Imaging of Kingella kingae musculoskeletal infections in children: a series of 5 cases.

PURPOSE: Kingella kingae musculoskeletal infections continue to be under-diagnosed and there remains a paucity of literature on its imaging features. The purpose of this manuscript is to review the imaging, clinical, and laboratory findings of microbiology-proven K. kingae infections. MATERIALS AND METHODS: A retrospective review of musculoskeletal infections between January 1, 2013 and Dec 31, 2016 yielded 134 patients from whom 5 patients had confirmed K. kingae infections (3 boys and 2 girls, mean age of 16 months, range 9-38 months). Picture archiving and communication system and electronic medical records were reviewed. RESULTS: At presentation, none of the patients had a fever and not all patients had abnormal inflammatory markers. Three patients had septic arthritis (2 knee and 1 sternomanubrial joints), one had epiphyseal osteomyelitis, and one had lumbar spondylodiscitis. The case of epiphyseal osteomyelitis of the distal humerus also had elbow joint involvement. A combination of radiography (n = 4), ultrasound (n = 2), and magnetic resonance (MR) imaging (n = 5) were performed. Prominent synovial thickening was observed for both knee and elbow joints and extensive regional myositis for all except for the patient with sternomanubrial joint infection. The diagnosis of K. kingae infection resulted in a change in the antibiotic regimen in 80% of the patients. CONCLUSION: Disproportionate synovial thickening, prominent peri-articular myositis, and/or characteristic sites of involvement demonstrating imaging features of infection or inflammation in a young child with mild infectious symptoms and elevated inflammatory markers should invoke the possibility of an underlying K. kingae infection.

Cornelia de Lange syndrome: Correlation of brain MRI findings with behavioral assessment.

Neurobehavioral and developmental issues with a broad range of deficits are prominent features of Cornelia de Lange syndrome (CdLS), a disorder due to disruption of the cohesin protein complex. The etiologic relationship of these clinical findings to anatomic abnormalities on neuro-imaging studies has not, however, been established. Anatomic abnormalities in the brain and central nervous system specific to CdLS have been observed, including changes in the white matter, brainstem, and cerebellum. We hypothesize that location and severity of brain abnormalities correlate with clinical phenotype in CdLS, as seen in other developmental disorders. In this study, we retrospectively evaluated brain MRI studies of 15 individuals with CdLS and compared these findings to behavior at the time of the scan. Behavior was assessed using the Aberrant Behavior Checklist (ABC), a validated behavioral assessment tool with several clinical features. Ten of fifteen (67%) of CdLS patients had abnormal findings on brain MRI, including cerebral atrophy, white matter changes, cerebellar hypoplasia, and enlarged ventricles. Other findings included pituitary tumors or cysts, Chiari I malformation and gliosis. Abnormal behavioral scores in more than one behavioral area were seen in all but one patient. All 5 of the 15 (33%) patients with normal structural MRI studies had abnormal ABC scores. All normal ABC scores were noted in only one patient and this was correlated with moderately abnormal MRI changes. Although our cohort is small, our results suggest that abnormal behaviors can exist in individuals with CdLS in the setting of relatively normal structural brain findings. © 2016 Wiley Periodicals, Inc.

MR Imaging of Pediatric Bone Marrow.

The bone marrow is one of the largest organs in the body and is visible in every magnetic resonance (MR) imaging study. It is composed of a combination of hematopoietic red marrow and fatty yellow marrow, and its composition changes throughout life in response to normal maturation (red to yellow conversion) and stress (yellow to red reconversion). MR imaging is highly sensitive for detection of altered marrow signal intensity, and the T1-weighted spin-echo sequence provides the most robust contrast between yellow marrow and disease. Heterogeneous red marrow and red marrow hyperplasia can mimic marrow disease, but should be distinguished from neoplastic replacement (leukemia, lymphoma, primary bone sarcomas, hematogenous metastases) and expected posttreatment changes (radiation therapy, chemotherapy, colony-stimulating factor, bone marrow transplant). Nonneoplastic edema-like processes can also alter marrow signal intensity, including trauma, infection, inflammation (chronic recurrent multifocal osteomyelitis, juvenile inflammatory arthritis), altered biomechanics, and chronic regional pain syndrome. Unfortunately, MR imaging findings are often nonspecific and overlap among many of these vastly different causes. Therefore, a definitive diagnosis is reliant on a combination of imaging findings, clinical evaluation, laboratory assessment, and occasionally tissue analysis. ©RSNA, 2016.

Bilateral Rasmussen Encephalitis: Good Outcome Following Hemispherotomy.

BACKGROUND: Bilateral Rasmussen encephalitis is a rare variant of a debilitating, typically unihemispheric disease with limited treatment options. Few cases with bilateral histopathology have been reported, all with poor seizure control following surgery. Here we report a favorable outcome following hemispherotomy in a four-year-old male with biopsy-confirmed bilateral disease. CASE: The patient presented with right hemispheric focal seizures with behavioral arrest and over a year progressed to left lower extremity clonic seizures, epilepsia partialis continua, and loss of ambulation, with transient response to steroids and tacrolimus. Histopathology confirmed bilateral disease. The patient developed super-refractory status epilepticus and underwent right functional hemispherotomy 4.5 years after initial presentation. In a 2.5-year follow-up period, an Engel 1D outcome classification was observed with substantially improved quality of life. CONCLUSION: Previous reports of bilateral Rasmussen encephalitis describe universally poor outcomes, and hemispherotomy is often considered contraindicated. However, hemispherotomy in a patient with bilateral Rasmussen encephalitis may have a good outcome if seizures are unihemispheric.

