Publications by authors named "Sarah S Milla"

38 Publications

Chest radiograph features of multisystem inflammatory syndrome in children (MIS-C) compared to pediatric COVID-19.

Authors:
Bradley S Rostad Jay H Shah Christina A Rostad Preeti Jaggi Edward J Richer Leann E Linam Adina L Alazraki Erica L Riedesel Sarah S Milla

Pediatr Radiol 2021 02 6;51(2):231-238. Epub 2021 Jan 6.

Department of Radiology and Imaging Sciences, Emory University School of Medicine, 1405 Clifton Rd. NE, Atlanta, GA, 30322, USA.

Background: Although the radiographic features of coronavirus disease 2019 (COVID-19) in children have been described, the distinguishing features of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 are not well characterized.

Objective: We compared the chest radiographic findings of MIS-C with those of COVID-19 and described other distinguishing imaging features of MIS-C.

Materials And Methods: We performed a retrospective case series review of children ages 0 to 18 years who were hospitalized at Children's Healthcare of Atlanta from March to May 2020 and who either met the Centers for Disease Control and Prevention (CDC) case definition for MIS-C (n=11) or who had symptomatic, laboratory-confirmed COVID-19 (n=16). Two radiologists reviewed the most severe chest radiographs for each patient. The type and distribution of pulmonary opacities and presence or absence of pleural effusions were recorded. The chest radiographs were categorized based on potential COVID-19 imaging findings as typical, indeterminate, atypical or negative. An imaging severity score was also assigned using a simplified version of the Radiographic Assessment of Lung Edema Score. Findings were statistically compared between patients with MIS-C and those with COVID-19. Additional imaging findings of MIS-C were also described.

Results: Radiographic features of MIS-C included pleural effusions (82% [9/11]), pulmonary consolidations (73% [8/11]) and ground glass opacities (91% [10/11]). All of the lung opacities (100% [10/10]) were bilateral, and the majority of the pleural effusions (67% [6/9]) were bilateral. Compared to children with COVID-19, children with MIS-C were significantly more likely to develop pleural effusions on chest radiograph (82% [9/11] vs. 0% [0/0], P-value <0.01) and a lower zone predominance of pulmonary opacifications (100% [10/10] vs. 38% [5/13], P-value <0.01). Children with MIS-C who also had abdominal imaging had intra-abdominal inflammatory changes.

Conclusion: Key chest radiographic features of MIS-C versus those of COVID-19 were pleural effusions and lower zone pulmonary opacifications as well as intra-abdominal inflammation. Elucidating the distinguishing radiographic features of MIS-C may help refine the case definition and expedite diagnosis and treatment.
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http://dx.doi.org/10.1007/s00247-020-04921-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7785920PMC
February 2021

Artificial intelligence research within reach: an object detection model to identify rickets on pediatric wrist radiographs.

Authors:
Karthik C Meda Sarah S Milla Bradley S Rostad

Pediatr Radiol 2021 Jan 5. Epub 2021 Jan 5.

Department of Radiology and Imaging Sciences, Children's Healthcare of Atlanta at Egleston, Emory University School of Medicine, 1405 Clifton Rd. NE, Atlanta, GA, 30322-1101, USA.

Background: Artificial intelligence models have been successful in analyzing ordinary photographic images. One type of artificial intelligence model is object detection, where a labeled bounding box is drawn around an area of interest. Object detection can be applied to medical imaging tasks.

Objective: To demonstrate object detection in identifying rickets and normal wrists on pediatric wrist radiographs using a small dataset, simple software and modest computer hardware.

Materials And Methods: The institutional review board at Children's Healthcare of Atlanta approved this study. The radiology information system was searched for radiographic examinations of the wrist for the evaluation of rickets from 2007 to 2018 in children younger than 7 years of age. Inclusion criteria were an exam type of "Rickets Survey" or "Joint Survey 1 View" with reports containing the words "rickets" or "rachitic." Exclusion criteria were reports containing the words "renal," "kidney" or "transplant." Two pediatric radiologists reviewed the images and categorized them as either rickets or normal. Images were annotated by drawing a labeled bounding box around the distal radial and ulnar metaphases. The training dataset was created from images acquired from Jan. 1, 2007, to Dec. 31, 2017. This included 104 wrists with rickets and 264 normal wrists. This training dataset was used to create the object detection model. The testing dataset consisted of images acquired during the 2018 calendar year. This included 20 wrists with rickets and 37 normal wrists. Model sensitivity, specificity and accuracy were measured.

Results: Of the 20 wrists with rickets in the testing set, 16 were correctly identified as rickets, 2 were incorrectly identified as normal and 2 had no prediction. Of the 37 normal wrists, 33 were correctly identified as normal, 2 were incorrectly identified as rickets and 2 had no prediction. This yielded a sensitivity and specificity of 80% and 95% for wrists with rickets and 89% and 90% for normal wrists. Overall model accuracy was 86%.

Conclusion: Object detection can identify rickets on pediatric wrist radiographs. Object detection models can be developed with a small dataset, simple software tools and modest computing power.
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http://dx.doi.org/10.1007/s00247-020-04895-8DOI Listing
January 2021

Routine Neuroimaging of the Preterm Brain.

Authors:
Ivan L Hand Renée A Shellhaas Sarah S Milla

Pediatrics 2020 11;146(5)

Departments of Radiology and Pediatrics, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, Georgia.

Neuroimaging of the preterm infant is a common assessment performed in the NICU. Timely and focused studies can be used for diagnostic, therapeutic, and prognostic information. However, significant variability exists among neonatal units as to which modalities are used and when imaging studies are obtained. Appropriate timing and selection of neuroimaging studies can help identify neonates with brain injury who may require therapeutic intervention or who may be at risk for neurodevelopmental impairment. This clinical report reviews the different modalities of imaging broadly available to the clinician. Evidence-based indications for each modality, optimal timing of examinations, and prognostic value are discussed.
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http://dx.doi.org/10.1542/peds.2020-029082DOI Listing
November 2020

Radiographic findings predictive of irreducibility and surgical resection in ileocolic intussusception.

Authors:
Dhruv M Patel Jonathan M Loewen Kiery A Braithwaite Sarah S Milla Edward J Richer

Pediatr Radiol 2020 08 9;50(9):1249-1254. Epub 2020 Jun 9.

Department of Radiology, Emory University/Children's Healthcare of Atlanta, 1405 Clifton Rd., Atlanta, GA, 30329, USA.

