Complex lumbosacral spinal cord lipomas: A longitudinal study on outcomes from a Singapore children's hospital.

BACKGROUND: Total/near-total resection (TR/NTR) of complex lumbosacral lipomas (CSL) is reported to be associated with better long-term functional outcomes and lower symptomatic re-tethering rates. We report our institutional experience for CSL resection in affected children. METHODS: This is a single-institution, retrospective study. Inclusion criteria consist of patients with CSL with dorsal, transitional and chaotic lipomas based on Pang et al's classification. The study population is divided into 2 groups: asymptomatic patients with a normal preoperative workup referred to as 'prophylactic intent' and 'therapeutic intent' for those with pre-existing neuro-urological symptoms. Primary aims are to review factors that affect post-operative clean intermittent catheterization (CIC), functional outcomes based on Necker functional score (NFS), and re-tethering rates. RESULTS: 122 patients were included from 2000 to 2021. There were 32 dorsal lipomas (26.2 %), 74 transitional lipomas (60.7 %), and 16 chaotic lipomas (13.1 %). 82 % patients achieved TR/NTR. Favourable NFS at 1-year was 48.2 %. The re-tethering rate was 6.6 %. After multivariable analysis, post-operative CIC was associated with median age at surgery (p = 0.026), lipoma type (p = 0.029), conus height (p = 0.048) and prophylactic intent (p < 0.001). Next, extent of lipoma resection (p = 0.012) and the post-operative CSF leak (p = 0.004) were associated with re-tethering. Favourable NFS was associated with lipoma type (p = 0.047) and prophylactic intent surgery (p < 0.001). CONCLUSIONS: Our experience shows that TR/NTR for CSL is a feasible option to prevent functional deterioration and re-tethering. Efforts are needed to work on factors associated with post-operative CIC.

Withdrawal notice to Spinal dysraphism-perspectives from a Southeast Asian paediatric neurosurgical unit: WNEU, 173 (2023) 20134.

The Publisher regrets that this article is an accidental duplication of an article that has already been published in WNEU, 172 (2023) 20066, https://doi.org/10.1016/j.wneu.2023.01.070. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/policies/article-withdrawal.

Fibrofatty filum terminale: long-term outcomes from a Singapore children's hospital.

OBJECTIVE: The role of prophylactic detethering a fibrofatty filum terminale (FFT) remains equivocal. Furthermore, long-term studies focusing on urological outcomes are sparse. The aims of this study were to present an institutional experience on the perioperative and long-term outcomes of FFT surgery and to assess for factors that contribute to postoperative clean intermittent catheterization (CIC). METHODS: This was a single-institution, retrospective study conducted over a 20-year period. Patients younger than 19 years of age who underwent surgery for FFT were included. Variables of interest included patient demographics, clinical presentation, radiological findings, postoperative complications, and long-term need for CIC. Outcomes were measured using the Necker functional score and modified Hoffer Functional Ambulation scale score at 3, 6, and 12 months postdischarge. RESULTS: A total of 164 surgeries were performed for FFT from 2000 to 2020. The median age at surgery was 1.1 years, and the mean follow-up duration was 8.3 years. There were 115 patients (70.1%) who underwent prophylactic-intent surgery and 49 patients (29.9%) who underwent therapeutic-intent surgery. The proportion of therapeutic-intent surgeries increased significantly with age percentiles (0-20th, 21.9%; 20th-40th, 9.1%; 40th-60th, 18.2%; 60th-80th, 36.4%; and 80th-100th, 63.6% [p < 0.001]). Thirty patients (18.3%) had an associated syndrome, the most common (n = 19, 11.6%) being VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities). Forty-eight patients (29.3%) had an associated malformation (anorectal anomaly = 37, urogenital anomaly = 16, and sacral anomaly = 3). Cutaneous manifestation was the most common presentation (n = 96, 58.5%), followed by lower-limb neurological deficits (n = 21, 12.8%). A low-lying conus was present in 36.0% of patients (n = 59), and 16.5% had an associated syrinx (n = 27). There were 26 patients (18.8%) with an abnormal preoperative urodynamic study. Three patients (1.8%) had postoperative complications that required repeat surgery. There were no cases of CSF leakage. One patient (0.6%) developed retethering requiring another surgery. Postoperative CIC was required in 11 patients (6.7%). Multivariable analyses showed that an abnormal preoperative urodynamic study (adjusted OR 5.5 [95% CI 1.27-23.9], p = 0.023) and having an intraspinal syrinx (adjusted OR 5.29 [95% CI 1.06-26.4], p = 0.042) were associated with the need for CIC. CONCLUSIONS: The authors' results demonstrate that detethering surgery for FFT is a relatively safe procedure and can be performed prophylactically. Nonetheless, the risks of postoperative CIC should be emphasized during the preoperative counseling process.

