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Biomedical subjects

Douglas L Brockmeyer

Publications and source records attributed to Douglas L Brockmeyer.

At least 19 recordsLinked to original sources

Chiari I malformation.

Chiari I malformation (CM1), the most common structural hindbrain disorder in humans, is traditionally characterized by the downward displacement of the cerebellar tonsils through the foramen magnum. However, this definition does not reflect the variability in clinical presentation, natural history and treatment response of this disorder. Some individuals with minimal tonsillar descent have severe neurological symptoms and syringomyelia, whereas others with extensive descent remain asymptomatic. Emerging evidence from neuroimaging, developmental biology and human genetics indicates that CM1 is not a single anatomical entity but a spectrum of disorders resulting from disruptions in coordinated growth and homeostasis across the cerebellum, posterior fossa, craniocervical junction, cerebrospinal fluid and neurovascular systems. CM1 may be best understood as a disorder of disrupted developmental scaling, in which the tightly regulated relationships between cerebellar growth and cranial accommodation are altered within a dynamic neurovascular and cerebrospinal fluid environment. In this context, tonsillar herniation is a geometric consequence rather than the primary disease process. This Primer synthesizes current knowledge on the epidemiology, mechanisms, diagnosis and management of CM1 across the lifespan. We highlight advances in neuroimaging, genomics and phenomics that support a shift from anatomy-based definitions towards an integrated genomic-phenomic classification.

Humans↗

The role of diffusion-weighted magnetic resonance imaging in pediatric brain tumors.

OBJECTIVES: Diffusion-weighted imaging (DWI) may enhance the radiographic diagnosis of pediatric brain tumors. This study reviews the DWI properties of pediatric brain tumors at our institution and examines their relationship to tumor grade and type. MATERIALS AND METHODS: The preoperative DWI and apparent diffusion coefficient (ADC) characteristics of brain tumors in 41 children were compared with histologic diagnosis. Signal characteristics on DWI and ADC maps correlated well with tumor grade. High-grade lesions were hyperintense on DWI and hypointense on ADC maps. Sensitivity, specificity, positive predictive value, and negative predictive value were 70, 100, 100, and 91%, respectively. Signal characteristics did not differ among different tumors of the same grade. All primitive neuroectodermal tumors showed diffusion restriction whereas none of the ependymomas did. CONCLUSIONS: The signal characteristics on DWI and ADC maps appeared to be strongly correlated to grade in pediatric brain tumors and they may assist with preoperative diagnostic predictions.

Brain Neoplasms↗

Cerebrospinal fluid dissecting into spinal epidural space after lumbar puncture causing cauda equina syndrome: review of literature and illustrative case.

OBJECTS: We report a case of epidural cerebrospinal fluid (CSF) leak after lumbar puncture caused by CSF dissecting into the spinal epidural space. The incidence of this phenomenon may be higher than suspected, although most cases may remain asymptomatic. MATERIALS AND METHODS: A 4-year-old girl with new-onset seizure underwent a diagnostic lumbar puncture, the results of which were normal; 3 h later, she began experiencing severe low-pressure headaches and lower back pain, bilateral lower extremity weakness, numbness, and pain, and urinary retention when upright. Spinal MRI demonstrated extensive epidural CSF collection posterior to the thecal sac extending from the cervicothoracic junction to the sacrum. After 48 h in the supine position and gradual mobilization, the patient had complete resolution of symptoms and no neurological sequelae. CONCLUSION: Patients usually recover without any neurological deficits after conservative treatment. Prone or lateral decubitus positioning immediately after lumbar puncture may decrease the incidence of this phenomenon.

Cerebrospinal Fluid↗

Cervical spine clearance after trauma in children.

