Don't make a mountain out of dermoid cysts of the anterior fontanelle.

Dermoid cysts are ectodermal inclusion lesions which can occur at the bregma, preventing complete closure of the anterior fontanelle. Various management strategies have been suggested for children presenting with such lesions. We describe the experience of managing a series of 13 consecutive patients at the University Hospital of Wales, Cardiff, including pre-operative workup, operative strategy, post operative care and follow up. Referred patients underwent a form of cross-sectional cranial imaging. All patients were given a general anaesthetic and had a small coronial incision over the lesion, large enough only to expose the lesion. Circumferential dissection of the lesion was then performed, taking particular care at the base of the lesion. We found no intra-dural or intra-sinus connection at the base of any lesion. All patients were discharged on the same day of surgery and were seen once in person or via telephone at follow-up prior to discharge. There have been no recurrences of any lesions. We conclude that these patients can be managed safely as day case procedures and discharged after single follow-up. Although a theoretical risk of intra-sinus or intra-dural connection exists, we suspect this is extremely rare.

Towards improved outcome in children treated surgically for spontaneous intracranial suppuration in South Wales.

INTRODUCTION: Spontaneous central nervous system (CNS) infections in children are rare. Treatment involves surgical intervention and antibiotic therapy. We describe a single centre experience of managing this condition in South Wales. METHODS: We performed a retrospective review of surgically managed cases in our unit for patients under 18 years of age between 2008 and 2018. Data were collected regarding aetiology, location, microbiology examination, treatment and outcomes. RESULTS: Twenty-six patients were identified of which 25 case notes were available. Fifteen were male and 10 were female. Median age was 12 years (age range 0.3-17 years). Seven patients (28%) had a burr-hole aspiration and 18 (72%) underwent craniotomy. A second procedure was performed in 10 (40%) and a third procedure in two (8%). Fourteen (56%) had a brain abscess, 10 (40%) had subdural empyema (one was bilateral) and one (4%) had an extradural empyema. Fifteen (60%) had a raised WCC (>11.5 × 109/L) and 22 (88%) had a CRP of >10 mg/L at presentation. Three (12%) patients had a normal WCC and CRP at presentation. Overall, 12 (48%) were secondary to sinus infection, with the most common organism being Streptococcus. Seven (28%) were due to otitis media or mastoiditis, six (24%) had no cause identified. The mean number of CT/MRI scans was 6.7 (range 3-13). The mean follow-up period was 16.7 months (range 1-117 months). At last follow up, 19 (76%) had a GOS of 5, five (20%) had a GOS of 4 and one (12%) had GOS of 3. There were no deaths. CONCLUSIONS: In Wales, outcomes have improved over time in keeping with other paediatric neurosurgical units in England. Increased availability of imaging resources in our hospital and use of neuro-navigation for all cases in our unit as well as earlier identification of sepsis, communication with microbiologists with dedicated ward rounds and, enhanced identification of causative organisms and contemporary anti-microbials have also contributed towards the improved management of this condition.

Suspected abusive head trauma in children - when do they really need to be transferred to a neurosurgical unit?