Tethered cord secondary to focal nondisjunction of the primary neural tube: experience from a Singapore children's hospital.

PURPOSE: Tethered cord due to focal nondisjunction of primary neuralisation (FNPN) is a rare form of spinal dysraphism. We present our institutional experience in managing children diagnosed with FNPN. MATERIALS AND METHODS: This is a single institution, retrospective study approved by the hospital ethics board. Patients below 18 years of age diagnosed with CDS, LDM or their mixed lesions, and subsequently underwent intervention by the Neurosurgical Service, KK Women's and Children's Hospital, are included. RESULTS: From 2001 to 2021, 16 FNPN patients (50% males) were recruited. Eight of them had CDS (50.0%), seven had LDM (43.8%), and one patient had a mixed CDS and LDM lesion (6.2%). The average duration of follow up was 5.7 years and the mean age of surgery was 6 months old. Thirteen patients underwent prophylactic intent surgery (81.2%) and three had therapeutic intent surgery (18.8%). All patients did not have new neurological deficit or required repeat surgery for cord retethering. We observed that detethering surgery performed at or less than three months old was associated with having a wound infection (p = .022). CONCLUSIONS: Our study reports that early recognition and timely intervention are mainstays of management for FNPN. We advocate a multi-disciplinary approach for good 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.

Monomodality versus Combined Therapy in Optic Pathway Gliomas-20-Year Experience from a Singapore Children's Hospital.

Introduction: The treatment of pediatric optic pathway gliomas (OPG) is challenging. At present, most centers provide individualized treatment to maximize progression free survival (PFS) and minimize morbidity. We aim to report our experience in the management of pediatric OPG, and investigate factors associated with an increased duration of remission after treatment. Methods: This is a single-institution study approved by the hospital ethics board. A retrospective review of consecutive OPGs managed from 2000 to 2020 was performed. Patients were divided into those managed with monomodality treatment (MT) and those who received combined therapy (CT). MT included various forms of surgery, chemotherapy and radiotherapy given alone, while CT involves a combination of surgery and adjuvant chemotherapy and/or radiotherapy. Results: Twenty-two patients were selected for this study. They had 40 treatment cycles; and a total follow up duration of 194.8 patient-years. Most of them were male (63.6%) and presented with visual deficits (72.7%). The mean age at initial presentation was 65 months and majority (86.4%) had their tumors arising directly from the optic chiasm, with 77.3% with hypothalamic extension. One patient had Neurofibromatosis type I (4.5%). The most common histological diagnosis was pilocytic astrocytoma (90.9%), followed by pilomyxoid astrocytoma (9.1%). The 5- and 10- year PFS were 46.2% and 36.4% respectively, while the 5- and 10-year OS were both 100%. When accounting for treatment type, there were 24 treatment cycles with MT (60.0%) and 16 CT (40.0%). After adjustment, treatments with MT were shown to have a shorter mean duration of remission (MT: 45 ± 49, CT: 84 ± 79 months; p = 0.007). Cox regression curve plotted after adjusting for patient's age at treatment demonstrated a significantly longer PFS in the CT group (p = 0.037). Conclusions: Our results suggest a significant survival benefit of CT over MT for affected patients due to the prolonged the duration of disease remission, for both primary and subsequent treatments. Nonetheless, we acknowledge that our study reflects the outcomes of treatment strategies that have evolved over time. We emphasize the need for collective efforts from a dedicated multidisciplinary team and international collaborations for better disease understanding.

