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Mark B. Ellis, Daniel Gridley, Suresh Lal, Geetha R. Nair and Iman Feiz-Erfan

Phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMT-MCT) are tumors that may cause tumor-induced osteomalacia and rarely appear intracranially. The authors describe the case of an 8-year-old girl who was found to have PMT-MCT with involvement of the cerebellar hemisphere and a small tumor pedicle breaching the dura mater and involving the skull. This was removed surgically in gross-total fashion without further complication. Histologically the tumor was confirmed to be a PMT-MCT. There was no evidence of tumor-induced osteomalacia. At the 42-month follow-up, the patient is doing well, has no abnormalities, and is free of recurrence. PMT-MCTs are rare tumors that may involve the brain parenchyma. A gross-total resection may be effective to cure these lesions.

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Tsinsue Chen, Karam Moon, Daphne E. deMello, Iman Feiz-Erfan, Nicholas Theodore and Ratan D. Bhardwaj

A 13-year-old boy presented with fever and neck pain and stiffness, which was initially misdiagnosed as culture-negative meningitis. Magnetic resonance images of the brain and cervical spine demonstrated what appeared to be an intradural extramedullary mass at the C1–3 level, resulting in moderate cord compression, and a Chiari Type I malformation. The patient underwent a suboccipital craniectomy and a C1–3 laminectomy with intradural exploration for excisional biopsy and resection. The lesion containing the parasite was extradural, extending laterally through the C2–3 foramina. Inflammatory tissue secondary to Onchocerca lupi infection was identified, and treatment with steroids and doxycycline was initiated. At the 6-month follow-up, the patient remained asymptomatic, with MR images demonstrating a significant reduction in lesional size. However, 10 weeks postoperatively, the infection recurred, necessitating a second operation. The patient was treated with an additional course of doxycycline and is currently maintained on ivermectin therapy. This is the second reported case of cervical O. lupi infection in a human. In the authors' experience, oral doxycycline alone was insufficient in controlling the disease, and the addition of ivermectin therapy was necessary.

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M. Yashar S. Kalani, Samuel Kalb, Nikolay L. Martirosyan, Salvatore C. Lettieri, Robert F. Spetzler, Randall W. Porter and Iman Feiz-Erfan

Object

Resection of cancer and the involved artery in the neck has been applied with some success, but the indications for such an aggressive approach at the skull base are less well defined. The authors therefore evaluated the outcomes of advanced skull base malignancies in patients who were treated with bypass and resection of the internal carotid artery (ICA).

Methods

The authors retrospectively reviewed the charts of all patients with advanced head and neck cancers who underwent ICA sacrifice with revascularization in which an extracranial-intracranial bypass was used between 1995 and 2010 at the Barrow Neurological Institute.

Results

Eighteen patients (11 male and 7 female patients; mean age 46 years, range 7–69 years) were identified. There were 4 sarcomas and 14 carcinomas that involved the ICA at the skull base. All patients underwent ICA sacrifice with revascularization. One patient died of a stroke after revascularization. A second patient died of the effects of a fistula between the oral and cranial cavities (surgery-related mortality rate 11.1%). Eight months after the operation, 1 patient developed occlusion of the bypass and died. Complications associated with the bypass surgery included 1 case of subdural hematoma (SDH) with blindness, 1 case of status epilepticus, and 1 case of asymptomatic bypass occlusion (bypass-related morbidity 16.7%). Complications associated with tumor resection included 3 cases of CSF leakage requiring repair and shunting, 1 case of hydrocephalus requiring shunting, 1 case of SDH, and 1 case of contralateral ICA injury requiring a bypass (tumor resection morbidity rate 33.3%). In 1 patient treated with adjuvant therapy before surgery, the authors identified only a radiation effect and no tumor on resection. In a second patient the bypass was occluded, and her tumor was not resected. The other 16 patients underwent gross-total resection of their tumor. Excluding the surgery-related deaths, the mean and median lengths of survival in this series were 13.2 and 8.3 months, respectively (range 1.5–48 months). Including the surgery-related deaths, the mean and median lengths of survival were 11.8 and 8 months, respectively (range 17 days–48 months). At last follow-up all patients had died of cancer or cancer-related causes.

Conclusions

Despite maximal surgical intervention, including ICA sacrifice at the skull base with revascularization, patient survival was dismal, and the complication rate was significant. The authors no longer advocate such an aggressive approach in this patient population. On rare occasions, however, such an approach may be considered for low-grade malignancies.

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Iman Feiz-Erfan, Benjamin D. Fox, Remi Nader, Dima Suki, Indro Chakrabarti, Ehud Mendel, Ziya L. Gokaslan, Ganesh Rao and Laurence D. Rhines

Object

Hematogenous metastases to the sacrum can produce significant pain and lead to spinal instability. The object of this study was to evaluate the palliative benefit of surgery in patients with these metastases.

