Understanding Bilateral Skull Fractures in Infancy: A Retrospective Multicenter Case Review.

BACKGROUND: Bilateral skull fractures in infancy often raise suspicion for abuse. Nevertheless, literature suggests that they may occur accidentally. However, empiric data are lacking. OBJECTIVE: This multicenter retrospective review aimed to characterize bilateral skull fractures in a large sample. PARTICIPANTS AND SETTING: Medical records for infants younger than 24 months with bilateral skull fractures involving hospital consultation with a child abuse pediatrician (CAP) were reviewed from 2005 to 2020 at 13 nationally represented institutions. METHODS: Standardized data collection across institutions included historical features, fracture characteristics, and additional injuries, as well as the CAP's determination of accident versus abuse. Pooled data were analyzed for descriptive and bivariate analyses. RESULTS: For 235 cases, 141 were accidental, and 94 abuse. The majority occurred in young infants, and a history of a fall was common in 70% of cases. More than 80% involved both parietal bones. Bilateral simple linear fractures were more common in accidental cases, 79% versus 35%, whereas a complex fracture was more frequent in abuse cases, 55% versus 21% ( P < 0.001). Almost two thirds of accidental cases showed approximation of the fractures at the sagittal suture, compared with one third of abuse cases ( P < 0.001). Whereas focal intracranial hemorrhage was seen in 43% of all cases, diffuse intracranial hemorrhage was seen more in abuse cases (45%) than accidents (11%). Skin trauma was more common in abusive than accidental injury (67% vs 17%, P < 0.001), as were additional fractures on skeletal survey (49% vs 3%, P < 0.001). CONCLUSIONS: A fall history was common in bilateral skull fractures deemed accidental by a CAP. Most accidental cases involved young infants with biparietal simple linear fractures, without skin trauma or additional fractures. A skeletal survey may aid in the determination of accidental or abusive injury for unwitnessed events resulting in bilateral skull fractures in infants.

Debunking Fringe Beliefs in Child Abuse Imaging: AJR Expert Panel Narrative Review.

Child abuse is a global public health concern. Injuries from physical abuse may be clinically occult and not appreciable on physical examination. Imaging is therefore critical in identifying and documenting such injuries. The radiologic approach for a child who has potentially been abused has received considerable attention and recommendations according to decades of experience and rigorous scientific study. Nonetheless, fringe beliefs describing alternative explanations for child abuse-related injuries have emerged and received mainstream attention. Subsequently, imaging findings identified in abused children have been attributed to poorly supported underlying medical conditions, clouding the evidence basis for radiologic findings indicative of nonaccidental trauma. Fringe beliefs that attribute findings seen in child abuse to alternate pathologies such as genetic disorders, birth trauma, metabolic imbalances, vitamin D deficiency, and short-distance falls typically have limited evidence basis and lack professional society support. Careful review of the scientific evidence and professional society consensus statements is important in differentiating findings attributable to child abuse from fringe beliefs used to discount the possibility that a child's constellation of injuries is consistent with abuse. This review refutes fringe beliefs used to provide alternative explanations in cases of suspected child abuse and reinforces the key literature and scientific consensus regarding child abuse imaging.

Macrocephaly and subdural collections.

Enlarged subarachnoid spaces are a common finding in infants and young children imaged for macrocephaly or an enlarging head circumference, and benign enlargement of the subarachnoid spaces is often diagnosed. Infrequently, presumed "spontaneous" subdural hemorrhages or subdural collections might complicate these enlarged subarachnoid spaces. Children with large bilateral subdural collections might also present for imaging with macrocephaly. Each scenario potentially raises concerns for prior injury because subdural hemorrhage is a frequent finding in children with abusive head trauma.

Pediatric hanging and strangulation: is vascular injury a true risk?

BACKGROUND: Acute screening of pediatric strangulation and hanging injuries has evolved at many institutions to include cervical arterial vascular imaging. As current standards in pediatric imaging support less radiation exposure and increased imaging appropriateness, it is questionable whether vascular arterial injury is a true risk in this population. OBJECTIVE: To determine the role of cervical vascular arterial imaging in the evaluation of pediatric hanging and strangulation injuries. MATERIALS AND METHODS: This is a retrospective study of patients who present at a Level 1 pediatric trauma center with a history of hanging and strangulation injuries. All relevant studies, including computed tomography (CT) angiography of the neck, contrast-enhanced neck CT, cervical magnetic resonance (MR) angiography, magnetic resonance imaging (MRI) and/or CT of the brain and cervical spine and associated clinical records, were reviewed. RESULTS: Sixty-six patients were identified, 60 with vascular arterial imaging studies. No vascular injury was identified. Soft-tissue injury was noted in 20/66 (30%) patients and craniocervical injury was suspected in 2/66 (3%), but no cervical spine fracture was identified. Only 49 patients had brain imaging, with 7/49 (14%) demonstrating changes consistent with cerebral edema, which correlated negatively with survival (P<0.01). CONCLUSION: Vascular arterial imaging, particularly with CT angiography, performed in the pediatric population after hanging and strangulation injury resulted in no positive studies for cervical arterial injury. This study supports the need to reevaluate routine screening CT angiography in this study population.

