Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review.

INTRODUCTION: Primary intracranial malignant melanoma (PIMM) is an extremely rare primary brain tumor with most cases diagnosed in adults. To date, there are only a few cases reported in the pediatric population. Owing to its infrequency, there are no established guidelines to treat this aggressive neoplasm. Recent insights suggest that PIMM are molecularly different between adults and children, whereby NRAS mutations drive tumor growth in the latter group. We present a unique case of PIMM in a pediatric patient and discuss the case in corroboration with current literature. CASE PRESENTATION: A previously well 15-year-old male presented with progressive symptoms of raised intracranial pressure. Neuroimaging reported a large solid-cystic lesion with significant mass effect. He underwent gross total resection of the lesion that was reported to be a PIMM with pathogenic single nucleotide variant NRAS p.Gln61Lys. Further workup for cutaneous, uveal, and visceral malignant melanoma was negative. A trial of whole-brain radiotherapy followed by dual immune checkpoint inhibitors was commenced. Despite concerted efforts, the patient had aggressive tumor progression and eventually demised from his disease. CONCLUSION: We therein report a case of pediatric PIMM, in the context of the patient's clinical, radiological, histopathological, and molecular findings. This case highlights the therapeutic difficulties faced in disease management and contributes to the very limited pool of medical literature for this devastating primary brain tumor.

Relevance of presenting risks of frailty, sarcopaenia and osteopaenia to outcomes from aneurysmal subarachnoid haemorrhage.

INTRODUCTION: Aneurysmal subarachnoid haemorrhage (aSAH) is a condition with significant morbidity and mortality. Traditional markers of aSAH have established their utility in the prediction of aSAH outcomes while frailty markers have been validated in other surgical specialties. We aimed to compare the predictive value of frailty indices and markers of sarcopaenia and osteopaenia, against the traditional markers for aSAH outcomes. METHODS: An observational study in a tertiary neurosurgical unit on 51 consecutive patients with ruptured aSAH was performed. The best performing marker in predicting the modified Rankin scale (mRS) on discharge was selected and an appropriate threshold for the definition of frail and non-frail was derived. We compared various frailty indices (modified frailty index 11, and 5, and the National Surgical Quality Improvement Program score [NSQIP]) and markers of sarcopaenia and osteopaenia (temporalis [TMT] and zygoma thickness), against traditional markers (age, World Federation of Neurological Surgery and modified Fisher scale [MFS]) for aSAH outcomes. Univariable and multivariable analysis was then performed for various inpatient and long-term outcomes. RESULTS: TMT was the best performing marker in our cohort with an AUC of 0.82, Somers' D statistic of 0.63 and Tau statistic 0.25. Of the frailty scores, the NSQIP performed the best (AUC 0.69), at levels comparable to traditional markers of aSAH, such as MFS (AUC 0.68). The threshold of 5.5 mm in TMT thickness was found to have a specificity of 0.93, sensitivity of 0.51, positive predictive value of 0.95 and negative predictive value of 0.42. After multivariate analysis, patients with TMT ≥ 5.5 mm (defined as non-frail), were less likely to experience delayed cerebral ischaemia (OR 0.11 [0.01 - 0.93], p = 0.042), any complications (OR 0.20 [0.06 - 0.069], p = 0.011), and had a larger proportion of favourable mRS on discharge (95.0% vs. 58.1%, p = 0.024) and at 3-months (95.0% vs. 64.5%, p = 0.048). However, the gap between unfavourable and favourable mRS was insignificant at the comparison of 1-year outcomes. CONCLUSION: TMT, as a marker of sarcopaenia, correlated well with the presenting status, and outcomes of aSAH. Frailty, as defined by NSQIP, performed at levels equivalent to aSAH scores of clinical relevance, suggesting that, in patients presenting with acute brain injury, both non-neurological and neurological factors were complementary in the determination of eventual clinical outcomes. Further validation of these markers, in addition to exploration of other relevant frailty indices, may help to better prognosticate aSAH outcomes and allow for a precision medicine approach to decision making and optimization of best outcomes.

Decompressive surgery in abusive head injury: Experience from a Singapore children's hospital and a review of literature.

PURPOSE: Abusive head trauma (AHT) is a major cause of morbidity and mortality in children. Studies on pediatric head injury observe that AHT patients often have a higher incidence of malignant cerebral oedema and, overall, worse prognosis. There are limited studies with a focus on the outcome of decompressive surgery in children with AHT. This is a study undertaken to review our institutional experience on the role of decompressive surgery in AHT patients and objectively assess its outcomes, in corroboration with current literature. METHODS: This is an ethics-approved, retrospective study. Inclusion criteria consist of all children with a diagnosis of AHT managed by the Neurosurgical Service, KK Women's and Children's Hospital. Demographical and clinical variables are incorporated in the statistical analyses. RESULTS: From 2011 to 2021, a total of 7 patients required decompressive surgery for AHT. Mean age of the cohort was 17.1 months (with the majority of patients being male (n = 5, 71.4%). During the follow-up period, there was 1 mortality (14.3%), 3 patients developed cerebral palsy (42.9%), and 3 patients had post-traumatic epilepsy (42.9%). With regards to functional outcome, 4 patients (57.1%) had a favorable KOSCHI score at 6 months follow-up. CONCLUSION: Decompressive surgery in children with AHT presents with its own unique challenges. We therein present our neurosurgical experience in decompressive surgery for this extremely vulnerable group of patients. Given the potential role of decompressive surgery in AHT, the development of an objective marker to select such patients who may benefit most from intervention should be the way forward.

