Multidisciplinary coordinated care of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease).

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare disorder with a case prevalence as high as one in 5000, causing arteriovenous malformations in multiple organ systems. HHT is familial with autosomal dominant inheritance, with genetic testing allowing confirmation of the diagnosis in asymptomatic kindreds. Common clinical manifestations are epistaxis and intestinal lesions causing anemia and requiring transfusions. Pulmonary vascular malformations predispose to ischemic stroke and brain abscess and may cause dyspnea and cardiac failure. Brain vascular malformations can cause hemorrhagic stroke and seizures. Rarely, liver arteriovenous malformations can cause hepatic failure. A form of HHT can cause juvenile polyposis syndrome and colon cancer. Specialists in multiple fields may be called to care for one or more aspects of HHT, but few are familiar with evidence-based guidelines for HHT management or see a sufficient number of patients to gain experience with the unique characteristics of the disease. Primary care physicians and specialists are often unaware of the important manifestations of HHT in multiple systems and the thresholds for their screening and appropriate management. To improve familiarity, experience, and coordinated multisystem care for patients with HHT, the Cure HHT Foundation, which advocates for patients and families with this disease, has accredited 29 centers in North America with designated specialists for the evaluation and care of patients with HHT. Team assembly and current screening and management protocols are described as a model for evidence-based, multidisciplinary care in this disease.

Patient Safety Analysis in Radiation Burden of Head Computed Tomography Imaging in 1185 Neurosurgical Inpatients.

OBJECTIVE: We performed a retrospective analysis in a cohort of 1185 patients at our institution who were identified as undergoing ≥1 head computed tomography (CT) examinations during their inpatient stay on the neurosurgery service, to quantify the number, type, and associated radiation burden of head CT procedures performed by the neurosurgery service. METHODS: CT procedure records and radiology reports were obtained via database search and directly validated against records retrieved from manual chart review. Next, dosimetry data from the head CT procedures were extracted via automated text mining of electronic radiology reports. RESULTS: Among 4510 identified adult head CT procedures, 88% were standard head CT examinations. A total of 3.65 ± 3.60 head CT scans were performed during an average adult admission. The most common primary diagnoses were neoplasms, trauma, and other hemorrhage. The median cumulative effective dose per admission was 5.66 mSv (range, 1.06-84.5 mSv; mean, 8.56 ± 8.95 mSv). The median cumulative effective dose per patient was 6.4 mSv (range, 1.1-127 mSv; mean, 9.26 ± 10.0 mSv). CONCLUSIONS: The median cumulative radiation burden from head CT imaging in our cohort equates approximately to a single chest CT scan, well within accepted limits for safe CT imaging in adults. Refined methods are needed to characterize the safety profile of the few pediatric patients identified in our study.

Patient Satisfaction Ratings of Male and Female Residents Across Subspecialties.

BACKGROUND: Females currently comprise approximately 50% of incoming medical students yet continue to be underrepresented in certain medical subspecialties. OBJECTIVE: To assess whether gender plays a role in patients' perception of physician competency among different specialties. METHODS: We administered surveys at 2 academic medical centers to patients who were stable, cognitively aware, and indicated English as their primary language. Survey questions evaluated communication, medical expertise, and quality of care. RESULTS: A total of 4222 surveys were collected. Females comprised around half (n = 2133, 50.7%) of evaluated residents. First-year (n = 1647, 39%) and second-year (n = 1416, 33.5%) residents were assessed most frequently. Internal medicine conducted the most surveys (n = 1111, 23.6%), whereas head and neck surgery conducted the least (n = 137, 3.24%). There was no statistically significant difference between patients' perception of male and female residents of the same year in overall communication skills, medical expertise, and quality of medical care. Female residents outperformed their male counterparts on specific questions evaluating the communication of treatment plans, patient education, and patient satisfaction (P < .001, P = .03, P = .04, respectively). Unsurprisingly, patients' perceptions of residents' overall communication skills, medical expertise, and quality of medical care significantly improved when comparing more experienced residents to newer residents. CONCLUSION: There is no difference between overall communication, medical expertise, and quality of care between sexes, and across subspecialties. Though gender inequalities currently exist most starkly in practitioners in surgical subspecialties, women in surgical residencies were much better communicators than their male counterparts, but still perceived to have similar levels of medical expertise and quality of care.

Racial Differences in Hospital Stays among Patients Undergoing Craniotomy for Tumour Resection at a Single Academic Hospital.

