Chronic non-bacterial osteomyelitis (CNO) is a rare auto-inflammatory bone disorder, with a prevalence of around one in a million patients. In the more severe form, it is referred to as chronic recurrent multifocal osteomyelitis (CRMO). We present the current knowledge on epidemiology, pathophysiology as well as diagnostic options and treatment regimens. CNO/CRMO most commonly affects children and lesions are often seen in the metaphyseal plates of the long bones, but cases have been described affecting all age groups as well as lesions in almost every bone. It is, therefore, a disease that clinicians can encounter in many different settings. Diagnosis is mainly a matter of exclusion from differential diagnoses such as bacterial osteomyelitis and cancer. Magnetic resonance imaging is the best radiological method for diagnosis coupled with a low-grade inflammation and a history of recurring episodes. Treatment is based on case reports and consists of alleviating symptoms with non-steroidal anti-inflammatory drugs since the disease is often self-limiting. Recently, more active treatments using either bisphosphonates or biological treatment are becoming more common, to prevent long term bone damage. In general, due to its rarity, much remains unclear regarding CNO/CRMO. We review the known literature on CNO/CRMO and propose areas of interest as well as possible ways to make current diagnostic criteria more detailed. We also find unifocal cases of the jaw to be a possible sub-type that may need its own set of criteria.

https://link.springer.com/content/pdf/10.1007%2Fs00223-018-0495-0.pdf

Chronic Non-bacterial Osteomyelitis: A Review.

Innate immune system represents the ancestral defense against infectious agents preserved along the evolution and species; it is phylogenetically older than the adaptive immune system, which exists only in the vertebrates. Cells with phagocytic activity such as neutrophils, macrophages, and natural killer (NK) cells play a key role in innate immunity. In 1999 Kastner et al. first introduced the term "autoinflammation" describing two diseases characterized by recurrent episodes of systemic inflammation without any identifiable infectious trigger: Familial Mediterranean Fever (FMF) and TNF Receptor Associated Periodic Syndrome (TRAPS). Autoinflammatory diseases (AIDs) are caused by self-directed inflammation due to an alteration of innate immunity leading to systemic inflammatory attacks typically in an on/off mode. In addition to inflammasomopathies, nuclear factor (NF)-κB-mediated disorders (also known as Rhelopathies) and type 1 interferonopathies are subjects of more recent studies. This review aims to provide an overview of the field with the most recent updates (see "Most recent developments in.." paragraphs) and a description of the newly identified AIDs.

Periodic fever syndromes and the autoinflammatory diseases (AIDs)

The use of PET/computed tomography (CT) for the evaluation and management of children, adolescents, and young adults continues to expand. The principal tracer used is 18F-fluorodeoxyglucose and the principal indication is oncology, particularly musculoskeletal neoplasms. The purpose of this article is to review the common applications of PET/CT for imaging of musculoskeletal issues in pediatrics and to introduce the use of PET/CT for nononcologic issues, such as infectious/inflammatory disorders, and review the use of 18F-sodium fluoride in trauma and sports-related injuries.

Pediatric Musculoskeletal Imaging: The Indications for and Applications of PET/Computed Tomography.

The enigmatic role and development of the clavicle

The proposed model describes in a quality way the process of tumor-imaging radiopharmaceutical 99mTc-MIBI distribution with taking into account radiopharmaceutical accumulation, elimination, and radioactive decay. The dependencies of concentration versus the time are analyzed. The model can be easily tested by the concentration data of the radioactive pharmaceuticals in the blood measured at early time point and late time point of the scanning, and the obtained data can be used for determination of the washout rate coefficient which is one of the existing oncology diagnostics methods.

/pdf/mathematical-simulation-of-transport-kinetics-of-tumor-3sdhaxdn4p.pdf

Mathematical Simulation of Transport Kinetics of Tumor-Imaging Radiopharmaceutical 99mTc-MIBI.

Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory disease of unknown cause. In general, CRMO follows a characteristic clinical course and is regarded at present as a distinct entity. It affects bone and occurs predominantly in children and adolescents. The clinical, radiologic and pathologic findings are non-specific. The recognition of this rare entity is often delayed and difficulties in patient management sometimes emerge from its usual protracted course. We present a 6-year-old girl diagnosed with CRMO involving tibia and lumbar vertebra where a multidisciplinary approach was essential in making the diagnosis.

