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Daniel M. Sciubba, Kaisorn L. Chaichana, Graeme F. Woodworth, Matthew J. McGirt, Ziya L. Gokaslan and George I. Jallo

Object

The indications remain unclear for fusion at the time of cervical laminectomy for intradural tumor resection. To identify patients who may benefit from initial fusion, the authors assessed clinical, radiological/imaging, and operative factors associated with subsequent symptomatic cervical instability requiring fusion after cervical laminectomy for intradural tumor resection.

Methods

The authors reviewed 10 years of data obtained in patients who underwent cervical laminectomy without fusion for intradural tumor resection and who had normal spinal stability and alignment preoperatively. The association of pre- and intraoperative variables with the subsequent need for fusion for progressive symptomatic cervical instability was assessed using logistic regression analysis, and percentages were compared using Fisher exact tests when appropriate.

Results

Thirty-two patients (mean age 41 ± 17 years) underwent cervical laminectomy without fusion for resection of an intradural tumor (18 intramedullary and 14 extramedullary). Each increasing number of laminectomies performed was associated with a 3.1-fold increase in the likelihood of subsequent vertebral instability (odds ratio 3.114, 95% confidence interval 1.207–8.034, p = 0.02). At a mean follow-up interval of 25.2 months, 33% (4 of 12) of the patients who had undergone a ≥ 3-level laminectomy required subsequent fusion compared with 5% (1 of 20) who had undergone a ≤ 2-level laminectomy (p = 0.03). Four (36%) of 11 patients initially presenting with myelopathic motor disturbance required subsequent fusion compared with 1 (5%) of 21 presenting initially with myelopathic sensory or radicular symptoms (p = 0.02). Age, the presence of a syrinx, intramedullary tumor, C-2 laminectomy, C-7 laminectomy, and laminoplasty were not associated with subsequent symptomatic instability requiring fusion.

Conclusions

In the authors' experience with intradural cervical tumor resection, patients presenting with myelopathic motor symptoms or those undergoing a ≥ 3-level cervical laminectomy had an increased likelihood of developing subsequent symptomatic instability requiring fusion. A ≥ 3-level laminectomy with myelopathic motor symptoms may herald patients most likely to benefit from cervical fusion at the time of tumor resection.

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Lori C. Jordan, George I. Jallo and Philippe Gailloud

✓ The authors report the case of a 4-year-old boy with a spontaneous intracerebral hemorrhage (ICH) related to an arteriovenous malformation (AVM) that was not found with good-quality magnetic resonance (MR) imaging and MR angiography. Both modalities were used serially in the acute phase and at 2 and 7 months of follow-up. Digital subtraction angiography identified the peripheral AVM when the patient experienced rehemorrhaging 1 year after his initial presentation. This case illustrates the need for a complete diagnostic evaluation including conventional angiography in cases of idiopathic ICH in children.

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George I. Jallo

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Matthew J. McGirt, Kaisorn L. Chaichana, April Atiba, Ali Bydon, Timothy F. Witham, Kevin C. Yao and George I. Jallo

Object

Gross-total resection of pediatric intramedullary spinal cord tumor (IMSCT) can be achieved in the majority of cases while preserving long-term neurological function. Nevertheless, postoperative progressive spinal deformity often complicates functional outcome years after surgery. The authors set out to determine whether laminoplasty in comparison with laminectomy has reduced the incidence of subsequent spinal deformity requiring fusion after IMSCT resection at their institution.

Methods

The first 144 consecutive patients undergoing resection of IMSCTs at a single institution underwent laminectomy with preservation of facet joints. The next 20 consecutive patients presenting for resection of IMSCTs underwent osteoplastic laminotomy regardless of patient or tumor characteristics. All patients were followed up with telephone interviews corroborated by medical records for the following outcomes: 1) neurological and functional status (modified McCormick Scale [MMS] score and Karnofsky Performance Scale [KPS] score); and 2) development of progressive spinal deformity requiring fusion. The incidence of progressive spinal deformity and the long-term neurological function were compared between the laminectomy and osteoplastic laminotomy cohorts. The means are expressed ± the standard deviation.

