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Robert P. Naftel, R. Shane Tubbs, Joshua Y. Menendez, John C. Wellons III, Ian F. Pollack and W. Jerry Oakes

Object

The effects of posterior fossa decompression on Chiari malformation Type I–induced syringomyelia have been well described. However, treatment of worsening syringomyelia after Chiari decompression remains enigmatic. This paper defines patient and clinical characteristics as well as treatment and postoperative radiological and clinical outcomes in patients experiencing this complication.

Methods

The authors performed a retrospective review of patients at the Children's Hospital of Pittsburgh and Children's of Alabama who developed worsening syringomyelia after Chiari decompression was performed.

Results

Fourteen children (age range 8 months to 15 years), 7 of whom had preoperative syringomyelia, underwent posterior fossa decompression. Aseptic meningitis (n = 3) and bacterial meningitis (n = 2) complicated 5 cases (4 of these patients were originally treated at outside hospitals). Worsening syringomyelia presented a median of 1.4 years (range 0.2–10.3 years) after the primary decompression. Ten children presented with new, recurrent, or persistent symptoms, and 4 were asymptomatic. Secondary Chiari decompression was performed in 11 of the 14 children. The other 3 children were advised to undergo secondary decompression. A structural cause for each failed primary Chiari decompression (for example, extensive scarring, suture in the obex, arachnoid web, residual posterior arch of C-1, and no duraplasty) was identified at the secondary operation. After secondary decompression, 8 patients' symptoms completely resolved, 1 patient's condition stabilized, and 2 patients remained asymptomatic. Radiologically, 10 of the 11 children had a decrease in the size of their syringes, and 1 child experienced no change (but improved clinically). The median follow-up from initial Chiari decompression was 3.1 years (range 0.8–14.1 years) and from secondary decompression, 1.3 years (range 0.3–4.5 years). No patient underwent syringopleural shunting or other nonposterior fossa treatment for syringomyelia.

Conclusions

Based on the authors' experience, children with worsening syringomyelia after decompression for Chiari malformation Type I generally have a surgically remediable structural etiology, and secondary exploration and decompression should be considered.

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Andrew Romeo, Robert P. Naftel, Christoph J. Griessenauer, Gavin T. Reed, Richard Martin, Chevis N. Shannon, Paul A. Grabb, R. Shane Tubbs and John C. Wellons III

Object

Endoscopic third ventriculostomy (ETV) is an alternative to shunt placement in children with hydrocephalus due to tectal plate gliomas (TPGs). However, controversy remains regarding the amount of ventricular size reduction that should be expected after ETV. This study investigates ventricular size change after ETV for TPGs.

Methods

Twenty-two children were identified from a 15-year retrospective database of neuroendoscopic procedures performed at the authors' institution, Children's Hospital of Alabama, in patients with a minimum of 1 year of follow-up. Clinical outcomes, including the need for further CSF diversion and symptom resolution, were recorded. The frontal and occipital horn ratio (FOR) was measured on pre- and postoperative, 1-year, and last follow-up imaging studies.

Results

In 17 (77%) of 22 children no additional procedure for CSF diversion was required. Of those in whom CSF diversion failed, 4 underwent successful repeat ETV and 1 required shunt replacement. Therefore, in 21 (96%) of 22 patients, CSF diversion was accomplished with ETV. Preoperative and postoperative imaging was available for 18 (82%) of 22 patients. The FOR decreased in 89% of children who underwent ETV. The FOR progressively decreased 1.7%, 11.2%, and 12.7% on the initial postoperative, 1-year, and last follow-up images, respectively. The mean radiological follow-up duration for 18 patients was 5.4 years. When ETV failed, the FOR increased at the time of failure in all patients. Failure occurred 1.6 years after initial ETV on average. The mean clinical follow-up period for all 22 patients was 5.3 years. In all cases clinical improvement was demonstrated at the last follow-up.

Conclusions

Endoscopic third ventriculostomy successfully treated hydrocephalus in the extended follow-up period of patients with TPGs. The most significant reduction in ventricular size was observed at the the 1-year followup, with only modest reduction thereafter.

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Joshua J. Chern, Amber S. Gordon, Robert P. Naftel, R. Shane Tubbs, W. Jerry Oakes and John C. Wellons III

Intracranial endoscopy in the treatment of hydrocephalus, arachnoid cysts, or brain tumors has gained wide acceptance, but the use of endoscopy for intradural navigation in the pediatric spine has received much less attention. The aim of the authors' present study was to analyze their experience in using spinal endoscopy to treat various pathologies of the spinal canal.

The authors performed a retrospective review of intradural spinal endoscopic cases at their institution. They describe 4 representative cases, including an arachnoid cyst, intrinsic spinal cord tumor, holocord syrinx, and split cord malformation.

Intradural spinal endoscopy was useful in treating the aforementioned lesions. It resulted in a more limited laminectomy and myelotomy, and it assisted in identifying a residual spinal cord tumor. It was also useful in the fenestration of a multilevel arachnoid cyst and in confirming communication of fluid spaces in the setting of a complex holocord syrinx. Endoscopy aided in the visualization of the spinal cord to ensure the absence of tethering in the case of a long-length Type II split spinal cord malformation.

Conclusions

Based on their experience, the authors found intradural endoscopy to be a useful surgical adjunct and one that helped to decrease morbidity through reduced laminectomy and myelotomy. With advances in technology, the authors believe that intradural endoscopy will begin to be used by more neurosurgeons for treating diseases of this anatomical region.

