Novel Development of a Large Cerebral Cavernous Malformation in an Adolescent With a History of Familial Cerebral Cavernous Malformation Syndrome.

Cerebral cavernous malformations (CCM) are capillary vascular malformations of the central nervous system (CNS). These lesions can be either familial or sporadic. We present a case of a 16-year-old girl with familial CCM syndrome who presented with a six-month history of chronic headaches. A magnetic resonance imaging (MRI) scan revealed a large cavernoma in the right frontal lobe that had not been present on a prior scan performed eight years earlier. This case presentation demonstrates the possibility of significant novel cavernoma development further into adolescence.

Tethered Cord Syndrome: Role of Imaging Findings in Surgical Decision-Making.

For infants presenting with urinary problems or lower extremity weakness, imaging is ordered to investigate spinal pathology. Tethered cord syndrome (TCS) often manifests without conclusive anatomic evidence. In our case, a premature infant presented with urosepsis and was found to have an asymmetric gluteal crease and a sacral dimple. Renal ultrasound showed mild hydronephrosis, and a cystourethrogram revealed bilateral high-grade vesicoureteral reflux. Ultrasound and magnetic resonance imaging demonstrated a borderline low-lying spinal cord at the mid-L3 vertebral level. Urodynamic testing to confirm neurogenic bladder could not be completed on the first attempt due to urinary tract infection and on the second attempt due to instrument intolerance. Despite the lack of conclusive imaging evidence of a tethered cord, enough supportive clinical data was present to proceed with surgical intervention with the goal of preventing the progression of neurological dysfunction. Because TCS is ultimately a clinical diagnosis, appropriate management should not be discouraged by inconclusive or borderline imaging findings.

Radiologic image sharing among U.S. children's hospitals.

BACKGROUND: There is no streamlined approach for sharing radiologic images among medical institutions. Common methods to transfer imaging between facilities include electronic image-sharing platforms and physical media, such as compact discs (CDs). The prompt and secure transfer of imaging is vital for patient safety as demand for imaging increases. OBJECTIVE: Use a survey-based study to outline the methods and difficulties of image sharing among U.S. children's hospitals. MATERIALS AND METHODS: A multi-question survey regarding radiologic image sharing was distributed to children's hospital department chairs in the United States in August 2021. Descriptive statistical analyses of the results were performed. RESULTS: Our results reveal 78% of responding U.S. children's hospitals have an electronic image-sharing platform. Twenty-seven percent of surveyed institutions experience daily difficulties with radiologic image sharing. Most of the difficulties are with CDs (67%) and a lack of interoperability among electronic image-sharing platforms (51%). CONCLUSION: Our study identified the various methods used by U.S. children's hospitals for radiologic image sharing and quantified the ongoing challenges with these systems.

Bifid Mandibular Condyle With Associated Temporomandibular Joint Ankylosis: A Rare Skeletal Abnormality.

Bifid mandibular condyle (BMC) is splitting the mandibular condyle into two separate articular surfaces. The etiology is poorly understood, but trauma and developmental issues are currently the most cited causes. Though most often asymptomatic, occasionally, this condition may cause the development of jaw pain, clicking, and restriction of motion. We present a rare case of a patient who developed unilateral ankylosis of the temporomandibular joint (TMJ) secondary to BMC in the absence of trauma or infection. The ankylosis developed due to abnormal biomechanical forces and degenerative arthritis secondary to the abnormal articulation of the TMJ caused due to BMC. CT imaging is the best modality to evaluate the bony anatomy of the TMJ. It is essential to consider BMC as a cause of TMJ pathology, as management is primarily surgical in nature.

Thyroid Teratoma in a Pediatric Patient.

Congenital thyroid teratomas are rare in the pediatric population as well as in the adult population. While they are typically found in the gonadal regions, extragonadally, they are commonly found in the sacrococcygeal region, with teratomas of the head and neck rarely found, comprising only about 1%-6% of all pediatric teratomas. Due to a concern for potential airway compromise and increased risk of malignancy with age, early surgical excision is recommended. In this case report, we present a two-year-old female who underwent laryngoscopy with subsequent right thyroid lobectomy for a large thyroid mass, which was found to be a congenital thyroid teratoma.

Neuroimaging of Propofol Infusion Syndrome: A Case Report and Review of Literature.

A school-age boy with a complex medical history underwent a minor elective surgical procedure. Propofol was used for sedation during the procedure. The patient could not be awakened post-operatively. Laboratory findings demonstrated metabolic lactic acidosis, leukocytosis with bandemia, and transaminitis. Neuroimaging demonstrated findings that were consistent with hypoxic-ischemic or toxic-metabolic brain injury involving the bilateral basal ganglia, hippocampi, and cerebellum. The patient's condition progressively worsened over the course of the following few weeks, and brain death was confirmed by scintigraphy seven weeks later. Prompt neuroimaging in unresponsive patients with suspected propofol infusion syndrome (PRIS) is of critical importance in detecting neurologic injuries, excluding alternative diagnoses, and determining prognostication.

