Abusive head injury in the very young: outcomes from a Singapore children's hospital.

PURPOSE: Abusive head trauma (AHT) is a serious problem in children. The aims of this study are to identify risk factors that correlate with outcomes for those requiring neurosurgical intervention for very young children with AHT, assessment of variables associated with outcomes, and corroboration of our results with literature. METHODS: This is an ethics-approved, retrospective study. Inclusion criteria consisted of patients aged 2 years old or less 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. Logistic regression was applied to statistically significant variables for the risk prediction model. RESULTS: From 2000 to 2020, 24 patients required surgery for AHT. Timepoint was set at 12 months post-diagnosis. Univariate analyses demonstrated that patients with mild TBI were likely to have a favourable GOS-E Peds (p = 0.01), whereas radiological presence of cerebral oedema (p < .001), development of scar epilepsy (p = 0.021), and progression to cerebral palsy (p = 0.001) were associated with unfavourable GOS-E Peds. CONCLUSION: This is the first study focused on neurosurgical outcomes for very young children with AHT in Singapore. We advocate multidisciplinary efforts to improve outcomes for this devastating condition.

Long-term outcomes in children with moderate to severe traumatic brain injury: a single-centre retrospective study.

Traumatic brain injury (TBI) is a significant cause of mortality and disability in the pediatric population. Non-accidental trauma (NAT) has specifically been reported to result in more severe injury as compared to accidental mechanisms of injury. We aim to investigate the long-term neurological outcomes in children with moderate to severe traumatic brain injury. Our secondary aim is to evaluate the difference in outcomes between children presenting with NAT and non-NAT, in our study population. We performed a retrospective study in a tertiary pediatric hospital between January 2008 to October 2017 of all patients with TBI <16 years old with a Glasgow Coma Scale (GCS) ≤13. The dual primary outcomes were mortality and Paediatric Functional Independence Measure (WeeFIM) scores, recorded at the start of rehabilitation, discharge, 3 months and 6 months post-injury. The secondary outcome was the development of post-traumatic epilepsy. There were 68 patients with a median age of 4.5 [interquartile range (IQR) 1.0-9.0] years old. The most common presenting symptom was vomiting for children <2 years (11/20, 55.0%) while confusion and disorientation were common for those ≥2 years (27/48, 56.3%). WeeFIM scores at the start of rehabilitation [median 122.0, IQR 33.8-126.0] improved at 6 months post-injury (median 126.0, IQR 98.5-126.0). There was a greater incidence of post-traumatic epilepsy in age <2 years (6/20, 30.0%) compared to age ≥2 years (1/48, 2.1%) (p = .002). When comparing NAT versus non-NAT survivors, cognition WeeFIM scores were significantly different at the start of rehabilitation (p = .017) and at 3 months post-injury (p = .025). NAT predicts for poorer long-term outcomes, specifically in cognition, as measured by WeeFIM scores. Younger children <2 years had a higher incidence of post-traumatic epilepsy compared to older children.

Selective dorsal rhizotomy using a 3D high definition exoscope.

Selective dorsal rhizotomy (SDR) is an established neurosurgical technique for children with spastic diplegia secondary to cerebral palsy. Meticulous intraoperative testing of individual nerve roots with electromyography in tandem with the on-site neurorehabilitation team is recommended for good clinical outcomes. The standard approach requires the neurosurgeons to spend extended time under the traditional operating microscope. In this video, the authors describe the use of a 3D exoscope system for SDR. Overall, the 3D exoscope improves ergonomics and reduces musculoskeletal fatigue for the operating neurosurgeons. Furthermore, it provides excellent visualization of important structures, allowing safe and efficient completion of the procedure. The video can be found here: https://stream.cadmore.media/r10.3171/2023.10.FOCVID23105.

Caregiver reported long-term outcomes in children with major trauma and traumatic brain injuries: A single-centre retrospective study.