Background: Ileocolic intussusception is a common cause of intestinal obstruction in young children. Radiographs may be of limited value in the diagnosis of intussusception and are sometimes utilized primarily to exclude pneumoperitoneum before therapeutic enema reduction.

Objective: The goal of this study was to determine if radiographic findings in ileocolic intussusception can offer prognostic information regarding the outcome of therapeutic air enema and, for those requiring surgical intervention, surgical outcomes and/or complications.

Materials And Methods: A single institution retrospective study was performed including all enemas for intussusception performed during a 5-year period from September 2012 to August 2017. Radiographs obtained before therapeutic enema, including our institution radiographs, outside facility radiographs, or scout images obtained during fluoroscopy or computed tomography (CT), were independently scored by two pediatric radiologists for normal bowel gas pattern, soft-tissue mass, paucity of bowel gas, meniscus sign and bowel obstruction. The reviewers were blinded to enema and surgical outcomes at the time of review. Differences were resolved by consensus. Cases were excluded in which there was no adequate pre-procedure radiograph. In total, 182 cases were reviewed. The medical records were reviewed for enema and surgical outcomes.

Results: Radiographic findings included normal bowel gas pattern in 13%, soft-tissue mass in 26%, paucity of bowel gas in 65%, meniscus sign in 12% and obstruction in 10% of the cases, with 17.5% of patients having more than one finding. In patients with bowel obstruction on radiographs, there was a statistically significant decrease in success of therapeutic enema (83% vs. 21%, P=0.0001), increase in complicated surgical reductions (47% vs. 4%, P=0.0012), and increase in bowel resection (42% vs. 4%, P=0.003) compared to patients with normal bowel gas pattern.

Conclusion: Radiographs can offer prognostic information regarding the potential for therapeutic enema success, as well as potential surgical outcomes in patients failing enema reduction. Particularly, bowel obstruction significantly decreases the success of therapeutic enema and increases the need for bowel resection.
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http://dx.doi.org/10.1007/s00247-020-04695-0DOI Listing
August 2020

Incidence and importance of portal venous gas in children with hypertrophic pyloric stenosis.

Authors:
Mary M Kelly Cera Cantu Edward J Richer Kiery A Braithwaite Leann E Linam Erica L Riedesel Jonathan Loewen Stephen F Simoneaux Sarah S Milla

Pediatr Radiol 2020 07 27;50(8):1102-1106. Epub 2020 May 27.

Department of Radiology and Imaging Sciences, Emory School of Medicine, Atlanta, GA, USA.

Background: Hypertrophic pyloric stenosis (HPS) is a common cause of gastric outlet obstruction in young infants. Infants with HPS present with projectile vomiting, sometimes have electrolyte abnormalities and typically undergo pyloromyotomy to alleviate the obstruction. Abdominal US is the gold standard imaging study for diagnosis. Case reports of incidental hepatic portal venous gas have been reported in infants with HPS; however, no large studies have been conducted to determine the incidence or possible clinical implications of this finding.

Objective: To assess the incidence of portal venous gas in infants with HPS and to determine whether the presence of this gas in infants with HPS indicates a more unstable patient, increased length of stay or worse outcome.

Materials And Methods: We conducted a retrospective review of sonographic reports containing "pyloric stenosis," excluding negative descriptor, at a tertiary-care children's hospital from November 2010 to September 2017. Data collected included pyloric thickness/length, liver evaluation, portal venous gas, any additional imaging, demographics, symptomatology days, electrolyte abnormality, and length of hospital stay.

Results: In a 7-year period, 545 US exams were positive for HPS. Of these, 334 exams included enough hepatic parenchyma to evaluate for portal venous gas. Infants in 6 of the 334 exams demonstrated portal venous gas (1.8%). Clinical presentation (length of symptoms and electrolyte abnormalities), demographics (male predominance and age at presentation) and imaging characteristics (pyloric thickness and length) were similar for the HPS groups with and without portal venous gas. There was no significant difference in outcome or length of hospital stay.

Conclusion: Visualization of portal venous gas in infants with HPS is not rare and appears benign, without need for further imaging. Portal venous gas in infants with HPS does not portend a more severe patient presentation or outcome.
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http://dx.doi.org/10.1007/s00247-020-04694-1DOI Listing
July 2020

Economically Motivated Patient Steerage: The Pediatric Perspective.

Authors:
Richard E Heller Sarah S Milla Richard A Barth

J Am Coll Radiol 2020 Aug 12;17(8):1025-1026. Epub 2020 May 12.

Radiologist-in-Chief, Lucile Packard Children's Hospital, Associate Chair of Radiology, Stanford University School of Medicine, Stanford, California.

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http://dx.doi.org/10.1016/j.jacr.2020.04.014DOI Listing
August 2020

ACR Appropriateness Criteria® Cerebrovascular Disease-Child.

Authors:
Richard L Robertson Susan Palasis Michael J Rivkin Sumit Pruthi Twyla B Bartel Nilesh K Desai Nadja Kadom Abhaya V Kulkarni H F Samuel Lam Mohit Maheshwari Sarah S Milla David M Mirsky John S Myseros Sonia Partap Rupa Radhakrishnan Bruno P Soares Andrew T Trout Unni K Udayasankar Matthew T Whitehead Boaz Karmazyn

J Am Coll Radiol 2020 May;17(5S):S36-S54

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Stroke is an uncommon but an important and under-recognized cause of morbidity and mortality in children. Strokes may be due to either brain ischemia or intracranial hemorrhage. Common symptoms of pediatric acute stroke include headache, vomiting, focal weakness, numbness, visual disturbance, seizures, and altered consciousness. Most children presenting with an acute neurologic deficit do not have an acute stroke, but have symptoms due to stroke mimics which include complicated migraine, seizures with postictal paralysis, and Bell palsy. Because of frequency of stroke mimics, in children and the common lack of specificity in symptoms, the diagnosis of a true stroke may be delayed. There are a relatively large number of potential causes of stroke mimic and true stroke. Consequently, imaging plays a critical role in the assessment of children with possible stroke and especially in children who present with acute onset of stroke symptoms. 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.
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http://dx.doi.org/10.1016/j.jacr.2020.01.036DOI Listing
May 2020

ACR Appropriateness Criteria® Head Trauma-Child.

