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Mark R. Proctor and R. Michael Scott

Object

Split cord malformations (SCMs) are relatively rare forms of occult spinal dysraphism (OSD) and tethered spinal cord syndrome. The majority of these cases present in early childhood, with neurocutaneous stigmata being an early presenting feature. Prophylactic detethering surgery is advocated by most neurosurgeons due to the risk of neurological deterioration over time caused by patient growth and activity. However, unlike other forms of OSD, the course of SCM progression after surgery is not well understood, and little has been published about long-term follow-up results. In this study the authors review the results obtained in 16 patients in whom the senior author performed surgery over a 13-year period (average length of follow up almost 8 years).

Methods

Presentation, surgical approach, and outcome are evaluated, and the long-term outcome of neurological status, pain, bowel/bladder disturbance, and spinal deformities are emphasized.

Conclusions

The primary conclusion is that patients with SCM generally tolerate surgery well and experience few complications. Neurological deterioration is rare except in cases in which retethering occurs, (two patients in this series). Although impaired bowel and bladder function was stabilized or improved and pain was reliably relieved postoperatively, preexisting vertebral column deformities usually progressed after surgery and, in most cases, required spinal fusion.

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Ian F. Dunn, Mark R. Proctor and Arthur L. Day

✓Lumbar spine injuries in athletes are not uncommon and usually take the form of a mild muscle strain or sprain. More severe injuries sustained by athletes include disc herniations, spondylolistheses, and various types of fracture. The recognition and management of these injuries in athletes involve the additional consideration that to return to play, the lumbar spine must be able to withstand forces similar to those that were injurious. The authors consider common lumbar spine injuries in athletes and discuss management principles for neurosurgeons that are relevant to this population.

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John M. K. Mislow, Mark R. Proctor, P. Daniel Mcneely, Arin K. Greene and Gary F. Rogers

✓Calvarial osteolysis is a relatively rare finding in patients with neurofibromatosis. The authors describe two patients with neurofibromatosis Type 1 (NF1) and extensive cranial defects associated with underlying dural ectasia. Cranioplasties were performed in both patients with mixed results. One patient underwent cranioplasty using titanium mesh and methylmethacrylate. The other patient underwent an extensive cranioplasty with autogenous iliac crest grafting, and after initial healing has since had further bone resorption. In conclusion, the results of cranial reconstruction in patients with NF1 and dural ectasia are unpredictable because of the tendency for further bone resorption; techniques that protect the graft material from cerebrospinal fluid pulsations via a rigid mesh should be considered.

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Cormac O. Maher, Lilliana Goumnerova, Joseph R. Madsen, Mark Proctor and R. Michael Scott

Object

Patients who have undergone prior myelomeningocele or lipomyelomeningocele repair may present with symptomatic retethering of the spinal cord. In some cases, symptomatic tethering may recur after previous untethering operations. The expected outcome following repeated untethering in a patient after two or more prior untethering operations is not well described.

Methods

The authors examined surgical indications, techniques, and outcomes for 30 repeated untethering operations in 22 patients who had undergone a previous repair of the primary spinal disorder and at least two subsequent untethering operations.

The mean age at repeated untethering was 12.3 years. Presenting symptoms were pain (70%), weakness (70%), urinary symptoms (57%), and sensory changes (27%). The mean duration of symptoms was 7.5 months, and a longer symptomatic interval correlated with an increased number of prior operations. Total circumferential untethering was accomplished in 11 cases (37%). Postoperative symptomatic improvement was noted most often for pain (81%), and less often for urinary symptoms (53%) and weakness (48%). Complications included postoperative cerebrospinal fluid leakage or pseudomeningocele and new postoperative lower-extremity dysesthesia in five cases (17%). An increasing number of prior untethering operations was associated with a worse result for pain relief and a greater chance of significant morbidity.

Conclusions

Multiple repeated untethering operations offer symptomatic relief to well-selected patients with this condition.