BACKGROUND: Racial differences in American patients undergoing brain tumour surgery remain poorly characterized within urban medical centres. Our objective was to assess racial differences in operative brain tumour patients at a single academic hospital in Los Angeles, California. METHODS: We reviewed medical records of adult patients undergoing craniotomy for tumour resection from March 2013 to January 2017 at UCLA Medical Centre. Patients were categorized as Asian, Hispanic, Black, or White. Racial cohorts were matched on demographic variables for comparisons. Our primary outcome was post-operative length of stay (LOS). Secondary outcomes included hospital mortality and discharge disposition. RESULTS: In this study, 462 patients identified as Asian (15.1%), Hispanic (8.7%), Black (3.9%), or White (72.3%). After cohort matching, non-White patients had elevated risk of prolonged LOS [odds ratio (OR)=2.62 (1.44, 4.76)]. No differences were observed in hospital mortality or non-routine discharge. Longer LOS was positively correlated with non-routine discharge [rpb (458)=0.41, p<0.001]. Black patients with government insurance had average LOS 2.84 days shorter than Black patients with private insurance (p=0.04). Among Hispanics, government insurance was associated with non-routine discharge [OR=4.93 (1.03, 24.00)]. CONCLUSION: Racial differences manifested as extended LOS for non-White patients, with comparable rates of hospital mortality and non-routine discharge across races. Prolonged LOS loosely reflected complicated clinical course with greater risk of adverse discharge disposition. Private insurance coverage predicted markedly lower risk of non-routine discharge for Hispanic patients, and LOS of three additional days among Black patients. Further research is needed to elucidate the basis of these differences.

Natural history of the aging spine: a cross-sectional analysis of spinopelvic parameters in the asymptomatic population.

OBJECTIVE: There have been numerous studies demonstrating increased pain and disability when patients' spinopelvic parameters fall outside of certain accepted ranges. However, these values were established based on patients suffering from spinal deformities. It remains unknown how these parameters change over a lifetime in asymptomatic individuals. The goal of this study was to define a range of spinopelvic parameters from asymptomatic individuals. METHODS: Sagittal scoliosis radiographs of 210 asymptomatic patients were evaluated. All measurements were reviewed by 2 trained observers, supervised by a trained clinician. The following parameters and relationships were measured or calculated: cervical lordosis (CL), thoracic kyphosis (TK), lumbar lordosis (LL), pelvic incidence (PI), sagittal vertical axis (SVA), cervical SVA (cSVA), and T1 slope, TK/LL, truncal inclination, pelvic tilt (PT), LL-PI, LL/PI, and T1 slope/PI. Patients were stratified by decade of life, and regression analysis was performed to delineate the relationship between each consecutive age group and the aforementioned parameters. RESULTS: Cervical lordosis (R2 = 0.61), thoracic kyphosis (R2 = 0.84), SVA (R2 = 0.88), cSVA (R2 = 0.51), and T1 slope (R2 = 0.77) all increase with age. Truncal inclination (R2 = 0.36) and T1 slope/CL remain stable over all decades (R2 = 0.01). LL starts greater than PI, but in the 6th decade of life, LL becomes equal to PI and in the 7th decade becomes smaller than PI (R2 = 0.96). The ratio of TK/LL is stable until the 7th decade of life (R2 = 0.81), whereas PT is stable until the 6th decade (R2 = 0.92). CONCLUSIONS: This study further refines the generally accepted LL = PI + 10° by showing that patients under the age of 50 years should have more LL compared to PI, whereas after the 5th decade the relationship is reversed. SVA was not as sensitive across age groups, exhibiting a marked increase only in the 7th decade of life. Given the reliable increase of CL with age, and the stability of T1 slope/CL, this represents another important relationship that should be maintained when performing cervical deformity/fusion surgery. This study has important implications for evaluating adult patients with spinal deformities and for establishing corrective surgical goals.

Age and gender considerations on the symptomology in patients with superior semicircular canal dehiscence: A systematic review and case illustration.

Superior semicircular canal dehiscence (SSCD) is caused by a fistula in the arcuate eminence, creating vestibular and auditory disturbances. We aim to determine the effects of gender and age on symptom prevalence and resolution in patients with SSCD. A Boolean search was conducted through four separate scientific databases. Full-text English articles for SSCD patients, who underwent surgery were included. Demographics and outcomes were extracted. A total of 198 patients were identified, and available for quantitative analysis. Between genders, there were no differences in the prevalence of pre- or post-operative symptomology. Both genders had statistically significant improvement in symptomatology with females experiencing significantly high rates of hearing loss improvement compared to male patients. Compared to their younger cohort, patients over 65 had similar symptom frequencies before and after surgery with similar rates of symptom resolution. SSCD repair is safe and effective for resolving auditory and vestibular symptoms. Gender and age may not be strong predictors of patient presentation or symptom resolution. Gender and age-associated factors may not influence patient outcomes.

The Meningioma Vascularity Index: a volumetric analysis of flow voids to predict intraoperative blood loss in nonembolized meningiomas.

