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Sharad Rajpal, M. Shahriar Salamat, R. Shane Tubbs, David R. Kelly, W. Jerry Oakes and Bermans J. Iskandar

Object

The goal of the present study goal was to systematically confirm the previously recognized nomenclature for tethering tracts that are part of the spectrum of occult spinal dysraphic lesions.

Methods

The tethering tract in 20 patients with spina bifida occulta underwent histological examination with H & E staining and epithelial membrane antigen (EMA) immunolabeling, and additional selected specimens were stained with Masson trichrome.

Results

All tethering tracts contained fibrous connective tissue. Four tracts were lined with epithelial cells and either originated within a dermoid cyst, terminated at a skin dimple/sinus opening, or had both of these characteristics. No tethering tracts exhibited EMA positivity or meningeal elements. Although all tethering tracts originated in juxtaposition to the spinal cord, their termination sites were variable.

Conclusions

Based on histological findings and presumed embryological origin, the authors broadly classified tethering tracts terminating within the dura mater, epidural space, or lamina as “short tethering tracts” (STTs). The STTs occurred mostly in conjunction with split cord malformations and had a purely fibrous composition. Tethering tracts terminating superficial to the overlying lamina were classified as “long tethering tracts” (LTTs), and the authors propose that these are embryologically distinct from STTs. The LTTs were of two varieties: epithelial and nonepithelial, the former being typically associated with a skin dimple or spinal cord (epi)dermoid cyst. In fact, analysis of the data suggested that not every tethering tract terminating in or on the skin should be classified as a dermal sinus tract without histological confirmation, and because no evidence of meningeal tissue–lined tracts was detected, the use of the term “meningocele manqué” may not be appropriate.

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R. Shane Tubbs, Cuong J. Bui, Marios Loukas, Mohammadali M. Shoja and W. Jerry Oakes

Object

The authors report on symptomatic patients with myelomeningocele (MMC) and lipomyelomeningocele (LMMC) who were found to have changes in their lumbosacral angle (LSA) corresponding to the onset of symptoms indicative of a tethered spinal cord.

Methods

The authors review data obtained in these two cohorts of patients and compare the LSAs measured in the perinatal period with those seen when the patients presented with symptoms of a tethered spinal cord.

Results

Children with LMMC, roughly one third of studied cases, were symptomatic due to a tethered spinal cord at their most recent follow-up. In children in whom the MMC was the closed form at birth, 20 of 30 had symptoms that could be indicative of a tethered spinal cord at their most recent follow-up. The LSA was altered in both groups with symptoms.

Conclusions

Signs and symptoms indicative of a tethered spinal cord appear to correspond to increases in the LSA.

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Jeffrey P. Blount, R. Shane Tubbs, John C. Wellons III, Leslie Acakpo-Satchivi, David Bauer and W. Jerry Oakes

✓ In certain highly selected circumstances, division of a distally nonfunctional or dysfunctional cord can be a means of definitive untethering that spares and protects more rostral neurological function and results in definitive untethering. The authors reviewed their institutional experience with such cases and evaluated the limited literature. Based on their experience, treatment can be effective in carefully selected patients who undergo spinal cord transection, and the rate of repetitive tethered spinal cord can be decreased. Although uncommon, spinal cord transection appears to be an effective therapy in carefully selected patents with symptoms of repetitive cord tethering.

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Cuong J. Bui, R. Shane Tubbs and W. Jerry Oakes

✓The treatment of a patient with symptoms of a tethered spinal cord and in whom a fatty infiltrated terminal filum is found is controversial. The authors review their experience and the literature regarding this aspect of occult spinal dysraphism. From experience, transection of a fatty terminal filum in patients with symptoms related to excessive caudal cord tension is a minor procedure that generally yields good results. The most problematic issue in the literature is what patients and symptoms are best suited to surgical treatment.

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R. Shane Tubbs, Marios Loukas, Robert G. Louis Jr., Mohammadali M. Shoja, Leslie Acakpo-Satchivi, Jeffrey P. Blount, E. George Salter, W. Jerry Oakes and John C. Wellons III

Object

The superior and inferior sagittal sinuses have been well studied. Interestingly, other venous structures within the falx cerebri have received scant attention in the medical literature. The present study was performed to elucidate the presence and anatomy of these midline structures.