Introduction: We aim to investigate the functional outcomes and long-term health-related quality of life (HRQOL) in children with major trauma associated with traumatic brain injury (TBI). Method: We performed a retrospective review of records among patients >2 and ≤16 years old in a tertiary paediatric hospital between January 2014 and October 2019 with major trauma (Injury Severity Score of ≥16) and TBI of all severities. We recorded each child's Glasgow Outcome Scale-Extended Pediatric Version (GOS-E Peds) at 12 months post-injury and Pediatric Quality of Life Inventory (PedsQL) scores at 6 and 12 months post-injury based on the parent proxy-report scales. Results: We included 53 patients with a median age of 9.0 years old (interquartile range 2.3-15.5). Most injuries were due to falls (30, 56.6%) or road traffic collisions (15, 28.3%); 41 patients (77.3%) required intensive care while 30 patients (56.6%) underwent neurosurgical intervention. Most patients (43, 81.1%) had GOS-E Peds scores of ≤2 at 12 months post-injury. We reported a significant mean difference between the 6- and 12-month parent-reported scores for physical functioning (6.6, 95% confidence interval [CI] 0.3-12.8, P=0.041), psychosocial functioning (4.1, 95% CI 1.0-7.2, P=0.012) and overall scores (5.0, 95% CI 1.4-8.7, P=0.008). Compared with the validated PedsQL scores, our mean scores were higher across all domains at 12 months. Conclusion: With current standard of care, parents of children with major trauma and TBI reported gains in quality of life, physical, psychosocial and overall function between 6 and 12 months post-injury.

Access to dental care and caries experience among children with cerebral palsy: a Singapore experience.

INTRODUCTION: Cerebral palsy (CP) is one of the most severe childhood neurodevelopmental disabilities resulting from non-progressive insult to the developing brain. We aimed to report our experience regarding dental visit attendance, caries prevalence and factors affecting dental access in children with CP in Singapore. METHODS: Patients diagnosed with CP who were born in or after 1994 were included in this study. We reviewed the data of all 151 patients recruited under the CP Registry in Singapore (SingCPR) from September 2017 to May 2020. The SingCPR was launched in September 2017 to assist in future planning of services and resources for CP in Singapore. RESULTS: The mean age of the patients was 7.8 years, with the interquartile range being 3 years and 8 months-10 years and 10 months. Only 41.7% reported a visit to the dentist ever, with 25.4% reporting presence of dental caries. Age was the only statistically significant factor influencing access to dental care. None of the children less than 2 years old ever received any dental care, and 20% of the children with CP aged 2-6 years had received dental care before. Age range with the highest percentage of dental visits was 7-12 years, with up to 44.0% having ever received dental care. We believe the prevalence of dental caries was underreported as many children did not receive any dental care and therefore may have undetected dental caries. CONCLUSION: Dental care in children with CP should be advocated early for prevention and detection of caries.

Evaluation of risk factors associated with fragility fractures and recommendations to optimise bone health in children with long-term neurological condition.

Introduction: The growing years are paramount for bone growth and mineral accrual. Children with long-term neurological condition (LTNC) have multiple risk factors for poor bone health and fragility fractures. In Singapore, this has not been studied systematically. Therefore, we aimed to evaluate the risk factors associated with fragility fractures in children with LTNC. Methods: In this study, the search for fragility fractures was done by a retrospective review of patients with LTNC on follow-up in the paediatric neurology clinic and patients who presented with fracture to the paediatric orthopaedic clinic. Information on patients' demographics, medical history, intervention, biochemical bone markers and fracture history was collected. Results: In a tertiary clinic population of 136 patients with LTNC, 65% were dependent on mobility (Gross Motor Function Classification System [GMFCS] V), 60% were underweight and 60% were fed via gastrostomy or nasogastric tube, or were on oral pureed diet. Furthermore, 60% were on anticonvulsants. The fracture rate was 3% in this population and was associated with low-impact activities such as transfer and dressing. Only 7.4% and 33% of the patients had undergone measurements of vitamin D and calcium levels, respectively. Conclusion: The local prevalence of fragility fractures in children with LTNC on follow-up at the neurology clinic was found to be 3%. Risk factors identified were limited ambulation and compromised nutritional status associated with feeding difficulty. Recommendations to optimise bone health in children with LTNC were made. These include promoting weight-bearing activities, looking out for underweight children, avoiding vitamin D deficiency and ensuring adequate calcium intake.

