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1.
Pediatr Emerg Care ; 40(8): e159-e163, 2024 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-38713845

RESUMO

BACKGROUND: A removable brace with home management is widely accepted treatment for distal radius buckle fractures, which most commonly involve the dorsal cortex. PURPOSE: The purpose of this study is to determine if a removable brace and home management treatment is safe for volar distal radius buckle fractures. MATERIALS AND METHODS: Isolated distal radius buckle fractures in children (3-16 years) diagnosed at an acute care visit (April 1, 2019 to May 31, 2022) were identified. Final diagnosis was confirmed using strict criteria including cortical buckling without cortical breach or physeal involvement. Cortical buckling was categorized as either dorsal or volar. Demographic data, mechanism of injury, treatment, and any complications were recorded and analyzed. RESULTS: Three hundred thirty-three fractures were either dorsal (254, 76%) or volar (79, 24%) buckle fractures. Mean age (SD) for volar fractures (9.3 [2.2 years]; range, 4-14 years) was significantly higher than for dorsal fractures (8.5 (3.0 years); range, 3-15 years; P = 0.012). More girls had volar fractures (48 [60%], P = 0.006). Most fractures occurred after a standing-height fall. Two hundred forty-four (96%) dorsal and 76 (96%) volar fractures were initially treated with a removable brace. Two hundred fourteen (84%) dorsal and 66 (84%) volar fractures had orthopedic follow-up. Brace treatment continued for 167 (167/204, 82%) dorsal and 56 (56/63, 89%) volar fractures. Treatment changed from initial brace to cast for 37 (37/204, 18%) dorsal fractures and 7 (7/63, 11%) volar fractures, influenced by caregiver preference and/or sport participation requirements. Only 1 (1/79, 1%) patient with a volar fracture returned for an additional visit for persistent pain. CONCLUSIONS: When diagnosis of volar buckle fracture is made using the same strict criteria used for dorsal buckle fractures, removable brace and home management treatment is safe. Shared decision making with caregivers may alter buckle fracture treatment.


Assuntos
Braquetes , Fraturas do Rádio , Humanos , Feminino , Masculino , Criança , Adolescente , Pré-Escolar , Fraturas do Rádio/terapia , Estudos Retrospectivos
2.
Pediatr Qual Saf ; 8(5): e681, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37780600

RESUMO

Background: Bowel management for children with chronic constipation may include repeated single-view abdomen radiographs (AXR) to monitor treatment success. Only one image of the abdomen is needed to include most of the colon, but technologists often make a second (or even third) exposure to be sure they have imaged the entire abdomen. Our quality improvement project aimed to reduce radiation exposure by decreasing the frequency of >1 exposure performed for AXR orders in children with chronic constipation from 27% to <10% by December 2022 and sustain. Methods: We counted baseline (01/2020-11/2020) and intervention (12/2020-5/2023) examinations with >1 exposure. Initial interventions were a structured communication to technologists and an article in the monthly department newsletter and later, a technologist education module. Additional interventions included communication to radiologists, project updates and encouragement to all technologists, and individual technologist feedback. A statistical process control chart tracked data to study process changes over time. Results: During the baseline and intervention periods, 525/1944 and 1329/8334 examinations, respectively, had >1 exposure performed for AXR orders. Interventions created 2 centerline shifts. Overall, examinations with >1 exposure decreased from 27% to 13.5%. Conclusions: Frequency of >1 exposure performed for AXR orders in children with chronic constipation decreased from 27% to 13.5% through education and communication. This was sustained. We plan to assign training modules for all new technologists, policy reminders (annual training in odd years) for all technologists, and continue individualized learning opportunities.

