Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort.

PURPOSE: Substantial evidence exists describing differences between paediatric and adult Chiari 1 malformation (CM1) patients. Differences in clinical presentation between very young (0-6 years old) and older (7-18 years old) paediatric patients is similarly well-established. However, progression on these findings with regard to surgical outcomes is limited. We aimed to establish whether inter-paediatric age group modifies surgical outcome for CM1 decompression. METHODS: Retrospective chart review was conducted for 65 patients receiving posterior fossa decompression between 2006 and 2018. Presenting features, surgical management, and outcome were evaluated and stratified into very young patients (0-6 years) or older patients (7-18 years). Outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS), a validated 16-point framework for comparison. RESULTS: Very young patients (21 patients) scored significantly lower in surgical outcome overall compared with older patients (44 patients) (12.1 ± 3.2/16 vs 14.2 ± 1.6/16, p = 0.011), and across 3/4 CCOS subscores: non-pain symptoms, functionality, and complications. Very young patients also returned to theatre more commonly (47.6% vs 13.6%, p = 0.003), primarily for re-do decompression (7/10 patients, 70%). Finally, the presentation of very young patients differed to older patients with significantly more oropharyngeal (38.1% vs 9.0%, p = 0.014) and motor symptoms (47.6% vs 22.7%, p = 0.042). DISCUSSION: Very young patients (0-6 years) do not appear to respond as well to standard posterior fossa decompression, as their older (7-18 years) paediatric counterparts, in the absence of several baseline cohort characteristic differences. We hypothesise underlying anatomical differences may contribute to this finding.

Brain abscess in a recent immigrant.

A 7-year-old boy who has recently immigrated from India presented with two episodes of focal seizure over a 3-month period. A cranial magnetic resonance imaging scan showed a ring-enhancing lesion in his cerebral cortex. He was diagnosed with a pyogenic brain abscess following resection of the lesion and also had positive serology to Taenia solium (which causes neurocysticercosis). We briefly review these two diseases and discuss the important diagnostic issues.

Access to imaging investigation and neurosurgical care is delayed in regional Queensland for paediatric primary brain tumours.

Central nervous system tumours are the leading oncology cause of paediatric mortality. The aim of this research was to identify stages within the diagnostic process of a primary paediatric brain tumour that could be improved resulting in better outcomes. METHODS: The electronic medical records of Queensland Children's Hospital patients with central nervous system tumours between the 17/12/2014 till 11/12/2019 were retrospectively accessed. Time intervals of symptom onset to first medical review,location, time till medical imaging,subspecialty or neurosurgical review, timing of surgery, diagnosis and mortality status were recorded then analysed. RESULTS: A total of 168 patients were included. Mean age to 7.5, 65% male, with pilocytic astrocytoma representing 31%. 71.4% of the population were from a major city as determined by Remoteness Area classification, ABS, with 19% inner regional and 9.5% being outer regional and remote. The average time from first medical review to diagnostic imaging was significantly different when comparing remoteness classification (p = 0.044). There was also a statistically significant difference in the duration of time from medical imaging to specialist review comparing major city and outer regional/remote (p = 0.016) and inner regional versus outer regional/remote areas (p = 0.026). CONCLUSIONS: Delays in imaging in outer regional and remote Queensland are contributing to a delay in diagnosis and intervention in paediatric brain tumours. Service provision for neurosurgery in outer regional and remote Queensland is currently on par with inner regional and city areas. Suspicion of paediatric brain tumours is needed with clear referral pathways for general practitioners to access diagnostic imaging.

Multifocal Gorham-Stout disease associated with Chiari I malformation and recurrent aseptic meningitis: Case report and review of literature.