Methods

The authors examined 27 adult latex- or ink-injected cadaveric specimens to observe the morphological features of the sinuses within the falx cerebri (excluding the inferior and superior sagittal sinuses).

Results

All specimens were found to have an extensive network of small tributaries within the falx cerebri that were primarily concentrated in its posterior one third. In this posterior segment, these structures were usually more pronounced in the inferior two thirds. The portion of the falx cerebri not containing significant falcine venous sinus was termed a “safe area.” These vascular channels ranged in size from 0.5 mm to 1.1 cm (mean 0.6 mm); 100% of these vessels communicated with the inferior sagittal sinus. Classification of the structures was then performed based on communication of the falcine venous sinus with the superior sagittal sinus. Type I falcine sinuses had no communication with the superior sagittal sinus, Type II falcine sinuses had limited communication with the superior sagittal sinus, and Type III falcine sinuses had significant communication with the superior sagittal sinus. Seventeen (63%) of 27 specimens communicated with the superior sagittal sinus (Types II and III). Further subdivision revealed 10 Type I, seven Type II, and 10 Type III falcine venous plexuses.

Conclusions

There are other venous sinuses in the falx cerebri in addition to the superior and inferior sagittal sinuses. Neurosurgical procedures that necessitate incising or puncturing the falx cerebri can be done more safely via a described safe area. Given that the majority of specimens in the authors' study were found to have a plexiform venous morphology within the falx cerebri, they propose that these channels be referred to as the falcine venous plexus and not sinus. The falcine venous plexus should be taken into consideration by the neurosurgeon.

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Cuong J. Bui, R. Shane Tubbs, Chevis N. Shannon, Leslie Acakpo-Satchivi, John C. Wellons III, Jeffrey P. Blount and W. Jerry Oakes

Object

There is scant literature regarding the long-term outcome in patients with cranial vault encephaloceles, and what literature there is may underestimate long-term deficits. The goal of this study was to address this lack of information.

Methods

The authors performed a retrospective chart review of cranial vault encephaloceles performed at our institution between 1989 and 2003. Fifty-two total patients were identified and 44 of these cases were reviewed. Additionally, 34 of the 44 patients were contacted and given an outcome survey (Hydrocephalus Outcome Questionnarie [HOQ]) to evaluate physical, emotional, cognitive, and overall health outcomes.

Results

The mean age for patients in this cohort was 9.6 years (range 4–17 years) and the mean follow-up time was 9.2 years. There was an equal sex distribution and there were no deaths. Hydrocephalus was found in 60% of occipital and 14% of frontal encephaloceles, and epilepsy was confirmed in 17% of occipital and 7% of frontal lesions. Outcome assessments performed using the HOQ showed that 50% of the patients with occipital encephaloceles had overall HOQ health scores of 0.5 or less and 55% had HOQ cognitive scores of 0.3 or less, compared with 0% of patients in both categories who had frontal encephaloceles. It was also found that the presence of hydrocephalus and epilepsy independently and significantly lowered the overall health scores.

Conclusions

Occipital encephaloceles carry a worse prognosis than frontal encephaloceles, with higher rates of hydrocephalus and seizure. Based on this study, the presence of hydrocephalus and epilepsy are significant additive adverse prognostic factors. Approximately half of the patients with occipital encephaloceles will be severely debilitated and will probably be unable to live and function independently in society. These data may be useful to clinicians in counseling patients and predicting long-term outcome following repair of cranial vault encephaloceles.

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John C. Wellons III, R. Shane Tubbs, Cuong J. Bui, Paul A. Grabb and W. Jerry Oakes

✓Patients with Chiari malformation Type I (CM-I) most commonly present with chronic symptoms. A search of the current medical literature revealed scant information regarding acute presentations of CM-I in either pediatric or adult patients. The authors report on two children who presented with rapidly worsening neurological symptoms attributable to a previously undiagnosed CM-I. One patient became profoundly hypopneic with dysphagia and right hemiparesis over a less than 48-hour period. The second patient presented with a few days of worsening right hemiparesis, gait disturbance, and anisocoria. In addition to a CM-I, magnetic resonance imaging in the second patient revealed a holocord syrinx. Following urgent posterior fossa decompression, both patients rapidly improved in the 24 hours immediately following surgery and continued to improve in the subsequent weeks. Few reports detail acute symptoms due to CM-I and those that do exist almost exclusively involve adult patients. Although seemingly rare, the clinician should consider CM-I in the differential diagnosis in pediatric patients presenting with acute brainstem or long tract signs.

