Volumetric segmentation in the context of posterior fossa-related pathologies: a systematic review.

BACKGROUND: Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior fossa-related conditions, such as Chiari malformation, trigeminal neuralgia, post-operative pediatric cerebellar mutism syndrome, and Crouzon syndrome. Herein, we present a summary of the current literature on segmentation of the posterior fossa. The review highlights the various segmentation techniques, and their respective strengths and weaknesses, employed along with objectives and outcomes of the various studies reported in the literature. METHODS: A literature search was conducted in PubMed, Embase, Cochrane, and Web of Science up to November 2023 for articles on segmentation techniques of posterior fossa. The two senior authors searched through databases based on the keywords of the article separately and then enrolled joint articles that met the inclusion and exclusion criteria. RESULTS: The initial search identified 2205 articles. After applying inclusion and exclusion criteria, 77 articles were selected for full-text review after screening of titles/abstracts. 52 articles were ultimately included in the review. Segmentation techniques included manual, semi-automated, and fully automated (atlas-based, convolutional neural networks). The most common pathology investigated was Chiari malformation. CONCLUSIONS: Various forms of segmentation techniques have been used to assess posterior fossa volumes/pathologies and each has its advantages and disadvantages. We discuss these nuances and summarize the current state of literature in the context of posterior fossa-associated pathologies.

STAT5b gain-of-function disease in a child with mycobacterial osteomyelitis of the skull: rare presentation of an emerging disease entity.

PURPOSE: STAT proteins play a key role in several cellular functions related to cell development, differentiation, proliferation, and survival. Persistent STAT activation due to somatic STAT5bN642H gain-of-function mutation is a rare mechanism of STAT dysregulation that results in hypereosinophilia, frequent infections, leukemias, and pulmonary diseases. Herein, we describe a case of a child with a rare early onset STAT5b gain-of-function disease treated with targeted JAK inhibition who developed a cranial Mycobacterium avium osteomyelitis. METHODS: A 3-year-old male with a known STAT5b gain-of-function mutation presented with a 10-day history of a firm, immobile, non-painful cranial mycobacterium mass with dural infiltration located anterior to the coronal suture. Stepwise management finalized with complete resection of the lesion with calvarial reconstruction. A case-based literature review was performed evaluating all patients with this mutation who developed cranial disease. RESULTS: The patient was symptom and lesion-free at 1 year since surgical resection and initiation of triple mycobacterial pharmacotherapy. Our literature review demonstrated the rarity of this disease, as well as other presentations of this disease in other patients. CONCLUSION: Patients with STAT5b gain-of-function mutations have attenuated Th1 responses and are treated with medications, such as JAK inhibitors, which further inhibit other STAT proteins that regulate immunity against rare infectious entities, such as mycobacterium. Our case highlights the importance of considering these rare infections in patients on JAK inhibitors and with STAT protein mutations. Possessing a clear mechanistic understanding of this genetic mutation, its downstream effect, and the consequences of treatment may enhance a physician's diagnostic and clinical management of similar patients in the future.

The selective dorsal rhizotomy technique for spasticity in 2020: a review.

This review looks at the advances in the surgical technique, selective dorsal rhizotomy, used for the management of spasticity in children.

Patient monitoring programs in oncology pharmacy practice: A survey of oncology pharmacists in Atlantic Canada.

BACKGROUND: There has been a dramatic increase in new drug approvals in oncology, consisting of both small molecule inhibitors and monoclonal antibodies. However, Health Canada approval for many of the new agents was based on single randomized trials consisting of only a few hundred patients. As more patients get treated with these newer agents, there is the potential for new and discrete toxicities. Pharmacists are in an ideal position to identify, monitor, manage, and even preempt future events, given their close proximity to the patient. However, the extent of pharmacists' involvement in formal patient programs is unknown. To address this knowledge gap, a survey of oncology pharmacists practicing in Atlantic Canada was conducted. METHODS: A structured mailing strategy was adopted as recommended by Dillman (1978). Standardized data collection forms were electronically mailed to 60 oncology pharmacists. Survey items consisted of respondent demographic information, practice setting, the existence of a formal patient monitoring program managed, and if patients are contacted by telephone following the completion of their anticancer cycle. RESULTS: Overall, 31 completed surveys were received, for an overall response rate of 50%. Respondents had a median age of 42 and a median of 18 years' (range 1 to 25) professional experience as a pharmacist. Only 18 of the 31 (58%) respondents indicated that there was a formal monitoring and call back program managed by pharmacy available at their institution. For those without such programs, the main reasons were due to staffing issues and lack of adequately trained clinical personnel. Overall, 100% of respondents would favor the development of a formal monitoring program in hospitals with a high volume of anticancer drug prescribing. CONCLUSIONS: Even though the number of new anticancer drugs being introduced into clinical pharmacy practice is increasing, formal patient monitoring and patient call back programs are not universal in Atlantic Canada hospitals.