OBJECT: Currently, no diagnostic or procedural standards exist for clearing the cervical spine in children after trauma. The purpose of this study was to determine if reeducation of nonneurosurgical personnel and initiation of a new protocol based on the National Emergency X-Radiography Utilization Study criteria could safely increase the number of pediatric cervical spines cleared of suspected injury without a neurosurgical consultation. METHODS: Data regarding cervical spine clearance in children (ages 0-18 years) after trauma protocol activation at Primary Children's Medical Center between 2001 and 2005 were collected and reviewed. Radiographic and clinical methods of clearing the cervical spine as well as the type and management of injuries were determined for two time frames: Period I (January 2001-December 2003) and Period II (January 2004-July 2005). Between 2001 and 2003, 95% of 936 cervical spines were cleared of suspected injury by the neurosurgical service. Twenty-one ligamentous injuries (2.2%) and 12 fracture-dislocations (1.3%) were detected, with five patients requiring surgical stabilization (0.5%). Between January 2004 and July 2005, 507 (68%) of 746 cervical spines were cleared by nonneurosurgical personnel. Six ligamentous injuries (0.8%) and 10 fracture-dislocations (1.3%) were identified, with three patients (0.4%) requiring surgical stabilization. No late injuries were detected in either period. CONCLUSIONS: The protocol used has been effective in enabling detection of cervical spine injuries in children after trauma, with the new protocol increasing by more than 60% the number of cervical spines cleared by nonneurosurgical personnel. Reeducation with establishment of the new protocols can safely facilitate clearance of the cervical spine by nonneurosurgical personnel after trauma.

Cervical Vertebrae↗

Cervical spine deformity associated with resection of spinal cord tumors.

Postoperative sagittal-plane cervical spine deformities are a concern when laminectomy is performed for tumor resection in the spinal cord. These deformities appear to occur more commonly after resection of intramedullary spinal cord lesions, compared with laminectomy for stenosis caused by degenerative spinal conditions. Postlaminectomy deformities are most common in pediatric patients with an immature skeletal system, but are also more common in young adults (< 25 years of age) in comparison with older adults. The extent of laminectomy and facetectomy, number of laminae removed, location of laminectomy, preoperative loss of lordosis, and postoperative radiation therapy in the spine have all been reported to influence the risk of postlaminectomy spinal deformities. When these occur, patients should be monitored closely with serial imaging studies, because a significant percentage will have progressive deformities. These can range from focal kyphosis to more complicated swan-neck deformities. General indications for surgical intervention include progressive deformity, axial pain in the area, and neurological symptoms attributable to the deformity. Surgical options include anterior, posterior, and combined anterior-posterior procedures. The authors have reviewed the literature on postlaminectomy kyphosis as it relates to resection of cervical spinal cord tumors, and they summarize some general factors to consider when treating these patients.

Cervical Vertebrae↗

Odontoid synchondrosis fractures in children.

OBJECT: Odontoid synchondrosis fractures, although rare in the overall incidence of spinal trauma, are one of the more common fractures in young children. The goal of this study was to evaluate the demographic data, incidence of neurological deficits, treatment strategies, and outcomes in a combined series of odontoid synchondrosis fractures treated at the authors' institution and reported in other series. METHODS: In a retrospective chart review, the authors identified four odontoid synchondrosis fractures treated at their hospital since January 2000; these were combined with cases reported in six other series in the literature, yielding a total of 55 patients. Data regarding the patients' age, sex, delayed diagnosis, odontoid displacement, neurological deficits, treatment, and fusion status were collected. The patients' ages ranged from 9 months to 7 years (mean 2.8 years), with neither sex predominating. Diagnosis was delayed in eight cases. The orientation of the odontoid fracture was reported for 36 patients, with 94% experiencing anterior displacement. Spinal cord injury (SCI) was noted in 15 patients, including 11 with complete injuries and eight with SCI at the cervicothoracic junction. Forty-two (93%) of 45 patients with fractures initially treated with external immobilization attained fusion. Eight patients were treated with surgery; four initially, with no attempt at conservative therapy, three after failed halo immobilization, and one after nonunion because of delayed diagnosis. CONCLUSIONS: Odontoid synchondrosis fractures can be difficult to diagnose. In children younger than 7 years of age who present with neck pain or neurological deficits attributable to SCI, this fracture should be suspected. Given the high rate of fusion attained with conservative therapy, it is recommended for most synchondrosis fractures, although surgery may be warranted for individual cases.

Cartilage, Articular↗

Multilevel cervical disconnection syndrome: initial description, embryogenesis, and management. Report of two cases.