3.
Pediatr Qual Saf ; 8(4): e673, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37551257

RESUMO

The involvement of pediatric imaging professionals in quality improvement (QI) in our department was low, with few available informatics tools to report issues or suggest improvement opportunities in a timely and efficient manner. We aimed to increase QI engagement in radiology by creating a real-time, encounter-specific reporting tool embedded into the clinical imaging workflow. Methods: A multidisciplinary team outlined requirements for a new electronic quality-reporting tool, including point-of-care access during imaging workflow and simultaneous automatic capture of encounter-specific clinical information from the hospital information system. Information system experts created a user-friendly interface for categories based on stages of imaging workflow (Planning, Acquisition, Processing, Interpretation, Communication, and Data Collection). Team members trained all department staff. Quality coordinators sorted entries and monitored personnel engagement for two 36-week periods: immediately after launch and 3 years later. Descriptive statistics were used to analyze proposed and completed QI projects during these periods. Results: There were 1,498 entries during the first 36 weeks. Ninety-three percent of radiologists and 56% of technologists participated. Three years later, there were 1,251 entries in 36 weeks. Data collection entries for established QI projects increased from 380 (25%) to 487(39%). The engagement continued among radiologists but decreased among technologists over time. Submissions for QI projects increased from baseline. The project completion rate increased. Conclusion: We created a QI reporting tool embedded into the clinical imaging workflow, which improved the participation of our imaging professionals and increased the number of completed QI projects.

4.
Pediatr Qual Saf ; 7(2): e547, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35919219

RESUMO

Introduction: Accurately distinguishing between stable and unstable isolated distal radius fractures (DRF) in children allows for appropriate fracture-specific treatment. Although fractures with cortical disruption, displacement, or angulation are unstable, distinguishing stable buckle fractures (BF) from more subtle potentially unstable DRF is challenging. Our quality improvement project aimed to improve radiology reporting accuracy for these subtle fractures from 23% to 90% in a large tertiary pediatric hospital. Methods: Exams with a reported isolated distal radius fracture during baseline (January-March 2016) and intervention (April 2016-June 2019) were reviewed for accuracy. We introduced 3 types of interventions: radiologist education (self-directed learning modules and individual feedback), a new standardized report template, and a measurement tool ("The 1 cm Rule"). In addition, a statistical process control chart tracked accuracy data to study process changes over time. Results: During the baseline and intervention period, 22 and 480 radiographs, respectively, had either a stable BF or a potentially unstable isolated DRF. Each intervention type created a centerline shift. Overall, reporting accuracy increased from 23% to 90%. Most reports (95%, 639/676) used the template and standard terminology for reporting DRF. Conclusions: Radiology reporting diagnostic accuracy for distinguishing between stable BF and potentially unstable DRF in children increased to 90% through education, standardized reporting, and a measurement tool to enhance radiologist performance. Our institution plans to expand fracture-specific treatment practices with improved radiology reporting accuracy, including bracing and home management of stable BF diagnosed during an acute care visit.

5.
Pediatr Radiol ; 52(7): 1347-1355, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35325266

RESUMO

BACKGROUND: Radiographic bone age assessment by automated software is precise and instantaneous. OBJECTIVE: The aim of this study was to evaluate the accuracy of an automated tool for bone age assessment. MATERIALS AND METHODS: We compared a total of 586 bone age radiographs from 451 patients, which had been assessed by three radiologists from 2013 to 2018, with bone age analysis by BoneXpert, using the Greulich and Pyle method. We made bone age comparisons in different patient groups based on gender, diagnosis and race, and in a subset with repeated bone age studies. We calculated Spearman correlation (r) and accuracy (root mean square error, or R2). RESULTS: Bone age analyses by automated and manual assessments showed a strong correlation (r=0.98; R2=0.96; P<0.0001), with the mean bone age difference of 0.12±0.76 years. Bone age comparisons by the two methods remained strongly correlated (P<0.0001) when stratified by gender, common endocrine conditions including growth disorders and early/precocious puberty, and race. In the longitudinal analysis, we also found a strong correlation between the automated software and manual bone age over time (r=0.7852; R2=0.63; P<0.01). CONCLUSION: Automated bone age assessment was found to be reliable and accurate in a large cohort of pediatric patients in a clinical practice setting in North America.