OBJECTIVES: Transferring paediatric patients with suspected abusive head trauma (AHT) to paediatric neurosurgical centres, disrupts safeguarding investigations. Therefore, it is desirable that suspected AHTs are transferred only when clinically necessary. The aim of this study was to describe referral patterns of patients referred to a tertiary paediatric neurosurgical centre with suspected AHT, with the view of informing future transfer of AHT patients. DESIGN: A retrospective review was performed of all suspected AHT patients referred to the University Hospital of Wales between 2012 and 2021. METHODS: Rates of surgery, intubation and ventilation, radiological findings and presenting GCS were compared between referred patients and those transferred for neurosurgical care. Variables were compared between the transferred and the non-transferred groups. For categorical variables, Chi-squared tests were performed, with Fisher's exact test used where the expected count was less than 5. Odds ratios (OR) for neurosurgical transfer with radiological or clinical features at presentation were calculated. RESULTS: A total of 76 patients were referred, of which 18 were transferred for neurosurgical care. Of these, six were intubated and ventilated. Only one transferred patient required surgery, with the remainder receiving supportive care. Amongst the transferred group, 77.8% had SDHs and 68.6% had a GCS ≥ 13 at presentation. Patients with a GCS ≥ 13 and SDHs, bilateral haematomas or a history of vomiting were significantly more likely to be transferred (OR = 4.27, 95%CI 1.01-18.00, p = .05). CONCLUSIONS: Most transferred patients with suspected AHT did not receive surgical intervention. We suggest that patients should be transferred when it is likely that they will require surgery, otherwise they should stay locally in order to complete their safeguarding investigations. Immediate transfer may not be necessary for AHT patients with a GCS ≥ 13 and either vomiting, SDHs or bilateral haematomas, provided they are unlikely to require emergency surgery.

Pre-operative dosing of dexamethasone for the management of children with posterior fossa tumours: are we getting it right?

INTRODUCTION: Posterior fossa (PF) tumours are associated with vasogenic oedema causing symptoms of raised intracranial pressure. Preoperatively this is managed with dexamethasone. To minimise steroid related complications, the lowest effective dose should be administered. No neurosurgical guidelines exist for pre-operative dosing of dexamethasone in PF tumours. METHODS: A retrospective review was performed of surgically managed cases for patients under 16 years of age between 2013 and 2018 to ascertain the initial dose of dexamethasone with symptomatic PF tumours. RESULTS: Thirty-six patients were identified of which 30 notes were available. Sixteen were male. Median age was 6 years (range 10 months - 15 years). Twenty-two (73%) were referrals from DGH and 8 (27%) presented to our neurosurgical centre. All patients presented with symptomatic PF tumours including headache (97%), vomiting (93%), gait disturbance (43%), and nystagmus (17%). Four (13%) had papilloedema. Average initial stat dexamethasone dose was 9.15 mg; 0.31 mg/kg (range 1-16.7 mg; 0.05 - 1.77 mg/kg). Stratified according to weight, average dose (and range) was 8.8 mg; 0.94 mg/kg (1-16.6 mg; 0.13 - 1.77 mg/kg) in those weighing <10 kg; 9.7 mg; 0.66 mg/kg (4-16.7 mg; 0.21 - 1.35 mg/kg) in 10-20 kg; 12.3 mg;0.52 mg/kg (8-16.7 mg; 0.27 - 0.73mg/kg) in 20-30 kg and 7.8 mg; 0.17mg/kg (2-16.7 mg; 0.0 - 0.39 mg/kg) in >30 kg up to a maximum of 16.6 mg in any 24h period. These results suggest that dosage was higher in those children weighing less. PPI was used in 24 (80%) of cases. All doses were reduced after review by the neurosurgical team and a PPI added. CONCLUSION: Pre-operative dexamethasone dosing does not always reflect the severity of clinical symptoms for PF tumours. Guidelines are required to correlate clinical symptoms with a suggested suitable dose of dexamethasone to prevent overdose and complications associated with corticosteroid use. We recommend a weight-based regimen as provided by the Food and Drug Administration. The current advice is for 0.02-0.3mg/kg/day in 3-4 divided doses.

Infected congenital cervical dermal sinuses leading to spinal cord abscess: two case reports and a review of the literature.

