Clinical characteristics and outcomes of children with non-sinogenic Brain infections.

BACKGROUND: Central Nervous System (CNS) suppurative infections are serious but rare conditions in the paediatric population. Data on long-term neurocognitive and quality of life outcomes in children recovering from these infections are lacking. METHODS: A retrospective cohort review of children <16 years with non-sinogenic infections undergoing neurosurgery was conducted. Data for patients admitted to Children's Health Ireland at Temple St between 2008-2021 were analysed for clinical and microbiological profiles. Follow-up reviews evaluating neurological and academic sequelae and quality of life were performed. Categorical variables were analysed for unfavourable outcome with a p < 0.05 significance value. RESULTS: Forty patients were included with a mean age of 4.5 years and equal gender distribution. Fever (68%) and vomiting (58%) were the most common presenting complaints. Only fourteen (35%) patients presented with the classic triad of fever, headache, and focal neurological deficit. Meningitis/Encephalitis was the most common cause of suppurative infection (40%). Predisposing factors included congenital heart disease (18%), prematurity (15%) and immunocompromised status (10%). More patients received an initial Burr hole aspiration (73%) than Craniotomy (27%). The re-operation rate was higher in the craniotomy group (45%) compared to the burr hole group (34%), but this was not statistically significant (p = 0.522). Four patients died (10%) including two intra-hospital deaths (5%). Male gender (p = 0.047) and multiple abscesses (p = 0.041) were associated with unfavourable outcome at discharge. Mobility impairment was the most affected determinant of quality of life. CONCLUSION: CNS suppurative infections are associated with long-term neurocognitive sequelae in children. Multiple abscesses and male gender are associated with unfavourable GOS on discharge. Children are still left with mobility impairment (25%), personality changes (23%) and intellectual disability (18%) at an average of 5 years. Long-term follow up with multidisciplinary input is required. Further research should focus on evaluating long-term HRQoL in children.

Effectiveness of subtemporal decompression for the management of slit ventricle syndrome.

OBJECTIVES: To assess the effectiveness of subtemporal decompression in the management of slit ventricle syndrome. METHODS: We conducted a retrospective review of all patients with slit ventricle syndrome (SVS) who underwent subtemporal decompression (STD) at our centre between 2010 and 2021. Cases were identified using the hospital database. Medical records for each patient were reviewed, including operative and radiological reports. RESULTS: Fifteen patients underwent STD for the management of SVS. Median age at time of STD was 9.18 years. Aetiology of hydrocephalus consisted of spinal dysraphism (5), idiopathic (4), post-infectious (1), post-haemorrhagic (3), secondary to tumour (1), and craniofacial anomalies (1). Median age at first shunt insertion was 3.4 months. Median pre-operative period assessed, from initial shunt insertion to STD, was 4.54 (interquartile range [IQR] 3.12-10.47) years. Twelve patients underwent ≥1 shunt revision prior to STD. All patients had a diagnosis of SVS at time of STD. Presenting symptoms, for the admission in which STD was performed, included nausea (9), vomiting (8), lethargy (8), headache (12), irritability (5), and visual disturbances (6). One third underwent shunt revision at the time of STD. Two patients developed post-operative complications requiring further surgery (meningitis requiring shunt revision: 1; wound debridement: 1). Three patients developed uncomplicated post-operative pyrexia, which was managed with antibiotics. Median duration of post-operative follow-up was 5.4 (IQR 1.73-8.54) years. Eleven patients underwent ≥1 shunt related procedure following STD. Wilcoxon signed-rank test demonstrated a significant difference in number of shunt related procedures before (median = 5, IQR 1-8) and after (median = 3, IQR 0-5) STD (Z = -2.083, p = .037). All patients reported subjective symptom improvement post-operatively. Thirteen patients experienced symptom recurrence at a median duration of 10 months post-operatively. CONCLUSIONS: STD was associated with a reduction in the amount of shunt related procedures required in this group of patients with SVS. Further study is required to confirm this association.

Multiple Abscesses in the Frontal, Temporal and Brainstem regions in a 4.5-year-Old Girl- An Illustrative Case Report.

