Cost-effectiveness of surgery for degenerative cervical myelopathy in the United Kingdom.

PURPOSE: Degenerative cervical myelopathy (DCM) is the commonest cause of adult spinal cord dysfunction worldwide, for which surgery is the mainstay of treatment. At present, there is limited literature on the costs associated with the surgical management of DCM, and none from the United Kingdom (UK). This study aimed to evaluate the cost-effectiveness of DCM surgery within the National Health Service, UK. MATERIALS AND METHODS: Incidence of DCM was identified from the Hospital Episode Statistics (HES) database for a single year using five ICD-10 diagnostic codes to represent DCM. Health Resource Group (HRG) data was used to estimate the mean incremental surgery (treatment) costs compared to non-surgical care, and the incremental effect (quality adjusted life year (QALY) gain) was based on data from a previous study. A cost per QALY value of <£30,000/QALY (GBP) was considered acceptable and cost-effective, as per the National Institute for Health and Clinical Excellence (NICE) guidance. A sensitivity analysis was undertaken (±5%, ±10% and ±20%) to account for variance in both the cost of admission and QALY gain. RESULTS: The total number of admissions for DCM in 2018 was 4,218. Mean age was 62 years, with 54% of admissions being of working age (18-65 years). The overall estimated cost of admissions for DCM was £38,871,534 for the year. The mean incremental (per patient) cost of surgical management of DCM was estimated to be £9,216 (ranged £2,358 to £9,304), with a QALY gain of 0.64, giving an estimated cost per QALY value of £14,399/QALY. Varying the QALY gain by ±20%, resulted in cost/QALY figures between £12,000 (+20%) and £17,999 (-20%). CONCLUSIONS: Surgery is estimated to be a cost-effective treatment of DCM amongst the UK population.

Toward Shared Decision-Making in Degenerative Cervical Myelopathy: Protocol for a Mixed Methods Study.

BACKGROUND: Health care decisions are a critical determinant in the evolution of chronic illness. In shared decision-making (SDM), patients and clinicians work collaboratively to reach evidence-based health decisions that align with individual circumstances, values, and preferences. This personalized approach to clinical care likely has substantial benefits in the oversight of degenerative cervical myelopathy (DCM), a type of nontraumatic spinal cord injury. Its chronicity, heterogeneous clinical presentation, complex management, and variable disease course engenders an imperative for a patient-centric approach that accounts for each patient's unique needs and priorities. Inadequate patient knowledge about the condition and an incomplete understanding of the critical decision points that arise during the course of care currently hinder the fruitful participation of health care providers and patients in SDM. This study protocol presents the rationale for deploying SDM for DCM and delineates the groundwork required to achieve this. OBJECTIVE: The study's primary outcome is the development of a comprehensive checklist to be implemented upon diagnosis that provides patients with essential information necessary to support their informed decision-making. This is known as a core information set (CIS). The secondary outcome is the creation of a detailed process map that provides a diagrammatic representation of the global care workflows and cognitive processes involved in DCM care. Characterizing the critical decision points along a patient's journey will allow for an effective exploration of SDM tools for routine clinical practice to enhance patient-centered care and improve clinical outcomes. METHODS: Both CISs and process maps are coproduced iteratively through a collaborative process involving the input and consensus of key stakeholders. This will be facilitated by Myelopathy.org, a global DCM charity, through its Research Objectives and Common Data Elements for Degenerative Cervical Myelopathy community. To develop the CIS, a 3-round, web-based Delphi process will be used, starting with a baseline list of information items derived from a recent scoping review of educational materials in DCM, patient interviews, and a qualitative survey of professionals. A priori criteria for achieving consensus are specified. The process map will be developed iteratively using semistructured interviews with patients and professionals and validated by key stakeholders. RESULTS: Recruitment for the Delphi consensus study began in April 2023. The pilot-testing of process map interview participants started simultaneously, with the formulation of an initial baseline map underway. CONCLUSIONS: This protocol marks the first attempt to provide a starting point for investigating SDM in DCM. The primary work centers on developing an educational tool for use in diagnosis to enable enhanced onward decision-making. The wider objective is to aid stakeholders in developing SDM tools by identifying critical decision junctures in DCM care. Through these approaches, we aim to provide an exhaustive launchpad for formulating SDM tools in the wider DCM community. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/46809.

