39 results on '"Bristol, RE"'
Search Results
2. De Novo Variants Disrupting the HX Repeat Motif of ATN1 Cause a Recognizable Non-Progressive Neurocognitive Syndrome
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Palmer, EE ; https://orcid.org/0000-0003-1844-215X, Hong, S, Al Zahrani, F, Hashem, MO, Aleisa, FA, Ahmed, HMJ, Kandula, T ; https://orcid.org/0000-0002-4355-4965, Macintosh, R ; https://orcid.org/0000-0001-5036-0264, Minoche, AE, Puttick, C, Gayevskiy, V, Drew, AP, Cowley, MJ ; https://orcid.org/0000-0002-9519-5714, Dinger, M ; https://orcid.org/0000-0003-4423-934X, Rosenfeld, JA, Xiao, R, Cho, MT, Yakubu, SF, Henderson, LB, Guillen Sacoto, MJ, Begtrup, A, Hamad, M, Shinawi, M, Andrews, MV, Jones, MC, Lindstrom, K, Bristol, RE, Kayani, S, Snyder, M, Villanueva, MM, Schteinschnaider, A, Faivre, L, Thauvin, C, Vitobello, A, Roscioli, T ; https://orcid.org/0000-0003-1502-5000, Kirk, EP ; https://orcid.org/0000-0002-4662-0024, Bye, A, Merzaban, J, Jaremko, Ł, Jaremko, M, Sachdev, RK, Alkuraya, FS, Arold, ST, Palmer, Elizabeth ; https://orcid.org/0000-0003-1844-215X, Palmer, EE ; https://orcid.org/0000-0003-1844-215X, Hong, S, Al Zahrani, F, Hashem, MO, Aleisa, FA, Ahmed, HMJ, Kandula, T ; https://orcid.org/0000-0002-4355-4965, Macintosh, R ; https://orcid.org/0000-0001-5036-0264, Minoche, AE, Puttick, C, Gayevskiy, V, Drew, AP, Cowley, MJ ; https://orcid.org/0000-0002-9519-5714, Dinger, M ; https://orcid.org/0000-0003-4423-934X, Rosenfeld, JA, Xiao, R, Cho, MT, Yakubu, SF, Henderson, LB, Guillen Sacoto, MJ, Begtrup, A, Hamad, M, Shinawi, M, Andrews, MV, Jones, MC, Lindstrom, K, Bristol, RE, Kayani, S, Snyder, M, Villanueva, MM, Schteinschnaider, A, Faivre, L, Thauvin, C, Vitobello, A, Roscioli, T ; https://orcid.org/0000-0003-1502-5000, Kirk, EP ; https://orcid.org/0000-0002-4662-0024, Bye, A, Merzaban, J, Jaremko, Ł, Jaremko, M, Sachdev, RK, Alkuraya, FS, Arold, ST, and Palmer, Elizabeth ; https://orcid.org/0000-0003-1844-215X
- Abstract
Polyglutamine expansions in the transcriptional co-repressor Atrophin-1, encoded by ATN1, cause the neurodegenerative condition dentatorubral-pallidoluysian atrophy (DRPLA) via a proposed novel toxic gain of function. We present detailed phenotypic information on eight unrelated individuals who have de novo missense and insertion variants within a conserved 16-amino-acid “HX repeat” motif of ATN1. Each of the affected individuals has severe cognitive impairment and hypotonia, a recognizable facial gestalt, and variable congenital anomalies. However, they lack the progressive symptoms typical of DRPLA neurodegeneration. To distinguish this subset of affected individuals from the DRPLA diagnosis, we suggest using the term CHEDDA (congenital hypotonia, epilepsy, developmental delay, digit abnormalities) to classify the condition. CHEDDA-related variants alter the particular structural features of the HX repeat motif, suggesting that CHEDDA results from perturbation of the structural and functional integrity of the HX repeat. We found several non-homologous human genes containing similar motifs of eight to 10 HX repeat sequences, including RERE, where disruptive variants in this motif have also been linked to a separate condition that causes neurocognitive and congenital anomalies. These findings suggest that perturbation of the HX motif might explain other Mendelian human conditions.
- Published
- 2019
3. Non-uniformity of contact stress on polyethylene inserts in total knee arthroplasty
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Bristol, RE, primary, Fitzpatrick, DC, additional, Brown, TD, additional, and Callaghan, JJ, additional
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- 1996
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4. Preoperative Frontal and Parietal Bone Thickness Assessment to Predict Blood Loss and Transfusion During Extended Suturectomy for Isolated Sagittal Craniosynostosis.
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Grove AM, Kirsch HM, Kurnik NM, Bristol RE, Sitzman TJ, Pfeifer C, and Singh DJ
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- Humans, Male, Retrospective Studies, Female, Infant, Osteotomy methods, Cranial Sutures diagnostic imaging, Craniosynostoses surgery, Craniosynostoses diagnostic imaging, Blood Transfusion, Blood Loss, Surgical, Parietal Bone diagnostic imaging, Frontal Bone diagnostic imaging, Tomography, X-Ray Computed
- Abstract
Objective: To predict the morbidity of sagittal suturectomy using preoperative computer tomographic measurement of frontal and parietal bone thickness in osteotomy sites., Design: Retrospective analysis., Setting: Tertiary children's hospital., Patients: Fifty infants with nonsyndromic, isolated sagittal craniosynostosis who underwent extended sagittal suturectomy from 2015-2022., Methods: Mean thickness of the frontal and parietal bone in regions of osteotomies were determined for each patient from preoperative CT images obtained within 30 days prior to suturectomy. The relationship between bone thickness (mm) and estimated blood loss (mL) was evaluated using Spearman's correlation and a multivariable model that adjusted for patient weight and surgery duration. The association between bone thickness and perioperative blood transfusion was evaluated using a multivariable logistic model controlling for patient weight and surgery duration., Main Outcome Measures: Estimated blood loss, perioperative blood transfusion., Results: Frontal and parietal bone thickness in the region of osteotomies were positively correlated with estimated blood loss (p < 0.01). After adjusting for patient weight and duration of operation, both parietal and frontal bone thickness were associated with intraoperative blood loss (R
2 = 0.292, p = 0.002 and R2 = 0.216, p = 0.026). Thicker frontal and parietal bone in the line of osteotomies resulted in significantly higher odds of blood transfusion. Bone thickness in the line of parietal osteotomies was 76% accurate at identifying patients who would require blood transfusion (p = 0.004)., Conclusions: Frontal and parietal bone thickness in the line of osteotomies is associated with blood loss and perioperative blood transfusion for sagittal suturectomy operations., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.- Published
- 2025
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5. Meningomyelocele Reconstruction: Comparison of Repair Methods via Systematic Review.
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Leach GA, Pflibsen LR, Roberts AD, O'Connor MJ, Bristol RE, Mabee MK, Almader-Douglas D, and Schaub TA
- Abstract
Purpose: The purpose of this article was to appraise the various methods of reconstruction for meningomyelocele (MMC) defects., Methods: A systematic review of the literature was performed to evaluate all reconstructions for MMC. The method of reconstruction was categorized by: primary closure with and without fascial flaps, random pattern flaps, VY advancement flaps (VY), perforator flaps, and myocutaneous flaps. Perforator flaps were subsequently subcategorized based on the type of flap., Results: Upon systematic review, 567 articles were screened with 104 articles assessed for eligibility. Twenty-nine articles were further reviewed and included for qualitative synthesis. Two hundred seventy patients underwent MMC repair. The lowest rates of major wound complications (MWC) were associated with myocutaneous and random pattern flaps. A majority of MWC was in the lumbrosacral/sacral region (87.5% of MWC). In this region, random patterns and perforator flaps demonstrated the lowest rate of MWC (4.5, 8.1%)., Conclusions: Plastic surgery consultation should be strongly considered for MMC with defects in the lumbosacral/sacral region. Perforator flaps are excellent options for the reconstruction of these defects., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)
- Published
- 2023
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6. Long term outcome after surgical treatment for hypothalamic hamartoma.
