✓ There is a growing body of evidence in the literature suggesting that cavernous malformations of the central nervous system may develop after neuraxis irradiation. The authors discuss the case of a 17-year-old man who presented with progressive back pain and myelopathy 13 years after undergoing craniospinal irradiation for a posterior fossa medulloblastoma. Spinal magnetic resonance (MR) imaging, performed at the time of his initial presentation with a medulloblastoma, demonstrated no evidence of a malformation. Imaging studies and evaluation of cerebrospinal fluid revealed no evidence of recurrence or dissemination. Spinal MR imaging demonstrated an extensive lesion in the thoracic spine with an associated syrinx suggestive of a cavernous malformation. A thoracic laminectomy was performed and the malformation was successfully resected. Pathological examination confirmed the diagnosis. The patient did well after surgery and was ambulating without assistance 6 weeks later. To the best of the authors' knowledge, this is the second reported case in the literature and the first in the young adult age group suggesting the de novo development of cavernous malformations in the spinal cord after radiotherapy. An increased awareness of these lesions and close follow-up examination are recommended in this setting.
Case report and review of the literature
Prithvi Narayan and Daniel L. Barrow
Alexander F. Post, Prithvi Narayan and Regis W. Haid Jr.
✓ The authors report on the management of occipital neuralgia secondary to an abnormality of the atlas in which the posterior arch was separated by a fibrous band from the lateral masses, resulting in C-2 nerve root compression. The causes and treatments of occipital neuralgia as well as the development of the atlas are reviewed.
Prithvi Narayan, Michael J. Workman and Daniel L. Barrow
✓ Aneurysms arising from a lenticulostriate artery (LSA) are uncommon. Their causes include hypertension, moyamoya disease, infection, systemic lupus erythematosis, and flow-related saccular aneurysms. Options for treating these aneurysms are limited. The authors present a case in which an LSA aneurysm was identified in a 69-year-old woman with no significant medical history, who experienced a sudden onset of right hemiparesis and aphasia due to a basal ganglia hemorrhage. The different causes and treatment options available for these rare and difficult-to-treat aneurysms are discussed.
James T. Rutka
Prithvi Narayan, Regis W. Haid, Brian R. Subach, Christopher H. Comey and Gerald E. Rodts
Object. Pedicle screw fixation with transverse process fusion has gained widespread acceptance since its inception. Improved rates of arthrodesis have been demonstrated when this technique is used. The authors present one of the largest series of patients to undergo this procedure at a single center; one of the goals was to correlate construct length and spinal disease with rates of successful arthrodesis by conducting a prospective analysis of lumbar fusion in which pedicle screws were placed.
Methods. During a 7-year period, the senior author performed pedicle screw fixation with posterolateral fusion in 457 patients; the mean follow-up period was 28.4 months. Indications for fusion included metastatic tumor, single-level degenerative disc disease (DDD), trauma, degenerative scoliosis, and translational vertebral instability. Successful fusion was based on the radiographic demonstration of a bilateral contiguous osseous bridge over the transverse processes and absence of movement on dynamic x-ray films.
Fusion rates were lowest in cases of tumors (54%) and highest in cases of trauma (96%). In patients with single-level DDD the rate was 91%, and in those with translational instability it was 89%. Fusion rates, however, declined steeply in relation to each additional motion segment in the translational instability group. In this group a strong linear trend for proportion was demonstrated (p < 0.001). The overall fusion rate in patients with degenerative scoliosis was 70%. The overall fusion rate for the entire group was 86%.
Conclusions. The data in this study can be used as a benchmark with which to compare newer technologies. Although overall pedicle screw—assisted fusion rate in cases of trauma or selected degenerative lesions approached 90%, the arthrodesis rates are not uniform for the different diagnoses. This appears to be related to the underlying spinal disease and the number of segments included in the fusion.
D. Ryan Ormond, Ibrahim Omeis, Avinash Mohan, Raj Murali and Prithvi Narayan
✓ Cysts occupying the third ventricle are rare lesions and may appear as an unusual cause of obstructive hydrocephalus. Various types of lesions occur in this location, and they generally have an arachnoidal, endodermal, or neuroepithelial origin. The authors present a case of acute hydrocephalus following minor trauma in a child due to cerebrospinal fluid outflow obstruction by a third ventricular cyst. Definitive diagnosis of this cystic lesion was possible only with contrast ventriculography and not routine computed tomography or magnetic resonance imaging. The investigation, treatment, and pathological findings are discussed.
Francesco T. Mangano, Jose A. Menendez, Matthew D. Smyth, Jeffrey R. Leonard, Prithvi Narayan and Tae Sung Park
All-terrain vehicles (ATVs) have been characterized as inherently unstable and are associated with significant pediatric injuries in the US. The authors performed a study to analyze data obtained in pediatric patients who had sustained neurological injuries in ATV-related accidents, identify potential risk factors, and propose preventive measures. The study is based on a 10-year experience at the St. Louis Children’s Hospital.
