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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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Joshua J. Chern, Mitchel Muhleman, R. Shane Tubbs, Joseph H. Miller, James M. Johnston, John C. Wellons III, Jeffrey P. Blount, W. Jerry Oakes and Curtis J. Rozzelle

Object

Most children with spina bifida aperta have implanted CSF shunts. However, the efficacy of adding surveillance imaging to clinical evaluation during routine follow-up as a means to minimize the hazard of shunt failure has not been thoroughly studied.

Methods

A total of 396 clinic visits were made by patients with spina bifida aperta and shunt-treated hydrocephalus in a spina bifida specialty clinic during the calendar years 2008 and 2009 (initial clinic visit). All visits were preceded by a 6-month period during which no shunt evaluation of any kind was performed and were followed by a subsequent visit in the same clinic. At the initial clinic visit, 230 patients were evaluated by a neurosurgeon (clinical evaluation group), and 166 patients underwent previously scheduled surveillance CT scans in addition to clinical evaluation (surveillance imaging group). Subsequent unexpected events, defined as emergency department (ED) visits and caregiver-requested clinic visits, were reviewed. The time to an unexpected event and the likelihood of event occurrence in each of the 2 groups were compared using Cox proportional hazards survival analysis. The outcome and complications of shunt surgeries were also reviewed.

Results

The clinical characteristics of the 2 groups were similar. In the clinical evaluation group, 2 patients underwent shunt revision based on clinical findings in the initial visit. In the subsequent follow-up period, there were 27 visits to the ED and 25 requested clinic visits that resulted in 12 shunt revisions. In the surveillance imaging group, 11 patients underwent shunt revision based on clinical and imaging findings in the initial visit. In the subsequent follow-up period, there were 15 visits to the ED and 9 requested clinic visits that resulted in 8 shunt revisions. Patients who underwent surveillance imaging on the day of initial clinic visit were less likely to have an unexpected event in the subsequent follow-up period (relative risk 0.579, p = 0.026). The likelihood of needing shunt revision and the morbidity of shunt malfunction was not significantly different between the 2 groups.

Conclusions

Surveillance imaging in children with spina bifida aperta and shunted hydrocephalus decreases the likelihood of ED visits and caregiver-requested clinic visits in the follow-up period, but based on this study, its effect on mortality and morbidity related to shunt malfunction was less clear.

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Joshua J. Chern, Jennifer L. Kirkman, Chevis N. Shannon, R. Shane Tubbs, Jeffrey D. Stone, Stuart A. Royal, W. Jerry Oakes, Curtis J. Rozzelle and John C. Wellons

Object

Various cutaneous stigmata and congenital anomalies are accepted as sufficient reasons to perform lumbar ultrasonography as a screening tool to rule out occult spinal dysraphism (OSD). The purpose of this study was to correlate presenting cutaneous findings with lumbar ultrasonography results based on a large number of lumbar ultrasonography tests obtained by regional primary care providers.

Methods

Over the course of 5 years, 1273 infants underwent lumbar ultrasonography screening at a major pediatric tertiary referral center. Of these infants, 1116 had adequate documentation for retrospective chart review. Referral sources included urban academic, urban private practice, and surrounding rural private practitioners. Presence of cutaneous stigmata and/or congenital anomalies and lumbar ultrasonography results were reviewed for all patients. When present, surgical findings were reviewed.

Results

A total of 943 infants were referred for presumed cutaneous stigmata, the most common of which was a sacral dimple (638 patients [68%]) followed by hairy patch (96 patients [10%]). Other reported cutaneous findings included hemangioma, deviated gluteal fold, skin tag, and skin discoloration. In comparison, 173 patients presented with congenital anomalies, such as imperforate anus (56 patients [32%]) and tracheoesophageal fistula/esophageal atresia (37 patients [21%]), most of which were detected prenatally by fetal ultrasonography. A total of 17 infants underwent surgical exploration. Occult spinal dysraphism was diagnosed in 7 infants in the cutaneous stigmata group and in 10 infants in the group with congenital abnormalities. None of the cutaneous stigmata as recorded were found to be indicative of the presence of OSD.

Conclusions

Cutaneous markers as currently defined by general practitioners are not useful markers for predicting OSD. The vast majority of findings on lumbar ultrasonography studies performed under these circumstances will be negative.

