Trends and Costs Associated With the Diagnosis and Treatment of Infantile Spasms: A 10-Year Multicenter Retrospective Review

OBJECTIVE Early treatment of infantile spasms (IS) may be imperative for improvement of neurodevelopmental outcomes. Existing studies have led to inconclusive recommendations with variation in treatment. Our objective was to determine the national average cost, initial diagnostic workup, treatments, and hospital length of stay for patients with IS. METHODS This retrospective cohort study was designed to review data of patients < 2 years from 43 non-profit institutions. Data obtained included patient demographics, length of stay, admission cost, and treatments used from 2004 to 2014. Cost data were collected and adjusted to 2014 dollars, the year data were analyzed. RESULTS A total of 6183 patients met study criteria (n = 3382, 55% male). Three-quarters of patients (n = 4684, 76%) had an electroencephalogram, 56.4% had brain imaging (n = 3487), and 17% (n = 1050) underwent a lumbar puncture. Medication for IS was initiated during inpatient hospital stay in two-thirds of all patients (n = 4139, 67%). Most patients were initiated on corticotropin (n = 2066, 33%) or topiramate (n = 1804, 29%). Average length of stay was 5.8 days with an average adjusted cost of $18,348. Over time there was an 86.6% increase in cost from an average $12,534.54 (2004) to $23,391.20 (2014), a significant change (p < 0.01). This correlated with an increase in average length of stay. CONCLUSIONS Variability exists in diagnostic workup and pharmacotherapy initiated for IS, which may lead to differences in the cost of hospital stay. Further studies may help determine contributing factors to increased cost and improve health care utilization for IS patients.

ACCURACY OF TRANSTHORACIC ECHOCARDIOGRAPHY IN DIAGNOSIS OF CARDIAC MYXOMA: SINGLE CENTRE EXPERIENCE

The differential diagnosis of cardiac myxomas (CM), the most common benign primary cardiac tumors, is broad and a thorough diagnostic workup is required to establish accurate diagnosis prior to surgical resection. Transthoracic echocardiography (TTE) is usually the first imaging modality used for diagnosis of suspected CM. Purpose In a single tertiary centre study, we sought to determine the accuracy, sensitivity, and specificity of TTE in the diagnosis of CM and to determine echocardiographic characteristics indicative of CM. Methods and results We retrospectively analyzed clinical, echocardiographic, and pathohistological findings of 73 patients consecutively admitted for suspected CM. After diagnostic workup, 53 (73%) patients were treated surgically at our institution. Based on preoperative TTE, patients were divided into a CM group (n=45, 85%) and non-myxoma (NM) group. Of the 53 pathohistological specimens obtained during surgery, 39 (73%) were CM. The sensitivity and specificity of preoperative echocardiography were 97% and 50%, respectively. The overall accuracy was 85%. All NM tumors were found in an atypical location and 72% of CM were found in a typical position in the left atrium (p<0.001). Tumors in NM group were significantly smaller than CM (24.3±13.2 mm vs 37.9±18.3 mm, p=0.017). Conclusion Our study confirms very good accuracy of TTE in the diagnosis of CM. The most important echocardiographic characteristics to differentiate between CM and tumors of different etiology are tumor location and size. Smaller tumors presenting at an atypical location are less likely to be diagnosed as CM, and these require additional imaging modalities for accurate diagnosis.

Spasticity and Dystonia are Underidentified in Young Children at High Risk for Cerebral Palsy

Background Early spasticity and dystonia identification in cerebral palsy is critical for guiding diagnostic workup and prompting targeted treatment early when it is most efficacious. However, differentiating spasticity from dystonia is difficult in young children with cerebral palsy. Methods We sought to determine spasticity and dystonia underidentification rates in children at high risk for cerebral palsy (following neonatal hypoxic-ischemic encephalopathy) by assessing how often child neurologists identified hypertonia alone versus specifying the hypertonia type as spasticity and/or dystonia by age 5 years. Results Of 168 children, 63 developed cerebral palsy and hypertonia but only 19 (30%) had their hypertonia type specified as spasticity and/or dystonia by age 5 years. Conclusions Child neurologists did not specify the type of hypertonia in a majority of children at high risk of cerebral palsy. Because early tone identification critically guides diagnostic workup and treatment of cerebral palsy, these results highlight an important gap in current cerebral palsy care.

Added diagnostic accuracy after [18F]flutemetamol PET scanning in young patients with dementia in an academic memory clinic setting

ABSTRACTIntroductionA timely diagnosis of Alzheimer’s Disease (AD) in an early stage of dementia is important to support timely access to treatment, advice, and care. The aim of this study was to estimate the diagnostic accuracy of [18F]flutemetamol PET in addition to the usual diagnostic workup for the diagnosis of AD in a memory clinic population with young onset dementia by means of a panel reference-based etiology diagnosis.Methodsin an academic memory clinic early onset dementia cohort (n=211) the nosological diagnosis was set by usual diagnostic workup and after including [18F]flutemetamol amyloid PET in a stepwise approach. To assess the change in proportion correctly diagnosed, the diagnosis with and without [18F]flutemetamol PET was related to a panel-based reference standard, serving as gold standard, consisting of 3 neurologists who relied on available clinical information over 2-year follow-up (n=152; blinded for PET).ResultsThe panel majority nosology was set as a reference diagnosis in 122 participants, leaving 30 (20%) participants with no majority reached. In 107 (88%) cases post-PET was in line with the reference, and in 103 (84%) the pre-PET diagnosis was in line with the reference. The difference was 3.3% (95% CI -3.5% to 10.1%; p=0.424).Discussion[18F]flutemetamol PET did not significantly improve the diagnostic accuracy in young patients with dementia in an academic memory clinic setting. The secondary analyses provided several indications for future research in a narrower subsample of persons with (very) high diagnostic uncertainty and to assess patient relevant health outcomes.

