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INTRODUCTION: At our institution, revascularization after indirect moyamoya surgery is routinely evaluated using magnetic resonance angiography (MRA) rather than catheter angiography. In this paper, we review how revascularization can be visualized on axial MRA and compare its visualization on MRA to that on catheter angiography. We also compare clinical outcomes of patients followed with routine postoperative MRA with outcomes of patients followed with routine catheter angiography. METHODS: We retrospectively reviewed the records of all patients treated at our institution who underwent unilateral encephaloduroarteriosynangiosis (EDAS) and/or pial synangiosis between the ages of 1 and 21 years and between December 31, 2003, and May 1, 2021. We included patients who underwent EDAS/pial synangiosis at other hospitals as long as they met all inclusion criteria. Inclusion criteria included having a preoperative MRA within 18 months of surgery and a postoperative MRA 3-30 months after surgery. Clinical outcomes included development of postoperative stroke and transient ischemic attacks (TIAs) and changes in symptoms (improved, unchanged, or worsened), including seizures, balance issues, and headaches. Clinical outcomes were compared between patients who had routine postoperative MRA only versus those who had routine postoperative angiograms, with or without routine MRA. For each surgery, we determined the ratios of the diameters and areas of the donor vessel and the contralateral corresponding vessel, as well as the relative signal intensities of these two vessels, on preoperative and 3- to 30-month postoperative MRA. We did the same for the middle meningeal artery (MMA) ipsilateral to the donor artery and the contralateral MMA. We assessed changes from pre- to post-operation in diameter ratios, area ratios, relative signal intensity, ivy sign, and in brain perfusion on arterial spin labeled (ASL) imaging. MRI and MRA measures of revascularization and flow were compared to Matsushima grades in patients who had postoperative catheter angiograms. RESULTS: Fifty-one operations for 42 unique patients were included. There were no significant differences in the rates of postoperative strokes, postoperative TIAs, changes in symptoms, or new symptoms after surgeries evaluated by routine postoperative MRA versus catheter angiogram (p = 0.282, 1, 0.664, and 0.727, respectively). There were significant associations between greater collateralization on postoperative MRA and greater median increases in preoperative-to-postoperative ratios of donor-vessel-over-contralateral-vessel diameter (p = 0.0461), ipsilateral-MMA-over-contralateral-MMA diameter (p = 0.0135), and the summed donor and ipsilateral MMA diameters over the summed contralateral vessel diameters (p < 0.001). The median increase in the ratio of the donor vessel and contralateral corresponding vessel diameters was significantly higher for Matsushima grade A versus B (p = 0.036). The median increase in the ratio of the sum of donor and ipsilateral MMA diameters over the sum of the contralateral vessel diameters was significantly higher for improved-versus-unchanged perfusion on ASL imaging (p = 0.0074). There was a nonsignificant association between greater postoperative collateralization on MRA and Matsushima grade (p = 0.1160). CONCLUSION: Cerebral revascularization after EDAS and pial synangiosis can be evaluated on axial MRA by comparing the diameter and/or signal intensity of the donor vessel and corresponding contralateral vessel, as well as the ipsilateral and contralateral MMA, on postoperative-versus-preoperative MRA. The use of routine postoperative MRA rather than catheter angiography does not appear to negatively affect outcomes.