ACR Appropriateness Criteria® Scoliosis-Child.

Scoliosis is frequently encountered in childhood, with prevalence of 2%. The majority is idiopathic, without vertebral segmentation anomaly, dysraphism, neuromuscular abnormality, skeletal dysplasia, tumor, or infection. As a complement to clinical assessment, radiography is the primary imaging modality used to classify scoliosis and subsequently monitor its progression and response to treatment. MRI is utilized selectively to assess for neural axis abnormalities in those at higher risk, including those with congenital scoliosis, early onset idiopathic scoliosis, and adolescent idiopathic scoliosis with certain risk factors. CT, although not routinely employed in the initial evaluation of scoliosis, may have a select role in characterizing the bone anomalies of congenital scoliosis and in perioperative planning. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Headache-Child.

Headaches in children are not uncommon and have various causes. Proper neuroimaging of these children is very specific to the headache type. Care must be taken to choose and perform the most appropriate initial imaging examination in order to maximize the ability to properly determine the cause with minimum risk to the child. This evidence-based report discusses the different headache types in children and provides appropriate guidelines for imaging these children. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Back Pain-Child.

It is now generally accepted that nontraumatic back pain in the pediatric population is common. The presence of isolated back pain in a child has previously been an indication for imaging; however, recently a more conservative approach has been suggested using clinical criteria. The presence of constant pain, night pain, and radicular pain, alone or in combination, lasting for 4 weeks or more, constitute clinical red flags that should prompt further imaging. Without these clinical red flags, imaging is likely not indicated. Exceptions include an abnormal neurologic examination or clinical and laboratory findings suggesting an infectious or neoplastic etiology, and when present should prompt immediate imaging. Initial imaging should consist of spine radiographs limited to area of interest, with spine MRI without contrast to evaluate further if needed. CT of the spine, limited to area of interest, and Tc-99m bone scan whole body with single-photon emission computed tomography may be useful in some patients. The addition of intravenous contrast is also recommended for evaluation of a potential neoplastic or infectious process. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Suspected Physical Abuse-Child.

The youngest children, particularly in the first year of life, are the most vulnerable to physical abuse. Skeletal survey is the universal screening examination in children 24 months of age and younger. Fractures occur in over half of abused children. Rib fractures may be the only abnormality in about 30%. A repeat limited skeletal survey after 2 weeks can detect additional fractures and can provide fracture dating information. The type and extent of additional imaging for pediatric patients being evaluated for suspected physical abuse depends on the age of the child, the presence of neurologic signs and symptoms, evidence of thoracic or abdominopelvic injuries, and social considerations. Unenhanced CT of the head is the initial study for suspected intracranial injury. Clinically occult abusive head trauma can occur, especially in young infants. Therefore, head CT should be performed in selected neurologically asymptomatic physical abuse patients. Contrast-enhanced CT of the abdomen/pelvis is utilized for suspected intra-abdominal or pelvic injury. Particular attention should be paid to discrepancies between the patterns of injury and the reported clinical history. Making the diagnosis of child abuse also requires differentiation from anatomical and developmental variants and possible underlying metabolic and genetic conditions. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Diffusion-weighted Imaging Using Readout-segmented EPI Reveals Bony Metastases from Neuroblastoma.

Identifying neuroblastoma (NBL) metastases is crucial to treatment and prognosis. Metaiodobenzylguanidine and Tc99M bone scans are standard for identifying bony metastases but can underestimate disease. Diffusion-weighted imaging (DWI) of the spine has shown promise in evaluating bony metastases but has been limited by artifacts. Readout-segmented echo planar imaging is a technique for DWI that minimizes artifacts allowing for improved identification of spinal disease. This report illustrates the utility of DWI of the spine using readout-segmented echo planar imaging in the detection of bony NBL metastases in a child, lending support that DWI should be included in magnetic resonance imaging scans for NBL.

The etiology and significance of fractures in infants and young children: a critical multidisciplinary review.

This paper addresses significant misconceptions regarding the etiology of fractures in infants and young children in cases of suspected child abuse. This consensus statement, supported by the Child Abuse Committee and endorsed by the Board of Directors of the Society for Pediatric Radiology, synthesizes the relevant scientific data distinguishing clinical, radiologic and laboratory findings of metabolic disease from findings in abusive injury. This paper discusses medically established epidemiology and etiologies of childhood fractures in infants and young children. The authors also review the body of evidence on the role of vitamin D in bone health and the relationship between vitamin D and fractures. Finally, the authors discuss how courts should properly assess, use, and limit medical evidence and medical opinion testimony in criminal and civil child abuse cases to accomplish optimal care and protection of the children in these cases.

Opioid overdose in a child: case report and discussion with emphasis on neurosurgical implications.