Developing recommendations for the safe handling of oral anti-cancer drugs in community pharmacies: A pan-Canadian consensus approach.

PURPOSE: To create a set of consensus-based and evidence-informed recommendations to provide guidance around the safe dispensing and handling of oral anti-cancer drugs in low-volume settings unique to the community pharmacy setting. METHODS: A review of published and grey literature (published in non-commercial domains such as national organizations and associations) documents and nine key informant interviews were conducted and a modified Delphi approach was taken to achieve consensus. The final list of 47 candidate recommendations was reviewed by a task force and validated by multi-disciplinary stakeholders. A draft of the statements was circulated broadly within the community pharmacy community in an effort to assess relevance and implementation feasibility. RESULTS: The final report included 44 recommendations that addressed 11 key areas germane to the safe handling of oral anti-cancer drugs in community pharmacies. Mean agreement increased from 70% to 95%. Early feedback from community pharmacy leaders during the external review suggests that many of the proposed recommendations can be feasibly implemented within a reasonable timeframe when released with appropriate education and resource materials. CONCLUSIONS: A modified-Delphi approach supplemented by key informant interviews and a comprehensive external review resulted in a set of evidence-informed, community-driven recommendations for community pharmacies. The recommendations address a gap in existing literature to improve understanding of the risks associated with handling and dispensing oral anti-cancer drugs for both community pharmacy staff and management and offer mitigating strategies to reduce those risks. Incorporating feasibility assessment actions early (through the key informant interviews) and late (through the external review) ensures recommendations are grounded in practicality and support broad and early knowledge translation strategies.

Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis: Impact of Family History.

OBJECTIVE: The main objective of this study was to determine if family history of malignant peripheral nerve sheath tumor (MPNST) increases risk of developing an MPNST in patients with neurofibromatosis-1 (NF-1). MATERIALS AND METHODS: Individuals with NF-1 registered with the Children's Tumor Foundation's Neurofibromatosis Registry were emailed an anonymous 15-minute survey with regard to personal and family history of NF-1, MPNST, ages of onset, and symptomatology. Participation was voluntary and information was self-reported. RESULTS: The survey was sent to 4801 registrants, 878 responded. Presence of a family history of MPNST was found to be a risk factor for the development of MPNST; 19.4% of respondents confirming a family history of MPNST developed MPNST compared with 7.5% of respondents with no family history (odds ratio, 2.975; 95% confidence interval, 1.232-7.187; P=0.021). NF-1 patients with a positive family history developed MPNST at a younger age than those with no family history (8.3% vs. 0.5% P=0.003 and 13.9% vs. 2.4% P=0.003, for onset before 10 and 20, respectively). In the MPNST population with a known family history, onset prior to age 10 was significantly more prevalent (42.9% vs. 7% P=0.029). CONCLUSIONS: These results suggest a positive family history of MPNST represents a risk factor for the development and early onset of MPNST in individuals with NF-1.

Virtual modeling, stereolithography, and intraoperative CT guidance for the optimization of sagittal synostosis reconstruction: a technical note.