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BACKGROUND: CT is the standard imaging technique to evaluate pediatric sinuses. Given the potential risks of radiation exposure in children, it is important to reduce pediatric CT dose and maintain image quality. OBJECTIVE: To study the utility of spectral shaping with tin filtration to improve dose efficiency for pediatric sinus CT exams. MATERIALS AND METHODS: A head phantom was scanned on a commercial dual-source CT using a conventional protocol (120 kV) and a proposed 100 kV with a 0.4-mm tin filter (Sn100 kV) protocol for comparison. Entrance point dose (EPD) of eye and parotid gland region was measured by an ion chamber. Sixty pediatric sinus CT exams (33 acquired with 120 kV, 27 acquired with Sn100 kV) were retrospectively collected. All patient images were objectively measured for image quality and blindly reviewed by 4 pediatric neuroradiologists for overall noise, overall diagnostic quality, and delineation of 4 critical paranasal sinus structures, using a 5-point Likert scale. RESULTS: Phantom CTDIvol from Sn100 kV is 4.35 mGy, compared to CTDIvol of 5.73 mGy from 120 kV at an identical noise level. EPD of sensitive organs decreases in Sn100 kV (e.g., right eye EPD 3.83±0.42 mGy), compared to 120 kV (5.26±0.24 mGy). Patients in the 2 protocol groups were age and weight (unpaired T test P>0.05) matched. The patient CTDIvol of Sn100 kV (4.45±0.47 mGy) is significantly lower than 120 kV (5.56±0.48 mGy, unpaired T test P<0.001). No statistically significant difference for any subjective readers' score (Wilcoxon test P>0.05) was found between the two groups, indicating proposed spectral shaping provides equivalent diagnostic image quality. CONCLUSION: Phantom and patient results demonstrate that spectral shaping can significantly reduce radiation dose for non-contrast pediatric sinus CT without compromising diagnostic quality.
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Estaño , Tomografía Computarizada por Rayos X , Humanos , Niño , Tomografía Computarizada por Rayos X/métodos , Estudios Retrospectivos , Reducción Gradual de Medicamentos , Dosis de RadiaciónRESUMEN
OBJECTIVE: We aimed to determine if T2-weighted hyperintense white matter lesions (WMLs) on brain magnetic resonance imaging (MRI) occur more frequently in pediatric patients with migraine and other primary headache disorders compared to the general pediatric population. BACKGROUND: Small foci of T2 hyperintensity in the white matter are frequently identified on brain MRI during the workup of pediatric headache. Such lesions have been reported to be more common among adults with migraine versus adults without migraine; however, this association has not been well established in the pediatric population. METHODS: We performed a retrospective cross-sectional single-center study of electronic medical records and radiologic studies, examining pediatric patients from 3 to 18 years old who underwent brain MRI between 2016 and 2021. Patients with existing intracranial disease or abnormalities were excluded. Patients with reports of headache were categorized. Imaging was reviewed to determine the number and location of WMLs. Headache-associated disability scores (Pediatric Migraine Disability Assessment) were noted, when available. RESULTS: Brain MRI of 248 patients with a diagnosis of headache (144 with migraine, 42 with non-migraine primary headache, and 62 with headache that could not be further classified) and 490 controls were reviewed. WMLs were encountered commonly among all study participants, with a prevalence of 40.5% (17/42) to 54.1% (265/490). There was no statistically significant difference comparing the number of lesions between each of the headache groups and the control group: migraine group versus control group median [interquartile range (IQR)], 0 [0-3] versus 1 [0-4], incidence rate ratio [95% confidence interval (CI)], 0.99 [0.69-1.44], p = 0.989, non-migraine headache group versus control group median [IQR], 0 [0-3] versus 1 [0-4], 0.71 [0.46-1.31], p = 0.156, headache not otherwise specified group versus control group median [IQR], 0 [0-4] versus 1 [0-4], 0.77 [0.45-1.31], p = 0.291. There was no significant correlation between headache-associated disability and the number of WMLs (0.07 [-0.30 to 0.17], rho [95% CI]). CONCLUSION: T2 hyperintense WMLs are common within the pediatric population and are not encountered more frequently in pediatric patients with migraine or other primary headache disorders. Thus, such lesions are presumably incidental and unlikely related to headache history.
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Trastornos Migrañosos , Sustancia Blanca , Adulto , Humanos , Niño , Preescolar , Adolescente , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/patología , Estudios Retrospectivos , Estudios Transversales , Trastornos Migrañosos/diagnóstico por imagen , Trastornos Migrañosos/epidemiología , Trastornos Migrañosos/patología , Cefalea/diagnóstico por imagen , Cefalea/epidemiología , Cefalea/patología , Imagen por Resonancia Magnética/métodosRESUMEN
INTRODUCTION: Encephaloduroarteriosynangiosis (EDAS) for moyamoya is predominantly performed using a branch of the superficial temporal artery (STA) as the donor artery. At times, other branches of the external carotid artery are better suited for EDAS than is the STA. There is little information in the literature concerning using the posterior auricular artery (PAA) for EDAS in the pediatric age-group. In this case series, we review our experience using the PAA for EDAS in children and adolescents. CASE PRESENTATIONS: We describe the presentations, imaging, and outcomes of 3 patients in whom the PAA was used for EDAS, as well our surgical technique. There were no complications. All 3 patients were confirmed to have radiologic revascularization from their surgeries. All patients also had improvement of their preoperative symptoms, and no patient has had a stroke postoperatively. CONCLUSION: The PAA is a viable option for use as a donor artery in EDAS for the treatment of moyamoya in children and adolescents.