Authors:
Maura E Ryan Sumit Pruthi Nilesh K Desai Richard A Falcone Orit A Glenn Madeline M Joseph Mohit Maheshwari Jennifer R Marin Catherine Mazzola Sarah S Milla David M Mirsky John S Myseros Sumit N Niogi Sonia Partap Rupa Radhakrishnan Richard L Robertson Bruno P Soares Unni K Udayasankar Matthew T Whitehead Jason N Wright Boaz Karmazyn

J Am Coll Radiol 2020 May;17(5S):S125-S137

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Head trauma is a frequent indication for cranial imaging in children. The majority of accidental pediatric head trauma is minor and sustained without intracranial injury. Well-validated pediatric-specific clinical decision guidelines should be used to identify very low-risk children who can safely forgo imaging. In those who require acute imaging, CT is considered the first-line imaging modality for suspected intracranial injury because of the short duration of the examination and its high sensitivity for acute hemorrhage. MRI can accurately detect traumatic complications, but often necessitates sedation in children, owing to the examination length and motion sensitivity, which limits rapid assessment. There is a paucity of literature regarding vascular injuries in pediatric blunt head trauma and imaging is typically guided by clinical suspicion. Advanced imaging techniques have the potential to identify changes that are not seen by standard imaging, but data are currently insufficient to support routine clinical use. 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.
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http://dx.doi.org/10.1016/j.jacr.2020.01.026DOI Listing
May 2020

The continuous lure of pediatric radiology.

Authors:
Peter J Strouse Erin K Romberg Delma Y Jarrett Jonathan R Dillman Daniel A Dessner Janet R Reid Sarah S Milla Richard E Heller Michael A DiPietro

Pediatr Radiol 2020 01 4;50(1):3-12. Epub 2020 Jan 4.

Section of Pediatric Radiology, C. S. Mott Children's Hospital, Room 3-231, Department of Radiology, University of Michigan Health System, 1540 E. Hospital Drive, Ann Arbor, MI, 48109, USA.

Pediatric radiology is an immensely rewarding career choice. Eight pediatric radiologists, enthusiastic for their profession, were asked six questions about their career choice. Their responses illustrate the common virtues of pediatric radiology and also demonstrate the diverse paths and activities that pediatric radiologists take and pursue.
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http://dx.doi.org/10.1007/s00247-019-04569-0DOI Listing
January 2020

Prospective Cohort Study of Next-Generation Sequencing as a Diagnostic Modality for Unexplained Encephalitis in Children.

Authors:
Julia C Haston Christina A Rostad Robert C Jerris Sarah S Milla Courtney McCracken Catherine Pratt Michael Wiley Karla Prieto Gustavo Palacios Andi L Shane Anita K McElroy

J Pediatric Infect Dis Soc 2020 Jul;9(3):326-333

Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia.

Background: Encephalitis is an inflammatory condition of the brain associated with long-term neurologic sequelae and even death in children. Although viruses are often implicated, an etiology is not identified in the majority of cases. Metagenomics-based next-generation sequencing (mNGS) is a high-throughput sequencing technique that can enhance the detection of novel or low-frequency pathogens.

Methods: Hospitalized immunocompetent children aged 6 months to 18 years with encephalitis of unidentified etiology were eligible for enrollment. Demographic, historical, and clinical information was obtained, and residual blood and cerebrospinal fluid (CSF) samples were subjected to mNGS. Pathogens were identified by querying the sequence data against the NCBI GenBank database.

Results: Twenty children were enrolled prospectively between 2013 and 2017. mNGS of CSF identified 7 nonhuman nucleic acid sequences of significant frequency in 6 patients, including that of Mycoplasma bovis, parvovirus B19, Neisseria meningitidis, and Balamuthia mandrillaris. mNGS also detected Cladophialophora species, tobacco mosaic virus, and human bocavirus, which were presumed to be contaminants or nonpathogenic organisms. One patient was found to have positive serology results for California encephalitis virus, but mNGS did not detect it. Patients for whom mNGS identified a diagnosis had a significantly higher CSF white blood cell count, a higher CSF protein concentration, and a lower CSF glucose level than patients for whom mNGS did not identify a diagnosis.

Conclusion: We describe here the results of a prospective cohort analysis to evaluate mNGS as a diagnostic tool for children with unexplained encephalitis. Although mNGS detected multiple nonpathogenic organisms, it also identified multiple pathogens successfully and was most useful in patients with a CSF abnormality.
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http://dx.doi.org/10.1093/jpids/piz032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7457329PMC
July 2020

ACR Appropriateness Criteria Suspected Spine Trauma-Child.

Authors:
Nadja Kadom Susan Palasis Sumit Pruthi Walter L Biffl Timothy N Booth Nilesh K Desai Richard A Falcone Jeremy Y Jones Madeline M Joseph Abhaya V Kulkarni Jennifer R Marin Sarah S Milla David M Mirsky John S Myseros Charles Reitman Richard L Robertson Maura E Ryan Gaurav Saigal Jacob Schulz Bruno P Soares Aylin Tekes Andrew T Trout Matthew T Whitehead Boaz Karmazyn

J Am Coll Radiol 2019 May;16(5S):S286-S299

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Choosing the appropriate imaging in children with accidental traumatic spine injuries can be challenging because the recommendations based on scientific evidence at this time differ from those applied in adults. This differentiation is due in part to differences in anatomy and physiology of the developing spine. This publication uses scientific evidence and a panel of pediatric experts to summarize best current imaging practices for children with accidental spine trauma. 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.
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http://dx.doi.org/10.1016/j.jacr.2019.02.003DOI Listing
May 2019

ACR Appropriateness Criteria Scoliosis-Child.

Authors:
Jeremy Y Jones Gaurav Saigal Susan Palasis Timothy N Booth Laura L Hayes Ramesh S Iyer Nadja Kadom Abhaya V Kulkarni Sarah S Milla John S Myseros Charles Reitman Richard L Robertson Maura E Ryan Jacob Schulz Bruno P Soares Aylin Tekes Andrew T Trout Boaz Karmazyn

J Am Coll Radiol 2019 May;16(5S):S244-S251

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

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.
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http://dx.doi.org/10.1016/j.jacr.2019.02.018DOI Listing
May 2019

Congenital Limb Overgrowth Syndromes Associated with Vascular Anomalies.

