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Paul Klimo Jr., Samuel R. Browd, Svetlana Pravdenkova, William T. Couldwell, Marion L. Walker and Ossama Al-Mefty

Object

Various lesions occur in deep locations or at the skull base in pediatric patients and require skull base approaches for resection. Skull base surgery confers the advantages of improved line of sight, a wider operative corridor, and reduced brain retraction. The posterior petrosal approach provides simultaneous access to lesions in the posterior middle fossa and posterior fossa from the top of the clivus to the level of the jugular foramen. It allows visualization of the ventrolateral brainstem and may be combined with various other supra- and infratentorial approaches, thus giving the surgeon a wide array of access routes to the lesion.

Methods

The authors conducted a retrospective review of all cases involving pediatric patients undergoing a posterior petrosal approach, either alone or in combination with other cranial approaches. Preoperative and postoperative data were collected, including presentation, neurological examination, imaging findings, pathological condition, operative details, perioperative complications, and postoperative outcomes.

Results

There were 13 patients (6 female, 7 male) with a mean age of 12.6 years (range 14 months–9 years). The posterior petrosal was the sole skull base cranial approach in 4 patients, whereas the posterior petrosal was combined with 1 or more other cranial approaches in 9. A gross-total resection was achieved in 7 patients, subtotal resection in 5, and a biopsy was performed in 1. Complications occurred in 9 patients, including 7 new or worsened cranial neuropathies. There was no perioperative mortality.

Conclusions

Although infrequently used in pediatric neurosurgery, the posterior petrosal approach is a highly versatile approach that can access intra- and extraaxial pathology centered on the petrous bone. The authors believe that patient outcomes are directly related to the degree of experience using this approach. Therefore, if this approach is to be used, they recommend collaboration with a skull base neurosurgeon.

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Paul Klimo Jr., Patrick J. Codd, Holcombe Grier and Liliana C. Goumnerova

Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery.

Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.

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Paul Klimo Jr., Peter Kan, Ganesh Rao, Ronald Apfelbaum and Douglas Brockmeyer

Object

The most contentious issue in the management of os odontoideum surrounds the decision to attempt atlantoaxial fusion in patients with asymptomatic lesions. The authors examined the clinical presentation and outcome in patients with os odontoideum who underwent surgical stabilization, with an emphasis on 3 patients who initially received conservative treatment and suffered delayed neurological injury.

Methods

Seventy-eight patients (mean age 20.5 years; median 15 years) were identified in a 17-year retrospective review. The median follow-up period was 14 months (range 1–115 months). Neck pain was the most common symptom (64%), and 56% of patients presented after traumatic injury. Eighteen patients had neurological signs or symptoms at presentation, and an additional 15 had a history of intermittent or prior neurological symptoms. Fifteen patients had undergone ≥ 1 attempt at atlantoaxial fusion elsewhere.

Results

Seventy-seven patients underwent posterior fusion and rigid screw fixation combined with a graft/wire construct: 75 had C1–2 fusion and 2 had occipitocervical fusion. One patient had an odontoid screw placed. Fusion was achieved in all patients at a median of 4.8 months (range 2–17 months). Approximately 90% of patients had resolution or improvement of their neck pain or neurological symptoms.

Conclusions

The authors believe that patients with os odontoideum are at risk for future spinal cord compromise. Forty-four percent of our patients had myelopathic symptoms at referral, and 3 had significant neurological deterioration when a known os odontoideum was left untreated. This risk of late neurological deterioration should be considered when counseling patients. Stabilization using internal screw fixation techniques resulted in 100% fusion, whereas 15% of patients had previously undergone unsuccessful wire and external bracing attempts.

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Ruchira M. Jha, Paul Klimo Jr. and Edward R. Smith

Achondroplasia has a known association with foramen magnum stenosis that can result in cervicomedullary compression, which is most often due to a hypertrophied posterior occipital rim and an undersized transverse diameter. The authors present a unique case of a child with achondroplasia with symptomatic craniocervical compression from marked overgrowth of his opisthion anterior to the posterior arch of the atlas. This 22-month-old child with achondroplasia presented with severe respiratory and motor disabilities, including progressive quadriparesis and apneic episodes requiring continuous positive airway pressure. Magnetic resonance imaging and CT scans revealed marked foramen magnum stenosis from overgrowth of the opisthion, a hypoplastic C-1 ring, and spinal cord edema at the cervicomedullary junction. Foramen magnum decompression and a C-1 laminectomy were performed. Postoperatively, steady motor improvement has been observed and the patient no longer requires ventilatory support. To the authors' knowledge, this is the first report of this unusual anatomical entity.

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Michael Finn, Paul Klimo Jr. and William T. Couldwell

✓Dural arteriovenous fistulas (dAVFs) are acquired direct arteriovenous shunts that often drain into the dural venous sinus. Treatment options generally involve disrupting the abnormal vascular conduits by using a combination of modalities, including surgical disconnection, radiosurgery, and transarterial and transvenous embolization. Often these modalities provide only partial treatment of fistulous lesions, and thus the fistula recurs and symptoms result. The authors report on a novel surgical technique in which the involved venous sinuses are skeletonized and an interpositional dural substitute is placed between the disconnected sinus and native dura mater and over the pial surface adjacent to the sinus. The technique, which is demonstrated in an illustrative case, is intended to preserve native venous drainage and to prevent recruitment of new vascularization to the venous sinus postoperatively. The authors have not observed reconstitution of fistulas over areas treated with this technique, which offers the advantage of inhibiting vascular ingrowth (refistulization) while maintaining venous sinus patency.

