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Stanley L. Barnwell, Christopher F. Dowd, Richard L. Davis, Michael S. B. Edwards, Philip H. Gutin and Charles B. Wilson

✓ The cases of seven patients with intramedullary, cryptic vascular malformations of the spinal cord are reported. In all patients, the clinical course was progressive; a Brown-Séquard syndrome was the most common presenting symptom complex. Magnetic resonance (MR) imaging was performed in all patients. The pattern seen most often was a focus of high signal (on both T1- and T2-weighted MR images) surrounded by a larger zone of low signal (best seen on T2-weighted images), and was remarkably similar for all patients. Six patients underwent surgical exploration; removal of the lesions halted the progression of symptoms in five patients, and one patient had worsened sensory function after surgery. Motor function did not decrease postoperatively in any patient. The one patient who refused surgery has continued to decline neurologically. Histopathological examination of surgical specimens showed a cavernous malformation in one patient, a venous malformation in one, venous varices in two, and organizing hematomas in two; these findings are markedly different from those in previously reported cases of cryptic vascular malformations.

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Maria Peris-Celda, Avital Perry, Lucas P. Carlstrom, Christopher S. Graffeo, Colin L. W. Driscoll and Michael J. Link

OBJECTIVE

Middle fossa surgery is challenging, and reliable surgical landmarks are essential to perform accurate and safe surgery. Although many descriptions of the middle fossa components have been published, a clinically practical description of this very complex anatomical region is lacking. Small structure arrangements in this area are often not well visualized or accurately demarcated with neuronavigation systems. The objective is to describe a “roadmap” of key surgical reference points and landmarks during middle fossa surgery to help the surgeon predict where critical structures will be located.

METHODS

The authors studied 40 dry skulls (80 sides) obtained from the anatomical board at their institution. Measurements of anatomical structures in the middle fossa were made with a digital caliper and a protractor, taking as reference the middle point of the external auditory canal (MEAC). The results were statistically analyzed.

RESULTS

The petrous part of the temporal bone was found at a mean of 16 mm anterior and 24 mm posterior to the MEAC. In 87% and 99% of the sides, the foramen ovale and foramen spinosum, respectively, were encountered deep to the zygomatic root. The posterior aspect of the greater superficial petrosal nerve (GSPN) groove was a mean of 6 mm anterior and 25 mm medial to the MEAC, nearly parallel to the petrous ridge. The main axis of the IAC projected to the root of the zygoma in all cases. The internal auditory canal (IAC) porus was found 5.5 mm lateral and 4.5 mm deep to the lateral aspect of the trigeminal impression along the petrous ridge (mean measurement values). A projection from this point to the middle aspect of the root of the zygoma, being posterior to the GSPN groove, could estimate the orientation of the IAC.

CONCLUSIONS

In middle fossa approaches, the external acoustic canal is a reliable reference before skin incision, whereas the zygomatic root becomes important after the skin incision. Deep structures can be related to these 2 anatomical structures. An easy method to predict the location of the IAC in surgery is described. Careful study of the preoperative imaging is essential to adapt this knowledge to the individual anatomy of the patient.

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Aladine A. Elsamadicy, Andrew B. Koo, Megan Lee, Adam J. Kundishora, Christopher S. Hong, Astrid C. Hengartner, Joaquin Camara-Quintana, Kristopher T. Kahle and Michael L. DiLuna

OBJECTIVE

In the past decade, a gradual transition of health policy to value-based healthcare has brought increased attention to measuring the quality of care delivered. In spine surgery, adolescents with scoliosis are a population particularly at risk for depression, anxious feelings, and impaired quality of life related to back pain and cosmetic appearance of the deformity. With the rising prevalence of mental health ailments, it is necessary to evaluate the impact of concurrent affective disorders on patient care after spinal surgery in adolescents. The aim of this study was to investigate the impact that affective disorders have on perioperative complication rates, length of stay (LOS), and total costs in adolescents undergoing elective posterior spinal fusion (PSF) (≥ 4 levels) for idiopathic scoliosis.

METHODS

A retrospective study of the Kids’ Inpatient Database for the year 2012 was performed. Adolescent patients (age range 10–17 years old) with AIS undergoing elective PSF (≥ 4 levels) were selected using the International Classification of Diseases, Ninth Revision, Clinical Modification coding system. Patients were categorized into 2 groups at discharge: affective disorder or no affective disorder. Patient demographics, comorbidities, complications, LOS, discharge disposition, and total cost were assessed. The primary outcomes were perioperative complication rates, LOS, total cost, and discharge dispositions.