The authors retrospectively analyzed data obtained in all patients admitted to the St. Louis Children’s Hospital between 1993 and 2003, limiting their focus to pediatric cases involving ATV-related accidents. A total of 185 patients were admitted with these criteria. Sixty-two patients (33.5%) suffered neurological injuries; there were 42 male and 20 female patients whose age ranged from 2 to 17 years. The most common injuries included skull fracture (37 cases) and closed head injury (30 cases). There were 39 cases of intracranial hemorrhage and 11 of spinal fracture. A total of 15 types of neurosurgical procedure were performed: six craniotomies for hematoma drainage, five craniotomies for elevation of depressed fractures, two procedures to allow placement of an intracranial pressure monitor, one to allow placement of an external ventricular drain, and one to allow the insertion of a ventriculoperitoneal shunt. Two patients had sustained spinal cord injury, and three procedures were performed for spinal decompression or stabilization. The duration of hospital stay ranged from 1 to 143 days (mean 6.6 days). Fifty-seven patients (30.8%) were eventually discharged from the hospital, three (1.6%) were transferred to another hospital, two (1.1%) died, and 123 (66.4%) required in-patient rehabilitation.
Children suffered significant injuries due to ATV accidents. In passengers there was a statistically significant increased risk of neurological injury. The relative risk of neurological injury in patients not wearing helmets was higher than that in those who wore helmets, but the difference did not reach statistical significance. Further efforts must be made to improve the proper operation and safety of ATVs, both through the education of parents and children and through the creation of legislation requiring stricter laws concerning ATV use.
Case report and review of the literature
William W. Ashley Jr., Prithvi Narayan, Tae Sung Park, Pang-hsien Tu, Arie Perry and Jeffrey R. Leonard
✓Juvenile xanthogranuloma (JXG) is a specialized form of non—Langerhans cell histiocyte proliferation that occurs in children. The majority of cases present as a solitary cutaneous lesion with a predilection for the head and neck region; however, isolated lesions occasionally have been identified in the central nervous system. The cutaneous forms of JXG usually follow a benign course. Other physicians have reported surgery as the first line of treatment in symptomatic patients with accessible lesions. Adjuvant therapies may be indicated for multicentric or surgically inaccessible lesions. The authors describe an unusual case of isolated intraparenchymal JXG in an asymptomatic child with no cutaneous manifestations and provide a review of the literature.
Francesco T. Mangano, Jose A. Menendez, Tracy Habrock, Prithvi Narayan, Jeffrey R. Leonard, Tae Sung Park and Matthew D. Smyth
The use of adjustable differential pressure valves has been recommended to improve ventriculoperitoneal (VP) shunt performance in selected patients; however, published data are scarce regarding their clinical reliability. Recently, the identification of a number of malfunctioning programmable valves during shunt revision surgery in children prompted a retrospective review of valve performance in this patient cohort.
The authors performed a retrospective chart analysis of 100 patients with programmable valve shunts and 89 patients with nonprogrammable valve shunts implanted at the St. Louis Children's Hospital between April 2002 and June 2004. They noted the cause of hydrocephalus, the type of shunt malfunction, and cerebrospinal fluid (CSF) protein levels. Regular clinical follow up ranged from 1 to 26 months, with a mean follow-up time of 9.75 months for patients with programmable valves and 10.4 months for patients with nonprogrammable valves.
Patient ages ranged from 2 weeks to 18 years. One hundred patients had 117 programmable valves implanted, and 35 of these patients (35%) underwent shunt revision because of malfunction. The programmable valve itself malfunctioned in nine patients who had undergone shunt revision (11.1%/year of follow up). The nonprogrammable valve group had no valve malfunctions. The overall VP shunt revision rate in the nonprogrammable valve group was 20.2%. No significant differences were identified when CSF protein levels and specific malfunction types were compared within the programmable valve and nonprogrammable valve groups.
In this study the authors demonstrated an annualized intrinsic programmable valve malfunction rate of 11.1%, whereas during the same period no intrinsic valve malfunctions were noted with nonprogrammable valve systems for similar causes of hydrocephalus. The CSF protein levels did not correlate with observed valve malfunction rates. Further evaluation in a prospective, randomized fashion will elucidate specific indications for programmable valve systems and better determine the reliability of these valves in the pediatric population.
David D. Limbrick Jr., Prithvi Narayan, Alexander K. Powers, Jeffrey G. Ojemann, Tae Sung Park, Mary Bertrand and Matthew D. Smyth
Hemispherotomy generally is performed in hemiparetic patients with severe, intractable epilepsy arising from one cerebral hemisphere. In this study, the authors evaluate the efficacy of hemispherotomy and present an analysis of the factors influencing seizure recurrence following the operation.
The authors performed a retrospective review of 49 patients (ages 0.2–20.5 years) who underwent functional hemispherotomy at their institution. The first 14 cases were traditional functional hemispherotomies, and included temporal lobectomy, while the latter 35 were performed using a modified periinsular technique that the authors adopted in 2003.
Thirty-eight of the 49 patients (77.6%) were seizure free at the termination of the study (mean follow-up 28.6 months). Of the 11 patients who were not seizure free, all had significant improvement in seizure frequency, with 6 patients (12.2%) achieving Engel Class II outcome and 5 patients (10.2%) achieving Engel Class III. There were no cases of Engel Class IV outcome. The effect of hemispherotomy was durable over time with no significant change in Engel class over the postoperative follow-up period. There was no statistical difference in outcome between surgery types. Analysis of factors contributing to seizure recurrence after hemispherotomy revealed no statistically significant predictors of treatment failure, although bilateral electrographic abnormalities on the preoperative electroencephalogram demonstrated a trend toward a worse outcome.
In the present study, hemispherotomy resulted in freedom from seizures in nearly 78% of patients; worthwhile improvement was demonstrated in all patients. The seizure reduction observed after hemispherotomy was durable over time, with only rare late failure. Bilateral electrographic abnormalities may be predictive of posthemispherotomy recurrent seizures.