Search

Background To date, children with hydrocephalus continue to have a considerable long-term outcome. However, current literature on health-related quality of life (HRQOL) among children with hydrocephalus are limited. This serves a call for research to validate a suitable HRQOL for children with hydrocephalus measuring the physical, emotional, social and cognitive well being. Objective To validate a technology-enhanced quality of life questionnaire (EITVAQ) as an effective assessment tool measuring the current well-being of a child with hydrocephalus (child-centred) To adopt a technology-enhanced quality of life assessment tool (EITVAQ) into a phone/tablet application to gain widespread usage To explore the use of technology to increase the learning potential and obtaining a new skill among children with hydrocephalus Methods This is a prospective study which took place since January 2018 to January 2019. This study received ethical approval All data was compiled and analysed using SPSS. The validation process involved: Content Validity, Feasibility and Reliability. A cohort of 70 healthy children from various primary and secondary schools participated as a control. Finally, a small cohort of children with hydrocephalus participated in technology-enhanced learning programme (TELP) to learn a new music skill and created a music video. Results This study reports the result of the validation process. In total there were 132 participants. Content validity was achieved by the nominal group technique. In this study, technology enhanced quality of life questionnaire (EITVAQ) is a feasible tool, having a high satisfaction rate of 78%, a response rate of 76.5% and an average completion time of 4 mins and 26 seconds. EITVAQ had a score of 0.92 (Cronbach Alpha). When comparing with the control, the two lowest scores were the social and emotional aspect. The two major differences in scores among both groups were the physical and cognitive well-being. All participants who participated in the TELP managed to complete the task learning the skills of creating a music video. Conclusion EITVAQ, an interactive and child-friendly tool to assess quality of life is now validated. It aims to be used widely among children with hydrocephalus, providing a baseline assessment to allow us to understand more about a child’s quality of life from their own perspective. This study concludes that technology has a huge potential in helping children with hydrocephalus and various neuro-disability to integrate into society.

Introduction Differentiation between plagiocephaly and craniosynostosis continues to provide a challenge for the clinician. Historically, initial investigations would have included a skull x-ray. However, it is widely accepted that this investigation is difficult to interpret and its value in diagnosis of craniosynostosis is debated. Aside from creating the burden of additional investigations, it also exposes children to ionising radiation. Aim and Objectives Examine the correlation between reported skull x-rays and clinical diagnosis of craniosynostosis to establish their diagnostic value and value in management of abnormal skull shape. Methods Retrospective chart review conducted as a 2 part study in children who were referred with a skull x-ray. Part A: Referrals of abnormal skull shapes to the National Pediatric Craniofacial Center (NPCC), Temple Street Children’s Hospital, between 1st January 2015 and 30th May 2017 Part B: Children who underwent surgery for a confirmed craniosynostosis between 1st January 2011 - 25th October 2017. Results and Findings Part A: 300 children were referred with 59 skull x-rays. This represented 20% of all patients referred during the time period. Of these 44 (75%) were found to be a match with 15 (25%) not matching the final clinical diagnosis. Part B: 274 children underwent surgery for a confirmed craniosynostosis between 1st January 2011 - 25th October 2017. 63 pts had skull x-rays on referral- this represents 23% of all operated children in the time period. Of these 41 (63%) were found to be a match with 17 (29%) not matching the final clinical diagnosis. 5 (8%) were inconclusive. Conclusions Part A: In 25% of the children referred to the NPCC with abnormal skull shape, their clinical diagnosis did not match their x-ray report. As such, skull x-rays did not contribute to their management. Part B: In 35% of children who underwent surgery for craniosynostosis, their clinical diagnosis did not match their radiological diagnosis. In the remaining children who had a skull x-ray performed and underwent surgery for craniosynostosis, the majority (88%) had a subtype of craniosynostosis which our clinical team who feel confident to diagnosis clinically without imaging. As such, it can be said that in 92% of children who underwent surgery at the NPCC the x-ray did not contribute to their management. We recommend clinicians should check with the NPCC with respect to the protocol for x-rays where craniosynostosis is suspected as in most cases the diagnosis is clinically obvious to the craniofacial surgeon.

