Publications by authors named "Tord D Alden"

45 Publications

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

J Neurosurg Pediatr 2021 Feb 12:1-10. Epub 2021 Feb 12.

1Department of Neurological Surgery, Washington University School of Medicine, St. Louis, MO.

Objective: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM.

Methods: The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft.

Results: A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain.

Conclusions: In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.
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http://dx.doi.org/10.3171/2020.8.PEDS2087DOI Listing
February 2021

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

Neurosurgery 2021 01;88(2):332-341

Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.

Background: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology.

Objective: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD.

Methods: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD.

Results: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups.

Conclusion: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.
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http://dx.doi.org/10.1093/neuros/nyaa460DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7803430PMC
January 2021

Use of Nimodipine in a Neonate With Cerebral Vasospasm With Delayed Ischemia From Subarachnoid Hemorrhage in the Posterior Fossa.

Pediatr Neurol 2020 10 3;111:44-45. Epub 2020 Jul 3.

Division of Neurology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2020.06.018DOI Listing
October 2020

Infratentorial choroid plexus tumors in children.

Childs Nerv Syst 2020 08 2;36(8):1761-1766. Epub 2020 Mar 2.

Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Avenue, Chicago, IL, USA.

Objective: Choroid plexus tumors (CPTs) are rare pediatric intracranial neoplasms, and mostly occur in the lateral ventricle. CPTs located in the infratentorial location are considered to be rare in the pediatric population. We present a series of eight patients treated in the last decade at our institution focusing on clinical presentations and their outcome after excision.

Methods: We performed an institutional retrospective review of patients who underwent surgical resection of infratentorial CPTs during the period from 2008 to 2017. Patients' charts were reviewed for demographic data, clinical presentation, surgical treatment, and follow-up.

Results: There were eight patients (6 females and 2 males), with mean age for the cohort at presentation was 9.0 years. They represent 75% of 12 CPTs of all locations treated at the same period in our institution. These 8 infratentorial CPTs were in the fourth ventricle in seven, and in the cerebellopontine angle (CPA) in one. Seven patients had choroid plexus papillomas (WHO grade I) and 1 had an atypical choroid plexus papilloma (WHO grade II). Gross total resection was attempted in all patients. However, two of 3 patients with fourth ventricle floor invasion had subtotal resection with a thin layer of tumor left on the floor. The remaining 6 had a gross total resection. Six patients with preoperative hydrocephalus had a perioperative external ventricular drainage but none required permanent shunting after tumor resection. None showed recurrence/tumor progression without adjuvant therapy during the follow-up period of 20 months to 11 years.

Conclusion: Infratentorial dominance among pediatric CPTs in this series contradicts previous reports. Infratentorial CPTs are amenable to surgical resection. Unresected small residuals due to invasion to the fourth ventricle floor showed no regrowth during 2 to 3 years follow-up without adjuvant therapy. However, these patients with incomplete resection need watchful observations.
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http://dx.doi.org/10.1007/s00381-020-04532-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7355280PMC
August 2020

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2020 Mar 6:1-11. Epub 2020 Mar 6.

34Department of Neurological Surgery, Washington University in St. Louis School of Medicine, St. Louis, Missouri.

Objective: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.

Methods: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.

Results: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.

Conclusions: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.
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http://dx.doi.org/10.3171/2020.1.PEDS19493DOI Listing
March 2020

Nasal dermoid cysts with intracranial extension: avoiding coronal incision through midline exposure and nasal bone osteotomy.

J Neurosurg Pediatr 2019 Dec 6:1-7. Epub 2019 Dec 6.

4Otolaryngology/Head and Neck Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Illinois.

Objective: Up to 10% of midline nasal dermoid cysts have intracranial extension. Previous techniques of excision include frontal and frontonasal craniotomies via a coronal approach, combined with a direct cutaneous excision of the dermoid cyst. While the coronal incision allows for wide visualization, it carries significant risks of transfusion, blood loss, and scarring. The authors present an alternative technique in which access is gained through a midline extension of the dermoid cyst excision that provides direct access for a keyhole frontal craniotomy.

Methods: The authors utilize a nasal bone osteotomy, pericranial flap, and keyhole-type craniotomy performed through a nasal midline incision for the treatment of nasal dermoid cysts with intracranial extension. They performed a retrospective chart review of all patients with nasal dermoid cysts treated at the Ann & Robert H. Lurie Children's Hospital of Chicago from 2009 to 2017. Patient demographic data, operative data, and in- and outpatient complication data were collected.

Results: In 10 patients with cyst extension near or into the intracranial cavity (7 with true intracranial extension), the nasal osteotomy technique was performed. The mean blood loss was 13 ml, with a 0% transfusion rate. The mean length of inpatient stay was 1 day. A durotomy was made and repaired as part of the dermoid cyst dissection in 3 patients. One patient underwent intraoperative placement of a lumbar drain. The mean operative time was 228 minutes. There were no intraoperative or postoperative complications, including the need for a reoperation. No patients had any long-term complications, and no patients have had dermoid cyst recurrence. The appearance of the scar was acceptable in all cases.

Conclusions: The midline approach to nasal dermoid cysts with intracranial extension is safe and results in limited blood loss, short operative times, and short lengths of inpatient hospital stay. This is a viable technique for the treatment of this challenging pathology.
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http://dx.doi.org/10.3171/2019.9.PEDS19132DOI Listing
December 2019

Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2019 Aug 16:1-8. Epub 2019 Aug 16.

1Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.

Objective: Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors' goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.

Methods: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).

Results: Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB-C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.