Diverse Clinical Manifestations of Cardiofaciocutaneous Syndrome Type 3 in Two Patients from South East Asia.

Background: Cardiofaciocutaneous syndrome (CFCS) is a rare genetic condition caused by mutations in BRAF, KRAS, MAP2K1, or MAP2K2. It is characterized by ectodermal abnormalities, cardiac defects, intellectual disability, and distinct craniofacial features. CFCS falls under a group of conditions caused by mutations in the RAS/MAPK pathway called RASopathies which share many features. In particular, CFCS has significant phenotypic overlaps with Costello syndrome (CS) and Noonan syndrome (NS). Objective: The aim of this study was to assess the patients‧ phenotypic features for syndromic disorders and evaluate the use of molecular testing to clarify the clinical diagnosis. Method: The patients were recruited for genetic testing with written informed consent. Genomic DNA from venous blood was sequenced and potential variants were identified via targeted next-generation sequencing. Their phenotypic features were compared with other CFCS cases carrying pathogenic variants in the same gene. Results and Discussion: One patient had a de novo variant (c.370C>T; p.P124S) in MAP2K1 and presented with mild and typical features which do not significantly affect her quality of life. The second patient presented with severe features, including failure to thrive, feeding difficulties, epileptic spasms, septal hypertrophy, and global developmental delay, and developed chronic lung disease and sequelae from multiple infections. She had a severe disease course and severe global developmental delay. The discovery of a de novo variant (c.371C>A; p.P124Q) in MAP2K1, which had been reported in another patient with a similar phenotype, clarifies her clinical diagnosis. Her presentations add to existing reports that support expanding the CFCS phenotype to include features previously thought to be more suggestive of CS. Conclusion: The genetic findings for the 2 patients affirm the use of identified gene mutations to confirm the clinical diagnosis of syndromic disorders and add to the phenotypic spectrum of CFCS.

Comparison of functional outcome scales in paediatric acute encephalitis: Responsiveness and outcome predictors.

PURPOSE: To compare scoring systems and their ability to capture short and long-term recovery of paediatric patients with acute encephalitis. To identify clinical predictors of short-term outcomes by correlating functional outcome measures at 1 month post diagnosis of acute encephalitis. METHODS: Patients with encephalitis diagnosed between July 2011 and 2016 based on Granerod's criteria were studied in this retrospective cohort study. Functional outcome scores on WeeFIM, LOS, GOS-E, mRS and ICF at initial presentation and 1, 3, 6 and 12 months later were compared. RESULTS: WeeFIM and LOS scores both showed maximum change in the first 3 months, reflecting highest recovery in this period. With WeeFIM, the greatest change occurred within the first month following diagnosis. On univariate analysis, seizure frequency in the first month, presence of movement disorder, presence of autonomic dysfunction and lower baseline functional score was associated with poorer WeeFIM scores at 1 month. The latter three variables remained statistically significant on multivariate analysis. CONCLUSION: WeeFIM is a potentially preferred functional outcome assessment tool as it demonstrated greatest recovery within the first month due to a trend of high responsiveness and relatively low ceiling effect. Presence of autonomic dysfunction and movement disorders at diagnosis correlated with poorer outcome at 1 month post diagnosis.

Cost of inpatient rehabilitation for children with moderate to severe traumatic brain injury.

