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

Chiari malformations without myelodysplasia are rarely diagnosed in the pediatric age group. With current neurodiagnostic techniques, however, they are being seen more frequently. Unfortunately, the prognosis is not clear because publications have included a number of different entities, used a variety of surgical approaches, and lacked long term follow-up. Sixteen patients younger than 20 years were treated for Chiari malformations (without myelodysplasia) between 1975 and 1985. The average age was 11 years, and the average duration of symptoms was 20 months. The common symptoms were isolated motor weakness (56%), pain (37.5%), and sensory loss (25%). Frequently seen signs were motor deficit (81%), sensory loss (50%), scoliosis (50%), and cranial nerve palsy (50%). The surgical procedures used were foramen magnum decompression (3 transoral clivus odontoid resections and 15 posterior fossa decompressions with dural grafting), alteration of cerebrospinal fluid (CSF) pathways at the cervicomedullary junction (plugging the foramen cecum and a 4th ventricle to subarachnoid shunt with posterior fossa decompression), and ventriculoperitoneal shunting (2 cases). In follow-up, 37.5% of the patients are asymptomatic, 50% are improved, and 12.5% are stable after an average follow-up period of 43 months. The asymptomatic group was younger (9.3 years) and had a shorter symptom duration (7.2 months) than both the improved (11.9 years, 16.4 months) and the stable groups (15 years, 20 months). Optimal outcome depends on complete evaluation of the abnormal CSF pathways and bony abnormalities at the craniovertebral junction. Operation is then directed toward correction of these abnormalities as delineated radiographically. Of our patients, 87.5% have at least shown improvement, which has been long term in all cases.
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PMID:Presentation and management of pediatric Chiari malformations without myelodysplasia. 275 73

A previously healthy 13-year-old boy without myelodysplasia who had mild scoliosis was seen with complaints of nasal congestion, noisy nighttime breathing, and difficulty sleeping. Flattening of the inspiratory loop on the flow-volume curve was found on pulmonary function testing, suggesting a variable extrathoracic obstruction due to a laryngeal lesion. Bilateral abductor vocal cord paralysis and sleep apnea developed precipitously following general anesthesia. Further workup demonstrated a type-I Chiari malformation with syringomyelia. Brainstem abnormalities such as Chiari malformation with secondary tenth cranial nerve deficits should be considered in previously healthy children and adolescents with signs and symptoms of upper airway obstruction and apnea.
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PMID:Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. 361 93

Two hundred and fifty cases of myelodysplasia were reviewed in relation to spinal deformity. Approximately half of the children had, or were expected to develop, curves severe enough to need operations and only 10 per cent maintained completely undeformed spines. The most frequent deformity was scoliosis which could be subdivided into congenital and developmental types. The latter was of mixed aetiology, neuromuscular imbalance and asymmetry of the neural arch both contributing, while in some cases no causative factors could be identified. The best early indicator that developmental scoliosis was likely to appear was a high segmental level of both the neurological deficit and the neural arch defect. Deformity was very unlikely to start after the age of nine years.
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PMID:The natural history of scoliosis in myelodysplasia. 698 15

Twenty-seven patients have undergone posterolateral spinal fusion for the treatment of scoliosis associated with myelodysplasia. Seventeen patients were girls, and 10 were boys. The average age at the time of surgery was 10.6 years in the girls and 11 years in the boys. The average preoperative curve was 78.52 degrees. All patients were fused at the sacrum; the upper level of fusion was at the fourth thoracic vertebra in 20 patients and at the third thoracic vertebra in 7 patients. Harrington instrumentation was used in 5 patients. The average improvement in curvature was 44.5%. Based on clinical and functional criteria, 16 patients had good results, 4 patients had fair results, and 7 patients had poor results.
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PMID:Posterolateral spinal fusion for the treatment of scoliosis in myelodysplasia. 716 86

The results of a prospective study of the surgical treatment of paralytic scoliosis and congenital lumbar kyphosis in 19 children with myelodysplasia and one child with sacral agenesis indicate that improved results can be achieved with the use of staged anterior and posterior fusions, more rigid spinal instrumentation, and bank and autogenous bone graft. No wound infections occurred as a result of preoperative treatment of urinary infection and with the use of prophylactic antibiotics. Improved posterior spinal fixation was accomplished with a double square end sacral hook in paralytic scoliosis and pelvic obliquity and with a cable-hook compression system in congenital lumbar kyphosis. Posterior lumbopelvic fusion with cable-hook compression instrumentation gave acceptable results in lumbopelvic kyphosis from complete sacral agenesis.
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PMID:Severe spine deformity in myelodysplasia and sacral agenesis: an aggressive surgical approach. 730 85

We attempted to characterize the signs and symptoms of patients presenting with scoliosis as a manifestation of occult syringomyelia, and to determine the effect of syrinx decompression on the deformity and neurologic deficits. This study represented 21 patients treated from 1981 through 1991 at the authors' institution. All patients with occult syringomyelia and no other central nervous system lesions underwent decompression of the syrinx, with improvement in neurologic signs and symptoms. Three of the four with scoliosis that were not treated by arthrodesis showed improvement of their deformity at an average follow-up of 35 months. Three of five patients with myelodysplasia, occult syringomyelia, and scoliosis not previously treated with spinal arthrodesis showed stabilization or improvement of their deformity at an average follow-up of 21 months. We conclude that syringomyelia is associated with a high incidence of developmental scoliosis, and that decompression of the syrinx leads to improvement in, or stabilization of, the majority of scoliotic curves, or postpones the need for fusion.
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PMID:Syringomyelia and developmental scoliosis. 796 97