Authors:
Frederic Bertino Kiery A Braithwaite C Matthew Hawkins Anne E Gill Michael A Briones Rachel Swerdlin Sarah S Milla

Radiographics 2019 Mar-Apr;39(2):491-515

From the Divisions of Pediatric Radiology (F.B., K.A.B., C.M.H., A.E.G., S.S.M.) and Interventional Radiology and Image Guided Medicine (F.B., C.M.H., A.E.G.), Department of Radiology and Imaging Sciences; and Department of Hematology and Medical Oncology (M.A.B.), Emory University School of Medicine, 1364 Clifton Rd NE, Suite D112, Atlanta, GA 30322; and Vascular Anomalies Clinic (F.B., K.A.B., C.M.H., A.E.G., M.A.B., R.S., S.S.M.) and Aflac Cancer and Blood Disorders Center (M.A.B.), Children's Healthcare of Atlanta, Atlanta, Ga.

Congenital limb length discrepancy disorders are frequently associated with a variety of vascular anomalies and have unique genetic and phenotypic features. Many of these syndromes have been linked to sporadic somatic mosaicism involving mutations of the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, which has an important role in tissue growth and angiogenesis. Radiologists who are aware of congenital limb length discrepancies can make specific diagnoses based on imaging findings. Although genetic confirmation is necessary for a definitive diagnosis, the radiologist serves as a central figure in the identification and treatment of these disorders. The clinical presentations, diagnostic and imaging workups, and treatment options available for patients with Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular anomalies, epidermal nevi, and scoliosis/spinal deformities) syndrome, fibroadipose vascular anomaly, phosphatase and tensin homolog mutation spectrum, Parkes-Weber syndrome, and Proteus syndrome are reviewed. RSNA, 2019.
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http://dx.doi.org/10.1148/rg.2019180136DOI Listing
March 2020

Quantitative magnetic resonance evaluation of the trigeminal nerve in familial dysautonomia.

Authors:
Eugene Won Jose-Alberto Palma Horacio Kaufmann Sarah S Milla Benjamin Cohen Lucy Norcliffe-Kaufmann James S Babb Yvonne W Lui

Clin Auton Res 2019 08 19;29(4):469-473. Epub 2019 Feb 19.

Department of Radiology, New York University School of Medicine, New York, NY, 10016, USA.

Purpose: Familial dysautonomia (FD) is a rare autosomal recessive disease that affects the development of sensory and autonomic neurons, including those in the cranial nerves. We aimed to determine whether conventional brain magnetic resonance imaging (MRI) could detect morphologic changes in the trigeminal nerves of these patients.

Methods: Cross-sectional analysis of brain MRI of patients with genetically confirmed FD and age- and sex-matched controls. High-resolution 3D gradient-echo T1-weighted sequences were used to obtain measurements of the cisternal segment of the trigeminal nerves. Measurements were obtained using a two-reader consensus.

Results: Twenty pairs of trigeminal nerves were assessed in ten patients with FD and ten matched controls. The median (interquartile range) cross-sectional area of the trigeminal nerves in patients with FD was 3.5 (2.1) mm, compared to 5.9 (2.0) mm in controls (P < 0.001). No association between trigeminal nerve area and age was found in patients or controls.

Conclusions: Using conventional MRI, the caliber of the trigeminal nerves was significantly reduced bilaterally in patients with FD compared to controls, a finding that appears to be highly characteristic of this disorder. The lack of correlation between age and trigeminal nerve size supports arrested neuronal development rather than progressive atrophy.
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http://dx.doi.org/10.1007/s10286-019-00593-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6697601PMC
August 2019

Epileptic Encephalopathy and Cerebellar Atrophy Resulting from Compound Heterozygous Variants.

Authors:
Kameryn M Butler Philip J Holt Sarah S Milla Cristina da Silva John J Alexander Andrew Escayg

Case Rep Genet 2018 15;2018:6308283. Epub 2018 Oct 15.

Emory University Department of Human Genetics, Atlanta, GA 30322, USA.

encodes an auxiliary subunit of the voltage-dependent calcium channel. To date, there have only been two reports of individuals with early-infantile epileptic encephalopathy due to mutations. In both reports, patients were homozygous for the identified variants. Here, we report a patient with epileptic encephalopathy and cerebellar atrophy who was found to have two novel variants in the gene: c.782C>T (p.Pro261Leu) and c.3137T>C (p.Leu1046Pro), by whole-exome sequencing. The variants were shown to be inherited in and the unaffected parents were confirmed to be heterozygous carriers. This is the third report of recessive variants associated with disease and the first report of compound heterozygous variants. The clinical description of this new case highlights the phenotypic similarities amongst individuals with -related disease and suggests that should be considered as a differential diagnosis in individuals with cerebellar dysfunction and multiple seizure types that begin in the first year of life.
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http://dx.doi.org/10.1155/2018/6308283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6205307PMC
October 2018

ACR Appropriateness Criteria Sinusitis-Child.

Authors:
Aylin Tekes Susan Palasis Daniel J Durand Sumit Pruthi Timothy N Booth Nilesh K Desai Jeremy Y Jones Nadja Kadom H F Samuel Lam Sarah S Milla David M Mirsky Sonia Partap Richard L Robertson Maura E Ryan Gaurav Saigal Gavin Setzen Bruno P Soares Andrew T Trout Matthew T Whitehead Boaz Karmazyn

J Am Coll Radiol 2018 Nov;15(11S):S403-S412

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Sinusitis is common in children that usually resolves spontaneously. Imaging is not part of the standard of care for initial diagnosis, however may be necessary in cases with persistent or chronic sinusitis to guide surgical intervention, or to rule out intracranial and vascular complications of sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) are the leading imaging modalities. In this article, appropriateness in use of imaging modalities are discussed under common/clinically relevant scenarios. 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.
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http://dx.doi.org/10.1016/j.jacr.2018.09.029DOI Listing
November 2018

Multiphase acquisitions in pediatric abdominal-pelvic CT are a common practice and contribute to unnecessary radiation dose.

Authors:
Bradley S Rostad Kimberly E Applegate Tammy Kim Renee M Mansour Sarah S Milla

Pediatr Radiol 2018 11 7;48(12):1714-1723. Epub 2018 Jul 7.

Department of Radiology, Children's Healthcare of Atlanta at Egleston, 1405 Clifton Rd., NE, Atlanta, GA, 30322-1101, USA.