Conclusions: Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.
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http://dx.doi.org/10.3171/2019.5.PEDS18527DOI Listing
August 2019

Pediatric primary spinal atypical teratoid rhabdoid tumor: a case series and review of the literature.

J Neurosurg Pediatr 2019 Jul 12:1-17. Epub 2019 Jul 12.

4Department of Surgery, Division of Pediatric Neurosurgery, Ann and Robert H. Lurie Children's Hospital of Chicago; and.

Atypical teratoid rhabdoid tumors (ATRTs) are rare malignant central nervous system tumors, commonly occurring before 3 years of age. Median overall survival (OS) of patients with these tumors is about 1 year, despite aggressive multimodal therapy. Pediatric primary spinal ATRTs are even more rare, with fewer than 50 cases reported. The authors present a series of four patients who were treated at Ann and Robert H. Lurie Children's Hospital of Chicago in the period from 1996 to 2017.These patients, with ages 2-11 years, presented with pain and a decline in motor functions. They were found to have lesions in the lumbar, thoracic, and/or cervical spine. One patient's tumor was intramedullary with exophytic components, while another patient's tumor had both intra- and extradural components. All patients underwent resection followed by chemotherapy (systemic and intrathecal). Two patients had fractionated radiation therapy and one had an autologous stem cell transplant. Three patients are known to be deceased (OS 8.5-45 months). The fourth patient was in remission 19 years after her initial diagnosis. To the authors' knowledge, this is the largest series of pediatric primary spinal ATRTs documented at a single institution. These cases illustrate a variety of presentations of spinal ATRT and add to the body of literature on this aggressive pathology.A systematic MEDLINE search was also conducted using the keywords "atypical teratoid rhabdoid tumor," "pediatric spinal rhabdoid tumor," and "malignant rhabdoid tumor spine." Reports were included for patients younger than 21 years, without evidence of intracranial or systemic disease at the time of diagnosis. Clinical characteristics and outcomes of the four institutional cases were compared to those in the literature. This review yielded an additional 48 cases of primary pediatric spinal ATRTs reported in the English-language literature. Patients (ages 2 months to 19 years) presented with symptoms of pain, regression of motor function, and spinal cord compression. The majority of tumors were intradural (14 extramedullary, 8 intramedullary, 1 both). Eleven cases in the literature described tumors limited to extradural structures, while 10 tumors involved the intra- and extradural spine. Four reports did not specify tumor location. Although rare, spinal ATRT should be considered in the differential diagnosis of pediatric patients presenting with a new spinal mass.
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http://dx.doi.org/10.3171/2019.4.PEDS19113DOI Listing
July 2019

Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance.

Orphanet J Rare Dis 2019 06 13;14(1):137. Epub 2019 Jun 13.

Center for Rare Diseases at Host Schmidt Kliniken, Wiesbaden, Germany and Department of Paediatrics University of Padova, Padova, Italy.

Introduction: Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA.

Methods: Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers.

Results: A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance).

Conclusion: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.
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http://dx.doi.org/10.1186/s13023-019-1074-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6567385PMC
June 2019

Safety and outcome of combined endovascular and surgical management of low grade cerebral arteriovenous malformations in children compared to surgery alone.

Eur J Radiol 2019 Jul 18;116:8-13. Epub 2019 Feb 18.

Department of Radiology, Northwestern University, Feinberg School of Medicine, Chicago, IL, United States; Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, IL, United States. Electronic address:

Purpose: To evaluate the outcomes of combined preoperative embolization and microsurgical resection in comparison with microsurgical resection alone as the current standard of care for low-grade cerebral arteriovenous malformations (AVM) in the pediatric population.

Materials & Methods: We performed a single-center retrospective study of pediatric patients presenting with Spetzler-Martin (SM) grade I and II cerebral AVMs at a high-volume tertiary pediatric hospital between January 2005 and September 2016. Low grade AVM patients were divided into two groups: pre-operative embolization with subsequent microsurgical resection or microsurgical resection alone. Patient demographics, clinical and imaging presentations, AVM morphological characteristics, post-operative complications, and mid to long-term clinical outcomes were studied. Post-embolization and post-surgical outcomes were assessed prior to and after treatment, at 3 months and at final follow-up using the modified Rankin Scale (mRS) to compare both final independent (mRS 0-2) and favorable (no change or improved mRS) clinical outcomes for comparison between study groups. Statistical associations of patient demographics, AVM characteristics/SM grading, and treatment modality group with post-operative complications were performed using univariate logistic regression analysis.

Results: Thirty-four patients with low grade cerebral AVMs met the study inclusion criteria (mean age 10.6 ± 3.4 years; range 3-16 years, 22M:12 F). Twenty patients (59%) presented with ruptured AVMs. Twenty-five patients (73.5%) underwent combined treatment with embolization and microsurgical resection, while 9/34 (26.5%) underwent microsurgical resection alone. A total of 35 embolization procedures performed in 25 patients (Mode, 1; Range, 1-7) were associated with two minor post-embolization and 7 subsequent post-surgical (28%) complications, resulting in clinical deterioration in a single patient. Microsurgical resection alone was associated with 3 post-surgical complications (33%), resulting in permanent neurological disability in a single patient. There was no significance association of post-operative complications with either treatment modality group, combined treatment versus surgical resection alone [OR:1.13; 95% CI:0.23-5.62; p-value 0.88]. SM Grade II and eloquent locations were found to be significantly associated with post-surgical complications of low grade pediatric cerebral AVMs [OR 13.2 and OR 8 respectively, p-value 0.004 and 0.005). On mean follow-up time of 35.7 months, final clinical outcome was favorable in the majority of both treatment arms with no dependent (mRS>2) patients in the combined endovascular and surgical cohort. Two patients in the surgical cohort failed to achieve independent functional status, primarily due to a pre-operative morbid status (p-value 0.015). However, there was no significant difference in favorable outcomes between the treatment groups [p-value 0.14].