Background: Brainstem located abscesses are rare in the pediatric population. Diagnosis of brain abscess can be challenging as patients may present with nonspecific symptoms and the classical triad of headache, fever, and focal neurological deficit is not always present. Treatment can be conservative or a combination of surgical intervention with antimicrobial therapy. Case Description: We present the first case of a 4.5-year-old girl with acute lymphoblastic leukemia that developed infective endocarditis (IE) and subsequently developed 3 suppurative collections (frontal, temporal, and brainstem). The patient had negative cerebrospinal, blood, and pus culture growth and subsequently underwent burr-hole drainage of the frontal and temporal abscesses with a 6-week course of intravenous antibiotic therapy with an uneventful postoperative course. At 1 year, the patient is left with minor right lower limb hemiplegia and no cognitive sequelae. Conclusion: The decision to surgically intervene for brainstem abscesses is dependent on surgeon and patient factors including the presence of multiple collections, midline shift, the aim of source identification in sterile cultures, and the patient's neurological condition. Patients with hematological malignancies should be monitored closely for IE which is a risk factor for hematogenous spread of brainstem located abscesses.

Spontaneous intracerebral haemorrhage secondary to 5-ALA-induced thrombocytopaenia in a paediatric patient: case report and literature review.

INTRODUCTION: The primary objective of neurosurgical management of malignant gliomas is maximal safe resection of the tumour. One of the main obstacles in achieving this is the ability to accurately discriminate between tumour edges and the surrounding healthy brain tissue. The use of fluorescence-guided surgery utilising 5-aminolevulinic acid (5-ALA), first introduced more than 20 years ago, has become an invaluable adjunct in high-grade glioma surgery in adults. However, as 5-ALA is not licensed for use in paediatric patients, the safety profile for such use remains undetermined. CASE REPORT: We describe the case of a 4-year-old boy who underwent 5-ALA-guided resection of a fourth ventricle anaplastic ependymoma. Although complete resection was achieved and the patient awoke from surgery well with no neurological deficits, the patient developed acute transaminitis, anaemia, thrombocytopaenia and coagulopathy postoperatively. The patient had a sudden neurological deterioration on postoperative day 2; imaging revealed that he had suffered a spontaneous right frontal intracerebral haemorrhage. The patient returned to theatre for surgical decompression and evacuation of the haematoma, and ultimately went on to make a full recovery. CONCLUSION: The use of 5-ALA in paediatric patients can be helpful in maximising surgical resection, but the associated safety profile remains undefined. Further research is urgently warranted in order to characterise the efficacy and risk of the use of 5-ALA in the paediatric population.

Prenatal findings and associated survival rates in fetal ventriculomegaly: A prospective observational study.

OBJECTIVES: Fetal ventriculomegaly is associated with varying degrees of genetic and structural abnormalities. The objective was to present the experience of fetal ventriculomegaly in a large European center in relation to: 1. grade of ventriculomegaly; 2. additional chromosomal/structural abnormalities; and 3. perinatal survival rates. METHODS: This was a prospective observational study of patients referred with fetal ventriculomegaly from January 2011 to July 2020. Data were obtained from the hospital database and analyzed to determine the rate of isolated ventriculomegaly, associated structural abnormalities, chromosomal/genetic abnormalities, and survival rates. Data were stratified into three groups; mild (Vp = 10-12 mm), moderate (Vp = 13-15 mm) and severe (Vp > 15 mm) ventriculomegaly. RESULTS: There were 213 fetuses included for analysis. Of these 42.7% had mild ventriculomegaly, 44.6% severe and 12.7% had moderate ventriculomegaly. Initial ultrasound assessment reported isolated ventriculomegaly in 45.5% fetuses, with additional structural abnormalities in 54.5%. The rate of chromosomal/genetic abnormalities was high,16.4%. After all investigations, the true rate of isolated VM was 36.1%. The overall survival was 85.6%. Survival was higher for those with isolated VM across all groups (P < 0.05). CONCLUSION: Ventriculomegaly is a complex condition and patients should be counselled that even with apparently isolated VM, there remains the possibility of additional genetic and/or structural problems being diagnosed in up to 10% of fetuses.

Severe fetal ventriculomegaly: Fetal morbidity and mortality, caesarean delivery rates and obstetrical challenges in a large prospective cohort.