Presentation, management, and outcomes of cauda equina syndrome up to one year after surgery, using clinician and participant reporting: A multi-centre prospective cohort study.

Background: Cauda equina syndrome (CES) results from nerve root compression in the lumbosacral spine, usually due to a prolapsed intervertebral disc. Evidence for management of CES is limited by its infrequent occurrence and lack of standardised clinical definitions and outcome measures. Methods: This is a prospective multi-centre observational cohort study of adults with CES in the UK. We assessed presentation, investigation, management, and all Core Outcome Set domains up to one year post-operatively using clinician and participant reporting. Univariable and multivariable associations with the Oswestry Disability Index (ODI) and urinary outcomes were investigated. Findings: In 621 participants with CES, catheterisation for urinary retention was required pre-operatively in 31% (191/615). At discharge, only 13% (78/616) required a catheter. Median time to surgery from symptom onset was 3 days (IQR:1-8) with 32% (175/545) undergoing surgery within 48 h. Earlier surgery was associated with catheterisation (OR:2.2, 95%CI:1.5-3.3) but not with admission ODI or radiological compression. In multivariable analyses catheter requirement at discharge was associated with pre-operative catheterisation (OR:10.6, 95%CI:5.8-20.4) and one-year ODI was associated with presentation ODI (r = 0.3, 95%CI:0.2-0.4), but neither outcome was associated with time to surgery or radiological compression. Additional healthcare services were required by 65% (320/490) during one year follow up. Interpretation: Post-operative functional improvement occurred even in those presenting with urinary retention. There was no association between outcomes and time to surgery in this observational study. Significant healthcare needs remained post-operatively. Funding: DCN Endowment Fund funded study administration. Castor EDC provided database use. No other study funding was received.

Iatrogenic post-surgical tension pneumosyrinx-a first reported case.

We describe the first ever-reported occurrence of a post-operative tension pneumosyrinx occurring after a resection of an intradural intramedullary spinal tumour in a 40-year-old patient. Post-operatively, he developed sudden onset paraplegia and imaging revealed a tension pneumosyrinx which was subsequently surgically decompressed. He made a gradual neurological recovery. This is an extremely rare complication with potentially long-lasting deleterious effects on patients' neurological status if not recognized. We aim to bring this pathology to the attention of our neurosurgical colleagues and share our surgical approach and management to assist those who may encounter this pathology in the future.

Epidemiological trends in COVID-19 pandemic: prospective critical appraisal of observations from six countries in Europe and the USA.

INTRODUCTION: Europe was the epicentre of the COVID-19 pandemic in March 2020, with the highest number of cases and deaths between March and April. In May, the infection numbers registered a fall followed by a second new rise, not proportionally reflected by an increase in the number of deaths. We aimed to investigate the relationship between disease prevalence and infection fatality rate (IFR), and the number of intensive care unit (ICU) and hospital admissions over time, to develop a predictive model, as well as appraising the potential contributing factors underpinning this complex relationship. METHODS: A prospective epidemiological study using data from six countries collected between 10 March and 4 September 2020. Data on the number of daily hospital and ICU admissions with COVID-19 were gathered, and the IFR and the prevalence were calculated. Trends over time were analysed. A linear regression model was used to determine the association between the fatality rates and the number of admissions. FINDINGS: The prediction model confirmed the linear association between the fatality rates and the numbers of ICU and hospital admissions. The exception was during the peak of the COVID-19 pandemic when the model underestimated the fatalities indicating that a substantial number of deaths occurred outside of the hospitals. The fatality rates decreased in all countries from May until September regardless of the trends in prevalence, differences in healthcare systems or strategic variations in handling the pandemic. INTERPRETATION: The observed gradual reduction in COVID-19 fatality rates over time despite varying disease prevalence and public health measures across multiple countries warrants search for a biological explanation. While our understanding of this novel virus grows, hospital and ICU admission rates remain effective predictors of patient outcomes which can be used as early warning signs for escalation of public health measures.