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.

Biomechanical Phenomena↗

Morphometric and qualitative analysis of congenital occipitocervical instability in children: implications for patients with Down syndrome.

OBJECT: Congenital occipitocervical (OC) instability is uncommon in healthy children but can occur in many children with Down syndrome. A simple morphometric method of evaluating the OC joint in children with OC instability is presented, supported by a qualitative image analysis based on computed tomography (CT). METHODS: Thin-cut CT scans of the OC joint were obtained in eight patients with Down syndrome and one patient with congenital OC instability. These patients' CT scans were compared with those of 15 healthy age-matched control individuals. Morphometric analysis was performed by measuring the depth and length of the superior articular surface (SAS) of C-1, and these values were normalized for a comparison between groups. Qualitative data were acquired using a surface-rendering technique for a visual comparison of the C-1 SAS. Morphometric analysis demonstrated an absence of the concave C-1 SAS anatomy in patients with congenital OC instability compared with age-matched control individuals (0.083 compared with 0.202, p < 0.001). Three-dimensional (3D) image analysis of the C-l SAS supported this finding. CONCLUSIONS: Congenital differences in the shape of the OC joint are highly associated with atraumatic OC instability in children with Down syndrome. High-resolution CT imaging combined with 3D rendering techniques and surface mapping provides support for this assessment. It appears that abnormal OC joint shape is a contributing factor to congenital OC instability, especially in patients with Down syndrome.

Adolescent↗

Long-term maintenance of cervical alignment after occipitocervical and atlantoaxial screw fixation in young children.

OBJECT: Despite decades of surgical experience, the long-term consequences of occipitocervical (OC) and atlantoaxial (C1-2) fusions in children are unknown. The purpose of this study was to determine the long-term effects of these fusions on growth and alignment of the maturing cervical spine. METHODS: A retrospective chart review was conducted for patients 6 years of age or younger (mean 4.7 years, range 1.7-6.8 years) who underwent OC or C1-2 fusion at the Primary Children's Medical Center at the University of Utah within the last 10 years. Immediate postoperative plain radiographs and computed tomography (CT) scans were compared with the most recent plain and dynamic radiographs to assess changes in spinal growth and alignment. Seventeen children met entry criteria for the study. All patients had fusion documented on follow-up radiography or CT scans. At a mean follow up of 28 months, there were no cases of sagittal malalignment (kyphotic or swan-neck deformity), subaxial instability (osteophyte formation or subluxation), or unintended fusion of adjacent levels. The lordotic curvature of the cervical spine increased from a mean of 15 degrees postoperatively to 27 degrees at follow up (p = 0.06). A mean of 34% of the vertical growth of the cervical spine occurred within the fusion segment. When data were analyzed pertaining to a subgroup of five patients who underwent follow-up periods for longer than 48 months (mean 50.2 months, range 48-54 months), similar results were seen. CONCLUSIONS: Preliminary follow-up results indicate that, compared with older children, children 6 years of age or younger undergoing OC or C1-2 fusion are not at an increased risk of spinal deformity or subaxial instability. Longer follow-up periods, during which measurements of the spinal canal are taken, will be necessary to determine precisely how children's spines grow and remodel after an upper cervical spine fusion.

Age Factors↗

Epidural venous engorgement resulting in progressive cervical myelopathy from shunt-related intracranial hypotension. Case report and review of the literature.

The authors report an unusual case of engorged epidural veins causing progressive cervical myelopathy after long-term cerebrospinal fluid (CSF) shunt therapy and intracranial hypotension. An 18-year-old woman, who had previously undergone shunt placement with a distal slit valve for a porencephalic cyst when 2 years of age, presented with progressive spastic quadriparesis, numbness, and gait difficulty. Postural headaches were absent and a lumbar puncture revealed low CSF pressure. Neuroimaging disclosed markedly engorged anterior epidural veins causing compression of the cervical spinal cord. The slit-valve shunt system was surgically removed and an external drain was placed. The patient's CSF pressure was gradually raised to clinically tolerable levels. Once the optimal pressure was identified, a programmable shunt was placed with the valve set at the same level. The patient's neurological status improved, and the epidural veins had returned to their normal size on follow-up imaging. The authors describe the unique treatment strategy used in this patient and review the literature on epidural venous engorgement as it relates to intracranial hypotension.