AIM: To evaluate the cost of inpatient rehabilitation for children with moderate to severe traumatic brain injury (TBI). Secondary aim was to identify factors associated with high inpatient rehabilitation cost. METHOD: Retrospective review of a tertiary hospital's trauma registry was performed from 2011-2017. All patients aged 16 years or younger who sustained TBI with Glasgow Coma Scale ≤13 were included. Data on patient demographics, mechanism and severity of injury, hospital duration and inpatient rehabilitation cost were collected. We performed a regression analysis to identify factors associated with high rehabilitation cost. RESULTS: There were a total of 51 patients. The median duration of inpatient rehabilitation was 13.5 days (interquartile range [IQR] 4-35), amounting to a median cost of SGD8,361 (IQR 3,543-25,232). Daily ward costs contributed the most to total inpatient rehabilitation cost. Those with severe TBI had longer duration of inpatient rehabilitation that resulted in higher cost of inpatient rehabilitation. Presence of polytrauma, medical complications, post-traumatic amnesia and TBI post-non-accidental injury (NAI) were associated with higher cost of inpatient rehabilitation. CONCLUSION: The cost of inpatient rehabilitation for paediatric patients post-TBI is significant in Singapore. Patients with TBI secondary to NAI had significantly higher cost of inpatient rehabilitation. Ways to reduce duration of hospitalisation post-TBI and early step-down care or outpatient rehabilitation should be explored to reduce cost.

Causes, functional outcomes and healthcare utilisation of people with cerebral palsy in Singapore.

INTRODUCTION: A voluntary cerebral palsy (CP) registry was established in 2017 to describe the clinical characteristics and functional outcomes of CP in Singapore. METHODS: People with CP born after 1994 were recruited through KK Women's and Children's Hospital, National University Hospital and Cerebral Palsy Alliance Singapore. Patient-reported basic demographics, service utilisation and quality of life measures were collected with standardised questionnaires. Clinical information was obtained through hospital medical records. RESULTS: Between 1 September 2017 and 31 March 2020, 151 participants were recruited. A majority (n=135, 89%) acquired CP in the pre/perinatal period, where prematurity (n=102, 76%) and the need for emergency caesarean section (n=68, 50%) were leading risk factors. Sixteen (11%) of the total participants had post-neonatally acquired CP. For predominant CP motor types, 109 (72%) had a spastic motor type; 32% with spastic mono/hemiplegia, 41% diplegia, 6% triplegia and 21% quadriplegia. The remaining (42, 27.8%) had dyskinetic CP. Sixty-eight (45.0%) participants suffered significant functional impairment (Gross Motor Functional Classification System levels IV-V). Most participants (n=102, 67.5%) required frequent medical follow-up (≥4 times a year). CONCLUSION: Optimisation of pre- and perinatal care to prevent and manage prematurity could reduce the burden of CP and their overall healthcare utilisation.

Assessment of the Understanding of Concussion and Care Protocols Amongst Student Athletes and Coaches: A Qualitative Study.

Background: Pediatric sports-induced concussions have become a topic of interest and concern in the scientific community. Already, the literature is rich with studies that have identified numerous short-term and long-term consequences of childhood sports-induced concussions. However, there are very few studies that have identified how well the students who participate in concussion-prone sports and their coaches understand these consequences and how they can be avoided. This study aimed to explore student athletes' and their coaches' understanding of the concept of concussion and how it is managed both immediately after the injury occurs and during long-term recovery. Methods: This study utilized a qualitative design. The study was conducted in local and international schools in Singapore. Participants were recruited through purposive sampling. 42 student athletes aged 13-18 who participated in rugby, softball, football, cricket, volleyball, and/or water polo were recruited. Fourteen coaches who coached these same sports were also recruited. Four focus groups and three semi-structured interviews were conducted. Data collected were then analyzed with thematic analysis. Risk factors were assessed through four domains of focus: understanding of what concussion is; attitudes toward concussion; existing protocols for treating concussion; and return-to-school and return-to-play protocols. As this is a qualitative study, outcome measures were not identified. Results: Analysis of the data revealed four themes for each group. For student-athletes these included: limited understanding of concussion; non-reporting of injuries; variable supervision of athletes; and a lack of established return-to-school and return-to-play guidelines. For coaches these included: variable understanding of concussion; insufficient formal training in concussion management; limited medical support in managing injuries; and lack of understanding and adherence to return-to-school and return-to-play protocols. Conclusions: Of the themes identified, the most pressing was a lack of clearly defined return-to-play guidelines. This is an urgent issue that needs to be jointly addressed by healthcare professionals and schools with evidence-based guidelines.