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Revascularización Cerebral , Enfermedad de Moyamoya , Accidente Cerebrovascular , Adolescente , Niño , Humanos , Arterias/cirugía , Revascularización Cerebral/métodos , Enfermedad de Moyamoya/diagnóstico por imagen , Enfermedad de Moyamoya/cirugía , Accidente Cerebrovascular/etiología , Resultado del TratamientoRESUMEN
BACKGROUND: Intracranial arachnoid cysts are relatively common in the pediatric population. Rarely, they rupture, leading to acute subdural fluid collections, which can cause a sudden increase in intracranial pressure. The purpose of this study was to characterize ophthalmic sequelae in a large cohort of these patients. METHODS: The medical records of all children treated for ruptured arachnoid cysts who presented at a single tertiary pediatric hospital for initial assessment between 2009 and 2021 were reviewed retrospectively. RESULTS: Of 35 children treated for ruptured arachnoid cysts during the study period, 30 received ophthalmological examination. Papilledema was found in 57% of these children, abducens palsy in 20%, and retinal hemorrhages in 10%. Of the 30 children, 22 were seen in outpatient follow-up, of whom 5 had a best-corrected visual acuity of 20/40 or worse in one or both eyes at most recent follow-up. Cranial nerve palsies resolved in all cases without strabismus surgery. CONCLUSIONS: Given high rates of papilledema, cranial nerve palsies, and vision loss, all children with ruptured arachnoid cysts should be evaluated by pediatric ophthalmologists.
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Quistes Aracnoideos , Papiledema , Niño , Humanos , Quistes Aracnoideos/complicaciones , Quistes Aracnoideos/diagnóstico , Quistes Aracnoideos/cirugía , Papiledema/diagnóstico , Papiledema/etiología , Estudios Retrospectivos , Trastornos de la Visión , Rotura/complicacionesRESUMEN
BACKGROUND: Cerebral sinovenous thrombosis (CSVT) has been proposed in legal settings to be an atraumatic mimic of abusive head trauma (AHT). OBJECTIVE: The objective of this study was to determine the prevalence of CSVT and subdural hemorrhage (SDH) in a large AHT population. MATERIALS AND METHODS: This retrospective cohort study measured the prevalence of CSVT and SDH on magnetic resonance venograms in 243 patients diagnosed with AHT at a single center. We also reported additional intra- and extracranial injuries, head injury severity and length of hospital stay. RESULTS: Among 243 patients diagnosed with AHT, 7% (16/243) had CSVT. SDH was present in 94% (15/16) of the CSVT cases. Cytotoxic edema and subarachnoid hemorrhage were in 88% (14/16) and 69% (11/16) of the CSVT cases, respectively. Extracranial signs of abuse were also in 100% (16/16) of the patients with CSVT. Critical to maximal head injury severity (abbreviated injury scale >=5) was in 75% (12/16) of the CSVT population vs. 33% (82/243) in the total AHT population. Length of hospital and pediatric intensive care unit stay was greater in those with CSVT (10 vs. 21.9 and 3.5 vs. 7.3 days). CONCLUSION: These findings suggest that CSVT is uncommon in AHT and is associated with additional traumatic injuries and greater injury severity.
