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Ambulation, lesion level, and health-related quality of life in children with myelomeningocele

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Ebru Şeker Abanoz
Ebru Şeker Abanoz
Ebru Şeker Abanoz
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Meral Ozmen at Istanbul University
Meral Ozmen
Mine Calışkan at Istanbul University
Mine Calışkan
Gülbin Gökçay at Istanbul University Institute of Child Health
Gülbin Gökçay
  • Istanbul University Institute of Child Health
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Abstract

Purpose The aim is to investigate the health-related quality of life (HRQOL) in children with myelomeningocele (MMC), compare the results with those of healthy children, and determine the factors related to HRQOL. Methods Fifty children with MMC with a mean age of 8.96 ± 2.57 and 50 healthy children with a mean age of 9.50 ± 2.42 were included in the study. The demographic information form and the CHQ-PF-50 (Child Health Questionnaire Parent form 50) were completed to determine the quality of life (QOL) for the children. Ambulation levels of children with MMC and disease-specific findings were recorded. The HRQOL scores of children with MMC were compared with healthy children and assessed according to lesion levels and ambulation status. Results The CHQ-PF-50 scores of healthy and MMC children had no significant difference in the sub-dimensions of health change (p > 0.05), but the mean QOL score of children with MMC was significantly lower in all other sub-dimensions (p < 0.05). In addition, QOL scores according to lesion levels in children with MMC were significantly different between the three groups (p < 0.05). The QOL scores were the highest in the sacral group and the lowest in the thoracic-high lumbar group. The QOL for non-ambulatory children was significantly lower than for ambulatory children with MMC (p < 0.05). Conclusions The present study confirms that children with MMC have diminished HRQOL and non-ambulatory and children with high lesion levels are affected the most. Our result suggests that focusing on the activities that will enable children to acquire the ability to walk can positively affect the HRQOL.
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ORIGINAL ARTICLE
Ambulation, lesion level, and health-related quality of life
in children with myelomeningocele
Ebru Şeker Abanoz
1
&Meral Özmen
2
&Mine Çalışkan
2
&Gülbin Gökçay
3
&Nur Aydınlı
2
Received: 26 February 2019 /Accepted: 7 August 2019
#Springer-Verlag GmbH Germany, part of Springer Nature 2019
Abstract
Purpose The aim is to investigate the health-related quality of life (HRQOL) in children with myelomeningocele (MMC),
compare the results with those of healthy children, and determine the factors related to HRQOL.
Methods Fifty children with MMC with a mean age of 8.96 ± 2.57 and 50 healthy children with a mean age of 9.50 ±2.42 were
included inthe study. The demographic information form and the CHQ-PF-50 (Child Health Questionnaire Parent form 50) were
completed to determine the quality of life (QOL) for the children. Ambulation levels of children with MMC and disease-specific
findings were recorded. The HRQOL scores of children with MMC were compared with healthy children and assessed according
to lesion levels and ambulation status.
Results The CHQ-PF-50 scores of healthy and MMC children had no significant difference in the sub-dimensions of health
change (p> 0.05), but the mean QOL score of children with MMC was significantly lower in all other sub-dimensions (p<0.05).
In addition, QOL scores according to lesion levels in children with MMC were significantly different between the three groups
(p< 0.05). The QOL scores were the highest in the sacral group and the lowest in the thoracic-high lumbar group. The QOL for
non-ambulatory children was significantly lower than for ambulatory children with MMC (p<0.05).
Conclusions The present study confirms that children with MMC have diminished HRQOL and non-ambulatory and children
with high lesion levels are affectedthe most. Our result suggests that focusing on the activities thatwill enablechildren to acquire
the ability to walk can positively affect the HRQOL.
Keywords Lesion level .Myelomeningocele .Quality of life .Ambulation
Introduction
Myelomeningocele (MMC), which has a major impact on
patient’s lives, is a complex congenital birth defect frequently
associated with many complications such as hydrocephaly,
muscle weakness/paralysis, neurogenic bowel and bladder,
epilepsy, and learning and behavioral problems [1,2]. The
degree of neurological disorder and orthopedic problems
change according to the size and localization of the defect
and high lesion levels lead to more severe results [3].
Previous studies with multidisciplinary treatment approaches
have shown an increased psychosocial well-being, emphasiz-
ing the importance of QOL studies beyond traditional out-
come measures [4–6]. Studies investigating health-related
quality of life (HRQOL) in children with MMC have shown
differing results and the studies assessing QOL according to
lesion levels are very limited. The present study investigated
the physical, emotional, and social aspects of HRQOL in chil-
dren with MMC; determined the HRQOL for different lesion
levels; and compared these results with healthy children.
Methods
Participants
The database of the Department of Pediatric Neurology of
Istanbul University, Istanbul Faculty of Medicine,
*Ebru Şeker Abanoz
ebruseker@gmail.com
1
Institute of Graduate Studies, Department of Physiotherapy and
Rehabilitation, Istanbul University - Cerrahpasa, Istanbul, Turkey
2
Istanbul Faculty of Medicine, Pediatric Neurology Section, Istanbul
University, Istanbul, Turkey
3
Institute of Child Health, Social Pediatrics Department, Istanbul
University, Istanbul, Turkey
https://doi.org/10.1007/s00381-019-04348-0
Child's Nervous System (2020) 36:611–616
/Published online: 19 Augu st 2019
Content courtesy of Springer Nature, terms of use apply. Rights reserved.