Conclusion: Our study suggests equivalent safety and favorable clinical outcomes related to combined endovascular embolization and microsurgical resection of low grade pediatric cerebral AVMs in comparison to microsurgical resection alone. On long term clinical follow-up, the vast majority of patients achieved an independent and favorable functional status irrespective of pre-operative embolization.
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http://dx.doi.org/10.1016/j.ejrad.2019.02.016DOI Listing
July 2019

Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance.

Orphanet J Rare Dis 2019 05 29;14(1):118. Epub 2019 May 29.

Center for Rare Diseases at Host Schmidt Kliniken, Wiesbaden, Germany and Department of Paediatrics, University of Padova, Padova, Italy.

Introduction: Mucopolysaccharidosis (MPS) VI or Maroteaux-Lamy syndrome (253200) is an autosomal recessive lysosomal storage disorder caused by deficiency in N-acetylgalactosamine-4-sulfatase (arylsulfatase B). The heterogeneity and progressive nature of MPS VI necessitates a multidisciplinary team approach and there is a need for robust guidance to achieve optimal management. This programme was convened to develop evidence-based, expert-agreed recommendations for the general principles of management, routine monitoring requirements and the use of medical and surgical interventions in patients with MPS VI.

Methods: 26 international healthcare professionals from various disciplines, all with expertise in managing MPS VI, and three patient advocates formed the Steering Committee group (SC) and contributed to the development of this guidance. Members from six Patient Advocacy Groups (PAGs) acted as advisors and attended interviews to ensure representation of the patient perspective. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with expertise and experience managing patients with MPS VI and the manuscript has been evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers.

Results: A total of 93 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions. Consensus was reached on all statements after two rounds of voting. The greatest challenges faced by patients as relayed by consultation with PAGs were deficits in endurance, dexterity, hearing, vision and respiratory function. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance).

Conclusion: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS VI and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.
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http://dx.doi.org/10.1186/s13023-019-1080-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6541999PMC
May 2019

Standardized Evaluation of Cerebral Arteriovenous Malformations Using Flow Distribution Network Graphs and Dual-venc 4D Flow MRI.

J Magn Reson Imaging 2019 12 9;50(6):1718-1730. Epub 2019 May 9.

Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.

Background: Cerebral arteriovenous malformations (AVMs) are pathological connections between arteries and veins. Dual-venc 4D flow MRI, an extended 4D flow MRI method with improved velocity dynamic range, provides time-resolved 3D cerebral hemodynamics.

Purpose: To optimize dual-venc 4D flow imaging parameters for AVM; to assess the relationship between spatial resolution, acceleration, and flow quantification accuracy; and to introduce and apply the flow distribution network graph (FDNG) paradigm for storing and analyzing complex neurovascular 4D flow data.

Study Type: Retrospective cohort study.

Subjects/phantom: Scans were performed in a specialized flow phantom: 26 healthy subjects (age 41 ± 17 years) and five AVM patients (age 27-68 years).

Field Strength/sequence: Dual-venc 4D flow with varying spatial resolution and acceleration factors were performed at 3T field strength.

Assessment: Quantification accuracy was assessed in vitro by direct comparison to measured flow. FDNGs were used to quantify and compare flow, peak velocity (PV), and pulsatility index (PI) between healthy controls with various Circle of Willis (CoW) anatomy and AVM patients.

Statistical Tests: In vitro measurements were compared to ground truth with Student's t-test. In vivo groups were compared with Wilcoxon rank-sum test and Kruskal-Wallis test.

Results: Flow was overestimated in all in vitro experiments, by an average 7.1 ± 1.4% for all measurement conditions. Error in flow measurement was significantly correlated with number of voxels across the channel (P = 3.11 × 10 ) but not with acceleration factor (P = 0.74). For the venous-arterial PV and PI ratios, a significant difference was found between AVM nidal and extranidal circulation (P = 0.008 and 0.05, respectively), and between AVM nidal and healthy control circulation (P = 0.005 and 0.003, respectively).

Data Conclusion: Dual-venc 4D flow MRI and standardized FDNG analysis might be feasible in clinical applications. Venous-arterial ratios of PV and PI are proposed as network-based biomarkers characterizing AVM nidal hemodynamics.

Level Of Evidence: 3 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2019;50:1718-1730.
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http://dx.doi.org/10.1002/jmri.26784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6842032PMC
December 2019

Choroidal artery embolization in the management of cerebrospinal fluid overproduction: case report and review of the literature.

J Neurosurg Pediatr 2019 03 22:1-12. Epub 2019 Mar 22.

Department of Surgery, Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois; and.

Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a rare cause of communicating hydrocephalus. DVHCP may be diagnosed radiographically and through histological evaluation. It may be associated with genetic abnormalities, particularly involving chromosome 9. Due to CSF overproduction, patients with DVHCP often fail management with shunting alone and may require adjuvant interventions. The authors present the case of a child with partial trisomy 9p and delayed diagnosis of hydrocephalus with radiographic evidence of DVHCP who was successfully managed with ventriculoperitoneal shunt (VPS) placement, adjuvant bilateral endoscopic choroid plexus coagulation (CPC), and the novel application of anterior choroidal artery embolization. In addition, a systematic MEDLINE search was conducted using the keywords "diffuse villous hyperplasia," "choroid plexus hypertrophy," and "idiopathic cerebrospinal fluid overproduction." Clinicopathological characteristics and outcomes of the present case were reviewed and compared to those in the literature.A 14-month-old girl with partial trisomy 9p presented with macrocephaly and radiographic evidence of communicating hydrocephalus and DVHCP. Ventriculoperitoneal shunting resulted in distal failure due to inadequate CSF absorption, and ventriculoatrial shunt (VAS) placement was not possible due to multiple cardiac anomalies. Daily CSF production was reduced via endoscopic third ventriculostomy and bilateral CPC, followed by distal choroidal artery embolization, enabling VPS re-internalization. The embolization was complicated by radiographic evidence of an iatrogenic cerebral infarct, but this was clinically occult. Thirty-two additional cases of communicating hydrocephalus due to DVHCP are reported in the literature: 27 pediatric, 3 adult, and 2 postmortem. Genetic abnormalities were noted in 14, with 7 (50%) involving chromosome 9. Twelve patients underwent plexectomy (9 bilateral, 2 unilateral, 1 partial), and 10 underwent CPC (4 bilateral, 3 unilateral, and 3 unspecified), with or without shunting. Eight patients were successfully managed with shunting alone (6 VASs, 2 VPSs), and none underwent arterial embolization.DVHCP is a rare cause of communicating hydrocephalus that may be associated with genetic abnormalities. A thorough review of the literature highlights diagnostic criteria and interventional options involved in managing this cause of CSF overproduction. The present case demonstrates that angiographic confirmation of prominent choroidal arteries may contribute to the diagnosis DVHCP. In addition, embolization of the distal choroidal arteries may be considered as a potential adjuvant treatment in patients for whom conventional treatments have failed or are not feasible.
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http://dx.doi.org/10.3171/2019.1.PEDS18519DOI Listing
March 2019

MynxGrip vascular closure device use in pediatric neurointerventional procedures.

J Neurosurg Pediatr 2018 05 2;21(5):466-470. Epub 2018 Mar 2.

Departments of1Radiology.

OBJECTIVE The aim of this paper was assess the efficacy and safety of using the MynxGrip arterial closure device in pediatric neuroendovascular procedures where the use of closure devices remains off-label despite their validation and widespread use in adults. METHODS A retrospective review of all pediatric patients who underwent diagnostic or interventional neuroendovascular procedures at the authors' institution was performed. MynxGrip use was predicated by an adequate depth of subcutaneous tissue and common femoral artery (CFA) diameter. Patients remained on supine bedrest for 2 hours after diagnostic procedures and for 3 hours after therapeutic procedures. Patient demographics, procedural details, hemostasis status, and complications were recorded. RESULTS Over 36 months, 83 MynxGrip devices were deployed in 53 patients (23 male and 30 female patients; mean age 14 years) who underwent neuroendovascular procedures. The right-side CFA was the main point of access for most procedures. The mean CFA diameter was 6.24 mm and ranged from 4 mm to 8.5 mm. Diagnostic angiography comprised 46% of the procedures. A single device failure occurred without any sequelae; the device was extracted, and hemostasis was achieved by manual compression with the placement of a Safeguard compression device. No other immediate or delayed major complications were recorded. CONCLUSIONS MynxGrip can be used safely in the pediatric population for effective hemostasis and has the advantage of earlier mobilization.
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http://dx.doi.org/10.3171/2017.11.PEDS17481DOI Listing
May 2018

Peripherally derived T regulatory and γδ T cells have opposing roles in the pathogenesis of intractable pediatric epilepsy.

J Exp Med 2018 04 27;215(4):1169-1186. Epub 2018 Feb 27.

Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL

The pathophysiology of drug-resistant pediatric epilepsy is unknown. Flow cytometric analysis of inflammatory leukocytes in resected brain tissues from 29 pediatric patients with genetic (focal cortical dysplasia) or acquired (encephalomalacia) epilepsy demonstrated significant brain infiltration of blood-borne inflammatory myeloid cells and memory CD4 and CD8 T cells. Significantly, proinflammatory (IL-17- and GM-CSF-producing) γδ T cells were concentrated in epileptogenic lesions, and their numbers positively correlated with disease severity. Conversely, numbers of regulatory T (T reg) cells inversely correlated with disease severity. Correspondingly, using the kainic acid model of status epilepticus, we show ameliorated seizure activity in both γδ T cell- and IL-17RA-deficient mice and in recipients of T reg cells, whereas T reg cell depletion heightened seizure severity. Moreover, both IL-17 and GM-CSF induced neuronal hyperexcitability in brain slice cultures. These studies support a major pathological role for peripherally derived innate and adaptive proinflammatory immune responses in the pathogenesis of intractable epilepsy and suggest testing of immunomodulatory therapies.
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http://dx.doi.org/10.1084/jem.20171285DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5881465PMC
April 2018

Surgical management of neurological manifestations of mucopolysaccharidosis disorders.

Mol Genet Metab 2017 12 28;122S:41-48. Epub 2017 Sep 28.

Department of Neuroradiology, DASA Group, São Paulo, Brazil.

The mucopolysaccharidosis (MPS) disorders are ultra-rare lysosomal storage disorders associated with progressive accumulation of glycosaminoglycans (GAGs) in cells and tissues throughout the body. Clinical manifestations and progression rates vary widely across and within the different types of MPS. Neurological symptoms occur frequently, and may result directly from brain damage caused by infiltration of GAGs, or develop secondary to somatic manifestations such as spinal cord compression, hydrocephalus, and peripheral nerve entrapment. Management of secondary neurological manifestations often requires surgical correction of the underlying somatic cause. The present review discusses the surgical management of neurological disease in patients with MPS, including diagnostic imaging. Background information is derived from presentations and discussions during a meeting on the brain in MPS, attended by an international group of experts (April 28-30, 2016, Stockholm, Sweden), and additional literature searches.
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http://dx.doi.org/10.1016/j.ymgme.2017.09.011DOI Listing
December 2017

The rare case of a large complex intraosseous cranial arteriovenous malformation with successful multidisciplinary management.

