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Daniel C. Lu and Peter P. Sun

✓The authors describe the use of bone morphogenetic protein (BMP) to promote bone fusion in an infant with craniovertebral instability after two attempts at arthrodesis had failed. To their knowledge, this is the first such report. Management of craniovertebral instability remains challenging in infants with Down syndrome. Surgical treatment may result in nonunion in this patient population. The authors report on a 4-month-old boy with Down syndrome who suffered a high cervical spinal cord injury secondary to craniovertebral instability. Two attempts to fuse and stabilize the craniovertebral junction resulted in nonunion. Finally, the use of BMP led to a stable fusion construct within 6 months without encroachment on the spinal canal. At 4 years of follow up, the patient has a solid fusion mass. The case suggests a role for the use of BMP to promote fusion in this patient population.

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Susan R. Durham, Peter P. Sun and Leslie N. Sutton

Object. This outcome study was undertaken to investigate the long-term results obtained in surgically treated pediatric patients with lumbar disc disease by using standardized medical outcome scales and clinical follow-up examination.

Methods. Twenty nine patients 17 years of age or younger underwent surgery between 1968 and 1998 for lumbar disc disease. The follow-up period ranged from 4 months to 30.5 years (mean 8.5 years). Outcome scores (health profiles) were generated using a standardized medical outcome scale, the Short Form health survey questionnaire (SF-36), and a condition-specific back pain outcome scale. Clinical follow-up data were obtained by telephone interview.

The health profile of the study population closely paralleled that of the normal population and was distinctly different from the health profile of adults with low-back pain. Only physical functioning, as measured by a scale of the SF-36, was found to be impaired in a subset of the study population. The rate of reoperation was 24% over the course of the follow-up period. In contrast to similar studies in adults, there were no identifiable predictive factors for either reoperation or poor outcome.

Conclusions. Lumbar disc disease in the pediatric population does not appear to lead to chronic complaints of back pain, and it does not appear to have a negative impact on overall health. This finding suggests that pediatric lumbar disc disease may be a separate entity distinct from adult lumbar disc disease, and therefore, the same conclusions regarding long-term outcome cannot be applied to the pediatric population.

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Doris D. Wang, Kenneth W. Martin, Kurtis I. Auguste and Peter P. Sun

Disorders of CSF dynamics such as syringomyelia and obstructive hydrocephalus can be caused by thin mobile obstructive lesions not visible on traditional MRI sequences. New imaging techniques with balanced steady-state free precession (bSSFP) and dynamic imaging with bSSFP cine allow visualization of these pulsatile structures within the CSF space. The authors present 2 cases involving pediatric patients—one who developed presumed idiopathic syringomyelia and one with presumed communicating hydrocephalus in association with Pfeiffer syndrome—who harbored thin dynamic obstructive lesions seen on bSSFP cine studies using 1.5-T MRI.

In combination with traditional CSF cine studies and bSSFP, bSSFP cine sequence was able to detect dynamic membranous adhesions not seen on traditional MRI sequences. These previously undetectable lesions on traditional MRI sequences were the etiology of CSF obstruction, and tailored surgical approaches were performed to avoid shunting in both patients. These reports demonstrate the clinical utility for using these novel imaging tools for the detection of thin adhesions and dynamic lesions in the central nervous system. Balanced SSFP cine sequences can supplement conventional MR modalities to identify these otherwise poorly visualized lesions responsible for presumed communicating hydrocephalus or idiopathic syringomyelia.

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Darryl Lau, Cecilia L. Dalle Ore, Kenneth W. Martin, James F. Policy and Peter P. Sun

Pedicle aplasia is an uncommon congenital anomaly most frequently involving the absence of a single pedicle at a single vertebral level. Bilateral pedicle aplasia at multiple levels is exceedingly rare and has only been described once previously in the literature. While single-level pedicle aplasia is often asymptomatic and discovered incidentally, pedicle aplasia of multiple levels may produce severe spinal deformities and neurological deficits. Due to the rarity of this condition, optimal management remains uncertain. In this case report, the authors describe the surgical management of a healthy 9-year-old boy who presented with frequent falls, difficulty running, and severe thoracic kyphotic deformity and was found to have bilateral pedicle aplasia from T3 to T9. A review of the literature regarding pedicle aplasia is also presented.

