Intrathecal baclofen for management of spastic cerebral palsy: multicenter trial.

Intrathecal baclofen infusion has demonstrated effectiveness in decreasing spasticity of spinal origin. Oral antispasticity medication is minimally effective or not well tolerated in cerebral palsy. This study assessed the effectiveness of intrathecal baclofen in reducing spasticity in cerebral palsy. Candidates were screened by randomized, double-blind, intrathecal injections of baclofen and placebo. Responders were defined as those who experienced an average reduction of 1.0 in the lower extremities on the Ashworth Scale for spasticity. Responders received intrathecal baclofen via the SynchroMed System and were followed for up to 43 months. Fifty-one patients completed screening and 44 entered open-label trials. Lower-extremity spasticity decreased from an average baseline score of 3.64 to 1.90 at 39 months. A decrease in upper extremity spasticity was evidenced over the same study period. Forty-two patients reported adverse events. Most common reports were hypotonia, seizures (no new onset), somnolence, and nausea or vomiting. Fifty-nine percent of the patients experienced procedural or system-related events. Spasticity in patients with cerebral palsy can be treated effectively by continuous intrathecal baclofen. Adverse events, although common, were manageable.

Untethered filum terminale presenting as cauda equina lesion.

We report the case of a young girl with recurrent bladder dysfunction. Magnetic resonance imaging performed for evaluation of initial urologic symptoms revealed a low-lying conus medullaris. She underwent an L5 laminectomy and cord untethering by sectioning of the filum terminale. After initial improvement of bladder function, her symptoms returned 4 years later. Repeat magnetic resonance imaging demonstrated a new intradural lesion at L2. At surgery she was found to have an untethered, thickened, coiled filum terminale at L2.

Are dermoid and epidermoid tumors preventable complications of myelomeningocele repair?

Dermoids and epidermoids found at reoperation for tethered cord following myelomeningocele repair have been attributed to inadequate excision of cutaneous elements and 'implantation' in the repair site. This study reviews the pathological findings in excised placodes from fresh myelomeningoceles and specimens from tethered cord release and concludes that hamartomatous lesions are a common feature of the myelodysplastic sequence.

Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome.

After primary repair of myelomeningoceles or lipomyelomeningoceles, late progressive neurologic deterioration commonly occurs due to a treatable cause. In our experience many of these patients have a tethered cord. With early untethering, most patients are stabilized and a significant percent of the patients show improvement in their clinical status. Of 341 tethered cord releases done from 1981 to 1988, we report on 153 patients reoperated upon following primary repair. One hundred were performed after primary closure of a myelomeningocele and 53 after repair of a lipomyelomeningocele. The average age of the patients with a myelomeningocele was 6 years old, and for the spinal lipoma patients, 8 years old. The presenting symptoms were similar; weakness, deterioration in gait, scoliosis, orthopedic deformities, and urinary incontinence represented the most common complaints. All 153 patients were noted to have a tethered cord at operation. Additional pathology (dermoid tumors, hydromyelia, tight filum and diastematomyelia) was present in 30% of the cases. With the use of the CO2 laser for dissection, all but 10 patients could be untethered. Follow-up over an average of 4 years revealed 93% of the patients with a myelomeningocele had stabilization or improvement of their presenting complaints, and 7% had progression of their presenting complaints. All of the lipomyelomeningocele patients had either stabilization or improvement of their presenting complaints. There were no mortalities. Close follow-up and early treatment of this patient population is indicated. With release of the cord a significant portion of the population will have relief or improvement of their presenting complaints.

Selective sacral rhizotomy in children with high pressure neurogenic bladders: preliminary results.

Recent successful introduction of selective rhizotomy in the management of lower extremity spasticity in patients with myelodysplasia has prompted us to use it as a means of managing high pressure neurogenic bladders occasionally encountered in myelodysplastic patients. During the last 1 1/2 years 8 children have undergone selective sacral rhizotomy in an attempt to avert urinary diversion or bladder augmentation. Patient age ranged from 6.5 to 18.5 years. The level of the respective lesions was evenly distributed throughout the spine. At spinal surgery each patient had an electrode placed in the detrusor of the bladder via a suprapubic approach, electromyography electrodes were placed in the perineum and slow fill water cystometry was performed throughout the procedure. Standard electrophysiological stimulation of the nerve roots was performed to identify the rootlets that would only affect the detrusor and spare the external sphincter. Postoperative followup has been obtained on all patients. Of the patients 4 have exhibited significant improvement and they have not required augmentation, 2 have not shown any further deterioration in bladder function, 1 has demonstrated deterioration and 1 still lacks urodynamic followup. Postoperative cystometric studies have revealed a bladder capacity increase of 69% for the group. Uninhibited bladder contractions were abated in all but 1 patient. No patient has been rendered incontinent of urine from the procedure and no patient has had a problem with stool continence as a result of the rhizotomy. It appears that selective rhizotomy of the sacral roots has been able to increase bladder capacity as well as compliance in patients who normally would have been relegated to either bladder augmentation or urinary diversion. While these are encouraging results, some further followup is required to ascertain if the early improvements will be long-lasting.

Anatomical progression of the Chiari II malformation.

To evaluate whether anatomic change of the relationship of the Chiari II malformation and the cranial base was occurring, 22 children with meningomyelocele had serial MRI scans reviewed. A ratio (B/A) was established between the distance from the foramen magnum to the caudalmost portion of herniated cerebellum (B) and the diameter of the foramen magnum (A) and this ratio was compared on serial MRI scans. Eighteen children had an increase in the B/A ratio, two children had a decrease, and two had no change. This indicates that continuous anatomic change of the Chiari II malformation and the skull base is occurring. Clinical deterioration in the older child may be explained by a combination of compressive and traction forces due to this change.

