Activated factor IX complex in treatment of surgical cases of hemophilia A with inhibitors.

Three patients with severe hemophilia A with inhibitors to factor VIII were treated with activated factor IX complex. Bleeding was controlled adequately during surgical procedures involving each of the three. Partial thromboplastin times showed a variable shortening and prothrombin times were significantly shortened to values less than normal. Hemostasis was substantiated by the use of epsilon aminocaproic acid. Neither anamnestic responses nor thrombotic complications were observed. A transient hypertension developed in two patients shortly after infusion with the activated factor IX complex.

Scoliosis in the institutionalized cerebral palsy population.

A prospective review of 272 institutionalized cerebral palsy residents was undertaken in order to determine the incidence and characteristics of neuromuscular scoliosis in this population. The types of cerebral palsy in the group consisted of 75% spastic, 8% dyskinetic, 4% ataxic, 8% mixed, and 5% undefined. There was a 64% incidence of roentgenographic scoliosis greater than 10 degrees. Two distinct curve patterns were determined with equal frequency, single and multiple. The significance of the curve patterns could not be determined. Scoliosis was most common in the spastic group with the highest incidence in the spastic quadriplegics. The incidence directly paralleled the severity of the neurologic deficit but also appeared to be aggravated by the effects of gravity when the individuals were artificially placed in the sitting position. There was a definite inverse relationship between the level of ambulation and scoliosis: the higher the level of ambulation the lower the incidence of scoliosis. Hip stability per se could not be correlated with the incidence of scoliosis. The most important factors in predicting scoliosis in this population are the presence of spasticity and the severity of the neurologic deficit.

A new technique to improve cortical-evoked potentials in spinal cord monitoring. A ratio method of analysis.

Systemic effects such as anesthesia, hypotension, hypothermia, and hypoxia affect the cortical evoked responses. We propose, that by sequential stimulation of the median and posterior tibial nerves, and the construction of a ratio from the value of their amplitudes, the systemic effects can be eliminated and thus improve the reliability of the cortical evoked responses. Two groups of scoliosis patients who underwent spinal surgery with instrumentation were analyzed retrospectively. Both groups had spinal cord monitoring using peripheral nerve stimulation and cortical recordings of the somatosensory-evoked response (SER). In Group I, 50 patients were analyzed for changes in posterior tibial nerve response before and after distraction. Wide variability in the response suggested this method to be less reliable in predicting spinal cord conduction deficits. Thirty-eight patients in Group 2 were analyzed using both the median and posterior tibial nerve amplitudes. A ratio of the posterior tibial to median nerve wave amplitude was constructed, thus eliminating any systemic variables. A critical value, alerting the surgeons to possible decreases in spinal cord conduction, was calculated by subtracting one standard deviation from the mean of the postdistraction ratios of the posterior tibial to median nerves (1.20-.633 = .567).

3-S prosthesis: a preliminary report.

We report a new prosthetic suspension system ideally suited for active young amputees. The silicone suction socket (3-S) utilizes a silicone sleeve that intimately fits the residual limb, providing a suction-type suspension for both upper and lower extremities. Seven juvenile amputees were evaluated. Six were successful and one was deemed a failure. Details of the design concepts, fabrication, and method of donning the prosthesis are described. We believe that the 3-S design is a significant improvement over conventional prosthetic suspension, offering important advantages to the active pediatric amputee.

Paralytic instability of the hip in myelomengoceles.

The results of a Colonna type arthroplasty with several modifications were evaluated in 29 hips of 22 myelomeningocele children with subluxations or dislocations. The operations were performed only in children with high neurosegmental levels where no muscle transfers were possible or when previous surgical procedures failed to maintain hip stability. Improvement in radiographic and clinical stability was demonstrated in 79 per cent of the hips. Eighty-three per cent of the hips lost motion in the flexion-extension arch with a mean loss of 30 degrees. The arthroplasty improved hip stability but at the expense of some loss of flexion-extension. Favorable results are more likely when the neurosegmental level is T-12 or above, the hip has had no previous operative procedure and the arthroplasty is accomplished without capsular interposition. Improvement of ambulatory status was impossible to evaluate because in any one case, the level of ambulation was determined by influences and physical factors other than the operation.