most cases, the clinical appearance, time of onset, and pattern of involution facilitate its diagnosis. Imaging evaluation is not always needed since the IH features at clinical presentation are usually characteristic, but when needed, US and frequently MRI are the imaging modalities of choice. Clinical photography or photographic documentation plays a central role in monitoring these lesions over their clinical course. Photographic documentation can also add confidence and alert the radiologist when interpreting imaging studies. Some vascular anomalies, especially vascular malformations, are a frequent source of confusion, as these may resemble IHs clinically and at imaging. The lack of uniform terminology also hinders an accurate diagnosis. To unify the terminology and minimize confusion, the International Society for the Study of Vascular Anomalies created a helpful classification in 1994. In addition, radiologists need to be aware of and become familiar with other neoplasms in children that may resemble IH to avoid misdiagnosis and unnecessary procedures. Fibrous and lipomatous tumors are examples of benign tumors that can mimic IHs clinically and at imaging, whereas rhabdomyosarcoma, infantile fibrosarcoma, neuroblastoma, and lymphoproliferative disorders are examples of malignant neoplasms. The authors review the features of IH at clinical presentation and imaging evaluation, highlighting its different phases of evolution and stressing the importance of photographic documentation. The authors also review pitfalls of IH with helpful pearls for differentiation. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material. See the invited commentary by Khanna and Briones in this issue.

Determination of Relative Weightings for Sacroiliac Joint Pathologies in the OMERACT Juvenile Arthritis Magnetic Resonance Imaging Sacroiliac Joint Score.

This study aims to determine the relative weights (point value) of items of the juvenile idiopathic arthritis magnetic resonance imaging-sacroiliac joint scoring system (JAMRIS-SIJ). An adaptive multicriteria decision analysis was performed using the 1000Minds web application to determine the relative weights of the items in the JAMRIS-SIJ inflammation and damage domains. Experts in imaging and rheumatology independently completed a conjoint analysis survey (CAS) to determine the point value of the measurement items of the JAMRIS-SIJ. Each CAS survey question asked the expert to compare two hypothetical patient profiles, which were otherwise similar but different at two items at a time, and to select which item showed a more severe stage of inflammation or osteochondral damage. In addition, experts ranked 14 JAMRIS-SIJ grade only or image + grade patient vignettes while blinded to the CAS-derived weights. The validity of the weighted JAMRIS-SIJ was tested by comparing the expert CAS-weighted score and the image + grade ranking method. Seventeen experts completed the CAS (11 radiologists and 6 rheumatologists). Considering the point value for inflammation domain items, osteitis (24.7%) and bone marrow edema (24.3%) had higher group-averaged percentage weights compared to inflammation in erosion cavity (16.9%), joint space enhancement (13.1%), joint space fluid (9.1%), capsulitis (7.3%), and enthesitis (4.6%). Similarly, concerning the damage domain, ankylosis (41.3%) and erosion (25.1%) showed higher group-averaged weights compared to backfill (13.9%), sclerosis (10.7%), and fat metaplasia lesion (9.1%). The Spearman correlation coefficients of the CAS-weighted vignette order and unweighted JAMRIS-SIJ grade only order vignettes for all experts were 0.79 for inflammation and 0.80 for damage. The correlations of image vignettes among imaging experts to CAS were 0.75 for inflammation and 0.90 for damage. The multicriteria decision analysis identified differences in relative weights among the JAMRIS-SIJ measurement items. The determination of the relative weights provided expert-driven score scaling and face validity for the JAMRIS-SIJ, enabling the future evaluation of its longitudinal construct validity.

Multiparametric MRI evaluation of bone sarcomas in children.