PURPOSE: Congenital dermal sinuses are a rare form of spinal dysraphism. The developmental defects are located along the midline neuroaxis, with sinuses in the cervical region being the least common. Congenital dermal sinuses can be associated with intraspinal infection as they act as a direct route from the skin and subcutaneous tissues into the spinal cord. METHODS: The authors present two cases of cervical dermal sinuses complicated by intramedullary abscess. Both children presented with neurological decline and febrile illness. MRI showed intraspinal abscess. Both underwent prompt surgical excision of the sinus tract, exploration of the cord and intravenous antibiotics. RESULTS: Both patients demonstrated excellent neurological recovery. CONCLUSIONS: Complete surgical excision of the sinus and tract in addition to long-term antimicrobials can yield excellent neurological outcomes. At surgery, do not expect to find pus when exploring the intramedullary component. Long-term follow-up is advocated due to potential late recurrence.

Surgical site infection in paediatric posterior fossa surgery: does pathology matter?

OBJECTIVES: The aim of this study was to explore the rates and potential risks of surgical site infection (SSI) after posterior fossa surgery for tumour resection in children. METHODS: We retrospectively reviewed our local paediatric (age < 16 years) database for all cases of posterior fossa (PF) brain tumour surgery between November 2008 and November 2019. We collected patient demographics, tumour histology/location, and the event of postoperative surgical site infection. RESULTS: Overall, 22.1% (n=15) developed SSI out of sixty-eight children undergoing PF surgery for resection of brain tumours; 73.3% of them had a confirmed diagnosis of medulloblastoma. There was no statistically significant difference in the age (5.1 ± 0.60 vs. 6.2 ± 0.97 years; p=0.47) and duration of operation (262 vs. 253 min; p = 0.7655) between the medulloblastoma group and other tumours. Although the rate of postoperative hydrocephalus was higher in the medulloblastoma group (12.9% vs. 0%), this was not associated with increased SSI. Rates of CSF leak between the 2 groups were not different. CONCLUSION: Medulloblastoma as a pathological entity seems to carry higher risk of postoperative surgical site infection compared to other types of paediatric posterior fossa tumours. Further larger studies are required to look into this causal relationship and other risk factors that might be involved.

Clinical features associated with the development of hydrocephalus following TBI in the paediatric age group.

INTRODUCTION: Predictive factors for post-traumatic hydrocephalus (PTH) in adults have been elucidated but remain uncertain for children. We aimed to identify the prevalence of PTH in paediatric patients and identify clinical/radiological factors which may increase the probability of children developing PTH. METHODS: This was a retrospective study of all patients < 16 years old admitted to our unit with traumatic brain injury (TBI) between March 2013 and June 2018, 108 patients in total. Patients were classified as mild (13-15), moderate (9-12) or severe (3-8) TBI based on admission GCS. Three independent reviewers collected data from case notes. CT scans were reviewed for hydrocephalus using Evan's index. Two-tailed Fisher's exact tests with a p value < 0.05 were considered statistically significant. RESULTS: Median patient age was 7 years, and 65% were males (n = 70). PTH wasn't identified in any patients with mild/moderate TBI (n = 79). In cases of severe TBI (n = 29), three patients developed PTH requiring ventriculoperitoneal shunting (10%; p = 0.02). Radiological features which were significantly more common in the PTH group were intraventricular haemorrhage (p = 0.05) and subarachnoid haemorrhage (p = 0.03). There was also a trend towards a statistically significant association with subdural haematoma (p = 0.07). The need for other neurosurgical procedures, such as fracture elevation and craniotomy, did not increase the probability of developing with PTH (p = 0.08). DISCUSSION: The prevalence of PTH in our study is 2.7%. Factors which may be associated with a higher probability of developing PTH may include IVH, SAH, severity of TBI, and subdural haematoma. We propose a national prospective multicentre database of paediatric PTH. The data collected on prevalence, presentation, risk factors, and management could guide contemporary management and improve the outcomes of children with PTH.

The need for hair removal in paediatric brain tumour surgery?