OBJECTIVEMeningiomas that appear hypervascular on neuroimaging could be amenable to preoperative embolization. However, methods for measuring hypervascularity have not been described, nor has the benefit of preoperative embolization been adjudicated. The objective of this study was to show a relationship between flow void volume (measured on MRI) and intraoperative estimated blood loss (EBL) in nonembolized meningiomas.METHODSThe authors performed volumetric analyses of 51 intracranial meningiomas (21 preoperatively embolized) resected at their institution. Through the use of image segmentation software and a voxel-based segmentation method, flow void volumes were measured on T2-weighted MR images. This metric was named the Meningioma Vascularity Index (MVI). The primary outcomes were intraoperative EBL and perioperative blood transfusion.RESULTSIn the nonembolized group, the MVI correlated with intraoperative EBL when controlling for tumor volume (r = 0.55, p = 0.002). The MVI also correlated with perioperative blood transfusion (point-biserial correlation [rpb] = 0.57, p = 0.001). A greater MVI was associated with an increased risk of blood transfusion (odds ratio [OR] 5.79, 95% confidence interval [CI] 1.15-29.15) and subtotal resection (OR 7.64, 95% CI 1.74-33.58). In the embolized group, those relationships were not found. There were no significant differences in MVI, intraoperative EBL, or blood transfusion across groups.CONCLUSIONSThis study clearly shows a relationship between MVI and intraoperative EBL in nonembolized meningiomas when controlling for tumor volume. The MVI is a potential biomarker for tumors that would benefit from embolization.

Risk of Brain Tumor Induction from Pediatric Head CT Procedures: A Systematic Literature Review.

Head computed tomography (CT) is instrumental for managing patients of all ages. However, its low dose radiation may pose a low but non-zero risk of tumor induction in pediatric patients. Here, we present a systematic literature review on the estimated incidence of brain tumor induction from head CT exams performed on children and adolescents. MEDLINE was searched using an electronic protocol and bibliographic searches to identify articles related to CT, cancer, and epidemiology or risk assessment. Sixteen studies that predicted or measured head CT-related neoplasm incidence or mortality were identified and reviewed. Epidemiological studies consistently cited increased tumor incidence in pediatric patients (ages 0-18) exposed to head CTs. Excess relative risk of new brain tumor averaged 1.29 (95% confidence interval, 0.66-1.93) for pediatric patients exposed to one or more head CTs. Tumor incidence increased with number of pediatric head CTs in a dose-dependent manner, with measurable excess incidence even after a single scan. Converging evidence from epidemiological studies supported a small excess risk of brain tumor incidence after even a single CT exam in pediatric patients. However, refined epidemiological methods are needed to control for confounding variables that may contribute to reverse causation, such as patients with pre-existing cancer or cancer susceptibility. CT remains an invaluable technology that should be utilized so long as there is clinical indication for the study and the radiation dose is as small as reasonably achievable.

Planned Subtotal Resection of Vestibular Schwannoma Differs from the Ideal Radiosurgical Target Defined by Adaptive Hybrid Surgery.

OBJECTIVE: To retrospectively compare ideal radiosurgical target volumes defined by a manual method (surgeon) to those determined by Adaptive Hybrid Surgery (AHS) operative planning software in 7 patients with vestibular schwannoma (VS). METHODS: Four attending surgeons (3 neurosurgeons and 1 ear, nose, and throat surgeon) manually contoured planned residual tumors volumes for 7 consecutive patients with VS. Next, the AHS software determined the ideal radiosurgical target volumes based on a specified radiotherapy plan. Our primary measure was the difference between the average planned residual tumor volumes and the ideal radiosurgical target volumes defined by AHS (dRVAHS-planned). RESULTS: We included 7 consecutive patients with VS in this study. The planned residual tumor volumes were smaller than the ideal radiosurgical target volumes defined by AHS (1.6 vs. 4.5 cm3, P = 0.004). On average, the actual post-operative residual tumor volumes were smaller than the ideal radiosurgical target volumes defined by AHS (2.2 cm3 vs. 4.5 cm3; P = 0.02). The average difference between the ideal radiosurgical target volume defined by AHS and the planned residual tumor volume (dRVAHS-planned) was 2.9 ± 1.7 cm3, and we observed a trend toward larger dRVAHS-planned in patients who lost serviceable facial nerve function compared with patients who maintained serviceable facial nerve function (4.7 cm3 vs. 1.9 cm3; P = 0.06). CONCLUSIONS: Planned subtotal resection of VS diverges from the ideal radiosurgical target defined by AHS, but whether that influences clinical outcomes is unclear.