Osteosarcoma and Ewing sarcoma are the most common bone sarcomas in children. Their clinical presentation is very variable depending on the age of the patient and tumor location. MRI is the modality of choice to assess these bone sarcomas and has an important function at diagnosis and also for monitoring recurrence or tumor response. Anatomic sequences include T1- and T2-weighted images and provide morphological assessment that is crucial to localize the tumor and describe anatomical boundaries. Multiparametric MRI provides functional information that helps in the assessment of tumor response to therapy by using different imaging sequences and biomarkers. This review manuscript illustrates the role of MRI in osteosarcoma and Ewing sarcoma in the pediatric population, with emphasis on a functional perspective, highlighting the use of diffusion-weighted imaging and dynamic contrast-enhanced MRI at diagnosis, and during and after treatment.

An algorithm combining procalcitonin and lung ultrasound improves the diagnosis of bacterial pneumonia in critically ill children: The PROLUSP study, a randomized clinical trial.

BACKGROUND: Lung ultrasound (LUS) and procalcitonin (PCT) are independently used to improve accuracy when diagnosing lung infections. The aim of the study was to evaluate the accuracy of a new algorithm combining LUS and PCT for the diagnosis of bacterial pneumonia. METHODS: Randomized, blinded, comparative effectiveness clinical trial. Children <18 years old with suspected pneumonia admitted to pediatric intensive care unit were included, and randomized into experimental group (EG) or control group (CG) if LUS or chest X-Ray (CXR) were done as the first pulmonary image, respectively. PCT was determined. In patients with bacterial pneumonia, sensitivity, specificity, and predictive values of LUS, CXR, and of both combined with PCT were analyzed and compared. Concordance between the final diagnosis and the diagnosis concluded through the imaging test was assessed. RESULTS: A total of 194 children, with a median age of 134 (interquartile range [IQR]: 39-554) days, were enrolled, 96 randomized into the EG and 98 into the CG. Bacterial pneumonia was diagnosed in 97 patients. Sensitivity and specificity for bacterial pneumonia diagnosis were 78% (95% confidence interval [CI]: 70-85) and 98% (95% CI: 93-99) for LUS, 85% (95% CI: 78-90) and 53% (95% CI: 43-62) for CXR, 90% (95% CI: 83-94) and 85% (95% CI: 76-91) when combining LUS and PCT, and 95% (95% CI: 90-98) and 41% (95% CI: 31-52) when combining CXR and PCT. The positive predictive value for LUS and PCT was 88% (95% C:I 79%-93%) versus 68% (95% CI: 60-75) for CXR and PCT. The concordance between the final diagnosis and LUS had a kappa value of 0.69 (95% CI: 0.62-0.75) versus 0.34 (95% CI: 0.21-0.45) for CXR, (p < 0.001). CONCLUSIONS: The combination of LUS and PCT presented a better accuracy for bacterial pneumonia diagnosis than combining CXR and PCT. Therefore, its implementation could be a reliable tool for pneumonia diagnosis in critically ill children.

Postsurgical Evaluation of the Pediatric Foot and Ankle.

This article reviews situations in which surgical treatment is required in the context of different types of pathology in the foot and ankle of children, focusing on the role of imaging in surgical planning and postsurgical assessment. The types of pathology analyzed from this perspective are congenital pathology, neuromuscular disorders, osteochondral lesions, fractures and infection, ligament injuries, and tumors.We address the most common pitfalls of postsurgical imaging of the ankle and foot in children. With some exceptions, postsurgical follow-up focuses on clinical assessment, with imaging follow-up only needed in patients where complications are suspected or symptoms recur. Postoperative assessment of the foot and ankle in children and adolescents requires knowing the indications for surgery and the most common types of interventions, along with their imaging characteristics. The radiologist needs to be aware of the normal sequence of development of structures and how to select adequate imaging techniques to assess anatomy at various stages or in different postsurgical scenarios.

Omphalomesenteric Duct Anomalies in Children: A Multimodality Overview.