Gorham-Stout disease is a rare condition of uncertain aetiology characterised by lymphatic proliferation within osseous structures and subsequent massive osteolysis. This report describes the index case of a patient with multifocal Gorham-Stout disease involving the skull base with Chiari I malformation and recurrent aseptic meningitis without fistula. A five-year-old male presented following decompression of a Chiari I malformation with headaches, vomiting, and stiff neck and cerebrospinal fluid pleocytosis without growth of a pathogenic organism. Ongoing symptoms prompted a further three presentations over several months revealing persistent aseptic cerebrospinal fluid monocytic pleocytosis. Further investigation revealed multifocal osseous cystic disease and subsequent bone biopsy suggested Gorham-Stout disease. Suboccipital decompression was not repeated despite craniocervical junction re-stenosis. A literature review demonstrated the extreme rarity of Gorham-Stout disease associated with Chiari I malformation and meningitis. Potential mechanisms of these entities occurring in concert are discussed. Consideration of Gorham-Stout disease as a secondary cause for Chiari I malformation is important amid local bone changes or cerebrospinal fluid leakage prior to pursuing suboccipital decompression considering the poor outcomes reported.

Traumatic brain injuries in a paediatric neurosurgical unit: A Queensland experience.

Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in children with a broad injury spectrum and associated continuum in the level of care required. A dearth of data exists regarding children requiring inpatient neurosurgical admission following TBI. A retrospective study of children 0-16 years-old admitted to the neurosurgical unit of a level-1 paediatric trauma hospital in Queensland, Australia following TBI was conducted focusing on the demographics, clinical characteristics, and management of these patients to guide those involved in their management, and identify areas for improvement in injury prevention and trauma system management. Over 48 months, 671 patients were identified (62.6% male) with median age 5.0 years, the majority transferred from peripheral centres. Falls (47.2%) and traffic accidents (21.5%) were the most common mechanisms. Non-displaced skull fracture was the most common injury. Moderate or severe TBI (GCS 3-12) was seen in 14.8% of whom were more likely to require surgery, intensive care, or suffer polytrauma. Clinically significant TBI, defined as moderate/severe TBI, polytrauma, death, requiring neurosurgery, intensive care admission, intubation, or admission three or more nights was detected in 57.97% with higher rates in transferred patients (62.9%) versus primary presentations (50.6%). Mechanisms involving low kinetic forces especially low-height falls and children with non-surgical pathology were less likely to meet criteria for clinically significant TBI. Opportunity exists to optimise triage and transfer practices within the trauma network to minimise the economic and social implications of over-triage with many children requiring only brief observation.

Chiari I malformation with significant motor and autonomic dysfunction in an infant.

Quadriparesis and cardiovascular instability are not symptoms commonly associated with Chiari I malformation diagnoses, less so in a 3-month-old toddler. Chiari I malformations have a wide array of symptoms, varying with regard to both the nature of onset and the age of the patient presenting. This variation is illustrated in this report, presenting a novel description of a set of symptoms in a particularly young patient, a case lacking the common insidious nature of Chiari I malformation symptoms. Acute onset of cardiovascular instability, global hypertonia, hyperreflexia, and proximal upper limb weakness in a 3-month-old toddler are discussed. Parallels between the presented case and previous case reports are few, however some key features are distinguishable among similar cases. A successful posterior fossa craniectomy allowed the acutely unwell toddler to become a healthy, fully functional child. We believe this case contributes to elucidating the rare presentations of a curious pathology.

Nonaccidental head injuries in children: a Sydney experience.