Background: Many patients at our pediatric hospital have had a contrast-enhanced CT of the abdomen and pelvis performed by an outside imaging facility before admission. We have noticed that many of these exams are multiphase, which may contribute to unnecessary radiation dose.

Objective: To determine the frequency of multiphase acquisitions and radiation dose indices in contrast-enhanced CTs of the abdomen and pelvis performed by outside imaging facilities in patients who were subsequently transferred to our pediatric hospital for care, and compare these metrics to contrast-enhanced CTs of the abdomen and pelvis performed internally.

Materials And Methods: A retrospective analysis was performed of contrast-enhanced CTs of the abdomen and pelvis from outside imaging facilities uploaded to our picture archiving and communication system (PACS) between January 1, 2012, and December 31, 2015. CT images and dose pages were reviewed to determine the number of phases and dose indices (CT dose index-volume [CTDI], dose-length product, size-specific dose estimate). Exams for abdominal or pelvic mass, trauma or urinary leak indications were excluded. Data were compared to internally acquired contrast-enhanced CTs of the abdomen and pelvis by querying the American College of Radiology (ACR) Dose Index Registry. This review was institutional review board and HIPAA compliant.

Results: There were 754 contrast-enhanced CTs of the abdomen and pelvis from 104 outside imaging facilities. Fifty-three percent (399/754) had 2 phases, and 2% (14/754) had 3 or more phases. Of the 939 contrast-enhanced CTs of the abdomen and pelvis performed internally, 12% (115) were multiphase exams. Of 88% (664) contrast-enhanced CTs of the abdomen and pelvis from outside imaging facilities with dose data, CTDI was 2.7 times higher than our institution contrast-enhanced CTs of the abdomen and pelvis (939) for all age categories as defined by the ACR Dose Index Registry (mean: 9.4 vs. 3.5 mGy, P<0.0001). The majority (74%) of multiphase exams were performed by 9 of 104 outside imaging facilities.

Conclusion: Multiphase acquisitions in routine contrast-enhanced CT of the abdomen and pelvis exams at outside imaging facilities are more frequent than those at a dedicated pediatric institution and contribute to unnecessary radiation dose. A contrast-enhanced CT of the abdomen and pelvis exam from an outside imaging facility with two passes may have as much as four times to six times the dose as the same exam performed with a single pass at a pediatric imaging center. We advocate for imaging facilities with high multiphase rates to eliminate multiple phases from routine contrast-enhanced CT of the abdomen and pelvis exams in children.
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http://dx.doi.org/10.1007/s00247-018-4192-yDOI Listing
November 2018

ACR Appropriateness Criteria Headache-Child.

Authors:
Laura L Hayes Susan Palasis Twyla B Bartel Timothy N Booth Ramesh S Iyer Jeremy Y Jones Nadja Kadom Sarah S Milla John S Myseros Ann Pakalnis Sonia Partap Richard L Robertson Maura E Ryan Gaurav Saigal Bruno P Soares Aylin Tekes Boaz K Karmazyn

J Am Coll Radiol 2018 May;15(5S):S78-S90

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

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.
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http://dx.doi.org/10.1016/j.jacr.2018.03.017DOI Listing
May 2018

Spontaneous epidural hematoma secondary to bone infarction in sickle cell anemia: case report.

Authors:
Rohini Komarla Bruno P Soares Joshua J Chern Sarah S Milla

J Neurosurg Pediatr 2018 07 20;22(1):18-21. Epub 2018 Apr 20.

Departments of1Radiology and.

Spontaneous epidural hematoma (EDH) is a rare occurrence in patients with sickle cell disease, with a small number of cases reported. Appropriate diagnosis is critical, because rapid neurosurgical intervention may be required. This unique case illustrates clinical and MRI features of an 18-year-old woman presenting with a headache and subsequent progression to severe focal neurological symptoms. Imaging demonstrated a large EDH of mixed signal characteristics and underlying calvarial infarction, requiring emergency decompression and evacuation. A second companion case is also presented. The authors discuss proposed pathophysiology of the formation of EDHs in sickle cell anemia.
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http://dx.doi.org/10.3171/2018.1.PEDS17407DOI Listing
July 2018

Pediatric Dental Clinic-Associated Outbreak of Mycobacterium abscessus Infection.

Authors:
Lindsay A Hatzenbuehler Melissa Tobin-D'Angelo Cherie Drenzek Gianna Peralta Lisa C Cranmer Evan J Anderson Sarah S Milla Shelly Abramowicz Jumi Yi Joseph Hilinski Roy Rajan Matthew K Whitley Verlia Gower Frank Berkowitz Craig A Shapiro Joseph K Williams Paula Harmon Andi L Shane

J Pediatric Infect Dis Soc 2017 Sep;6(3):e116-e122

Division of Pediatric Infectious Diseases.

Background: Mycobacterium abscessus is an uncommon cause of invasive odontogenic infection.

Methods: M abscessus-associated odontogenic infections occurred in a group of children after they each underwent a pulpotomy. A probable case-child was defined as a child with facial or neck swelling and biopsy-confirmed granulomatous inflammation after a pulpotomy between October 1, 2013, and September 30, 2015. M abscessus was isolated by culture in confirmed case-children. Clinical presentation, management, and outcomes were determined by medical record abstraction.

Results: Among 24 children, 14 (58%) were confirmed case-children. Their median age was 7.3 years (interquartile range, 5.8-8.2 years), and the median time from pulpotomy to symptom onset was 74 days (range, 14-262 days). Clinical diagnoses included cervical lymphadenitis (24 [100%] of 24), mandibular or maxillary osteomyelitis (11 [48%] of 23), and pulmonary nodules (7 [37%] of 19). Each child had ≥1 hospitalization and a median of 2 surgeries (range, 1-6). Of the 24 children, 12 (50%) had surgery alone and 11 (46%) received intravenous (IV) antibiotics. Nineteen of the 24 (79%) children experienced complications, including vascular access malfunction (7 [64%] of 11), high-frequency hearing loss (5 [56%] of 9), permanent tooth loss (11 [48%] of 23), facial nerve palsy (7 [29%] of 24), urticarial rash (3 [25%] of 12), elevated liver enzyme levels (1 [20%] of 5), acute kidney injury (2 [18%] of 11), incision dehiscence/fibrosis (3 [13%] of 24), and neutropenia (1 [9%] of 11).