INTRODUCTION: Preoperative hair removal is conventional practice within neurosurgery in an attempt to maintain antisepsis. However, there is a lack of evidence to suggest that it makes a difference with regards to infection. This article aims to relate preoperative hair removal to SSIs for paediatric patients. METHODS AND MATERIALS: A retrospective analysis was conducted from a single paediatric neurosurgical database at the University Hospital of Wales. Patients were grouped according to whether they underwent preoperative hair removal or not. Findings were reviewed in light of the previously published literature. RESULTS: One hundred eighty two paediatric intracranial tumours were operated on between November 2008 and 2019. A total of twenty-six patients (14%) developed an infection post-operatively, of which meningitis was the most common (77%). Eighty-nine operations were undertaken without preoperative hair removal, of which there were a total of fifteen infections (17%). In the hair removal group, there were a total of eleven infections out of ninety-three operations (12%). Overall, the patients without hair removal had a higher infection rate when compared to those with hair removal (17 and 12% respectively), however, this result was not statistically significant (p-value 0.3989). CONCLUSION: We did not find evidence that hair removal in paediatric neurosurgery effects postoperative infection risk.

Unilateral akathisia and choreoathetosis as an unusual presentation of a frontal lobe dermoid cyst.

OBJECTIVE: To consider an unusual presentation of a frontal lobe dermoid cyst. Intracranial masses very rarely present with movement disorders. We describe a highly unusual presentation of an intracranial dermoid with unilateral choreoathetosis, akathisia and facial tics. MATERIAL: A 63-year-old man presented with left-sided akathisia and tardive dyskinesia of his upper limb, attributed to a dermoid cyst in the right frontal lobe. Resection of the cyst led to a complete resolution of his symptoms without neurological deficit. CONCLUSION: Pathogenic mechanisms of such symptoms may include compression and ischaemia of the basal ganglia and other nigrostriatal structures, as well as dysfunction of the supplementary motor area and mesolimbic dopaminergic pathways.

Paediatric posterior fossa tumour resection rates in a small volume centre: the past decade's experience.

BACKGROUND: Paediatric brain tumour resection rates have been shown to correlate with outcome and, it is argued, are linked to operator volume and caseload. The British paediatric neurosurgery community has previously debated centralisation of paediatric neuro-oncology. At the 2018 British Paediatric Neurosurgery Group (BPNG) meeting, a commitment was made to prospectively collect tumour resection data at each Neurosurgical Unit (NSU). Here we review our prospectively-collected 10-year database of the three commonest paediatric posterior fossa tumours - astrocytomas, medulloblastomas and ependymomas. MATERIALS AND METHODS: Our primary outcome was extent of resection (EOR) on post-operative MRI scans reviewed by neuro-radiologists. Secondary outcomes comprised neurosurgical morbidity including infection, need for cerebrospinal fluid (CSF) diversion and the occurrence of posterior fossa syndrome (PFS). RESULTS: 55 children had 62 operations, where our complete resection rates for pilocytic astrocytomas, medulloblastomas and ependymomas were 77%, 79% and 63%, respectively. Both our primary and secondary outcomes were in keeping with the published literature and we discuss here some of the factors which may contribute towards favourable outcomes in a small volume centre. CONCLUSION: Our results suggest that small volume centres can expect equivalent results to larger volume NSUs with regards to paediatric brain tumour surgery. Continuing efforts nationally for data collection on resection rates and operative outcomes is a key step towards optimising management in these children.

Meningioma with rhabdoid features combined with meningioangiomatosis in infancy: a novel combination.

Meningioangiomatosis is a rare histologically distinct abnormality that is occasionally associated with intracranial meningioma. The rhabdoid variant of meningioma is also uncommon and is classified as a World Health Organization Grade III tumour. We report a case of meningioangiomatosis in conjunction with a meningioma with prominent rhabdoid features, in an infant male who underwent complete surgical resection of the lesion. The patient has been followed up for 6 years with no disease recurrence. To our knowledge, this is the first report in the literature describing meningioangiomatosis combined with a meningioma with rhabdoid features.

Surgical management of raised intracranial pressure secondary to otogenic infection and venous sinus thrombosis.