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R. Shane Tubbs, Charles Law, Drew Davis, Mohammadali M. Shoja, Leslie Acakpo-Satchivi, John C. Wellons III, Jeffrey P. Blount and W. Jerry Oakes

Object

Postoperative epidural morphine is commonly used to control pain in children following dorsal rhizotomy for spasticity. The authors report their experience in using a regimen of scheduled minor analgesic drugs to manage postoperative pain, with the goal of avoiding opiate use following a spinal intradural procedure.

Methods

Postoperative pain scores were analyzed in a group of 22 children who underwent a partial dorsal rhizotomy. According to a preestablished standard regimen for postoperative pain control after dorsal rhizotomy, in each patient an intraoperative epidural catheter was placed for the potential infusion of postoperative morphine. Additionally, this cohort underwent a scheduled regimen of acetaminophen (10 mg/kg) and ibuprofen (10 mg/kg), alternating every 2 hours. For comparison, a retrospective chart review was performed in 20 patients with rhizotomies completed prior to the use of this oral analgesic protocol.

Only one patient received a postoperative dose of morphine epidurally. None of the remaining patients required postoperative epidural morphine for pain control. Pain scores were significantly lower in this group compared with a retrospective review of patients treated according to the standard regimen. Length of hospital stay was shorter in these patients and antiemetic requirements were lower.

Conclusions

A regimen of minor analgesic therapy, when given in alternating doses every 2 hours immediately after partial dorsal rhizotomy for spasticity and throughout hospitalization, significantly reduced postoperative pain scores, hospitalization, and antiemetic requirements in these patients.

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R. Shane Tubbs, Cuong J. Bui, Marios Loukas, Mohammadali M. Shoja and W. Jerry Oakes

✓ Cerebral palsy is a common affliction in childhood. In some cases, the spasticity that often occurs can be treated with dorsal rhizotomies. Classically, these procedures have not been performed in children in whom there are known specific congenital brain malformations.

The authors report on two patients with holoprosencephaly and unilateral schizencephaly who underwent dorsal rhizotomy to treat their spasticity. The results were good. The long-term benefits during a mean follow-up period of 3.5 years included the transition from using a walker to quad canes for ambulation. Additionally, the outcomes in these two children appeared comparable to those found in other children with spastic diplegia undergoing dorsal rhizotomy at the authors' institution.

Dorsal rhizotomy may prove useful for treating spasticity in children with known congenital brain deformities.

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R. Shane Tubbs, Marios Loukas, Robert G. Louis Jr., Mohammadali M. Shoja, Cameron S. Askew, April Phantana-Angkool, E. George Salter and W. Jerry Oakes

Object

The basal vein of Rosenthal (BV) courses from the premesencephalic cistern, through the ambient cistern, and terminates in the quadrigeminal cistern. The aim of this study was to describe and quantitate the surgical anatomy of this structure and specifically to provide landmarks for identifying this vessel along its course. These data may be of use, for example, to surgeons using subtemporal operative approaches through regions where this vessel is concealed.

Methods

The authors examined 15 latex-injected adult cadaveric brains (30 sides) to delineate the morphological characteristics of the BV. Dissections of the BV were then performed and measurements were made between this structure and the tentorial incisura at the anterior, middle, and posterior borders of the lateral midbrain.

All specimens were found to have a left and right BV with varying morphological characteristics. The mean distance between the BV and posterior cerebral artery at the midpoint of the lateral midbrain was 16 mm. The BV was always found superomedial to the posterior cerebral artery along the lateral aspect of the midbrain, and the BV ranged in diameter from 1 to 5 mm. The BV drained into the vein of Galen in all but two specimens. The mean distances from the tentorial edge to the BV at the anterior, middle, and posterior borders of the lateral midbrain were 11, 13, and 4 mm, respectively. No statistically significant differences were found when comparing left and right sides or male and female specimens.

Conclusions

The authors hope that these data will help the neurosurgeon operating near the BV to avoid injury to this important structure.