Conclusions: M abscessus infection was associated with significant medical morbidity and treatment complications. Unique manifestations included extranodal mandibular or maxillary osteomyelitis and pulmonary nodules. Challenges in the identification of case-children resulted from an extended incubation period and various clinical manifestations. Clinicians should consider the association between M abscessus infection and pulpotomy in children who present with subacute cervical lymphadenitis. The use of treated/sterile water during pulpotomy might prevent further outbreaks.
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http://dx.doi.org/10.1093/jpids/pix065DOI Listing
September 2017

Unilateral lung agenesis, aplasia or hypoplasia: Which one is it?

Authors:
Khristopher M Nguyen Snehal Vala Sarah S Milla Lokesh Guglani

Congenit Anom (Kyoto) 2018 Mar 29;58(2):75-76. Epub 2017 Aug 29.

Pulmonology, Allergy/Immunology, Cystic Fibrosis and Sleep Medicine (PACS) Division, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, USA.

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http://dx.doi.org/10.1111/cga.12239DOI Listing
March 2018

Rare presentation of four primary pediatric cardiac tumors.

Authors:
Lauren R Schaffer Shelley A Caltharp Sarah S Milla Brian F Kogon Caitlin A Cundiff Aarti Dalal Phillip C Quigley Bahig M Shehata

Cardiovasc Pathol 2016 Jan-Feb;25(1):72-7. Epub 2015 Aug 31.

Department of Pathology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, US. Electronic address:

Pediatric cardiac tumors are extremely rare and usually benign. We selected four unique cases of pediatric cardiac tumors from a 15-year period at our institution. The four chosen cases represent unique, rare primary tumors of the heart. Our selection includes a case of Rosai Dorfman disease without systemic involvement, which is, to our knowledge, the second case of isolated cardiac Rosai Dorfman disease in a child. We present a case of subtotal replacement of myocardium by granulocytic sarcoma with minimal bone marrow involvement, representing the first reported case in a child manifested as hypertrophic cardiomyopathy, as well as a case of a primary synovial sarcoma arising from the atrioventricular (AV) node, representing the fourth reported pediatric case of a cardiac synovial sarcoma, and it is the first to arise from the AV node. Finally, we present a primary congenital infantile fibrosarcoma of the heart, which is, to our knowledge, the first confirmed cardiac congenital infantile fibrosarcoma. These four cases represent the need for continued inclusion of rare cardiac conditions in a clinician's differential diagnosis. Furthermore, they present the need for more in-depth molecular and genomic analysis of pediatric cardiac tumors in order to identify their etiopathogenesis.
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http://dx.doi.org/10.1016/j.carpath.2015.08.011DOI Listing
October 2016

One diffusion acquisition and different white matter models: how does microstructure change in human early development based on WMTI and NODDI?

Authors:
Ileana O Jelescu Jelle Veraart Vitria Adisetiyo Sarah S Milla Dmitry S Novikov Els Fieremans

Neuroimage 2015 Feb 9;107:242-256. Epub 2014 Dec 9.

Center for Biomedical Imaging, Department of Radiology, New York University Langone Medical Center, New York, NY, USA.

White matter microstructural changes during the first three years of healthy brain development are characterized using two different models developed for limited clinical diffusion data: White Matter Tract Integrity (WMTI) metrics from Diffusional Kurtosis Imaging (DKI) and Neurite Orientation Dispersion and Density Imaging (NODDI). Both models reveal a non-linear increase in intra-axonal water fraction and in tortuosity of the extra-axonal space as a function of age, in the genu and splenium of the corpus callosum and the posterior limb of the internal capsule. The changes are consistent with expected behavior related to myelination and asynchrony of fiber development. The intra- and extracellular axial diffusivities as estimated with WMTI do not change appreciably in normal brain development. The quantitative differences in parameter estimates between models are examined and explained in the light of each model's assumptions and consequent biases, as highlighted in simulations. Finally, we discuss the feasibility of a model with fewer assumptions.
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http://dx.doi.org/10.1016/j.neuroimage.2014.12.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4300243PMC
February 2015

The challenging ultrasound diagnosis of perforated appendicitis in children: constellations of sonographic findings improve specificity.

Authors:
Sheryl Tulin-Silver James Babb Lynne Pinkney Naomi Strubel Shailee Lala Sarah S Milla Sandra Tomita Nancy R Fefferman

Pediatr Radiol 2015 Jun 4;45(6):820-30. Epub 2014 Dec 4.

Department of Radiology, New York University School of Medicine, 660 1st Ave., New York, NY, 10016, USA.

Background: Rapid and accurate diagnosis of appendicitis, particularly with respect to the presence or absence of perforation, is essential in guiding appropriate management. Although many studies have explored sonographic findings associated with acute appendicitis, few investigations discuss specific signs that can reliably differentiate perforated appendicitis from acute appendicitis prior to abscess formation.

Objective: The purpose of our study was to identify sonographic findings that improve the specificity of US in the diagnosis of perforated appendicitis. Our assessment of hepatic periportal echogenicity, detailed analysis of intraperitoneal fluid, and formulation of select constellations of sonographic findings expands upon the literature addressing this important diagnostic challenge.

Materials And Methods: We retrospectively reviewed 116 abdominal US examinations for evaluation of abdominal pain in children ages 2 to 18 years from January 2008 to September 2011 at a university hospital pediatric radiology department. The study group consisted of surgical and pathology proven acute appendicitis (n = 51) and perforated appendicitis (n = 22) US exams. US exams without a sonographic diagnosis of appendicitis (n = 43) confirmed by follow-up verbal communication were included in the study population as the control group. After de-identification, the US exams were independently reviewed on a PACS workstation by four pediatric radiologists blinded to diagnosis and all clinical information. We recorded the presence of normal or abnormal appendix, appendicolith, appendiceal wall vascularity, thick-walled bowel, dilated bowel, right lower quadrant (RLQ) echogenic fat, increased hepatic periportal echogenicity, bladder debris and abscess or loculated fluid. We also recorded the characteristics of intraperitoneal fluid, indicating the relative quantity (number of abdominal regions) and quality of the fluid (simple fluid or complex fluid). We used logistic regression for correlated data to evaluate the association of diagnosis with the presence versus absence of each US finding. We conducted multivariable analysis to identify constellations of sonographic findings that were predictive of perforated appendicitis.