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Aaron J. Clark, Rachel A. Kuperman, Kurtis I. Auguste and Peter P. Sun

Vagus nerve stimulation (VNS) is used as palliation for adult and pediatric patients with intractable epilepsy who are not candidates for curative resection. Although the treatment is generally safe, complications can occur intraoperatively, perioperatively, and in a delayed time frame. In the literature, there are 2 reports of pediatric patients with implanted VNS units who had refractory bradycardia that resolved after the stimulation was turned off. The authors report the case of a 13-year-old boy with a history of vagus nerve stimulator placement at 2 years of age, who developed intractable episodic bradycardia that persisted despite the cessation of VNS and whose imaging results suggested vagus nerve tethering by the leads. He was subsequently taken to the operating room for exploration, where it was confirmed that the stimulator lead was exerting traction on the vagus nerve, which was displaced from the carotid sheath. After the vagus nerve was untethered and the leads were replaced, the bradycardia eventually resolved with continual effective VNS therapy. When placing a VNS unit in a very young child, accommodations must be made for years of expected growth. Delayed intractable bradycardia can result from a vagus nerve under traction by tethered stimulator leads.

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Dario J. Englot, Jonathan D. Breshears, Peter P. Sun, Edward F. Chang and Kurtis I. Auguste

While temporal lobe epilepsy (TLE) is the most common epilepsy syndrome in adults, seizures in children are more often extratemporal in origin. Extra–temporal lobe epilepsy (ETLE) in pediatric patients is often medically refractory, leading to significantly diminished quality of life. Seizure outcomes after resective surgery for pediatric ETLE vary tremendously in the literature, given diverse patient and epilepsy characteristics and small sample sizes. The authors performed a systematic review and meta-analysis of studies including 10 or more pediatric patients (age ≤ 19 years) published over the last 20 years examining seizure outcomes after resective surgery for ETLE, excluding hemispherectomy. Thirty-six studies were examined. These 36 studies included 1259 pediatric patients who underwent resective surgery for ETLE. Seizure freedom (Engel Class I outcome) was achieved in 704 (56%) of these 1259 patients postoperatively, and 555 patients (44%) continued to have seizures (Engel Class II–IV outcome). Shorter epilepsy duration (≤ 7 years, the median value in this study) was more predictive of seizure freedom than longer (> 7 years) seizure history (odds ratio [OR] 1.52, 95% confidence interval [CI] 1.07–2.14), suggesting that earlier intervention may be beneficial. Also, lesional epilepsy was associated with better seizure outcomes than nonlesional epilepsy (OR 1.34, 95% CI 1.19–1.49). Other predictors of seizure freedom included an absence of generalized seizures (OR 1.61, 95% CI 1.18–2.35) and localizing ictal electroencephalographic findings (OR 1.55, 95% CI 1.24–1.93). In conclusion, seizure outcomes after resective surgery for pediatric ETLE are less favorable than those associated with temporal lobectomy, but seizure freedom may be more common with earlier intervention and lesional epilepsy etiology. Children with continued debilitating seizures despite failure of multiple medication trials should be referred to a comprehensive pediatric epilepsy center for further medical and surgical evaluation.

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Ramin A. Morshed, Darryl Lau, Peter P. Sun and Lauren R. Ostling

Choroid plexus papillomas (CPPs) are typically benign tumors that can occur in any age group but are more commonly found in pediatric patients. Although these tumors are benign, there are several reports in adult patients of distant metastases present either at the time of diagnosis or occurring months to years after initial resection. Here, the authors report the case of a 14-year-old boy who presented with symptoms of elevated intracranial pressure due to obstructive hydrocephalus that was caused by a large fourth ventricular mass. Preoperative imaging included a full MRI of the spine, which revealed an intradural lesion that encased the distal sacral nerve roots at the tip of the thecal sac and was concerning for a drop metastasis. The patient underwent gross-total resection of both the fourth ventricular and sacral tumors with histology of both lesions consistent with benign CPP (WHO Grade I). In addition, the authors review prior reports of both pediatric and adult patients in whom benign CPPs have metastasized with either benign or atypical pathology found at a distant site. Taking into account this unusual case and reports in the literature, patients with even benign CPPs may warrant initial and routine follow-up imaging of the total neural axis in search of the rare, but possible, occurrence of drop metastasis.

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Peter P. Sun, Glen J. Poffenbarger, Susan Durham and Robert A. Zimmerman

Object. Injuries of the occipitoatlantoaxial (Oc—C2) region are the predominant form of cervical injury in children younger than 10 years of age. Magnetic resonance (MR) imaging can be used to visualize directly the traumatic ligamentous and soft-tissue abnormalities of the Oc—C2 region. A retrospective review was undertaken to examine the spectrum of pediatric Oc—C2 injuries seen on MR imaging, their correlation with plain x-ray film and computerized tomography findings, and their clinical course.

