Laura-Nanna Lohkamp

"Challenges and opportunities in neurosurgical care transition" published on 01 Aug 2024 by American Association of Neurological Surgeons.

"Introduction. Transitional care in neurosurgery" published on 01 Aug 2024 by American Association of Neurological Surgeons.

Craniopharyngioma (CP) represent 1.2-4.6% of all intracranial tumors in children and carry a significant morbidity due to their lesional intimacy with structures involved in neurological, visual, and endocrinological functions. Variable treatment modalities being available, including surgery, radiation therapy, alternative surgeries, and intracystic therapies or combinations of them, their common goal is to reduce immediate and long-term morbidity while preserving these functions. Multiple attempts have been made to re-evaluate surgical and irradiation strategies in order to optimize their complication and morbidity profile. However, despite significant advances in “function sparing” approaches, such as limited surgery and improved technologies of radiation therapies, achieving interdisciplinary consensus on the optimal treatment algorithm remains a challenge. Furthermore, there remains a significant span of improvement given the number of specialties involved as well as the complex and chronic nature of CP disease. This perspective article aims to summarize recent changes and knowledge gains in the field of pediatric CP, outlining updated treatment recommendations, a concept of integrative interdisciplinary care and the implication of novel potential diagnostic tools. A comprehensive update on the multimodal treatment of pediatric CP is presented, focusing on “function-preserving” therapies and their implications.

BACKGROUND: Transorbital penetrating head injuries (PHIs) are uncommon but can lead to substantial deficits, depending on intracranial involvement and the neuroanatomical structures affected. Complete recovery after such injuries is rare. OBSERVATIONS: A 7-year-old boy sustained a PHI when he fell onto a garden spike while climbing a fence. Initial imaging showed an orbital roof fracture, focal subarachnoid hemorrhage, and an intraparenchymal hemorrhage in the right frontal lobe with associated linear tract extending to the contralateral superior temporal gyrus. Relevant neuroanatomical structures, including the anterior cerebral arteries (ACAs) and the basal ganglia, were spared. This is in keeping with superior transorbital PHI caused by a garden spike, which had transgressed the skull entering from the right superior orbit. Clinically, he experienced some transient right-sided weakness and mild speech disturbance. Some questionable vasospasm of the ACAs observed on interim magnetic resonance imaging was absent in a repeat imaging study, followed by an unremarkable radiographic follow-up at 6 months after injury. At 18 months after injury, he is neurologically intact without deficit. LESSONS: Most PHIs bear serious lifelong consequences, but here was a case of a deep, penetrating object that managed to avoid all significant neuroanatomical pathways, leading to complete recovery in follow-up.

Purpose Congenital cervicothoracic dissociation (CCTD) of the spine is a rare condition while having major impacts on stability and neurological function. Surgical treatment includes decompression and instrumented fusion. Only few cases of CCTD have been reported in children. This report intends to demonstrate the complexity of this condition and its surgical management options based on two cases. Methods Retrospective illustration of two cases with CCTD treated with instrumented occipito-thoracic fusion. Timing and options of surgical management are discussed. Results Two patients aged 9 and 12 were treated. Patient 1 presented with asymmetric chronic weakness of the upper extremity and unstable neurogenic bladder. Imaging showed a CCTD with severe distortion of the posterior facets and a dysplastic spinal cord. Patient 2 presented with mild spasticity and unilateral weakness of the upper extremity. A low energy trauma resulted in tetraplegia by increasing the amount of antero-posterior displacement. Both patients were treated with Halo vest application, followed by posterior decompression, and instrumented occipito-thoracic fusion. Additional upfront Halo traction was applied in patient 2 for distraction and sagittal alignment improvement. The last follow-up confirmed solid fusion and minor residual neurological impairment in both patients at 5, and 6 years, respectively. Conclusion CCTD is a rare congenital condition, which can be successfully managed by posterior instrumented fusion. A sudden cervicothoracic displacement with neurological deterioration may respond to careful Halo traction with preoperative sagittal alignment correction. Timing of surgery depends on the actual instability in neurologically stable patients.