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Ambulation in Patients with Myelomeningocele
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  • Sep 1988
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Factors determining ambulation in 163 patients with myelomeningocele were studied by a multi-variate statistical method. Neurological dysfunction unrelated to the plaque was analyzed by magnetic resonance imaging. There were no ambulators at the thoracic or L1-L2 level. At the L3 level, 54% ambulated, and at the L4 level, 67% ambulated. Eighty percent were ambulators at L5 and all at the sacral level. Below L1-L2, one-half of the nonambulators had neurological deficiencies caused by syringohydromyelia or Chiari malformations preventing ambulation. Severe scoliosis was closely, age moderately, and hip flexion contracture slightly related to the inability of the other nonambulators to walk, while pelvic obliquity, hip dislocation, or knee flexion contracture was not.
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Assessing health-related quality of life in children with spina bifida
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  • Feb 2015
Object: The purpose of this study is to explore various aspects of health-related quality of life (HRQOL) in children with spinal dysraphism. Methods: The authors enrolled a prospective cohort of 159 patients from the multidisciplinary spina bifida clinic. Surveys were distributed to caregivers of patients with spina bifida who were 5 years old and older. Data were collected using the Health Utilities Index Mark 3 focusing on vision, speech, hearing, dexterity, ambulation, cognition, emotions, and pain. Each participant received an overall HRQOL utility score and individual domain subscores. These were correlated with demographic and treatment variables. Analysis was done using SPSS statistics (version 21). Results: There were 125 patients with myelomeningocele, 25 with lipomyelomeningocele, and 9 with other dysraphisms. Among patients with myelomeningocele, 107 (86%) had CSF shunts in place, 14 (11%) had undergone Chiari malformation Type II decompression, 59 (47%) were community ambulators, and 45 (36%) were nonambulatory. Patients with myelomeningocele had significantly lower overall HRQOL scores than patients with closed spinal dysraphism. Among patients with myelomeningocele, younger patients had higher HRQOL scores. Patients with impaired bowel continence had lower overall HRQOL scores. History of a ventriculoperitoneal shunt was associated with worse HRQOL (overall score, ambulation, and cognition subscores). History of Chiari malformation Type II decompression was associated with worse overall, speech, and cognition scores. Patients who could ambulate in the community had higher overall and ambulation scores. A history of tethered cord release was correlated with lower pain subscore. No association was found between sex, race, insurance type, or bladder continence and HRQOL. Conclusions: Patients with myelomeningocele have significantly lower HRQOL scores than those with other spinal dysraphisms. History of shunt treatment and Chiari decompression correlate with lower HRQOL scores.
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Health-related quality of life is compromised in individuals with spina bifida: Results from qualitative and quantitative studies
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  • Aug 2014
  • EUR J OBSTET GYN R B
Spina bifida (SB) accounts for approximately 90% of total neural tube defects. According to the degree of SB severity, the range of associated symptoms and complications varies greatly. Given the complexity and diversity of these complications, individuals with SB might suffer from livelong impairment. This review presents an overview of the impact of SB on patients' health-related quality of life (HRQoL) and explores results from published quantitative and qualitative studies regarding the HRQoL impact of SB on patients, as well as comparing results of existing studies to national norms. A literature search using three electronic databases PUBMED, PsycINFO, and Embase was performed to identify relevant studies dating from January 1976 to November 2010. To satisfy the initial inclusion criteria, articles had to contain studies that were specific to HRQoL in patients with SB. Findings highlight that HRQoL is significantly impaired in patients with SB. Making sure that the public is aware of the disease and how to minimise the risk of NTD, such as SB (e.g., through adequate folate levels at time of conception, etc.) is essential to ensure that fewer individuals face the burden of NTDs in the future.
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Health-related quality of life in children and adolescents with spinal dysraphism: Results from a Taiwanese sample
Article
  • Sep 2013
  • CHILD NERV SYST
This study was designed to investigate health-related quality of life (HRQL) in children and adolescents with spinal dysraphism and to determine the differences between the myelomeningocele (MMC) and lipomyelomeningocele (LMMC) groups. Thirty-two patients (mean age, 13.2 years) with MMC and 28 patients (mean age, 11.3 years) with LMMC participated in this study. HRQL was measured using the Child Health Questionnaire-Parent Form 50 (CHQ-PF50). The CHQ-PF50 scores for the study participants were compared with those of a reference sample of healthy Taiwanese children. Outcomes for children with MMC were compared with those for children with LMMC. We also explored the relationships of the lesion level and associated medical problems with HRQL in these subjects. Patients with MMC had significantly lower scores on the physical and psychosocial aspects of CHQ-PF50 than did healthy children. Patients with LMMC had lower scores on some domains but had comparable psychosocial summary scores. Significant differences were noted between the MMC and LMMC groups for all subscales and both summary scores. Children with a higher lesion level had poor physical health (r = -0.52, P < 0.001) but not poor psychosocial health. The number of associated medical problems, indicating their severity, was related to both physical (r = -0.77, P < 0.001) and psychosocial health (r = -0.57, P < 0.001). Children and adolescents with MMC have reduced HRQL compared with those with LMMC. Children and adolescents with more associated medical problems have the greatest risk of diminished physical and psychosocial well-being.
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