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  • Author or Editor: Joshua J. Chern x
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Hector E. James

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Mohammadali M. Shoja and Joshua J. Chern

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Editorial

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James M. Drake

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Brandon A. Miller, David I. Bass and Joshua J. Chern

Arteriovenous malformations (AVMs) are typically considered congenital lesions, although there is growing evidence for de novo formation of these lesions as well. The authors present the case of an AVM in the same cerebral cortex that had been affected by a severe traumatic brain injury (TBI) more than 6 years earlier. To the best of the authors' knowledge, this is the first report attributing the formation of an AVM directly to TBI.

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Joshua J. Chern, Akash J. Patel, Andrew Jea, Daniel J. Curry and Youssef G. Comair

Object

Focal cortical dysplasia (FCD) is an important cause of intractable epilepsy and is at times treatable by resection. The now widespread use of MR imaging and recent advancement of functional imaging have increased the number of patients undergoing surgical treatment for FCD. The objective of this review is to critically examine and to provide a summary of surgical series on FCD published since 2000.

Methods

Studies concerning surgery for FCD were identified from MEDLINE and references of selected articles and book chapters. Data from these included studies were summarized and analyzed to identify factors correlated with seizure outcome.

Results

Sixteen studies were identified, and 469 patients met our selection criteria. Seizure-free outcome at 1-year postoperatively was achieved in 59.7% of the patients. Children and adults were equally likely to benefit from the surgery. Complete resection (OR 13.7, 95% CI 6.68–28.1; p < 0.0001) and temporal location (OR 2.15, 95% CI 1.26–3.69; p = 0.0073) were two positive prognostic indicators of seizure-free outcome. Utilization of invasive monitoring did not affect the chance of seizure remission, but firm conclusions could not be drawn because patients were not randomized.

Conclusions

The advancement of modern imaging has transformed the process of surgical candidate selection for partial epilepsy due to FCD. Patients from recent surgical series were more homogeneous in their clinical presentations and might represent FCD as an independent pathological entity. This likely explained the improved surgical outcome for this group of patients. These reports also documented the increased utilization of functional imaging, but their efficacy needs to be verified with further studies.

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Joshua J. Chern, Andrew J. Tsung, William Humphries, Raymond Sawaya and Frederick F. Lang

Object

Intracranial hemorrhage (ICH) is a frequent complication found in leukemia patients with thrombocytopenia. At the University of Texas MD Anderson Cancer Center, when a leukemia patient is found to have ICH, a platelet transfusion is generally recommended until 50,000/μl is reached. The authors examine the feasibility and outcome of their intervention strategy in this study.

Methods

Records were reviewed from 76 consecutive leukemia patients with newly diagnosed ICH at the University of Texas MD Anderson Cancer Center from January 1, 2007, to December 31, 2009. Variables of interest included age, platelet count at presentation, leukemia subtype, history of trauma, Glasgow Coma Scale score at presentation, whether the 50,000/μl goal was reached after transfusion, and whether the patient was a transfusion responder (platelet count increase > 2000/μl/unit transfused). Outcome parameters were mortality rates at 72 hours and 30 days and imaging-documented hemorrhage progression.

Results

Thrombocytopenia was prevalent at the time of presentation (68 of 76 patients had platelet levels < 50,000/μl at presentation). Despite an aggressive transfusion protocol, only 24 patients reached the 50,000/μl target after an average of 16 units of transfusion. Death due to ICH occurred in 15 patients within the first 72 hours (mortality rate 19.7%). Death correlated with the presenting Glasgow Coma Scale score (p = 0.0075) but not with other transfusion-related parameters. A significant mortality rate was again observed after 30 days (32.7%). The 30-day mortality rate, however, was largely attributable to non-ICH related causes and correlated with patient age (p = 0.032) and whether the patient was a transfusion responder (p = 0.022). Reaching and maintaining a platelet count > 50,000/μl did not positively correlate with the 30-day mortality rate (p = 0.392 and 0.475, respectively).

Conclusions

Platelet transfusion in the setting of ICH in leukemia patients is undoubtedly necessary, but whether the transfusion threshold should be 50,000/μl remains unclear. Factors other than thrombocytopenia likely contribute to the overall poor prognosis.

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Joshua J. Chern, Amber J. Gordon, Martin M. Mortazavi, R. Shane Tubbs and W. Jerry Oakes

Object

In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the foramen magnum was the suspected cause. The label “Chiari 0” was used to categorize these unique cases with no tonsillar ectopia. All of the patients underwent posterior fossa decompression and duraplasty identical to the technique used to treat patients with CM-I. Significant syrinx and symptom resolution occurred in these patients. Herein, the authors report on a follow-up study of patients with CM-0 who were derived from over 400 operative cases of pediatric CM-I decompression.

Methods

The authors present their 12-year experience with this group of patients.

Results

Fifteen patients (3.7%) were identified. At surgery, many were found to have physical barriers to CSF flow near the foramen magnum. In most of them, the syringomyelia was greatly diminished postoperatively.

Conclusions

The authors stress that this subgroup represents a very small cohort among patients with Chiari malformations. They emphasize that careful patient selection is critical when diagnosing CM-0. Without an obvious CM-I, other etiologies of a spinal syrinx must be conclusively ruled out. Only then can one reasonably expect to ameliorate the clinical course of these patients via posterior fossa decompression.

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Andrew Reisner, Matthew F. Gary, Joshua J. Chern and J. Damien Grattan-Smith

Spinal cord infarctions following seemingly innocuous trauma in children are rare, devastating events. In the majority of these cases, the pathophysiology is enigmatic. The authors present 3 cases of pediatric spinal cord infarction that followed minor trauma. An analysis of the clinical, radiographic, and laboratory features of these cases suggests that thromboembolism of the nucleus pulposus into the spinal cord microcirculation is the likely mechanism. A review of the human and veterinary literature supports this notion. To the authors' knowledge, this is the largest pediatric series of myelopathy due to thromboembolism of the nucleus pulposus reported to date, and it is the first report of this condition occurring in an infant.