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  • Author or Editor: Hugh J. L. Gartonx
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Cormac O. Maher, Hugh J. L. Garton, Wajd N. Al-Holou, Jonathan D. Trobe, Karin M. Muraszko, and Eric M. Jackson

Object

Arachnoid cysts may occasionally be associated with subdural hygromas. The management of these concurrent findings is controversial.

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The authors reviewed their experience with arachnoid cysts and identified 8 patients with intracranial arachnoid cysts and an associated subdural hygroma. The medical records and images for these patients were also examined.

Results

In total, 8 patients presented with concurrent subdural hygroma and arachnoid cyst. Of these 8 patients, 6 presented with headaches and 4 had nausea and vomiting. Six patients had a history of trauma. One patient was treated surgically at the time of initial presentation, and 7 patients were managed without surgery. All patients experienced complete resolution of their presenting signs and symptoms.

Conclusions

Subdural hygroma may lead to symptomatic presentation for otherwise asymptomatic arachnoid cysts. The natural course of cyst-associated subdural hygromas, even when symptomatic, is generally benign, and symptom resolution can be expected in most cases. The authors suggest that symptomatic hygroma is not an absolute indication for surgical treatment and that expectant management can result in good outcomes in many cases.

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Wajd N. Al-Holou, Andrew Y. Yew, Zackary E. Boomsaad, Hugh J. L. Garton, Karin M. Muraszko, and Cormac O. Maher

Object

Arachnoid cysts are a frequent finding on intracranial imaging in children. The prevalence and natural history of these cysts are not well defined. The authors studied a large consecutive series of children undergoing MR imaging to better define both the MR imaging–demonstrated prevalence and behavior of these lesions over time.

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The authors reviewed a consecutive series of 11,738 patients who were 18 years of age or younger and had undergone brain MR imaging at a single institution during an 11-year period. In the patients in whom intracranial arachnoid cysts were identified, clinical and demographic information was recorded and imaging characteristics, such as cyst size and location, were evaluated. Prevalence data were analyzed using univariate and multivariate logistic regression, linear regression, and ANOVA. All patients with sufficient data (repeat MR imaging studies as well as repeated clinical evaluation over at least 5 months) for a natural history analysis were identified. This group was assessed for any change in symptoms or imaging appearance during the follow-up interval.

Results

Three hundred nine arachnoid cysts (2.6% prevalence rate) were identified. There was an increased prevalence of arachnoid cysts in males (p < 0.000001). One hundred eleven patients met all criteria for inclusion in the natural history analysis. After a mean follow-up of 3.5 years, 11 arachnoid cysts increased in size, 13 decreased, and 87 remained stable. A younger age at presentation was significantly associated with cyst enlargement (p = 0.001) and the need for surgery (p = 0.05). No patient older than 4 years of age at the time of initial diagnosis had cyst enlargement, demonstrated new symptoms, or underwent surgical treatment.

Conclusions

Arachnoid cysts are a common incidental finding on intracranial imaging in pediatric patients. An older age at the time of presentation is associated with a lack of clinical or imaging changes over time.

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Cordelie E. Witt, Anthony C. Wang, Cormac O. Maher, Khoi D. Than, Hugh J. L. Garton, and Karin M. Muraszko

In this report, the authors describe the first known case of inducible hemifacial weakness in a patient with Chiari malformation Type I (CM-I). The patient was a 14-year-old girl with a 1-year history of right facial paresis induced by sustained leftward head rotation. These episodes were characterized by weak activation of her right facial muscles with preserved eye opening and closure. Additionally, she had hypernasal speech, persistent headaches, and intermittent left arm twitching. Magnetic resonance imaging demonstrated a CM-I. A suboccipital craniectomy and C-1 laminectomy were performed for decompression of the CM-I, with duraplasty and coagulation of the pial surface of the cerebellar tonsils. At the 9-month follow-up, the patient's inducible hemifacial weakness had completely resolved. Her symptoms were thought to have resulted from the CM-I, perhaps due to traction on the right facial nerve by the ectopic tonsils with head rotation.