INTRODUCTION: Severe fetal ventriculomegaly (VM) is defined as an enlargement of the atria of the lateral cerebral ventricles (Vp) of greater than 15 mm. While it is well established that it confers significant risk of morbidity and mortality to the neonate, there is limited information pertaining to the caesarean delivery rates and the obstetric management of these complex cases. The aim of this study was twofold: firstly, to determine survival rates in fetuses with severe VM, and secondly to determine the caesarean delivery rates in continuing pregnancies. We explore the obstetric challenges associated with these difficult cases. METHODS: This was a prospective observational study of patients with antenatal severe VM, attending the Department of Fetal Medicine, National Maternity Hospital, Dublin, Ireland, from 1st January 2011 to 31st July 2020. Data were obtained from the hospital database and those with severe VM (Vp > 15 mm) were identified. The rates of chromosomal abnormalities, the survival rates and the caesarean delivery (CD) rates for the overall group were then determined. The data were then further sub-divided into two groups: 1. Vp < 20 mm and 2. Vp > 20 mm, and the results compared. Statistical analysis was performed using the Chi-Square test. RESULTS: A total of N = 95 pregnancies with severe VM were included for analysis, of which additional structural abnormalities on ultrasound were apparent in 67/95 (70.5%) and 28/95 (29.5%) had isolated severe VM. Chromosomal abnormalities were diagnosed in 15/95 (15.8%) of cases, with (2/28) 7.1% in the isolated SVM group versus (13/67) 19.4% in the non-isolated SVM group. The overall survival rate (excluding TOP) was 53/74 (71.6%), with 20/23 (86.9%) in the isolated SVM group. The overall CD rate was 47/72 (65.3%), which was significantly higher than the CD for the hospital during the same time period of 25.4% (P < 0.01). The data were subdivided into Vp < 20 and Vp > 20 and those with a Vp > 20 had higher rates of additional intracranial findings on ultrasound (Vp < 20 13/41 (31.7%) versus Vp > 20 32/54 (59.3%) (P < 0.05)) and macrocrania (Vp < 20 14/41 (34.1%) versus Vp > 20 35/54 (64.8%) (P < 0.05)). No significant difference was observed in the overall survival or CD rates between the two groups. CONCLUSION: In conclusion this study reports significant fetal morbidity and mortality with severe VM with high CD rates observed in this cohort. Significant challenges exist in relation to the obstetric management and counseling of parents regarding an often uncertain neonatal prognosis. In continuing pregnancies with significant macrocrania delivery plans should be individualized to improve neonatal outcomes where possible and minimize harm to the mother.

A multidisciplinary fetal neurosurgical service-5 years of fetal outcomes from a national referral centre.

BACKGROUND: A specialist fetal neurosurgical clinic was set up in order to improve patient care in a tertiary referral fetal medicine centre. The clinic provides a targeted clinical service for women diagnosed with fetal neurological abnormalities. The service consists of fetal MRI, fetal ultrasound and joint assessment and counselling from neurosurgery and fetal medicine teams. AIMS: We aimed to review this service that provides MDT expertise directly to parents and record the cases and pregnancy outcomes involved. METHODS: This is a prospective study of clinic data from Jan 2013 to Dec 2017. Information includes ultrasound scan findings, MRI results, karyotype results and pregnancy outcome data including post mortem results and data from the paediatric neurosurgery service at the affiliated children's hospital. RESULTS: From 2013 to 2017, there were 1852 major fetal anomalies diagnosed antenatally at the tertiary referral fetal medicine service and n = 306/1852 [16%] were primarily neurological in origin. The neurosurgical clinic reviewed 125 patients since 2013. The most common reasons for referral were spina bifida, n = 60 [48%] and isolated ventriculomegaly n = 43 [34%]. Other reasons for referral include agenesis of the corpus callosum n = 4 [3%], encephalocoele n = 5 [4%] and intracranial mass lesions n = 3 [2.4%]. Cases with borderline ventriculomegaly and cases with known chromosomal or genetic abnormalities were not typically referred to the clinic. Full outcome data were available on 110 of 125 women seen. Thirty-two women [29%] underwent invasive testing and 14 women [12.7%] had a termination of pregnancy. CONCLUSION: Multidisciplinary antenatal counselling supported with in utero MRI provides families with optimum information to inform them of likely neonatal outcome.

Predicting outcomes in Dandy-Walker malformation: a retrospective cohort study.

