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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hip instability occurs in meningomyelocele patients with lumbar level paralysis because of muscle imbalance. Transferring the hip adductor origins to the ischium can be used as the primary procedure to restore muscle balance and prevent hip instability in myelodysplasia patients if performed by the age of one year. Twelve lumbar meningomyelocele patients underwent bilateral adductor transfers. Secondary procedures, such as iliopsoas release or transfer or varus derotation osteotomy, were done in 14 hips. Ten hips needed no further surgery. Twenty of 24 hips were clinically and roentgenographically stable and showed roentgenographic evidence of improving hip development. Instability was associated with scoliosis, congenital dislocation, and age greater than one year at the time of the transfer.
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PMID:The adductor transfer in the high-risk hip in myelodysplasia: a preliminary report. 67 26

The results of this study support the concept that the levels of the bifid vertebral arch and of functional motor activity are significant influences on the development of scoliosis and kyphosis in patients born with myelodysplasia. 'Significant' degrees of scoliosis and kyphosis may appear at any time during life, but most frequently occur during late childhood or adolescence. Not all 'significant' curvatures require treatment, nor do all children with myelodysplasia and factors predisposing to kyphosis and scoliosis develop these complications. It is believed that the preliminary cumulative percentage curves presented in this study will be of value in counselling parents of afflicted newborn infants, and in planning the management of patients born with myelodysplasia.
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PMID:Myelodysplasia: the natural history of kyphosis and scoliosis. A preliminary report. 107 Oct 79

Of a total of 143 myelodysplastic patients treated between 1928 and 1951, there were sixty-three patients with severe myelodysplasia whose records allowed long-term review. At the time of writing twenty-nine were alive and were twenty to forty-three years old. All were walking in the hospital while under an intensive physical therapy program. However only two of the nine with twelfth thoracic-second lumbar function were walking at final follow-up as adults, while nineteen of the twenty with function at the third lumbar level were doing so. The status of the hips did not correlate with the ability to walk. One-third of the survivors were self-supporting at the time of writing. About one-half had scoliosis and in one-third was greater than 20 degrees.
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PMID:Myelodysplastics--fate of those followed for twenty years or more. 109 48

Management of the myelodysplasia patient and his family is best performed in the multidisciplinary setting of a comprehensive care clinic. Careful initial evaluation by each subspecialist on the team followed by family counseling provides the basis for selection of patients for treatment. Subsequently each subspecialist contributes up-to-date assessment and on-going medical care. Prevention of progressive orthopedic deformity through the use of plaster casts may minimize the need for surgical treatment. Severe deformities may require muscle balancing procedures. Aggressive surgical intervention is indicated in cases of progressive scoliosis and lordoscoliosis. Minimal urologic evaluation and surveillance guidelines have been developed, and prevention of irreversible renal damage is possible. Chemotherapy, adequate bladder drainage, and sophisticated techniques including sphincter and bladder electromyography and the use of artificial urinary sphincters and bladder pacemakers contribute to improved urologic management. The emotional needs of the patient and his family are complex, and support by the psychiatrist and the social worker as well as all other team members is necessary for adequate development of a well adjusted child in the face of physical handicaps.
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PMID:The role of the psychiatrist in myelodysplasia. 126 39

A computerized pressure plate system was used to analyze the sitting pressure distribution and balance of 100 subjects with both normal and abnormal spines. Normal subjects had a balanced sitting posture with the weight taken evenly on each ischial tuberosity. Patients with idiopathic scoliosis showed significant sitting imbalance especially in the presence of lumbar curves. Bracing improved the sitting balance and stability in most patients. Abnormal sitting patterns were seen with the spinal deformities of myelodysplasia or cerebral palsy. Some patients with severe scoliosis had very asymmetric patterns, which correlated with their loss of sitting stability. The kyphosis of myelodysplasia produced an abnormal tripod sitting pattern due to pelvic flexion and a hyperlordosis of cerebral palsy a pubic sitting pattern due to pelvic extension. The patients studied after spinal fusion also had poor sitting balance and occasionally persisting decubitus ulceration.
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PMID:Sitting balance in spinal deformity. 141 65

To investigate the relationship between unilateral hip dislocation or subluxation and scoliosis, the records were examined of 31 patients aged 10 to 26 years with myelomeningocele and an initial neurosegmental level of L3 or L4. 11 patients had deteriorated neurologically over time; the remainder had stable neurological examinations, with an average follow-up of 14.3 years. Scoliosis occurred in three of the nine patients with unilateral hip instability, compared with five of the 11 remaining patients. Only two patients had the expected pattern of unilateral hip instability on the high side of an oblique pelvis, with scoliosis convex to the opposite (low) side. Several unexpected patterns were observed. The functional neurosegmental level is not stable in many patients with myelodysplasia. In neurologically stable patients, unilateral hip instability and scoliosis are comorbid factors and no causal relationship was identified.
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PMID:The myelodysplastic hip and scoliosis. 155 3