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Kulkarni N, Nageotte RA, Klamer BG, Rekate HL, Bristol RE, Scharnweber T, Bobrowitz M, and Kerrigan JF
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- Humans, Treatment Outcome, Magnetic Resonance Imaging, Sudden Unexpected Death in Epilepsy, Hypothalamic Diseases complications, Hypothalamic Diseases surgery, Epilepsy etiology, Hamartoma complications, Hamartoma surgery
- Abstract
Purpose: To determine long-term outcome for seizure control and clinical predictors for seizure freedom in patients undergoing surgical treatment for epilepsy associated with hypothalamic hamartoma (HH)., Methods: 155 patients underwent surgical treatment for HHs and treatment-resistant epilepsy at one center (Barrow Neurological Institute at St. Joseph's Hospital and Medical Center, Phoenix, Arizona, USA) between February 2003 and June 2010. Data collection included medical record review and direct follow-up interviews to determine seizure outcome. Statistical analysis included descriptive summaries of patient characteristics and time-to-event analysis for seizure freedom., Results: Long-term survival with follow-up of at least five years since first surgical treatment was available for 108 patients (69.7% of the treatment cohort). The surgical approach for first HH intervention consisted of transventricular endoscopic resection (n = 57; 52.8%), transcallosal interforniceal resection (n = 35; 32.4%), pterional resection (n = 7; 6.5%), and gamma knife radiosurgery (n = 9; 8.3%). Multiple surgical procedures were required for 39 patients (36.1%). There were 10 known deaths from all causes in the treatment cohort (6.5%). Of these, one (0.6%) was related to immediate complications of HH surgery, three (1.9%) were attributed to Sudden Unexpected Death in Epileptic Persons (SUDEP), and one (0.6%) to complications of status epilepticus. For surviving patients with long-term follow-up, 55 (50.9%) were seizure-free for all seizure types. Univariable analysis showed that seizure-freedom was related to 1) absence of a pre-operative history for central precocious puberty (p = 0.01), and 2) higher percentage of HH lesion disconnection after surgery (p = 0.047). Kaplan-Meier survival analysis shows that long-term seizure outcome following HH surgery is comparable to short-term results., Summary: These uncontrolled observational results show that long-term seizure control following HH surgical treatment is comparable to other forms of epilepsy surgery. Late relapse (at least one year after surgery) and SUDEP do occur, but in a relatively small number of treated patients. These results inform clinical practice and serve as a comparable benchmark for newer technologies for HH surgery, such as magnetic resonance imaging-guided laser interstitial thermal therapy, where long-term outcome results are not yet available., (Copyright © 2023 Elsevier B.V. All rights reserved.)
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- 2023
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7. Hydrocephalus surveillance following CSF diversion: a modified Delphi study.
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Hersh DS, Martin JE, Bristol RE, Browd SR, Grant G, Gupta N, Hankinson TC, Jackson EM, Kestle JRW, Krieger MD, Kulkarni AV, Madura CJ, Pindrik J, Pollack IF, Raskin JS, Riva-Cambrin J, Rozzelle CJ, Smith JL, and Wellons JC
- Abstract
Objective: Long-term follow-up is often recommended for patients with hydrocephalus, but the frequency of clinical follow-up, timing and modality of imaging, and duration of surveillance have not been clearly defined. Here, the authors used the modified Delphi method to identify areas of consensus regarding the modality, frequency, and duration of hydrocephalus surveillance following surgical treatment., Methods: Pediatric neurosurgeons serving as institutional liaisons to the Hydrocephalus Clinical Research Network (HCRN), or its implementation/quality improvement arm (HCRNq), were invited to participate in this modified Delphi study. Thirty-seven consensus statements were generated and distributed via an anonymous electronic survey, with responses structured as a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A subsequent, virtual meeting offered the opportunity for open discussion and modification of the statements in an effort to reach consensus (defined as ≥ 80% agreement or disagreement)., Results: Nineteen pediatric neurosurgeons participated in the first round, after which 15 statements reached consensus. During the second round, 14 participants met virtually for review and discussion. Some statements were modified and 2 statements were combined, resulting in a total of 36 statements. At the conclusion of the session, consensus was achieved for 17 statements regarding the following: 1) the role of standardization; 2) preferred imaging modalities; 3) postoperative follow-up after shunt surgery (subdivided into immediate postoperative imaging, delayed postoperative imaging, routine clinical surveillance, and routine radiological surveillance); and 4) postoperative follow-up after an endoscopic third ventriculostomy. Consensus could not be achieved for 19 statements., Conclusions: Using the modified Delphi method, 17 consensus statements were developed with respect to both clinical and radiological follow-up after a shunt or endoscopic third ventriculostomy. The frequency, modality, and duration of surveillance were addressed, highlighting areas in which no clear data exist to guide clinical practice. Although further studies are needed to evaluate the clinical utility and cost-effectiveness of hydrocephalus surveillance, the current study provides a framework to guide future efforts to develop standardized clinical protocols for the postoperative surveillance of patients with hydrocephalus. Ultimately, the standardization of hydrocephalus surveillance has the potential to improve patient care as well as optimize the use of healthcare resources.
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- 2022
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8. Hydrogel Check-Valves for the Treatment of Hydrocephalic Fluid Retention with Wireless Fully-Passive Sensor for the Intracranial Pressure Measurement.
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Lee S, Liu S, Bristol RE, Preul MC, and Blain Christen J
- Abstract
Hydrocephalus (HCP) is a neurological disease resulting from the disruption of the cerebrospinal fluid (CSF) drainage mechanism in the brain. Reliable draining of CSF is necessary to treat hydrocephalus. The current standard of care is an implantable shunt system. However, shunts have a high failure rate caused by mechanical malfunctions, obstructions, infection, blockage, breakage, and over or under drainage. Such shunt failures can be difficult to diagnose due to nonspecific systems and the lack of long-term implantable pressure sensors. Herein, we present the evaluation of a fully realized and passive implantable valve made of hydrogel to restore CSF draining operations within the cranium. The valves are designed to achieve a non-zero cracking pressure and no reverse flow leakage by using hydrogel swelling. The valves were evaluated in a realistic fluidic environment with ex vivo CSF and brain tissue. They display a successful operation across a range of conditions, with negligible reverse flow leakage. Additionally, a novel wireless pressure sensor was incorporated alongside the valve for in situ intracranial pressure measurement. The wireless pressure sensor successfully replicated standard measurements. Those evaluations show the reproducibility of the valve and sensor functions and support the system's potential as a chronic implant to replace standard shunt systems.
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- 2022
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9. Accessory Cranial Suture Leading to Abnormal Head Shape.
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Leach GA, Kurnik NM, Bristol RE, and Singh DJ
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- Cranial Sutures diagnostic imaging, Cranial Sutures surgery, Humans, Infant, Parietal Bone diagnostic imaging, Parietal Bone surgery, Skull, Tomography, X-Ray Computed, Craniosynostoses diagnostic imaging, Craniosynostoses surgery, Skull Fractures
- Abstract
Abstract: Accessory cranial sutures have been described in the literature and are most commonly associated with the parietal bone. These sutures are typically identified incidentally and there have been no reported cases of accessory cranial sutures leading to abnormal head shape.The authors present the case of a 3-month-old patient with multiple congenital anomalies and an accessory parietal suture leading to abnormal head shape. The patient was successfully treated with cranial orthotic therapy. To our knowledge, this is the first reported case of an accessory cranial suture leading to abnormal head shape., Competing Interests: The authors report no conflicts of interest, (Copyright © 2020 by Mutaz B. Habal, MD.)
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- 2021
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10. Pediatric intracranial arterial injuries by penetrating gunshot wounds: an institutional experience.
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Catapano JS, Hutchens DM, Cadigan MS, Srinivasan VM, Albuquerque FC, and Bristol RE
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- Child, Glasgow Outcome Scale, Humans, Retrospective Studies, Tomography, X-Ray Computed, Firearms, Head Injuries, Penetrating diagnostic imaging, Wounds, Gunshot diagnostic imaging, Wounds, Penetrating
- Abstract
Purpose: Pediatric intracranial injuries due to penetrating gunshot wounds are a rare entity that is often fatal. A subset of patients may experience an intracerebral arterial injury; however, literature on the pediatric population is limited. This study analyzes a large institution's experience with pediatric head gunshot wounds and intracranial arterial injuries., Methods: All pediatric patients ≤ 18 years of age who presented to our institution with a penetrating gunshot wound from 2008 to 2018 were retrospectively analyzed., Results: Thirty-seven patients presented with an intracerebral penetrating gunshot injury. There were 18 deaths (49%) in the cohort. A total of 20 patients (54%) had vascular imaging. Of the remaining 17 patients with no vascular imaging, 13 (35%) died before any vascular studies were obtained. Four (20%) of the 20 patients with vascular imaging experienced an intracerebral arterial injury. Three of these 4 patients died before treatment could be administered. One patient with a firearm injury underwent embolization of a distal middle cerebral artery pseudoaneurysm and was discharged home with a Glasgow Outcome Scale score of 5 on follow-up., Conclusion: Pediatric patients with penetrating intracranial gunshot wounds often die before vascular imaging can be obtained.