OBJECTIVE: Dandy-Walker malformation (DWM) is a disorder that most neurologists and neurosurgeons will manage at some point during their careers. It is characterized by partial or complete dysgenesis of the cerebellar vermis. Outcomes are highly variable and range from functionally normal to severely disabled. Predicting these outcomes has classically been focused on the radiological findings that constitute DWM. Other anomalies that can be commonly found in these patients are potentially more indicative of outcome than the tenet markers of DWM. Furthermore, hydrocephalus is an ever-present danger in these patients, many of whom will be admitted to the hospital due to this condition. This study aims to identify these items as potential predictors of outcome. METHODS: All referrals from antenatal anatomy scans between 1992 and 2013 that were suspicious for DWM were reviewed. Neurosurgery archives were reviewed for outpatient letters and other correspondence. The number of DWM diagnoses was quantified. Outcomes were judged based on patient status, ranging from death to attending normal school. The presence of any other anomalies was quantified and measured against patient outcomes. RESULTS: Cyst size and the presence of another CNS anomaly were shown to portend worse outcomes. Non-CNS anomalies and hydrocephalus were not predictive of worse outcomes. Furthermore, of all the treatments assessed, ventriculoperitoneal shunts were shown to be the most effective in this data set. CONCLUSIONS: Results from this study suggest a pivot in how prognoses in DWM should be established and how parents should be counseled, along with a view of hydrocephalus and its treatment that challenges the current literature.

Intrathecal baclofen pumps in the management of hypertonia in childhood: a UK and Ireland wide survey.

BACKGROUND: Intrathecal baclofen (ITB) is a useful treatment for hypertonia where non-invasive treatments have been ineffective or poorly tolerated. There is an absence of national guidance on selection criteria and a lack of literature regarding patient characteristics and treatment details for children and young people (CYP) receiving ITB therapy in the UK and Ireland. We aimed to gather patient and treatment characteristics for CYP receiving ITB in the UK and Ireland. METHODS: An electronic survey was sent to all paediatric ITB centres in the UK and Ireland. Anonymised data were returned between December 2019 and April 2020. CYP >16 years and those awaiting ITB pump removal were excluded from the dataset. RESULTS: 176 CYP were identified as receiving ITB therapy across the UK and Ireland. The majority of CYP with ITB pumps were non-ambulant (93%) with a diagnosis of cerebral palsy (79%). Median age of ITB insertion was 9 years; median current age was 14 years. 79% of CYP had significant spasticity, 55% had significant dystonia. The most commonly used ITB dosing modes were continuous (73%) and flexible (23%). CONCLUSIONS: ITB pumps were most frequently used for non-ambulant CYP with cerebral palsy and existence of spasticity and/or dystonia in the UK and Ireland. Most CYP were receiving a continuous dose of ITB. There is significant variation in the number of paediatric ITB pumps across UK and Ireland. There is a need for development of nationally accepted paediatric referral criteria and clinical standards for ITB use.

Endoscopic extended transsphenoidal surgery for newly diagnosed paediatric craniopharyngiomas.

PURPOSE: Endoscopic extended transsphenoidal surgery (EETSS) has gained popularity for treatment of craniopharyngiomas. The aim of this study is to assess the outcome of endoscopic extended transsphenoidal surgery (EETSS) for newly diagnosed paediatric craniopharyngiomas. METHODS: Patient details were obtained from a prospective database of all endoscopic transnasal operations performed by a single surgeon. Outcomes including visual function, pituitary function, body mass index (BMI), postoperative neurological deficit, extent of resection and recurrence on follow-up were obtained. Obesity was defined as BMI percentile of equal to or greater than 95%. RESULTS: Between January 2011 and January 2020, 15 of 16 children (5-18 years old) with newly diagnosed craniopharyngiomas underwent EETSS. Four patients had a conchal-type sphenoid sinus. Gross total resection (GTR) was achieved in 4 patients and near total resection (NTR) in 5 patients. The remaining 6 had subtotal resection (STR). Postoperative radiotherapy was used in 6 patients (4 with STR, 2 with NTR). There were no postoperative deaths, strokes or CSF leaks. Normalisation of visual fields (VF) occurred in 9/13 patients with preoperative VF defects. One patient developed a new visual field defect. During a median follow-up period of 74 (8-104) months, 2 patients have required further surgery for tumour progression following initial STR, where a tumour remnant was left in situ to preserve the pituitary stalk. 6/11 patients developed new anterior pituitary dysfunction as a result of surgery and 9/12 developed new diabetes insipidus (DI). At the time of last follow-up, 14/15 children had anterior panhypopituitarism, 13/15 had DI and 1 patient developed new onset obesity. Two patients, who were obese preoperatively, were no longer obese at last follow-up. CONCLUSIONS: EETSS can be performed as the first option in the majority of children with newly diagnosed craniopharyngioma, despite factors such as small nose, non-pneumatised sphenoid sinus, small sella or purely suprasellar tumour location. Preservation of the pituitary stalk at the expense of leaving residual tumour may not be in the best interests of the patient.