In environments in which opioids are increasingly abused for recreation, children are becoming more at risk for both accidental and nonaccidental intoxication. In toxic doses, opioids can cause potentially lethal acute leukoencephalopathy, which has a predilection for the cerebellum in young children. The authors present the case of a 2-year-old girl who suffered an accidental opioid overdose, presenting with altered mental status requiring cardiorespiratory support. She required emergency posterior fossa decompression, partial cerebellectomy, and CSF drainage due to cerebellar edema compressing the fourth ventricle. To the authors' knowledge, this is the first report of surgical decompression used to treat cerebellar edema associated with opioid overdose in a child.

Acquired pathology of the pediatric spine and spinal cord.

Pediatric spine pathology poses a diagnostic challenge for radiologists. Acquired spine pathology often yields nonspecific signs and symptoms in children, especially in the younger age groups, and diagnostic delay can carry significant morbidity. This review is focused on some of the more common diagnostic dilemmas we face when attempting to evaluate and diagnose acquired pediatric spine anomalies in daily practice. An understanding of some of the key differentiating features of these disease processes in conjunction with pertinent history, physical exam, and advanced imaging techniques can indicate the correct diagnosis.

ACR Appropriateness Criteria Vomiting in Infants up to 3 Months of Age.

Vomiting is a commonly reported symptom in infants less than three months of age. There are a multitude of pathologies to consider, both within and outside the gastrointestinal tract. In addition to conducting a thorough history and physical examination, a clinician formulates a reasonable differential diagnosis by consideration of two main factors: the infant's age and the characterization of the vomit as bilious or nonbilious. In this endeavor, the clinician is able to determine if an imaging study is needed and, if so, the urgency of the request. A review of the appropriate imaging evaluation of vomiting infants in the newborn to three-month-old age group is provided by organizing the discussion around the following three clinical scenarios: bilious vomiting, intermittent nonbilious vomiting since birth, and new-onset bilious vomiting. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every three years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Neurocognitive improvements following endovascular repair of vein of Galen malformation in a child.

Cognitive regression is a well-described presentation of vein of Galen aneurysmal malformations (VGAMs) in childhood. However, it remains unclear whether successful treatment of the malformation can reverse cognitive regression. Here, the authors present the case of a 5-year-old girl with a VGAM that was treated with staged endovascular embolization procedures. Comprehensive neurocognitive assessments were completed before intervention and approximately 6 years after initial presentation. There were significant age-matched improvements in this child's neurocognitive profile over this period. The authors believe that timely and successful treatment of VGAM in children may not only stabilize the associated cognitive deterioration but, in some cases, may ameliorate these deficits. Details of this case and a discussion of neurocognitive deficits related to VGAM are presented.

ACR Appropriateness Criteria head trauma--child.

Head trauma is a frequent indication for cranial imaging in children. CT is considered the first line of study for suspected intracranial injury because of its wide availability and rapid detection of acute hemorrhage. However, the majority of childhood head injuries occur without neurologic complications, and particular consideration should be given to the greater risks of ionizing radiation in young patients in the decision to use CT for those with mild head trauma. MRI can detect traumatic complications without radiation, but often requires sedation in children, owing to the examination length and motion sensitivity, which limits rapid assessment and exposes the patient to potential anesthesia risks. MRI may be helpful in patients with suspected nonaccidental trauma, with which axonal shear injury and ischemia are more common and documentation is critical, as well as in those whose clinical status is discordant with CT findings. Advanced techniques, such as diffusion tensor imaging, may identify changes occult by standard imaging, but data are currently insufficient to support routine clinical use. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Response to bevacizumab, irinotecan, and temozolomide in children with relapsed medulloblastoma: a multi-institutional experience.

PURPOSE: Chemotherapy for relapsed medulloblastoma has been inadequate, and most patients succumb to disease. METHODS: We retrospectively reviewed nine cases of relapsed medulloblastoma treated with bevacizumab, irinotecan, ± temozolomide. Patients received one to three prior therapeutic regimens. Five patients received 10 mg/kg bevacizumab and 125-150 mg/m(2) irinotecan IV every 2 weeks, with temozolomide, starting at a median dose of 150 mg/m(2) orally for 5 days monthly. Two patients received bevacizumab and irinotecan, but not temozolomide, due to provider preference. Two of nine patients received 15 mg/kg bevacizumab IV, 90 mg/m(2) irinotecan orally for five consecutive days, 100 mg/m(2)/day temozolomide IV for 5 days, and 1.5 mg/m(2) vincristine IV, each administered every 21 days. RESULTS: Median time to progression was 11 months. Median overall survival was 13 months. Objective tumor response at 3 months was 67 %, including six patients with partial response (PR) and three patients with stable disease (SD). At 6 months, objective response was 55 %, with two patients with PR and three with complete response. Additionally, one patient had SD and three had PD. Two patients remain alive and progression free at 15 and 55 months; another is alive with disease at 20 months. Toxicities included two patients with grade III neutropenia, two with grade III thrombocytopenia, one with grade III elevation of liver function tests, and one patient with grade III diarrhea. CONCLUSIONS: The combination of bevacizumab and irinotecan, with or without temozolomide, produces objective responses with minimal toxicity in children with recurrent medulloblastoma. Prospective clinical trials are needed to evaluate the efficacy of this strategy.