Low-Grade Glioma Case Series: Lessons Learned from an Evolving 10-Year Single-Institution Multidisciplinary Team Practice.

BACKGROUND: Low-grade gliomas are a heterogeneous group with significant changes in their management during the last decade. OBJECTIVE: To assess how our multidisciplinary team approach to the management of low-grade glioma has evolved over the past 10 years and its implications for outcomes. METHODS: Retrospective single-center cohort study of adult patients with a pathologically confirmed diagnosis of World Health Organization grade II glioma between 2009 and 2018. Demographic, clinical, and pathologic data were collected. RESULTS: Ninety-five patients were included. There was a statistically significant difference in the surgical approach, with more patients having gross total resection (45.7% vs. 18.4%) and fewer patients having a biopsy (21.8% vs. 49.0%) (P = 0.002) after 2014. There was a significantly better overall survival after 2014 (<2014, 16.3%; ≥2014, 0 deaths; P = 0.010) measured at the mean time of follow-up. The use of adjuvant chemotherapy (P = 0.045) and radiotherapy (P = 0.001) significantly decreased after 2014. A subgroup analysis showed that the impact of extent of surgical resection was the greatest for survival in the 1p19q noncodeleted tumors (P = 0.029) and for seizure outcomes in the 1p19q codeleted group (P = 0.018). There was no statistically significant increase in neurologic disability with more radical surgery, incorporating intraoperative neuromonitoring, as measured by modified Rankin Scale score (P > 0.05). CONCLUSIONS: More radical surgery was associated with increased survival, less need for postoperative adjuvant therapy and better seizure control, without significant morbidity. Molecular markers are useful tools for stratification of benefits after such surgery.

Neurosurgery and coronavirus: impact and challenges-lessons learnt from the first wave of a global pandemic.

INTRODUCTION AND OBJECTIVES: The novel severe acute respiratory syndrome coronavirus 2 (COVID-19) pandemic has had drastic effects on global healthcare with the UK amongst the countries most severely impacted. The aim of this study was to examine how COVID-19 challenged the neurosurgical delivery of care in a busy tertiary unit serving a socio-economically diverse population. METHODS: A prospective single-centre cohort study including all patients referred to the acute neurosurgical service or the subspecialty multidisciplinary teams (MDT) as well as all emergency and elective admissions during COVID-19 (18th March 2020-15th May 2020) compared to pre-COVID-19 (18th of January 2020-17th March 2020). Data on demographics, diagnosis, operation, and treatment recommendation/outcome were collected and analysed. RESULTS: Overall, there was a reduction in neurosurgical emergency referrals by 33.6% and operations by 55.6% during the course of COVID-19. There was a significant increase in the proportion of emergency operations performed during COVID-19 (75.2% of total, n=155) when compared to pre-COVID-19 (n = 198, 43.7% of total, p < 0.00001). In contrast to other published series, the 30-day perioperative mortality remained low (2.0%) with the majority of post-operative COVID-19-infected patients (n = 13) having underlying medical co-morbidities and/or suffering from post-operative complications. CONCLUSION: The capacity to safely treat patients requiring urgent or emergency neurosurgical care was maintained at all times. Strategies adopted to enable this included proactively approaching the referrers to maintain lines of communications, incorporating modern technology to run clinics and MDTs, restructuring patient pathways/facilities, and initiating the delivery of NHS care within private sector hospitals. Through this multi-modal approach we were able to minimize service disruptions, the complications, and mortality.