Adolescent↗

Cranial base strategies for resection of craniopharyngioma in children.

The optimal treatment of craniopharyngioma in children remains a challenge. The use of complete excision to minimize recurrence continues to be controversial because of the risk of postoperative morbidity and death. Advances in skull base approaches, modern microsurgical techniques, neuroimaging, and hormone replacement therapy, however, have allowed safe gross- or near-total resection in the majority of cases. Total removal of these tumors, if possible, offers the best chance of cure for the patient. Although craniopharyngiomas are not strictly tumors of skull base origin, their intimate relationship with the neurovascular structures of this region often requires a skull base approach to maximize the surgical corridor and facilitate adequate microsurgical resection. In this review, the authors focus on commonly used skull base approaches for the surgical management of craniopharyngioma. They discuss the relative indications, advantages, disadvantages, and complications associated with each approach. Illustrative cases and intraoperative videos are presented.

Child↗

Expansion of arachnoid cysts in children: report of two cases and review of the literature.

Arachnoid cysts are intracranial, space-occupying lesions that typically remain stable in size on serial imaging. The authors describe two cases of rapidly enlarging arachnoid cysts, including one located in the anterior fossa. In the first case a 7-month-old boy presented with increasing head circumference and a rapidly enlarging arachnoid cyst in the left middle fossa, which had been documented by serial imaging over the preceding 6 months. In the second case a 4-year-old girl presented with an arachnoid cyst compressing the right frontal lobe. The cyst had not been present on imaging studies performed during the perinatal period. In both cases, a craniotomy for open fenestration of the cyst was performed with successful resolution of the mass effect. Rare cases of expansion of arachnoid cysts have been reported in the literature. In this article the authors report the dramatic enlargement of two arachnoid cysts, including the first description of enlargement of an arachnoid cyst located in the anterior fossa.

Arachnoid Cysts↗

Atlantoaxial transarticular screw fixation: a review of surgical indications, fusion rate, complications, and lessons learned in 67 pediatric patients.

OBJECT: In this, the second of two articles regarding C1-2 transarticular screw fixation, the authors discuss their surgical experience in treating patients 16 years of age and younger, detailing the rate of fusion, complication avoidance, and lessons learned in the pediatric population. METHODS: The authors retrospectively reviewed 67 consecutive patients (23 girls and 44 boys) younger than 16 years of age in whom at least one C1-2 transarticular screw fixation procedure was performed. A total of 127 transarticular screws were placed in these 67 patients whose mean age at time of surgery was 9 years (range 1.7-16 years). The indications for surgery were trauma in 24 patients, os odontoideum in 22 patients, and congenital anomaly in 17 patients. Forty-four patients underwent atlantoaxial fusion and 23 patients underwent occipitocervical fusion. Two of the 67 patients underwent halo therapy postoperatively. All patients were followed for a minimum of 3 months. In all 67 patients successful fusion was achieved. Complications occurred in seven patients (10.4%), including two vertebral artery injuries. CONCLUSIONS: The use of C1-2 transarticular screw fixation, combined with appropriate atlantoaxial and craniovertebral bone/graft constructs, resulted in a 100% fusion rate in a large consecutive series of pediatric patients. The risks of C1-2 transarticular screw fixation can be minimized in this population by undertaking careful patient selection and meticulous preoperative planning.

Adolescent↗

Spinal cord schwannomas mimicking drop metastases in a patient with intramedullary ependymoma and neurofibromatosis 2.

The authors describe a 9-year-old boy with a large spinal cord ependymoma whose apparent drop metastases on imaging studies were later demonstrated to be schwannomas. This finding led to the discovery of small bilateral acoustic neuromas and an intracranial meningioma, establishing the diagnosis of neurofibromatosis 2. The presence of additional radiographic abnormalities in a patient with a spinal cord tumor should prompt careful consideration of the diagnosis of neurofibromatosis 2, as early identification of this disorder may significantly affect patient management.