J Neurosurg Pediatr 2017 Dec 29;20(6):591-597. Epub 2017 Sep 29.

Departments of1Radiology and.

Intraosseous cranial arteriovenous malformations (AVMs) are very rare, challenging entities. The authors report the case of an extracranial parietooccipital vascular lesion. A 12-year-old boy presented with accelerated growth of a right scalp lesion over a few months. Digital subtraction angiography showed a large, right parietooccipital intraosseous AVM with multiple complex arterial feeders. Treatment of these lesions is difficult and can necessitate a multidisciplinary approach. In the featured case, 6 embolization procedures were performed over 1 year, including both transarterial and transvenous approaches, followed by total resection. The authors describe what is thought to be the second case of an AVM originating in the cranial bones and the first case with successful multidisciplinary management.
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http://dx.doi.org/10.3171/2017.7.PEDS17161DOI Listing
December 2017

Seizure Outcomes in Children Following Electrocorticography-Guided Single-Stage Surgical Resection.

Pediatr Neurol 2017 Jun 7;71:35-42. Epub 2017 Feb 7.

Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago and Feinberg School of Medicine, Northwestern University, Chicago, Illinois.

Background: In children with abnormal imaging, single-stage epilepsy surgery is an attractive alternative to the two-stage approach that relies on invasive recording of seizures. Implanted electrodes carry risks of their own and extend hospitalization, but the efficacy of one-stage resections in a variety of pathologies and cerebral locations is not well established. We report our center's experience with single-stage epilepsy surgery guided by intraoperative electrocorticography (ECoG).

Methods: We retrospectively analyzed 130 consecutive patients who underwent single-stage epilepsy surgery before age 19 years and had at least a two-year follow-up. Intraoperative ECoG was available for review in 113. Patients were considered seizure-free if they were continuously Engel Class I up to the two-year postoperative mark. ECoG findings were classified according to the presence of interictal attenuation, spikes, both, or neither. Complications and hospital length of stay were evaluated.

Results: Eighty percent of 130 patients were seizure-free at two years. All but one had an abnormal MRI. Patients with tumor had a better seizure outcome than patients with cortical malformation. Frontal resections had worse outcome, especially among tumors. Intraoperative ECoG revealed both attenuation and spikes in 48%, attenuation only in 23%, spikes only in 20%, and neither in 9%. The complication rate was 6.9%, with no major neurological complications. The average length of stay was 5.7 nights.

Conclusions: With ECoG-guided single-stage surgery, we achieved results comparable with other pediatric surgical series and with a low complication rate. An extensive two-stage approach may not be required when there is a lesion on imaging and other information is concordant, even when the MRI abnormality is subtle and unclearly delineated. Frontal foci may present a challenge because of their proximity to "eloquent" nonresectable cortex or critical structures.
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http://dx.doi.org/10.1016/j.pediatrneurol.2017.01.024DOI Listing
June 2017

Familial incidence of the congenital torcular dural arteriovenous shunt: Case report and review of the literature.

Clin Neurol Neurosurg 2016 May 21;144:129-32. Epub 2016 Mar 21.

Departments of Radiology Northwestern University, Feinberg of School of Medicine, Chicago, IL, USA; Neurological Surgery, Northwestern University, Feinberg of School of Medicine, Chicago, IL, USA; Neuro-interventional Surgery, Divisions, Ann & Robert H. Lurie Children's, Hospital of Chicago. Chicago, IL, USA.

Congenital dural sinus malformations are rare but can be major causes of mortality and morbidity in the pediatric population if not detected and managed urgently. Lesions involving large draining sinus structures such as superior sagittal sinus and torcular herophili can result in significant intracranial circulation impairment mostly due to venous drainage disturbance. Early detection plays a pivotal role in the outcome of the patients. Rarely familial incidence of some types of arteriovenous malformations in isolation from other congenital hereditary disorders has been reported. Knowledge of the familial association of congenital dural sinus malformations may raise the awareness for considering the possibility of occurrence of these lesions in the relatives of index cases. Herein, we describe the occurrence of giant torcular dural shunt in two pediatric cousins treated with endovascular embolization.
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http://dx.doi.org/10.1016/j.clineuro.2016.03.015DOI Listing
May 2016

Focal stenosis of the sigmoid sinus causing intracranial venous hypertension: Case report, endovascular management, and review of the literature.

Interv Neuroradiol 2016 Apr 14;22(2):240-5. Epub 2016 Jan 14.

Department of Radiology, Northwestern University Feinberg of School of Medicine, USA Department of Neurological Surgery, Northwestern University Feinberg of School of Medicine, USA Division of Neuro-interventional Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, USA.

Regardless of the underlying pathology, elevated intracranial pressure is the endpoint of any impairment in either cerebrospinal fluid (CSF) absorption (including arachnoid villi) or intracranial venous drainage. In all age groups, the predominant final common pathway for CSF drainage is the dural venous sinus system. Intracranial venous hypertension (ICVH) is an important vascular cause of intracranial hypertension (and its subsequent sequelae), which has often been ignored due to excessive attention to the arterial system and, specifically, arteriovenous shunts. Various anatomical and pathological entities have been described to cause ICVH. For the second time, we present a unique case of severe focal stenosis in the distal sigmoid sinus associated with concurrent hypoplasia of the contralateral transverse sinus causing a significant pressure gradient and intracranial hypertension, which was treated with endovascular stent placement and angioplasty.
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http://dx.doi.org/10.1177/1591019915622160DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4984344PMC
April 2016

Pediatric skull base reconstruction: case report of a tunneled temporoparietal fascia flap.