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Katherine G. Holste, Jack Vernamonti, Momodou G. Bah, Karin M. Muraszko, Samir K. Gadepalli, Cormac O. Maher, and Hugh J. L. Garton

OBJECTIVE

Necrotizing enterocolitis (NEC) and posthemorrhagic hydrocephalus are both conditions that can affect preterm infants. The peritoneum is the preferred terminus for shunt placement, but another terminus is sometimes used due to subjective concerns about infection and complications related to NEC. The aim of this study was to examine the rates of ventriculoatrial (VA) and ventriculoperitoneal (VP) shunt infection and failure in pediatric patients with a history of NEC.

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一个单中心回顾性研究of medical records from 2009 to 2021 was performed to identify pediatric patients with NEC who underwent shunt placement before 2 years of age. Patients were excluded if shunt placement preceded NEC diagnosis. Patient demographic characteristics, timing of shunt placement, type of shunt, shunt infections or revisions, and timing and management of NEC were extracted. The Student t-test and Fisher exact test were used to calculate significance. Kaplan-Meier curves were calculated.

RESULTS

Twenty-two patients met the inclusion criteria. Most patients underwent VP shunt placement (16 [71.4%]). Patients who underwent surgical management of NEC compared with those who underwent medical management were more likely to have a VA shunt placed (p = 0.02). One VA shunt and 3 VP shunts became infected during follow-up (p = 0.7). The mean time until infection was not significantly different between VA and VP shunts (p = 0.73). Significantly more VA shunts required revision (83% vs 31%, p = 0.04), and VA shunts had a significantly shorter time until failure (3.0 ± 0.8 vs 46.3 ± 7.55 months, p = 0.03).

CONCLUSIONS

VP shunts had a significantly longer time until failure than VA shunts; these shunts had similar infection rates in infants with prior NEC. When feasible, neurosurgeons and pediatric general surgeons can consider placing a VP shunt even if the patient has a history of NEC.

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Katherine G. Holste, Clare M. Wieland, Mohannad Ibrahim, Hemant A. Parmar, Sara Saleh, Hugh J. L. Garton, and Cormac O. Maher

OBJECTIVE

Benign expansion of the subarachnoid spaces (BESS) is a condition seen in macrocephalic infants. BESS is associated with mild developmental delays which tend to resolve within a few years. It is accepted that patients with BESS are at increased risk of spontaneous subdural hematomas (SDHs), although the exact pathophysiology is not well understood. The prevalence of spontaneous SDH in BESS patients is poorly defined, with only a few large single-center series published. In this study the authors aimed to better define BESS prevalence and developmental outcomes through the longitudinal review of a large cohort of BESS patients.

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A large retrospective review was performed at a single institution from 1995 to 2020 for patients 2 years of age or younger with a diagnosis of BESS by neurology or neurosurgery and head circumference > 85th percentile. Demographic data, head circumference, presence of developmental delay, occurrence of SDH, and need for surgery were extracted from patient charts. The subarachnoid space (SAS) size was measured from the available MR images, and the sizes of those who did and did not develop SDH were compared.

RESULTS

自由文本本身arch revealed BESS mentioned within the medical records of 1410 of 2.6 million patients. After exclusion criteria, 480 patients remained eligible for the study. Thirty-two percent (n = 154) of patients were diagnosed with developmental delay, most commonly gross motor delay (53%). Gross motor delay resolved in 86% of patients at a mean age of 22.2 months. The prevalence of spontaneous SDH in this BESS population over a period of 25 years was 8.1%. There was no significant association between SAS size and SDH formation.

CONCLUSIONS

This study represents results for one of the largest cohorts of patients with BESS at a single institution. Gross motor delay was the most common developmental delay diagnosed, and a majority of patients had resolution of their delay. These data support that children with BESS have a higher prevalence of SDH than the general pediatric population, although SAS size was not significantly associated with SDH development.