Child↗

Juvenile pilocytic astrocytoma of the brainstem in children.

OBJECT: In reports involving the operative treatment of brainstem tumors, multiple histological types are often grouped together. To determine prognosis after resection, histology-specific data may be helpful. METHODS: Twenty-eight patients with juvenile pilocytic astrocytoma (JPA) of the brainstem (six in the midbrain, four in the pons, and 18 in the medulla) were identified from the medical records. Initial treatment was resection in 25 and biopsy sampling in three. Postoperative imaging revealed gross-total resection (GTR) or resection with linear enhancement (RLE) in 12 of 25 patients and solid residual tumor in the other 13. In 10 of the 13 patients harboring solid residual tumor, observation was undertaken; the residual lesion disappeared in one, was stable in four, and progressed in five. Of the 12 patients with complete excision or RLE only, seven underwent no further treatment, with tumor progression occurring in one. All patients were alive at last follow-up examination (range 0.3-20.4 years, mean 6 years). New neurological deficits commonly appeared immediately after resection but often resolved. In six of the 28 patients, the new postoperative deficit was still present at last follow-up visit. The 5- and 10-year progression-free survival was 74 and 62%, respectively, after GTR or RLE and 19 and 19%, respectively, when solid residual tumor was present. CONCLUSIONS: Long-term survival after resection of JPAs of the brainstem has been observed and appears to be related to the extent of initial excision.

Adolescent↗

Complications of intracranial pressure monitoring in children with head trauma.

OBJECT: Intracranial pressure (ICP) monitoring has become routine in the management of patients with traumatic brain injury (TBI). Many surgeons prefer to use external ventricular drains (EVDs) over fiberoptic monitors to measure ICP because of the added benefit of cerebrospinal fluid drainage. The purpose of this study was to examine a consecutive series of children with TBI and compare the incidence of complications after placement of an EVD, a fiberoptic intraparencyhmal monitor, or both. METHODS: A retrospective chart review was conducted to identify children with TBI who met the criteria for insertion of an ICP monitor. All patients underwent head CT scanning on admission and after placement of an ICP monitor. During a 5-year period 80 children met the criteria for inclusion in the study. Eighteen children (22.5%) underwent EVD placement only, 18 (22.5%) underwent placement of a fiberoptic device only, and 44 (55%) received both. A total of 62 fiberoptic devices (48%) were inserted, and 68 EVDs (52%) were placed. Overall, there was a fourfold increased risk of complications in children who received an EVD compared with those in whom a fiberoptic monitor was placed (p = 0.004). Hemorrhagic complications were detected in 12 (17.6%) of 62 patients who received an EVD compared with four (6.5%) of 62 patients who received a fiberoptic monitor (p = 0.025). Six (8.8%) of 68 EVDs were malpositioned and required replacement; in three (50%) of these cases a hemorrhagic complication occurred. Only one infection was noted in a patient with an EVD (1.5%). CONCLUSIONS: In this retrospective cohort of pediatric patients with TBI, complication rates were significantly higher in those receiving EVDs than in those in whom fiberoptic monitors were placed. Although the majority of these complications did not entail clinical sequelae, surgeons should be aware of the different complication rates when choosing the most appropriate device for each patient.

Adolescent↗

Surgical management of aneurysmal bone cysts of the spine.

OBJECT: Aneurysmal bone cysts of the spine are benign, highly vascular osseous lesions of unknown origin that may present difficult diagnostic and therapeutic challenges. They are expansile lesions containing thin-walled, blood-filled cystic cavities that cause bone destruction and sometimes spinal deformity and neurological compromise. The treatment of aneurysmal bone cysts of the spine remains controversial according to the literature. In this review, the authors discuss the clinical manifestations, pathophysiological features, neuroimaging characteristics, and treatment strategies for these lesions. METHODS: Treatment options include simple curettage with bone grafting, complete excision, embolization, and radiation therapy. Reconstruction and stabilization of the spine may be warranted if deformity and instability are present. Special factors need to be considered in the management of these lesions. CONCLUSIONS: Complete excision of aneurysmal bone cysts offers the best chance of cure and spinal decompression if neurological deficits are present.

Adolescent↗