Paediatric brain tumours in Singapore: A 15-year epidemiological and outcome study.

Paediatric brain tumours (PBTs) are the most common solid tumours in children. Previous publications reflect variations in incidence rates and frequency of histological types in different global populations. However, there are limited studies on the epidemiology of PBTs in Singapore. This study aims to summarise the epidemiology of paediatric brain tumours managed in Singapore. This is an ethics-approved retrospective study of all patients below 19 years old diagnosed with PBTs managed by Singapore's 2 tertiary paediatric neurosurgical centres, KK Women's and Children's Hospital (KKH) and the National University Hospital (NUH) over a 15-year period from 01 January 2002 to 31 December 2017. Data collected was analysed for age, gender, tumour characteristics, presenting complaints, location, treatment modalities, 1-year and 5-year overall survival (OS). A total of 396 patients were included. The mean age of diagnosis was 7.05 years (0.25-18; ± 4.83) and male-to-female ratio was 1.41:1. Top histological groups were astrocytic (30.6%), embryonal (26.0%), germ cell (11.1%), ependymoma (30, 7.58%) and craniopharyngioma (27, 6.82%). Outcomes included recurrence rate (31.2%), 1-year OS (89.5%) and 5-year OS (72.2%). Poorer 5-year OS were noted in embryonal tumours (47.0%; p < 0.001) and ependymoma (50.0%; p = 0.0074) patients. Of note, the following cohorts also had poorer OS at 5 years: supratentorial tumours (76.2%; p = 0.0426), radiotherapy (67.4%; p = 0.0467) and surgery (74.9%, HR; p < 0.001). Overall, our data reflects patient demographics, presenting complaints, treatment modalities and survival outcomes, that are comparable to other international paediatric neurosurgical centres.

Programmable Shunt Valves for Pediatric Hydrocephalus: 22-Year Experience from a Singapore Children's Hospital.

(1) Background: pediatric hydrocephalus is a challenging condition. Programmable shunt valves (PSV) have been increasingly used. This study is undertaken to firstly, to objectively evaluate the efficacy of PSV as a treatment modality for pediatric hydrocephalus; and next, review its associated patient outcomes at our institution. Secondary objectives include the assessment of our indications for PSV, and corroboration of our results with published literature. (2) Methods: this is an ethics-approved, retrospective study. Variables of interest include age, gender, hydrocephalus etiology, shunt failure rates and incidence of adjustments made per PSV. Data including shunt failure, implant survival, and utility comparisons between PSV types are subjected to statistical analyses. (3) Results: in this case, 51 patients with PSV are identified for this study, with 32 index and 19 revision shunts. There are 3 cases of shunt failure (6%). The mean number of adjustments per PSV is 1.82 times and the mean number of adjustments made per PSV is significantly lower for MEDTRONIC™ Strata PSVs compared with others (p = 0.031). Next, PSV patients that are adjusted more frequently include cases of shunt revisions, PSVs inserted due to CSF over-drainage and tumor-related hydrocephalus. (4) Conclusion: we describe our institutional experience of PSV use in pediatric hydrocephalus and its advantages in a subset of patients whose opening pressures are uncertain and evolving.

Paediatric arachnoid cysts: Surgical outcomes from a Singapore children's hospital.