Magnetic Resonance Imaging Findings in Infants with Severe Traumatic Brain Injury and Associations with Abusive Head Trauma.

Young children with severe traumatic brain injury (TBI) have frequently been excluded from studies due to age and/or mechanism of injury. Magnetic resonance imaging (MRI) is now frequently being utilized to detect parenchymal injuries and early cerebral edema. We sought to assess MRI findings in infants with severe TBI, and to determine the association between specific MRI findings and mechanisms of injury, including abusive head trauma (AHT). MRI scans performed within the first 30 days after injury were collected and coded according to NIH/NINDS Common Data Elements (CDEs) for Neuroimaging in subjects age < 2 years old with severe TBI enrolled in the Approaches and Decisions in Acute Pediatric Traumatic Brain Injury Trial. Demographics and injury characteristics were analyzed. A total of 81 children were included from ADAPT sites with MRI scans. Median age was 0.77 years and 57% were male. Most common MRI finding was ischemia, present in 57/81 subjects (70%), in a median of 7 brain regions per subject. Contusion 46/81 (57%) and diffuse axonal injury (DAI) 36/81 (44.4%) subjects followed. Children were dichotomized based on likelihood of AHT with 43/81 subjects classified as AHT. Ischemia was found to be significantly associated with AHT (p = 0.001) and "inflicted" injury mechanism (p = 0.0003). In conclusion, the most common intracerebral injury seen on MRI of infants with severe TBI was ischemia, followed by contusion and DAI. Ischemia was associated with AHT, and ischemia affecting > 4 brain regions was predictive of AHT.

Epidural Fibrin Sealant Injection for the Management of Cerebrospinal Fluid Leak Following Dural Puncture in Children.

Cerebrospinal fluid (CSF) leak, intracranial hypotension, and postdural puncture headaches are common following dural punctures. Management usually consists of conservative treatments with medications (e.g. caffeine, nonsteroidal anti-inflammatory drugs, steroids, opioids), increased fluid intake, and bed rest. In more severe and persistent cases, epidural blood patches (EBPs) are indicated. When multiple EBPs fail, epidural injection of fibrin sealant has been successful in a few reported adult cases. The authors describe the first reported clinical experiences of epidural fibrin patch in children for repair of CSF leak and resolution of intracranial hypotension. This technique was used in three cases where serial EBPs failed to resolve symptoms related to intracranial hypotension following dural puncture. Following the procedure, each patient had resolution of their presenting clinical symptoms and radiographic abnormalities, and there were no noted complications. Epidural fibrin sealant injection is a reasonable option for relieving intracranial hypotension due to CSF leak following dural puncture in children.

Pediatric extraosseous sacral chordoma: case report and literature review of embryonic derivation and clinical implications.

An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Neoplastic transformation of the notochord is reviewed to illustrate the developmental basis for the surgical anatomy and pathogenesis of the classic chordoma variant. Clinical and pathological features are reviewed to differentiate this chordoma presentation from classic osseous chordomas and ecchordosis physaliphora, a related benign developmental notochordal lesion. Finally, the role of developmental signaling in the pathogenesis of chordomas from postembryonic notochordal tissue is discussed.

Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study.

OBJECTIVE: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction. METHODS: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx. RESULTS: Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications. CONCLUSIONS: This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Overdrainage-related ependymal bands: a postulated cause of proximal shunt obstruction.

OBJECTIVEVentricular shunts have an unacceptably high failure rate, which approaches 50% of patients at 2 years. Most shunt failures are related to ventricular catheter obstruction. The literature suggests that obstructions are caused by in-growth of choroid plexus and/or reactive cellular aggregation. The authors report endoscopic evidence of overdrainage-related ventricular tissue protrusions ("ependymal bands") that cause partial or complete obstruction of the ventricular catheter.METHODSA retrospective review was completed on patients undergoing shunt revision surgery between 2008 and 2015, identifying all cases in which the senior author reported endoscopic evidence of ependymal tissue in-growth into ventricular catheters. Detailed clinical, radiological, and surgical findings are described.RESULTSFifty patients underwent 83 endoscopic shunt revision procedures that revealed in-growth of ventricular wall tissue into the catheter tip orifices (ependymal bands), producing partial, complete, or intermittent shunt obstructions. Endoscopic ventricular explorations revealed ependymal bands at various stages of development, which appear to form secondarily to siphoning. Ependymal bands are associated with small ventricles when the shunt is functional, but may dilate at the time of obstruction.CONCLUSIONSVentricular wall protrusions are a significant cause of proximal shunt obstruction, and they appear to be caused by siphoning of surrounding tissue into the ventricular catheter orifices.