Chronic Recurrent Multifocal Osteomyelitis in Children: A Multidisciplinary Approach is needed to establish a Diagnosis.

Kikuchi-Fujimoto disease, known as Kikuchi disease, is a rare benign and self-limiting disorder that typically affects the regional cervical lymph nodes. Generalized lymphadenopathy and extranodal involvement are rare. We report a rare case of a 19-year-old female with a history of persistent fever, nausea, and debilitating malaise. Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) revealed multiple hypermetabolic generalized lymph nodes in the cervical, mediastinum, axillary, abdomen and pelvic regions with diffuse spleen, diffuse thyroid gland, and focal parotid involvement, bilaterally. In addition, subcutaneous lesions were noted in the left upper paraspinal and occipital regions. An excisional lymph node biopsy guided by 18F-FDG PET/CT revealed the patient's diagnosis as Kikuchi syndrome.

/pdf/kikuchi-disease-with-generalized-lymph-node-spleen-and-nis58bzpuj.pdf

Kikuchi Disease with Generalized Lymph Node, Spleen and Subcutaneous Involvement Detected by Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

Metabolic bone disease due to hyperparathyroidism is characterized by increased bone resorption and new bone formation. (99m)Tc- hexakis-2-methoxyisobutylisonitrile ((99m)Tc MIBI) accumulation is controlled by metabolic function and cell viability. To investigate, for the first time, the potential of whole body (99m)Tc MIBI scan for detecting, visually and with the aid of quantitative analysis, bony changes associated with hyperparathyroidism. Eighty-six patients with hyperparathyroidism, referred routinely for parathyroid localization, were included in this case-control prospective study. Each patient was injected with 20-25 mCi of (99m)Tc MIBI. Routine anterior cervico-thoracic images were obtained for parathyroid localization. Two extra whole body images were acquired and assessed visually and by drawing regions of interest over the mandible, sternum, femur, humeri, spine, and the soft tissue adjacent to the bone. The ratios of bone to soft tissue were calculated and compared to ratios drawn in a control group routinely referred for cardiac imaging and injected with (99m)Tc MIBI, after confirming the absence of any bone disease. The visual interpretation of the scans showed 48 patients to have increased bone uptake. Quantitative assessment showed significant difference between the mean ratios of the case and control groups. The Kruskal-Wallis test showed significant agreement between visual and quantitative ratios drawn from delayed right and left femora and left humerus images (P < 0.05). (99m)Tc MIBI whole body imaging is a potentially useful technique for assessing metabolic bone disease associated with hyperparathyroidism. Quantitative analysis helped in confirming the visual findings.

(99m)Tc-MIBI Whole Body Scan: A Potentially Useful Technique for Evaluating Metabolic Bone Disease

Gardner syndrome, also known as familial colorectal polyposis, is an autosomal dominant disease and is part of the spectrum of familial adenomatous polyposis (FAP) syndromes. It is characterized by the presence of multiple polyps in the colon together with neoplasms outside the colon. Gardner syndrome is also associated with desmoid tumors in approximately 15% of affected individuals. Desmoid tumors are rare benign slow-growing neoplasms, arising from connective tissue (musculoaponeurotic fibrous tissue). Due to their locally aggressive nature, the term aggressive fibromatosis have been used to describe these tumors. Seven percent of patients with FAP or its variants may also have adrenal masses, most being benign and asymptomatic. We report the PET/CT of a 42 year old patient who was diagnosed with Gardner syndrome, demonstrating the rare coexistence of the intestinal polyps, extra and intra-abdominal desmoids and adrenal adenoma by PET/CT. In addition, the characteristic cutaneous lesions and osteomas of the skull were also identified.

/pdf/gardner-syndrome-with-extra-and-intra-abdominal-desmoid-31yw0o1qs6.pdf

Rasha A Ashkanani

Papers

Chronic Recurrent Multifocal Osteomyelitis in Children: A Multidisciplinary Approach is needed to establish a Diagnosis.

Kikuchi Disease with Generalized Lymph Node, Spleen and Subcutaneous Involvement Detected by Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

(99m)Tc-MIBI Whole Body Scan: A Potentially Useful Technique for Evaluating Metabolic Bone Disease

Gardner Syndrome with Extra and Intra-Abdominal Desmoid Tumors and Adrenal Involvement: PET/CT Findings