PURPOSE: This study reviews paediatric patients with raised intracranial pressure as a result of venous sinus thrombosis secondary to otogenic mastoiditis, requiring admission to the paediatric neuroscience centre at the University Hospital Wales, Cardiff. The consensus regarding the management of otogenic hydrocephalus in the published literature is inconsistent, with a trend towards conservative over surgical management. We reviewed our management of this condition over a 9-year period especially with regard to ventriculo-peritoneal (VP) shunting. METHODS: Analysis of a prospectively collected database of paediatric surgical patients was analysed and patients diagnosed with otogenic hydrocephalus from November 2010 to August 2018 were identified. Our data was compared with the published literature on this condition. RESULTS: Eleven children, 7 males and 4 females, were diagnosed with otogenic hydrocephalus over the 9-year period. Five (45.5%) required VP shunt insertion to manage their intracranial pressure and protect their vision. The remaining six patients (54.5%) were managed medically. CONCLUSIONS: When children with mastoiditis and venous sinus thrombosis progress to having symptoms or signs of raised intracranial pressure, they should ideally be managed within a neuroscience centre. Of those children, almost half will need permanent cerebrospinal fluid diversion to protect their sight.

Outcomes with respect to extent of surgical resection for pediatric atypical teratoid rhabdoid tumors.

PURPOSE: To evaluate overall survival for atypical teratoid rhabdoid tumors (ATRTs) in relation to extent of surgical resection. METHODS: The neurosurgical tumor databases from three UK Pediatric centers (University Hospital of Wales, Alder Hey and Royal Manchester Children's Hospital) were analyzed. Patients with a diagnosis of ATRT were identified between 2000 and 2018. Data was collected regarding demographics, extent of resection, complications, and overall survival. RESULTS: Twenty-four patients diagnosed with ATRT underwent thirty-eight operations. The age range was 20 days to 147 months (median 17.5 months). The most common location for the tumor was the posterior fossa (nine patients; 38%). Six patients (25%) underwent a complete total resection (CTR), seven (29%) underwent a near total resection (NTR), eight (33.3%) underwent a subtotal resection (STR), and three patients (12.5%) had biopsy only. Two-thirds of patients who underwent a CTR are still alive, as of March 2019, compared to 29% in the NTR and 12.5% in the STR groups. Out of the thirty-eight operations, there were a total of twenty-two complications, of which the most common was pseudomeningocele (27%). The extent of surgical resection (p = 0.021), age at surgery (p = 0.00015), and the presence of metastases at diagnosis (0.015) significantly affected overall survival. CONCLUSIONS: Although these patients are a highly vulnerable group, maximal resection is recommended where possible, for the best chance of long-term survival. However, near total resections are likely beneficial when compared with subtotal resections and biopsy alone. Maximal surgical resection should be combined with adjuvant therapies for the best long-term outcomes.

The effect of the revised WHO classification on the incidence of grade II meningioma.

Introduction: A retrospective study over a three-year period at University Hospital of Wales (UHW) of the incidence of atypical cranial grade II meningioma both pre and post 2016 revision of The World Health Organisation (WHO) classification of tumours of the central nervous system.Subjects: All available histology from January 2015 to December 2017 of patients with cranial meningiomas.Method: Institutional online reporting system Welsh Clinical Portal to identify patients and view histology reports.Results: Overall 164 patient histology results were analysed (median age 59, age range 23-82, 74% females). There were 55 patients in 2015: 69.1% grade I, 29.1% grade II and 1.8% grade III. There were 109 patients in 2016/17; 68.8% grade I, 29.4% grade II, 1.8% grade III.Discussion: There is significant variability in the reported incidence of grade II meningioma, likely due to variation in local interpretation of diagnostic criteria. Neuropathologists at our institution have reported brain invasion as grade II prior to 2016. This was due to compelling published evidence that brain invasive meningiomas have recurrence and mortality rates similar to that of grade II meningioma as defined using other criteria. The new 2016 WHO criteria now recognise this specifically. As other institutions adapt to the amended 2016 guidelines we anticipate that there will be a greater consensus in line with our incidence rates of grade II meningioma.

