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Matthew J. McGirt, Frank J. Attenello, Ghazala Datoo, Muraya Gathinji, April Atiba, Jon D. Weingart, Benjamin Carson and George I. Jallo

Object

Indications for duraplasty in treatment of Chiari malformation Type I (CM-I) remain unclear. In the present study, the authors evaluate their surgical experience to determine whether intraoperative ultrasonography is effective in the selection of patients with CM-I who can be adequately treated with craniectomy alone without duraplasty.

Methods

The authors reviewed the records of 256 children who underwent first-time hindbrain decompression for CM-I. Craniectomy alone (without duraplasty) was performed when intraoperative ultrasonography suggested adequate decompression of the subarachnoid spaces ventral and dorsal to the tonsils after suboccipital craniectomy alone. Duraplasty was performed if intraoperative ultrasonography demonstrated persistent dural compression of the tonsils following craniectomy. Symptom recurrence as a function of time was compared between cases of duraplasty versus suboccipital decompression alone stratified by extent of tonsillar herniation.

Results

Duraplasty was performed in 140 patients (55%), and suboccipital decompression alone was performed in 116 patients (45%). Patients underwent follow-up for 29 ± 15 months. Symptoms included headache in 192 patients (75%) and lower cranial nerve and brainstem dysfunction in 68 (27%). In 38 patients (15%) there was tonsillar herniation rostral to the C-1 lamina, in 195 (76%) it extended between the C-1 and C-2 lamina, and in 23 patients (9%) there was herniation caudal to the lower border of the C-2 lamina. In children with tonsillar herniation caudal to C-1, ultrasonography-guided suboccipital decompression alone was associated with a 2-fold increase in the risk of symptom recurrence compared with those who also underwent duraplasty (p = 0.01). In children with tonsillar herniation rostral to C-1, outcome was equivalent between suboccipital decompression alone and duraplasty (p = 0.41).

Conclusions

In the setting of moderate-to-severe tonsillar CM-I, intraoperative ultrasonography demonstrating decompression of the subarachnoid spaces ventral and dorsal to the tonsils may not effectively select patients in whom bone decompression alone is sufficient. Duraplasty may be warranted in cases of tonsillar herniation that extends below the C-1 lamina regardless of intraoperative ultrasonography findings. More objective cerebrospinal fluid flow or volumetric measures may be needed intraoperatively to guide duraplasty in patients with more pronounced tonsillar herniation.

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Frank J. Attenello, Matthew J. McGirt, April Atiba, Muraya Gathinji, Ghazala Datoo, Jon Weingart, Benjamin Carson and George I. Jallo

Object

Chiari malformation Type I (CM-I) is often associated with scoliosis. It remains unclear which subgroups of patients are most likely to experience progression of spinal deformity after cervicomedullary decompression. The authors' goal was to determine the time frame of curvature progression and assess which patient subgroups are at greatest risk for progression of spinal deformity after surgery.

Methods

The authors retrospectively reviewed the records of all pediatric patients with significant scoliosis in whom suboccipital decompression was performed to treat for CM-I during a 10-year period at a single academic institution. Clinical, radiological, and operative variables were assessed as independent factors for failure (worsening of scoliosis) by using a univariate regression analysis.

Results

Twenty-one children (mean age 9 ± 3 years; 4 male) underwent hindbrain decompression for CM-I–associated scoliosis and were followed for a mean of 39 months. All patients harbored a syrinx. Eight patients (38%) experienced improvement in scoliosis curvature, whereas 10 (48%) suffered a progression. Thoracolumbar junction scoliosis (p = 0.04) and failure of the syrinx to improve (p = 0.05) were associated with 5- and 4-fold respective increases in the likelihood of deformity progression. Each increasing degree of preoperative Cobb angle was associated with an 11% increase in the likelihood of scoliotic curve progression (p < 0.05).

Conclusions

Over one third of patients with CM-I–associated scoliosis will improve after cervicomedullary decompression alone. Cervicomedullary decompression is a good first-line option, particularly in children with concordant posterior fossa symptoms. Patients presenting with more severe scoliosis (increasing Cobb angle) or scoliosis that crosses the thoracolumbar junction may benefit from earlier orthopedic involvement and should be monitored regularly for curvature progression after cervicomedullary decompression. In cases in which there is a failure of the syrinx to show improvement after suboccipital decompression, the patients are also more likely to develop curvature progression.

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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.