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Shawn L. Hervey-Jumper, Hugh J. L. Garton, Darryl Lau, David Altshuler, Douglas J. Quint, Patricia L. Robertson, Karin M. Muraszko, and Cormac O. Maher

Object

Vascular endothelial growth factor (VEGF) is the major proangiogenic factor in many solid tumors. Vascular endothelial growth factor receptor (VEGFR) is expressed in abundance in pediatric patients with medulloblastoma and is associated with tumor metastasis, poor prognosis, and proliferation. Gadolinium enhancement on MRI has been suggested to have prognostic significance for some tumors. The association of VEGF/VEGFR and Gd enhancement in medulloblastoma has never been closely examined. The authors therefore sought to evaluate whether Gd-enhancing medulloblastomas have higher levels of VEGFR and CD31. Outcomes and survival in patients with enhancing and nonenhancing tumors were also compared.

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A retrospective analysis of patients with enhancing, nonenhancing, and partially enhancing medulloblastomas was performed. Primary end points included risk stratification, extent of resection, and perioperative complications. A cohort of 3 enhancing and 3 nonenhancing tumors was selected for VEGFR and CD31 analysis as well as microvessel density measurements.

Results

Fifty-eight patients were analyzed, and 20.7% of the medulloblastomas in these patients were nonenhancing. Enhancing medulloblastomas exhibited strong VEGFR1/2 and CD31 expression relative to nonenhancing tumors. There was no significant difference in perioperative complications or patient survival between the 2 groups.

Conclusions

These results suggest that in patients with medulloblastoma the presence of enhancement on MRI may correlate with increased vascularity and angiogenesis, but does not correlate with worse patient prognosis in the short or long term.

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Michael J. Cools, Wajd N. Al-Holou, William R. Stetler Jr., Thomas J. Wilson, Karin M. Muraszko, Mohannad Ibrahim, Frank La Marca, Hugh J. L. Garton, and Cormac O. Maher

Object

Filum terminale lipomas (FTLs) are being identified with increasing frequency due to the increasing utilization of MRI. Although an FTL may be associated with tethered cord syndrome (TCS), in many cases FTLs are diagnosed incidentally in patients without any symptoms of TCS. The natural history of FTLs is not well defined.

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The authors searched the clinical and imaging records at a single institution over a 14-year interval to identify patients with FTLs. For patients with an FTL, the clinical records were reviewed for indication for imaging, presenting symptoms, perceived need for surgery, and clinical outcome. A natural history analysis was performed using all patients with more than 6 months of clinical follow-up.

Results

A total of 436 patients with FTL were identified. There were 217 males and 219 females. Of these patients, 282 (65%) were adults and 154 (35%) were children. Symptoms of TCS were present in 22 patients (5%). Fifty-two patients underwent surgery for FTL (12%). Sixty-four patients (15%) had a low-lying conus and 21 (5%) had a syrinx. The natural history analysis included 249 patients with a mean follow-up time of 3.5 years. In the follow-up period, only 1 patient developed new symptoms.

Conclusions

Filum terminale lipomas are a common incidental finding on spinal MRI, and most patients present without associated symptoms. The untreated natural history is generally benign for asymptomatic patients.

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Silky Chotai, Jeffrey L. Nadel, Katherine G. Holste, James M. Mossner, Brandon W. Smith, Joseph R. Kapurch, Karin M. Muraszko, Hugh J. L. Garton, Cormac O. Maher, and Jennifer M. Strahle

OBJECTIVE

The objective of this study was to understand the natural history of scoliosis in patients with Chiari malformation type I (CM-I) with and without syringomyelia.

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A retrospective review of data was conducted. Patients with CM-I were identified from a cohort of 14,118 individuals age 18 years or younger who had undergone MRI over an 11-year period at the University of Michigan. Patients eligible for study inclusion had a coronal curve ≥ 10° on radiography, associated CM-I with or without syringomyelia, and at least 1 year of clinical follow-up prior to any surgery. Curve magnitude at initial diagnosis, prior to posterior fossa decompression (PFD; if applicable), and at the last follow-up (prior to any surgical correction of scoliosis) was recorded, and clinical and radiographic characteristics were noted. The change in curve magnitude by 10° was defined as curve progression (increase by 10°) or regression (decrease by 10°).