Introduction Children with craniopharyngiomas (CP) can experience significant morbidities caused by extensive surgery and/or radiation. Ommaya reservoir insertion (ORI) into cystic CP represents a minimally invasive approach allowing immediate decompression and aims to avoid additional injuries. The purpose of this study was to determine the surgical outcome and relevance of upfront ORI (± intracystic treatment) for preservation of endocrine function. Methods We performed a retrospective chart review of children with CP treated at the Hospital for Sick Children between 01/01/2000 and 15/01/2020. Endocrine function was reviewed at the time of initial surgery and throughout follow-up. New endocrinological deficits related to the index procedure were defined as immediate failure (IF), whereas postoperative duration of endocrinological stability (ES) was analyzed using the Kaplan-Meier method. The rate of IF and ES was compared between the treatment groups. Results Seventy-nine patients were included and had a median age of 8.3 years (range 2.1–18.0 years); 31 were males. Fifty-three patients with upfront surgical treatment, including 29 ORI and 24 gross total or partial resections had sufficient endocrinological follow-up data. Endocrine dysfunction occurring immediately after the index procedure (IF) was observed in 15 patients (62.5%) in the resection group compared to two patients (6.8%) in the ORI group, odds ratio: 0.05 (CI: 0.01–0.26, p < 0.0001). Excluding those with immediate endocrinological deficits, mean ES after ORI was 19.4 months (CI: 11.6–34.2), compared to 13.4 months (CI:10.6-NA) after surgical resection. Conclusions Endocrine function was preserved in patients with upfront ORI (± intracystic treatment), which was confirmed as a minimally invasive procedure with an overall low morbidity profile.

Introduction: Neurofibromatosis type 1 (NF1) has an incidence of 1 in 2,000 to 3,000 individuals and in 15% is associated with optic pathway glioma (OPG). Given the variability in clinical presentation and related morbidity, a multidisciplinary approach for diagnosis and management of children with NF1 and OPG is required, but often lacks coordination and regular information exchange. Herein we summarize our experience and describe the care pathways/network provided by a multidisciplinary team. The role of the distinct team members is elucidated as well as the care amendments made over time. Methods: We performed a retrospective single-center observational study, including children treated at our institution between 1990 and 2021. Inclusion criteria were clinical diagnosis of NF1, radiographic and/or histopathological diagnosis of OPG and age below 18 years. Patients being treated elsewhere were excluded from the study. Data was abstracted from each child’s health record using a standardized data collection form. Characteristics of children with NF1 and OPG were described using means (SD) and percentages. Outcomes were determined using Kaplan-Meier estimates. Results: From 1990 to 2021, 1,337 children were followed in our institution. Of those, 195 were diagnosed with OPG (14.6%), including 94 (48.21%) females and 101 (51.79%) males. Comprehensive data were available in 150 patients. The mean (SD) age at diagnosis was 5.31(4.08) years (range: 0.8–17.04 years). Sixty-two (41.3%) patients remained stable and did not undergo treatment, whereas 88 (58.7%) patients required at least one treatment. The mean (SD) duration of follow up was 8.14 (5.46) years (range: 0.1–25.9 years; median 6.8 years). Overall survival was of 23.6 years (±1.08), comprising 5 deaths. A dedicated NF clinic, including pediatricians and a nurse, provides regular follow up and plays a central role in the management of children with NF1, identifying those at risk of OPG, coordinating referrals to Neuroradiology and other specialists as indicated. All children are assessed annually by Ophthalmology. Comprehensive care was provided by a multidisciplinary team consisting of Dermatology, Genetics, Neuro-oncology, Neuroradiology, Neurosurgery, Ophthalmology and Pediatrics. Conclusions: The care of children with NF1 and OPG is optimized with a multidisciplinary team approach, coordinated by a central specialty clinic.