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Patrick J. Codd, Ron I. Riesenburger, Paul Klimo Jr., Jonathan R. Slotkin and Edward R. Smith

✓ Aneurysmal bone cysts (ABCs) are benign, highly vascular osseous lesions characterized by cystic, blood-filled spaces surrounded by thin perimeters of expanded bone. Children and young adults are most often affected by spinal ABCs; more than 75% of patients are younger than 20 years old at presentation. Although ABCs have been documented in all areas of the axial and appendicular skeleton, ABCs of the spine present unique challenges due to the risk of vertebral destabilization, pathological fracture and vertebral body (VB) collapse, and neurological compromise. The authors describe the case of an 8-year-old child who presented with low-back pain and was subsequently found to have a lumbar ABC causing vertebra plana of the L-3 VB. They also review the literature on ABCs of the spine. This case highlights the importance of considering an ABC in the differential diagnosis when vertebra plana is seen in pediatric patients.

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Paul Klimo Jr. and Liliana C. Goumnerova

Object

The authors retrospectively reviewed the charts of all patients harboring brainstem tumors treated at their institution, excluding those with tectal gliomas, who underwent an endoscopic third ventriculocisternostomy.

Methods

Endoscopic third ventriculocisternostomy was performed in 13 patients with tumors involving the brainstem: nine patients with diffuse pontine gliomas, two with posterior fossa ependymomas, one with a cervicomedullary tumor, and one with a pontine primitive neuroectodermal tumor. No technical difficulties attributable to the location of the tumors or surgery-related complications were encountered. Immediate symptomatic relief of hydrocephalus was achieved in all patients, and there was an associated decrease in steroid and analgesic agent requirements. Only one patient eventually required a shunt.

Conclusions

Endoscopic third ventriculocisternostomy can be used in the terminal treatment of patients with brainstem tumors, yielding good results without significant surgical morbidity.

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Paul Klimo Jr. and Richard H. Schmidt

✓The elucidation of predictive factors of cerebral vasospasm following aneurysmal subarachnoid hemorrhage (SAH) is a major area of both clinical and basic science research. It is becoming clear that many factors contribute to this phenomenon. The most consistent predictor of vasospasm has been the amount of SAH seen on the postictal computed tomography scan. Over the last 30 years, it has become clear that the greater the amount of blood within the basal cisterns, the greater the risk of vasospasm. To evaluate this risk, various grading schemes have been proposed, from simple to elaborate, the most widely known being the Fisher scale. Most recently, volumetric quantification and clearance models have provided the most detailed analysis. Intraventricular hemorrhage, although not supported as strongly as cisternal SAH, has also been shown to be a risk factor for vasospasm.

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Paul Klimo Jr., Richard C. E. Anderson and Douglas L. Brockmeyer

✓Two cases of a previously undescribed cervical spinal anomaly distinct from cervical spondylolysis are presented. The authors report the first detailed description of a congenital vertebral anomaly characterized by multilevel cervical spondylolysis, sagittal deformity, and spinal cord compression. The sine qua non of the condition is a lack of communication between the anterior and posterior columns of the cervical spinal canal, which may occur over several vertebral levels. A kyphotic deformity of the anterior column occurs, whereas the posterior column may have relatively normal alignment. The underlying biomechanical stresses caused by the anterior–posterior column disconnection result in spinal instability and progressive kyphotic deformity, often to a profound degree. Two children, 2 and 3 years of age, presented with congenital multilevel disconnection and myelopathy. In the first stage of treatment, each underwent an anterior decompression, reduction, and reconstruction of the involved segments. This was followed by posterior stabilization and fusion as a separate procedure. In both patients, the myelopathy improved and a solid, circumferential fusion was achieved. The authors’ success in treating these patients indicates that management of these conditions can be based on the principles of deformity correction, spinal cord decompression, and combined anterior–posterior arthrodesis.

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Paul Klimo Jr. and John R. W. Kestle

✓The choice of outcome (or outcomes) and their measurement are critical for a sound clinical trial. Surgeons have traditionally measured simple outcomes such as death, duration of survival, or tumor recurrence but have recently developed more sophisticated measures of the effect of an intervention. Many outcome measures require a lengthy maturation process, which includes a determination of the instrument's validity, reliability, and sensitivity; thus, using established instruments rather than creating new ones is recommended. The authors illustrate several guidelines for the determination of appropriate outcome measures by using examples from their experience and describe several outcome measures that can be used in pediatric neurosurgery. These include general outcome measures such as the Pediatric Evaluation of Disability Inventory and the Functional Independence Measure for Children, which measure physical function and independence in chronically ill and disabled children as well as disease-specific measures for hydrocephalus (Hydrocephalus Outcome Questionnaire), cerebral palsy (gross motor function and performance measures), head injury (Pediatric Cerebral Performance Category and Children's Coma Scale), and oncology (Pediatric Cancer Quality-of-Life Inventory).