Assuntos
Determinação da Idade pelo Esqueleto , Software , Determinação da Idade pelo Esqueleto/métodos , Osso e Ossos , Criança , Transtornos do Crescimento , Mãos/diagnóstico por imagem , Humanos , Lactente , Radiografia
6.
Pediatr Radiol ; 52(1): 12-21, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34518936

RESUMO

Children with cerebral palsy are at increased risk of hip dislocation. Detecting hip subluxation through radiographic hip screening is an essential component of hip surveillance and has been shown to prevent hip dislocations. Large-scale hip surveillance programs are being implemented nationwide, highlighting the importance of uniform technical and reporting standards.


Assuntos
Paralisia Cerebral , Luxação do Quadril , Paralisia Cerebral/diagnóstico por imagem , Criança , Diagnóstico por Imagem , Luxação do Quadril/diagnóstico por imagem , Humanos , Radiografia
7.
Pediatr Qual Saf ; 6(6): e485, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34934874

RESUMO

Detection of hip migration in children with cerebral palsy (CP) through radiographic surveillance can prevent dislocations. Migration Percentage (MP) is the accepted method for quantifying hip subluxation in CP on pelvis x-ray but was not being reported at our institution. Our objective was to improve care for children with CP by standardizing radiographic techniques and reporting radiographs obtained as part of a hip surveillance program. METHODS: A baseline retrospective review of CP surveillance pelvis x-ray reports was performed. We then educated radiologists and technologists, standardized imaging techniques, and required structured radiology reporting to include MP measurement and dislocation risk categories. We tracked compliance with the reporting template for 10 months. Images and reports were also assessed for quality and accuracy by an orthopedic surgeon. RESULTS: Baseline period reports showed no consistency. In total, 449 children with CP (mean age: 7.3 years ± 4.2) had a surveillance pelvis radiograph during the postintervention study period (May 2019-February 2020). An estimated 90% reporting compliance was achieved and sustained by 5 months. Eight (89%) of the children with high-risk hips were newly diagnosed during our study period; all had a progressive increase in MP from prior examinations. All clinicians surveyed agreed that the standardized reports, including MP, were helpful to their practice. CONCLUSIONS: Using evidence-based process measures and quality improvement methodology, we standardized hip surveillance for children with CP. Radiology reports that include MP and risk category for hip dislocation enable clear communication for referrals across specialties and early detection and treatment for better outcomes.

8.
J Child Neurol ; 36(4): 253-261, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33522373

RESUMO

AIM: To examine associations between the deep medullary vein white matter injury global severity scoring system and neurodevelopmental impairment. METHODS: This is a prospective observational cohort study of infants born at ≥32 weeks, diagnosed with deep medullary vein thrombosis and infarction on neuroimaging in the first month of life. Developmental testing was performed using validated measures for early, preschool, and school-age follow-up. RESULTS: Nineteen (37%) patients had major neurodevelopmental impairment. Global severity score was higher among patients with neurodevelopmental impairment (21.6 vs 13.4, P = .04). Overall, 78% of patients with epilepsy had neurodevelopmental impairment. A greater degree of asymmetry with right-sided injury predominance was associated with lower Bayley-III cognitive scores and presence of neurodevelopmental impairment (P < .01). CONCLUSIONS: Results suggest a need for targeted clinical surveillance for patients with a high global severity score and/or asymmetric, predominantly right cerebral white matter injury and for those who develop epilepsy.