Results

Overall, the patients' mean age was 8.6 ± 5 years, and they presented with median MMS scores of 2 (interquartile range [IQR] 2–4). A > 95% resection was achieved in 125 cases (76%). There were no differences (p > 0.10) between patients treated with osteoplastic laminotomy and those treated with laminectomy in terms of the following characteristics: age; sex; duration of symptoms; location of tumor; incidence of preoperative scoliosis (Cobb angle > 10°: 7 [35%] with laminoplasty compared with 49 [34%] with laminectomy); involvement of the cervicothoracic junction (7 [35%] compared with 57 [40%]); thoracolumbar junction (4 [20%] compared with 36 [25%]); tumor size; extent of resection; radiation therapy; histopathological findings; or mean operative spinal levels (7.5 ± 2 compared with 7.5 ± 3). Nevertheless, patients who underwent osteoplastic laminotomy had better median preoperative MMS scores than those treated with laminectomy (2 [IQR 2–2] compared with 2 [IQR 2–4]; p = 0.04). A median of 3.5 years (IQR 1–7 years) after surgery, only 1 patient (5%) in the osteoplastic laminotomy cohort required fusion for progressive spinal deformity, compared with 43 (30%) in the laminectomy cohort (p = 0.027). Adjusting for the inter-cohort difference in preoperative MMS scores, osteoplastic laminotomy was associated with a 7-fold reduction in the odds of subsequent fusion for progressive spinal deformity (odds ratio 0.13, 95% confidence interval 0.02–1.00; p = 0.05). The median MMS and KPS scores were similar between patients who underwent osteoplastic laminotomy and those in whom laminectomy was performed (MMS Score 2 [IQR 2–3] for laminotomy compared with 2 [IQR 2–4] for laminectomy, p = 0.54; KPS Score 90 [IQR 70–100] for laminotomy compared with 90 [IQR 80–90] for laminectomy, p = 0.545) at a median of 3.5 years after surgery.

Conclusions

In the authors' experience, osteoplastic laminotomy for the resection of IMSCT in children was associated with a decreased incidence of progressive spinal deformity requiring fusion but did not affect long-term functional outcome. Laminoplasty used for pediatric IMSCT resection may decrease the incidence of progressive spinal deformity requiring subsequent spinal stabilization in some patients.

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Matthew J. McGirt, Kaisorn L. Chaichana, April Atiba, Frank Attenello, Kevin C. Yao and George I. Jallo

Object

With modern surgical advances, radical resection of pediatric intramedullary spinal cord tumors (IMSCTs) can be expected to preserve long-term neurological function. Nevertheless, postoperative neurological decline is not uncommon after surgery, and many patients continue to experience long-term dysesthetic symptoms. Preoperative predictors of postoperative neurological decline and sensory syndromes have not been investigated and may serve as a guide for surgical risk stratification.

Methods

Neurological function (as determined using the modified McCormick Scale [mMS]) preoperatively, postoperatively, and 3 months after surgery was retrospectively recorded from patient charts in 164 consecutive patients undergoing resection of IMSCTs. A median 4 years (interquartile range [IQR] 1–8 years) after surgery, long-term motor and sensory symptoms were assessed by telephone interviews and corroborated by subsequent medical visits in 120 available patients. This long-term assessment was retrospectively reviewed for the purposes of this study. The authors reviewed this series to assess long-term motor, sensory, and urinary outcomes and to determine independent risk factors of postoperative neurological decline and long-term sensory dysfunction.

Results

Patients were 8.6 ± 5.7 years old and presented with a median mMS of 2 (IQR 2–4). Three months after surgery, 38 patients (23%) continued to experience decreased neurological function (1 mMS point) incurred perioperatively. Increasing age (p = 0.028), unilateral symptoms (p = 0.046), and urinary dysfunction at presentation (p = 0.004) independently predicted persistent 3-month perioperative decline. At long-term follow-up (median 4 years), 39 (33%) exhibited improvements in their mMS scores, 13 (30%) had improvement in their urinary dysfunction, and 27 (30%) had resolution of their dysesthesias. Seventy-eight patients (65%) experienced long-term dysesthetic symptoms. Increasing age (p = 0.024), preoperative symptom duration > 12 months (p = 0.027), and worsened postoperative mMS score at hospital discharge (p = 0.013) independently increased the risk of long-term dysesthesias.