A prospective study was undertaken in 1985 to better understand how the surgical manipulation of hindbrain herniation affected abnormal spinal curvature. Eleven patients under 16 years of age with Chiari malformation (not associated with myelodysplasia) and scoliosis of at least 15 degrees were studied. The mean curve angle at the time of original treatment was 29 degrees, with the convexity to the right in seven patients. The curvature was rapidly progressing in four patients. The most common presenting signs were myelopathy and weakness. Investigative procedures included spine radiographs with the patient standing and magnetic resonance (MR) imaging of the brain, spinal cord, and craniovertebral junction. Eight children had associated hydrosyringomyelia. Surgical intervention consisted of a dorsal posterior fossa decompression in all patients and a transoral ventral decompression of the cervicomedullary junction in five. All patients were followed at 3, 6, and 12 months, and at yearly intervals thereafter with clinical evaluations, spine radiographs in the standing position, and postoperative MR imaging. The mean follow-up period was 35 months. The scoliosis improved in eight patients, stabilized in one, and progressed in two. Only one child required postoperative spinal fusion and instrumentation for progression of scoliosis. Hematomyelia or hematobulbia was associated with persistent scoliosis in two patients. The presence of hydrosyringomyelia and bone erosion did not preclude curve improvement. All patients under 10 years of age had resolution of their scoliosis, despite preoperative curves of more than 40 degrees. These findings emphasize the importance of early surgical intervention, with the restoration of normal cerebrospinal fluid dynamics at the craniovertebral junction in children with symptomatic Chiari malformations.
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PMID:Scoliosis in pediatric Chiari malformations without myelodysplasia. 160 74

The physical therapy and orthopedic management of patients with myelodysplasia from infancy to adulthood are reviewed. The overall goal for the child with myelodysplasia is functional independence. Physical therapy and orthopedic intervention enable the individual to achieve this goal. Associated problems, however, such as Arnold-Chiari malformation, hydrocephalus, and tethered spinal cord, influence functional expectations. Physical therapy management begins in the neonatal period and continues through adolescence. Treatment is modified at the various stages of development. Knowledge of current orthotic and adaptive equipment is necessary to achieve optimal locomotor function. Orthopedic management decisions are based on musculoskeletal and neurologic assessments, to which the physical therapist provides a significant contribution. Controversies exist over the orthopedic management of dislocated hips, scoliosis, and kyphosis.
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PMID:Myelodysplasia--the musculoskeletal problem: habilitation from infancy to adulthood. 194 27

Transiliac limb lengthening osteotomy is a modified Salter innominate osteotomy which uses a trapezoidal interposition bone graft instead of the usual triangular graft to achieve up to 3 cm of intrapelvic lengthening. It allows correction of certain forms of postural imbalance and pelvic obliquity, as well as allowing an optimal and variable amount of acetabular redirection. This review of 105 patients who have undergone the procedure at our institution revealed the following indications: decompensated scoliosis, 26 patients; acetabular dysplasia with ipsilateral femoral shortening, 34 patients; intrapelvic asymmetry, 7 patients; pure limb length inequality, 38 patients. Intrapelvic lengthening averaged 2.5 cm. Correction of decompensated scoliotic curves averaged 7 degrees reduction in Cobb angle. Improvement in center-edge angle in patients with hip dysplasia averaged 17 degrees. Reduction in size of shoe lift required to balance the trunk in all cases was correlated closely with the intrapelvic lengthening achieved. Complications included two transient neurapraxias (one femoral, one femoral and sciatic), two cases of sacroiliac subluxation (one patient with myelodysplasia, and one patient with polio), three cases of partial graft collapse, and six instances of broken fixation pins, and three deep wound infections. No patient had chondrolysis or avascular necrosis of the femoral head. After average follow-up of nearly 5 years (minimum follow-up 2 years), patients who underwent transiliac limb lengthening osteotomy for correction of postural imbalance for various causes retained satisfactory correction.
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PMID:[Transiliac leg lengthening: experiences with a modified Salter osteotomy]. 223 60

Objective criteria are available for decision making in children with ventriculomegaly and spina bifida cystica. Figure 29.7 is the evaluation algorithm used in the Hydrocephalus/Myelodysplasia Clinic at Rainbow Babies and Children's Hospital. In children without serious neurosurgical complications such as the Chiari crisis or problems with wound healing, we rely on three reasonably objective measurements for decision making. Head circumference: Measured daily while in hospital and at each visit. If the pattern of head growth crosses multiple percentile lines indicating that the child will be severely megalencephalic, a shunt will be performed. Ultrasonography: Ultrasound determinations are made in the first few days of life, prior to discharge, at 6 weeks of age, and each 6 weeks of age until 6 months. Some measurements of ventricular size (usually CT scan because of a small anterior fontanelle) should be made at age 1 year. Denver Developmental Testing (DDST): These are performed at age 6 weeks and each 6 weeks thereafter. If the child shows significant ventriculomegaly, a shunt is performed. When the results are questionable the decision is delayed and the test repeated in 6 weeks. Whether a shunt is or is not placed in an infant with ventriculomegaly and myelodysplasia, follow-up must remain compulsive. Following shunting, not only should the head circumference stabilize, but the cortical mantle should increase. Often children shunted in this situation fail to show signs of increased intracranial pressure with shunt malfunction and must be followed with serial head circumference measurements as well as ultrasounds and CT scans. If the decision is made not to shunt the child the work of Hall et al. (10) would suggest the possibility that later in life shunts may be needed to prevent scoliosis secondary to hydromyelia. More information is needed as the aggressively treated population become adults.
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PMID:To shunt or not to shunt: hydrocephalus and dysraphism. 241 85


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