Study Design: Systematic review. Objective: Ehlers-Danlos Syndrome (EDS) comprises a spectrum of connective tissue disorders, which may be associated with cranio-cervical instability (CCI). There is a lack of consensus on diagnostic imaging parameters, indications, and outcomes of surgical treatment. Methods: This systematic review analyses the literature on diagnostic methods and/or criteria for CCI, screening the databases Ovid Medline, Embase, Cochrane Library, and PubMed. Articles were included based on the PRISMA guidelines and assessed using the Newcastle-Ottawa Quality Assessment Scale (NOS) and according to their evidence level. Results: Sixteen articles, including 78 surgical patients, met the inclusion criteria. The main diagnostic measures for CCI were dynamic x-rays and CT imaging. Ten different radiographic parameters were reported, of which 4 were the most frequently applied for surgical decision-making: the clivo-axial angle (CXA), the Harris measurement, the Grabb–Mapstone–Oakes measurement, and the angular displacement of C1 to C2. The evidence level ranged between III and V and the article quality between 4 and 8 out of 9 stars on the NOS Scale. Conclusions: There is a lack of high quality, prospective evidence regarding the evaluation of suspected CCI in patients with EDS. Based on our systematic review, we recommend that the CXA, Harris measurement, Grabb–Mapstone–Oakes measurement, and the angular displacement of C1 to C2 be used to evaluate suspected CCI in EDS patients. Surgical fixation of suspected CCI should only be performed in cases with clear radiographic presence of instability and concordant symptoms/signs. Consensus-based guidelines and care pathways are required.

Background: CyberKnife-based robotic radiosurgery (RRS) is a widely used treatment modality for various benign and malignant tumors of the central nervous system (CNS) in adults due to its high precision, favorable safety profile, and efficacy. Although RRS is emerging in pediatric neuro-oncology, scientific evidence for treatment indications, treatment parameters, and patient outcomes is scarce. This systematic review summarizes the current experience and evidence for RRS and robotic stereotactic radiotherapy (RSRT) in pediatric neuro-oncology. Methods: We performed a systematic review based on the databases Ovid Medline, Embase, Cochrane Library, and PubMed to identify studies and published articles reporting on RRS and RSRT treatments in pediatric neuro-oncology. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were applied herein. Articles were included if they described the application of RRS and RSRT in pediatric neuro-oncological patients. The quality of the articles was assessed based on their evidence level and their risk for bias using the original as well as an adapted version of the Newcastle Ottawa Quality Assessment Scale (NOS). Only articles published until 1 August 2021, were included. Results: A total of 23 articles were included after final review and removal of duplicates. Articles reported on a broad variety of CNS entities with various treatment indications. A majority of publications lacked substantial sample sizes and a prospective study design. Several reports included adult patients, thereby limiting the possibility of data extraction and analysis of pediatric patients. RRS and RSRT were mostly used in the setting of adjuvant, palliative, and salvage treatments with decent local control rates and acceptable short-to-intermediate-term toxicity. However, follow-up durations were limited. The evidence level was IV for all studies; the NOS score ranged between four and six, while the overall risk of bias was moderate to low. Conclusion: Publications on RRS and RSRT and their application in pediatric neuro-oncology are rare and lack high-quality evidence with respect to entity-related treatment standards and long-term outcomes. The limited data suggest that RRS and RSRT could be efficient treatment modalities, especially for children who are unsuitable for surgical interventions, suffer from tumor recurrences, or require palliative treatments. Nevertheless, the potential short-term and long-term adverse events must be kept in mind when choosing such a treatment. Prospective studies are necessary to determine the actual utility of RRS and RSRT in pediatric neuro-oncology.

Study Design: Narrative Review. Objectives: The objective of this review is to provide a stepwise approach to the assessment of patients with potential symptoms of degenerative cervical myelopathy (DCM). Methods: N/A Results: DCM is an overarching term used to describe progressive compression of the cervical spinal cord by age-related changes to the spinal axis. These alterations to normal anatomy narrow the spinal canal, reduce the space available for the spinal cord, and may ultimately compress the ascending and descending neural tracts. Patients with DCM present with a wide range of symptoms that can significantly impact quality of life, including bilateral hand numbness and paresthesia, gait impairment, motor weakness of the upper and lower extremities, and bladder and bowel dysfunction. Unfortunately, DCM is often misdiagnosed, resulting in delayed assessment and management by the appropriate specialist. The proper evaluation of a patient with suspected DCM includes obtaining a detailed patient history, conducting a comprehensive neurological examination, and ordering appropriate tests to rule in or out other diagnoses. Conclusion: This review summarizes a stepwise approach to the diagnosis of patients with DCM.