Conclusions

In the authors' experience, nearly one third of patients may experience improvement in motor, sensory, and urinary dysfunction years after IMSCT resection, whereas the majority will continue to experience long-term dysesthetic symptoms. Improvement in motor deficits preceded improvement in sensory syndromes, and urinary dysfunction typically resolved much longer after surgery. The risk of persistent perioperative motor decline was increased with older age, unilateral symptoms, preoperative urinary symptoms, and less severe preoperative neurological deficit. The risk of long-term dysesthesias was increased with older age, increased duration of symptoms prior to resection, and greater postoperative neurological deficit.

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Kevin C. Yao, Matthew J. McGirt, Kaisorn L. Chaichana, Shlomi Constantini and George I. Jallo

Object

Gross-total resection of pediatric intramedullary spinal cord tumors (IMSCTs) can be achieved in the majority of cases, with preservation of long-term neurological function. However, progressive spinal deformity requiring subsequent fusion occurs in many cases. It remains unknown which subgroups of patients have the greatest risk for progressive spinal deformity.

Methods

Data for 161 patients undergoing resection of IMSCTs at a single institution were retrospectively collected and analyzed with regard to the development of progressive spinal deformity requiring fusion and patient functional status (based on the modified McCormick Scale [mMS] and Karnofsky Performance Scale [KPS]) by conducting telephone interviews corroborated by medical records. The independent association of all clinical, radiographic, and operative variables to subsequent progressive spinal deformity was assessed using multivariate logistic regression analysis.

Results

Patients were a mean of 8.6 ± 5.7 years old at the time of surgery. The tumor spanned a mean of six ± three spinal levels. Preoperative scoliotic deformity was present in 56 cases (35%). Seventy-six patients (47%) had undergone a previous biopsy procedure, and 28 (17%) a prior resection. Gross-total resection (> 95%) was achieved in 122 cases (76%). A median of 9 years (range 1–21) after surgery, progressive spinal deformity requiring fusion developed in 43 patients (27%). The median functional scores at the last follow-up were worse in patients who required fusion compared with those who did not (mMS: 3 compared with 2, p = 0.006; KPS: 80 compared with 90, p = 0.04) despite similar mMS scores between the groups at 3 months postoperatively. An age less than 13 years, preoperative scoliotic deformity (Cobb angle > 10°), involvement of the thoracolumbar junction, and tumor-associated syrinx independently increased the odds of a postoperative progressive deformity requiring fusion 4.4-, 3.2-, 2.6-, and 3.4-fold, respectively (p < 0.05). Each subsequent resection increased the odds of a progressive deformity 1.8-fold (p < 0.05). Symptoms lasting less than 1 month before resection decreased the odds of spinal deformity requiring fusion ninefold (p < 0.05).

Conclusions

Progressive spinal deformity requiring fusion occurred in 27% of children undergoing resection of an IMSCT and was associated with a decreased functional status. Preoperative scoliotic deformity, an increasing number of resections, an age less than 13 years, tumor-associated syrinx, and surgery spanning the thoracolumbar junction increased the risk for progressive spinal deformity. Patients possessing one or more of these characteristics should be monitored closely for progressive spinal deformity following surgery.

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Samuel Pak, George I. Jallo, Ann Biser and Harvey A. Ziessman

Object

Baclofen pump infusion systems are used for the treatment of patients with severe spasticity. When symptoms do not respond to infusion rate increases, the cause may be pump malfunction or catheter obstruction. The purpose of this investigation was to review the authors' experience with indium-111 diethylene-triamine-pentaacetic acid (In-111 DTPA) scintigraphy for evaluation of baclofen infusion system function and patency and to correlate scintigraphic findings with patient treatment and outcome to determine optimal methodology and diagnostic criteria.

Methods

Ten scintigraphic studies acquired in eight patients (five adults, three adolescents) were retrospectively reviewed. Imaging was routinely performed at 4, 24, 48, and 72 hours after injection of In-111 DTPA into the pump reservoir. The results of the studies were interpreted by two nuclear medicine physicians who had no knowledge of the patients' clinical history or outcome. The scintigraphic findings (for example, radiotracer detected in the basal cisterns, cervical or thoracolumbar regions, infusion catheter, and pump) were recorded and correlated with neuroimaging findings, patient history, treatment, and clinical response to therapy.

