Understanding Orthopaedic and Health-related Quality of Life Outcomes in Patients With Myelomeningocele Functional Classification 1 Spina Bifida in Relationship to Supportive Standing.

Abstract

Background: The Myelomeningocele Functional Classification (MMFC) provides a comprehensive summary of functional mobility in patients with spina bifida (SB). The hallmark of MMFC1 patients is the deficiency of gravity-dependent quadriceps musculature strength which precludes independent ambulation but not supportive standing. Limited data exist on the clinical value of supportive standing in this population. The purpose of this study was to identify orthopaedic outcomes and patient-reported quality of life (QOL) scores in MMFC1 spina bifida patients and investigate their relationship to continued supportive standing.

Methods: A retrospective chart review was performed between 2004 and 2023 of all patients with SB treated at a regional tertiary pediatric care center. Patients were excluded if manual muscle testing did not fit the criteria for MMFC1 classification. Demographic, clinical outcomes, and the Patient-Reported Outcomes Measurement Information System (PROMIS) scores were reviewed. Supportive standing history was extracted from chart review and analyzed relative to outcome measures. Statistical analyses were reported with P < .05 reaching significance.

Results: In total, 2,228 patients in our SB clinic were screened with 78 (3.5%) meeting the MMFC1 inclusion criteria. Thirty-three patients (42%) had at least one fracture with 25 (32%) of those patients having a femur fracture. Thirty-nine patients (50%) had at least one pressure sore with 23 (29%) of those patients having a sacral/ischial wound. Those who continued to stand at the most recent follow-up showed a statistically significant decrease in the number of skin wounds compared with those who discontinued standing, but no difference in number of fractures. A significant difference in the Neurogenic Bladder scores was noted in standing patients with higher bladder QOL scores.

Conclusion: MMFC1 patients with spina bifida are at risk for sustaining fractures and/or sacral/ischial pressure sores, but discontinuing supported standing is associated with an increased pressure wound risk. Additionally, patients who continued to stand had higher bladder QOL scores. Utilizing supportive standing in conjunction with multidisciplinary care may decrease the risk of fractures and pressure sores and improve QOL in MMFC1 patients with spina bifida.

Key concepts: (1)Myelomeningocele Functional Classification 1 (MMFC1) patients with spina bifida are at risk of incurring a femur fracture regardless of supportive standing.(2)MMFC1 spina bifida patients who continue to stand have significantly fewer number of pressure sores.(3)Improved neurogenic bladder quality of life scores were correlated with MMFC1 spina bifida patients who continued supportive standing.(4)A multidisciplinary team approach is needed to prevent barriers to care and improve quality of life in MMFC1 spina bifida patients.

Level of evidence: Level III.