Altered Motor Excitability in Patients With Diffuse Gliomas Involving Motor Eloquent Areas: The Impact of Tumor Grading.

BACKGROUND: Diffuse gliomas have an increased biological aggressiveness across the World Health Organization (WHO) grading system. The implications of glioma grading on the primary motor cortex (M1)-corticospinal tract (CST) excitability is unknown. OBJECTIVE: To assess the excitability of the motor pathway with navigated transcranial magnetic stimulation (nTMS). METHODS: Retrospective cohort study of patients admitted for surgery with diffuse gliomas within motor eloquent areas. Demographic, clinical, and nTMS-related variables were collected. The Cortical Excitability Score (CES 0 to 2 according to the number of abnormal interhemispheric resting motor threshold (RMT) ratios) was calculated for patients where bilateral upper and lower limb mapping was performed. RESULTS: A total of 45 patients were included: 9 patients had a low-grade glioma and 36 patients had a high-grade glioma. The unadjusted analysis revealed an increase in the latency of the motor evoked potential of the lower limb with an increase of the WHO grade (P = .038). The adjusted analysis confirmed this finding (P = .013) and showed a relation between the increase in the WHO and a decreased RMT (P = .037) of the motor evoked responses in the lower limb. When CES was calculated, an increase in the score was related with an increase in the WHO grade (unadjusted analysis-P = .0001; adjusted analysis-P = .001) and in isocitrate dehydrogenase (IDH) wild-type tumors (unadjusted analysis-P = .020). CONCLUSION: An increase in the WHO grading system and IDH wild-type tumors are associated with an abnormal excitability of the motor eloquent areas in patients with diffuse gliomas.

Management evaluation of metastasis in the brain (MEMBRAIN)-a United Kingdom and Ireland prospective, multicenter observational study.

BACKGROUND: In recent years an increasing number of patients with cerebral metastasis (CM) have been referred to the neuro-oncology multidisciplinary team (NMDT). Our aim was to obtain a national picture of CM referrals to assess referral volume and quality and factors affecting NMDT decision making. METHODS: A prospective multicenter cohort study including all adult patients referred to NMDT with 1 or more CM was conducted. Data were collected in neurosurgical units from November 2017 to February 2018. Demographics, primary disease, KPS, imaging, and treatment recommendation were entered into an online database. RESULTS: A total of 1048 patients were analyzed from 24 neurosurgical units. Median age was 65 years (range, 21-93 years) with a median number of 3 referrals (range, 1-17 referrals) per NMDT. The most common primary malignancies were lung (36.5%, n = 383), breast (18.4%, n = 193), and melanoma (12.0%, n = 126). A total of 51.6% (n = 541) of the referrals were for a solitary metastasis and resulted in specialist intervention being offered in 67.5% (n = 365) of cases. A total of 38.2% (n = 186) of patients being referred with multiple CMs were offered specialist treatment. NMDT decision making was associated with number of CMs, age, KPS, primary disease status, and extent of extracranial disease (univariate logistic regression, P < .001) as well as sentinel location and tumor histology (P < .05). A delay in reaching an NMDT decision was identified in 18.6% (n = 195) of cases. CONCLUSIONS: This study demonstrates a changing landscape of metastasis management in the United Kingdom and Ireland, including a trend away from adjuvant whole-brain radiotherapy and specialist intervention being offered to a significant proportion of patients with multiple CMs. Poor quality or incomplete referrals cause delay in NMDT decision making.

Patient-reported experience measures in patients undergoing navigated transcranial magnetic stimulation (nTMS): the introduction of nTMS-PREMs.

