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.
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.
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.
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.
Although many children with spina bifida and associated scoliosis or dislocated hips undergo spine or hip surgery, the benefits are uncertain.
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.
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.
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.
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°.
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).
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.
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.
In contrast with treatment recommendations for adolescent idiopathic scoliosis, there are no clear algorithms for treating patients with early-onset scoliosis. There has been rapid expansion of treatment options for children with early-onset scoliosis, including casting, growth rods, the vertical expandable prosthetic titanium rib, and anterior vertebral stapling.
Early-onset scoliosis, when left untreated, leads to severe deformity. Until the last decade, treatment of progressive curves in young children often consisted of definitive spinal fusion. The recognition of thoracic insufficiency syndrome associated with definitive early fusion has led to the development of new surgical techniques developed to preserve spinal and thoracic growth in young patients with progressive scoliosis.
Structure-Respiration Function Relationships Before and After Surgical Treatment of Early-onset Scoliosis
Spine and chest wall deformities in children with early onset scoliosis (EOS) frequently impair respiratory function and postnatal growth of the lung. While a relationship between deformity and such impairment has been reported in children with adolescent idiopathic scoliosis it is not well understood in children with early-onset scoliosis (EOS).
The Vertical Expandable Prosthetic Titanium Rib (VEPTR™; Synthes North America, West Chester, PA) reportedly controls spinal deformity associated with constrictive chest wall conditions.
Can VEPTR® Control Progression of Early-onset Kyphoscoliosis?: A Cohort Study of VEPTR® Patients With Severe Kyphoscoliosis
Kyphoscoliosis is considered a relative contraindication to treatment with the Vertical Expandable Prosthetic Titanium Rib (VEPTR; Synthes Inc, Paoli, PA). Nevertheless, patients do present with early-onset kyphoscoliosis and thoracic insufficiency syndrome, and no suitable alternative treatments are currently available. However, it is unclear whether VEPTRis reasonable for treating patients with kyphoscoliosis.
Early-onset scoliosis describes progressive spinal deformity of varying etiologies in the growing child. The management of early-onset scoliosis is challenging, with many treatment options but no conclusive evidence for the best treatment method.
The challenge when managing early-onset scoliosis (EOS) is to prevent curve progression while maintaining spinal growth. Current surgical treatments (growing rods, VEPTR) require repetitive interventions to lengthen the implants.
Does Removing the Spinal Tether in a Porcine Scoliosis Model Result in Persistent Deformity?: A Pilot Study
Using a tethering technique, a porcine model of scoliosis has been created. Ideally, tether release before placement and evaluation of corrective therapies would lead to persistent scoliosis.
Thoracic insufficiency syndrome represents a novel form of postnatal restrictive respiratory disease occurring in children with early-onset scoliosis and chest wall anomalies. Expansion thoracoplasty improves lung volumes in children with thoracic insufficiency syndrome; however, how it affects lung development is unknown.
Unilateral pedicle screw epiphysiodesis of the neurocentral synchondrosis (NCS) can produce asymmetric growth of the synchondrosis to create scoliosis in an immature animal model.
2010 Mid-America Orthopaedic Association Physician in Training Award: Predictors of Early Adverse Outcomes after Knee and Hip Arthroplasty in Geriatric Patients
Geriatric patients experience more adverse events owing to early complications after TKA or THA related to preexisting comorbidities. However, associations between patient and surgery variables, including age, BMI, and comorbidities with complications are unclear. Knowing these relationships is necessary for developing risk stratification, defining contraindications, and predicting complications and adverse outcomes.
It is believed that some cases of aseptic failure of THA may be attributable to occult infections. However, it is unclear whether preoperative erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are more likely elevated in these patients than those without overt infection.
Custom Cementless THA in Patients with Skeletal Dysplasia Results in Lower Apparent Revision Rates than Other Types of Femoral Fixation
Patients with skeletal dysplasia are prone to degenerative hip disease thus requiring THA at a younger age than the general population. This is a technically demanding procedure with high complication and revision rates. Achieving good femoral fixation can be challenging because of the abnormal features of the hip.
Various priority criteria for waiting lists for THA have been proposed. These criteria, however, are not typically included in clinical practice, resulting in unclear management procedures. Further, the clinical effects of waiting times on subsequent pain control or function are unclear.
Is Correctional Osteotomy Crucial in Primary Varus Knees Undergoing Anterior Cruciate Ligament Reconstruction?
Valgus high tibial osteotomy (HTO) has been recommended for ligament stability and enhanced function after anterior cruciate ligament (ACL) reconstruction in varus-angulated knees. However, it is not clear whether HTO should be performed in patients undergoing ACL reconstruction who have primary varus knees without medial compartment arthrosis.
More than half of patients with hereditary hemochromatosis (HH) have painful arthritis, often including hindfoot osteoarthritis. Total ankle arthroplasty (TAA) is increasingly recommended for patients with painful ankle osteoarthritis. However, the pain relief and function experienced by patients continues to be debated particularly as compared with ankle fusion.
We believe a curved periacetabular osteotomy is indicated for treatment of severe dysplastic hips with center-edge angles less than 0°, classified as Severin Group IV-b. However, the lower limit of the center-edge angle in hips classified as Severin Group IV-b is not clearly defined to determine which patients should receive periacetabular osteotomy alone.
Lower extremity vascular anomalies have been described for patients with clubfoot but few imaging studies have investigated effects on soft tissues such as fat and muscle. To make these assessments we need noninvasive, noncontrast agents to more safely image children.
Resection of a tumor of the pelvis is most disabling when the acetabulum is excised and a durable reconstruction of the defect is hard to achieve. All available methods are associated with frequent complications. Few large series have been published, and fewer have focused entirely on complete resections of the acetabulum. The use of an allograft-prosthetic composite allows customization on the operating table. However, while such composites restore anatomy and function of the pelvis the use of pelvic allografts is controversial and the durability is unknown.
Displaced scapular body fractures most commonly are treated conservatively. However there is conflicting evidence in the literature regarding the outcomes owing to retrospective design of studies, different classification systems, and diverse outcome tools.
Depot delivery of antimicrobial agents is used for treatment and prevention of bacterial orthopaedic infections; there is little information regarding newer antifungal agents and their potential use in polymethylmethacrylate (PMMA) depot delivery.
Osteogenic Protein-1 Delivered by Hydroxyapatite-coated Implants Improves Bone Ingrowth in Extracortical Bone Bridging
Extracortical bone bridging for treatment of massive bone loss can improve stability and longevity of massive endoprostheses. Osteogenic protein-1 (OP-1), when used with allograft bone, reportedly improves extracortical bone bridging and bone ingrowth.
Healing of Long-term Frozen Orthotopic Bone Allografts is not Affected by MHC Differences Between Donor and Recipient
The use of bone grafting in orthopaedic surgery has increased dramatically in recent years. However, the degree to which immune responses are important for the survival of the allograft is not fully understood. In particular it remains unclear whether differences in the major histocompatibility complex (MHC) influence incorporation of bone allografts and their subsequent biologic performance.
The os peroneum is a sesamoid bone in the peroneus longus tendon. Fractures of the os peroneum are rare. Some authors recommend surgery for active patients.
Currently, the movement that occurs at the site of soft tissue repair cannot be measured accurately in vivo. Radiostereometric analysis (RSA) is the gold standard for measuring movement between two skeletal segments in vivo but its application to studying soft tissue migration has been limited by the unknown stability of tantalum beads in tendons and ligaments and their ability to define rigid bodies in these structures.