Arachnoid cysts (AC) are reported to have a prevalence of up to 2.6% in children. Most AC remain indolent, but others may expand or rupture to cause life-threatening symptoms of raised intracranial pressure. Currently, there are 2 controversial topics with regards to the management of ACs: the indications for surgery and the choice of surgical procedure. We therein report our institution's neurosurgical experience for symptomatic AC over a 22-year period and corroborate our results with published literature. This is a single institution, retrospective study conducted at KK Women's and Children's Hospital from 01 January 1998 to 31 December 2019. A total of 38 patients with ACs that required surgery were recruited. The 3 most common anatomical locations were in the middle cranial fossa (40.5%), posterior fossa (24.3%) and interhemispheric (13.5%). Typical clinical presentations included symptoms of raised intracranial pressure (34.2%), obstructive hydrocephalus (28.9%) and AC rupture (21.1%). Surgical approaches included 17 craniotomy-based procedures, 7 endoscopic fenestrations, 11 cystoperitoneal shunts, 2 burrhole drainage operations and 1 excision of spinal AC. Thirteen patients (34.2%) underwent either another operation due to the lack of resolution of their AC-related symptoms or secondary to complications directly related to their initial surgery. Average length of followup from time of first operation was 84.1 months. Overall, our results demonstrate similarities in epidemiology, clinical presentation and surgical experience, in comparison to larger cohort studies. We advocate collaborative efforts to better understanding of the pathophysiology of paediatric ACs, particularly for deciding between the various surgical treatment modalities.

Cerebrospinal fluid cytokines in metastatic group 3 and 4 medulloblastoma.

BACKGROUND: Metastatic medulloblastoma (MB) portends a poor prognosis. Amongst the 4 molecular subtypes, Group 3 and Group 4 patients have a higher incidence of metastatic disease, especially involving the neuroaxis. At present, mechanisms underlying MB metastasis remain elusive. Separately, inflammation has been implicated as a key player in tumour development and metastasis. Cytokines and their inflammation-related partners have been demonstrated to act on autocrine and, or paracrine pathways within the tumour microenvironment for various cancers. In this study, the authors explore the involvement of cerebrospinal fluid (CSF) cytokines in Group 3 and 4 MB patients with disseminated disease. METHODS: This is an ethics approved, retrospective study of prospectively collected data based at a single institution. Patient clinicpathological data and corresponding bio-materials are collected after informed consent. All CSF samples are interrogated using a proteomic array. Resultant expression data of selected cytokines are correlated with each individual's clinical information. Statistical analysis is employed to determine the significance of the expression of CSF cytokines in Group 3 and 4 patients with metastatic MB versus non-metastatic MB. RESULTS: A total of 10 patients are recruited for this study. Median age of the cohort is 6.6 years old. Based on Nanostring gene expression analysis, 5 patients have Group 3 as their molecular subtype and the remaining 5 are Group 4. There are 2 non-metastatic versus 3 metastatic patients within each molecular subtype. Proteomic CSF analysis of all patients for both subtypes show higher expression of CCL2 in the metastatic group versus the non-metastatic group. Within the Group 3 subtype, the MYC-amplified Group 3 MB patients with existing and delayed metastases express higher levels of CXCL1, IL6 and IL8 in their CSF specimens at initial presentation. Furthermore, a longitudinal study of metastatic Group 3 MB observes that selected cytokines are differentially expressed in MYC-amplified metastatic Group 3 MB, in comparison to the non-MYC amplified metastatic Group 3 MB patient. CONCLUSION: This study demonstrates higher expression of selected CSF cytokines, in particular CCL2, in metastatic Group 3 and 4 MB patients. Although our results are preliminary, they establish a proof-of-concept basis for continued work in a larger cohort of patients affected by this devastating disease.

Extraneural metastatic paediatric glioblastoma: Case report and literature review.

The incidence of paediatric glioblastoma is uncommon in comparison to their adult counterpart. Even more infrequent are extraneural metastases in glioblastoma. A previously well 14-year-old female presented with progressive right hemiparesis secondary to a left fronto-temporal lobe glioblastoma (WHO IV). She underwent successful gross total resection for her brain tumour. Prior to commencement of her adjuvant treatment, she developed tumour recurrence associated with intra-lesional haemorrhage. Although she underwent a second surgery, the patient developed bilateral malignant pleural effusion secondary to extraneural pulmonary metastases. Early awareness of its existence enables prompt diagnosis for this devastating disease. The authors emphasize the urgent need for international collaborations to work together for children affected by this challenging brain tumour.