Results: The individual US findings of abscess/loculated fluid, appendicolith, dilated bowel and increased hepatic periportal echogenicity were significantly associated with perforated appendicitis when compared with acute appendicitis (P < 0.01). The sonographic observation of increased hepatic periportal echogenicity demonstrated a statistically significant association with perforated appendicitis compared with acute appendicitis (P < 0.01). The presence of complex fluid yielded a specificity of 87.7% for perforated appendicitis compared with the acute appendicitis group. The US findings of ≥2 regions or ≥3 regions with fluid had specificity of 87.3% and 99.0%, respectively, for perforated appendicitis compared with the acute appendicitis group. Select combinations of sonographic findings yielded high specificity in the diagnosis of perforated appendicitis compared with acute appendicitis. These constellations yielded higher specificity than that of each individual finding in isolation. The constellation of dilated bowel, RLQ echogenic fat, and complex fluid had the highest specificity (99.5%) for perforated appendicitis (P < 0.01).

Conclusion: Our study demonstrates that identification of select constellations of findings using abdominal sonography, in addition to focused US examination of the right lower quadrant, can improve sonographic diagnosis of perforated appendicitis in the pediatric population.
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http://dx.doi.org/10.1007/s00247-014-3232-5DOI Listing
June 2015

Bilateral first rib anomalous articulations with pseudarthroses mimicking healing fractures in an infant with abusive head injury.

Authors:
Melissa A Pasquale-Styles Christian M Crowder Jeannette Fridie Sarah S Milla

J Forensic Sci 2014 Nov;59(6):1668-71

Fulton County Medical Examiner Center, Atlanta, GA.

Bilateral symmetric bone nodules were observed in the anterolateral first ribs of an infant with shaking injuries at autopsy. The location prompted diagnostic considerations of healing fractures versus anomalous articulations with pseudarthroses. The forensic pathologist worked with forensic anthropologists and pediatric radiologists to evaluate autopsy findings and compare premortem and postmortem X-rays. Gross examination of the bones by the pathologist and anthropologists confirmed bilateral, callus-like bone nodules in first-rib locations associated with pseudarthroses. Histologic examination of one of the bones further showed features most consistent with pseudarthrosis, not a healing fracture. Radiologists then compared multiple premortem and postmortem radiographs that showed no remodeling of the bone over a 2-week interval between the time of injury and death, which would be unexpected for a healing fracture in an infant. This multidisciplinary approach resulted in the appropriate diagnosis of pseudarthroses due to anomalous articulations, an uncommon finding in forensic pathology.
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http://dx.doi.org/10.1111/1556-4029.12609DOI Listing
November 2014

Benefits of brain magnetic resonance imaging over computed tomography in children requiring emergency evaluation of ventriculoperitoneal shunt malfunction: reducing lifetime attributable risk of cancer.

Authors:
In Kim Susan B Torrey Sarah S Milla Marisa C Torch Michael G Tunik Jessica C Foltin

Pediatr Emerg Care 2015 Apr;31(4):239-42

From the Department of Pediatric Emergency Medicine, New York University Langone Medical Center, New York, NY.

Objectives: The rapid growth of computed tomography (CT) has resulted in increased concerns of ionizing radiation exposure and its subsequent risk of cancer development. We evaluated the impact of a new protocol using rapid sequence magnetic resonance imaging (rsMRI) instead of CT in children presenting with possible ventriculoperitoneal shunt (VPS) malfunction to promote patient safety.

Methods: This is a retrospective case series of pediatric patients who received a neuroimaging study for emergency evaluation of possible VPS malfunction at New York University's Tisch Hospital Emergency Department between January 2010 and July 2011. Radiology Charge Master was queried to identify the patient database. The trend in the use of rsMRI and CT was calculated for 3 patient age groups and compared across 3 chronological intervals. The effective dose of ionizing radiation per CT scan was calculated using the CT dose index and dose-length product for each patient.

Results: Total of 365 patients with the mean age of 8.87 years received either rsMRI or CT study during the study period. One hundred forty-four of these patients required the imaging studies because of VPS malfunction. Overall, 62% of all VPS malfunction cases used rsMRI instead of CT. The ratio of the number of patients receiving rsMRI divided by CT studies has progressively increased from 1.4:1 to 2.1:1 over 3 chronologic periods.

Conclusions: Children with VPS are subject to multiple neuroimaging studies throughout their lifetime. Rapid sequence MRI is an effective alternative to CT while providing no ionizing radiation exposure or risk of developing radiation-induced cancer.
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http://dx.doi.org/10.1097/PEC.0000000000000248DOI Listing
April 2015

Phase II study of sorafenib in children with recurrent or progressive low-grade astrocytomas.

Authors:
Matthias A Karajannis Geneviève Legault Michael J Fisher Sarah S Milla Kenneth J Cohen Jeffrey H Wisoff David H Harter Judith D Goldberg Tsivia Hochman Amanda Merkelson Michael C Bloom Angela J Sievert Adam C Resnick Girish Dhall David T W Jones Andrey Korshunov Stefan M Pfister Charles G Eberhart David Zagzag Jeffrey C Allen

Neuro Oncol 2014 Oct 6;16(10):1408-16. Epub 2014 May 6.

NYU Comprehensive Neurofibromatosis Center, Division of Pediatric Hematology/Oncology, Department of Pediatrics and Laura and Isaac Perlmutter Cancer Center at NYU Langone Medical Center, New York, New York (M.A.K., G.L., A.M., J.C.A.); Division of Oncology, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania (M.J.F., A.J.S.); Department of Radiology, NYU Langone Medical Center, New York, New York (S.S.M., M.C.B.); The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, Maryland (K.J.C.); Division of Pediatric Neurosurgery, Department of Neurosurgery, NYU Langone Medical Center, New York, New York (J.H.W., D.H.H.); Division of Biostatistics, Department of Population Health and The Laura and Isaac Perlmutter Cancer Center at NYU Langone Medical Center, New York, New York (J.D.G., T.H.); Department of Neurosurgery, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania (A.C.R); Division of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, California (G.D.); German Cancer Research Center and University Hospital, Heidelberg, Germany (D.T.W.J., A.K., S.M.P.); Division of Neuropathology, Department of Pathology, Johns Hopkins University, Baltimore, Maryland (C.G.E.); Division of Neuropathology, Department of Pathology, Department of Neurosurgery and Laura and Isaac Perlmutter Cancer Center at NYU Langone Medical Center, New York, New York (D.Z.).