Assuntos
Infarto Encefálico/psicologia , Transtornos do Neurodesenvolvimento/diagnóstico , Transtornos do Neurodesenvolvimento/etiologia , Trombose Venosa/psicologia , Substância Branca/irrigação sanguínea , Substância Branca/lesões , Adolescente , Infarto Encefálico/complicações , Infarto Encefálico/diagnóstico , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Escala de Gravidade do Ferimento , Imageamento por Ressonância Magnética , Masculino , Valor Preditivo dos Testes , Trombose Venosa/complicações , Trombose Venosa/diagnóstico , Substância Branca/diagnóstico por imagem
9.
Pediatr Radiol ; 50(12): 1735-1741, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32809066

RESUMO

BACKGROUND: Tibial stress fractures are not uncommon in pediatric athletes. The severity of injury may be graded using magnetic resonance imaging (MRI). OBJECTIVE: To determine whether Fredericson MRI grading of tibial stress fractures can differentiate times to recovery across different grades in pediatric athletes. MATERIALS AND METHODS: A medical record search identified all athletes younger than 19 years old who had tibial stress fractures confirmed by MRI and were treated by sports medicine specialists in our clinic system over a 5-year period. Two pediatric radiologists graded MRI exams using the Fredericson system. Time to recovery (in days) was defined in four ways: pain onset to full participation, pain onset to zero pain, first treatment to full sport participation and first treatment to zero pain. Recovery times were compared to tibial stress fracture Fredericson MRI grade and to the use of a recovery device. RESULTS: Thirty-eight pediatric athletes (age range: 7-18 years, mean: 15.4±2.2 years) had 42 tibial stress fractures while participating in 12 different sports. About half (55%) were track and/or cross-country athletes. The mean time from diagnosis to report of no pain for all patients was 55.6±5.0 days. We found no significant difference in time to recovery across stress fracture grade or with the use of a recovery device. CONCLUSION: No differences were noted between Fredericson stress fracture grades and different time periods to recovery or between differences in recovery time and the return to full participation in sports, regardless of the use of assistive devices.


Assuntos
Traumatismos em Atletas/diagnóstico por imagem , Fraturas de Estresse/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Recuperação de Função Fisiológica , Fraturas da Tíbia/diagnóstico por imagem , Adolescente , Atletas/estatística & dados numéricos , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Tíbia/diagnóstico por imagem , Tíbia/lesões , Tempo , Índices de Gravidade do Trauma
10.
Pediatr Radiol ; 49(3): 358-364, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30547221

RESUMO

BACKGROUND: Treatment pathways for isolated distal radius fractures in children are becoming more tailored to specific fracture types and include different management plans for stable buckle fractures versus potentially unstable distal radius fractures. OBJECTIVE: To propose a measurement rule to aid in differentiating stable buckle fractures from other distal radius fractures in children. MATERIALS AND METHODS: A medical record search identified 203 children with closed distal radius fractures during a 14-month period. A consensus of two senior readers served as a reference standard to distinguish buckle fractures from other distal radius fractures. We measured fracture-to-physis distance on posteroanterior (PA) and lateral radiographs for each of the isolated distal radius fractures. We analyzed diagnostic accuracy, using fracture-to-physis distance as a predictor for buckle fractures, in children ages 3-16 years. We used a receiver operating characteristic (ROC) curve to determine the cut-off values tested. RESULTS: There were 148 buckle fractures (73%) and 55 isolated potentially unstable distal radius fractures (27%). For children 7-16 years, buckle fracture-to-physis distance was <1 cm in only 1/106 (0.9%) on the PA projection and none on the lateral projection. Diagnostic accuracy for buckle fracture diagnosis was 82% using a cut-off of 14 mm (PA) and 13 mm (lateral). In children 3-6 years old, the buckle fracture-to-physis distance was <1 cm in 1/42 (2.4%) on the PA radiographs and another 1/42 (2.4%) on the lateral radiographs. Diagnostic accuracy was low for younger children for all tested distances. CONCLUSION: We propose utilizing a measurement rule to increase diagnostic accuracy for buckle fractures in children ≥7 years old. An isolated distal radius fracture in a child 7 years or older is not likely to be a buckle fracture if the fracture-to-physis distance is <1 cm.