Paediatric meningiomas in Singapore - Case series of a rare entity.

Paediatric meningiomas are extremely rare. These tumours constitute only 2 to 3% of all childhood brain tumours. Despite similarities in histological features between PMs and their adult counterparts, there are important distinctions between them. In this case series, the authors describe their experience in paediatric meningiomas in Singapore's 2 children's hospitals from 1998 to 2018. The primary aim of this retrospective study is to evaluate the clinical, radiological and pathological characteristics, and associated outcomes of paediatric patients diagnosed with meningioma managed in our local institutions. Following that, the study's findings are secondary aims are corroborated with published literature. A total of 10 patients (4 males and 6 females) were identified for this study within the period of 01 January 1998 to 31 December 2018. Their ages ranged from 1 year old to 18 years old (median age 10.5 years old). Two of the patients had NF1 and NF2 respectively. There were 9 intracranial and 1 intraspinal paediatric meningiomas. Seven patients achieved gross total resection and 3 patients had subtotal resection. Eight patients did not have tumour recurrence or increase in size of tumour remnant during the course of their follow-up. In congruency with the literature, up to 40% of our patients had higher grade meningiomas and 55.6% had large tumour volumes more than 30 cm3. Owing to the paucity of knowledge for this unusual tumour, the authors emphasize the need for closer surveillance and in-depth genomic studies to identify novel therapies for this challenging condition.

Sporadic Meningioangiomatosis Presenting as a Middle Cranial Fossa Arachnoid Cyst.

BACKGROUND: Meningioangiomatosis is an extremely rare meningovascular disease of the central nervous system that is characterized by the proliferation of leptomeninges, cortical vessels, and perivascular spindled cells. Although it is a benign, neoplastic disorder that carries a good prognosis after surgical excision, initial diagnosis may be challenging as radiologic findings are often variable and nonspecific. CASE DESCRIPTION: In this report, we describe an unusual presentation of meningioangiomatosis presenting as a symptomatic middle cranial fossa arachnoid cyst. CONCLUSIONS: In view of the unexpected diagnosis and infrequency of this condition, the case is discussed in collaboration with current literature and management strategies.

Use of an Offsite Intraoperative MRI Operating Theater for Pediatric Brain Tumor Surgery: Experience from a Singapore Children's Hospital.

BACKGROUND: Intraoperative magnetic resonance imaging (iMRI) has been recognized as a useful adjunct for brain tumor surgery in pediatric patients. There is minimal data on the use of an offsite intraoperative magnetic resonance imaging operating theater (iMRI OT), whereby vehicle transfer of patients is involved. The primary aim of this study is to validate the feasibility of perioperative patient transfer to use an offsite iMRI OT for patients with pediatric brain tumor. Secondary objectives include the assessment of tumor resection efficacy and perioperative outcomes in our patient cohort. METHODS: This is a retrospective, single-institution clinical study of prospectively collected data from Singapore's largest children hospital. Variables of interest include issues encountered during interhospital transfer, achievement of surgical aims, length of stay in hospital, and postoperative complications. Our findings were compared with results of related studies published in the literature. RESULTS: From January 1, 2009 to December 31, 2018, a total of 35 pediatric operative cases were performed in our offsite iMRI OT. Within this cohort, 24 of these were brain tumor surgery cases. For all the patients in this study, use of the iMRI OT influenced intraoperative decisions. Average ambulance transport time from parent hospital to the iMRI OT was 30.5 minutes, and from iMRI OT back to the parent hospital after surgery was 27.7 minutes. The average length of hospitalization stay was 7.9 days per patient. There were no ferromagnetic accidents during perioperative iMRI scanning and no airway/hemodynamic incidents in patients encountered during interhospital transfer. CONCLUSIONS: In our local context, the use of interhospital transfers for access to iMRI OT is a safe and feasible option in ensuring good patient outcomes for a select group of patients with pediatric brain tumors.