Moyamoya disease and moyamoya syndrome in Ireland: patient demographics, mode of presentation and outcomes of EC-IC bypass surgery.

BACKGROUND: There are no previously published reports regarding the epidemiology and characteristics of moyamoya disease or syndrome in Ireland. AIMS: To examine patient demographics, mode of presentation and the outcomes of extracranial-intracranial bypass surgery in the treatment of moyamoya disease and syndrome in Ireland. METHODS: All patients with moyamoya disease and syndrome referred to the National Neurosurgical Centre during January 2012-January 2019 were identified through a prospective database. Demographics, clinical presentation, radiological findings, surgical procedures, postoperative complications and any strokes during follow-up were recorded. RESULTS: Twenty-one patients were identified. Sixteen underwent surgery. Median age at diagnosis was 19 years. Fifteen were female. Mode of presentation was ischaemic stroke in nine, haemodynamic TIAs in eight, haemorrhage in three and incidental in one. Sixteen patients had Moyamoya disease, whereas five patients had moyamoya syndrome. Surgery was performed on 19 hemispheres in 16 patients. The surgical procedures consisted of ten direct (STA-MCA) bypasses, five indirect bypasses and four multiple burr holes. Postoperative complications included ischaemic stroke in one patient and subdural haematoma in one patient. The median follow-up period in the surgical group was 52 months; there was one new stroke during this period. Two patients required further revascularisation following recurrent TIAs. One patient died during follow-up secondary to tumour progression associated with neurofibromatosis type 1. CONCLUSIONS: Moyamoya is rare but occurs in Caucasians in Ireland. It most commonly presents with ischaemic symptoms. Surgical intervention in the form of direct and indirect bypass is an effective treatment in the majority of cases.

Role of routine post-operative CT brain following evacuation of extradural haematoma in children: a single-centre experience.

PURPOSE: Extradural haematoma (EDH) is a serious neurosurgical emergency in children, which confers significant morbidity and mortality rates. The objective of this study was to retrospectively evaluate the role of post-operative imaging in children with EDH who were managed surgically in a national paediatric neurosurgical unit over a 9-year period (January 2008 to December 2016). METHODS: A retrospective case review of paediatric patients who underwent surgical evacuation of extradural haematoma between January 2008 and December 2016 was performed. This included demographic and clinical details, indications for post-operative imaging and outcomes. RESULTS: Seventy patients underwent surgical management of EDH during this time period, with a male preponderance (69%) and a mean age of 8 years. The commonest location of haematoma in this cohort was in the parietal region (n = 24), with a mean maximum thickness of 25.9 mm and mean volume of 57 ml. Post-operative imaging was performed in 84% of patients. However, only one patient had a change in the course of their post-operative management as a result of post-operative imaging findings. CONCLUSIONS: Post-operative imaging in asymptomatic paediatric patients after evacuation of EDH could therefore be avoided as a routine investigation.

Correction to: International experience in the development of patient-derived xenograft models of diffuse intrinsic pontine glioma.

There are two errors and one omission in the original article. Author Gottardo's correct name is Nicholas G. Gottardo, author Hulleman's correct affiliation is no. 3 (VUMC, Amsterdam), and the Acknowledgements should include the following sentence: "We would like to thank Dr Angel Montero Carcaboso (Hospital Sant Joan de Deu, Barcelona, Spain) for generously supplying the HSJD-DIPG007 cells."

International experience in the development of patient-derived xenograft models of diffuse intrinsic pontine glioma.