Assuntos
Fraturas do Rádio/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Sensibilidade e Especificidade
11.
J Neurosurg Pediatr ; 20(2): 141-148, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28548617

RESUMO

OBJECTIVE Sporadic meningiomas have been classified in many different ways. Radiographically, these lesions can be described as occurring in either typical or atypical locations. The purpose of this study was to determine if there are any histopathological differences between sporadic meningiomas that arise in these varying locations in children. METHODS The neuroimaging, histopathological findings, and clinical records in patients with sporadic pediatric meningiomas not associated with neurofibromatosis Type 2 or prior radiation therapy were retrospectively reviewed. Tumors were classified by radiological findings as either typical or atypical, and they were categorized histopathologically by using the latest WHO nomenclature and grading criteria. RESULTS Fifteen sporadic meningiomas in pediatric patients were biopsied or resected at the authors' institution between 1989 and 2013. Five (33%) were typical in radiographic appearance and/or location and 10 (67%) were atypical. Four (80%) typical meningiomas were WHO Grade I tumors. Most (60%) of the atypical meningiomas were WHO Grade II or III. CONCLUSIONS This study is the largest series of sporadic pediatric meningiomas in atypical locations to date. Although sporadic meningiomas are relatively infrequent in children, those with atypical imaging, specifically those with apparently intraparenchymal and intraosseous locations, may be more common than previously recognized. In this study, pediatric sporadic meningiomas arising in atypical locations, in particular intraparenchymal meningiomas, may be of higher histopathological grade. The authors' findings should alert clinicians to the potential for more aggressive clinical behavior in these tumors.


Assuntos
Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Meningioma/diagnóstico por imagem , Meningioma/patologia , Adolescente , Criança , Feminino , Humanos , Lactente , Masculino , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Gradação de Tumores , Estudos Retrospectivos , Adulto Jovem
12.
Pediatr Neurol ; 72: 70-75.e1, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28495146

RESUMO

BACKGROUND: The vein of Labbé is a superficial cortical vein, which drains the lateral surface of the temporal lobe. Thrombosis of the vein of Labbé can occur in the neonatal period. The developmental outcomes of infants who had vein of Labbé thrombosis are unknown as few studies of outcomes exist. METHODS: We completed a retrospective review of infants born ≥34 weeks of gestation, diagnosed with vein of Labbé thrombosis, and/or infarction on neuroimaging during the first 30 days of life. Size of each temporal lobe infarction was estimated based on the number of temporal lobe segments involved. Primary outcomes were the presence of major neurodevelopmental impairments in childhood and Bayley scores at two years. RESULTS: Our cohort of 19 infants had a median gestational age of 38 weeks (interquartile range 36 to 39) and mean birth weight 2892 ± 920 grams. The most common presenting symptoms of vein of Labbé thrombosis and infarction of surrounding tissue were seizures, apnea, lethargy, and either hypertonia or hypotonia. At the latest clinical follow-up appointment documented in the electronic medical record (mean 4.4 ± 3.08 years), 44% had major neurodevelopmental impairment. Patients with large vein of Labbé infarctions had significantly worse average Bayley scores than those with small to moderate lesions, and differences in language composite were statistically significant (72.7 vs 107.8, P = 0.017). CONCLUSIONS: Neonates with large vein of Labbé infarctions are more likely to have poor language outcomes. This finding suggests a need for targeted surveillance to ensure early identification of deficits and referral for intervention.


Assuntos
Infarto Cerebral/complicações , Veias Cerebrais/patologia , Desenvolvimento da Linguagem , Transtornos da Linguagem/etiologia , Idioma , Infarto Cerebral/patologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Transtornos da Linguagem/patologia , Masculino , Estudos Retrospectivos
13.
Pediatr Neurol ; 55: 37-45, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26724889