Background Craniosynostosis is a congenital condition resulting from premature fusion of the cranial sutures resulting in a phenotypic headshape. Nonsyndromic craniosynostosis, involving a single suture only; is the most common presentation involving the fusion of one of the following sutures; sagittal, metopic, coronal or lambdoid. The National Paediatric Craniofacial Centre in Temple Street Children’s University Hospital is the designated craniofacial centre in the Republic of Ireland. The treatment of craniosynostosis involves appearance-changing surgery designed to normalise the headshape. As with most other designated craniofacial centres the aim is to perform surgery as close to 12 months of age as possible for these children. The clinical presentation of craniosynostosis as with most rare diseases can result in an over-reliance on different types of radiology imaging, a subsequent later referral and surgery being performed at a later age. The aim of this review was to report the referral patterns of children with metopic, coronal and lambdoid nonsyndromic craniosynostosis presenting to the NPCC. Methods A retrospective chart review of children who underwent surgery for metopic, coronal or lambdoid craniosynostosis between January 2009 and December 2018 was undertaken. Data collected included: patient demographics, hospital of birth, referrer specialty, date of referral and any radiology investigations performed. Children subsequently diagnosed with a syndrome were excluded. Results 130 patients were included in the review. 80% (n=104) were referred before 10 months of age. There was a high use of radiology imaging with 71% (n= 92) of all children having at least one radiology investigation prior to referral. 15% (n=19) had multiple radiology investigations performed. Paediatricians and Neonatologists referred the largest number of children; 58% (n=75) and 22% (n=28) respectively. Surgery was performed before 15 months of age for 44% (n=57) of children and before 18 months of age for a further 26% (n=34) of all children. Conclusion The incidence of metopic, coronal and lambdoid craniosynostosis is in keeping with reported rates in Europe. Although most children were referred before 10 months of age complex referral patterns and use of radiology imaging were associated with later referrals, leading to subsequent delays in surgery performed. It is hoped that an increased awareness of recognising and referring children with craniosynostosis will lead to subsequent treatment as early as possible. The NPCC encourages that referral letters for children with suspected craniosynostosis are accompanied by photographs of the child’s headshape and not radiology investigations.

Introduction There is a paucity of knowledge regarding Sleep Disordered Breathing (SDB) in paediatric patients with Spina Bifida/Myelomeningocoele (SB/MMC). Undiagnosed, untreated SDB has detrimental effects on growth, development (1) and cognition (2) which is especially important to recognise in those with other co-morbidities as is the case in SB/MMC. Aim There are currently 317 patients aged 0–16 years attending the Spina Bifida service at Temple Street Children’s University (TSCUH). Fourteen of 317 patients (4.4%) currently require Non-Invasive Ventilation (NIV) therapy for SDB under the care of the Respiratory Team. We hypothesize that a subpopulation of the SB/MMC patients are at risk of undiagnosed SDB. We aimed to initially review the demographics of the sub-group of SB/MMC patients currently requiring NIV by assessing for potential predictors of SDB. Method We used up to date medical records of the patients under our care to look for potential predictors for SDB, in particular hydrocephalus with VP shunt, Chiari II malformation and intellectual disability (ID). We then compared our data to what is known about the general SB/MMC population. Results 100% of SB patients with SDB requiring NIV had hydrocephalus with VP shunt compared to 80% in general SB/MMC patients. 92% also had Chiari II malformation. 50% had a moderate to severe ID in contrast to 25–25% of the general SB/MMC population. Conclusion A higher proportion of SB/MMC patients known to have SDB have hydrocephalus and ID compared to the SB/MMC population mean. At present there is no standardised screen for SDB in our SB/MMC patients. The results of this review have prompted a clinical audit of all of SB/MMC patients at TSCUH to identify those at higher risk of SDB and ultimately determine the prevalence of SDB in this population. References ‘How do we recognize the child with OSAS?’ Joosten KF, et al. Pediatric Pulmonology 2017 Feb;52(2):260–271. ‘Association of Sleep-Disordered Breathing With Cognitive Function and Risk of Cognitive Impairment: A Systematic Review and Meta-analysis.’ Leng Y, et al. JAMA Neurology 2017 Oct 1;74(10):1237–1245.