INTRODUCTION: Sagittal synostosis affects 1 in 1000 live births and may result in increased intracranial pressure, hindrance of normal neural development, and cosmetic deformity due to scaphocephaly. Historically, several approaches have been utilized for surgical correction and recently, computed tomography (CT)-guided reconstruction procedures are increasingly used. In this report, the authors describe the use of a CT-derived virtual and stereolithographic (3D printed) craniofacial models, which are used to guide intraoperative bone placement, and intraoperative CT guidance for confirmation of bone placement, to ensure the accuracy of surgical correction of scaphocephaly, as demonstrated to parents. METHODS: Preoperative high-resolution CT imaging was used to construct 3D image sets of the skulls of two infants (a 14-month-old female and a 6-month-old male) with scaphocephaly. These 3D image sets were then used to create a virtual model of the proposed surgical correction for each of the infants' deformities, which was then printed and made available for use intraoperatively to plan the bone flap, fashion the bone cuts, and optimize graft placement. After the remodeling, adherence to the preoperative plan was assessed by overlaying a CT scan of the remodeled skull with the virtual model. Deviations from the preoperative model were noted. RESULTS: Both patients had excellent postoperative cosmetic correction of head shape and contouring. The mean operative time was 5 h, blood loss was 100 ml, and one child required modification of the subocciput after intraoperative imaging showed a deviation of the reconstruction from the surgical goal as depicted by the preoperative model. CONCLUSION: The addition of neuro-navigation to stereolithographic modeling ensured the accuracy of the reconstruction for our patients and provided greater confidence to both surgeons and parents. While unisutural cases are presented for clarity, correction was still required for one patient. The cost of the models and the additional CT required must be weighed against the complexity of the procedure and possibly reserved for patients with potentially complicated corrections.

Diffusion tensor imaging and ventricle volume quantification in patients with chronic shunt-treated hydrocephalus: a matched case-control study.

OBJECTIVEThe object of this study was to use diffusion tensor imaging (DTI) and tract-based spatial statistics (TBSS) to characterize the long-term effects of hydrocephalus and shunting on white matter integrity and to investigate the relationship of ventricular size and alterations in white matter integrity with headache and quality-of-life outcome measures.METHODSPatients with shunt-treated hydrocephalus and age- and sex-matched healthy controls were recruited into the study and underwent anatomical and DTI imaging on a 3-T MRI scanner. All patients were clinically stable, had undergone CSF shunt placement before 2 years of age, and had a documented history of complaints of headaches. Outcome was scored based on the Headache Disability Inventory and the Hydrocephalus Outcome Questionnaire. Fractional anisotropy (FA) and other DTI-based measures (axial, radial, and mean diffusivity; AD, RD, and MD, respectively) were extracted in the corpus callosum and internal capsule with manual region-of-interest delineation and in other regions with TBSS. Paired t-tests, corrected with a 5% false discovery rate, were used to identify regions with significant differences between patients and controls. Within the patient group, linear regression models were used to investigate the relationship between FA or ventricular volume and outcome, as well as the effect of shunt-related covariates.RESULTSTwenty-one hydrocephalus patients and 21 matched controls completed the study, and their data were used in the final analysis. The authors found significantly lower FA for patients than for controls in 20 of the 48 regions, mostly posterior white matter structures, in periventricular as well as more distal tracts. Of these 20 regions, 17 demonstrated increased RD, while only 5 showed increased MD and 3 showed decreased AD. No areas of increased FA were observed. Higher FA in specific periventricular white matter tracts, tending toward FA in controls, was associated with increased ventricular size, as well as improved clinical outcome.CONCLUSIONSThe study shows that TBSS-based DTI is a sensitive technique for elucidating changes in white matter structures due to hydrocephalus and chronic CSF shunting and provides preliminary evidence that DTI may be a valuable tool for tailoring shunt procedures to monitor ventricular size following shunting and achieve optimal outcome, as well as for guiding the development of alternate therapies for hydrocephalus.

Incidental choroid plexus papilloma in a child: A difficult decision.

BACKGROUND: Choroid plexus tumors (CPT) in the pediatric population are usually discovered in symptomatic patients often with symptoms of increased intracranial pressure, with hydrocephalus as the most common presentation, along with seizures, subarachnoid hemorrhage, or focal neurological deficit. Most CPTs are found to be benign choroid plexus papillomas (CPP), whereas a small number are intermediate and malignant choroid plexus carcinomas (CPC). Total surgical resection is the established definitive treatment for symptomatic CPP. CASE DESCRIPTION: We describe a young female who was found to have an incidental CPT during workup for recent head trauma without neurological deficits or hydrocephalus. She underwent a surgical operation to remove the tumor successful, with 1-year follow-up showing no recurrence and normal developmental milestones. CONCLUSION: This rare presentation of an asymptomatic CPT brings attention to the fact that there is no clear evidence for how or when to treat such patients. Because discovery of a CPT in an asymptomatic patient is uncommon, the treatment plan appears to be developed on a case-by-case basis. We hope to generate discussion for establishing an agreed upon treatment approach for CPTs in asymptomatic patients.