Cerebrospinal fluid diversion versus bariatric surgery in the management of idiopathic intracranial hypertension.

Background: Idiopathic intracranial hypertension (IIH) is a condition affecting predominantly young women with increased body mass index (BMI). Obesity with the related metabolic and biochemical complications are thought to be involved in the pathogenesis of the condition. The aim of this study is to evaluate the safety, outcomes and economic implications of two treatment options for IIH.Methods: We retrospectively analysed cases of morbidly obese IIH patients treated by cerebrospinal fluid (CSF) shunting procedures between 2006 and 2016 in our department and compared their outcome with that of 69 patients undergoing bariatric surgery between 2015 and 2016.Results: A total of 42 female patients with IIH underwent de-novo shunting procedures during the study period. There was a high rate of shunt revisions (67%) and further weight gain in the majority of patients who had the insertion of CSF shunts. Of the 69 female patients undergoing bariatric surgery 4.3% required interventions related to their surgery with a significantly fewer number of hospital inpatient days. Furthermore, in the patients undergoing bariatric surgery, there was a significant improvement in all obesity-related complications.Conclusions: CSF shunting procedures do not address the aetiological factor of IIH and are associated with high rates of morbidity and further weight gain. Bariatric surgery is not only efficacious in the management of patients with IIH but is associated with significant improvements in other obesity-related comorbidities. Bariatric surgery is safe and more cost-effective than CSF shunting.

Is 30-day readmission an accurate measure of morbidity in cranial meningioma surgery?

Introduction: 30-day readmission rate is a widely adopted marker of quality and performance of acute care, but validity is not well demonstrated. Readmission data following cranial meningioma surgery is not well described and we sought to assess risk factors for readmission. Subjects: All adult patients who underwent cranial meningioma resection from January 2015 to December 2017 in a single institution. Methods: Using Welsh Clinical Portal electronic data to identify readmission within and beyond 30 days to both the index hospital and regional hospitals. Causes of readmission were recorded. Results: 160 patients were included (76.3% female, median age 58, age range 23-78). 28 cases (17.5%) were emergency admissions, median length of initial admission 7 days. 42 patients (26.3%) had seizures at presentation. Total readmission rate was 13.8% (median age 54.5, pre-operative seizure rate 40.9%, median length of readmission 9 days). 13 (59.1%) patients presented within 30 days and 9 (40.9%) >30 days. Readmission causes were seizure, neurological deficit, thromboembolic, infection, CSF, bleeding and social. Causes after 30 days were the same except social or neurological deficit. Discussion: Readmission rates are not associated with age, tumour location, tumour grade, admission route or initial length of stay. 40.9% of readmissions presented outside of the 30-day post-operative time. 30-day readmission rates may not be the most suitable method to demonstrate neurosurgical unit performance in meningioma surgery.

How much do plastic surgeons add to the closure of myelomeningoceles?

PURPOSE: This study reviews the outcomes of children undergoing myelomeningocele (MMC) repair in the paediatric neurosurgical department in Cardiff. These procedures are historically performed by paediatric neurosurgeons with occasional support from plastic surgeons for the larger lesions. We reviewed the postoperative outcomes over a 9-year period to assess the efficacy of having a plastic surgeon present at all MMC closures. METHODS: Analysis of a prospectively collected database of all MMC closures performed at University Hospital Wales from April 2009 to August 2017 was used. Comparison was made with the published literature especially with regard to complications. RESULTS: Thirty-one children, 13 males and 18 females, underwent MMC closure over the 9-year period. Twenty-four (77.4%) defects were closed by direct approximation. Seven patients (22.5%) required a more complex plastic procedure to obtain closure. Two patients (6.5%) had a wound complication, one wound infection and one flap edge necrosis both healing with dressings alone. Two patients had cerebrospinal fluid (CSF) leaks that responded to ventriculo-peritoneal shunting. Two patients died from unrelated conditions during the study period. CONCLUSION: In our series, 7/31 (22.5%) cases involved a more complex closure in keeping with the literature. The authors feel that having the plastic surgeon at all closures has led to a low wound complication rate.