Everolimus for subependymal giant cell astrocytoma: 5-year final analysis.

OBJECTIVE: To analyze the cumulative efficacy and safety of everolimus in treating subependymal giant cell astrocytomas (SEGA) associated with tuberous sclerosis complex (TSC) from an open-label phase II study (NCT00411619). Updated data became available from the conclusion of the extension phase and are presented in this ≥5-year analysis. METHODS: Patients aged ≥ 3 years with a definite diagnosis of TSC and increasing SEGA lesion size (≥2 magnetic resonance imaging scans) received everolimus starting at 3mg/m(2) /day (titrated to target blood trough levels of 5-15ng/ml). The primary efficacy endpoint was reduction from baseline in primary SEGA volume. RESULTS: As of the study completion date (January 28, 2014), 22 of 28 (78.6%) initially enrolled patients finished the study per protocol. Median (range) duration of exposure to everolimus was 67.8 (4.7-83.2) months; 12 (52.2%) and 14 (60.9%) of 23 patients experienced SEGA volume reductions of ≥50% and ≥30% relative to baseline, respectively, after 60 months of treatment. The proportion of patients experiencing daily seizures was reduced from 7 of 26 (26.9%) patients at baseline to 2 of 18 (11.1%) patients at month 60. Most commonly reported adverse events (AEs) were upper respiratory tract infection and stomatitis of mostly grade 1 or 2 severity. No patient discontinued treatment due to AEs. The frequency of emergence of most AEs decreased over the course of the study. INTERPRETATION: Everolimus continues to demonstrate a sustained effect on SEGA tumor reduction over ≥5 years of treatment. Everolimus remained well-tolerated, and no new safety concerns were noted.

The striated MR nephrogram, not a reflection of pathology.

BACKGROUND: We have intermittently observed low signal striations in the kidneys on delayed post-contrast MR exams of the spine. While we suspected these striations were due to concentrated gadolinium, the clinical importance of this finding was uncertain. OBJECTIVE: To describe the striated MR nephrogram (low signal striations in the kidney) and assess its clinical relevance. MATERIALS AND METHODS: Retrospective review of delayed post-contrast MRIs of the spine (mean: 45 min after contrast administration). The presence of the striated MR nephrogram was correlated with imaging parameters (field strength, time since contrast), and findings (gadolinium in the bladder, inferior vena cava and aorta diameters) and with clinical factors (history of renal disease, laboratory values). RESULTS: Seven hundred seventy-three exams performed on 229 patients, 8.3 ± 5.3 years of age, were reviewed. The striated MR nephrogram was observed in 102/773 examinations (13.2%) and was present on at least one study in 54/229 patients (23.6%). The presence of striations was associated with the specific magnet on which the exam was performed (P < 0.01) but not with magnet field strength. Serum creatinine was minimally lower in patients with striations (0.43 ± 0.12 vs. 0.49 ± 0.18 mg/dL, P = 0.002), but no other clinical or historical data, including time from contrast administration (P = 0.54), fluid status (P = 0.17) and clinical history of renal disease (P = 0.14), were predictive of the presence of striations. CONCLUSION: The striated MR nephrogram was observed in 13% of delayed post-contrast MR exams of the spine. Precipitating factors are unclear, but the striated nephrogram does not appear to be a marker of clinically apparent renal dysfunction.

Incidental Thalamic Lesions Identified on Brain MRI in Pediatric and Young Adult Patients: Imaging Features and Natural History.

