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  • By Author: Bui, Cuong J. x
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Leslie Acakpo-Satchivi, R. Shane Tubbs, Audie L. Woolley, Cuong J. Bui, Peter Liechty, Yuki Hammers, John Wellons III, Jeffrey P. Blount and W. Jerry Oakes

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Cuong J. Bui, R. Shane Tubbs, Gigi Pate, Traci Morgan, Douglas C. Barnhart, Leslie Acakpo-Satchivi, John C. Wellons III, W. Jerry Oakes and Jeffrey P. Blount

Object

There is conflicting information in the literature regarding the increased risk of ventriculoperitoneal (VP) shunt infection after abdominal surgical procedures such as gastrostomy tube (GT) placement and Nissen fundoplication (NF) in the young patient. To further elucidate this potential association, the authors reviewed their institutional experience with such cases.

Methods

The authors retrospectively reviewed the records of all patients with shunted hydrocephalus who also underwent NF procedures and/or GT placements over a 9-year period.

Results

During this 9-year period, 3065 cerebrospinal fluid (CSF) shunts were placed and 1630 NF procedures/GT placements were performed. Ninety-six patients were identified who received both a CSF shunt and NF procedures/GT placements. Seventy-nine patients had a functioning VP shunt (without recent [< 6 months] CSF infection or shunt revision) at the time of their abdominal procedure (NF procedure or GT placement). Of this latter cohort, there were 38 males and 41 females with a mean age of 6.4 months; 12.7% of these patients developed a shunt infection and 60% of these infections were due to Staphylococcus species.

Conclusions

Based on this study, an NF procedure/GT placement in a child with a VP shunt appears to carry approximately the same risk of shunt infection as a shunt placement operation. Moreover, the microbiology observed in these patients with infection did not differ significantly from the usual microorganisms responsible for VP shunt infections in infants/neonates. Finally, but not conclusively, an open NF procedure/GT placement may carry a higher infection risk than a laparoscopic-assisted NF procedure/GT placement.

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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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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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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, 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, Cuong J. Bui, William C. Rice, Marios Loukas, Robert P. Naftel, Michael Paul Holcombe and W. Jerry Oakes

Object

Occasional comments are found in the literature regarding patients with lipomyelomeningocele and concomitant Chiari malformation Type I (CM-I). The object of this study was to explore the association between these two conditions.

Methods

The authors performed a retrospective database analysis of lipomyelomeningocele cases to identify cases of concomitant CM-I. Analysis of posterior fossa volume (based on the Cavalieri principle) was performed in all identified cases in which appropriate neuroimages were available, and the results were compared with those obtained in age-matched controls.

Seven (13%) of 54 patients with lipomyelomeningocele were found to also have CM-I. Two of these were symptomatic (cervicothoracic syrinx and occipital headaches) and required posterior fossa decompression. No correlation was found between the amount of hindbrain herniation and the level of the conus medullaris or the type of lipomyelomeningocele (for example, caudal or transitional). Volumetric studies of the posterior fossa revealed normal age-matched volumes in all but one patient (who had asymptomatic CM-I).

Conclusions

The incidence of CM-I in patients with lipomyelomeningocele appears to be significantly greater than that of the general population and the association rate is too high for the finding to be a chance occurrence. Decreases in the volume of the posterior cranial fossa were not found in the majority of patients in this small cohort; therefore, the cause of the concomitant occurrence of lipomyelomeningocele and CM-I remains undetermined. Clinicians should consider obtaining imaging studies of the entire neuraxis in patients with lipomyelomeningoceles and should investigate other causes for syringes found in association with lipomyelomeningoceles.