Congenital tethered spinal cord syndrome in adults.

OBJECT: The management of tethered cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. METHODS: The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Surgery-related complications included one cerebrospinal fluid leak and five pseudomeningoceles. Two patients complained of worsening motor or sensory dysfunction and one of worsening bladder dysfunction, whereas four patients experienced persistent pain and one complained of worsening pain postoperatively. According to the patients, the vast majority of these complications were minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. CONCLUSIONS: Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.

Neonatal ventriculosubgaleal shunts.

OBJECTIVE: We report on 32 neonates treated with ventriculosubgaleal (VSG) shunts to determine VSG shunt survival and associated complications. METHODS: Between 1993 and 1997, 37 VSG shunts were placed in 32 neonates when the cerebrospinal fluid (CSF) or the abdomen was considered unsuitable for ventriculoperitoneal shunt placement. In each child, a ventricular catheter was attached to 3 cm of a closed-end peritoneal tube via a right-angle connector, which drained into a surgically created subgaleal pocket. RESULTS: The causes of hydrocephalus were as follows: intraventricular hemorrhage (IVH) in 20 neonates, meningitis/ventriculitis in 6, IVH and infection in 2, and other causes in 4. The mean postconception age at the time of VSG shunt insertion was 37.2 weeks (33.1 wk in the IVH group), and the mean weight was 2227 g (1724 g in the IVH group). The average preoperative head circumference was 33.6 cm. The average survival of these 37 VSG shunts (five children had two VSG shunts) was 35.1 days. The complications were as follows: one CSF leakage occurred when sutures were removed; one catheter fell into the ventricle and required removal, and one child died immediately after VSG shunt revision. There were no VSG shunt infections. All surviving children followed for a minimum of 4 months after insertion of a VSG shunt (n = 24) have required a ventriculoperitoneal shunt. Mean follow-up from the time of first VSG shunt insertion was 21.6 months. Four children died as a result of causes unrelated to the VSG shunt. CONCLUSION: VSG shunts offer a simple, effective, and relatively safe means of temporizing hydrocephalus, and they avoid the need for external drainage or frequent CSF aspiration in these medically unstable infants until the CSF characteristics and abdomen are acceptable for ventriculoperitoneal shunting.

Two cases of cerebral aneurysms in HIV+ children.

Two cases of fusiform cerebral aneurysms in human immunodeficiency virus (HIV) positive children are presented. To our knowledge, only 9 patients with this association have been reported. One of our patients represents the first report of a patient with an aneurysm associated with varicella-zoster vasculitis. One patient presented with a subarachnoid hemorrhage, Hunt-Hess grade IV, and posed difficult surgical management. The other patient suffered a cerebral infarct with a resulting hemiparesis. The first patient had a ventriculostomy placed, initially improved, and subsequently died from rebleeding. The second patient improved with medical management. AIDS arteriopathy, and specifically fusiform aneurysms, are being increasingly reported. The various presentations of this surgically challenging entity in light of other AIDS-related syndromes pose difficult management decisions. On occasion, the intracranial aneurysm may be the initial form of presentation as was present in our first patient.

Congenital tethered spinal cord syndrome in adults.

OBJECT: The management of tethered spinal cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. METHODS: The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. CONCLUSIONS: Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.

Midline dorsal appendages.

A case of a mature posterior cervical teratoma resembling two fingers and a case of a lumbosacral limb are presented. These are at least the seventh and eighth reported paraspinous or occipital teratomas with recognizable extremity development. Both anomalies had intradural extension and required exploration to decrease the likelihood of future spinal cord tethering. Preoperative evaluation, surgical technique, postoperative follow-up, and review of the literature are presented.

Radiation-induced glioma of the spinal cord.

We report a case of a radiation-induced spinal cord glioma. A 20-year-old girl presented with neck pain and new significant neurological deficits 17 years after resection of a posterior fossa medullomyoblastoma and subsequent craniospinal irradiation. She was found to have a cervical intramedullary tumor that was resected using a standard microsurgical technique. The permanent histopathological diagnosis was anaplastic astrocytoma. Her neurological status was worse immediately following the operation. She improved to her pre-operative status, but then had a relentless neurological decline resulting in death 16 weeks following surgery. Because of the high incidence of malignancy in the few radiation-induced spinal cord gliomas reported in the literature, and the poor outcome independent of therapy in patients with malignant spinal cord gliomas, an initial strategy of biopsy only may be more appropriate than attempted resection for the patient with a suspected radiation-induced intramedullary spinal cord tumor.