Evaluation of community pharmacist ability to ensure the safe use of oral anticancer agents: a nationwide survey in Japan.

OBJECTIVES: A recent study of community pharmacists in Canada reported that they required additional education. We conducted a survey of community pharmacists to evaluate the adequacy of education and training in oral anticancer agents in Japan. METHODS: Between May and June 2014, community pharmacists were asked to complete a questionnaire by using two different survey strategies, one online and one via postal mail. RESULTS: Three hundred community pharmacists responded to an online survey and 283 community pharmacists responded to a mailed survey. Only 6-10% of respondents felt that they had received adequate education in oncology or oral chemotherapy. Although 81% of Japanese pharmacists had attended at least one continuing education event related to oncology in the past 2 years, only 54% felt comfortable dispensing oral anticancer agents and only 40% felt comfortable educating patients about oral chemotherapy. In a multivariate analysis, confidence in educating patients about oral chemotherapy was associated with an understanding of chemotherapy cycles and doses (odds ratio = 4.89, 95% confidence interval [2.53-9.45]) and the number of continuing education events they had attended (odds ratio = 1.67, 95% confidence interval [1.35-2.08]). CONCLUSIONS: This is the first report to evaluate whether community pharmacists are equipped to ensure the safe use of oral anticancer agents in Japan. The results are similar to those previously reported for Canadian pharmacists, namely a low rate of positive responses for education in oncology and oral chemotherapy, demonstrating a similar need for additional education and training in oral chemotherapy.

Prenatally Diagnosed Aggressive Intracranial Immature Teratoma-Clinicopathological Correlation.

OBJECTIVE: To describe clinicopathological correlation of congenital intracranial immature teratoma. METHODS: A retrospective case analysis from a tertiary medical center. RESULTS: We report a case of an intracranial immature teratoma detected prenatally at 35 weeks of gestation. The tumor showed rapid growth, causing acute hydrocephalus requiring subsequent ventriculoperitoneal shunting. Resective surgery was performed within 2 weeks after birth. The infant died at day of life 29. Histological examination revealed an immature teratoma, with high MIB1/Ki-67 proliferation index. CONCLUSION/IMPLICATIONS: Intracranial immature teratoma with high MIB1/Ki-67 proliferation index may serve as an independent poor prognostic factor.

Use of a customized 3D "basket" to create a solitary split-thickness cranial graft from numerous split fragments in an infant.

While autologous split calvaria remains the preferred material for use in pediatric cranioplasty, it may be difficult to split the bone neatly into two distinct pieces, especially in infants and young children. In this paper, the authors present a technique in which numerous split pieces of bone can be readily joined together and conformed to the shape of the specific defect using a customized template and 3D trellis-like basket.

Outcomes assessment of a pharmacist-directed seamless care program in an ambulatory oncology clinic.

The primary goal of seamless care is improved patient outcomes and improved standards of care for patients with cancer. The pharmacy service of the Newfoundland Cancer Treatment and Research Foundation conducted a randomized control study that measured clinical and humanistic outcomes of a pharmacist-directed seamless care program in an ambulatory oncology clinic. This article focuses on the intervention group, particularly the identification of drug-related problems (DRPs) and utilization of health care services as well the satisfaction of 3 types of health professionals with the services provided by the pharmacist-directed seamless care program. Overall, the seamless care pharmacist (SCP) identified an average of 3.7 DRPs per intervention patient; the most common DRP reported was a patient not receiving or taking a drug therapy for which there is an indication. The SCP identified more DRPs in patients receiving adjuvant treatment compared to those receiving palliative treatment. On average, family physicians, oncology nurses, and hospital pharmacists were satisfied with the SCP intervention indicating that they agreed the information collected and distributed by the SCP was useful to them. Pharmacist-directed seamless care services in an ambulatory oncology clinic have a significant impact on clinical outcomes and processes of patient care. The presence of a SCP can help identify and resolve DRPs experienced by patients in an outpatient oncology clinic, ensuring that patients are receiving the highest standard of care.

Misdiagnosing recurrent medulloblastoma: the danger of examination and imaging without histological confirmation.