The omphalomesenteric duct is an embryologic structure that connects the yolk sac with the primitive midgut of the developing fetus. Omphalomesenteric duct anomalies include a group of entities that result from failed resorption of the omphalomesenteric duct. These anomalies include Meckel diverticulum, omphalomesenteric fistula, fibrous bands, cysts, and umbilical polyps. Meckel diverticulum is the most common congenital anomaly of the gastrointestinal tract and is usually asymptomatic. Symptoms develop when Meckel diverticulum involves complications such as hemorrhage, inflammation, and perforation, or when it causes intussusception or bowel obstruction. Hemorrhage is the most common complication of Meckel diverticulum, and technetium 99m-pertechnetate scintigraphy is the imaging modality of choice for detecting acute bleeding. US and CT are commonly used for the evaluation of patients with other complications such as obstruction and inflammation. Nevertheless, the diagnosis of these complications can be challenging, as their clinical manifestations are usually nonspecific and can masquerade as other acute intraabdominal entities such as appendicitis, inflammatory bowel disease, or other causes of bowel obstruction. There are other umbilical disorders, such as urachal remnants and umbilical granuloma, that may present with symptoms and imaging findings similar to those of omphalomesenteric duct anomalies. An accurate preoperative diagnosis of omphalomesenteric duct anomaly is crucial for appropriate management and a better outcome, particularly when these anomalies manifest as a life-threatening condition. The authors review the anatomy, clinical features, and complications of omphalomesenteric duct anomalies in children, describing the relevant differential diagnoses and associated imaging findings seen with different imaging modalities. ©RSNA, 2021.

Percutaneous cryoablation of chondroblastoma and osteoblastoma in pediatric patients.

BACKGROUND: To review the safety and efficacy of percutaneous cryoablation for the treatment of chondroblastoma and osteoblastoma in the pediatric and adolescent population. MATERIALS AND METHODS: A retrospective review from 2016 to 2020 was performed to evaluate clinical and imaging response to percutaneous cryoablation in 11 symptomatic patients with diagnosis of chondroblastoma and osteoblastoma treated from two pediatric hospitals with at least 12-month follow-up. Technical success (correct needle placement and potential full coverage of the tumor with the planned ablation zone) and clinical success (relief of the symptoms) were evaluated. The primary objective was to alleviate pain related to the lesion(s). Immediate and late complications were recorded. Patients were followed in clinic and with imaging studies such as MRI or CT for a minimum of 6 months. RESULTS: A total of 11 patients were included (mean 14 years, age range 9-17; male n = 8). Diagnoses were osteoblastoma (n = 4) and chondroblastoma (n = 7). Locations were proximal humerus (n = 1), femur condyle (n = 1), and proximal femur (n = 1) tibia (n = 3), acetabulum (n = 3), thoracic vertebra (n = 1) and lumbar vertebra (n = 1). Cryoablation was technically successful in all patients. Clinical success (cessation of pain) was achieved in all patients. No signs of recurrence were observed on imaging follow-up in any of the patients. One of the patients developed periprocedural right L2-L3 transient radiculopathy as major immediate complication. CONCLUSIONS: Percutaneous image-guided cryoablation can be considered potentially safe and effective treatment for chondroblastoma and osteoblastoma in children and adolescents.

MRI of Rhabdomyosarcoma and Other Soft-Tissue Sarcomas in Children.

Soft-tissue sarcomas in children comprise a heterogeneous group of entities with variable manifestation depending on the age of the patient and the location of the tumor. MRI is the modality of choice for evaluating musculoskeletal soft-tissue tumors and plays a paramount role in both initial diagnosis and assessment of tumor response during and after treatment. Conventional MRI sequences, such as T1- and T2-weighted imaging, offer morphologic information, which is important for localizing the lesion and describing anatomic relationships but not accurate for determining its malignant or benign nature and may be limited in differentiating tumor response from therapy-related changes. Advanced multiparametric MRI offers further functional information that can help with these tasks by using different imaging sequences and biomarkers. The authors present the role of MRI in rhabdomyosarcoma and other soft-tissue sarcomas in children, emphasizing a multiparametric approach with focus on the utility and potential added value of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced MRI in characterization and staging, determination of pretreatment extent, and evaluation of tumor response and recurrence after treatment. ©RSNA, 2020.