Closure intracranial pressure is an objective intraoperative determinant of the adequacy of surgical decompression in traumatic acute subdural haematoma: a multicentre observational study.

PURPOSE: Acute subdural haematoma (ASDH) is associated with severe traumatic brain injury and poor outcomes. Although guidelines exist for the decompression of ASDH, the question of adequate decompression remains unanswered. The authors examined the relationship of intracranial pressure (ICP) on closure with outcomes to determine its utility in the determination of adequate ASDH decompression. METHODS: A multicentre retrospective review of 105 consecutive patients with ASDH who underwent decompressive surgery was performed. Receiver operating characteristic (ROC) analysis with internal validation was performed to determine an ICP threshold for the division of patients into the inadequate and good ICP groups. Multivariable analyses were performed for both inpatient and long-term outcomes. RESULTS: An ICP threshold of 10 mmHg was identified with a 91.5% specificity, 45.7% sensitivity, and a positive and negative predictive value of 80.8% and 68.4%. There were 26 patients (24.8%) and 79 patients (75.2%) in the inadequate and good ICP groups, respectively. After adjustment, the inadequate ICP group was associated with increased postoperative usage of mannitol (OR 14.2, p < 0.001) and barbiturates (OR 150, p = 0.001). Inadequate ICP was also associated with increased inpatient mortality (OR 24.9, p < 0.001), and a lower rate of favourable MRS at 1 year (OR 0.08, p = 0.008). The complication rate was similar amongst the groups. CONCLUSIONS: Closure ICP is a novel, objective, and actionable intraoperative biomarker that correlates with inpatient and long-term outcomes in ASDH. Various surgical manoeuvres can be undertaken to achieve this target safely. Large-scale prospective studies should be performed to validate this ICP threshold.

Cerebral X-linked Adrenoleukodystrophy Presenting As Enlarging Cavum Vergae Cyst: A Case Report.

The cavum vergae cyst (CVC) is an uncommon brain malformation. Most patients with CVC are asymptomatic and do not require neurosurgical intervention. Separately, cerebral X-linked adrenoleukodystrophy (X-ALD) is one of the phenotypes of a genetic peroxisomal disorder that is seldom managed by neurosurgeons. We report an unusual case of cerebral X-ALD presenting as an enlarging CVC in a child, and discuss its nuances in corroboration with the literature. A previously well six-year-old male presented with confusion and fever. Urgent neuroimaging demonstrated a large CVC with resultant hydrocephalus. Of note, there were symmetrical areas of signal changes in the periventricular white matter bilaterally involving the corpus callosum, thalami, cerebral peduncles, midbrain, and pons in his MRI. Further investigations performed as part of his medical workup reported high plasma concentrations of very long-chain fatty acids (VLCFA). Put together, a diagnosis of cerebral X-ALD was confirmed. Initially, an external ventricular drain was inserted directly into the CVC under stereotaxy to decompress it. Subsequently, endoscopic fenestration of the CVC was performed as the definitive treatment. He recovered uneventfully from the neurosurgical interventions and proceeded for the treatment of his cerebral X-ALD. To our knowledge, this is the first report of cerebral X-ALD presenting as a CVC in a patient. This case adds to the limited literature for both rare conditions and highlights the importance of a multidisciplinary approach to management.

Intracranial Pressure as an Objective Biomarker of Decompression Adequacy in Large Territory Infarction: A Multicenter Observational Study.

Background: Decompressive craniectomy (DC) improves the survival and functional outcomes in patients with malignant cerebral infarction. Currently, there are no objective intraoperative markers that indicates adequate decompression. We hypothesise that closure intracranial pressure (ICP) correlates with postoperative outcomes. Methods: This is a multicentre retrospective review of all 75 DCs performed for malignant cerebral infarction. The patients were divided into inadequate ICP (iICP) and good ICP (gICP) groups based on a suitable ICP threshold determined with tiered receiver operating characteristic and association analysis. Multivariable logistic regression was performed for various postoperative outcomes. Results: An ICP threshold of 7 mmHg was determined, with 36 patients (48.0%) and 39 patients (52.0%) in the iICP and gICP group, respectively. After adjustment, postoperative osmotherapy usage was more likely in the iICP group (OR 6.32, p = 0.003), and when given, was given for a longer median duration (iICP, 4 days; gICP, 1 day, p = 0.003). There was no difference in complications amongst both groups. When an ICP threshold of 11 mmHg was applied, there was significant difference in the duration on ventilator (ICP ≥11 mmHg, 3-9 days, ICP <11 mmHg, 3-5 days, p = 0.023). Conclusion: Surgical decompression works complementarily with postoperative medical therapy to manage progressive cerebral edema in malignant cerebral infarctions. This is a retrospective study which showed that closure ICP, a novel objective intraoperative biomarker, is able to guide the adequacy of DC in this condition. Various surgical manoeuvres can be performed to ensure that this surgical aim is accomplished.