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- 2021
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11. Three-Dimensionally Printed Microelectromechanical-System Hydrogel Valve for Communicating Hydrocephalus.
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Lee S, Bristol RE, Preul MC, and Chae J
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- Animals, Brain, Catheters, Printing, Three-Dimensional, Sheep, Hydrocephalus surgery, Hydrogels
- Abstract
Hydrocephalus (HCP) is a chronic neurological brain disorder caused by a malfunction of the cerebrospinal fluid (CSF) drainage mechanism in the brain. The current standard method to treat HCP is a shunt system. Unfortunately, the shunt system suffers from complications including mechanical malfunctions, obstructions, infections, blockage, breakage, overdrainage, and/or underdrainage. Some of these complications may be attributed to the shunts' physically large and lengthy course making them susceptible to external forces, siphoning effects, and risks of infection. Additionally, intracranial catheters artificially traverse the brain and drain the ventricle rather than the subarachnoid space. We report a 3D-printed microelectromechanical system-based implantable valve to improve HCP treatment. This device provides an alternative approach targeting restoration of near-natural CSF dynamics by artificial arachnoid granulations (AGs), natural components for CSF drainage in the brain. The valve, made of hydrogel, aims to regulate the CSF flow between the subarachnoid space and the superior sagittal sinus, in essence, substituting for the obstructed arachnoid granulations. The valve, operating in a fully passive manner, utilizes the hydrogel swelling feature to create nonzero cracking pressure, P
T ≈ 47.4 ± 6.8 mmH2 O, as well as minimize reverse flow leakage, QO ≈ 0.7 μL/min on benchtop experiments. The additional measurements performed in realistic experimental setups using a fixed sheep brain also deliver comparable results, PT ≈ 113.0 ± 9.8 mmH2 O and QO ≈ 3.7 μL/min. In automated loop functional tests, the valve maintains functionality for a maximum of 1536 cycles with the PT variance of 44.5 mmH2 O < PT < 61.1 mmH2 O and negligible average reverse flow leakage rates of ∼0.3 μL/min.- Published
- 2020
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12. De Novo Variants Disrupting the HX Repeat Motif of ATN1 Cause a Recognizable Non-progressive Neurocognitive Syndrome.
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Palmer EE, Hong S, Al Zahrani F, Hashem MO, Aleisa FA, Jalal Ahmed HM, Kandula T, Macintosh R, Minoche AE, Puttick C, Gayevskiy V, Drew AP, Cowley MJ, Dinger M, Rosenfeld JA, Xiao R, Cho MT, Yakubu SF, Henderson LB, Guillen Sacoto MJ, Begtrup A, Hamad M, Shinawi M, Andrews MV, Jones MC, Lindstrom K, Bristol RE, Kayani S, Snyder M, Villanueva MM, Schteinschnaider A, Faivre L, Thauvin C, Vitobello A, Roscioli T, Kirk EP, Bye A, Merzaban J, Jaremko Ł, Jaremko M, Sachdev RK, Alkuraya FS, and Arold ST
- Published
- 2019
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13. Magnetic Resonance Imaging-Guided Laser Interstitial Thermal Therapy for the Treatment of Hypothalamic Hamartomas: A Retrospective Review.
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Xu DS, Chen T, Hlubek RJ, Bristol RE, Smith KA, Ponce FA, Kerrigan JF, and Nakaji P
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- Adolescent, Adult, Aged, Child, Child, Preschool, Female, Hamartoma complications, Humans, Hypothalamic Diseases complications, Laser Therapy adverse effects, Magnetic Resonance Imaging, Male, Middle Aged, Postoperative Complications epidemiology, Retrospective Studies, Seizures etiology, Seizures prevention & control, Treatment Outcome, Young Adult, Hamartoma surgery, Hypothalamic Diseases surgery, Laser Therapy methods, Surgery, Computer-Assisted methods
- Abstract
Background: Hypothalamic hamartomas (HH) are rare lesions associated with treatment-resistant epilepsy. Open surgery results in modest seizure control (about 50%) but has a significant associated morbidity. Radiosurgery is limited to a subset of patients due to latent therapeutic effects. Magnetic resonance imaging-guided laser interstitial thermal therapy (LITT) offers a novel minimally invasive option., Objective: To evaluate a single center's outcomes for the LITT treatment of HH., Methods: We retrospectively reviewed our experience with LITT for the treatment of HH using our institution's prospectively maintained patient database., Results: Eighteen patients (mean age, 21.1 yr; median age, 11 yr) underwent 21 total LITT treatments for HH. Mean follow-up was 17.4 mo. The length of stay was 1 night for 16 (89%) patients. At the end of follow-up, 11 of 18 patients (61%) had full disconnection of the HH, and 12 of 15 (80%) patients with gelastic seizures and 5 (56%) of 9 patients with nongelastic seizures were seizure free (International League Against Epilepsy Class 1). Immediate complications included a 39% (7/18) incidence of neurological deficits, including 1 case of hemiparesis. At the end of follow-up, 22% of patients (4/18) had persistent deficits. The hypothyroidism that occurred was delayed in 11% of patients (2/18), as was short-term memory loss (22%, 4/18) and weight gain (22%, 4/18)., Conclusion: LITT therapy for HH can achieve excellent rates of seizure control with low morbidity and a short postoperative stay in a majority of patients. Additional research is needed to assess the durability of results and the full spectrum of cognitive outcomes.
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- 2018
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14. Outcomes of Titanium Mesh Cranioplasty in Pediatric Patients.
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Ma IT, Symon MR, Bristol RE, Beals SP, Joganic EF, Adelson PD, Shafron DH, and Singh DJ
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- Adolescent, Bone Transplantation methods, Child, Child, Preschool, Female, Humans, Infant, Male, Outcome and Process Assessment, Health Care, Plastic Surgery Procedures adverse effects, Plastic Surgery Procedures instrumentation, Plastic Surgery Procedures methods, Retrospective Studies, United States, Craniocerebral Trauma surgery, Craniofacial Abnormalities surgery, Craniotomy adverse effects, Craniotomy instrumentation, Craniotomy methods, Postoperative Complications etiology, Postoperative Complications surgery, Skull surgery, Surgical Mesh, Titanium therapeutic use
- Abstract
Purpose: Cranial defects in children have been repaired with various materials ranging from autologous bone to synthetic materials. There is little published literature on the outcomes of titanium mesh cranioplasty (TMC) in calvarial reconstruction in the pediatric population. This study evaluates a pediatric cohort who underwent calvarial defect reconstruction with titanium mesh and assesses the efficacy and outcomes of TMC., Methods: An Institutional Review Board approved retrospective review of patients ≤18 years of age who underwent cranioplasty from 1999 to 2014 at 2 centers was performed. The cohort undergoing TMC was studied., Results: A total of 159 cranioplasties were performed. Autologous reconstruction included 84 bone flap replacements and 36 split calvarial bone graft reconstructions. Six patients underwent PEEK implant reconstruction. Titanium mesh cranioplasty was performed on 33 patients. Two patients underwent 2 separate cranioplasties. The median age of patients was 6 years (19 months to 18 years). The most common underlying etiologies were congenital syndromes/craniosynostosis (13 patients), and trauma (11). The majority of patients had prior cranial surgeries (85%). Various types of titanium mesh were used with sizes ranging from 2×3 cm to 19×20 cm, with some patients requiring distinct areas of defect reconstruction. Perioperative complications were noted in 2 patients that subsequently improved. Two patients had late soft tissue problems with complications of wound infections requiring resection of a portion of the mesh. Patients were followed an average of 4 years (range 13 days to 6.8 years), with 2 patients lost to follow-up. Overall, all patients with follow-up achieved a cranial contour with good symmetry to the unaffected side, as well as effective protection to the brain., Conclusions: Titanium mesh cranioplasty is an effective option for correcting pediatric cranial defects when autologous bone availability is limited and soft tissue coverage allows placement of an implant. The interim outcome for these patients is favorable with few complications and no evidence of growth restriction in the authors' series. Follow-up will be ongoing for these patients.