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Chima O. Ohaegbulam, Ian F. Dunn, Pierre d'Hemecourt and Mark R. Proctor

✓ This report describes 3 young male patients with multiple lumbar spondylolyses in combination with a symptomatic epidural hematoma. The records of all 3 patients were reviewed for clinical details. All patients were successfully treated without surgical intervention. Initial neuroimaging results for all patients revealed epidural hematomas, and follow-up imaging confirmed resolution of the hematomas. The relevant literature is briefly reviewed to examine the rarity of this combination. Spontaneous epidural hematomas may occur in the setting of spondylolysis, and this diagnosis should be considered when imaging reveals an unusual epidural lesion in a young active patient.

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Brian V. Nahed, Manuel Ferreira Jr., Matthew R. Naunheim, Kristopher T. Kahle, Mark R. Proctor and Edward R. Smith

Clinical and radiographic evidence of subarachnoid hemorrhage (SAH)-related vasospasm is rare in children and has not been reported in infants. In this report the authors present the case of a 22-month-old child who developed clinically symptomatic, radiographically identifiable vasospasm after traumatic SAH. To the authors' knowledge, this is the first report of vasospasm associated with SAH in a child this young. This 22-month-old boy fell and had a dense SAH. He had a history of surgically corrected craniosynostosis and nonsymptomatic ventriculomegaly. The boy was evaluated for occult vascular lesions using imaging; none were found and normal vessel caliber was noted. Ten days later, the child developed left-sided weakness and a right middle cerebral artery infarct was identified. Evaluation disclosed significant intracranial vasospasm. This diagnosis was supported by findings on CT angiography, transcranial Doppler ultrasonography, MR imaging, and conventional angiography. The child was treated using intraarterial verapamil with a good result, as well as with conventional intensive care measures to reduce vasospasm. This report documents the first known case of intracranial vasospasm with stroke after SAH in a patient under the age of 2 years. This finding is important because it demonstrates that the entity of SAH-associated vasospasm can affect the very young, widening the spectrum of ages susceptible to this condition. This case is also important because it demonstrates that even very young children can respond to conventional therapeutic interventions such as intraarterial verapamil. Thus, clinicians need to be alert to the possibility of vasospasm as a potential diagnosis when evaluating young children with SAH.

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Donald J. Blaskiewicz, Durga R. Sure, Daniel J. Hedequist, John B. Emans, Frederick Grant and Mark R. Proctor

Object

Osteoid osteomas (OOs) are benign lesions of the spine, but can cause significant pain and spinal deformity in the pediatric population. They are often surgically elusive, and may require multiple surgical procedures to ensure complete resection. Nuclear medicine intraoperative bone scans (IOBSs) are highly sensitive for lesion localization and verification of complete surgical extirpation.

Methods

A retrospective review of 20 consecutive patients who had undergone resection of a spinal OO at the authors' institution was undertaken. In all cases, IOBSs were used for lesion localization and verification of resection. Postoperative imaging and clinical follow-up were obtained.

Results

The average length of follow-up was 56 months, with a range of 8–156 months. Five patients had undergone a total of 12 unsuccessful prior procedures for resection at other institutions where IOBSs were not used. In these patients, complete resection was accomplished with the use of IOBSs at the authors' institution. Of the 15 patients who presented to this institution with a newly diagnosed OO and who underwent IOBS-assisted resection, 14 had complete resection without recurrence. One patient, however, was found to have a discrete recurrence adjacent to the initial resection bed at the time of follow-up.

Conclusions

Osteoid osteomas are benign lesions of the spine, and complete resection is curative. If resection is incomplete, then recurrence is likely. The IOBS modality is highly sensitive for detecting OO and for guiding complete resection. The IOBS modality should be considered as a first-line surgical adjunct in cases of suspected OO.

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Cormac O. Maher, Stuart B. Bauer, Liliana Goumnerova, Mark R. Proctor, Joseph R. Madsen and R. Michael Scott

Object

Patients who have undergone myelomeningocele or lipomyelomeningocele repair may present with symptomatic retethering of the spinal cord. The authors describe the results as well as the utility of urological testing in pediatric patients undergoing multiple repeat untethering operations.

Methods

The authors reviewed the records of 13 patients with lipomyelomeningocele or myelomeningocele who underwent at least 2 untethering procedures after their initial repair and who had urodynamic testing within 6 months prior to and 6 months following each untethering operation. In each case, urological testing included a slow-fill cystometrogram and an external urethral sphincter electromyogram using a concentric needle electrode to analyze individual motor unit action potentials at rest, in response to sacral reflexes, and during bladder filling and emptying.