OBJECT: The purpose of this study was to evaluate the demographics, clinical and radiological features, and clinical outcomes of nonaccidental pediatric head injury. METHODS: The authors reviewed 65 consecutive cases of nonaccidental head injury in a single pediatric neurosurgical unit during a period of 7 years. The mean patient age was 8.2 months (range 0.5-46 months). There were 39 boys and 26 girls. A history of abuse was present in 24% of families. There was a high incidence of family disruption, substance abuse, and premature birth. Fathers were the most common perpetrators. Fifteen patients had a Glasgow Coma Scale score of less than 10. Thirty-five patients had seizures on or preceding admission. Subdural hematoma was the most common finding (81.5%). Skull fractures were present in 36.9% of patients, skeletal injuries in 50% (of which 67% were subclinical), and retinal hemorrhages in 59%. The radiological finding of ischemia or edema had a significant correlation with a poor outcome. Magnetic resonance imaging revealed additional pathological findings not visible on computerized tomography scanning in 18 (49%) of 37 cases. Surgery was performed in 17 patients; recurrence of the subdural collection occurred in 46% of them. In this group, reevacuations were followed by further recurrences, and a subdural-peritoneal shunt was eventually required. Four patients died. Of the 56 surviving patients reviewed on a long-term basis, 19 made a full recovery, and epilepsy was reported in 17%. CONCLUSIONS: Magnetic resonance imaging should be routinely used in depicting ischemia, which is associated with a poor outcome. The high incidence of subclinical skeletal injuries stresses the importance of assessment of suspected cases of nonaccidental trauma with skeletal surveys and bone scans. Recurrence of subdural collection following burr hole drainage is common and is best treated with a subdural-peritoneal shunt.

Aqueduct stenosis-?Benign.

'Benign' aqueduct stenosis is a common cause of hydrocephalus in the paediatric population and is frequently treated by endoscopic third ventriculostomy. Occasionally, aqueduct stenosis can be a prelude to the development of other pathology, as is seen in these two cases of pineal tumours developing in patients whose hydrocephalus was successfully treated with endoscopic third ventriculostomy. The case histories are presented, along with the recommendation for increased radiological screening of patients with this usually 'benign' presentation.

Traumatic spinal epidural hematoma-unusual cause of torticollis in a child.

Traumatic spinal epidural hematoma is rare in children. The presentation could be nonspecific, leading to a delay in diagnosis. We present an infant who sustained an epidural hematoma after a "trivial" injury. The delay in diagnosis led to minor neurological deficit at 6-week follow-up. Irritability and torticollis after a neck trauma in an afebrile child should alert to the possibility of spinal cord compression. Early imaging and early decompression will minimize morbidity.

Spinal cord detethering procedures in children: a 5 year retrospective cohort study of the early post-operative course.

Tethered spinal cord can cause neurological, orthopaedic and sphincteric problems in children and detethering surgery may prevent or reverse these problems. This 5 year retrospective cohort study aimed to review our experience of detethering surgery at The Children's Hospital at Westmead, Sydney, Australia, particularly examining the early post-operative complications of this procedure. Between 2007 and 2012, 61 children underwent 63 detethering procedures. The median age at detethering surgery was 1.4 years old (interquartile range: 0.7-5.6 years). Fifty-five children (90.1%) had lumbosacral procedures, 31 (50.8%) were asymptomatic from tethering, 11 (18.0%) had motor or gait disturbance, 11 (18.0%) sphincteric disturbance, eight (13.1%) lower limb orthopaedic deformities, eight (13.1%) scoliosis, six (9.8%) back or leg pain and two (3.3%) sensory disturbance. The most common tethering pathologies were spinal lipomas in 32 children (52.5%), filum abnormalities in 23 (37.7%), dorsal sinus tracts in eight (13.1%) and diastematomyelia in seven (11.5%). Twenty-six children (42.6%) had either a syrinx or central canal dilatation preoperatively. The most common complications were wound infection and cerebrospinal fluid leak. Six children (9.8%) required reoperation for wound issues and two patients (3.3%) required subsequent reoperation for cord retethering during the study period. There were no deaths and no new neurological deficits. Of the children with the above preoperative deficits, 26.7% were documented to have improvement or resolution of their symptoms post-operatively. The highest rate of improvement occurred in children with motor or gait disturbance (36.4%) or sphincteric disturbance (27.3%).

Subtemporal decompression for slit-ventricle syndrome: successful outcome after dramatic change in intracranial pressure wave morphology. Report of two cases.