Background: Activation of the RAS-RAF-MEK-ERK signaling pathway is thought to be the key driver of pediatric low-grade astrocytoma (PLGA) growth. Sorafenib is a multikinase inhibitor targeting BRAF, VEGFR, PDGFR, and c-kit. This multicenter phase II study was conducted to determine the response rate to sorafenib in patients with recurrent or progressive PLGA.

Methods: Key eligibility criteria included age ≥ 2 years, progressive PLGA evaluable on MRI, and at least one prior chemotherapy treatment. Sorafenib was administered twice daily at 200 mg/m(2)/dose (maximum of 400 mg/dose) in continuous 28-day cycles. MRI, including 3-dimensional volumetric tumor analysis, was performed every 12 weeks. BRAF molecular testing was performed on tumor tissue when available.

Results: Eleven patients, including 3 with neurofibromatosis type 1 (NF1), were evaluable for response; 5 tested positive for BRAF duplication. Nine patients (82%) came off trial due to radiological tumor progression after 2 or 3 cycles, including 3 patients with confirmed BRAF duplication. Median time to progression was 2.8 months (95% CI, 2.1-31.0 months). Enrollment was terminated early due to this rapid and unexpectedly high progression rate. Tumor tissue obtained from 4 patients after termination of the study showed viable pilocytic or pilomyxoid astrocytoma.

Conclusions: Sorafenib produced unexpected and unprecedented acceleration of tumor growth in children with PLGA, irrespective of NF1 or tumor BRAF status. In vitro studies with sorafenib indicate that this effect is likely related to paradoxical ERK activation. Close monitoring for early tumor progression should be included in trials of novel agents that modulate signal transduction.
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http://dx.doi.org/10.1093/neuonc/nou059DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4165419PMC
October 2014

Focal resection of leptomeningeal angioma in a rare case of Sturge-Weber syndrome without facial nevus.

Authors:
Yevgenia Shekhtman Irene Kim James J Riviello Sarah S Milla Howard L Weiner

Pediatr Neurosurg 2013 9;49(2):99-104. Epub 2014 Jan 9.

Division of Pediatric Neurosurgery, Department of Neurosurgery, New York University, New York, N.Y., USA.

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder comprised typically of a facial nevus, leptomeningeal angioma with calcifications, and seizures. SWS without a port-wine stain is a rare variant with only 30 cases reported in the literature. Here, a case of an 8-year-old girl with no cutaneous abnormalities presenting with medically intractable epilepsy and MRI and CT findings consistent with SWS is described. The patient underwent multistage surgery with subdural electrode monitoring before and after resection of the epileptogenic focus, with complete excision of the lesion and postoperative resolution of her seizures. This is the first reported case of three-stage surgery for localized resection of the seizure focus for SWS.
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http://dx.doi.org/10.1159/000357357DOI Listing
December 2014

Recurrent ascites in a patient with low-grade astrocytoma and ventriculo-peritoneal shunt treated with the multikinase inhibitor sorafenib.

Authors:
Geneviève Legault Mark W Kieran Robert Michael Scott Christine Chordas Sarah S Milla Matthias A Karajannis

J Pediatr Hematol Oncol 2014 Nov;36(8):e533-5

Departments of *Pediatrics ∥Radiology, NYU Langone Medical Center, New York, NY Departments of †Pediatric Oncology §Neurosurgery, Boston Children's Hospital ‡Department of Pediatric Neuro-Oncology, Dana-Farber Cancer Institute, Boston, MA.

This report describes a 6-year-old boy with disseminated low-grade astrocytoma and ventriculo-peritoneal shunt, who developed recurrent ascites while receiving sorafenib on a clinical trial. Laboratory analysis of the peritoneal fluid showed no elevation of protein content and no evidence of underlying infection or tumor dissemination. This report highlights ascites as a previously unrecognized adverse reaction to sorafenib in a patient with a ventriculo-peritoneal shunt. We conclude that such patients should be closely monitored for this complication when treated with sorafenib.
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http://dx.doi.org/10.1097/MPH.0000000000000094DOI Listing
November 2014

Free-breathing contrast-enhanced T1-weighted gradient-echo imaging with radial k-space sampling for paediatric abdominopelvic MRI.

Authors:
Hersh Chandarana Kai T Block Matthew J Winfeld Shailee V Lala Daniel Mazori Emalyn Giuffrida James S Babb Sarah S Milla

Eur Radiol 2014 Feb 25;24(2):320-6. Epub 2013 Sep 25.

Department of Radiology, New York University Langone Medical Center, 660 First Avenue, New York, NY, 10016, USA,

Objective: To compare the image quality of contrast-enhanced abdominopelvic 3D fat-suppressed T1-weighted gradient-echo imaging with radial and conventional Cartesian k-space acquisition schemes in paediatric patients.

Methods: Seventy-three consecutive paediatric patients were imaged at 1.5 T with sequential contrast-enhanced T1-weighted Cartesian (VIBE) and radial gradient echo (GRE) acquisition schemes with matching parameters when possible. Cartesian VIBE was acquired as a breath-hold or as free breathing in patients who could not suspend respiration, followed by free-breathing radial GRE in all patients. Two paediatric radiologists blinded to the acquisition schemes evaluated multiple parameters of image quality on a five-point scale, with higher score indicating a more optimal examination. Lesion presence or absence, conspicuity and edge sharpness were also evaluated. Mixed-model analysis of variance was performed to compare radial GRE and Cartesian VIBE.

Results: Radial GRE had significantly (all P < 0.001) higher scores for overall image quality, hepatic edge sharpness, hepatic vessel clarity and respiratory motion robustness than Cartesian VIBE. More lesions were detected on radial GRE by both readers than on Cartesian VIBE, with significantly higher scores for lesion conspicuity and edge sharpness (all P < 0.001).

Conclusion: Radial GRE has better image quality and lesion conspicuity than conventional Cartesian VIBE in paediatric patients undergoing contrast-enhanced abdominopelvic MRI.

Key Points: • Numerous techniques are required to provide optimal MR images in paediatric patients. • Radial free-breathing contrast-enhanced acquisition demonstrated excellent image quality. • Image quality and lesion conspicuity were better with radial than Cartesian acquisition. • More lesions were detected on contrast-enhanced radial than on Cartesian acquisition. • Radial GRE can be used for performing abdominopelvic MRI in paediatric patients.
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http://dx.doi.org/10.1007/s00330-013-3026-4DOI Listing
February 2014