BACKGROUND: Patient-reported experience measures (PREMs) are a unique measure of experience of patients which can help address the quality of care of the patients. OBJECTIVE: Our aim of the study is to collect quality of care outcomes with our newly navigated transcranial magnetic stimulation patient-reported experience measure (nTMS-PREMs) questionnaire among neurosurgical patients undergoing nTMS. METHODS: A single-centre prospective nTMS-PREMs 19-item questionnaire study was performed between February 2018 and December 2018 on patient referred for nTMS at our hospital. The Data was analysed using Likert scale, linear and logistic regression using statistical software (STATA 13.0®). RESULTS: Fifty patient questionnaires were collected (30 males, 20 females, mean age of 47.6 ± 2.1 years) among which 74% of patients underwent both motor and language mapping with a mean duration of 103.3 ± 5.1 min. An overall positive response was noted from the results of the questionnaire, tiredness and anxiety being the common effects noted. Patients with the left-sided disease appreciated more the conditions provided in our laboratory (Q4, p = 0.040) and increasing age was related to less confidence and trust (Q6, p = 0.038) in the staff performing the exam. Younger patients tolerated nTMS better than older patients (> 65 years). PubMed literature search resulted in no relevant articles on the use of PREMs in nTMS patients. CONCLUSION: nTMS is a well-tolerated non-invasive tool and nTMS-PREMS provides a promising role in identifying the unmet needs of the patients and improving the quality of their care.

Integrated Approach Reveals Role of Mitochondrial Germ-Line Mutation F18L in Respiratory Chain, Oxidative Alterations, Drug Sensitivity, and Patient Prognosis in Glioblastoma.

Glioblastoma is the most common and malignant primary brain tumour in adults, with a dismal prognosis. This is partly due to considerable inter- and intra-tumour heterogeneity. Changes in the cellular energy-producing mitochondrial respiratory chain complex (MRC) activities are a hallmark of glioblastoma relative to the normal brain, and associate with differential survival outcomes. Targeting MRC complexes with drugs can also facilitate anti-glioblastoma activity. Whether mutations in the mitochondrial DNA (mtDNA) that encode several components of the MRC contribute to these phenomena remains underexplored. We identified a germ-line mtDNA mutation (m. 14798T > C), enriched in glioblastoma relative to healthy controls, that causes an amino acid substitution F18L within the core mtDNA-encoded cytochrome b subunit of MRC complex III. F18L is predicted to alter corresponding complex III activity, and sensitivity to complex III-targeting drugs. This could in turn alter reactive oxygen species (ROS) production, cell behaviour and, consequently, patient outcomes. Here we show that, despite a heterogeneous mitochondrial background in adult glioblastoma patient biopsy-derived cell cultures, the F18L substitution associates with alterations in individual MRC complex activities, in particular a 75% increase in MRC complex II_III activity, and a 34% reduction in CoQ10, the natural substrate for MRC complex III, levels. Downstream characterisation of an F18L-carrier revealed an 87% increase in intra-cellular ROS, an altered cellular distribution of mitochondrial-specific ROS, and a 64% increased sensitivity to clomipramine, a repurposed MRC complex III-targeting drug. In patients, F18L-carriers that received the current standard of care treatment had a poorer prognosis than non-carriers (373 days vs. 415 days, respectively). Single germ-line mitochondrial mutations could predispose individuals to differential prognoses, and sensitivity to mitochondrial targeted drugs. Thus, F18L, which is present in blood could serve as a useful non-invasive biomarker for the stratification of patients into prognostically relevant groups, one of which requires a lower dose of clomipramine to achieve clinical effect, thus minimising side-effects.

Symptomatic Idiopathic Spinal Epidural Lipomatosis in 9 Patients: Clinical, Radiologic, and Pathogenetic Features.