Systematic review of clinical evidence on postoperative delirium: literature search of original studies based on validated diagnostic scales

Background Postoperative delirium is a serious complication that can occur within the 5th postoperative day. In 2017, the European Society of Anesthesiologists delivered dedicated guidelines that reported the need for routine monitoring using validated scales. Objective Aim of this systematic review is to identify clinical studies related to postoperative delirium that included postoperative monitoring with validated scales. Design Systematic review Methods Searched keywords included the following terms: postoperative, postsurgical, post anesthesia, anesthesia recovery, delirium, and confusion. Two researchers independently screened retrieved studies using a data extraction form. Results Literature search led to retrieve 6475 hits; of these, 260 studies (5.6% of the retrieved), published between 1987 and 2021, included in their methods a diagnostic workup with the use of a postoperative delirium validated scale and monitored patients for more than 24 h, therefore are qualified to be included in the present systematic review. Conclusion In conclusion, available clinical literature on postoperative delirium relies on a limited number of studies, that included a validated diagnostic workup based on validated scales, extracted from a large series of studies that used inconsistent diagnostic criteria. In order to extract indications based on reliable evidence-based criteria, these are the studies that should be selectively considered. The analysis of these studies can also serve to design future projects and to test clinical hypothesis with a more standardized methodological approach.

Racial Disparities in the Diagnostic Evaluation of Multiple Myeloma

Introduction: Multiple myeloma (MM) is the most common hematologic malignancy in Black individuals with a 2-to-3-fold higher incidence rate among Black compared to White individuals. While therapeutic advances have led to significant increases in survival rates in MM across races, there still exist racial disparities in outcomes that have been attributed to inferior access to novel therapies and autologous stem cell transplant among Black patients. Risk stratification is an important strategy that allows clinicians to identify high-risk disease and potentially tailor therapy based on staging to try to abrogate its poor prognosis. Current risk stratification schemata in MM, such as the International Staging System (ISS) and Revised ISS (R-ISS), necessitate serum laboratory data and fluorescence in-situ hybridization (FISH) cytogenetic analysis of bone marrow specimens. To the best of our knowledge, it is unknown if discrepancies exist in the initial diagnostic workup of MM between Blacks and Whites, which ultimately may have treatment and outcome implications. We sought to assess the presence of racial disparities in the diagnostic workup of newly diagnosed MM among Black and White patients. Methods: We performed a retrospective cohort study using the Surveillance, Epidemiology and End Results (SEER)-Medicare linked database, which includes 16,174 MM patients with patient-level demographics, survival data, and health care claims information. The data included patients ≥ 65 years-old with the diagnosis of MM between 2001-2015. Race was documented by SEER registries. Lab and imaging data were collected from 180 days before and after diagnosis to capture an ample and appropriate allotted time for diagnostic workup. CPT codes were queried to determine the frequency of the diagnostic tests of interest. Data were analyzed through R version 4.0.2. Pearson chi-squared tests were used to compare frequency of diagnostic testing between racial groups. Results: A total of 18,267 MM patients were identified in the SEER-Medicare linked database. Of that, 15,360 MM patients (12,645 White and 2,715 Black) were identified with peripheral blood laboratory, bone marrow, and imaging health care claims data available . The remaining 2,907 patients with a documented race other than White or Black were excluded. Complete blood count and comprehensive metabolic panel serum tests were used to evaluate completeness of CPT codes use, which demonstrated that &gt;89% (13,723/15,360) of individuals had both tests performed. Overall, Black patients had lower frequency of nearly all serum and imaging tests completed relative to White patients (Table 1). Only 61% of White patients underwent the testing components necessary to adequately risk-stratify disease by ISS (e.g., beta-2 microglobulin) compared to 50% of Black patients (relative difference 21%). 30% of White individuals underwent the components to determine R-ISS (e.g., FISH cytogenetics). There were low overall rates of FISH cytogenetics obtained in the study population, with White individuals undergoing FISH cytogenetics at a rate of 30% compared to 25% among Black individuals (relative difference 18%). Magnetic resonance imaging (MRI) of the lumbar spine, the most commonly imaged portion of the spine by MRI, was performed more commonly in White vs Black individuals (33% vs 24%, relative difference 35%). PET/CT was performed in only a small percentage of patients (Whites 9% vs Blacks 5%, relative difference 94%). Conclusions: In a real-world analysis of patients with newly diagnosed MM, we found that Black patients were less likely than White patients to undergo a complete initial diagnostic evaluation. While rates of beta-2 microglobulin and FISH cytogenetics testing were low among all patients, Black patients were less likely to undergo testing needed to complete staging for ISS/R-ISS or proper imaging to assess for extramedullary disease (i.e., PET/CT). Whether these differences between the races in the initial diagnostic workup lead to differences in treatment strategies and survival outcomes deserves additional investigation. Further work is needed to improve access to complete diagnostic evaluation among Black patients with newly diagnosed MM. Figure 1 Figure 1. Disclosures Calip: Pfizer: Research Funding; Roche: Current equity holder in publicly-traded company; Flatiron Health: Current Employment. Derman: Sanofi: Membership on an entity's Board of Directors or advisory committees.