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- 2018
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15. Tranexamic Acid Reduces Blood Loss in Craniosynostosis Surgery.
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Kurnik NM, Pflibsen LR, Bristol RE, and Singh DJ
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- Blood Transfusion, Critical Care, Humans, Infant, Length of Stay, Retrospective Studies, Treatment Outcome, Antifibrinolytic Agents therapeutic use, Blood Loss, Surgical prevention & control, Craniosynostoses surgery, Tranexamic Acid therapeutic use
- Abstract
Methods: The authors retrospectively reviewed 79 patients with craniosynostosis who underwent either bifrontal or biparietal-occipital calvarial vault remodeling before institution of tranexamic acid (TXA) administration (non-TXA group) and 35 patients after institution of TXA administration (TXA group). The patients were analyzed in 2 groups: all open calvarial vault remodeling patients (anterior and posterior) as a whole, and anterior vaults only as a subset analysis. Primary outcomes accessed were: total intraoperative blood transfused, postoperative blood transfused, and estimated blood loss (EBL). Secondary outcomes evaluated were length of stay in the pediatric intensive care unit and hospital length of stay., Results: When comparing all open calvarial vault remodeling patients, patients who received TXA required significantly less total blood transfusion during their operation (264 cc TXA group versus 428 cc non-TXA, P < 0.0001). Patients who received TXA required no blood transfusions postoperatively, compared with the non-TXA group, in which 45% of patients required postoperative blood transfusion. Weight-based EBL was also significantly lower in those patients receiving TXA (25 cc/kg in the TXA group versus 34 cc/kg in the non-TXA group [P = 0.0143]). All patients required transfusion intraoperatively. Pediatric intensive care unit length of stay was shorter in the TXA group, but there was no significant difference in total hospital length of stay. These findings also reached statistical significance when comparing only the anterior vault patients., Conclusion: Intraoperative TXA administration has a correlation with reduced blood transfusion requirements, as well as EBL, in patients undergoing open calvarial vault remodeling. There were no adverse events related to TXA administration.
- Published
- 2017
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16. Current Trends in Craniosynostosis.
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Bristol RE
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- Evidence-Based Medicine, Humans, Treatment Outcome, Craniosynostoses diagnosis, Craniosynostoses surgery, Neurosurgical Procedures trends, Plastic Surgery Procedures trends
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- 2016
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17. In vitro hydrodynamic, transient, and overtime performance of a miniaturized valve for hydrocephalus.
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Schwerdt HN, Amjad U, Appel J, Elhadi AM, Lei T, Preul MC, Bristol RE, and Chae J
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- Cerebrospinal Fluid, Equipment Design, Humans, Hydrodynamics, Intracranial Pressure, Hydrocephalus therapy, Hydrogels therapeutic use, Miniaturization instrumentation
- Abstract
Reliable cerebrospinal fluid (CSF) draining methods are needed to treat hydrocephalus, a chronic debilitating brain disorder. Current shunt implant treatments are characterized by high failure rates that are to some extent attributed to their length and multiple components. The designed valve, made of hydrogel, steers away from such protracted schemes and intends to provide a direct substitute for faulty arachnoid granulations, the brain's natural CSF draining valves, and restore CSF draining operations within the cranium. The valve relies on innate hydrogel swelling phenomena to strengthen reverse flow sealing at idle and negative pressures thereby alleviating common valve failure mechanisms. In vitro measurements display operation in range of natural CSF draining (cracking pressure, PT ~ 1-110 mmH2O and outflow hydraulic resistance, Rh ~ 24-152 mmH2O/mL/min), with negligible reverse flow leakage (flow, QO > -10 µL/min). Hydrodynamic measurements and over-time tests under physically relevant conditions further demonstrate the valve's operationally-reproducible properties and strengthen its validity for use as a chronic implant.
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- 2015
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18. The rocking chair.
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Bristol RE
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- Brain diagnostic imaging, Extracorporeal Membrane Oxygenation, Humans, Infant, Newborn, Male, Respiratory Distress Syndrome, Newborn diagnostic imaging, Tomography, X-Ray Computed, Respiratory Distress Syndrome, Newborn complications
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- 2014
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19. Transvenous to arterial Onyx embolization.
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Albuquerque FC, Ducruet AF, Crowley RW, Bristol RE, Ahmed A, and McDougall CG
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- Aged, Carotid Artery, Common abnormalities, Carotid Artery, Common diagnostic imaging, Carotid Artery, Internal abnormalities, Carotid Artery, Internal diagnostic imaging, Central Nervous System Vascular Malformations diagnostic imaging, Cerebral Angiography, Female, Humans, Infant, Vein of Galen Malformations therapy, Central Nervous System Vascular Malformations therapy, Embolization, Therapeutic methods
- Abstract
Background/objective: Transarterial infusion of Onyx is now widely used in the treatment of arteriovenous malformations (AVMs) and dural arteriovenous fistulas (DAVFs). Transvenous Onyx embolization is rarely performed, and usually in cases of carotid cavernous fistulas. We describe a novel technique of transvenous Onyx embolization in the treatment of three high risk DAVFs and one vein of Galen (VOG) AVM., Methods: Cases were evaluated with specific attention to technical caveats, clinical indications, and complications. Patients were treated during 2011-2012 by two operators (FCA and CGM). Standard transfemoral venous approaches were employed in order to obtain access into the draining venous system in all cases., Results: Four female patients, aged 5 months to 68 years, were treated. Two patients, one presenting with headache and the other with a bruit, demonstrated high risk DAVFs involving the transverse-sigmoid sinuses. One patient, who was asymptomatic, manifested a high risk ethmoidal DAVF that was discovered incidentally during treatment of a basilar aneurysm. The final patient presented with symptoms of a pineal region mass and was found to have a VOG AVM. In all cases, a microcatheter was navigated through the draining venous pouch and into the ostium of an arterial feeder. A single Onyx infusion from this position filled multiple arterial feeders and resulted in cures in the first three patients and a significant reduction in AVM nidus in the fourth patient. No complications occurred., Conclusions: Transvenous Onyx infusion into a single arterial feeder can produce retrograde embolization of numerous other arterial feeders supplying high risk DAVFs and complex cerebral AVMs. This technique appears to be safe and potentially curative in select cases.
- Published
- 2014
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20. Miniaturized passive hydrogel check valve for hydrocephalus treatment.
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Schwerdt HN, Bristol RE, and Junseok Chae
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- Equipment Design, Humans, Infant, Monitoring, Physiologic methods, Hydrocephalus physiopathology, Hydrocephalus therapy, Hydrogel, Polyethylene Glycol Dimethacrylate therapeutic use, Intracranial Pressure physiology, Microtechnology instrumentation, Monitoring, Physiologic instrumentation
- Abstract
Improvements in cerebrospinal fluid (CSF) draining techniques for treatment of hydrocephalus are urgently sought after to substitute for current CSF shunts that are plagued by high failure rates. The passive check valve aims to restore near natural CSF draining operations while mitigating possible failure mechanisms caused by finite leakage or low resilience that frequently constrain practical implementation of miniaturized valves. A simple hydrogel diaphragm structures core passive valve operations and enforce valve sealing properties to substantially lower reverse flow leakage. Experimental measurements demonstrate realization of targeted cracking pressures (PT ≈ 20-110 mmH2O) and operation at -800 <; ΔP <; 600 mmH2O without observable degradation or leakage.
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- 2014
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21. Surgical and endovascular treatment of pediatric spinal arteriovenous malformations.