Epidemiology of Pediatric Tracheostomy and Risk Factors for Poor Outcomes: An 11-Year Single-Center Experience.

OBJECTIVE: Children with long-term tracheostomies are at higher risk of complications. This study aims to describe the epidemiology, outcomes, and factors associated with successful decannulation in children undergoing tracheostomy. STUDY DESIGN: Case series with chart review. SETTING: Tertiary hospital. SUBJECTS AND METHODS: A retrospective analysis was conducted on pediatric tracheostomies performed from 2006 to 2016. Demographics, preexisting comorbidities, indications for tracheostomy, and pretracheostomy ventilatory requirements were collected. A multivariate regression model with covariates of age, failure to thrive (FTT), and comorbidities was used to identify factors associated with successful decannulation. Secondary outcomes were ventilation and oxygen requirements at hospital discharge, hospital and intensive care unit length of stay, and complications. RESULTS: In total, 105 patients received a tracheostomy at a median age of 8.0 months (interquartile range, 2.0-45.0). The most common indication was anatomic airway obstruction (55 of 105, 52.5%). Forty-four (41.9%) patients had preexisting FTT. In-hospital mortality was 14 of 105 (13.3%). None were directly related to tracheostomy. At discharge, 40 of 91 (44.0%) and 12 of 91 (13.2%) required home mechanical ventilation and supplemental oxygen, respectively. Forty-one (39%) patients underwent successful decannulation at a median 408 days (interquartile range, 170-1153) posttracheostomy. On adjusted analysis, unsuccessful decannulation was more common in patients with FTT and neurologic comorbidities. Postoperative complications were more common in younger patients and those with a longer time to decannulation. CONCLUSION: Neurologic comorbidities and FTT were risk factors for unsuccessful decannulation after pediatric tracheostomy. Nutritional interventions may have a role in improving long-term outcomes following pediatric tracheostomies and should be investigated in future studies.

Long-Term Morbidities in Children with Critical Illness: Gaps and Opportunities.

INTRODUCTION: Improved mortality rates in paediatric critical care may come with the cost of increased morbidity. Goals of modern paediatric intensive care unit (PICU) management should focus on restoring long-term function of paediatric critical illness survivors. This review outlines our current knowledge on trajectories and risk factors of long-term morbidities in PICU survivors. Specifically, we aimed to identify current limitations and gaps in this area so as to identify opportunities for future investigations to reduce the burden of morbidities in these children. MATERIALS AND METHODS: A review of primary studies published in PubMed, EMBASE, and Cochrane databases in the last decade (2008-2017) describing long-term morbidities in PICU survivors was conducted. RESULTS: Children surviving critical illness continue to experience morbidities after discharge. A set of risk factors modify their long-term trajectories of recovery, with some children achieving their premorbid level of function, while some others deteriorate or die. Limitations in current methodologies of morbidity research impair our understanding on the causes of these morbidities. Opportunities for future endeavours to reduce the burden of these morbidities include identifying patients who are more likely to develop morbidities, evaluating the efficacy of early rehabilitation, identifying patients who might benefit from tight glycaemic control, characterising the optimal nutritional intervention, and improving management of increased intracranial pressure. CONCLUSION: Survivors of paediatric critical illness experience differing trajectories of recovery from morbidities. Future research is needed to expand our repertoire on management strategies to improve long-term function in these children.

Early Hyperglycemia in Pediatric Traumatic Brain Injury Predicts for Mortality, Prolonged Duration of Mechanical Ventilation, and Intensive Care Stay.