Results

In cases in which the basal cistern could be visualized at any time and those in which radiotracer was visualized in the thoracolumbar intrathecal region within 48 hours, the pump systems were functional and symptoms responded to an increase in infusion rate. One patient in whom activity was visualized in the thoracolumbar intrathecal region by 72 hours also experienced clinical improvement with a flow rate increase. Visualization of the pump alone or only the pump and the subcutaneous catheter was consistent with catheter obstruction or pump malfunction.

Conclusion

The use of In-111 DTPA scintigraphy of intrathecal baclofen infusion systems can play an important role in determining the functional status of these systems and guiding subsequent patient care.

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Daniel M. Sciubba, Joseph C. Noggle, Neena I. Marupudi, Carlos A. Bagley, Markus J. Bookland, Benjamin S. Carson Sr., Michael C. Ain and George I. Jallo

Object

Achondroplasia is a hereditary form of dwarfism caused by a defect in endochondral bone formation, resulting in skeletal abnormalities including short stature, shortened limb bones, macrocephaly, and small vertebral bodies. In the pediatric population, symptomatic spinal stenosis occurs at all spinal levels due to the abnormally narrow bone canal. In this study, clinical outcomes were assessed in children with achondroplasia after spinal canal decompression.

Methods

A retrospective review was conducted involving pediatric patients with heterozygous achondroplasia and symptomatic stenosis after decompressive procedures at the authors' institution within a 9-year period. Measured outcomes included resolution of symptoms, need for repeated surgery, presence of fusion, development of deformity, and complications.

Forty-four pediatric patients underwent a total of 60 decompressive procedures. The average patient age at surgery was 12.7 years (range 5–21 years). Forty-nine operations were performed for initial treatment of stenosis, and 11 were performed as revision surgeries on previously operated levels. A large proportion of patients (> 60%) required additional cervicomedullary decompressions, most often preceding the symptoms of spinal stenosis. Of the initial procedures, decompression locations included 32 thoracolumbar (65%), 10 lumbar (20%), four cervical (8%), two cervicothoracic (4%), and one thoracic (2%). Forty-three of the decompressive procedures (72%) included spinal fusion procedures. Of the 11 revisions, five were fusion procedures for progressive deformity at levels previously decompressed but not fused (all thoracolumbar), five were for decompressions of symptomatic junctional stenosis with extension of fusion, and one was for repeated decompression at the same level due to recurrence of symptomatic stenosis.

Conclusions

Decompression of the spinal canal in pediatric patients with achondroplasia can be accomplished safely with significant clinical benefit. Patients with a history of cervicomedullary compression may be at an increased risk of developing symptomatic stenosis prior to adolescence. Fusion procedures are recommended in patients with a large decompression overlying a thoracolumbar kyphosis to avoid progressive postoperative deformity.

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Daniel M. Sciubba, Li-Mei Lin, Graeme F. Woodworth, Matthew J. McGirt, Benjamin Carson and George I. Jallo

Object

Antibiotic-impregnated shunt (AIS) systems may decrease the incidence of cerebrospinal fluid (CSF) shunt infections. However, there is a reluctance to use AIS components because of their increased cost. In the present study the authors evaluated factors contributing to the medical costs associated with the treatment of CSF shunt infections in a hydrocephalic pediatric population, those implanted with AIS systems compared with those implanted with standard shunt systems.

Methods

The authors retrospectively reviewed data obtained in all pediatric patients who had undergone CSF shunt insertion at their institution over a 3-year period. All patients were followed up for 12 months after surgery. The independent association between AIS catheter use and subsequent shunt infection was assessed by performing a multivariate proportional hazards regression analysis. Factors contributing to the medical costs associated with shunt infection were evaluated.

Results

Two hundred eleven pediatric patients underwent 353 shunting procedures. Two hundred eight shunts (59%) were placed with nonimpregnated catheters and 145 shunts (41%) were placed with AIS catheters. Twenty-five patients (12%) with non-AIS catheters experienced shunt infection, whereas only two patients (1.4%) with AIS catheters had a shunt infection within the 6-month follow-up period (p < 0.01). Among infected patients, infected patients with standard shunt components had a longer average hospital stay, more inpatient complications related to infection treatment, and more multiple organism infections and multiple antibiotic regimens, compared with those with AIS components.

Conclusions

Although individual AIS components are more expensive than standard ones, factors contributing to medical costs are fewer in pediatric patients with infected shunts when the components are antibiotic-impregnated rather than standard.