Quality of Life Evaluation Following Limb Lengthening Surgery in Patients with Achondroplasia

Batıbay, Sefa Giray; Balcı, Halil İbrahim; Bayram, Serkan; Chodza, Mehmet; Göksoy, Şeyda Çelik; Hürmeydan, Önder Murat; Kardelen, Aslı Derya; Şen, Cengiz; Kocaoğlu, Mehmet

doi:10.1007/s43465-020-00127-1

Cited by 13 publications

(15 citation statements)

References 14 publications

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“…One study reported an improvement in mobility and self-care following simultaneous lower and upper (humeral) limb lengthening, however, this study did not use standardized tools to assess functionality or QoL, and conclusions were based primarily on an indirect assessment of arm span and joint range of motion [52]. Three further studies showed humeral lengthening improves physical functioning and/or generic QoL (as measured by the SF-36 or PedsQL) [40,41,51]. In contrast, Kim et al and Batibay et al reported no improvement in physical functioning or QoL in patients with ACH who (mainly) underwent lower limb lengthening [41,50].…”

Section: Impact Of Limb Lengtheningmentioning

confidence: 96%

“…and/or adolescents, 18 years) [33][34][35][36][37][38][39][40][41], 4 in adults (!16years) [42][43][44][45], and 6 included both pediatric and adult patients [46][47][48][49][50][51][52].…”

Section: Characteristics Of Included Studiesmentioning

confidence: 99%

“…Using the pediatric PedsQL scale, Batibay et al [41] retrospectively assessed QoL in young patients with ACH who mainly underwent lower limb lengthening (n ¼ 49; 3-13 years at the time of initial surgery), with a subgroup of patients who also underwent upper limb lengthening (n ¼ 22), in comparison with similarly aged patients not undergoing limb lengthening surgery. There were statistically significant differences between the surgery and the non-surgery groups, though the former group had higher average scores in both the physical and psychosocial domains of PedsQL.…”

Section: Lower and Upper Limb Lengtheningmentioning

confidence: 99%

“…Three further studies showed humeral lengthening improves physical functioning and/or generic QoL (as measured by the SF-36 or PedsQL) [40,41,51]. In contrast, Kim et al and Batibay et al reported no improvement in physical functioning or QoL in patients with ACH who (mainly) underwent lower limb lengthening [41,50]. As suggested by Kim et al, the findings of their casecontrol study could be explained by the fact that long and complicated surgical procedures such as bilateral lower limb lengthening are likely to be followed by post-operative complications or sequelae causing a decline in these outcome measures [50]; however, it is worth mentioning that Batibay et al found only minimal, non-statistically significant differences in mid-term QoL between patients with transient or minor complications (as observed in the study) and those without complications [41].…”

Section: Impact Of Limb Lengtheningmentioning

confidence: 99%

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Quality of life, physical functioning, and psychosocial function among patients with achondroplasia:a targeted literature review

Constantinides

Landis

Jarrett

et al. 2021

Disability and Rehabilitation

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Purpose: Achondroplasia (ACH) is the most common form of skeletal dysplasia, resulting in disproportionate short stature and medical complications. We review the literature on physical functioning, psychosocial function, and quality of life (QoL) in ACH individuals compared to average stature individuals or other short stature conditions. Studies that assess the association between these outcomes and height, limb length/lengthening surgery in ACH patients are also summarized. Materials and methods: PubMed/MEDLINE and Embase were searched through April 2021. Study inclusion criteria were: (1) quantitative design; (2) study population consisting solely/mainly of ACH patients;(3) reports of physical functioning, psychosocial functioning, and/or QoL. Included studies were summarized separately for pediatric and adult populations. Results: Of 1664 records identified, 23 primary studies (sample size 8-437 participants) were included. Multiple tools were used across studies, including the generic PedsQL and SF-36 and height-specific QoLISSY. Conclusions: The literature demonstrates that ACH patients experience limitations in physical functioning and poorer QoL outcomes compared to average stature people across the life span. This appeared to be at least in part due to disproportionate short stature. Future research to better characterize QoL in ACH patients will assist clinicians to better evaluate the effectiveness of management programs including novel interventions. ä IMPLICATIONS FOR REHABILITATIONPatients with achondroplasia experience limitations in physical functioning and poorer quality of life throughout their life course when compared to average statured individuals. Psychosocial issues are also heightened in adults with achondroplasia compared to average statured peers but are observed less frequently in children and adolescents with achondroplasia. The overall impact that limb lengthening has on physical functioning and QoL remains unclear, although there is some evidence that greater height or upper limb length may lead to an improvement in these parameters. Rehabilitation professionals should regularly assess physical functioning, psychosocial wellbeing, and quality of life in individuals with achondroplasia using condition-specific tools.

