Health-related quality of life following spinal cordectomy for syringomyelia.

BACKGROUND: Spinal cordectomy has been described as an effective treatment option in paraplegic patients for the treatment of syringomyelia to manage spasticity, pain and ascending neurological dysfunction. The objective of this study was to investigate the long-term health-related quality of life (HRQoL) after cordectomy in patients with intractable symptoms caused by syringomyelia. METHODS: Seventeen patients underwent spinal cordectomy for syringomyelia between February 2000 and July 2009. The etiology of syringomyelia was traumatic in 16 patients and spinal ependymoma in one patient. The mean follow-up was 3.8 years (range, 0.9-10.3). The HRQoL was assessed pre- and postoperatively using the EuroQol (EQ; degree of discomfort: 1 = none, 2 = moderate and 3 = extreme) and the short-form SF-36 quality of life score (SF-36). All patients underwent a telephone interview. RESULTS: The mean pre- and postoperative EuroQol-levels for mobility were 1.8 and 1.5; for self-care, 1.9 and 1.5; for usual activities, 2.1 and 1.5; for pain/discomfort, 2.3 and 2.0; and for anxiety/depression, 1.7 and 1.5, respectively. The mean overall EQ visual analogue scale improved postoperatively from 42 points (range, 15-80) to 67 points (range, 10-95) (p = 0.006). The component summary measure for mental health (SF-36) significantly improved postoperatively (p = 0.01). A telephone interview revealed a high subjective patient satisfactory (94.1%) in terms of postoperative sequelae. Following the intervention, 58.8% of all patients were employed full or part-time. CONCLUSIONS: Spinal cordectomy may increase the quality of life and can be considered as an ultimo ratio therapy in a selective group of patients with intractable symptoms caused by syringomyelia.

Impact of cordectomy as a treatment option for posttraumatic and non-posttraumatic syringomyelia with tethered cord syndrome and myelopathy.

OBJECT: Spinal cordectomy has recently become more important in the treatment of end-stage posttraumatic or postoperative syringomyelia and arachnopathy as a last resort to manage ascending neurological dysfunction, spasticity, and pain in paraplegic patients. The aim in this study was to confirm a clinical benefit in strict indications for cordectomy. METHODS: Between February 2000 and September 2007, 15 spinal cordectomies were performed at the Department of Neurosurgery, Cantonal Hospital, St. Gallen. Indications for treatment were end-stage myelopathies caused by syringomyelia, tethered cord syndrome, and arachnopathy with progressive spasticity and pain or progressive upper-level neurological deficits related to the tethered cord syndrome. All patients had severe motor and sensory deficits with no residual voluntary function below the affected level. RESULTS: Fourteen of 15 patients showed stabilization or even an improvement in motor and sensory function. Four patients suffered from progressive spasticity and 3 from deterioration due to pain. There were no other adverse surgical events. CONCLUSIONS: Cordectomy can be a useful instrument to preserve functions of the upper extremities and to improve spasticity and pain in patients with severe myelopathy and tethered cord, syringomyelia, or arachnopathy of various etiologies.

Midbrain syndrome with eye movement disorder: dramatic improvement after cranioplasty.

BACKGROUND: Patients with skull defects sometimes develop neurological deficits, which have been grouped under "the syndrome of the trephined". The deficits are usually nonspecific or nonlocalizing, such as apathy or diffuse headaches. We report, to our knowledge, a first case of severe midbrain syndrome associated with a skull defect. Cranioplasty dramatically resolved the patient's symptoms. A midbrain syndrome represents the main manifestation of the syndrome of the trephined and can be corrected by cranioplasty. CLINICAL PRESENTATION: A 38-year-old man with head trauma and epidural hematoma initially had normal eye motility. He developed a skull defect after infection following cranioplasty. He presented with onset of neurological symptoms one year after head trauma with a skull defect, a small divergent and vertical strabismus, elevation deficit of both eyes, headaches and fatigue. Over several months he developed severe bilateral deficit of adduction, elevation, depression and convergence. He had neuropsychological deficits, fatigue, headaches and impaired coordination. Neuroimaging and lumbar puncture did not show evidence of increased intracranial pressure or hydrocephalus. INTERVENTION: Cranioplasty using Palacos was performed one-and-a-half years after trauma. Immediately after surgery, the patient noted remarkable improvement in his symptoms. Headaches and fatigue disappeared within two days. Two weeks after cranioplasty the patient had orthotropia and virtually normal ocular motility. Neurological symptoms completely disappeared. Recovery remained sustained for over 5 years after cranioplasty. CONCLUSION: To our knowledge, this represents the first case of the syndrome of the trephined in which the neurological deficits map primarily to the brainstem and show rapid improvement following cranioplasty. We show that cranioplasty in patients with large skull defects is indicated for more then cosmetic reasons and should be considered even after longer periods following a trauma.