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Maltrato a los Niños , Traumatismos Craneocerebrales , Trombosis , Niño , Humanos , Lactante , Estudios Retrospectivos , Prevalencia , Traumatismos Craneocerebrales/diagnóstico por imagen , Traumatismos Craneocerebrales/epidemiología , Traumatismos Craneocerebrales/complicaciones , Hematoma Subdural/diagnóstico por imagen , Hematoma Subdural/epidemiología , Maltrato a los Niños/diagnóstico , Trombosis/complicacionesRESUMEN
Fetal MRI is a safe, noninvasive examination of the fetus and placenta, a complement to ultrasonography. MRI provides detailed CNS evaluation, including depicting parenchymal architecture and posterior fossa morphology, and is key in prenatal assessment of spinal dysraphism, neck masses, and ventriculomegaly. Fetal MRI is typically performed after 22 weeks gestation, and ultrafast T1 and T2-weighted MRI sequences are the core of the exam, with advanced sequences such as diffusion weighted imaging used for specific questions. The fetal brain grows and develops rapidly, and familiarity with gestational age specific norms is essential to MRI interpretation.
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Feto , Diagnóstico Prenatal , Femenino , Feto/diagnóstico por imagen , Edad Gestacional , Humanos , Imagen por Resonancia Magnética/métodos , Embarazo , Atención Prenatal , Diagnóstico Prenatal/métodosRESUMEN
Congenital malformations of the spine and spinal cord are a large and diverse group of diagnoses, which are often broadly referred to as spinal dysraphisms (SDs). Derived from the Greek words dys (bad) and raphe (suture), the term dysraphism describes missteps in the process of forming a midline seam during the zipper-like fusion of the neural folds in primary neurulation. As such, the term "spinal dysraphism" is a designation that should technically be reserved for malformations resulting from aberrations in primary neurulation. In medical practice, however, it is a catch-all designation regularly used to describe any of the numerous abnormalities demonstrating incomplete midline closure of mesenchymal, osseous, and nervous tissue, occurring at any point during embryologic development. For the sake of clarity and completeness, this article will also include that breadth in the discussion of congenital abnormalities of the spine.
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Anomalías Múltiples , Disrafia Espinal , Humanos , Imagen por Resonancia Magnética , Médula Espinal/anomalías , Disrafia Espinal/diagnóstico por imagen , Columna Vertebral/anomalías , Columna Vertebral/diagnóstico por imagenRESUMEN
Macrocephaly is a common diagnosis in the pediatric population, particularly in the infantile time period. There is a wide range of causes of macrocephaly, from benign to malignant, for which imaging plays a key role in the diagnosis and clinical guidance. Our aim is to review the distinct and prevalent neuroimaging findings in the evaluation of the macrocephalic infant.
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Megalencefalia , Niño , Humanos , Lactante , Megalencefalia/diagnóstico por imagen , NeuroimagenRESUMEN
BACKGROUND: Use of magnetic resonance imaging (MRI) as a tool to aid in neuroprognostication after cardiac arrest (CA) has been described, yet details of specific indications, timing, and sequences are unknown. We aim to define the current practices in use of brain MRI in prognostication after pediatric CA. METHODS: A survey was distributed to pediatric institutions participating in three international studies. Survey questions related to center demographics, clinical practice patterns of MRI after CA, neuroimaging resources, and details regarding MRI decision support. RESULTS: Response rate was 31% (44 of 143). Thirty-four percent (15 of 44) of centers have a clinical pathway informing the use of MRI after CA. Fifty percent (22 of 44) of respondents reported that an MRI is obtained in nearly all patients with CA, and 32% (14 of 44) obtain an MRI in those who do not return to baseline neurological status. Poor neurological examination was reported as the most common factor (91% [40 of 44]) determining the timing of the MRI. Conventional sequences (T1, T2, fluid-attenuated inversion recovery, and diffusion-weighted imaging/apparent diffusion coefficient) are routinely used at greater than 97% of centers. Use of advanced imaging techniques (magnetic resonance spectroscopy, diffusion tensor imaging, and functional MRI) were reported by less than half of centers. CONCLUSIONS: Conventional brain MRI is a common practice for prognostication after CA. Advanced imaging techniques are used infrequently. The lack of standardized clinical pathways and variability in reported practices support a need for higher-quality evidence regarding the indications, timing, and acquisition protocols of clinical MRI studies.