Right middle cerebral artery infarct after minor head trauma in an infant: Case report and literature review.

Ishaemic stroke (IS) in the paediatric population is extremely rare. In this age group, the occurrence of IS often concurs with underlying congenital heart disease, haematological, metabolic or immunological conditions. In contrast, the association between IS and minor head injury in children has been sparse in current literature. The authors report a case of a healthy 9-month-old male who was found to have a right middle cerebral artery territory infarct after a minor head injury. An extensive medical workup was performed, and it was negative for any previously undiagnosed co-morbidities. Given the paucity of such cases, the condition and its management are discussed in corroboration with current literature.

The Seow Operative Score (SOS) as a decision-making adjunct for paediatric Chiari I malformation: a preliminary study.

PURPOSE: The natural history of Chiari I malformation (C1M) in the paediatric population is poorly understood. There are conflicting reports with regards to surgical indications, operative techniques and peri-operative prognostic indicators. In this paper, we report our institutional experience in the management of paediatric C1M. METHODS: The workflow process which includes preferred imaging modalities, surgical techniques and indications for intervention is discussed. In particular, we describe one of our current projects-an in-house designed Seow Operative Score (SOS) as a feasibility scoring system for neurosurgical intervention in our local cohort of paediatric C1M patients. RESULTS: In our series, we have 2 groups: 10 non-operated patients versus 19 operated patients. In the non-operated group, the majority of patients had a SOS of 0 to 1. One patient had a score of 2.5 and was kept under close surveillance. Follow-up imaging demonstrated resolution of the cerebellar herniation and intraspinal syrinx. In the operated group, 17 patients had a SOS of 3 or more. Two patients had a SOS of 2. For these 2, 1 developed progressive symptoms, and the other had an extensive cervico-thoracic syrinx. Decision was made for surgery after a period of surveillance. CONCLUSIONS: In this paper, we report our institutional experience in managing paediatric C1M and, at the same time, highlight salient points of our practices. Meanwhile, we advocate collective global efforts and in-depth research for better disease understanding of this challenging condition.

Giant Aneurysm Arising from Anomalous Branch of the Middle Cerebral Artery in a Pediatric Patient: Case Report and Review of the Literature.

BACKGROUND: Pediatric intracranial aneurysms are extremely rare. In this age group, cerebral vascular anomalies have been associated with the development of intracranial aneurysms. CASE DESCRIPTION: We present a case of a previously well 11-year-old boy who presented with seizures secondary to a giant, unruptured, and partially thrombosed right middle cerebral artery (MCA) aneurysm. Extensive workup for underlying infective and autoimmune etiology was negative. Of interest, this vascular lesion was found to originate from an anomalous M2 branch, which ran an aberrant parallel course within the Sylvian fissure to the main and distally bifurcating MCA. The patient underwent successful surgical clipping and excision of the giant aneurysm. CONCLUSIONS: Because of the infrequency of the diagnosis, clinical presentation, and its unique neurovascular anatomy, the management of this case is discussed in corroboration with current literature. In addition, highlighting this unusual case in an individual adds to the growing body of literature for better disease understanding, especially in the pediatric population.

Spontaneous Intracranial Hypotension Presenting As Cervicogenic Headache: Case Report and Review of Literature.

BACKGROUND: Spontaneous intracranial hypotension is uncommon. There is a lack of understanding of its exact pathophysiology along with significant variability in its clinical management. CASE DESCRIPTION: The authors report the case of a previously well man with cervicogenic headache associated with magnetic resonance imaging features of intracranial hypotension. The salient features of this case include the details of various treatments prescribed for his condition and the therapeutic difficulties encountered. CONCLUSION: Owing to the infrequency of the diagnosis, the challenges of clinical management for this patient are discussed in relationship to current literature. Next, the highlights of this unusual case in an individual add to the growing body of literature reports for better understanding of this disease.