We aim to study the association between hyperglycemia and in-hospital outcomes among children with moderate and severe traumatic brain injury (TBI). This retrospective cohort study was conducted in a tertiary pediatric hospital between 2003 and 2013. All patients < 16 years old who presented to the Emergency Department within 24 hours of head injury with a Glasgow Coma Scale (GCS) ≤ 13 were included. Our outcomes of interest were death, 14 ventilation-free, 14 pediatric intensive care unit- (PICU-) free, and 28 hospital-free days. Hyperglycemia was defined as glucose > 200 mg/dL (11.1 mmol/L). Among the 44 patients analyzed, the median age was 8.6 years (interquartile range (IQR) 5.0-11.0). Median GCS and pediatric trauma scores were 7 (IQR 4-10) and 4 (IQR 3-6), respectively. Initial hyperglycemia was associated with death (37% in the hyperglycemia group versus 8% in the normoglycemia group, p = 0.019), reduced median PICU-free days (6 days versus 11 days, p = 0.006), and reduced median ventilation-free days (8 days versus 12 days, p = 0.008). This association was however not significant in the stratified analysis of patients with GCS ≤ 8. Conclusion. Our findings demonstrate that early hyperglycemia is associated with increased mortality, prolonged duration of mechanical ventilation, and PICU stay in children with TBI.

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome may have a hypothalamus-periaqueductal gray localization.

BACKGROUND: Anatomical localization of the rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome has proved elusive. Most patients had neuroimaging after cardiorespiratory collapse, revealing a range of ischemic lesions. PATIENT DESCRIPTION: A 15-year-old obese boy with an acute febrile encephalopathy had hypoventilation, autonomic dysfunction, visual hallucinations, hyperekplexia, and disordered body temperature, and saltwater regulation. These features describe the ROHHAD syndrome. Cerebrospinal fluid analysis showed pleocytosis, elevated neopterins, and oligoclonal bands, and serology for systemic and antineuronal antibodies was negative. He improved after receiving intravenous steroids, immunoglobulins, and long-term mycophenolate. Screening for neural crest tumors was negative. CONCLUSION: Magnetic resonance imaging of the brain early in his illness showed focal inflammation in the periaqueductal gray matter and hypothalamus. This unique localization explains almost all symptoms of this rare autoimmune encephalitis.

Nonimmune hydrops fetalis in a children's hospital: a six-year series.

INTRODUCTION: We aimed to study the profile of nonimmune hydrops fetalis (NIHF) in the local population and identify its outcomes and causes. METHODS: We carried out a retrospective review of the medical records in KK Women's and Children's hospital, a single tertiary referral centre, for pregnancies with an antenatal diagnosis of NIHF in the six-year period from 1 January 2005 to 31 December 2010. RESULTS: A total of 29 cases of NIHF were identified; 19 (66%) cases underwent karyotype evaluation, 17 (59%) underwent intrauterine infection screening, and all underwent antenatal thalassaemia screening. The median gestational age at diagnosis was 27 (range 12-37) weeks, median gestational age at birth was 33 (range 27-37) weeks, and median birth weight of live births was 2,480 (range 1,230-3,900) g. The aetiologies for NIHF were identified in 20 (69%) cases, which included cardiac anomalies (n = 5), haematological problems (n = 4), congenital tumours (n = 4), genetic/metabolic disorders (n = 4) and cystic hygromas (n = 3). The cause of NIHF was not identified in the remaining 9 (31%) cases. There were 19 live births - 8 (42%) survived and 11 (58%) died in the neonatal period - and one stillbirth. Nine women opted for medical termination of pregnancy following the diagnosis of NIHF. CONCLUSION: It is important to thoroughly investigate all cases of NIHF and identify its causes in order to provide appropriate antenatal and postnatal counselling. In our series, almost one-third of NIHF cases had no identified aetiology. The neonatal mortality rate was approximately 58%.