Occipital meningoceles in patients with the Dandy-Walker syndrome.

Occipital cephaloceles in the Dandy-Walker syndrome are not rare. To date, 28 cases have been reported. We report on 8 cases of associated occipital meningocele, totalling 16% of all patients with the Dandy-Walker syndrome in our series. In all patients, communication existed between the posterior fossa cyst and the occipital meningocele. In 2 patients, the occipital meningocele disappeared after cerebrospinal fluid shunting and never required surgical repair. It appears that the cranial defect had ossified and resulted in self-closure. Poor intellectual development in persons with the Dandy-Walker syndrome is associated with the presence of other central nervous system and systemic abnormalities and not with occipital meningoceles alone.

Two siblings with the Chiari I malformation.

Two siblings with symptoms characteristic of the Chiari I malformation are described. The malformations were confirmed by magnetic resonance imaging scans and at surgery. Upon review of the world literature, no instance of the Chiari I malformation in a single family has been reported.

Management of the Dandy-Walker syndrome.

The charts of 50 patients with the Dandy-Walker Syndrome were reviewed. Initial therapy was cystoperitoneal (CP) shunt in 21 (42%), ventriculoperitoneal (VP) shunt in 13 (26%), and both (CPVP) in 7 (14%), with the remaining 9 (18%) requiring no shunting procedure. Conversion from single to double shunt secondary to expansion of the unshunted compartment occurred in 9 (42%) of CP only and 4 (30%) of VP only patients. Final therapy was CP in 12 (24%), VP in 9 (18%), CPVP in 20 (40%) and none in 9 (18%). Patients who were unshunted remained shunt-free. When CPVP shunts were present, there was a statistically significant probability that shunt malfunction was due to the CP component only or both components, but not the VP component only. However, there was no statistical difference in malfunction or complication rates between patients with CP only or VP only shunts. Associated anomalies, intellectual outcome, and mortality were also analyzed. Seizures, hearing or visual problems, various CNS abnormalities, and systemic abnormalities were associated with poor intellectual development and could be used to predict intellectual outcome.

A prospective, randomized study of shunt function and infections as a function of shunt placement.

Much controversy still exists about the relative advantages and disadvantages of anteriorly vs. posteriorly placed shunts in terms of infection rate and duration of function. During a 27-month period, all 121 patients seen requiring new shunt insertions were prospectively randomized to anterior or posterior placement. The mean duration of follow-up was 15 months. 70% of the shunts in the posteriorly placed group vs. 59% of the shunts in the anteriorly placed group did not require further surgery during the study period. In a life-table analysis of shunt survival as a function of placement, the shunts in the posteriorly placed group 'survived' slightly longer without malfunctioning or becoming infected than the anteriorly placed shunts. Therefore, the authors conclude that anteriorly placed shunts offer no advantage over posteriorly placed shunts in terms of shunt malfunction or infection.

Suprarenal occurrence of an adenomatoid tumor.

Adenomatoid tumors are rare, benign mesotheliomas that usually are associated with genito-urinary tract. The suprarenal occurrence of this tumor is uncommon. We present such a case and review the origin of the adenomatoid tumor.

Radiation exposure to the ocular lens during CT scanning.

Measurements of radiation exposure during routine CT scanning were found to average 4.2 R at the level of the ocular lens. This data was combined with CT scanning rate data from a sample of 100 patients followed in the myelomeningocele clinic. The number of scans averaged 7.75/patient with a range of 0-34 scans. Cumulative radiation exposure to date ranged from 0 to 143 R. Radiation dosage nomenclature and acceptable limits are discussed and recommendations for reduction of dosage are presented.

Use of the 'H' reflex recovery curve in selective posterior rhizotomy.

Selective posterior rhizotomy is a safe and effective treatment in carefully selected patients. It produces objective and lasting changes in muscle tone and function without serious sensory complications. The 'H' reflex recovery curve technique allows objective evaluation of sensory rootlets and a physiologic rationale for the selection of abnormal rootlets. In 95 patients this technique has provided good results without significant sensory compromise.

Selective posterior rhizotomy for treatment of progressive spasticity in patients with myelomeningocele. Preliminary report.

This preliminary report describes two cases of progressive spasticity and scoliosis in children with myelomeningocele. Both patients had evidence of tethered spinal cord. Untethering of the spinal cord was successful in arresting the scoliosis but not the spasticity. Selective posterior rhizotomy was successful in resolving the spasticity in both cases. The technique of selective posterior rhizotomy is reviewed.

Surgical treatment of sagittal synostosis.

A technique for the correction of scaphocephaly is described. It is safe, is technically straightforward, and rapidly corrects the deformity. The results of a standardized operative procedure in 50 patients are reviewed.

Lasers in pediatric neurosurgery.

The carbon dioxide (CO2) laser is a microsurgical instrument to be used in conjunction with good neurosurgical technique. Laser surgery has special applications in pediatric neurosurgery because of the type and location of many pediatric tumors as well as the laser qualities of precision and decreased tissue trauma. The CO2 laser has proven to be a valuable surgical tool in this series of pediatric spinal cord and brain tumor patients. The areas of most effective laser treatment were the posterior fossa, ventricles, spinal cord and the suprasellar area. The laser was least helpful in tumors of the hemispheres and the hypothalamus. Criteria need to be developed for absolute and relative indications for CO2 laser use.