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Kalani MY, Ahmed AS, Martirosyan NL, Cronk K, Moon K, Albuquerque FC, McDougall CG, Spetzler RF, and Bristol RE
- Subjects
- Adolescent, Age Factors, Arteriovenous Malformations pathology, Arteriovenous Malformations physiopathology, Child, Child, Preschool, Endovascular Procedures statistics & numerical data, Female, Humans, Klippel-Trenaunay-Weber Syndrome pathology, Klippel-Trenaunay-Weber Syndrome physiopathology, Klippel-Trenaunay-Weber Syndrome surgery, Male, Paraparesis etiology, Paraparesis physiopathology, Paraparesis surgery, Pediatrics methods, Postoperative Complications etiology, Postoperative Complications physiopathology, Postoperative Complications prevention & control, Process Assessment, Health Care methods, Retrospective Studies, Secondary Prevention, Spinal Cord blood supply, Spinal Cord pathology, Spinal Cord surgery, Spinal Cord Compression pathology, Spinal Cord Compression physiopathology, Spinal Cord Compression surgery, Spinal Cord Diseases pathology, Spinal Cord Diseases physiopathology, Subarachnoid Hemorrhage etiology, Subarachnoid Hemorrhage physiopathology, Subarachnoid Hemorrhage surgery, Subarachnoid Space pathology, Subarachnoid Space surgery, Treatment Outcome, Arteriovenous Malformations surgery, Endovascular Procedures methods, Neurosurgical Procedures methods, Spinal Cord Diseases surgery
- Abstract
Objective: Pediatric spinal arteriovenous malformations (AVMs) are rare and complex lesions to treat. There are few reports of the endovascular and microsurgical treatment of these lesions in the pediatric population, and the treatment outcomes of these patients are not well described. The aim of this study was the clinical and radiographic outcomes of spinal AVMs in pediatric patients treated via endovascular and microsurgical modalities., Methods: We identified nine children (5 boys, 4 girls; average age 11 years, range 3-17 years) treated for spinal AVMs between 1998 and 2010. Their charts were reviewed., Results: Spinal AVMs most frequently involved the thoracic spinal cord. Four patients had associated Klippel-Trènaunay-Weber syndrome and one had hereditary hemorrhagic telangiectasia. There were two intramedullary, four conus medullaris, and three mixed extradural-intradural lesions. The most common presenting signs and symptoms were subarachnoid hemorrhage (n = 3) and paraparesis (n = 5). Endovascular intervention was used exclusively in two patients, and combined endovascular and microsurgical intervention was used in four patients. Surgery was the sole treatment in three patients with excellent results. There were two treatment-related complications: one case of subarachnoid hemorrhage and one case of scrotal swelling. The mean follow-up was 28.5 months and the median was 8 months (range, 1-65 months). The mean pretreatment World Health Organization (WHO)/Zubrod score was 2.4 (range, 1-4), and the mean post-treatment score was 1.4 (range, 0-4). One patient (11%) had a recurrence., Conclusions: Pediatric spinal AVMs require complex combined microsurgical and endovascular techniques to achieve favorable outcomes., (Copyright © 2012 Elsevier Inc. All rights reserved.)
- Published
- 2012
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22. Evaluation of operative procedures for symptomatic outcome after decompression surgery for Chiari type I malformation.
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Kalb S, Perez-Orribo L, Mahan M, Theodore N, Nakaji P, and Bristol RE
- Subjects
- Adolescent, Adult, Aged, Arachnoid surgery, Child, Child, Preschool, Craniotomy, Female, Fourth Ventricle surgery, Humans, Infant, Laminectomy, Magnetic Resonance Imaging, Male, Middle Aged, Nervous System Diseases etiology, Retrospective Studies, Scoliosis complications, Syringomyelia complications, Treatment Outcome, Wound Healing, Young Adult, Arnold-Chiari Malformation surgery, Decompression, Surgical methods, Neurosurgical Procedures methods
- Abstract
The wide spectrum of symptoms and radiographic findings in patients with Chiari I malformation makes the decision to proceed with intervention controversial. We evaluated symptomatic outcomes using diverse surgical techniques in 104 patients who underwent decompression surgery. The symptoms of most patients improved. Patients with syringomyelia showed less symptomatic improvement; however, syringomyelia was not associated with postoperative symptomatic worsening. Durotomy was performed in 97.1% and arachnoid opening was performed in 60.6% with visualization of the fourth ventricle in 51.9% of patients. Neither arachnoid opening nor fourth ventricle visualization was associated with the clinical outcome. Duraplasty was performed in 94.2% of patients. A Chiari plate was used in 13.4% of patients and was associated with favorable outcomes. Use of postoperative steroids or muscle relaxants was not associated with outcome. Syringomyelia showed a 62.5% improvement rate on postoperative MRI. In conclusion, bony decompression and dural opening are important aspects of Chiari I surgery, with symptomatic improvement observed in most patients., (Copyright © 2012. Published by Elsevier Ltd.)
- Published
- 2012
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23. Supracerebellar infratentorial endoscopically controlled resection of pineal lesions: case series and operative technique.
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Uschold T, Abla AA, Fusco D, Bristol RE, and Nakaji P
- Subjects
- Adolescent, Adult, Biopsy, Brain Neoplasms pathology, Cerebellum pathology, Child, Female, Follow-Up Studies, Humans, Male, Pineal Gland pathology, Retrospective Studies, Treatment Outcome, Young Adult, Brain Neoplasms surgery, Cerebellum surgery, Neuroendoscopy methods, Pineal Gland surgery
- Abstract
Object: The heterogeneous clinical manifestations and operative characteristics of pathological entities in the pineal region represent a significant challenge in terms of patient selection and surgical approach. Traditional surgical options have included endoscopic transventricular resection; open supratentorial microsurgical approaches through the midline, choroidal fissure, lateral ventricle, and tentorium; and supracerebellar infratentorial (SCIT) approaches through the posterior fossa. The object of the current study was to review the preoperative characteristics and outcomes for a cohort of patients treated purely via the novel endoscopically controlled SCIT approach., Methods: A single-institution series of 9 consecutive patients (4 male and 5 female patients [10 total cases]; mean age 21 years, range 6-37 years) treated via the endoscopically controlled SCIT approach for a pathological entity in the pineal region was retrospectively reviewed. The mean follow-up time was 13.2 months., Results: The endoscopically controlled SCIT approach was successfully used to approach a variety of pineal lesions, including pineal cysts (6 patients), epidermoid tumor, WHO Grade II astrocytoma (initial biopsy and recurrence), and malignant mixed germ cell tumor (1 patient each). Gross-total resection and/or adequate cyst fenestration was achieved in 8 cases. Biopsy with conservative debulking was performed for the single case of low-grade astrocytoma and again at the time of recurrence. The mean preoperative tumor and cyst volumes were 9.9 ± 4.4 and 3.7 ± 3.2 cm(3), respectively. The mean operating times were 212 ± 71 minutes for tumor cases and 177 ± 72 minutes for cysts. Estimated blood loss was less than 150 ml for all cases. A single case (pineal cyst) was converted to an open microsurgical approach to enhance visualization. There were no operative complications, as well as no documented CSF leaks, additional CSF diversion procedures, or air emboli. Seven patients underwent concomitant third ventriculostomy into the quadrigeminal cistern. At the time of the last follow-up evaluation, all patients had a stable or improved modified Rankin Scale score., Conclusions: The endoscopically controlled SCIT approach may be used for the biopsy and resection of appropriately selected solid tumors of the pineal region, in addition to the fenestration and/or resection of pineal cysts. Preoperative considerations include patient presentation, anticipated disease and vascularity, degree of local venous anatomical distortion, and selection of optimal paramedian trajectory.
- Published
- 2011
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24. Normally shaped heads with no sutures, normally shaped heads with abnormal sutures, and abnormally shaped heads with normal sutures.
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Bristol RE, Krieger MD, and McComb JG
- Subjects
- Cephalometry, Child, Female, Humans, Infant, Infant, Newborn, Male, Tomography, X-Ray Computed, Cranial Sutures abnormalities, Cranial Sutures diagnostic imaging, Craniosynostoses diagnostic imaging
- Abstract
Unlabelled: We present a series of children whose head shapes and suture status do not make sense. There were 3 patients with complete absence of sutures and normal head shapes. One patient was evaluated for microcephaly at 6 years of age. In the second, the absence of sutures was discovered on workup for headaches at 8 years of age. The third underwent x-ray imaging for parasagittal bony nodules at 1 month of age. The head circumferences fell at the 4th, 25th, and 50th percentiles, respectively. The 2 older children were in normal classes, and the youngest was meeting milestones appropriately. We encountered 3 patients with fusion of the sagittal suture and normal head shapes. One had a flattened occiput, the second patient was thought to be brachycephalic, and the third was macrocephalic. Head circumferences were at the fourth, 50th, and 75th percentiles. The patient with head circumference at the fourth percentile had fetal alcohol syndrome and speech delay. The other 2 were developing normally with follow-up of 14 months. Finally, 3 patients underwent surgery for characteristic craniosynostotic head shapes and were found to have patent sutures at surgery. One patient had classic unilateral coronal synostosis and a patent suture on the side of the defect. The second patient had Crouzon syndrome with characteristic head shape, but open coronal sutures. The third patient had the appearance of bicoronal synostosis, with a patent suture on one side. They have had good results from their craniofacial reconstructions at 24, 12, and 6 months' follow-up., Conclusions: We have no explanation for these interesting findings.