J Neurosurg Pediatr 2016 Mar 6;17(3):371-7. Epub 2015 Nov 6.

Pediatric Neurosurgery, Ann & Robert H. Lurie Children's Hospital of Chicago; and 

The authors of this report present a pediatric case involving the use of a tunneled temporoparietal fascia flap to reconstruct a skull base defect for a multiply recurrent clival chordoma and cerebrospinal fluid leak, demonstrate the surgical technique through illustrations and intraoperative photos, and review the pertinent literature. A 9-year-old female patient underwent extensive clival chordoma resection via both the endoscopic and open approaches, which ultimately exhausted the bilateral nasoseptal flaps and other intranasal reconstructive options. Following proton beam radiation and initiation of chemotherapy, tumor recurrence was managed with further endoscopic resection, which was complicated by a recalcitrant cerebrospinal fluid leak. A tunneled temporoparietal fascia flap was used to provide vascular tissue to augment an endoscopic repair of the leak and reconstruction of the skull base. While the nasoseptal flap remains the workhorse for many pediatric and adult endoscopic skull base reconstructions, the tunneled temporoparietal fascia flap has a demonstrated efficacy in adults when the nasoseptal flap and other intranasal flaps are unavailable. This report documents a pediatric case, serving as a step toward establishing this technique in the pediatric population.
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http://dx.doi.org/10.3171/2015.6.PEDS1588DOI Listing
March 2016

Squamosal Suture Craniosynostosis Due to Hyperthyroidism Caused by an Activating Thyrotropin Receptor Mutation (T632I).

Thyroid 2015 Oct 21;25(10):1167-72. Epub 2015 Jul 21.

5 Division of Endocrinology Metabolism and Molecular Medicine, Northwestern University Feinberg School of Medicine , Chicago, Illinois.

Background: Congenital hyperthyroidism can be a cause of failure to thrive, hyperactivity, developmental delay, and craniosynostosis during infancy. Most commonly, the condition occurs in the setting of maternal autoimmune thyroid disease. Rarely, congenital hyperthyroidism can also occur secondary to activating mutations within the thyrotropin (TSH) receptor.

Patient Findings: A Hispanic male infant presented at age 6 months with severe thyrotoxicosis. At the time of presentation he was being evaluated for squamosal suture synostosis and he was noted to have significant developmental delays.

Summary: The patient's thyrotoxicosis was initially treated with antithyroid medication, and he subsequently underwent craniosynostosis repair leading to neurodevelopmental improvement. DNA from the patient and his parents was submitted for mutational analysis of exons 9 and 10 of the TSH receptor. He was found to carry a monoallelic transition 1895C>T in exon 10 that resulted in the substitution of threonine at position 632 by isoleucine (T32I). This mutation resulted in constitutive activation of the TSH receptor. Neither parent carried this mutation indicating that the child has acquired a de novo germline mutation.

Conclusions: We report the first case of squamosal suture craniosynostosis in a patient with non-autoimmune hyperthyroidism. Squamosal suture craniosynotosis is rare, often has a subtle presentation, and should be considered in all patients with this condition because prompt treatment of hyperthyroidism and craniosynotosis repair can lead to normalization of neurodevelopment.
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http://dx.doi.org/10.1089/thy.2014.0503DOI Listing
October 2015

Diagnostic evaluation, monitoring, and perioperative management of spinal cord compression in patients with Morquio syndrome.

Mol Genet Metab 2015 Jan 30;114(1):11-8. Epub 2014 Oct 30.

Department of Anesthesiology & Critical Care, Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803-3607, USA. Electronic address:

Mucopolysaccharidosis IVA is an autosomal recessive condition caused by mutations in the GALNS gene, which encodes N-acetylgalactosamine-6-sulfatase, also called galactosamine-6-sulfatase (GALNS). A reduction in or absence of effective GALNS leads to faulty catabolism of keratan sulfate and chondroitin-6-sulfate within the lysosome; their accumulation causes cell, tissue, and organ dysfunction. The connective tissue, cartilage, ligaments, and bone of patients with Morquio A syndrome are particularly affected. Patients with Morquio A syndrome are at high risk of neurological complications because of their skeletal abnormalities; many patients are in danger of cervical myelopathy due to odontoid hypoplasia and ligamentous laxity leading to atlantoaxial subluxation. The multisystemic involvement of patients with Morquio A syndrome requires treatment by multidisciplinary teams; not all members of these teams may be aware of the potential for subluxation and quadriparesis. A multinational, multidisciplinary panel of 10 skeletal dysplasia or Morquio A syndrome specialists convened in Miami, FL on December 7 and 8, 2012 to develop consensus recommendations for early identification and effective management of spinal cord compression, for anesthesia and surgical best practices, and for effectual cardiac and respiratory management in patients with Morquio A syndrome. The target audience for these recommendations includes any physician who may encounter a patient with Morquio A syndrome, however doctors who do not have access to the full spectrum of specialists and resources needed to support patients with Morquio A syndrome should attempt to refer patients to a center that does. Physicians who manage Morquio A syndrome or comorbid conditions within specialty centers should review these expert panel recommendations and fully understand the implications of spinal cord instability for their own practices.
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http://dx.doi.org/10.1016/j.ymgme.2014.10.010DOI Listing
January 2015

Endoscopic endonasal surgery for sinonasal and skull base lesions in the pediatric population.

