The authors believe that the preferred treatment for pineal region tumors in children requires definitive surgery with a histological diagnosis and that a conservative approach consisting of shunting and radiation therapy no longer seems to be appropriate. The results are reported of a retrospective review of the presentation, treatment, and outcome of 36 children under the age of 18 years treated between 1974 and 1986. Eleven children had germinomas (two-cell type), seven had astrocytomas, and the remaining 18 had 15 histologically different tumor types. Surgery was performed on 30 patients; there were no deaths, but a 10% rate of persistent morbidity was found. The median follow-up period was 4 years. Nine (82%) of 11 patients with germinomas are alive without evidence of recurrence; one child died from recurrent tumor in the pineal region and another is presently being treated for recurrent tumor of the spinal cord. Six (86%) of the seven patients with astrocytoma are well after biopsy and radiation therapy. Of the remaining 18 children, five (28%) died from tumor progression. The cerebrospinal fluid (CSF) tumor markers alpha-fetoprotein and beta-human chorionic gonadotropin were helpful in determining the presence of malignant germ-cell tumors, particularly those with a poor prognosis. Magnetic resonance imaging was useful for diagnosis and for planning the operative approach. Magnetic resonance images showed the presence of pineal region tumors in four children with hydrocephalus who had no evidence of tumor on computerized tomography scans. Because the great variety of tumor types found in the pineal region must be treated in different ways and because improved microsurgical and stereotaxic surgical techniques have made mortality and morbidity rates acceptably low, a biopsy diagnosis should be obtained in all patients. Preoperative assessment of CSF tumor markers and cytology is useful for the identification of patients who have a poor prognosis.

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Object The goal of this paper is to elucidate the clinical presentation of tethered cord syndrome (TCS) following repair of a myelomeningocele. Methods Approximately 10 to 30% of children will develop TCS following repair of a myelomeningocele. Because essentially all children with repaired myelomeningocele will have a tethered spinal cord, as demonstrated on MR imaging, the diagnosis of TCS is made based on clinical criteria. The six common clinical presentations of TCS are increased weakness (55%), worsening gait (54%), scoliosis (51%), pain (32%), orthopedic deformity (11%), and urological dysfunction (6%). The primary goal of surgery is to detach the spinal cord where it is adherent to the thecal sac, relieving the stretch on the terminal portion of the cord. Conclusions Early diagnosis and surgical release of the tethered cord results in stabilization or improvement in most cases. Because TCS may present with orthopedic and/or urological signs or symptoms, children with myelomeningocele should by followed, ideally in a multidisciplinary clinic, by neurosurgeons, orthopedic surgeons, and urologists who are aware of this condition.

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