RESUMO

BACKGROUND: Autoimmune encephalitis is currently a clinical diagnosis without widely accepted diagnostic criteria, often leading to a delay in diagnosis. The utility of magnetic resonance imaging (MRI) and electroencephalography (EEG) in this disease is unknown. The objective of this study was to identify disease-specific patterns of neurodiagnostic studies (MRI and EEG) for autoimmune encephalitis in children. METHODS: We completed a retrospective chart review of encephalopathic patients seen at a large pediatric hospital over a four year interval. Clinical presentation, autoantibody status, and MRI and EEG findings were identified and compared. Individuals with autoantibodies were considered "definite" cases, whereas those without antibodies or those with only thyroperoxidase antibodies were characterized as "suspected." RESULTS: Eighteen patients met the inclusion criteria and autoantibodies were identified in nine of these. The patients with definite autoimmune encephalitis had MRI abnormalities within limbic structures, most notably the anteromedial temporal lobes (56%). Only individuals with suspected disease had nontemporal lobe cortical lesions. Sixteen patients had an EEG and 13 (81%) of these were abnormal. The most common findings were abnormal background rhythm (63%), generalized slowing (50%), focal slowing (43%), and focal epileptiform discharges (31%). Sleep spindle abnormalities occurred in 38% of patients. There were no specific differences in the EEG findings between the definite and suspected cases. Focal EEG findings only correlated with a focal lesion on MRI in a single definite case. CONCLUSIONS: Pediatric patients with definite autoimmune encephalitis have a narrow spectrum of MRI abnormalities. Conversely, EEG abnormalities are mostly nonspecific. All patients in our cohort had abnormalities on one or both of these neurodiagnostic studies.


Assuntos
Doenças Autoimunes/diagnóstico , Eletroencefalografia/métodos , Encefalite/diagnóstico , Imageamento por Ressonância Magnética/métodos , Adolescente , Autoanticorpos/sangue , Doenças Autoimunes/sangue , Doenças Autoimunes/patologia , Doenças Autoimunes/fisiopatologia , Criança , Pré-Escolar , Encefalite/sangue , Encefalite/patologia , Encefalite/fisiopatologia , Feminino , Humanos , Masculino , Estudos Retrospectivos
15.
Pediatr Radiol ; 45(6): 872-81, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25425210

RESUMO

BACKGROUND: Excessive cervical flexion-extension accompanying mild to severe impact injuries can lead to C2 synchondrosal fractures in young children. OBJECTIVE: To characterize and classify C2 synchondrosal fracture patterns. MATERIALS AND METHODS: We retrospectively reviewed imaging and medical records of children who were treated for cervical spine fractures at our institution between 1995 and 2014. We reviewed all fractures involving the five central C2 synchondroses with regard to patient demographics, mechanism of injury, fracture pattern, associated fractures and other injuries, treatment plans and outcome. RESULTS: Fourteen children had fractures involving the central C2 synchondroses. There were nine boys and five girls, all younger than 6 years. We found four distinct fracture patterns. Eleven complete fractures were further divided into four subtypes (a, b, c and d) based on degree of anterior displacement of the odontoid segment and presence of distraction. Nine of these 11 children had fractures through both odontoneural synchondroses and the odontocentral synchondrosis; one had fractures involving both neurocentral synchondroses and the odontoneural synchondrosis; one had fractures through bilateral odontoneural and bilateral neurocentral synchondroses. Three children had incomplete fractures, defined as a fracture through a single odontoneural synchondrosis with or without partial extension into either the odontocentral or the adjacent neurocentral synchondroses. All complete fractures were displaced or angulated. Four had associated spinal cord injury, including two contusions (subtype c fractures) and two fatal transections (subtype d fractures). Most children were treated with primary halo stabilization. Subtype c fractures required surgical fixation. CONCLUSION: We describe four patterns of central C2 synchondrosal fractures, including two unique patterns that have not been reported. We propose a classification system to distinguish these fractures and aid in treatment planning.