Methods

The authors retrospectively reviewed all cases involving patients undergoing surgery for metastatic disease to the sacrum at a single tertiary cancer center between 1993 and 2005.

Results

Twenty-five patients (21 men, 4 women) were identified as having undergone sacral surgery for hematogenous metastatic disease during the study period. Their median age was 57 years (range 25–71 years). The indications for surgery included palliation of pain (in 24 cases), need for diagnosis (in 1 case), and spinal instability (in 3 cases). The most common primary disease was renal cell carcinoma.

Complications occurred in 10 patients (40%). The median overall survival was 11 months (95% CI 5.4–16.6 months). The median time from the initial diagnosis to the diagnosis of metastatic disease in the sacrum was 14 months (95% CI 0.0–29.3 months). The numerical pain scores (scale 0–10) were improved from a median of 8 preoperatively to a median of 3 postoperatively at 90 days, 6 months, and 1 year (p < 0.01). Postoperative modified Frankel grades improved in 8 cases, worsened in 3 (due to disease progression), and remained unchanged in 14 (p = 0.19). Among patients with renal cell carcinoma, the median overall survival was better in those in whom the sacrum was the sole site of metastatic disease than in those with multiple sites of metastatic disease (16 vs 9 months, respectively; p = 0.053).

Conclusions

Surgery is effective to palliate pain with acceptable morbidity in patients with metastatic disease to the sacrum. In the subgroup of patients with renal cell carcinoma, those with the sacrum as their solitary site of metastatic disease demonstrated improved survival.

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Carl H. Snyderman, Paul A. Gardner and Juan C. Fernandez-Miranda

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M. Yashar S. Kalani, Maziyar A. Kalani, Samuel Kalb, Felipe C. Albuquerque, Cameron G. McDougall, Peter Nakaji, Robert F. Spetzler, Randall W. Porter and Iman Feiz-Erfan

Object

Craniofacial approaches provide excellent exposure to lesions in the anterior and middle cranial fossae. The authors review their experience with craniofacial approaches for resection of large juvenile nasopharyngeal angiofibromas.

Methods

Between 1992 and 2009, 22 patients (all male, mean age 15 years, range 9–27 years) underwent 30 procedures. These cases were reviewed retrospectively.

Results

Gross-total resection of 17 (77%) of the 22 lesions was achieved. The average duration of hospitalization was 8.2 days (range 3–20 days). The rate of recurrence and/or progression was 4 (18%) of 22, with recurrences occurring a mean of 21 months after the first resection. All patients underwent preoperative embolization. Nine patients (41%) developed complications, the most common of which was CSF leakage (23%). The average follow-up was 27.7 months (range 2–144 months). The surgery-related mortality rate was 0%. Based on their mean preoperative (90) and postoperative (90) Karnofsky Performance Scale scores, 100% of patients improved or remained the same.

Conclusions

The authors' experience shows that craniofacial approaches provide an excellent avenue for the resection of large juvenile nasopharyngeal angiofibromas, with acceptable rates of morbidity and no deaths.

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Ganesh Rao, Dima Suki, Indro Chakrabarti, Iman Feiz-Erfan, Milan G. Mody, Ian E. McCutcheon, Ziya Gokaslan, Shreyaskumar Patel and Laurence D. Rhines

Object

Sarcomas of the spine are a challenging problem due to their frequent and extensive involvement of multiple spinal segments and high recurrence rates. Gross-total resection to negative margins, with preservation of neurological function and palliation of pain, is the surgical goal and may be achieved using either intralesional resection or en bloc excision. The authors report outcomes of surgery for primary and metastatic sarcomas of the mobile spine in a large patient series.

Methods

A retrospective review of patients undergoing resection for sarcomas of the mobile spine between 1993 and 2005 was undertaken. Sarcomas were classified by histology study results and as either primary or metastatic. Details of the surgical approach, levels of involvement, and operative complications were recorded. Outcome measures included neurological function, palliation of pain, local recurrence, and overall survival.

Results

Eighty patients underwent 110 resections of either primary or metastatic sarcomas of the mobile spine. Twenty-nine lesions were primary sarcomas (36%) and 51 were metastatic sarcomas (64%). Intralesional resections were performed in 98 surgeries (89%) and en bloc resections were performed in 12 (11%). Median survival from surgery for all patients was 20.6 months. Median survival for patients with a primary sarcoma of the spine was 40.2 months and was 17.3 months for patients with a metastatic sarcoma. Predictors of improved survival included a chondrosarcoma histological type and a better preoperative functional status, whereas osteosarcoma and a high-grade tumor were negative influences on survival. Multivariate analysis showed that only a high-grade tumor was an independent predictor of shorter overall survival. American Spinal Injury Association scale grades were maintained or improved in 97% of patients postoperatively, and there was a significant decrease in pain scores postoperatively. No significant differences in survival or local recurrence rates between intralesional or en bloc resections for either primary or metastatic spine sarcomas were found.