RESULTS

Forty-three patients met the study inclusion criteria and were analyzed. About one-third (35%) of the patients presented with symptoms attributed to their CM-I. The mean degree of scoliosis at presentation was 32.6° ± 17.7°. Twenty-one patients (49%) had an associated syrinx. The mean tonsil position below the level of the foramen magnum was 9.8 ± 5.8 mm. Patients with a syrinx were more likely to have a curve > 20° (86% vs 41%, p = 0.002). Curve magnitude remained stable (≤ ±10°) in 77% of patients (33/43), progressed in 16% (7/43), and regressed in 7% (3/43). Mean age was higher (14.8 ± 0.59 years) among patients with regressed curves (p = 0.026). All regressed curves initially measured ≤ 20° (mean 14° ± 5.3°), and none of the patients with regressed curves had a syrinx. The change in curve magnitude was statistically similar in patients with (7.32° ± 17.7°) and without (5.32° ± 15.8°) a syrinx (p = 0.67). After a mean follow-up of 3.13 ± 2.04 years prior to surgery, 27 patients (63%) ultimately underwent posterior fossa or scoliosis correction surgery. For those who eventually underwent PFD only, the rate of change in curve magnitude prior to surgery was 0.054° ± 0.79°. The rate of change in curve magnitude was statistically similar before (0.054° ± 0.79°) and after (0.042° ± 0.33°) surgery (p = 0.45) for patients who underwent PFD surgery only.

CONCLUSIONS

The natural history of scoliosis in the presence of CM-I is variable, though most curves remained stable. All curves that regressed were ≤ 20° at initial diagnosis, and most patients in such cases were older at scoliosis diagnosis. Patients who underwent no surgery or PFD only had similar profiles for the change in curve magnitude, which remained relatively stable overall, as compared to patients who underwent PFD and subsequent fusion, who demonstrated curve progression. Among the patients with a syrinx, no curves regressed, most remained stable, and some progressed. Understanding this variability is a first step toward building a prediction model for outcomes for these patients.

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Jennifer Strahle, Ndi Geh, Béla J. Selzer, Regina Bower, Mai Himedan, MaryKathryn Strahle, Nicholas M. Wetjen, Karin M. Muraszko, Hugh J. L. Garton, and Cormac O. Maher

OBJECT

There is currently no consensus on the safety of sports participation for patients with Chiari I malformation (CM-I). The authors' goal was to define the risk of sports participation for children with the imaging finding of CM-I.

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A prospective survey was administered to 503 CM-I patients at 2 sites over a 46-month period. Data were gathered on imaging characteristics, treatment, sports participation, and any sport-related injuries. Additionally, 81 patients completed at least 1 subsequent survey following their initial entry into the registry and were included in a prospective group, with a mean prospective follow-up period of 11 months.

RESULTS

Of the 503 CM-I patients, 328 participated in sports for a cumulative duration of 4641 seasons; 205 of these patients participated in contact sports. There were no serious or catastrophic neurological injuries. One patient had temporary extremity paresthesias that resolved within hours, and this was not definitely considered to be related to the CM-I. In the prospective cohort, there were no permanent neurological injuries.

CONCLUSIONS

No permanent or catastrophic neurological injuries were observed in CM-I patients participating in athletic activities. The authors believe that the risk of such injuries is low and that, in most cases, sports participation by children with CM-I is safe.

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Shokei Yamada and Daniel J. Won

Abstract (N. R. Selden)

Object

Controversy exists regarding proper indications for surgical lysis of the terminal filum in children with voiding dysfunction and tethered spinal cord. Recently, surgery has been offered to children who have a normally positioned conus medullaris and no terminal filum abnormality visible on 1.5-tesla magnetic resonance images (referred to as minimal or occult tethered cord syndrome [TCS]). The author evaluates existing clinical and scientific evidence relevant to this controversy.

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Five retrospective, observational, noncontrolled studies of surgical terminal filum lysis for occult TCS in children were identified. Two further studies in which the authors reported surgical results in children with a normal-level conus medullaris were also identified.

Conclusions

These studies document encouraging clinical outcomes following surgery. Clinicopathological evidence suggests that occult TCS may result from radiographically occult structural abnormalities of the terminal filum. Although a preponderance of Class III clinical evidence supports the use of surgical filum lysis to treat occult TCS, no Class I or II evidence exists. Clinical practice varies; therefore, performance of a prospective randomized clinical trial of surgical terminal filum lysis for the treatment of occult TCS is advocated.

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