Autoinflammatory diseases in childhood, part 1: monogenic syndromes.

Autoinflammatory diseases constitute a family of disorders defined by aberrant stimulation of inflammatory pathways without involving antigen-directed autoimmunity. They may be divided into monogenic and polygenic types. Monogenic autoinflammatory syndromes are those with identified genetic mutations, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency or hyperimmunoglobulin D syndrome, cryopyrin-associated periodic fever syndromes (CAPS), pyogenic arthritis pyoderma gangrenosum and acne (PAPA) syndrome, interleukin-10 and interleukin-10 receptor deficiencies, adenosine deaminase 2 deficiency and pediatric sarcoidosis. Those without an identified genetic mutation are known as polygenic and include systemic-onset juvenile idiopathic arthritis, idiopathic recurrent acute pericarditis, Behçet syndrome, chronic recurrent multifocal osteomyelitis and inflammatory bowel disease among others. Autoinflammatory disorders are defined by repeating episodes or persistent fever, rash, serositis, lymphadenopathy, arthritis and increased acute phase reactants, and thus may mimic infections clinically. Most monogenic autoinflammatory syndromes present in childhood. However, because of their infrequency, diverse and nonspecific presentation, and the relatively new genetic recognition, diagnosis is usually delayed. In this article, which is Part 1 of a two-part series, the authors update monogenic autoinflammatory diseases in children with special emphasis on imaging features that may help establish the correct diagnosis.

Imaging Evaluation of Pediatric Parotid Gland Abnormalities.

Parotid gland lesions in children can be divided into benign or malignant. The age of the patient helps narrow the differential diagnosis, with vascular and congenital lesions being more frequent in the 1st year of life, while solid tumors are more frequent in older children. Inflammatory disease usually has rapid onset in comparison with that of neoplastic or congenital processes, which have more gradual clinical evolution. Currently, multiple imaging techniques are available to study the parotid region, such as US, CT, and MRI. However, it is still a challenge to distinguish nonmalignant lesions from malignant ones. US is the first-line diagnostic approach in children to characterize the morphology and vascularity of these lesions. CT in children may be indicated for evaluation of abscesses or sialolithiasis. MRI is the imaging modality of choice for investigating the nature of the lesion and its extent. In addition to complete and detailed clinical information, knowledge of parotid gland anatomy and characteristic radiologic features of parotid disorders is essential for optimal radiologic evaluation and avoiding unnecessary interventional diagnostic procedures or treatment. This article illustrates a variety of entities (congenital, inflammatory, vascular, neoplastic) that can occur in the parotid gland, highlighting the most frequent radiologic patterns of manifestation and correlating them with clinical, surgical, and pathologic findings. ©RSNA, 2018.

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.

A primary inflammatory myofibroblastic tumor of the scapula in a child: imaging findings.

Inflammatory myofibroblastic tumor (IMT) is an uncommon tumor characterized by inflammatory cell infiltration and differentiated myofibroblastic spindle cells. IMT was first described in the lung and retroperitoneum. Occurrence in bone has been well described in the maxilla and occasionally in the long bones in the adult population. We present a unique case of IMT arising primarily from the scapula in an 8-year-old patient, not described previously in the pediatric or adult literature. Imaging demonstrated an ill-defined and aggressive osteolytic lesion with cortical bone destruction associated with an important soft tissue component that extended into the adjacent muscles. Histologically, the tumor was composed of spindle and polygonal cells distributed in an inflammatory background with different proportions of plasma cells, lymphocytes, eosinophils and neutrophils. The absence of cellular atypia helped to differentiate this entity from malignant spindle cell tumors, and imaging could differentiate the tumor from the nontumoral inflammatory reaction.