PURPOSE: Diffuse intrinsic pontine glioma is the most aggressive form of high grade glioma in children with no effective therapies. There have been no improvements in survival in part due poor understanding of underlying biology, and lack of representative in vitro and in vivo models. Recently, it has been found feasible to use both biopsy and autopsy tumors to generate cultures and xenograft models. METHODS: To further model development, we evaluated the collective international experience from 8 collaborating centers to develop DIPG pre-clinical models from patient-derived autopsies and biopsies. Univariate and multivariate analysis was performed to determine key factors associated with the success of in vitro and in vivo PDX development. RESULTS: In vitro cultures were successfully established from 57% of samples (84.2% of biopsies and 38.2% of autopsies). Samples transferred in DMEM media were more likely to establish successful culture than those transported in Hibernate A. In vitro cultures were more successful from biopsies (84.2%) compared with autopsies (38.2%) and as monolayer on laminin-coated plates than as neurospheres. Primary cultures successfully established from autopsy samples were more likely to engraft in animal models than cultures established from biopsies (86.7% vs. 47.4%). Collectively, tumor engraftment was more successful when DIPG samples were directly implanted in mice (68%), rather than after culturing (40.7%). CONCLUSION: This multi-center study provides valuable information on the success rate of establishing patient-derived pre-clinical models of DIPG. The results can lead to further optimization of DIPG model development and ultimately assist in the investigation of new therapies for this aggressive pediatric brain tumor.

Neurosurgical management in children with bleeding diathesis: auditing neurological outcome.

OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation. RESULTS Five patients with a bleeding diathesis who underwent surgery for intracranial hematoma were identified; the diagnosis was hemophilia A in 3 cases, idiopathic thrombocytopenic purpura in 1 case, and severe aplastic anemia in 1 case. Intracerebral hematoma (ICH) (n = 4) and acute subdural hematoma (n = 1) were confirmed on radiological investigations. In 2 of the 4 patients with ICH, the diagnosis of bleeding diathesis was made for the first time on presentation. Four patients (all male) were younger than 2 years; the patient with severe aplastic anemia and spontaneous ICH was 15 years old and female. The duration of symptoms varied from 24 hours to 5 days. Neurological examination at 1 year's follow-up showed complete recovery (GOS-E Peds score of 1) in 3 cases and mild weakness (GOS-E Peds score of 2) in 2 cases. CONCLUSIONS Neurosurgical management of patients with bleeding diathesis should be carried out in a tertiary-care setting with multidisciplinary team management, including members with expertise in neuroimaging and hematology, in addition to neurosurgery. Early diagnosis and prompt treatment of a bleeding diathesis is crucial for full neurological recovery.

Prenatal surgery for spina bifida: a therapeutic dilemma. Proceedings of the SHINE conference, Belfast.

This is a transcript of a scientific conference on the subject of prenatal surgery for spina bifida. It represents the views of three patients, an obstetrician, a postnatal neurosurgeon, a neonatologist, a paediatric neurologist, two surgeons who practice open spina bifida foetal surgery, a fetoscopic surgeon and an obstetrician experienced in randomised trials and systematic reviews. Implications for current practice and recommendations for future research are also discussed in detail.

Integrated Molecular Meta-Analysis of 1,000 Pediatric High-Grade and Diffuse Intrinsic Pontine Glioma.

We collated data from 157 unpublished cases of pediatric high-grade glioma and diffuse intrinsic pontine glioma and 20 publicly available datasets in an integrated analysis of >1,000 cases. We identified co-segregating mutations in histone-mutant subgroups including loss of FBXW7 in H3.3G34R/V, TOP3A rearrangements in H3.3K27M, and BCOR mutations in H3.1K27M. Histone wild-type subgroups are refined by the presence of key oncogenic events or methylation profiles more closely resembling lower-grade tumors. Genomic aberrations increase with age, highlighting the infant population as biologically and clinically distinct. Uncommon pathway dysregulation is seen in small subsets of tumors, further defining the molecular diversity of the disease, opening up avenues for biological study and providing a basis for functionally defined future treatment stratification.

Indirect cavernous carotid fistula in a 12-year-old girl.

We present a very rare case of indirect cavernous carotid fistula (CCF) in a 12-year-old girl. Indirect CCF is extremely rare in the paediatric population. A 12-year-old girl presented with a 7-month history of frontal headaches and intermittent left-sided proptosis. On examination, she had dilated and engorged scleral veins on the left eye, mild dysdiadochokinesia and past pointing on the left side. A brain computer tomography with contrast, brain magnetic resonance imaging (MRI) and interventional radiography (IR) cerebral angiogram confirmed the diagnosis of CCF. The CCF was embolized and a follow-up brain MRI and an IR cerebral angiogram were conducted over the course of 8 months that revealed no evidence of residual CCF. CCF, though rare in the paediatric population, should be highly considered in the differential diagnosis when dilated scleral veins, proptosis and dysdiadokinesis are present in the clinical setting. Prompt treatment has good prognostic results.