Otolaryngol Clin North Am 2015 Feb;48(1):79-99

Department of Neurological Surgery, University of Pittsburgh School of Medicine, 200 Lothrop Street, PUH B-400, Pittsburgh, PA, 15213, USA; Division of Pediatric Neurosurgery, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA, 15224, USA.

Endoscopic endonasal skull base surgical techniques, initially developed in adult patients, are being utilized with increasing frequency in pediatric patients to treat sinonasal and skull base lesions. This article reviews the current state of endoscopic endonasal approaches to the skull base to both treat disease and reconstruct the skull base in pediatric patients. Sinonasal and skull base embryology and anatomy are reviewed as a foundation for understanding the disease processes and surgical techniques. Selected skull base pathologies and conditions that involve the pediatric skull base are also reviewed.
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http://dx.doi.org/10.1016/j.otc.2014.09.007DOI Listing
February 2015

Use of Onyx for endovascular embolization of pediatric spinal perimedullary (Type IV) fistula: case report.

Clin Neurol Neurosurg 2013 Oct 7;115(10):2260-3. Epub 2013 Aug 7.

Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. Electronic address:

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http://dx.doi.org/10.1016/j.clineuro.2013.07.022DOI Listing
October 2013

Onyx embolization of intracranial arteriovenous malformations in pediatric patients.

J Neurosurg Pediatr 2013 Apr 8;11(4):431-7. Epub 2013 Feb 8.

Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA.

Object: The authors undertook this study to assess the safety and efficacy of Onyx embolization in the treatment of intracranial arteriovenous malformations (AVMs) in pediatric patients.

Methods: All pediatric Onyx embolization of intracranial AVM cases performed consecutively at a single children's hospital over a 5-year period were collected and evaluated.

Results: Twenty-five patients (mean age 10.5 years) underwent a total of 38 procedures. An aggregate of 56 pedicles were embolized (mean 1.47 per session). The Spetzler-Martin grade was determined in all cases. Onyx embolization resulted in complete obliteration of the AVM in 3 cases (12%) and partial obliteration in 22 cases (88%). A total of 23 patients underwent surgical treatment. The mean preoperative AVM devascularization in these cases was 72%. One patient was treated with radiosurgery following Onyx embolization. Overall, 10 complications occurred in a total of 38 procedures (26.3%). None of the complications resulted in permanent neurological morbidity. The rate of transient neurological complications was 10.5% (4 of 38 procedures) and the rate of transient nonneurological complications was 5.3% (2 of 38 procedures). The remaining 4 complications were clinically silent (rate of 10.5%). There were no procedure-related deaths in this study population. There was no significant difference in patients with and without complications in terms of demographic characteristics, AVM grade, or embolization features (p ≥ 0.2). Deep venous drainage was associated with higher complication rates (p = 0.03).

Conclusions: Onyx utilization is feasible for preoperative or primary embolization in the treatment of pediatric intracranial AVMs; however, the spectrum of complications encountered is broad, and attention must be paid to the technical nuances of and indications for its use to avoid many potential dangerous effects. Although the overall complication rates were higher than expected, all were either clinically silent or had only transient clinical effects. Thus, this experience suggests that Onyx embolization can be performed safely with a low rate of permanent morbidity in pediatric patients harboring these difficult lesions.
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http://dx.doi.org/10.3171/2013.1.PEDS12286DOI Listing
April 2013

Safety and efficacy of onyx embolization for pediatric cranial and spinal vascular lesions and tumors.

Neurosurgery 2012 Oct;71(4):773-84

Department of Neurological Surgery, University of Miami Miller School of Medicine, Lois Pope LIFE Center, Miami, Florida 33136-1060, USA.

Background: Although Onyx is widely used to embolize vascular lesions in adults, the safety and efficacy of this liquid embolic agent for use in children are not well studied.

Objective: To report our experience using Onyx in pediatric patients for a variety of cranial and spinal vascular lesions and tumors to determine its procedural complication rates, types, and clinical consequences and to highlight the indications for and principles of Onyx embolization in pediatric patients.

Methods: All pediatric Onyx embolization cases performed consecutively by the neuroendovascular services at our 2 institutions over a 5-year period were collected retrospectively and analyzed.

Results: Over the study period, 105 Onyx embolization procedures were performed in 69 pediatric patients with a mean follow-up of 112 days. Fifty-two patients harbored "primary" vascular lesions (malformations, fistulas, etc), whereas 17 patients had tumors. Complications occurred in 25 of 105 procedures (23.8%) and included ischemic infarct (7), asymptomatic nontarget embolization (4), intracerebral hemorrhage (3), microcatheter-related vessel perforation (3), retained microcatheter (2), cerebral edema (2), dimethyl sulfoxide-induced pulmonary edema (2), facial ischemia (1), and contrast-induced bronchospasm (1). Neurological morbidity occurred transiently after 10 procedures (9.5%) and permanently after 2 procedures (1.9%). There were no procedure-related deaths. Statistical analysis revealed no predictors of complications among the multiple potential risk factors evaluated.

Conclusion: Our experience suggests that Onyx can be used effectively for embolization of pediatric cranial and spinal vascular lesions and tumors with low permanent morbidity; however, attention must be paid to the technical nuances of and indications for its use to avoid potential complications.
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http://dx.doi.org/10.1227/NEU.0b013e3182648db6DOI Listing
October 2012

A multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VI.

Mol Genet Metab 2012 Sep 21;107(1-2):15-24. Epub 2012 Jul 21.