BACKGROUND: Symptomatic spinal epidural lipomatosis (SSEL) is characterized by hypertrophy of adipose tissue within the spinal canal and consequent neural compromise. The exact pathogenesis remains enigmatic. The authors describe a retrospective case series, define the full clinical spectrum, and discuss possible pathogenetic mechanisms. METHODS: The medical notes and imaging of 9 patients with SSEL undergoing surgery from 2008-2018 were analyzed. Seven patients presented secondary to lumbosacral spinal epidural lipomatosis (SEL); 3 patients with chronic incomplete cauda equina syndrome (CES), 3 patients with acute CES (including a 25-week gravid patient and a 40-year-old patient with intravenous leiomyomatosis, both of whom had mild SEL) and 1 patient with chronic lumbar radiculopathy. In addition, 2 patients presented with progressive myelopathy secondary to thoracic SEL. RESULTS: Patients presenting with acute CES had a mean age of 37 years (range 23-49 years) and mean extradural fat (EF)-to-spinal canal (SC) ratio of 47% (range 41%-58%), in comparison with patients with chronic CES; mean age 61 years (range 58-65 years) and EF:SC ratio 72% (range 65%-80%). Patients underwent laminectomy and resection of EF at compressive levels. All patients with CES experienced complete resolution of symptoms at follow-up (range 1-48 months). CONCLUSIONS: The clinician should be astute to the radiologic features of SEL, particularly in patients presenting with CES in the absence of acute disk herniation. The outcome of patients with CES and SEL after surgery is excellent regardless of symptom duration. Venous impedance related to increased body mass index and EF deposition may play the predominant role in addition to mechanical compression in the pathogenesis of SSEL.

First United Kingdom Experience of Navigated Transcranial Magnetic Stimulation in Preoperative Mapping of Brain Tumors.

BACKGROUND: Surgery for lesions in eloquent brain areas remains challenging owing to the risk of permanent neurological deficits. To date, direct electrical stimulation (DES) and intraoperative neuromonitoring represent the reference standard. Recently, navigated transcranial magnetic stimulation (nTMS) has emerged as a mapping tool to optimize surgical planning. Our aim was to correlate nTMS with the intraoperative findings and assess its effects on surgical decision-making. METHODS: We retrospectively reviewed our series of patients who had undergone craniotomy for removal of a brain tumor at our institution with preoperative nTMS, intraoperative neuromonitoring, and DES from February 2017 to February 2018. Demographic data, tumor entity and location, extent of resection, change in surgical approach, and neurological outcome were collected. RESULTS: Of 35 patients, 24 (68.6%) had undergone preoperative motor mapping and 11 (31.4%) had undergone mapping for language. Histopathological examination demonstrated glioma in 88.6% (high grade, n = 24; low grade, n = 6), metastasis (n = 2), epidermoid cyst (n = 1), and cavernoma (n = 1). nTMS resulted in change in the surgical strategy in 31.5% (craniotomy size, n = 7; access pathway, n = 3; surgical indication, n = 1). The specificity of nTMS for language was 66.7%, with a negative predictive value of 74.1%. nTMS motor mapping correlated with DES in all cases, with a mean abductor pollicis brevis hotspot distance of 3.50 ± 0.66 mm (n = 9). CONCLUSIONS: nTMS is a safe, noninvasive adjunctive tool for preoperative mapping of brain tumors in eloquent areas. Furthermore, nTMS can influence the surgical decisions in up to one third of patients in our experience.

Navigated Transcranial Magnetic Stimulation in Patient with Cranioplasty in Situ: Safe and Accurate Procedure.

BACKGROUND: Navigated transcranial magnetic stimulation (nTMS) is a nonsurgical mapping technique used in mapping of motor and language eloquent areas within and/or surrounding brain tumors. Previous reports support this as a safe technique with minor side effects associated with minor headaches and discomfort around the stimulation area. Currently there are no published reports concerning the accuracy and safety of this procedure in patients with a titanium cranioplasty in situ. CASE PRESENTATION: A 59-year-old lady was diagnosed with a recurrent glioma in the context of increasing seizure frequency, left-sided numbness, and weakness. She was diagnosed with a World Health Organization grade 2 oligodendroglioma 10 years before her presentation, which was initially treated with radiotherapy and then surgical resection of this lesion 5 years later. The procedure was complicated with a wound infection, treated with a craniectomy and wound washout, followed by a titanium cranioplasty. Before proceeding with surgery for recurrence, nTMS was performed for motor mapping. No complications were identified. She underwent a craniotomy for tumor resection with aminolevulinic acid HCl (Gliolan), and the tumor was completely removed. Intraoperatively, the direct cortical stimulation correlated with the preoperative nTMS. The pathologic diagnosis on recurrence was an anaplastic oligodendroglioma grade III, and the patient is currently undergoing adjuvant chemotherapy. CONCLUSION: This report confirms that nTMS is a safe and accurate procedure in patients who have a titanium cranioplasty in situ.