Methods. Seventy-one consecutive children younger than 10 years of age underwent cervical MR imaging for evaluation of traumatic injury. Magnetic resonance imaging was used to document abnormalities in 23 children; 20 of these injuries involved the Oc—C2 region. Abnormalities in the Oc—C2 region included disruptions of the musculature, apical ligament, atlantooccipital joint(s), tectorial membrane, and spinal cord. A spectrum of injury with progressive involvement of these structures was seen, ranging from isolated muscular injury to the multiple soft-tissue and ligamentous disruptions with craniocervical dislocation. Involvement of the tectorial membrane was the critical threshold in the transition from stable to unstable injury. Analysis of plain x-ray films revealed that a novel interspinous C1–2:C2–3 ratio criteria of greater than or equal to 2.5 was predictive of tectorial membrane abnormalities on MR imaging, with 87% sensitivity and 100% specificity. In patients with tectorial membrane abnormalities who underwent immobilization alone, interim platybasia was demonstrated on follow-up MR images.

Conclusions. A progressive spectrum of distinct Oc—C2 injuries can occur in young children; the tectorial membrane is a critical stabilizing ligamentous structure in the Oc—C2 complex; and tectorial membrane abnormalities may be identified by a C1–2:C2–3 ratio of greater than or equal to 2.5.

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Tene A. Cage, Aaron J. Clark, Derick Aranda, Nalin Gupta, Peter P. Sun, Andrew T. Parsa and Kurtis I. Auguste

Object

Ependymoma is the third most common primary brain tumor in children. Tumors are classified according to the WHO pathological grading system. Prior studies have shown high levels of variability in patient outcomes within and across pathological grades. The authors reviewed the results from the published literature on intracranial ependymomas in children to describe clinical outcomes as they relate to treatment modality, associated mortality, and associated progression-free survival (PFS).

Methods

A search of English language peer-reviewed articles describing patients 18 years of age or younger with intracranial ependymomas yielded data on 182 patients. These patients had undergone treatment for ependymoma with 1 of 5 modalities: 1) gross-total resection (GTR), 2) GTR as well as external beam radiation therapy (EBRT), 3) subtotal resection (STR), 4) STR as well as EBRT, or 5) radiosurgery. Mortality and outcome data were analyzed for time to tumor progression in patients treated with 1 of these 5 treatment modalities.

Results

Of these 182 patients, 69% had supratentorial ependymomas and 31% presented with infratentorial lesions. Regardless of tumor location or pathological grade, STR was associated with the highest rates of mortality. In contrast, GTR was associated with the lowest rates of mortality, the best overall survival, and the longest PFS. Children with WHO Grade II ependymomas had lower mortality rates when treated more aggressively with GTR. However, patients with WHO Grade III tumors had slightly better survival outcomes after a less aggressive surgical debulking (STR+EBRT) when compared with GTR.

Conclusions

Mortality, PFS, and overall survival vary in pediatric patients with intracranial ependymomas. Pathological classification, tumor location, and method of treatment play a role in outcomes. In this study, GTR was associated with the best overall and PFS rates. Patients with WHO Grade II tumors had better overall survival after GTR+EBRT and better PFS after GTR alone. Patients with WHO Grade III tumors had better overall survival after STR+EBRT. Patients with infratentorial tumors had improved overall survival compared with those with supratentorial tumors. Progression-free survival was best in those patients with infratentorial tumors following STR+EBRT. Consideration of all of these factors is important when counseling families on treatment options.

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Dario J. Englot, John D. Rolston, Doris D. Wang, Peter P. Sun, Edward F. Chang and Kurtis I. Auguste

Temporal lobe epilepsy (TLE) is the most common form of epilepsy in adults and is responsible for 15%–20% of epilepsy cases in children. Class I evidence strongly supports the use of temporal lobectomy for intractable TLE in adults, but fewer studies have examined seizure outcomes and predictors of seizure freedom after temporal lobectomy in pediatric patients. The authors performed a systematic review and meta-analysis of studies including 10 or more pediatric patients (age ≤ 19 years) published over the last 20 years examining seizure outcomes after temporal lobectomy for TLE. Thirty-six studies met their inclusion criteria. These 36 studies included 1318 pediatric patients with a mean age (± SEM) of 10.7 ± 0.3 years. Overall, seizure freedom (Engel Class I outcome) was achieved in 1002 cases (76%); 316 patients (24%) continued to have seizures (Engel Class II–IV outcome). All patients had at least 1 year of follow-up. Statistically significant predictors of seizure freedom after surgery included lesional epilepsy etiology (odds ratio [OR] 1.08, 95% confidence interval [CI] 1.02–1.15), abnormal findings on preoperative MRI (OR 1.27, 95% CI 1.16–1.40), and lack of generalized seizures (OR 1.36, 95% CI 1.20–1.56). Among lesional epilepsy cases, there was a trend toward better outcome with gross-total lesionectomy than with subtotal resection. Approximately three-fourths of pediatric patients with TLE attain seizure freedom after temporal lobectomy. Favorable outcomes may be predicted by lesional epilepsy etiology, abnormal MRI, and lack of generalized seizures. Pediatric patients with medically refractory TLE should be referred to a comprehensive pediatric epilepsy center for surgical evaluation.