MOBILITY AIDS FOR CHILDREN WITH HIGH-LEVEL MYELOMENINGOCELE: PARAPODIUM VERSUS WHEELCHAIR

The optimal method for providing mobility for children with myelomeningocele remains controversial. 39 children using a parapodium were compared with 29 children in wheelchairs. There were no significant differences between the two groups for medical complications, use of health-care services or activities of daily living, although there were differences in the patterns of complications. Children using the parapodium were more likely to develop lesions of the lower extremities, to have dislocated hips, to be more obese and to watch more television; children using wheelchairs were more likely to develop lesions of the gluteal region, to have knee-flexion contractures and to have fewer fecal accidents. The parapodium was judged by families to be less effective as a mobility aid; however, the upright posture it allows was considered extremely advantageous. A combined approach allowing upright posture and wheeled mobility would appear to be optimal.     

External fixation of lower limb fractures in children.

Thirty-four tibial and femoral shaft fractures in 32 children between the ages of 3 and 15 years were treated by external fixation over a 5-year period. The indications were fractures occurring in association with other major injuries and failure of conservative treatment to maintain satisfactory reduction. There was one case of delayed union and one early refracture. The overall pin track infection rate was 6%, but the rate for the tibial pins (2.1%) was much lower than for the femoral pins (10.3%). Union was achieved at an average of 11.7 weeks in the femoral fractures and 10.0 weeks in the tibial fractures. The use of external fixation is recommended for childhood femoral and tibial fractures, particularly in children with multiple injuries.     

Executive cognitive deficits in primary dystonia.

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.     

Arthroscopic distal clavicle resection

The acromioclavicular joint is a potential source of pain in the shoulder. There are a variety of disorders that can affect this joint, including distal clavicle osteolysis, posttraumatic arthritis, osteoarthritis, and rheumatoid arthritis. Nonoperative treatment for this condition with nonsteroidal medication and activity modification can alleviate the pain. When conservative treatment is exhausted, surgical resection of the distal clavicle is often necessary. Arthroscopic resection of the distal clavicle preserves the acromioclavicular ligaments to prevent postoperative distal clavicle instability. The procedure is performed in either the beach chair or lateral position and requires the use of a shaver, electrocautery, and a burr for soft tissue and debridement and bone resection.     

Early experience with a novel plaque excision system for the treatment of complex coronary lesions.

The use of directional coronary atherectomy (DCA) in current practice has been limited. The SilverHawk System is a newly developed plaque excision device that aims to overcome the drawbacks of prior DCA platforms. The device was evaluated in a porcine coronary model and in a series of patients. Procedural variables along with outcomes were reviewed. Quantitative angiography (QCA) was performed and excised tissue fragments were weighed and examined histologically. In porcine cases, pretreatment MLD increased from 0.51 +/- 0.26 to 2.36 +/- 0.59 mm postdebulking and 19.9 +/- 7.6 mg of tissue was retrieved. In human cases, pretreatment MLD increased from 0.8 +/- 0.4 to 2.2 +/- 0.5 mm postdebulking and 15.2 +/- 7.8 mg of tissue was retrieved without complications. These data show that the SilverHawk System may offer significant utility in treating a wide variety of complex coronary lesions.     

Effect of the Aldehyde Dehydrogenase Inhibitor, Cyanamide, on the Ex Vivo Sensitivity of Murine Multipotent and Committed Hematopoietic Progenitor Cells to Mafosfamide (Asta Z 7557)

The ex vivo sensitivity of murine multipotent (CFU-GEMM) and committed (CFU-Mk, CFU-GM, BFU-E and CFU-E) hematopoietic progenitor cells to mafosfamide was quantified with and without concurrent exposure to cyanamide, an inhibitor of aldehyde dehydrogenase activity. In the absence of cyanamide, CFU-GEMM, CFU-Mk and CFU-GM were approximately equisensitive to mafosfamide while the erythroid progenitors were more sensitive to the drug. Cyanamide potentiated the cytotoxicity of mafosfamide toward CFU-GEMM and CFU-Mk, but not toward CFU-GM, BFU-E and CFU-E. Cellular aldehyde dehydrogenases are known to catalyze the oxidation of 4-hydroxycyclophos-phamide/aldophosphamide, the major intermediate in cyclophosphamide and mafosfamide activation, to the relatively nontoxic acid, carboxyphosphamide. Thus, our findings indicate that 1) murine CFU-GEMM contain the relevant aldehyde dehydrogenase activity, and 2) the relevant aldehyde dehydrogenase activity is retained upon differentiation to progenitors committed to the megakaryocytoid lineage, but lost upon differentiation to progenitors committed to the granulocytoid/monocytoid and erythroid lineages. The relative insensitivity of CFU-GM to mafosfamide is apparently due to a cellular determinant that influences their sensitivity to all cross-Unking agents since CFU-GM were found to be relatively insensitive to non-oxazaphosphorine cross-linking agents as well.     

Infantile myofibromatosis

 Infantile myofibromatosis is a mesenchymal tumor most commonly seen in infancy. The tumors have a variable appearance on CT/MR and often simulate a more aggressive neoplasm. This report describes CT/MR findings in cases of infantile myofibromatosis with pathologic correlation. Discussion into the success of imaging in suggesting the correct diagnosis is also addressed. Infantile myofibromatosis is a mesenchymal disorder of infancy characterized by the presence of tumorous nodules in the skin, subcutaneous tissue, muscle, viscera, and bone. Cases of solitary and multiple lesions have been described. We present the clinical, histologic, and radiographic findings of one case of the solitary form of infantile myofibromatosis that was recently diagnosed at our hospital.     

Intracapsular implant rupture: MR findings of incomplete shell collapse

The objective of this study was to determine the frequency and significance of the MR findings of incomplete shell collapse for detecting implant rupture in a series of surgically removed breast prostheses. MR images of 86 breast implants in 44 patients were studied retrospectively and correlated with surgical findings at explanation. MR findings included (a) complete shell collapse (linguine sign), 21 implants; (b) incomplete shell collapse (subcapsular line sign, teardrop sign, and keyhole sign), 33 implants; (c) radial folds, 31 implants; and (d) normal, 1 implant. The subcapsular line sign was seen in 26 implants, the teardrop sign was seen in 27 implants, and the keyhole sign was seen in 23 implants. At surgery, 48 implants were found to be ruptured and 38 were intact. The MR findings of ruptured implants showed signs of incomplete collapse in 52% (n = 25), linguine sign in 44% (n = 21), and radial folds in 4% (n = 2). The linguine sign perfectly predicted implant rupture, but sensitivity was low. Findings of incomplete shell collapse improved sensitivity and negative predictive values, and the subcapsular line sign produced a significant incremental increase in predictive ability. MRI signs of incomplete shell collapse were more common than the linguine sign in ruptured implants and are significant contributors to the high sensitivity and negative predictive values of MRI for evaluating implant integrity.     

Post-operative progress of dystonia patients following globus pallidus internus deep brain stimulation

In the current era of functional surgery for movement disorders, deep brain stimulation (DBS) of the globus pallidus internus (GPi) is emerging as the favoured intervention for patients with dystonia. Here we report our results in 20 patients with medically intractable dystonia treated with GPi stimulation. The series comprised 14 patients with generalized dystonia and six with spasmodic torticollis. Although comparisons were limited by differences in their respective neurological rating scales, chronic DBS clearly benefited both patient groups. Data conveying the rate of change in neurological function following intervention are also presented, demonstrating the gradual but progressive and sustained nature of improvement following stimulation of the GPi in dystonic patients.