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Robert P. Naftel, Joshua L. Argo, Chevis N. Shannon, Tracy H. Taylor, R. Shane Tubbs, Ronald H. Clements and Mark R. Harrigan

Object

Traditional ventriculoperitoneal (VP) shunt surgery involves insertion of the distal catheter by minilaparotomy. However, minilaparotomy may be a significant source of morbidity during shunt surgery. Laparoscopic insertion of the distal catheter is an alternative technique that may simplify and improve the safety of shunt surgery.

Methods

The authors performed a retrospective review of hospital records of all patients undergoing new VP shunt insertion at a tertiary care center between 2004 and 2009. Patient characteristics and outcomes were compared between patients undergoing open or laparoscopic insertion of the distal catheter. Independent variables in the analysis included age, sex, race, body mass index, surgical technique, previous VP shunt placement, previous abdominal procedures, American Society of Anesthesiology (ASA) score, and indication for shunt placement. Dependent variables included the occurrence of shunt failure, cause of shunt failure, complications, length of stay (LOS), LOS after shunt placement, estimated blood loss, and operative time.

Results

The authors identified 810 patients who met the inclusion criteria; open or laparoscopic distal catheter insertion was performed in 335 and 475 patients, respectively. There were no significant differences between the groups regarding age, race, ASA score, or indication for shunt placement. The most common indication was hydrocephalus due to subarachnoid hemorrhage, followed by tumor-associated hydrocephalus, normal pressure hydrocephalus (NPH), and hydrocephalus due to trauma. The incidence of shunt failure was not statistically different between cohorts, occurring in 20.0% of laparoscopic and 20.9% of open catheter placement cases (p = 0.791). With analysis of causes of shunt failure, shunt obstruction occurred significantly more often in the open surgery cohort (p = 0.012). In patients with a known cause shunt obstruction, distal obstruction occurred in 35.7% of the open cohort obstructions and 4.8% of the laparoscopic cohort obstructions (p = 0.014). The relative risk of distal obstruction in open cases compared with laparoscopic cases was 7.50. Infections occurred in 8.2% of laparoscopic cases compared with 6.6% of open cases (p = 0.419). Within the NPH subgroup, the laparoscopically treated patients had significantly more overdrainage (p = 0.040), whereas those in the open cohort experienced significantly more shunt obstructions (p = 0.034). Laparoscopically treated patients had shorter operative times (p < 0.0005), inpatient LOS (p < 0.001), and inpatient LOS after VP shunt placement (p = 0.01) as well as less blood loss (p = 0.058).

Conclusions

To our knowledge this is the largest reported comparison of distal VP shunt catheter insertion techniques. Compared with minilaparotomy, the laparoscopic approach was associated with decreased time in the operating room and a decreased LOS. Moreover, laparoscopy was associated with fewer distal shunt obstructions. Laparoscopic shunt surgery is a viable alternative to traditional shunt surgery.

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R. Shane Tubbs, Justin D. Hallock, Virginia Radcliff, Robert P. Naftel, Martin Mortazavi, Mohammadali M. Shoja, Marios Loukas and Aaron A. Cohen-Gadol

The specialized ligaments of the craniocervical junction must allow for stability yet functional movement. Because injury to these important structures usually results in death or morbidity, the neurosurgeon should possess a thorough understanding of the anatomy and function of these ligaments. To the authors' knowledge, a comprehensive review of these structures is not available in the medical literature. The aim of the current study was to distill the available literature on each of these structures into one offering.

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R. Shane Tubbs, Joshua Beckman, Robert P. Naftel, Joshua J. Chern, John C. Wellons III, Curtis J. Rozzelle, Jeffrey P. Blount and W. Jerry Oakes

Object

The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation.

Methods

The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010.

Results

The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and “return to school” time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed.

Conclusions

The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.

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Robert P. Naftel, Chevis N. Shannon, Gavin T. Reed, Richard Martin, Jeffrey P. Blount, R. Shane Tubbs and John C. Wellons III

Object

The use of intraventricular endoscopy to achieve diagnosis or to resect accessible intraventricular or paraventricular tumors has been described in the literature in both adults and children. Traditionally, these techniques have not been used in patients with small ventricles due to the perceived risk of greater morbidity. The authors review their experience with the effectiveness and safety of endoscopic brain tumor management in children with small ventricles.

Methods

Between July 2002 and December 2009, 24 children with endoscopically managed brain tumors were identified. Radiological images were reviewed by a radiologist blinded to study goals and clinical setting. Patients were categorized into small-ventricle and ventriculomegaly groups based on frontal and occipital horn ratio. Surgical success was defined a priori and analyzed between groups. Trends were identified in selected subgroups, including complications related to pathological diagnosis and surgeon experience.

Results

Six children had small ventricles and 18 had ventriculomegaly. The ability to accomplish surgical goals was statistically equivalent in children with small ventricles and those with ventriculomegaly (83% vs 89%, respectively, p = 1.00). There were no complications in the small-ventricle cohort, but in the ventriculomegaly cohort there were 2 cases of postoperative hemorrhages and 1 case of infection. All hemorrhagic complications occurred in patients with high-grade tumor histopathological type and were early in the surgeon's endoscopic career.

Conclusions

Based on our experience, endoscopy should not be withheld in children with intraventricular tumors and small ventricles. Complications appear to be more dependent on tumor histopathological type and surgeon experience than ventricular size.

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Robert P. Naftel, R. Shane Tubbs, Gavin T. Reed and John C. Wellons III

The authors describe a new technique that may be used in conjunction with neuronavigation or freehand techniques for obtaining small ventricular access. Using this modification, the introducer sheath and trocar can be guided down a ventriculostomy tract with endoscopic visual control. With increasing focus on endoscopic therapies in patients without hydrocephalus, this adjunct, based on the authors' experience, may provide an additional technique for safely treating patients.

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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.