Neurological and spinal manifestations of the Ehlers-Danlos syndromes.

The Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin extensibility, and tissue fragility. This communication briefly reports upon the neurological manifestations that arise including the weakness of the ligaments of the craniocervical junction and spine, early disc degeneration, and the weakness of the epineurium and perineurium surrounding peripheral nerves. Entrapment, deformation, and biophysical deformative stresses exerted upon the nervous system may alter gene expression, neuronal function and phenotypic expression.

Microsurgical treatment of sacral perineural (Tarlov) cysts: case series and review of the literature.

OBJECTIVE Tarlov cysts (TCs) occur most commonly on extradural components of the sacral and coccygeal nerve roots. These lesions are often found incidentally, with an estimated prevalence of 4%-9%. Given the low estimated rates of symptomatic TC and the fact that symptoms can overlap with other common causes of low-back pain, optimal management of this entity is a matter of ongoing debate.

Prevention of percutaneous spinal cord stimulation electrode migration: a 15-year experience.

Objectives: Percutaneous spinal cord stimulation electrodes have a propensity to migrate longitudinally, which is a costly complication that often compromises therapeutic effect. After implementing simple changes to our percutaneous electrode anchoring technique, we no longer encounter this migration. The current retrospective study updates previously reported results.

Off-label use of drugs and devices: role of medical professionals in the establishment of parameters for their use.

Unlabelled: Physicians may use approved drugs and devices in ways not approved by the US Food and Drug Administration, a process termed off-label use. Often, because there is not suitable data defining safety and efficacy, the results of such use may become problematic, as occurred with the off-label use by spine surgeons of bone morphogenetic proteins in anterior cervical fusions. Using this undesirable history of bone morphogenetic proteins, and the historical record of the introduction of another drug, chymopapain, a method is developed and presented whereby such drugs or devices in the future may be studied by the professional medical specialties themselves outside the New Drug Application process, but with appropriate input from government and industry.

Tarlov cysts: an overlooked clinical problem.

Symptomatic Tarlov cysts typically cause chronic pelvic and lower extremity pain and sacral nerve root radiculopathy. Historically, open surgical treatment involved significant patient morbidity, particularly postoperative cerebrospinal fluid (CSF) leaks and infection. These CSF leaks often required multiple surgical procedures to seal.

Perioperative and long-term outcomes from the management of parasagittal meningiomas invading the superior sagittal sinus.

Background: Parasagittal meningiomas invading the superior sagittal sinus (SSS) pose formidable obstacles to surgical management. Invasion is often considered a contraindication to surgery because of associated morbidity, such as cerebral venous thrombosis.

Objective: We report our most recent experience with the resection of parasagittal meningiomas invading the SSS.

The role of assessment in competency-based medical education.

Competency-based medical education (CBME), by definition, necessitates a robust and multifaceted assessment system. Assessment and the judgments or evaluations that arise from it are important at the level of the trainee, the program, and the public. When designing an assessment system for CBME, medical education leaders must attend to the context of the multiple settings where clinical training occurs.

Competency-based medical education: theory to practice.

Although competency-based medical education (CBME) has attracted renewed interest in recent years among educators and policy-makers in the health care professions, there is little agreement on many aspects of this paradigm. We convened a unique partnership - the International CBME Collaborators - to examine conceptual issues and current debates in CBME. We engaged in a multi-stage group process and held a consensus conference with the aim of reviewing the scholarly literature of competency-based medical education, identifying controversies in need of clarification, proposing definitions and concepts that could be useful to educators across many jurisdictions, and exploring future directions for this approach to preparing health professionals.

Long-term back pain after a single-level discectomy for radiculopathy: incidence and health care cost analysis.

Object: The most common spinal procedure performed in the US is lumbar discectomy for disc herniation. Longterm disc degeneration and height loss occur in many patients after lumbar discectomy. The incidence of mechanical back pain following discectomy varies widely in the literature, and its associated health care costs are unknown.

Recurrent lumbar disc herniation after single-level lumbar discectomy: incidence and health care cost analysis.

Objective: Same-level recurrent lumbar disc herniation complicates outcomes after primary discectomy in a subset of patients. The health care costs associated with the management of this complication are currently unknown. We set out to identify the incidence and health care cost of same-level recurrent disc herniation after single-level lumbar discectomy at our institution.

Genesis of the use of corticosteroids in the treatment and prevention of brain edema.

Background: Since the groundbreaking article from the University of Minnesota in 1961 by Drs. Galicich, French, and Melby describing the use of dexamethasone for peritumoral cerebral edema, the use of corticosteroids in patients with brain tumors has become routine. Unfortunately, little has been reported regarding the environment that fostered arguably the greatest translational research contribution in the history of neurosurgery.

Laminar screw fixation of the axis.