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Section: Impact Of Limb Lengtheningmentioning

confidence: 96%

“…and/or adolescents, 18 years) [33][34][35][36][37][38][39][40][41], 4 in adults (!16years) [42][43][44][45], and 6 included both pediatric and adult patients [46][47][48][49][50][51][52].…”

Section: Characteristics Of Included Studiesmentioning

confidence: 99%

Section: Lower and Upper Limb Lengtheningmentioning

confidence: 99%

Section: Impact Of Limb Lengtheningmentioning

confidence: 99%

See 2 more Smart Citations

Quality of life, physical functioning, and psychosocial function among patients with achondroplasia:a targeted literature review

Constantinides

Landis

Jarrett

et al. 2021

Disability and Rehabilitation

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“…Although we did not do quality of life (QOL) or psychologic analysis, prior studies of QOL have shown that despite frequent complications and despite the limited height gain as previously described, the QOL improved with ELL [ 26 ]. Batibay et al showed that despite complications with lower limb lengthening the QOL scores improved significantly if humeral lengthening was also performed [ 27 ]. They also showed that QOL scores were higher in the lengthened group vs. a control non-lengthened achondroplasia group.…”

Section: Discussionmentioning

confidence: 99%

Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia

Paley¹

2021

Children

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Extensive limb lengthening (ELL) was completed in 75 patients: 66 achondroplasia and 9 hypochondroplasia. The average lengthening was 27 cm for achondroplasia (12–40 cm) and 17 cm for hypochondroplasia (range 10–25 cm). There were 48 females and 27 males. Lengthening was done either by 2-segment (14 patients; both tibias and/or both femurs) or by 4-segment lengthenings (64 patients; both femurs and tibias at the same time). Most patients also had bilateral humeral lengthening. Patients had 2 or 3 lower limb lengthenings and one humeral lengthening. Lengthenings were either juvenile-onset (31), adolescent-onset (38) or adult-onset (6). The average age at final follow-up was 26 years old (range 17–43 years). There were few permanent sequelae of complications. The most serious was one paraparesis. All patients returned to activities of normal living and only one was made worse by the surgery (paraparesis). This is the first study to show that ELL can lead to an increase of height into the normal height range. Previous studies showed mean increases of height of up to 20 cm, while this study consistently showed an average increase of 30 cm (range 15–40 cm) for juvenile-onset and mean increase of 26 cm (range 15–30 cm) for adolescent-onset. This results in low normal height at skeletal maturity for males and females. The adult-onset had a mean increase of 16.8 (range 12–22 cm). This long-term follow-up study shows that ELL can be done safely even with large lengthenings and that 4-segment lengthening may offer advantages over 2-segment lengthening. While all but the more recent cases were performed using external fixation, implantable limb lengthening promises to be an excellent alternative and perhaps an improvement.

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A comprehensive systematic review of health-related quality of life measures in short stature paediatric patients

Adedeji,

Witt,

Innig

et al. 2024

Endocrine

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This systematic review investigates Patient-reported Outcome Measures (PROMs) and Observed Reported Outcome Measures (ObsROMs) pertinent to assessing Health-Related Quality of Life (HRQoL) in short-stature paediatric patients, focusing on Achondroplasia (ACH), Growth Hormone Deficiency (GHD), Isolated Growth Hormone Deficiency (IGHD), and Small-for-Gestational-Age (SGA) diagnoses. Utilising rigorous selection criteria, 53 studies published from 1998 to 2023 were analysed, revealing a predominance of European-based research. Notably, the review elucidated the utilisation of disease-specific and generic HRQoL measures, showcasing the multifaceted nature of short-stature conditions and their impact across physical, emotional, and social domains. The Quality of Life in Short Stature Youth (QoLISSY), Paediatric Quality of Life Inventory (PedsQL), and KIDSCREEN emerged as frequently employed instruments, offering nuanced insights into HRQoL perceptions across diverse age demographics. Additionally, the review highlighted the adaptation of adult HRQoL measures for adolescent populations, signalling a need for age-appropriate assessment tools. Furthermore, integrating PROMs and ObsROMs in HRQoL assessment underscored a comprehensive approach, considering both subjective patient perspectives and observed outcomes. Future research directions encompass comprehensive search strategies, longitudinal studies with diverse populations, and the development of age-appropriate HRQoL assessment tools. In conclusion, this review emphasises the importance of comprehensive HRQoL assessment to address the diverse needs of short-stature paediatric patients effectively.

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Quality of Life Evaluation Following Limb Lengthening Surgery in Patients with Achondroplasia

Cited by 13 publications

References 14 publications

Quality of life, physical functioning, and psychosocial function among patients with achondroplasia:a targeted literature review

Quality of life, physical functioning, and psychosocial function among patients with achondroplasia:a targeted literature review

Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia

A comprehensive systematic review of health-related quality of life measures in short stature paediatric patients

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