Symposium: Myelomeningocele 14 articles
Knee flexion contracture leading to crouch gait is commonly seen in children with myelomeningocele. Progressive increase in knee flexion contracture increases energy cost, which interferes with efficient, functional ambulation. To prevent this, surgical release has been recommended when a knee flexion contracture exceeds 15° to 20°.
Youth with disabilities are at risk for decreased participation in community activities. However, little is known about participation at different developmental periods of childhood and adolescence among youth with spina bifida (SB) or whether child, family, and SB-associated factors influence participation.
Spinal deformity, a common problem in children with myelodysplasia, is associated with alterations in pulmonary function and sitting balance. Sitting imbalance causes areas of high pressure in patients already at high risk for developing pressure ulcers due to insensate skin.
Children with spina bifida often present with functional deficits of the lower limb associated with neurosegmental lesion levels and require orthotic management. The most used orthosis for children with spina bifida is the ankle–foot orthosis (AFO). The AFO improves ambulation and reduces energy cost while walking. Despite the apparent benefits of using an AFO, limited evidence documents the influence of factors predicting the ability of an AFO to improve function and when they are indicated. These variables include AFO design, footwear, AFO–footwear combination, and data acquisition. When these variables are not adequately considered in clinical decision-making, there is a risk the AFO will be abandoned prematurely or the patient’s stability, function, and safety compromised.
Activity Level, Functional Health, and Quality of Life of Children with Myelomeningocele as Perceived by Parents
To provide the best health care for individuals with myelomeningocele (MM), clinicians and researchers need to understand their health and functional status as well as quality of life. The literature is mixed regarding the relationship between motor level and health-related quality of life (HRQOL) for these individuals.
Progressive kyphosis occurs in up to 20% of patients with myelomeningocele. Severely affected patients can develop recurrent skin breakdown, osteomyelitis, sitting imbalance, and poor cosmetic appearance.
Patients with myelomeningocele and rigid lumbar and thoracolumbar kyphosis face substantial functional difficulties with sitting and lying supine and are prone to skin breakdown over the gibbus and risk of infection. Kyphectomy, along with cordotomy and segmental spinal instrumentation down to the pelvis, is one alternative that can provide reliable correction of the deformity but also can maintain that correction over a period of time.
Patients with spina bifida frequently sustain lower extremity fractures which may be difficult to diagnose because they feel little or no pain, although the relative contributions of low bone density to pain insensitivity are unclear. Routine dual-energy xray absorptiometry (DXA) scanning is unreliable because these patients lack bony elements in the spine, and many have joint contractures and/or implanted hardware.
Is the Vertebral Expandable Prosthetic Titanium Rib a Surgical Alternative in Patients with Spina Bifida?
Nonambulatory children with myelodysplasia are most likely to develop spinal deformity. As the deformity progresses, the overall health of the patient deteriorates. Traditional management of the deformity with fusion results in a short trunk, crankshaft deformity, and spine and lung growth inhibition. One alternative that potentially minimizes these problems is the vertebral expandable prosthetic titanium rib (VEPTR).
Posterior Kyphectomy for Myelomeningocele With Anterior Placement of Fixation: A Retrospective Review
Kyphosis in myelomeningocele is a rare and difficult problem. Many strategies have been used with no single procedure universally agreed on. Techniques involving anterior and posterior fixation may provide better fusion.
Electrical stimulation is an established treatment for muscle weakness. However, traditional tectonic stimulation is poorly tolerated in children as a result of discomfort. Threshold electrical stimulation performed at night reportedly increases muscle strength in a variety of neuromuscular conditions and has been well tolerated in children.
Although many children with spina bifida and associated scoliosis or dislocated hips undergo spine or hip surgery, the benefits are uncertain.
Patients with spina bifida potentially experience social isolation with limited employment opportunities and restriction of independent living, neurologic and intellectual impairment, and orthopaedic and urologic consequences of their condition. However, the degree of disability as these individuals age into adulthood has not been completely delineated.