Ruptured pseudoaneurysm as a cause of spontaneous intracerebral bleed in a 3-month old infant.

Ruptured intracranial aneurysms in infants are very rare but if missed can lead to poor outcomes. Spontaneously dissecting false aneurysms have been described only in a handful of cases. We report a case of a three-month old girl with deteriorating neurological function due to a ruptured distal middle cerebral artery pseudoaneurysm.

Surgical treatment of long-standing overt ventriculomegaly in adults (LOVA).

BACKGROUND: Longstanding overt ventriculomegaly in adults (LOVA) is characterised by chronic hydrocephalus presumed to begin during infancy, but arresting before becoming clinically detectable. Later in life clinical features of hydrocephalus ensue, typically in the 5th or 6th decades. Only a relatively small number of LOVA case series have been published, and ambiguity remains regarding optimal management. This case series describes a series of patients with LOVA treated successfully at a single neurosurgical institution using endoscopic third ventriculostomy (ETV). METHODS: A series of 14 patients were diagnosed with LOVA using established clinical and radiological criteria. All patients underwent an ETV and their clinical conditions were followed up for up to 5 years post-operatively. RESULTS: Fourteen patients (100 %) reported either improvement or halt of progression in their presenting symptoms 3 months after ETV; 93 % of patients (n = 13) did not require any further surgical intervention. One patient (7 %) reported deterioration in symptoms beyond 3 months post-operatively, which necessitated further surgery (ventriculoperitoneal shunt). These promising outcomes after ETV are mirrored in numerous other LOVA case series. Other works have analysed the value of CSF shunting procedures in LOVA, with mixed results. A direct, prospective comparison of outcomes after shunt procedures and ETV, with a specific focus on LOVA patients, is yet to be completed. A minority of patients fail to respond, or develop recurrence of symptoms, months or years after initial surgical intervention. CONCLUSIONS: ETV is an attractive option for surgical treatment of LOVA. After surgical treatment for LOVA, long-term follow-up should be considered to screen for late recurrence of the condition.

Asymptomatic Chiari Type I malformation: should patients be advised against participation in contact sports?

BACKGROUND: Chiari type I malformation (CM-I) is characterised by caudal displacement of the cerebellar tonsils through the foramen magnum, crowding the craniocervical junction. It is being increasingly diagnosed in asymptomatic patients due to the widespread availability of MRI, and there are case reports of these patients suffering sudden death or neurological injury following head or neck trauma, raising the issue of whether they should be prohibited from contact sport participation, given the likelihood of frequent trauma. General neurosurgical opinion is that patients who are symptomatic and those with an associated spinal cord syrinx should be offered foramen magnum decompression, however asymptomatic patients without syringomyelia are not offered this in the majority of cases. METHODS: The authors performed a full review of the published literature, including all case reports, case series, studies and literature reviews regarding CM-I and either contact sports or trauma, excluding patients that had undergone surgical intervention and those becoming symptomatic in circumstances other than head or neck trauma. RESULTS: 21 case reports of CM-I patients deteriorating following trauma were identified, including four cases of sudden death following head or neck injury. However, studies of large samples of CM-I patients are yet to capture an incident of sudden death or acute neurological deterioration, suggesting that the risk is very low. CM-I patients may have an increased risk of concussion and post-concussion syndrome compared to the background population however. CONCLUSION: Overall, the authors feel that there should be no restriction of sports participation for CM-I patients, but a discussion to make them and their families aware of the possible increased risks is important.