BACKGROUND AND PURPOSE: Nonspecific, localized thalamic signal abnormalities of uncertain significance are occasionally found on pediatric brain MR imaging. The goal of this study is to describe the MR imaging appearance and natural history of these lesions in children and young adults. MATERIALS AND METHODS: This retrospective study evaluated clinically acquired brain MR imaging examinations obtained from February 1995 to March 2022 at a large, tertiary care pediatric hospital. Examinations with non-mass-like and nonenhancing thalamic lesions were identified based on term search of MR imaging reports. A total of 221 patients formed the initial group for imaging assessment. Additional exclusions during imaging review resulted in 171 patients. Imaging appearance and size changes were assessed at baseline and at follow-up examinations. RESULTS: A total of 171 patients (102 male) at a median age of 11 years (range: 1-23 years), 568 MR imaging examinations, and 180 thalamic lesions were included. Median time from baseline to the last follow-up MR imaging was 542 days (range: 46-5730 days). No lesion enhanced at any time point. On imaging follow-up, 11% of lesions (18/161) became smaller, 10% (16/161) resolved, 73% (118/161) remained stable, and 6% (9/161) increased in size at some point during evaluation. Median time interval from baseline to enlargement was 430 days (range: 136-1074 days). CONCLUSIONS: Most incidental, non-mass-like thalamic signal abnormalities were stable, decreased in size, or resolved on follow-up imaging and are likely of no clinical significance. Surveillance strategies with longer follow-up intervals may be adequate in the management of such findings.

Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis.

BACKGROUND: Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous sclerosis complex. An alternative may be the use of everolimus, which inhibits the mammalian target of rapamycin, a protein regulated by gene products involved in the tuberous sclerosis complex. METHODS: Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label study. The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months. We gave everolimus orally, at a dose of 3.0 mg per square meter of body-surface area, to achieve a trough concentration of 5 to 15 ng per milliliter. RESULTS: We enrolled 28 patients. Everolimus therapy was associated with a clinically meaningful reduction in volume of the primary subependymal giant-cell astrocytoma, as assessed on independent central review (P<0.001 for baseline vs. 6 months), with a reduction of at least 30% in 21 patients (75%) and at least 50% in 9 patients (32%). Marked reductions were seen within 3 months and were sustained. There were no new lesions, worsening hydrocephalus, evidence of increased intracranial pressure, or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma. Of the 16 patients for whom 24-hour video electroencephalography data were available, seizure frequency for the 6-month study period (vs. the previous 6-month period) decreased in 9, did not change in 6, and increased in 1 (median change, -1 seizure; P=0.02). The mean (±SD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100, with higher scores indicating a better quality of life) was improved at 3 months (63.4±12.4) and 6 months (62.1±14.2) over the baseline score (57.8±14.0). Single cases of grade 3 treatment-related sinusitis, pneumonia, viral bronchitis, tooth infection, stomatitis, and leukopenia were reported. CONCLUSIONS: Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases, though long-term studies are needed. (Funded by Novartis; ClinicalTrials.gov number, NCT00411619.).

Parenchymal Insults in Abuse-A Potential Key to Diagnosis.

Subdural hemorrhage is a key imaging finding in cases of abusive head trauma and one that many radiologists and radiology trainees become familiar with during their years of training. Although it may prove to be a marker of trauma in a young child or infant that presents without a history of injury, the parenchymal insults in these young patients more often lead to the debilitating and sometimes devastating outcomes observed in this young population. It is important to recognize these patterns of parenchymal injuries and how they may differ from the imaging findings in other cases of traumatic injury in young children. In addition, these parenchymal insults may serve as another significant, distinguishing feature when making the medical diagnosis of abusive head injury while still considering alternative diagnoses, including accidental injury. Therefore, as radiologists, we must strive to look beyond the potential cranial injury or subdural hemorrhage for the sometimes more subtle but significant parenchymal insults in abuse.

Cerebral vascular injury in transplant-associated thrombotic microangiopathy.

Transplant-associated thrombotic microangiopathy (TA-TMA) and atypical hemolytic uremic syndrome (aHUS) are complement-mediated TMAs. The central nervous system (CNS) is the most common extrarenal organ affected by aHUS, and, despite mechanistic overlap between aHUS and TA-TMA, CNS involvement is rarely reported in TA-TMA, suggesting that CNS involvement in TA-TMA may be underdiagnosed and that these patients may benefit from complement blockers. In addition, there are no widely used histologic or radiologic criteria for the diagnosis of TMA in the brain. Thirteen recipients of pediatric hematopoietic cell transplants (HCTs) who had TA-TMA and who underwent autopsy were studied. Seven of 13 brains had vascular injury, and 2 had severe vascular injury. Neurologic symptoms correlated with severe vascular injury. Classic TMA histology was present and most often observed in the cerebellum, brainstem, and cerebral white matter. Abnormalities in similar anatomic regions were seen on imaging. Brain imaging findings related to TMA included hemorrhages, siderosis, and posterior reversible encephalopathy syndrome. We then studied 100 consecutive HCT recipients to identify differences in neurologic complications between patients with and those without TA-TMA. Patients with TA-TMA were significantly more likely to have a clinical concern for seizure, have an electroencephalogram performed, and develop altered mental status. In summary, our study confirms that TA-TMA involves the brains of recipients of HCT and is associated with an increased incidence of neurologic symptoms. Based on these findings, we propose that patients with low- or moderate-risk TA-TMA who develop neurologic complications should be considered for TA-TMA-directed therapy.