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Imagen de Difusión Tensora , Paro Cardíaco , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Niño , Imagen de Difusión por Resonancia Magnética/métodos , Paro Cardíaco/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética/métodos , Encuestas y CuestionariosRESUMEN
PURPOSE: The circle of Willis is a circulatory anastomosis that supplies blood to the brain. If any of the bridging segments are hypoplastic or absent, the capacity for collateral flow in the setting of large vessel occlusion may be decreased. Outside of the neonatal period, the prevalence of a complete circle of Willis (CoW) in the pediatric population has not been well described. Our objectives include determining the prevalence of a complete CoW in children and identifying if there is an age-related "loss" of arterial segments. METHODS: Following IRB approval, angiograms of the CoW performed on a 3-T MR platform from 2016 to 2020 on patients 21 years or younger were retrospectively reviewed. Any patient with underlying arterial pathology that may affect the CoW was excluded. Patient age and gender at the time of imaging were obtained. RESULTS: In total, 592 pediatric CoW were assessed. Frequencies of completeness were calculated in two different fashions: scenario 1 where a CoW was characterized as complete even if it contained hypoplastic vessels (88.8%), and scenario 2 where it was characterized as complete after excluding hypoplastic vessels (44.0%). In both scenarios, our data showed that older age was more associated with an incomplete CoW (p < 0.0001). In addition, we found a higher percentage of males with an incomplete CoW compared with females (p < 0.0001). CONCLUSIONS: The presence of a complete CoW is greater in our pediatric population than what has been reported in adults. The prevalence of an incomplete circle of Willis also increases significantly with age.
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Encéfalo , Círculo Arterial Cerebral , Niño , Círculo Arterial Cerebral/diagnóstico por imagen , Círculo Arterial Cerebral/patología , Femenino , Humanos , Masculino , Prevalencia , Estudios RetrospectivosRESUMEN
We herein report a case of Childhood Primary Central Nervous System (CNS) Angiitis. This case consisted of a 14-year-old girl who presented with right-sided weakness, aphasia, and a fever. A Magnetic Resonance Imaging of the brain showed multifocal areas of ischemia. Magnetic Resonance Angiography of the head and neck showed narrowing and irregularities of the left middle cerebral artery and right posterior cerebral artery. Cerebrospinal Fluid studies showed a lymphocytic pleocytosis and brain biopsy revealed leptomeningeal and perivascular inflammation. The epidemiology, presenting symptoms, work-up, pathophysiology, diagnostic criteria, and treatment of Childhood Primary CNS angiitis are discussed. This case serves as a reminder that when pediatric patients present with stroke-like symptoms inflammatory etiologies including Primary CNS Angiitis must be considered and treated appropriately in a time sensitive manner.
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INTRODUCTION: While cases of acquired Chiari I malformation following ventriculoperitoneal shunting for posthemorrhagic hydrocephalus have been reported, true disproportionate cerebellar growth is rare, with no previous cases requiring posterior fossa decompression reported. CLINICAL PRESENTATION: We present a premature neonate who underwent ventriculoperitoneal shunt placement for suspected posthemorrhagic hydrocephalus. He subsequently developed a symptomatic Chiari I malformation with volumetric measurements demonstrating disproportionate growth of the cerebellum. He did not demonstrate thickening of the supratentorial or posterior fossa cranium. The patient underwent an extradural posterior fossa decompression, with resolution of symptoms. OUTCOME AND CONCLUSIONS: We review the extant literature regarding the development of Chiari malformation type I as a manifestation of craniocerebral disproportion (CCD) following shunt placement for posthemorrhagic hydrocephalus of prematurity. Most previous reports reflect a mechanism that includes underdevelopment of the intracranial posterior fossa (or supratentorial) volume. The case presented in this report, as well as one additional case, indicates that there may exist a variant mechanism, characterized by rapid growth of the cerebellum itself, in the absence of one of the rare syndromes associated with primary macrocerebellum. While this case was effectively managed with extradural posterior fossa decompression, previous reports indicate that supratentorial cranial expansion procedures are preferable in some cases. As such, pediatric neurosurgeons should be able to distinguish the patterns of craniocerebral disproportion when considering treatment options for these patients. Further investigation regarding these uncommon patients may better describe the underlying mechanisms.