Bone Metabolic Markers in the Clinical Assessment of Patients with Superior Semicircular Canal Dehiscence.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is a bony defect in the osseous shell of the petrous temporal bone. The pathophysiological association between osteoporosis and SSCD remains poorly understood. We investigated the relationship between bone metabolic markers and symptoms in patients with SSCD. METHODS: We collected patient demographics and clinical parameters for adult patients diagnosed with SSCD on high-resolution computed tomography scans. We used point-biserial correlation analysis to investigate the relationship between bone metabolic markers and symptoms in patients with SSCD. We compared clinical symptoms before and after surgical repair of SSCD through a middle fossa craniotomy using McNemar's test for paired comparisons of binary measures. RESULTS: We included a total of 99 patients (64 females and 35 males; average age 52 years; 118 surgeries). The level of serum calcium correlated with the need for a second surgery (rpb = -0.35, P = 0.001). Postoperative calcium supplementation negatively correlated with improvement in dizziness (rpb = -0.36, P = 0.01). The level of 25-hydroxyvitamin D correlated with preoperative hyperacusis (rpb = -0.98, P = 0.02) and postoperative autophony (rpb = 0.96, P = 0.04). Postoperative vitamin D supplementation positively correlated with hearing decline (rpb = 0.04, P = 0.04) The level of thyroid stimulating hormone correlated with preoperative autophony, amplification, and tinnitus (rpb = -0.71, rpb = -0.75, rpb = -0.70, all P < 0.001). CONCLUSIONS: Bone metabolic markers could be important in the clinical assessment of SSCD patients and could be potential targets for symptom management.

Dural Repair in Cranial Surgery Is Associated with Moderate Rates of Complications with Both Autologous and Nonautologous Dural Substitutes.

OBJECTIVE: Duraplasty, a common neurosurgical intervention, involves synthetic or biological graft placement to ensure dural closure. The objective of this study is to advance our understanding of the use of dural substitutes in cranial surgery. METHODS: The PubMed database was systematically searched to identify studies published over the past decade (2007-2017) that described duraplasty procedures. Clinical data were disaggregated and analyzed for the comparisons of biological versus synthetic grafts. RESULTS: A total of 462 cases were included in the quantitative synthesis. Overall, the most common indication for duraplasty was tumor resection (53%). Allografts were more frequently used in decompression for Chiari malformations compared with xenografts and synthetic grafts (P < 0.001). Xenografts were more frequently used in decompressive hemicraniectomy procedures for evacuation of acute subdural hematomas over allografts and synthetics (P < 0.001). Synthetic grafts were more frequently used in tumor cases than biological grafts (P = 0.002). The cumulative complication rate for dural substitutes of all types was 11%. There were no significant differences in complication rates among the 3 types of dural substitutes. CONCLUSIONS: Dural substitutes are commonly used to ensure dural closure in a variety of cranial procedures. This study provides greater insight into duraplasty practices and highlights the moderate complication rate associated with the procedure. Future studies are needed to determine the safety and efficacy of such procedures in larger prospective cohorts.

Restrictive transfusion threshold is safe in high-risk patients undergoing brain tumor surgery.

OBJECTIVE: To assess the safety of a restrictive threshold for the transfusion of red blood cells (RBCs) compared to a liberal threshold in high-risk patients undergoing brain tumor surgery. PATIENTS AND METHODS: We reviewed patients who were 50 years of age or older with a preoperative American Society of Anesthesiologists physical status class II to V who underwent open craniotomy for tumor resection and were transfused packed RBCs during or after surgery. We retrospectively assigned patients to a restrictive-threshold (a pretransfusion hemoglobin level <8g/dL) or a liberal-threshold group (a pretransfusion hemoglobin level of 8-10/dL). The primary outcome was in-hospital mortality rate. Secondary outcomes were in-hospital complication rates, length of stay, and discharge disposition. RESULTS: Twenty-five patients were included in the study, of which 17 were assigned to a restrictive-threshold group and 8 patients to a liberal-threshold group. The in-hospital mortality rates were 12% for the restrictive-threshold group (odds ratio [OR] 0.93, 95% confidence interval [CI] 0.07-12.11) and 13% for the liberal-threshold group. The in-hospital complication rates were 52.9% for the restrictive-threshold group (OR 1.13, 95% CI 0.21-6.05) and 50% for the liberal-threshold group. The average number of days in the intensive care unit and hospital were 8.6 and 22.4 days in the restrictive-threshold group and 6 and 15 days in the liberal-threshold group, respectively (P=0.69 and P=0.20). The rates of non-routine discharge were 71% in the restrictive-threshold group (OR 2.40, 95% CI 0.42-13.60) and 50% in the liberal-threshold group. CONCLUSIONS: A restrictive transfusion threshold did not significantly influence in-hospital mortality or complication rates, length of stay, or discharge disposition in patients at high operative risk.