Refusal of Vitamin K Injection: Survey of the Current Literature and Practical Tips for Pediatricians.

Vitamin K refusal and associated sequelae of vitamin K deficiency bleed (VKDB) in the newborn period is becoming a more common occurrence. We present six recent cases from a 4-month period in 2017 of parent refusal of vitamin K and describe the reasons for refusal and the clinical outcomes of these infants. There have been a number of case reports citing the rising incidence of VKDB and the reasons why parents refuse. However, there is a gap in the literature and clinical practice guidelines describing how a physician should approach a refusal in the hospital and in the office, and the need to report a refusal to child welfare. In addition, we describe a scenario in which the caregivers provide a religious reason for refusal of vitamin K that, to the best of our knowledge, has yet to be cited in the literature. [Pediatr Ann. 2018;47(8):e334-e338.].

Lead Toxicity in the Pediatric Patient with Sickle Cell Disease: Unique Risks and Management.

Lead toxicity is the result of lead ingestion, one of the most common ingestions in the pediatric population. Nationwide and statewide efforts to recognize and curtail this epidemic have led to declining rates of toxicity. In patients with sickle cell disease (SCD), lead toxicity can be an elusive diagnosis due to overlapping symptom profiles, and inconsistent follow-up with a primary care physician can make the diagnosis even more difficult. In this article, two illustrative cases of lead toxicity in patients with SCD are described. The discussion reviews the current risk factors, screening, and inpatient management of lead toxicity, as well as describing the unique and sometimes confounding presentations of lead toxicity versus sickle cell crisis. [Pediatr Ann. 2018;47(1):e36-e40.].

Conus Medullaris Arteriovenous Malformation Presenting with Acute Dysphagia and Intractable Hiccups.

BACKGROUND: Conus medullaris arteriovenous malformations (AVMs) are rare spinal vascular malformations presenting most frequently with features of myelopathy (Foix-Alajounine syndrome), radiculopathy, bowel/bladder dysfunction, or acute spinal hemorrhage (Coup de poignard of Michon) causing profound neurological deficit. Here we present the case of a young patient with progressive dysphagia and intractable hiccups as a rare first presentation symptom of later verified conus medullaris AVM. CASE DESCRIPTION: A 21-year-old male patient presented with acute onset of dysphagia and persistent hiccups. His magnetic resonance imaging of the spine demonstrated a lesion at the T11 and T12 levels with an associated holocord syrinx and syringobulbia to the level of the medulla. The patient underwent a decompressive suboccipital craniectomy and C1 (atlas) laminectomy with wide myelotomy of the medulla followed by T11 and T12 laminectomy and AVM reduction. Two days after partial AVM occlusion the patient developed transient worsening of his symptoms. Repeat magnetic resonance imaging showed recurrence of dilatation of the central canal. A syringo-subarachnoid shunt was sited at the level of the previous myelotomy of the medulla, after which his neurological symptoms resolved completely. CONCLUSIONS: This is the first case report in the English literature to date of a conus AVM presenting with intractable hiccups. These are extremely rare sporadic vascular malformations, and although their natural history is poorly understood, symptomatic patients generally deteriorate, culminating in severe disability. Management requires a multimodality approach including combined endovascular and microsurgical treatment. The patient in our case made a full recovery confirmed at 2-year follow-up.