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Malformación de Arnold-Chiari , Hidrocefalia , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/cirugía , Cerebelo/diagnóstico por imagen , Cerebelo/cirugía , Niño , Fosa Craneal Posterior/diagnóstico por imagen , Fosa Craneal Posterior/cirugía , Descompresión Quirúrgica , Humanos , Hidrocefalia/diagnóstico por imagen , Hidrocefalia/etiología , Hidrocefalia/cirugía , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Derivación VentriculoperitonealRESUMEN
Krabbe disease is a progressive neurologic disorder caused by deficiency of the lysosomal enzyme galactocerebrosidase. The disease commonly has an early-infantile onset, but can have late-infantile, juvenile, or adult-onset phenotypes. Classic computed tomography (CT) and magnetic resonance imaging (MRI) findings in Krabbe have been well described. We report a patient, ultimately diagnosed with juvenile-onset Krabbe, who presented with atypical CT imaging and rapid disease progression. Our patient was a previously healthy and developmentally appropriate female who presented at 3 years 4 months of age with ataxia and motor regression that had progressed over the course of 6 weeks without an identifiable catalyst. CT, performed in the emergency setting, demonstrated extensive white matter hyperdensity. Subsequent MRI showed T2 hyperintensity of the white matter corresponding to the areas of hyperdensity on the CT, as well as enhancement of multiple cranial nerves bilaterally, suggestive of Krabbe disease. Enzymatic testing demonstrated low galactocerebrosidase activity and molecular testing of GALC revealed compound heterozygosity for 2 known pathogenic mutations, consistent with a diagnosis of Krabbe Disease. This included the common 30-kb deletion and a known pathogenic mutation associated with juvenile/adult-onset disease. Our patient's diffuse hyperdensity on CT offers a new radiographic finding to include in the repertoire of Krabbe imaging, and thus aide in the diagnostic evaluation. The rapidity of progression our patient demonstrated is additionally unique and should be considered in the identification of juvenile Krabbe as well as the complicated decision-making process regarding potential treatments.
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Leucodistrofia de Células Globoides/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Neuroimagen/métodos , Tomografía Computarizada por Rayos X/métodos , Encéfalo/diagnóstico por imagen , Preescolar , Progresión de la Enfermedad , Femenino , HumanosRESUMEN
PURPOSE: To describe the ocular complications experienced by patients with pontine tegmental cap dysplasia (PTCD) and the management strategies used to care for these children. METHODS: Subjects with PTCD were recruited through social media advertisement and completed a survey gathering information on potential ocular problems related to the patient's PTCD disease and any current or previous treatments. RESULTS: Twenty-two patients or guardians completed the survey. Neurotrophic cornea was the most common ocular diagnosis (82%), followed by facial palsy (59%), dry eye syndrome (59%), and blepharitis (55%). Other diagnoses included cortical visual impairment (27%), strabismus (27%), amblyopia (18%), and nystagmus (18%). Common treatment modalities included lubricating eye drops (59%) or ointment (50%), contact lenses (14%), punctal plugs (27%), glasses (45%), and patching (18%). The most common surgical interventions were temporary or permanent tarsorrhaphy (64%) and amniotic membrane grafts (23%). In total, 68% of families reported self-injury to eyes and 91% reported the child to be primarily a visual learner. CONCLUSIONS: PTCD is a newly described, very rare disorder with a variety of vision-threatening ocular manifestations. It is essential that the ophthalmologist be aware of the potential for neurotrophic cornea because timely treatment could prevent corneal scarring, perforation, and blindness.