- Published
- 2011
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25. Low-grade glial tumors: are they all the same?
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Bristol RE
- Subjects
- Astrocytoma diagnosis, Astrocytoma therapy, Brain Neoplasms pathology, Chemotherapy, Adjuvant, Child, Ganglioglioma diagnosis, Ganglioglioma therapy, Glioma classification, Glioma pathology, Humans, Oligodendroglioma diagnosis, Oligodendroglioma therapy, Radiotherapy, Adjuvant, Treatment Outcome, Brain Neoplasms diagnosis, Brain Neoplasms therapy, Glioma diagnosis, Glioma therapy
- Abstract
The most common diagnosis for supratentorial brain tumors in children is a form of low-grade glioma. In addition to being a diagnosis on its own, this term has become a general category that encompasses many specific diagnoses. Advances in immunohistochemistry and pathology classification schemes have led to the recognition of these diverse pathologies. Even though the numbers of any given tumor type are small, the question has been raised as to whether different pathologies require different treatments. We reviewed the published articles on treatment and outcomes for all pathologies included under the heading "low-grade glioma" to answer this question. Once intervention is deemed necessary, attempted complete surgical resection remains the mainstay of treatment for all diagnoses. Surgically accessible recurrences are also best managed with repeat resection. Unresectable residuals or recurrences can be treated with adjuvant therapies. The only pathologic subgroups that may benefit from more aggressive up-front treatment are the grade II astrocytomas. Radiation is reserved for older children, and chemotherapy protocols are favored in younger children. Although the data from adult radiosurgical studies are promising, data for the pediatric population are not yet available. Many small lesions and small residuals remain unchanged for many years.
- Published
- 2009
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26. Treatment options for third ventricular colloid cysts: comparison of open microsurgical versus endoscopic resection.
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Horn EM, Feiz-Erfan I, Bristol RE, Lekovic GP, Goslar PW, Smith KA, Nakaji P, and Spetzler RF
- Abstract
Objective: We retrospectively reviewed our experience treating third ventricular colloid cysts to compare the efficacy of endoscopic and transcallosal approaches., Methods: Between September 1994 and March 2004, 55 patients underwent third ventricular colloid cyst resection. The transcallosal approach was used in 27 patients; the endoscopic approach was used in 28 patients. Age, sex, cyst diameter, and presence of hydrocephalus were similar between the two groups., Results: The operating time and hospital stay were significantly longer in the transcallosal craniotomy group compared with the endoscopic group. Both approaches led to reoperations in three patients. The endoscopic group had two subsequent craniotomies for residual cysts and one repeat endoscopic procedure because of equipment malfunction. The transcallosal craniotomy group had two reoperations for fractured drainage catheters and one operation for epidural hematoma evacuation. The transcallosal craniotomy group had a higher rate of patients requiring a ventriculoperitoneal shunt (five versus two) and a higher infection rate (five versus none). Intermediate follow-up demonstrated more small residual cysts in the endoscopic group than in the transcallosal craniotomy group (seven versus one). Overall neurological outcomes, however, were similar in the two groups., Conclusion: Compared with transcallosal craniotomy, neuroendoscopy is a safe and effective approach for removal of colloid cysts in the third ventricle. The endoscope can be considered a first-line treatment for these lesions, with the understanding that a small number of these patients may need an open craniotomy to remove residual cysts.
- Published
- 2008
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27. Treatment options for third ventricular colloid cysts: comparison of open microsurgical versus endoscopic resection.
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Horn EM, Feiz-Erfan I, Bristol RE, Lekovic GP, Goslar PW, Smith KA, Nakaji P, and Spetzler RF
- Subjects
- Corpus Callosum surgery, Female, Humans, Male, Middle Aged, Retrospective Studies, Treatment Outcome, Arachnoid Cysts surgery, Brain Diseases surgery, Endoscopy methods, Microsurgery methods, Neurosurgical Procedures methods, Third Ventricle surgery
- Abstract
Objective: We retrospectively reviewed our experience treating third ventricular colloid cysts to compare the efficacy of endoscopic and transcallosal approaches., Methods: Between September 1994 and March 2004, 55 patients underwent third ventricular colloid cyst resection. The transcallosal approach was used in 27 patients; the endoscopic approach was used in 28 patients. Age, sex, cyst diameter, and presence of hydrocephalus were similar between the two groups., Results: The operating time and hospital stay were significantly longer in the transcallosal craniotomy group compared with the endoscopic group. Both approaches led to reoperations in three patients. The endoscopic group had two subsequent craniotomies for residual cysts and one repeat endoscopic procedure because of equipment malfunction. The transcallosal craniotomy group had two reoperations for fractured drainage catheters and one operation for epidural hematoma evacuation. The transcallosal craniotomy group had a higher rate of patients requiring a ventriculoperitoneal shunt (five versus two) and a higher infection rate (five versus none). Intermediate follow-up demonstrated more small residual cysts in the endoscopic group than in the transcallosal craniotomy group (seven versus one). Overall neurological outcomes, however, were similar in the two groups., Conclusion: Compared with transcallosal craniotomy, neuroendoscopy is a safe and effective approach for removal of colloid cysts in the third ventricle. The endoscope can be considered a first-line treatment for these lesions, with the understanding that a small number of these patients may need an open craniotomy to remove residual cysts.
- Published
- 2007
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28. Segmental spinal dysgenesis: report of four cases and proposed management strategy.
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Bristol RE, Theodore N, and Rekate HL
- Subjects
- Abnormalities, Multiple diagnostic imaging, Abnormalities, Multiple surgery, Cervical Vertebrae abnormalities, Cervical Vertebrae diagnostic imaging, Cervical Vertebrae surgery, Child, Preschool, Female, Humans, Infant, Infant, Newborn, Lumbar Vertebrae abnormalities, Lumbar Vertebrae diagnostic imaging, Lumbar Vertebrae surgery, Magnetic Resonance Imaging, Male, Neural Tube Defects diagnostic imaging, Neural Tube Defects surgery, Spinal Fusion, Spine diagnostic imaging, Spine surgery, Thoracic Vertebrae abnormalities, Thoracic Vertebrae diagnostic imaging, Thoracic Vertebrae surgery, Tomography, X-Ray Computed, Treatment Outcome, Abnormalities, Multiple therapy, Neural Tube Defects therapy, Orthotic Devices, Spine abnormalities
- Abstract
Introduction: Segmental spinal dysgenesis, a rare developmental malformation, usually manifests during pregnancy or at birth. The resulting gross spinal instability necessitates spinal stabilization, which is inherently challenging in neonates., Methods: We report four cases of segmental dysgenesis: three in the thoracolumbar region and one at the cervicothoracic junction. The latter was maintained in a custom orthosis that restricted all craniospinal motion while allowing routine care. Two neonates underwent surgical stabilization. The fourth patient will remain in a brace until 12-14 months old when fusion is planned., Results: Fusion with rib autografts failed in the two neonates. One patient has been followed for 13 years and is paraplegic. The second patient was lost to follow up. The patient with the cervicothoracic dysgenesis maintained normal neurologic function until his death at 8 months of cardiac failure. The fourth patient is 12 months old and has been maintained in a thoracolumbar orthosis with stable neurologic function., Conclusion: Several factors contribute to the challenge of creating a stable fusion in neonates. Incomplete ossification of the vertebral bodies and poor results with allograft materials restrict fusion options. Neurologic deficits often prevent ambulation and decrease the axial-loading forces that enhance fusion. To allow children to grow and develop, we advocate rigid spinal immobilization for 12-18 months before spinal fusion (preferably, rib or fibular autograft). Given the already narrow spinal canal, the use of instrumentation is controversial. We advocate the use of instrumentation in infants only when a sound construct cannot be obtained with the graft alone.
- Published
- 2007
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29. Arachnoid cysts: spontaneous resolution distinct from traumatic rupture. Case report.