There are advantages to an upright posture and ambulation in pediatric myelodysplasia patients. The reciprocating gait orthosis (RGO) is a useful tool to enable this activity in selected individuals. We evaluated the long-term usage, pitfalls, and contraindications of this orthosis. Mean daily usage was 6.9 h/day, requiring an average of 10 min to don or doff; all patients required assistance. Obesity, advanced age, lack of patient or family motivation, scoliosis, and spasticity were significant negative factors in long-term usage of the RGO. Good upper extremity strength, trunk balance, previous standing or walking, and active hip flexion were important positive variables. Eleven of 21 patients did not persist with long-term usage of this orthosis, averaging 25.8 months of usage. An energy study was performed on three patients, comparing the efficiency of reciprocating gait and swing-through gait. All three were more energy efficient and two were faster with the swing-through gait but each preferred the reciprocating pattern. Care should be taken when selecting patients for RGO usage because not all children with myelomeningocele are able to function effectively with this orthosis.
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PMID:Use of the reciprocating gait orthosis in myelodysplasia. 849 69

Chiari malformation is a developmental disorder that is often associated with other abnormalities of the cerebrospinal axis. Despite widespread recognition of this association, there is relatively little information on the treatment of these coexisting disorders in the setting of cerebellar tonsillar ectopia. In an effort to improve the care provided to pediatric patients with Chiari malformations the authors reviewed their management practices over the last 20 years. Specifically, they recorded presenting symptoms, radiological studies, comorbidities, and management (surgical and nonsurgical) of 112 patients (all < 20 years of age) with Chiari malformation without myelodysplasia. They found an associated syrinx in 29% of patients, basilar invagination in 17%, and scoliosis in 14%. The basal angle varied from 120 to 190 degrees and Boogard's angle varied from 120 to 220 degrees ; both angles were larger than those measured in normal controls. The vertical height of the posterior fossa was shortened and the volume decreased as compared with normal controls. The surgical management of this group of patients included posterior decompressive (44%), combined transoral and posterior decompressive (31%), combined posterior decompressive and posterior fusion (8%), and multiple posterior decompressive procedures in the same patient (5%). The authors conclude that pediatric patients with a Chiari malformation should be specifically examined for evidence of additional craniovertebral malformations so that procedures are directed at correcting both the comorbidities and the herniation of the cerebellar tonsils through the foramen.
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PMID:Comprehensive approach to Chiari malformation in pediatric patients. 1697 50

In this review, basic knowledge and recent innovation of surgical treatment for scoliosis will be described. Surgical treatment for scoliosis is indicated, in general, for the curve exceeding 45 or 50 degrees by the Cobb's method on the ground that:1) Curves larger than 50 degrees progress even after skeletal maturity.2) Curves of greater magnitude cause loss of pulmonary function, and much larger curves cause respiratory failure.3) Larger the curve progress, more difficult to treat with surgery.Posterior fusion with instrumentation has been a standard of the surgical treatment for scoliosis. In modern instrumentation systems, more anchors are used to connect the rod and the spine, resulting in better correction and less frequent implant failures. Segmental pedicle screw constructs or hybrid constructs using pedicle screws, hooks, and wires are the trend of today.Anterior instrumentation surgery had been a choice of treatment for the thoracolumbar and lumbar scoliosis because better correction can be obtained with shorter fusion levels. Recently, superiority of anterior surgery for the thoracolumbar and lumbar scoliosis has been lost. Initial enthusiasm for anterior instrumentation for the thoracic curve using video assisted thoracoscopic surgery technique has faded out.Various attempts are being made with use of fusionless surgery. To control growth, epiphysiodesis on the convex side of the deformity with or without instrumentation is a technique to provide gradual progressive correction and to arrest the deterioration of the curves. To avoid fusion for skeletally immature children with spinal cord injury or myelodysplasia, vertebral wedge ostetomies are performed for the treatment of progressive paralytic scoliosis. For right thoracic curve with idiopathic scoliosis, multiple vertebral wedge osteotomies without fusion are performed. To provide correction and maintain it during the growing years while allowing spinal growth for early onset scoliosis, technique of instrumentation without fusion or with limited fusion using dual rod instrumentation has been developed. To increase the volume of the thorax in thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis, vertical expandable prosthetic titanium ribs has been developed.
Scoliosis 2008 Apr 18
PMID:Surgical treatment of scoliosis: a review of techniques currently applied. 1842 27

The incidence of surgical site infection (SSI) after spinal deformity surgery for adolescent idiopathic scoliosis ranges from 0.5-6.7%. The risk of infection following spinal fusion in patients with neuromuscular scoliosis is greater, with reported rates of 6.1-15.2% for cerebral palsy and 8-41.7% for myelodysplasia. SSIs result in increased patient morbidity, multiple operations, prolonged hospital stays, and significant financial costs. Recent literature has focused on elucidating the most common organisms involved in SSIs, as well as identifying modifiable risk factors and prevention strategies that may decrease the rates of infection. These include malnutrition, positive urine cultures, antibiotic prophylaxis, surgical site antisepsis, antibiotic-loaded allograft, local application of antibiotics, and irrigation solutions. Acute and delayed SSIs are managed differently. Removal of instrumentation is required for effective treatment of delayed SSIs. This review article examines the current literature on the prevention and management of SSIs after pediatric spinal deformity surgery.
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PMID:Surgical site infection after pediatric spinal deformity surgery. 2231 61


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