Assuntos
Vértebras Cervicais/lesões , Fraturas da Coluna Vertebral/classificação , Fraturas da Coluna Vertebral/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fraturas da Coluna Vertebral/terapia
16.
Pediatr Neurol ; 51(5): 734-6, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25286830

RESUMO

BACKGROUND: Nuclear polymerase gamma (POLG) mutations are the most common cause of inherited mitochondrial disease. POLG mutation diseases have a broad spectrum of clinical manifestations; the lethal infantile form is myocerebrohepatopathy spectrum. PATIENT: A 4-month-old boy was referred for poor feeding, emesis, failure to thrive, and hypotonia. RESULTS: Brain computed tomography was normal. Brain magnetic resonance imaging with and without contrast demonstrated bilateral enhancement of cranial nerves III, V-X, and the upper and midcervical nerve roots. Liver biopsy revealed early cirrhosis, steatosis, and focal necrosis. Muscle biopsy did not demonstrate specific abnormalities of mitochondrial morphology or number. Electron transport chain analysis of both fibroblasts and muscle demonstrated deficiencies. Because of suspected mitochondrial depletion disorder, testing was performed for mitochondrial abnormalities including analysis of the POLG gene, which revealed two pathogenic mutations, c.1399G>A (p.A467T) and c.3285C>G (p.S1095R). CONCLUSIONS: We report abnormal gadolinium enhancement of multiple cranial nerves and cervical nerve roots in an infant with myocerebrohepatopathy spectrum disease whose brain MRI otherwise revealed only mild atrophy. Mitochondrial disease should be included in the differential diagnosis of cranial nerve enhancement. Contrast-enhanced MRI aids in the diagnostic evaluation of infants with developmental delay and suspected neurological disease.


Assuntos
DNA Polimerase Dirigida por DNA/genética , Doenças Mitocondriais/genética , Mutação/genética , Encéfalo/patologia , DNA Polimerase gama , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Doenças Mitocondriais/patologia , Medula Espinal/patologia
17.
Pediatr Radiol ; 44(11): 1393-402, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25304462

RESUMO

BACKGROUND: Deep white matter hemorrhagic venous infarction with subsequent cavitation due to necrosis and liquefaction has been described in neonates and may be associated with infection and meningitis. In our experience, the MRI pattern of these lesions is confused with the pattern seen with cerebral abscesses. OBJECTIVE: The purpose of our study was to characterize the MRI findings of post infarction necrosis and liquefaction after hemorrhagic deep white matter venous infarction in infants and to distinguish these lesions from cerebral abscesses. MATERIALS AND METHODS: An institutional review board approved a retrospective review of imaging records to identify all patients with cerebral venous infarction at a children's hospital during a 10-year period. Nine infants had deep white matter hemorrhagic venous infarction with white matter fluid signal cavitary lesions. A diagnosis of cerebral abscess was considered in all. The imaging and laboratory findings in these patients are reviewed and compared to descriptions of abscesses found in the literature. RESULTS: There were six female and three male infants. The mean age at presentation was 20 days (range: 0-90 days), while the corrected age at presentation was less than 30 days for all patients. Seven patients presented with seizures and signs of infection; one infant presented with lethargy and later proved to have protein C deficiency. MRI was performed 0-12 days from presentation in these eight patients. Another patient with known protein C deficiency underwent MRI at 30 days for follow-up of screening US abnormalities. There were a total of 38 deep cerebral white matter fluid signal cavitary lesions: 25 frontal, 9 parietal, 2 temporal, 2 occipital. Larger lesions had dependent debris. All lesions had associated hemorrhage and many lesions had evidence of adjacent small vessel venous thrombosis. Lesions imaged after gadolinium showed peripheral enhancement. Three lesions increased in size on follow-up imaging. Three patients, two with meningitis confirmed via microbiology and one with presumed meningitis by CSF counts, underwent surgical aspiration of a total of six lesions. All specimens were sent for pathology and culture and were negative for microorganisms. CONCLUSION: Recognizing the MR appearance of cavitary necrosis and liquefaction after deep white matter cerebral venous infarction in neonates can distinguish this entity from cerebral abscess and potentially avoid an unnecessary neurosurgical aspiration procedure.