Department of Paediatric Neurosurgery, Birmingham Children's Hospital, Birmingham B4 6NH, UK.

Cervical cord compression is a sequela of mucopolysaccharidosis VI, a rare lysosomal storage disorder, and has devastating consequences. An international panel of orthopedic surgeons, neurosurgeons, anesthesiologists, neuroradiologists, metabolic pediatricians, and geneticists pooled their clinical expertise to codify recommendations for diagnosing, monitoring, and managing cervical cord compression; for surgical intervention criteria; and for best airway management practices during imaging or anesthesia. The recommendations offer ideal best practices but also attempt to recognize the worldwide spectrum of resource availability. Functional assessments and clinical neurological examinations remain the cornerstone for identification of early signs of myelopathy, but magnetic resonance imaging is the gold standard for identification of cervical cord compression. Difficult airways of MPS VI patients complicate the anesthetic and, thus, the surgical management of cervical cord compression. All patients with MPS VI require expert airway management during any surgical procedure. Neurophysiological monitoring of the MPS VI patient during complex spine or head and neck surgery is considered standard practice but should also be considered for other procedures performed with the patient under general anesthesia, depending on the length and type of the procedure. Surgical interventions may include cervical decompression, stabilization, or both. Specific techniques vary widely among surgeons. The onset, presentation, and rate of progression of cervical cord compression vary among patients with MPS VI. The availability of medical resources, the expertise and experience of members of the treatment team, and the standard treatment practices vary among centers of expertise. Referral to specialized, experienced MPS treatment centers should be considered for high-risk patients and those requiring complex procedures. Therefore, the key to optimal patient care is to implement best practices through meaningful communication among treatment team members at each center and among MPS VI specialists worldwide.
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http://dx.doi.org/10.1016/j.ymgme.2012.07.018DOI Listing
September 2012

Choroid plexus tumors in pediatric patients.

Br J Neurosurg 2012 Feb 4;26(1):32-7. Epub 2011 Oct 4.

Division of Neurosurgery, Children's Memorial Hospital, Chicago, IL, USA.

Objective: Choroid plexus tumors are rare intraventricular tumors, accounting for less than 1% of all intracranial tumors and 2-4% of brain tumors in children. The authors present their experience in the management of these lesions, and a review of the literature is performed.

Methods: We retrospectively analyzed the outcome of pediatric patients with choroid plexus tumors treated with surgical resection. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy and follow-up.

Results: This study involves 18 consecutive choroid plexus tumors: 14 papillomas, 2 atypical papillomas and 2 carcinomas. The tumor was located in the lateral ventricles (12), the fourth ventricle (4) and the third ventricle (2). The mean age at presentation was 4.6 years. Surgical resection was performed in all cases and no patients died perioperatively. Survival rate of papilloma patients was 100% without evidence of recurrent disease (mean follow-up for 73 months). Survival rate of carcinoma patients was 50% (mean follow-up for 23.5 months). One carcinoma patient died of disseminated disease 13 months after surgery. The functional outcome in long-term survivors after papilloma surgery was excellent. Postoperative extraventricular drainage (EVD) was performed in 12 patients. Five patients (27.8%) had persistent hydrocephalus after tumor resection and required a ventriculoperitoneal shunt.

Conclusion: Choroid plexus papilloma is a surgically curable disease. Postoperative EVD was considered effective in lowering the rate of shunt requirement through releasing the blood-tinged CSF and small particles of tumor residue.
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http://dx.doi.org/10.3109/02688697.2011.601820DOI Listing
February 2012

Retethering of transected fatty filum terminales.

J Neurosurg Pediatr 2011 Jan;7(1):42-6

Division of Neurosurgery, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614, USA.

Object: Untethering of a tethered spinal cord (TSC) by transecting or removing a fatty filum terminale is a relatively simple procedure that can prevent or ameliorate neurological symptoms, and the postoperative prognosis is usually good. Progressive neurological deterioration caused by recurrent tethering has been rarely reported. The authors present their experience in cases in which a sectioned fatty filum terminale has become retethered.

Methods: The authors retrospectively analyzed the surgical results of pediatric patients with fatty filum terminale-TSC treated by transection of the filum. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy, and follow-up data.

Results: Of the 225 children who underwent TSC release by sectioning the fatty filum from 1992 to 2005, there were 6 patients (2.7%; 3 males, 3 females) in whom the fatty filum retethered. The mean age at the first diagnosis of TSC was 5.2 years (range 2 months-12.3 years). The mean duration from the first untethering procedure to retethering was 5.4 years. The mean age at the time of retethering was 10.6 years (range 7-17.5 years). Symptoms of retethering were urinary incontinence, low-back pain, difficulty walking, constipation, leg pain, and worsening foot deformity. Patients underwent cystometrography at the time retethering was indicated by increased bladder capacity, large post-void residual volume, decreased bladder capacity, increase in filling pressure, and poor sensation of filling. Magnetic resonance imaging revealed adherence of the rostral stump of the sectioned filum to the midline dorsal dural surface. All patients underwent the second untethering procedure. Four patients improved neurologically and experienced no retethering thereafter (mean follow-up period 5.5 years). Two patients experienced additional retethering after temporary improvement following the second untethering procedure.

Conclusions: Retethering of the spinal cord is a rare condition occurring after the sectioning of a fatty filum terminale. Awareness of this rare sequela is necessary for appropriate long-term management of TSC caused by a fatty filum terminale. Cystometrography is useful for detecting the lesion and confirming the diagnosis of retethering.
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http://dx.doi.org/10.3171/2010.10.PEDS09550DOI Listing
January 2011