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Enfermedades Cerebelosas/diagnóstico , Enfermedades de los Nervios Craneales/diagnóstico , Discapacidades del Desarrollo/diagnóstico , Oftalmopatías/diagnóstico , Malformaciones del Sistema Nervioso/diagnóstico , Tegmento Pontino/anomalías , Adolescente , Enfermedades Cerebelosas/etiología , Preescolar , Enfermedades de los Nervios Craneales/etiología , Discapacidades del Desarrollo/etiología , Oftalmopatías/etiología , Femenino , Humanos , Masculino , Malformaciones del Sistema Nervioso/etiologíaRESUMEN
BACKGROUND: In May, 2018, Children's Hospital Colorado noted an outbreak of enterovirus A71 (EV-A71) neurological disease. We aimed to characterise the clinical features of EV-A71 neurological disease during this outbreak. METHODS: In this retrospective observational cohort study, children (younger than 18 years) who presented to Children's Hospital Colorado (Aurora, CO, USA) between March 1 and November 30, 2018, with neurological disease (defined by non-mutually exclusive criteria, including meningitis, encephalitis, acute flaccid myelitis, and seizures) and enterovirus detected from any biological specimen were eligible for study inclusion. The clinical characteristics of children with neurological disease associated with EV-A71 were compared with those of children with neurological disease associated with other enteroviruses during the same period. To explore the differences in clinical presentation of acute flaccid myelitis, we also used a subgroup analysis to compare clinical findings in children with EV-A71-associated acute flaccid myelitis during the study period with these findings in those with enterovirus D68 (EV-D68)-associated acute flaccid myelitis at the same hospital between 2013 and 2018. FINDINGS: Between March 10 and Nov 10, 2018, 74 children presenting to Children's Hospital Colorado were found to have enterovirus neurological disease; EV-A71 was identified in 43 (58%) of these children. The median age of the children with EV-A71 neurological disease was 22·7 months (IQR 4·0-31·9), and most of these children were male (34 [79%] children). 40 (93%) children with EV-A71 neurological disease had findings suggestive of meningitis, 31 (72%) children showed evidence of encephalitis, and ten (23%) children met our case definition of acute flaccid myelitis. All children with EV-A71 disease had fever and 18 (42%) children had hand, foot, or mouth lesions at or before neurological onset. Children with EV-A71 disease were best differentiated from those with other enteroviruses (n=31) by the neurological findings of myoclonus, ataxia, weakness, and autonomic instability. Of the specimens collected from children with EV-A71, this enterovirus was detected in 94% of rectal, 79% of oropharyngeal, 56% of nasopharyngeal, and 20% of cerebrospinal fluid specimens. 39 (93%) of 42 children with EV-A71 neurological disease who could be followed up showed complete recovery by 1-2 months. Compared with children with EV-D68-associated acute flaccid myelitis, children with EV-A71-associated acute flaccid myelitis were younger, showed neurological onset earlier after prodromal symptom onset, had milder weakness, showed more rapid improvement, and were more likely to completely recover. INTERPRETATION: This outbreak of EV-A71 neurological disease, the largest reported in the Americas, was characterised by fever, myoclonus, ataxia, weakness, autonomic instability, and full recovery in most patients. Because EV-A71 epidemiology outside of Asia remains difficult to predict, identification of future outbreaks will be aided by prompt recognition of these distinct clinical findings, testing of non-sterile and sterile site specimens, and enhanced enterovirus surveillance. FUNDING: None.
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Infecciones por Enterovirus/epidemiología , Enterovirus/aislamiento & purificación , Enfermedades del Sistema Nervioso/virología , Preescolar , Colorado/epidemiología , Brotes de Enfermedades , Infecciones por Enterovirus/virología , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
COL4A1 mutations present with a spectrum of clinical phenotypes often involving the cerebrovascular and ophthalmic systems. We report 2 cases of COL4A1 mutations that presented with congenital cataracts and porencephaly. Both patients had posterior cortical cataracts and radiographically defined bilateral posterior lenticonus. Considering the long-term clinical implications of these mutations, posterior cortical cataracts, bilateral posterior lenticonus, and porencephaly should raise clinical suspicion for COL4A1 mutations.