Assuntos
Abscesso Encefálico/patologia , Infarto Encefálico/patologia , Hemorragia Cerebral/patologia , Imageamento por Ressonância Magnética/métodos , Substância Branca/irrigação sanguínea , Substância Branca/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
18.
Hawaii Med J ; 68(1): 8-11, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19365922

RESUMO

A young Marshallese woman presented with the insidious development of fever, cough, fatigue, profound weakness, massive weight loss, cachexia, alopecia, amenorrhea, and periumbilical hyperpigmentation. Limited laboratory studies revealed anemia, leukocytosis, and hyponatremia. Imaging studies, as well as digital photographs, transmitted over the Internet, using the secure Pacific Island Health Care Project (PIHCP), store-and-forward telemedicine system, suggested the diagnosis of disseminated tuberculosis, and antimycobacterial antibiotics were begun. Sputum cultures eventually grew Mycobacterium tuberculosis. Based on the constellation of clinical signs and symptoms, the transmitted images, and limited laboratory data, adrenal tuberculosis (Addison's disease) with adrenal insufficiency was diagnosed and corticosteroids were initiated. The patient responded dramatically This case underscores the utility of telemedicine in the diagnosis and treatment of patients with unusual conditions, rarely seen today in the United States, from remote sites in the Developing World.


Assuntos
Doença de Addison/etiologia , Telemedicina/organização & administração , Tuberculose Pulmonar/complicações , Doença de Addison/tratamento farmacológico , Adulto , Antituberculosos/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Micronésia , Tuberculose Pulmonar/tratamento farmacológico , Adulto Jovem
19.
Pediatr Radiol ; 35(11): 1125-7, 2005 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-15931517

RESUMO

We report on a 10-year-old girl who presented with worsening pain and anorexia after blunt trauma to the abdomen. Contrast-enhanced CT of the abdomen was performed, and a counterclockwise rotation of the superior mesenteric vein around the superior mesenteric artery was seen. An upper gastrointestinal (UGI) series with small-bowel follow-through demonstrated a normally located duodenal-jejunal junction. This is the first case report of a counterclockwise barber-pole sign seen by CT with UGI that was negative for malrotation or volvulus.


Assuntos
Dor Abdominal/diagnóstico , Anorexia/diagnóstico , Volvo Intestinal/complicações , Volvo Intestinal/diagnóstico por imagem , Artérias Mesentéricas/anormalidades , Artérias Mesentéricas/diagnóstico por imagem , Dor Abdominal/etiologia , Anorexia/etiologia , Criança , Feminino , Humanos , Radiografia
20.
J Digit Imaging ; 17(4): 253-7, 2004 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-15692868

RESUMO

A study was carried out to determine whether digitized radiologic images added valuable information to Internet consultations from a remote Pacific Island. Chuuk State Hospital (Federated States of Micronesia) has limited film screen radiology, minimal ultrasound capability, and no radiologist. Providers initiate Web-based referrals for consultation or patient transfer. Digitized images (via low-cost digital camera or flatbed scanner) were uploaded to a Web site. Images were assessed for impact on referral decisions. A radiologist scored image quality and confidence (scale: 1-7). Of 97 referrals with images that were reviewed, 74 (76%) image sets were abnormal, 20 (20%) were normal, and 3 (4%) were indeterminate. Median scores were 4 for image quality and 5 for diagnostic confidence. In most cases with abnormal radiology (52/74, 70%), images were considered valuable. Radiologic images digitized with a low-cost camera or flatbed scanner provided valuable information for decision making in an Internet-based consultation and referral process from a remote, impoverished Pacific Island jurisdiction, despite relatively low image quality.


Assuntos
Internet , Encaminhamento e Consulta , Consulta Remota , Telerradiologia , Humanos , Micronésia , Intensificação de Imagem Radiográfica , Sistemas de Informação em Radiologia
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