Brain malformations associated with epignathus: a clue for the correct prenatal diagnosis.

We present a case of epignathus (oral teratoma) with midline intracranial associated malformations. This is a rare report of epignathus diagnosed in utero with fetal MRI description of these midline brain malformations, including duplication of the pituitary gland. This case is an example of how these intracranial findings led to the correct diagnosis of a small fetal oral mass.

Pediatric Emergencies: Imaging of Pediatric Head Trauma.

Pediatric head trauma is an important cause of morbidity and mortality in children and may be seen in the setting of accidental or abusive injuries. Although many of the patterns of head injury are similar to adults, the imaging manifestations of head injury in children are more complex due to the developing brain and calvarium. Additionally, there are unique considerations for mechanisms of injury in children, to include abusive head trauma and birth-related injuries. The primary role of the radiologist is to identify and characterize the type and severity of head injury to help guide appropriate patient management.

Reduced Field of View Diffusion-Weighted Imaging in the Evaluation of Congenital Spine Malformations.

BACKGROUND: Reduced field of view diffusion-weighted imaging (rFOV DWI) is a more recently described technique in the evaluation of spine pathology. In adults, this technique has been shown to increase clinician confidence in identification of diffusion restricting lesions. PURPOSE: In this study, we evaluate the image quality and diagnostic confidence of the rFOV DWI technique in pediatric spine MRI. METHODS: We included patients with MRI of the lumbar spine for suspected congenital abnormalities who had conventional SS-EPI (single shot echo planar imaging) with full field of view (fFOV) and rFOV DWI performed. Images were graded for image quality and observer confidence for detection of lesions with reduced diffusion. Position of the conus and L3 vertebral body measurements were recorded. Comparisons were made between the fFOV and rFOV scores. RESULTS: Fifty children (30 girls, 20 boys) were included (median 3.6 years). Compared to the fFOV images, the rFOV images scored higher in image quality (P < 0.0001) and for confidence in detecting lesions with reduced diffusion (P < 0.0001). The average spread of identified conus position was smaller for in rFOV compared to fFOV (P = 0.0042). There was no significant difference in the L3 vertebral body measurements between the two methods. In rFOV, the anterior aspects of the vertebral bodies were excluded in a few studies due to narrow FOV. CONCLUSION: rFOV DWI of the lumbar spine in the pediatric population has qualitatively improved image quality and observer confidence for lesion detection when compared to conventional fFOV SS-EPI DWI.

Adding Insult to Injury: Nonconvulsive Seizures in Abusive Head Trauma.

The primary objectives of this study were to determine the prevalence of nonconvulsive seizures and nonconvulsive status epilepticus in patients with abusive head trauma who underwent electroencephalography (EEG) monitoring and to describe predictive factors for this population. Children with a diagnosis of abusive head trauma were studied retrospectively to determine the rate of EEG monitoring, the rate of nonconvulsive seizures and nonconvulsive status epilepticus, and the associated neuroimaging findings. Over 11 years, 73 of 199 (36.8%) children with abusive head trauma had electroencephalography monitoring performed. Of these, 20 (27.4%) had nonconvulsive seizures and 3 (4.1%) had nonconvulsive status epilepticus. The presence of subarachnoid hemorrhage and cortical T2 / fluid-attenuated inversion recovery signal abnormalities were both significantly associated with the presence of nonconvulsive seizures / nonconvulsive status epilepticus. Nonconvulsive seizures are relatively common in abusive head trauma and may go unrecognized. Specific neuroimaging characteristics increase the likelihood of nonconvulsive seizures on EEG.

Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma.