Results

New urinary symptoms were identified in 7 of 13 cases prior to surgery. Postoperative subjective improvement in urinary symptoms was noted in 5 of these 7 cases. Improvement in bladder function on urodynamic testing correlated with symptomatic improvement. Sphincter electromyography findings did not correlate with changes in preoperative symptoms or postoperative improvement.

Conclusions

In patients undergoing multiple repeat spinal cord untethering operations, measuring bladder function is more useful than sphincter electromyographty when selecting candidates for surgery and for measuring surgical outcomes.

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Kevin S. Cahill, Ian Dunn, Thorsteinn Gunnarsson and Mark R. Proctor

Object

Lumbar disc herniation is a rare but significant cause of pain and disability in the pediatric population. Lumbar microdiscectomy, although routinely performed in adults, has not been described in the pediatric population. The objective of this study was to determine the surgical results of lumbar microdiscectomy in the pediatric population by analyzing the experiences at Children's Hospital Boston over the past decade.

Methods

A series of 87 consecutive cases of lumbar microdiscectomy performed by the senior author (M.R.P.) from 1999 to 2008 were reviewed. Presenting symptoms, physical examination findings, and preoperative MR imaging findings were obtained from medical records. Immediate operative results were assessed including operative duration, blood loss, length of stay, and complications, along with long-term outcome and need for repeat surgery.

Results

This series represents the first surgical series of pediatric microdiscectomies. The mean patient age was 16.6 years (range 12–18 years) and 60% were female. The preoperative physical examination results were notable for motor deficits in 26% of patients, sensory changes in 41%, loss of deep tendon reflex in 22%, and a positive straight leg raise in 95%. Conservative management was the first line of treatment in all patients and the mean duration of symptoms until surgical treatment was 12.2 months. The mean operative time was 110 minutes and the mean postoperative length of stay was 1.3 days. Complications were rare: postoperative infection occurred in 1%, postoperative CSF leak in 1%, and new postoperative neurological deficits in 1%. Only 6% of patients needed repeat lumbar surgery and 1 patient ultimately required lumbar fusion.

Conclusions

The treatment of pediatric lumbar disc herniation with microdiscectomy is a safe procedure with low operative complications. Nuances of the presentation, treatment options, and surgery in the pediatric population are discussed.

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Todd C. Hankinson, Anthony M. Avellino, David Harter, Andrew Jea, Sean Lew, David Pincus, Mark R. Proctor, Luis Rodriguez, David Sacco, Theodore Spinks, Douglas L. Brockmeyer and Richard C. E. Anderson

Object

The object of this study was to assess a multiinstitutional experience with pediatric occipitocervical constructs to determine whether a difference exists between the fusion and complication rates of constructs with or without direct C-1 instrumentation.

Methods

Seventy-seven cases of occiput-C2 instrumentation and fusion, performed at 9 children's hospitals, were retrospectively analyzed. Entry criteria included atlantooccipital instability with or without atlantoaxial instability. Any case involving subaxial instability was excluded. Constructs were divided into 3 groups based on the characteristics of the anchoring spinal instrumentation: Group 1, C-2 instrumentation; Group 2, C-1 and C-2 instrumentation without transarticular screw (TAS) placement; and Group 3, any TAS placement. Groups were compared based on rates of fusion and perioperative complications.

Results

Group 1 consisted of 16 patients (20.8%) and had a 100% rate of radiographically demonstrated fusion. Group 2 included 22 patients (28.6%), and a 100% fusion rate was achieved, although 2 cases were lost to follow-up before documented fusion. Group 3 included 39 patients (50.6%) and demonstrated a 100% radiographic fusion rate. Complication rates were 12.5, 13.7, and 5.1%, respectively. There were 3 vertebral artery injuries, 1 (4.5%) in Group 2 and 2 (5.1%) in Group 3.

Conclusions

High fusion rates and low complication rates were achieved with each configuration examined. There was no difference in fusion rates between the group without (Group 1) and those with (Groups 2 and 3) C-1 instrumentation. These findings indicated that in the pediatric population, excellent occipitocervical fusion rates can be accomplished without directly instrumenting C-1.