The screening and detection of recurrent medulloblastoma presents the clinician with significant diagnostic challenges, including the risk of misdiagnosis. The authors present the case of a young girl with a history of a treated standard-risk medulloblastoma that highlights the risk of assuming recurrence has occurred when clinical and/or imaging changes are observed. This girl developed both new clinical deficits and had radiographic evidence of recurrence. She subsequently experienced a complete resolution of symptoms and radiographic findings with steroids alone.

Are community pharmacists equipped to ensure the safe use of oral anticancer therapy in the community setting? Results of a cross-country survey of community pharmacists in Canada.

PURPOSE: Oral anticancer agents offer significant benefits over parenteral anticancer therapy in terms of patient convenience and reduced intrusiveness. Oral anticancer agents give many cancer patients freedom from numerous hospital visits, allowing them to obtain their medications from their local community pharmacy. However, a major concern with increased use of oral anticancer agents is shift of responsibility in ensuring the proper use of anticancer agents from the hospital/clinical oncology team to the patient/caregiver and other healthcare providers such as the community pharmacists who may not be appropriately trained for this. This study assessed the readiness of community pharmacists across Canada to play this increased role with respect to oral anticancer agents. METHODS: Using a structured electronic mailing strategy, a standardized survey was mailed to practicing pharmacists in five provinces where community pharmacists were dispensing the majority of oral anticancer agents. In addition to collecting basic demographic and their practice setting, the survey assessed the pharmacists' knowledge regarding cancer therapy and oral anticancer agents in particular, their education needs and access to resources on oral anticancer agents, the quality of prescriptions for oral anticancer agents received by them in terms of the required elements, their role in patient education, and steps to enhance patient and personal safety. RESULTS: There were 352 responses to the survey. Only 13.6% of respondents felt that they had received adequate oncology education at the undergraduate level and approximately 19% had attended a continuing education event related to oncology in the past 2 years. Only 24% of the pharmacists who responded were familiar with the common doses of oral anticancer agents and only 9% felt comfortable educating patients on these medications. CONCLUSIONS: A substantial portion of community pharmacists in Canada lack a solid understanding of oral anticancer agents and thus are poorly equipped to play a major role in ensuring their appropriate use. More education and training on oral anticancer agents are urgently required.

Craniocerebral disproportion: a topical review and proposal toward a new definition, diagnosis, and treatment protocol.

INTRODUCTION: At some point in their lives, patients previously shunted for hydrocephalus may experience chronic, debilitating headaches, despite the fact that their shunts are functioning properly. Previously published reports have suggested that a subset of these patients may be suffering from an iatrogenic craniocerebral disproportion (CCD) and, therefore, may benefit from procedures that expand the available intracranial space. A unified definition of this disorder, however, is lacking. DISCUSSION: Here, the authors chart the history (including historical terminology) of CCD, review its incidence, describe its signs, symptoms, and associated radiologic findings, and expound upon its pathophysiology. Next, a concise clinical definition of CCD based on the temporal correlation of headaches with the appearance of plateau waves on intracranial pressure (ICP) monitoring is proposed. The authors conclude with a discussion of the various therapeutic strategies employed previously to treat this disorder and present their individualized treatment strategy based upon the simultaneous utilization of ICP monitors and gradual external cranial vault expansion.

Successful treatment of post-shunt craniocerebral disproportion by coupling gradual external cranial vault distraction with continuous intracranial pressure monitoring.

A subset of hydrocephalic patients in whom shunts are placed at an early age will develop craniocerebral disproportion (CCD), an iatrogenic mismatch between the fixed intracranial volume and the growing brain. The lack of a reliable, reproducible method to diagnose this condition, however, has hampered attempts to treat it appropriately. For those practitioners who acknowledge the need to create more intracranial space in these patients, the lack of agreed-upon therapeutic end points for cranial vault expansion has limited the use of such techniques and has sometimes led to problems of underexpansion. Here, the authors present a definition of CCD based primarily on the temporal correlation of plateau waves on intracranial pressure (ICP) monitoring and headache exacerbation. The authors describe a technique of exploiting continued ICP monitoring during progressive cranial expansion in which the goal of distraction is the cessation of plateau waves. Previously encountered problems of underexpansion may be mitigated through the simultaneous use of ICP monitors and gradual cranial expansion over time.