- Author
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Bristol RE, Albuquerque FC, McDougall C, and Spetzler RF
- Subjects
- Adolescent, Arachnoid Cysts diagnosis, Arachnoid Cysts surgery, Brain Stem surgery, Child, Preschool, Cranial Fossa, Middle surgery, Humans, Magnetic Resonance Imaging methods, Male, Rupture, Spontaneous, Tomography, X-Ray Computed methods, Arachnoid Cysts complications, Brain Stem injuries, Cranial Fossa, Middle injuries
- Abstract
Although many arachnoid cysts are discovered incidentally and require no intervention, a small subset has been known to rupture. Note that rupture can occur either spontaneously or in association with trauma. Based on a review of the literature on ruptured arachnoid cysts, it appears that patients with middle fossa cysts are more likely to experience symptomatic traumatic rupture than those with cysts in other locations. Middle fossa cysts are more commonly associated with hemispheric subdural collections and hematomas than are any other cysts. The authors report on two representative cases illustrating the distinct presentation, imaging characteristics, and management of these cysts.
- Published
- 2007
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30. The role of neurosurgery in status epilepticus.
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Ng YT, Bristol RE, Schrader DV, and Smith KA
- Subjects
- Astrocytoma complications, Astrocytoma surgery, Brain Neoplasms complications, Brain Neoplasms surgery, Humans, Male, Middle Aged, Status Epilepticus etiology, Neurosurgical Procedures methods, Status Epilepticus surgery
- Abstract
Introduction: Status epilepticus remains a life-threatening condition that afflicts both adults and children which although occurs in patients with epilepsy, often presents as new-onset seizure activity also. Refractory status epilepticus poses a management challenge for neurological and neurosurgical teams., Case Report and Methods: Subdural grid electrodes were used to record cortical discharges and guide tumor resection involving eloquent cortex and multiple subpial transections in a 48-year-old man with left hemiparesis in status epilepticus. He had been refractory to multiple medical therapies in persistent epilepsia partialis continua for a prolonged period. As an alternative to higher-dose suppressive medical therapy, the patient elected to proceed with subdural grid mapping after seizure semiology ("negative" scalp electroencephalogram) localized the seizure focus to the right hemisphere, motor cortex. Following tumor removal, multiple subpial transections were subsequently performed over large areas of the motor and sensory strips and successfully resolved the status epilepticus., Results: The patient made an excellent recovery, became seizure free, had improved left-sided strength and was discharged home shortly after., Conclusion: This case illustrates a potentially life-saving technique for the treatment of refractory status epilepticus. Multiple subpial transections and other neurosurgical intervention should be considered for patients with status epilepticus. When localization with surface electrodes is poor, especially in eloquent cortex, subdural grid recording can be used to direct focal resection and/or multiple subpial transections to minimize neurological deficits. A review and summary of previously published neurosurgery cases for status epilepticus is discussed.
- Published
- 2007
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31. Invasive intracerebral schwannoma mimicking meningioma in a child.
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Bristol RE, Coons SW, Rekate HL, and Spetzler RF
- Subjects
- Child, Humans, Magnetic Resonance Imaging, Male, Tomography, X-Ray Computed, Brain Neoplasms pathology, Meningeal Neoplasms pathology, Meningioma pathology, Neurilemmoma diagnosis
- Abstract
Case Report: An 8-year-old boy presented to the emergency department after a generalized tonic-clonic seizure that lasted for 5 min. Magnetic resonance imaging showed a 2x2 cm, intraaxial, contrast-enhanced cortical lesion in the posterior right frontal lobe. On several images the lesion appeared to be dural-based and was presumed to be a meningioma. The patient was placed on dilantin and returned 1 month later for elective surgical resection., Outcome: At surgery, a rim of intact pia was identified between the dura and the tumor. Although initial frozen-section analysis was consistent with meningioma, subsequent immunohistochemical staining and review at an outside institution established the diagnosis of intracerebral schwannoma. The patient's postoperative course was uncomplicated and he remains seizure-free with no sign of recurrence at 18 months., Conclusion: Intracerebral schwannomas are uncommon cortical lesions in children. Imaging characteristics alone can be misleading; neuropathological support is essential for accurate diagnosis.
- Published
- 2006
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32. Spinal cord compression from traumatic anterior cervical pseudomeningoceles. Report of three cases.
- Author
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Horn EM, Bristol RE, Feiz-Erfan I, Beres EJ, Bambakidis NC, and Theodore N
- Subjects
- Adult, Humans, Male, Meningocele therapy, Spinal Cord Compression therapy, Cervical Vertebrae injuries, Meningocele diagnosis, Meningocele etiology, Spinal Cord Compression diagnosis, Spinal Cord Compression etiology
- Abstract
Pseudomeningoceles rarely develop after cervical trauma; in all reported cases the lesions have extended outside the spinal canal. The authors report the first known cases of anterior cervical pseudomeningoceles contained entirely within the spinal canal and causing cord compression and neurological injury. The authors retrospectively reviewed the cases of three patients with traumatic cervical spine injuries and concomitant compressive anterior pseudomeningoceles. The lesion was recognized in the first case when the patient's neurological status declined after he sustained a severe atlantoaxial injury; the pseudomeningocele was identified intraoperatively and decompressed. After the decompressive surgery, the patient's severe tetraparesis partially resolved. In the other two patients diagnoses of similar pseudomeningoceles were established by magnetic resonance imaging. Both patients were treated conservatively, and their mild to moderate hemiparesis due to the pseudomeningocele-induced compression abated. The high incidence of anterior cervical pseudomeningoceles seen at the authors' institution within a relatively brief period suggests that this lesion is not rare. The authors believe that it is important to recognize the compressive nature of these lesions and their potential to cause devastating neurological injury.
- Published
- 2006
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33. Surgical management of arteriovenous malformations in children.
- Author
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Bristol RE, Albuquerque FC, Spetzler RF, Rekate HL, McDougall CG, and Zabramski JM
- Subjects
- Cerebral Hemorrhage mortality, Child, Craniotomy, Embolization, Therapeutic, Follow-Up Studies, Humans, Intracranial Arteriovenous Malformations mortality, Postoperative Complications, Radiosurgery, Recurrence, Stroke mortality, Stroke surgery, Stroke therapy, Treatment Outcome, Cerebral Hemorrhage surgery, Cerebral Hemorrhage therapy, Intracranial Arteriovenous Malformations surgery, Intracranial Arteriovenous Malformations therapy
- Abstract
Object: Children compose 3 to 20% of the patients with arteriovenous malformations (AVMs); however, AVMs are responsible for 30 to 50% of intracranial hemorrhages in children., Methods: The medical records of 82 children with 84 AVMs treated surgically between 1983 and 2005 were reviewed. Fifty-two patients (63%) presented with hemorrhage, 13.4% presented with seizures, and AVMs in 12% were found incidentally. Patients with brainstem lesions presented at a significantly younger age (p = 0.002) than those harboring lesions in other locations. Frontal lobe lesions were significantly smaller than those in other locations, and thalamic lesions were significantly larger (p = 0.012 and 0.005, respectively). Most patients with Spetzler-Martin Grades I to III lesions underwent craniotomy only. Half of the patients with Grade IV and V lesions underwent embolization, craniotomy, and radiosurgery. The mean follow-up period was 43 months. Postoperatively, the initial obliteration rate was 65%, with a long-term obliteration rate of 90%. The perioperative mortality rate was 3.7%. Altogether, 81% of patients had excellent outcomes, and patients with Grade I lesions had the best outcomes. Of the 52 patients who presented with hemorrhage, 17% had fair or poor outcomes. The recurrence rate was 5.6%., Conclusions: Children with AVMs may be more prone than adults to present with a hemorrhage and to experience recurrence of the lesion after treatment. The authors favor resection for most AVMs in children and use embolization as a preoperative strategy for Grades II to V lesions treated surgically. Prehemorrhagic Grade IV and V lesions may best be treated conservatively and observed carefully for the development of symptoms. Long-term follow up of all patients is essential.
- Published
- 2006
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34. The evolution of endovascular treatment for intracranial arteriovenous malformations.
- Author
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Bristol RE, Albuquerque FC, and McDougall CG
- Subjects
- Embolization, Therapeutic trends, History, 20th Century, History, 21st Century, Humans, Intracranial Arteriovenous Malformations therapy, Embolization, Therapeutic history, Intracranial Arteriovenous Malformations history
- Abstract
Endovascular therapy for arteriovenous malformations (AVMs) remains a relatively new approach. Beginning in the 1960s with the use of flow-directed techniques for selective embolization, hemodynamic alterations have been used to treat these lesions. In every aspect of treatment, technological advances, including catheters, embolic materials, angiography suites, and pharmacological agents, have improved outcomes while lowering the risk to patients. In this article, the authors review the technical evolution of endovascular AVM therapy. Developments in embolic materials, beginning with foreign bodies and autografts and continuing through to highly engineered contemporary substances, are discussed. Finally, changes in treatment paradigms that have occurred over the years are traced. Within neurosurgery, this specialty has shown some of the fastest growth and development in recent decades. As minimally invasive approaches are embraced in all areas of medicine, it is clear that this treatment modality will continue to be refined.
