Susan Palasis

Surgical Treatment of Epilepsy in Children Caused by Focal Cortical Dysplasia

Focal cortical dysplasia (FCD) is a congenital disorder of neuronal migration that is increasingly recognized as a common cause of seizures in children, occurring in 20–30% of all surgically treated cases of epilepsy in the pediatric population. Advances in neuroimaging have contributed to recognition of FCD. We report 15 children (9 female, 6 male) with FCD and surgically treated intractable epilepsy. In 9 cases, a surgical strategy of anatomic (frameless stereotactic) grid placement and physiologic (electrocorticography) resection was employed. Postoperative MRI scans were obtained, the pathologic specimen was graded according to the Brannstrom system, and seizure outcome was defined using the Engel classification. There were no deaths and no permanent morbidity. After, on average, 4 years since treatment, 10 children are seizure free, 2 are 2A, 2 are 2B and 1 is 3A. Predictors of good outcome are an MRI-defined lesion and increased cortical disorganization (higher Brannstrom grade). Subtotal resection did not preclude a seizure-free outcome.

Regionally Specific Cortical Thinning in Children with Sickle Cell Disease

Sickle cell disease (SCD) is a chronic disease with a significant rate of neurological complications in the first decade of life. In this retrospective study, cortical thickness was examined in children with SCD who had no detectable abnormalities on conventional magnetic resonance imaging/magnetic resonance angiography. Regional differences in cortical thickness from SCD were explored using age-matched healthy controls as comparison. A comparison analysis was done for SCD (n = 28) and controls (n = 29) based on age (5-11; 12-21 years), due to the age-dependent variation in cortex maturation. Distinct regions of thinning were found in SCD patients in both age groups. The number, spatial extent, and significance (P < 0.001) of these areas of thinning were increased in the older SCD group. Regions of interest (ROIs) were defined on the areas of highly significant thinning in the older group and then mapped onto the younger cohort; a multiparametric linear regression analysis of the ROI data demonstrated significant (P < 0.001) cortical thinning in SCD subjects, with the largest regions of thinning in the precuneus and the posterior cingulate. The regionally specific differences suggest that cortical thickness may serve as a marker for silent insults in SCD and hence may be a useful tool for identifying SCD patients at risk for neurological sequelae.

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.

Drop metastases to the pediatric spine revealed with diffusion-weighted MR imaging

Identifying drop metastases to the spine from pediatric brain tumors is crucial to treatment and prognosis. MRI is currently the gold standard for identifying drop metastases, more sensitive than CSF cytology, but imaging is not uncommonly inconclusive. Although diffusion‐weighted imaging (DWI) of the brain is very useful in the evaluation of hypercellular tumors, DWI of the spine has not been clinically useful in children because of susceptibility artifacts and lack of spatial resolution. A new technique, readout-segmented echo planar imaging (EPI), has improved these images, allowing for identification of hypercellular drop metastases. We report a case that illustrates the utility of spine DWI in the detection of metastatic disease in children with primary central nervous system (CNS) tumors. This case suggests that DWI of the spine with readout-segmented EPI should be included in the evaluation for drop metastases.

Strokes, Cutis Marmorata Telangiectatica Congenita, and Factor V Leiden

Cutis marmorata telangiectatica congenita is an uncommon, congenital cutaneous condition typified by persistent cutis marmorata and other associated abnormalities. Progressive neurologic complications are generally not a feature of the disorder. A case is reported of cutis marmorata telangiectatica congenita associated with diffuse cerebrovascular infarcts at 7 months of age. Moyamoya-like vascular abnormalities were demonstrated in addition to the factor V Leiden mutation, a congenital hypercoagulable disorder. This novel case illustrates the importance of evaluating children with strokes for congenital thrombophilic disorders.

Central nervous system relapse of treated stage IV neuroblastoma.

Abstract Neuroblastoma is the most common extracranial solid tumor in pediatrics. The long-term survival of patients with advanced-stage neurobastoma has remarkably improved secondary to aggressive treatment protocols including autologous bone marrow transplant (BMT). As a result, a different natural history of this disease is being reported with unusual, late manifestations. The central nervous system (CNS), once a rare site of disease, is being involved with increasing frequency. Appropriate neuroimaging in these patients is important. Two cases of patients with treated stage IV neuroblastoma who developed isolated CNS metastases are presented. The proposed pathogenesis and neuroradiologic manifestations of this complication are reviewed.

Laryngeal and tracheal anomalies in an infant with oral-facial-digital syndrome type VI (Váradi-Papp): report of a transitional type

The oral-facial-digital syndromes (OFDS) comprise a group of disorders involving malformations of the mouth, face, and digits. There are 13 subtypes of the OFDS, and much overlap exists among OFDS patients. Distinct syndromes such as Joubert and Pallister-Hall display many of the same features. This report describes an infant with abnormalities including a hypoplastic/absent cerebellar vermis and forked third metacarpals, consistent with a diagnosis of OFDS type VI (Váradi-Papp). The girl’s abnormalities also included malformations of the larynx and trachea, findings never before described in type VI but described in other OFDS subtypes and similar syndromes. Our patient represents a transitional OFDS type, further supporting evidence of a common molecular pathway among these disorders. This report highlights the importance of the radiologist’s role in diagnosis.

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 Central Role of Community-Practicing Pediatricians in Contemporary Concussion Care: A Case Study of Children's Healthcare of Atlanta's Concussion Program.

Increased lay media coverage of the long-term impact of concussions and the widespread adoption of “concussion laws” mandating professional clearance for returnto-play and return-to-learn are driving an increasing number of children with suspected concussions to seek clinical care. Providing concussion-specific decision support tools for community-practicing, pediatric care providers is critical to address both the surging demand and the need for practice guidance for clinicians unaccustomed to routinely treating concussion patients. To address these challenges in the contemporary management of youth concussions, a multidisciplinary group of clinicians at Children’s Healthcare of Atlanta (CHOA), a tertiary care pediatric hospital, developed a comprehensive, evidence-based, community concussion program. The core feature of the program was to enable community pediatricians to play the central role in managing these patients. Extensive partnering and education programs were instituted with the goal of ensuring that the necessary tools were readily available to support primary care physicians. Other elements of CHOA’s Concussion Program include providing appropriate tertiary support for those children who remain symptomatic beyond the expected course, partnering with statewide preventive programs, and supporting concussion-related research efforts. The design and implementation of the CHOA Concussion Program is presented here, with the hope that it will serve as a template for other communities. Increasing Case Load

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.