Conclusions

Surgery for primary or metastatic sarcoma of the spine is associated with an improvement in neurological function and palliation of pain. The results of this study show a significant difference in patient survival for primary versus metastatic spine sarcomas. The results do not show a statistically significant benefit in survival or local recurrence rates for en bloc versus intralesional resections for either metastatic or primary sarcomas of the spine, but this may be due to the small number of patients undergoing en bloc resections.

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Sam Safavi-Abbasi, Mehmet Senoglu, Nicholas Theodore, Ryan K. Workman, Alireza Gharabaghi, Iman Feiz-Erfan, Robert F. Spetzler and Volker K. H. Sonntag

Object

The authors conducted a study to evaluate the clinical characteristics and surgical outcomes in patients with spinal schwannomas and without neurofibromatosis (NF).

Methods

The data obtained in 128 patients who underwent resection of spinal schwannomas were analyzed. All cases with neurofibromas and those with a known diagnosis of NF Type 1 or 2 were excluded. Karnofsky Performance Scale (KPS) scores were used to compare patient outcomes when examining the anatomical location and spinal level of the tumor. The neurological outcome was further assessed using the Medical Research Council (MRC) muscle testing scale.

Results

Altogether, 131 schwannomas were treated in 128 patients (76 males and 52 females; mean age 47.7 years). The peak prevalence is seen between the 3rd and 6th decades. Pain was the most common presenting symptom. Gross-total resection was achieved in 127 (97.0%) of the 131 lesions. The nerve root had to be sacrificed in 34 cases and resulted in minor sensory deficits in 16 patients (12.5%) and slight motor weakness (MRC Grade 3/5) in 3 (2.3%). The KPS scores and MRC grades were significantly higher at the time of last follow-up in all patient groups (p = 0.001 and p = 0.005, respectively).

Conclusions

Spinal schwannomas may occur at any level of the spinal axis and are most commonly intradural. The most frequent clinical presentation is pain. Most spinal schwannomas in non-NF cases can be resected totally without or with minor postoperative deficits. Preoperative autonomic dysfunction does not improve significantly after surgical management.

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Nicholas C. Bambakidis, Iman Feiz-Erfan, Eric M. Horn, L. Fernando Gonzalez, Seungwon Baek, K. Zafer Yüksel, Anna G. U. Brantley, Volker K. H. Sonntag and Neil R. Crawford

Object

The stability provided by 3 occipitoatlantal fixation techniques (occiput [Oc]–C1 transarticular screws, occipital keel screws rigidly interconnected with C-1 lateral mass screws, and suboccipital/sublaminar wired contoured rod) were compared.

Methods

Seven human cadaveric specimens received transarticular screws and 7 received occipital keel–C1 lateral mass screws. All specimens later underwent contoured rod fixation. All conditions were studied with and without placement of a structural graft wired between the skull base and C-1 lamina. Specimens were loaded quasistatically using pure moments to induce flexion, extension, lateral bending, and axial rotation while recording segmental motion optoelectronically. Flexibility was measured immediately postoperatively and after 10,000 cycles of fatigue.

Results

Application of Oc–C1 transarticular screws, with a wired graft, reduced the mean range of motion (ROM) to 3% of normal. Occipital keel–C1 lateral mass screws (also with graft) offered less stability than transarticular screws during extension and lateral bending (p < 0.02), reducing ROM to 17% of normal. The wired contoured rod reduced motion to 31% of normal, providing significantly less stability than either screw fixation technique. Fatigue increased motion in constructs fitted with transarticular screws, keel screws/lateral mass screw constructs, and contoured wired rods, by means of 19, 5, and 26%, respectively. In all constructs, adding a structural graft significantly improved stability, but the extent depended on the loading direction.

Conclusions

Assuming the presence of mild C1–2 instability, Oc–C1 transarticular screws and occipital keel–C1 lateral mass screws are approximately equivalent in performance for occipitoatlantal stabilization in promoting fusion. A posteriorly wired contoured rod is less likely to provide a good fusion environment because of less stabilizing potential and a greater likelihood of loosening with fatigue.

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Iman Feiz-Erfan, Mary Harrigan, Volker K. H. Sonntag and Timothy R. Harrington

Object

In a double-blind randomized study, platelet concentrate was used to treat 50 patients who underwent anterior cervical fusion with allograft bone and internal fixation, predominantly for degenerative disc disease or soft herniated cervical disc. The goal in this study was to compare the outcomes in patients treated with and without the platelet gel.

Methods

Patients were assessed radiographically at 6, 12, and 52 weeks and at 2 years if needed. Clinically, patients were evaluated with the visual analog scale, Neck Disability Index, Short Form–36, and a modified Prolo Scale.

Results

Follow-up included 90% of the patients at 1 year and 84% at 2 years. The overall fusion rate was 84%.

Conclusions

Whereas patients with degenerative discs treated with platelet gel demonstrated early fusion at the 12-week follow-up interval, no consistent early fusion was obtained with the use of the platelet gel preparation in patients with a soft disc herniation.