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Anomalías Múltiples , Catarata/genética , Colágeno Tipo IV/genética , ADN/genética , Mutación , Porencefalia/genética , Encéfalo/diagnóstico por imagen , Catarata/congénito , Catarata/diagnóstico , Colágeno Tipo IV/metabolismo , Análisis Mutacional de ADN , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética/métodos , Masculino , Linaje , Fenotipo , Porencefalia/diagnósticoRESUMEN
Understanding the abundance and fate of human viral pathogens in wastewater is essential when assessing the public health risks associated with wastewater discharge to the environment. Typically, however, the microbiological monitoring of wastewater is undertaken on an infrequent basis and peak discharge events may be missed leading to the misrepresentation of risk levels. To evaluate diurnal patterns in wastewater viral loading, we undertook 3-day sampling campaigns with bi-hourly sample collection over three seasons at three wastewater treatment plants. Untreated influent was collected at Ganol and secondary-treated effluent was sampled at Llanrwst and Betws-y-Coed (North Wales, UK). Our results confirmed the presence of human adenovirus (AdV), norovirus genotypes I and II (NoVGI and NoVGII) in both influent and effluent samples while sapovirus GI (SaVGI) was only detected in influent water. The AdV titre was high and relatively constant in all samples, whereas the NoVGI, NoVGII and SaVGI showed high concentrations during autumn and winter and low counts during the summer. Diurnal patterns were detected in pH and turbidity for some sampling periods; however, no such changes in viral titres were observed apart from slight fluctuations in the influent samples. Our findings suggest that viral particle number in wastewater is not affected by daily chemical fluctuations. Hence, a grab sample taken at any point during the day may be sufficient to enumerate the viral load of wastewater effluent within an order of magnitude while four samples a day are recommended for testing wastewater influent samples.
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Adenovirus Humanos/aislamiento & purificación , Enterovirus/aislamiento & purificación , Estaciones del Año , Aguas Residuales/virología , Microbiología del Agua/normas , Purificación del Agua/métodos , Adenovirus Humanos/genética , Enterovirus/genética , Genotipo , Humanos , Salud Pública , Gales , Aguas Residuales/químicaRESUMEN
Changes in the cytoskeletal organization within cells can be characterized by large spatial and temporal variations in rheological properties of the cell (e.g., the complex shear modulus G∗). Although the ensemble variation in G∗ of single cells has been elucidated, the detailed temporal variation of G∗ remains unknown. In this study, we investigated how the rheological properties of individual fibroblast cells change under a spatially confined environment in which the cell translational motion is highly restricted and the whole cell shape remains unchanged. The temporal evolution of single-cell rheology was probed at the same measurement location within the cell, using atomic force microscopy-based oscillatory deformation. The measurements reveal that the temporal variation in the power-law rheology of cells is quantitatively consistent with the ensemble variation, indicating that the cell system satisfies an ergodic hypothesis in which the temporal statistics are identical to the ensemble statistics. The autocorrelation of G∗ implies that the cell mechanical state evolves in the ensemble of possible states with a characteristic timescale.
Asunto(s)
Fibroblastos/citología , Reología , Análisis de la Célula Individual , Animales , Movimiento Celular , Cinética , Ratones , Modelos Biológicos , Células 3T3 NIHRESUMEN
OBJECTIVE: We describe long-term functional, neurodiagnostic, and psychosocial outcomes of a cohort of 12 children from Colorado diagnosed with acute flaccid myelitis (AFM) in 2014. METHODS: Children were assessed every 3 months for 1 year or until clinical resolution. Assessments included neurologic examination, MRI, EMG/nerve conduction studies (NCS), functional measures (Assisting Hand Assessment, Hammersmith Functional Motor Scale), and Patient-Reported Outcomes Measurement Information System questionnaires. RESULTS: Eight of 12 children completed the study. Six of 8 had persistent motor deficits at 1 year; 2 demonstrated full recovery. Four were not enrolled, 2 of whom reported full recovery. The 6 affected were weakest in proximal muscles, showing minimal to no improvement and significant atrophy at 1 year. All patients improved in distal muscle groups. Cranial nerve dysfunction resolved in 2 of 5 and improved in all. Four of 5 showed progressive functional improvement at 6 and 12 months. Two of 8 reported pain at 1 year. Three of 8 reported depressive symptoms. Repeat MRI was performed in 7 of 8 children a median of 7 months after onset and showed significant improvement or normalization in all but one child. Repeat EMG/NCS was performed on 4 children a median of 8 months after onset and showed ongoing denervation and chronic reinnervation in 3 children with persistent deficits. CONCLUSIONS: At 1 year, children with AFM demonstrated functional gains but weakness persisted. EMG changes correlated with persistent deficits better than imaging. Despite improvements, AFM had substantial long-term functional effects on affected children.