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Joshua J. Chern, Joseph H. Miller, R. Shane Tubbs, Thomas R. Whisenhunt, James M. Johnston, John C. Wellons III, Curtis J. Rozzelle, Jeffrey P. Blount and W. Jerry Oakes

Object

A large volume of patients presented to a Level I pediatric trauma center during and after a recent tornado disaster. Injuries of the central and peripheral nervous systems and the medical responses of a pediatric neurosurgical team are reviewed.

Methods

The clinical courses of patients who suffered cranial, spinal, and peripheral nerve injuries due to the tornado storm are reported. The clinical actions taken by the neurosurgical team during and after the event are reviewed and the lessons learned are discussed.

Results

The tornado storm system moved through the Tuscaloosa and Birmingham metropolitan areas on the early evening hours of April 27, 2011. Twenty-four patients received care from the neurosurgical team. A total of 11 cranial (including placement of an external ventricular drain), 2 spine, and 2 peripheral procedures were performed for the victims. Nine procedures were performed within the first 12 hours of the event, and an additional 6 surgeries were performed in the following 24 hours. Injuries of the peripheral nervous system often presented in a delayed fashion. Several key components were identified that enabled adequate neurosurgical care for a large influx of acute patients.

Conclusions

Massive casualties due to tornados are rare. A well-organized physician team working with the hospital administration may decrease the mortality and morbidity of such events.

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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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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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R. Shane Tubbs, Marios Loukas, Mohammadali M. Shoja, Ghaffar Shokouhi, John C. Wellons III, W. Jerry Oakes and Aaron A. Cohen-Gadol

Object

Various donor nerves, including the ipsilateral long thoracic nerve (LTN), have been used for brachial plexus neurotization procedures. Neurotization to proximal branches of the brachial plexus using the contralateral long thoracic nerve (LTN) has, to the authors' knowledge, not been previously explored.

Methods

In an attempt to identify an additional nerve donor candidate for proximal brachial plexus neurotization, the authors dissected the LTN in 8 adult human cadavers. The nerve was transected at its distal termination and then passed deep to the clavicle and axillary neurovascular bundle. This passed segment of nerve was then tunneled subcutaneously and contralaterally across the neck to a supra- and infraclavicular exposure of the suprascapular and musculocutaneous nerves. Measurements were made of the length and diameter of the LTN.

Results

All specimens were found to have a LTN that could be brought to the aforementioned contralateral nerves. Neural connections remained tension free with left and right neck rotation of ~ 45°. The mean length of the LTN was 22 cm with a range of 18–27 cm. The overall mean diameter of this nerve was 3.0 mm. No gross evidence of injury to surrounding neurovascular structures was identified in any specimen.

Conclusions

Based on the results of this cadaveric study, the use of the contralateral LTN may be considered for neurotization of the proximal musculocutaneous and suprascapular nerves.

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

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R. Shane Tubbs, Marios Loukas, Mohammadali M. Shoja, Robert J. Spinner, Erik H. Middlebrooks, William R. Stetler Jr., Leslie Acakpo-Satchivi, John C. Wellons III, Jeffrey P. Blount and W. Jerry Oakes

Object

The suprascapular nerve may become entrapped as it travels deep to the suprascapular ligament, necessitating decompression. The present study was performed to verify the feasibility of a minimally invasive, endoscopically assisted technique for decompressing the suprascapular nerve in the supraspinous fossa.

Methods

The authors performed dissection and decompression of the suprascapular ligament using an endoscopically assisted technique via a 3-cm skin incision in 10 adult cadavers (20 sides). Measurements were also made of the depth from the skin to the suprascapular ligament.

Results

A mean depth of 4 cm was necessary to reach the suprascapular ligament from the skin surface. With the authors' approach, no obvious injury occurred to the suprascapular or other vicinal neurovascular structures (such as the spinal accessory nerve and suprascapular vessels).

Conclusions

The results of this cadaveric study demonstrate that access to the suprascapular nerve can be obtained endoscopically via a small suprascapular incision. This approach obviates a large incision, entry into the glenohumeral joint, and reduces the risk of spinal accessory nerve injury in the posterior cervical triangle, or atrophy of the trapezius or supraspinatus muscles from a standard larger dissection. To the authors' knowledge an endoscopically assisted approach to decompressing the suprascapular nerve as it courses deep to the suprascapular ligament has not been reported previously.

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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.