Subtemporal decompression is recognized as an effective treatment for slit-ventricle syndrome; however, the effects of this procedure have not been demonstrated using both pre- and postsurgical intracranial pressure (ICP) monitoring. The authors report two cases in which slit-ventricle syndrome and elevated ICP had been diagnosed. Each patient underwent ICP monitoring before and after subtemporal decompression; the dramatic changes in the ICP measurements are presented along with findings from 1-year follow-up examinations.

Epidemiology of traumatic head injury from a major paediatric trauma centre in New South Wales, Australia.

BACKGROUND: Traumatic brain injury (TBI) is common and is a leading cause of presentations to emergency departments. Understanding the epidemiology of TBI can aid in improving overall management and identifying opportunities for prevention. Currently, there is a paucity of data on paediatric TBI in NSW. The purpose of this study was to determine the demographics, causes, treatment and outcome of TBI at The Children's Hospital at Westmead (CHW), a large trauma referral paediatric hospital. METHODS: A retrospective chart review was conducted of patients admitted to CHW emergency from 2006 to 2011 with a TBI. Patients who presented to the emergency department and had a history of TBI with either symptoms of concussion and/or positive computed tomography (CT) findings of head injury were selected. Information regarding demographics, injury pattern, CT findings, treatment and outcome were retrieved. RESULTS: Over the 6-year period, there were 1489 presentations at the CHW. Of these, 65% were male and 35% were female. The mean age was 7 years. A total of 93% were classified as mild, 1.5% as moderate and 5.5% as severe. Sports and recreational injuries accounted for 26% of all TBI presentations, while motor vehicle accidents (MVAs) accounted for 77% of all TBI deaths. Sixty-two per cent of children underwent a CT brain, and of those, 40% were normal. CONCLUSION: The majority of TBI are mild in nature, with younger children and males at greatest risk. There was a low rate of operative intervention and a high rate of good outcomes. Many injuries may be preventable with the adaptation of better public health education programmes, particularly in very young children and those related to MVAs.

Paediatric brain tumours treated at a single, tertiary paediatric neurosurgical referral centre from 1999 to 2010 in Australia.

Paediatric brain tumours are the most common solid tumour of childhood and the most common cancer cause of death among children. A retrospective review of 313 histopathologically proven brain tumours over an 11-year period has been performed at the Children's Hospital Westmead, New South Wales, Australia, to determine proportions and locations of different tumours, age distribution, survival rates and usage of various treatment modalities. Pilocytic astrocytoma was the most common paediatric brain tumour (29%) followed by medulloblastoma (12%) and ependymoma (6%). Most tumours were histologically benign (59%), and 42% of tumours were located in the posterior fossa. The average age at diagnosis was 7.9 years. About 50% of children were treated with surgery alone, whereas the other 50% had surgery or biopsy plus adjuvant treatment. The overall one-year survival rate was 89% and the five-year survival rate was 80%. The five-year survival rates for pilocytic astrocytoma was 91%; medulloblastoma, 75%; ependymoma, 82%; and high grade glioma, 15%. Thus, a large proportion of paediatric brain tumours were histologically benign and were treated with surgery alone, but a subset of benign tumours required adjuvant treatment and were associated with mortality (25%). The overall survival rates were high and are improving, although for some tumours, such as high grade glioma, the outlook remains poor.

Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome.

Desmoid-type fibromatosis, aggressive fibromatosis, or desmoid tumor is an uncommon benign but locally aggressive fibroblastic lesion. Although intraabdominal desmoid-type fibromatoses are well described in association with adenomatous polyposis syndrome, their occurrence along the neuraxis is extremely rare. The authors report the case of a 14-year-old boy with metachronous intracranial and spinal desmoid-type fibromatoses with preceding medulloblastoma. He was ultimately diagnosed with adenomatous polyposis syndrome. This is the first reported case of spinal desmoid-type fibromatosis in association with adenomatous polyposis syndrome. The identification of an underlying genetic instability allows for screening to detect lesions and institute measures to avoid preventable mortality from nonneurological tumors.