OBJECTIVE: To report long-term efficacy and safety data for everolimus for the treatment of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC). METHODS: This was an open-label extension phase of a prospective, phase 1-2 trial (NCT00411619) in patients ≥3 years of age with SEGA associated with TSC. Patients received oral everolimus starting at 3 mg/m2 per day and subsequently titrated, subject to tolerability, to attain whole blood trough concentrations of 5-15 ng/mL. Change in SEGA volume, seizures, and safety assessments were the main outcome measures. RESULTS: Of 28 patients enrolled, 25 were still under treatment at the time of analysis. Median dose was 5.3 mg/m2/day and median treatment duration was 34.2 months (range 4.7-47.1). At all time points (18, 24, 30, and 36 months), primary SEGA volume was reduced by ≥30% from baseline (treatment response) in 65%-79% of patients. All patients reported ≥1 adverse event (AE), mostly grade 1/2 in severity, consistent with that previously reported, and none led to everolimus discontinuation. The most commonly reported drug-related AEs were upper respiratory infections (85.7%), stomatitis (85.7%), sinusitis (46.4%), and otitis media (35.7%). No drug-related grade 4 or 5 events occurred. CONCLUSION: Everolimus therapy is safe and effective for longer term (median exposure 34.2 months) treatment of patients with TSC with SEGA. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that everolimus, titrated to trough serum levels of 5-15 ng/mL, was effective in reducing tumor size in patients with SEGA secondary to TSC for a median of 34 months.

Combined analysis of sMRI and fMRI imaging data provides accurate disease markers for hearing impairment.

In this research, we developed a robust two-layer classifier that can accurately classify normal hearing (NH) from hearing impaired (HI) infants with congenital sensori-neural hearing loss (SNHL) based on their Magnetic Resonance (MR) images. Unlike traditional methods that examine the intensity of each single voxel, we extracted high-level features to characterize the structural MR images (sMRI) and functional MR images (fMRI). The Scale Invariant Feature Transform (SIFT) algorithm was employed to detect and describe the local features in sMRI. For fMRI, we constructed contrast maps and detected the most activated/de-activated regions in each individual. Based on those salient regions occurring across individuals, the bag-of-words strategy was introduced to vectorize the contrast maps. We then used a two-layer model to integrate these two types of features together. With the leave-one-out cross-validation approach, this integrated model achieved an AUC score of 0.90. Additionally, our algorithm highlighted several important brain regions that differentiated between NH and HI children. Some of these regions, e.g. planum temporale and angular gyrus, were well known auditory and visual language association regions. Others, e.g. the anterior cingulate cortex (ACC), were not necessarily expected to play a role in differentiating HI from NH children and provided a new understanding of brain function and of the disorder itself. These important brain regions provided clues about neuroimaging markers that may be relevant to the future use of functional neuroimaging to guide predictions about speech and language outcomes in HI infants who receive a cochlear implant. This type of prognostic information could be extremely useful and is currently not available to clinicians by any other means.

Small pituitary size in children with Fanconi anemia.

BACKGROUND: Fanconi anemia (FA) is a genetic disorder associated with multiple congenital anomalies, bone marrow failure, and pituitary hypofunction including hypogonadism, thyroid dysfunction, and growth hormone (GH) deficiency. PROCEDURE: Among 44 patients with FA referred to Cincinnati Children's Hospital Medical Center (CCHMC) between 1975 and 2005, 33 had neuroimaging studies, including 11 cranial magnetic resonance imaging (MRIs). Two separate measurements per patient from these MRIs were used to evaluate pituitary height compared to on-site control data of similar measurements of cranial MRIs on 22 age and gender-matched children without any pathology involving the hypothalamic-pituitary system. Growth pattern and endocrine studies were reviewed to assess potential correlation with pituitary size. RESULTS: When compared to the age-gender matched on-site control sample, the mean pituitary height of FA patients was significantly smaller (P < 0.0001; mean +/- SE from mixed effects model with age and gender as covariates: 3.96 +/- 0.32 vs. 5.76 +/- 0.24). Upon further adjusting for the effect of the small head size by including bi-parietal diameter (BPD) as a covariate, the difference remained statistically significant (P = 0.0013). Findings on the growth pattern and endocrinological measurements are as follows: 50% of patients with small pituitary gland were short. GH and adrenal function tests were normal in all tested patients. Thyroid, pubertal status, and glucose regulation were abnormal in 30, 50, and 75% of patients tested. CONCLUSIONS: Children with FA tend to have unsuspected small pituitary glands beyond what is expected from the effects of their stunted growth. Further studies are required to reveal the clinical implications of this finding.

Tailgut cyst in a child.

Tailgut cyst, or retrorectal cystic hamartoma, is a rare congenital lesion found in the presacral space. The lesion has been infrequently reported in the literature. We report the MRI findings of a tailgut cyst in a 2-year-old girl who presented with a sacral dimple and skin discoloration.