Object: Laminar fixation of the axis with crossing bilateral screws has been shown to provide rigid fixation with a theoretically decreased risk of vertebral artery damage compared with C1-2 transarticular screw fixation and C-2 pedicle screw fixation. Some studies, however, have shown restricted rigidity of such screws compared with C-2 pedicle screws, and others note that anatomical variability exists within the posterior elements of the axis that may have an impact on successful placement. To elucidate the clinical impact of such screws, the authors report their experience in placing C-2 laminar screws in adult patients over a 2-year period, with emphasis on clinical outcome and technical placement.

Laparoscopic treatment of anterior sacral meningocele.

Background: Anterior sacral meningocele is a rare congenital malformation, whose open surgical treatment is well accepted. We present a laparoscopic approach as an adjunctive approach.

Methods: Five women who underwent laparoscopic transperitoneal surgery were clinically, radiologically, and surgically evaluated.

Reduction of cerebrospinal fluid rhinorrhea after vestibular schwannoma surgery by reconstruction of the drilled porus acusticus with hydroxyapatite bone cement.

Object: Cerebrospinal fluid (CSF) rhinorrhea remains a significant cause of morbidity after resection of vestibular schwannomas (VSs), with rates of rhinorrhea after this procedure reported to range between 0 and 27%. The authors investigated whether reconstruction of the drilled posterior wall of the porus acusticus with hydroxyapatite cement (HAC) would decrease the incidence of postoperative CSF rhinorrhea.

Methods: A prospective observational study of 130 consecutive patients who underwent surgery for reconstruction of the posterior wall of the drilled porus acusticus with HAC was conducted between October 2002 and September 2005.

Prospective evaluation of pain and analgesic use following major elective intracranial surgery.

Object: Opioid administration after major intracranial surgery is often limited by a presumed lack of need and a concern that opioids will adversely affect the postoperative neurological examination. The authors conducted a prospective study to evaluate the incidence, severity, and treatment of postoperative pain in patients who underwent major intracranial surgery.

Methods: One hundred eighty-seven patients (77 men and 110 women, mean age 52 +/- 15 years, mean weight 78.

The Lumbar Spine Outcomes Questionnaire: its development and psychometric properties.

Background Content: There are currently a number of generic and disease-specific instruments for assessing complaints of low back pain (LBP). None provide the comprehensive coverage of the wide range of factors that are considered essential in evaluating treatment outcomes.

Purpose: To develop and evaluate the psychometric properties of a comprehensive, disease-specific questionnaire for characterizing complaints of LBP and evaluating the outcomes of treatments for these complaints.

Microsatellite analysis of recurrent vestibular schwannoma (acoustic neuroma) following stereotactic radiosurgery.

Hypothesis: Genetic and immunohistochemical studies may provide insight into the mechanisms of vestibular schwannoma (VS) recurrence following radiation therapy.

Background: Stereotactic radiation therapy is an increasingly common alternative to microsurgical resection for the primary management of sporadic VS. The molecular mechanisms associated with recurrent vestibular schwannoma (VS) following radiation therapy are not known.

The efficacy of postoperative ondansetron (Zofran) orally disintegrating tablets for preventing nausea and vomiting after acoustic neuroma surgery.

Postoperative nausea and vomiting is a frequent complication of craniotomy. We evaluated the ability of intraoperative IV ondansetron followed by postoperative ondansetron in an orally disintegrating tablet formulation to reduce the frequency and severity of postoperative nausea and vomiting in a prospective, randomized, placebo-controlled double-blind trial of 60 patients undergoing acoustic neuroma resection. Each patient received intraoperative ondansetron (4 mg IV) or placebo 30 min before case end.

Acoustic neuromas after failed radiation therapy: challenges of surgical salvage.

Objectives: As stereotactic radiation has emerged as a treatment option for acoustic neuromas, cases that require surgical salvage after unsuccessful radiation have emerged. We present a comparison of the technical challenges faced by the surgeons in the treatment of irradiated versus nonirradiated acoustic neuromas.

Study Design: Matched case-control series.

Clinical and histopathologic features of recurrent vestibular schwannoma (acoustic neuroma) after stereotactic radiosurgery.

Objective: Stereotactic radiosurgery for vestibular schwannoma entails uncertain long-term risk of tumor recurrence and delayed cranial neuropathies. In addition, the underlying histopathologic changes to the tumor bed are not fully characterized. We seek to understand the clinical and histologic features of recurrent vestibular schwannoma after stereotactic radiation therapy.

Outcome and cost of craniotomy performed to treat tumors in regional academic referral centers.

Objective: Improved clinical and economic outcomes for high-risk surgical procedures have been previously cited in support of regionalization. The goal of this study was to examine the effects of regionalization by analyzing the cost and outcome of craniotomy for tumors and to compare the findings in academic medical centers versus community-based hospitals.

Methods: Outcomes and charges were analyzed for all adult patients undergoing craniotomy for tumor in 33 nonfederal acute care hospitals in Maryland using the Maryland Health Service Cost Review Commission database for the years 1990 to 1996.