- Published
- 2006
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35. Management of cranial base chondrosarcomas.
- Author
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Wanebo JE, Bristol RE, Porter RR, Coons SW, and Spetzler RF
- Subjects
- Adolescent, Adult, Aged, Child, Chondrosarcoma diagnostic imaging, Disease Management, Female, Follow-Up Studies, Humans, Male, Middle Aged, Radiography, Retrospective Studies, Skull Base Neoplasms diagnostic imaging, Survival Rate, Chondrosarcoma radiotherapy, Chondrosarcoma surgery, Skull Base Neoplasms radiotherapy, Skull Base Neoplasms surgery
- Abstract
Objective: Chondrosarcomas are rare, infiltrative, progressive lesions that occur at the cranial base. Their intimate association with cranial nerves and major vessels of the head and neck often precludes complete surgical resection., Methods: Between 1983 and 2003, 23 patients (14 females, 9 males) were treated at our institution with the diagnosis of chondrosarcoma of the cranial base (mean age at presentation, 43 yr). A retrospective chart review was performed to evaluate presentation, management, and adjunctive treatment. All living patients were contacted for a current examination and disease status., Results: The 23 patients underwent 43 surgical resections. Follow-up ranged from 8 months to 25 years (mean, 97 mo). Ten patients underwent various adjuvant radiation therapies. Five patients have died. Four patients have no evidence of disease, and 13 have residual tumor. One was lost to follow-up. Of 14 patients with 5 years of follow-up, 13 are living. Therefore, the absolute 5 year survival rate is 93%. The 10 year survival rate is 71%., Conclusion: Because of the intricate nature of the cranial base, a team approach is preferable for managing these challenging lesions. Maximum cytoreductive surgery should be pursued as an initial strategy to minimize neurological injury. Adjuvant stereotactic radiosurgery can be used to treat residual disease or small recurrences. This cohort also illustrates that patients with chondrosarcomas have better long-term survival rates than patients with chordomas of the cranial base.
- Published
- 2006
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36. Bedside twist drill craniostomy for chronic subdural hematoma: a comparative study.
- Author
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Horn EM, Feiz-Erfan I, Bristol RE, Spetzler RF, and Harrington TR
- Subjects
- Aged, Humans, Length of Stay, Middle Aged, Postoperative Complications, Prospective Studies, Reoperation, Treatment Outcome, Craniotomy instrumentation, Craniotomy methods, Hematoma, Subdural, Chronic surgery
- Abstract
Background: Although the bedside twist drill craniostomy is used to treat chronic subdural hematomas, the efficacy of this technique has not been compared with that of standard treatments (operative bur hole or craniotomy)., Methods: Twist drill craniostomy was compared with operative bur hole or craniotomy in a prospective nonrandomized trial. The inclusion criteria were computed tomographic evidence of chronic subdural hematoma (isodense or hypodense compared with brain) and symptoms indicating the need for drainage. Selection of the procedure depended on the on-call surgeon's preference. Clinical success of the procedure, recurrence, length of hospitalization, complications, and neurologic outcome were compared., Results: Between August 2001 and October 2002, 79 consecutive patients with 91 chronic subdural hematomas were treated (67 unilateral and 12 bilateral) at our institution. Fifty-five patients were treated with twist drill craniostomy and 24 with bur hole or craniotomy. There were no differences in the mean age of presentation, thickness of hematoma, length of hospitalization, reoperation rate, mortality rates, or ability to be discharged to home between the 2 groups. There was no difference in the neurologic outcomes in the 57 of the 79 patients available for follow-up., Conclusions: Twist drill craniostomy performed at the bedside is just as effective in treating chronic subdural hematomas as bur holes or craniotomy in the operating room. This procedure can most often be the first line of treatment in patients with symptomatic chronic subdural hematomas.
- Published
- 2006
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37. De novo presentation of an arteriovenous malformation. Case report and review of the literature.
- Author
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Gonzalez LF, Bristol RE, Porter RW, and Spetzler RF
- Subjects
- Accidents, Traffic, Arteriovenous Malformations surgery, Cerebral Angiography, Child, Child, Preschool, Epilepsy etiology, Female, Frontal Lobe blood supply, Frontal Lobe injuries, Humans, Magnetic Resonance Imaging, Radiosurgery, Arteriovenous Malformations etiology, Arteriovenous Malformations therapy, Craniocerebral Trauma complications, Embolization, Therapeutic
- Abstract
The authors report the case of a patient with a de novo arteriovenous malformation (AVM), indicating that the origin of these lesions may not always be congenital. A 3-year-old girl who was struck by a car suffered a mild head injury and experienced posttraumatic epilepsy. The initial magnetic resonance (MR) image obtained in this child revealed only a small contusion in the left frontal lobe. Intractable epilepsy subsequently developed. A second MR image obtained almost 4 years after the injury demonstrated an AVM in the right posterior temporal lobe that was verified using angiography. The lesion was classified as a Spetzler-Martin Grade III AVM. The patient underwent embolization of the feeding vessels followed by gamma knife surgery. Fourteen months after treatment she was asymptomatic. Follow-up MR images demonstrate no evidence of an AVM and no changes in the white matter. This case presents a de novo AVM that developed within approximately 4 years. The findings indicate that AVMs may not always be congenital and reinforce the concept that the natural history of AVMs is dynamic. Lesions may appear de novo, grow, and thrombose spontaneously.
- Published
- 2005
- Full Text
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38. Cognitive impact of craniosynostosis.
- Author
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Lekovic GP, Bristol RE, and Rekate HL
- Subjects
- Child, Child Development, Cognition Disorders surgery, Craniosynostoses surgery, Humans, Cognition physiology, Cognition Disorders etiology, Craniosynostoses physiopathology, Developmental Disabilities etiology
- Abstract
This article reviews the data on the effects of synostosis on cognitive development and the role of surgical intervention in ameliorating these effects. The literature on children with single-suture craniosynostosis, treated both surgically and conservatively, is reviewed. The evidence for possible pathophysiological mechanisms by which brain restriction might affect cognitive development is also reviewed. Although children with single-suture isolated craniosynostosis may be at risk of developmental delay, learning disability, or both, especially with regard to speech or language skills, available testing methodologies provide no evidence of an association between surgical intervention and ultimate intellectual outcome. Despite the controversies about the role of surgery in addressing the cognitive sequelae of simple craniosynostosis, in the absence of definitive data, the indications for surgery include correction of deformity and minimization of cognitive sequelae. The relative risks of performing surgery early or late must be determined on an individual basis, balancing potential cosmetic and cognitive benefits against the heightened risk of reoperation or perioperative morbidity.
- Published
- 2004
- Full Text
- View/download PDF
39. The effects of craniosynostosis on the brain with respect to intracranial pressure.
- Author
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Bristol RE, Lekovic GP, and Rekate HL
- Subjects
- Cranial Sutures pathology, Cranial Sutures physiopathology, Craniosynostoses genetics, Humans, Models, Biological, Receptor Protein-Tyrosine Kinases genetics, Receptor, Fibroblast Growth Factor, Type 2, Receptors, Fibroblast Growth Factor genetics, Brain physiopathology, Craniosynostoses physiopathology, Intracranial Pressure physiology
- Abstract
Intracranial pressure (ICP) and skull volume are intricately related. Craniosynostosis alters skull volume, and the many forms of craniosynostosis complicate the relationship to ICP even further. Patients with single-suture synostosis are less likely to experience elevated ICP than patients in whom multiple sutures, craniofacial syndromes, or both are involved. Among patients with more than one suture involved, the multifactorial mechanisms underlying elevated ICP include cephalocranial disproportion and venous outflow obstruction. Direct monitoring of ICP for at least 24 hours can aid in the diagnosis and decision making process. The management of craniosynostotic patients is diverse and necessitates a long-term plan for follow-up.
- Published
- 2004
- Full Text
- View/download PDF
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