RESULTS

There were 3759 adolescents included in this study, of whom 164 (4.4%) were identified with an affective disorder (no affective disorder: n = 3595). Adolescents with affective disorders were significantly older than adolescents with no affective disorders (affective disorder: 14.4 ± 1.9 years vs no affective disorder: 13.9 ± 1.8 years, p = 0.001), and had significantly different proportions of race (p = 0.005). Aside from hospital region (p = 0.016), no other patient- or hospital-level factors differed between the cohorts. Patient comorbidities did not differ significantly between cohorts. The number of vertebral levels involved was similar between the cohorts, with the majority of patients having 9 or more levels involved (affective disorder: 76.8% vs no affective disorder: 79.5%, p = 0.403). Postoperative complications were similar between the cohorts, with no significant difference in the proportion of patients experiencing a postoperative complication (p = 0.079) or number of complications (p = 0.124). The mean length of stay and mean total cost were similar between the cohorts. Moreover, the routine and nonroutine discharge dispositions were also similar between the cohorts, with the majority of patients having routine discharges (affective disorder: 93.9% vs no affective disorder: 94.9%, p = 0.591).

CONCLUSIONS

This study suggests that affective disorders may not have a significant impact on surgical outcomes in adolescent patients undergoing surgery for scoliosis in comparison with adults. Further studies are necessary to elucidate how affective disorders affect adolescent patients with idiopathic scoliosis, which may improve provider approach in managing these patients perioperatively and at follow-up in hopes to better the overall patient satisfaction and quality of care delivered.

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Gregory C. Wiggins, Michael J. Rauzzino, Christopher I. Shaffrey, Russ P. Nockels, Richard Whitehill, Mark E. Shaffrey, James Wagner and Tord D. Alden

This study was conducted to determine the safety, efficacy, and complication rate associated with the anterior approach in the use of a new titanium mesh interbody fusion cage for the treatment of unstable thoracolumbar burst fractures. The experience with this technique is compared with the senior authors' (C.S., R.W., and M.S.) previously published results in the management of patients with unstable thoracolumbar burst fractures.

Between 1996 and 1999, 21 patients with unstable thoracolumbar (T12-L3) burst fractures underwent an anterolateral decompressive procedure in which a titanium cage and Kaneda device were used. Eleven of the 21 patients had sustained a neurological deficit, and all patients improved at least one Frankel grade (average 1.2 grades). There was improvement in outcome in terms of blood loss, correction of kyphosis, and pain, as measured on the Denis Pain and Work Scale, in our current group of patients treated via an anterior approach when compared with the results in those who underwent a posterior approach.

In our current study the anterior approach was demonstrated to be a safe and effective technique for the management of unstable thoracolumbar burst fractures. It offers superior results compared with the posterior approach. The addition of the new titanium mesh interbody cage to our previous anterior technique allows the patient's own bone to be harvested from the corpectomy site and used as a substrate for fusion, thereby obviating the need for iliac crest harvest. The use of the cage in association with the Kaneda device allows for improved correction of kyphosis and restoration of normal sagittal alignment in addition to improved functional outcomes.

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Michael Safaee, Andrew T. Parsa, Nicholas M. Barbaro, Dean Chou, Praveen V. Mummaneni, Philip R. Weinstein, Tarik Tihan and Christopher P. Ames

OBJECT

Intradural extramedullary spine tumors represent two-thirds of all primary spine neoplasms. Approximately half of these are peripheral nerve sheath tumors, mainly neurofibromas and schwannomas. Given the rarity of this disease and, thus, the limited analyses of clinical outcomes, the authors examined the association of tumor location, extent of resection, and neurofibromatosis (NF) status with clinical outcomes.

METHODS

Patients were identified through a search of the University of California, San Francisco, neuropathology database and a separate review of current procedural terminology codes. Data recorded included patient age, patient sex, clinical presentation, presence of NF, tumor type, tumor location, extent of resection (gross-total resection [GTR] or subtotal resection [STR]), and clinical follow-up.

RESULTS

Of 221 tumors in 199 patients (mean age 45 years), 53 were neurofibromas, 163 were schwannomas, and 5 were malignant peripheral nerve sheath tumors. The most common presenting symptom was spinal pain (76%), followed by weakness (36%) and sensory abnormalities (34%). Mean symptom duration was 16 months. In terms of spinal location, neurofibromas were more common in the cervical spine (74% vs 27%, p < 0.001), and schwannomas were more common in the thoracic and lumbosacral spine (73% vs 26%, p < 0.001). Rates of GTR were lower for neurofibromas than schwannomas (51% vs 83%, p < 0.001), regardless of location. Rates of GTR were lower for cervical (54%) than thoracic (90%) and lumbosacral (86%) lesions (p < 0.001). NF was associated with lower rates of GTR among all tumors (43% vs 86%, p < 0.001). The mean follow-up time was 32 months. Recurrence/progression was more common for neurofibromas than schwannomas (17% vs 7%, p = 0.03), although the mean time to recurrence/progression did not differ according to tumor type (45 vs 53 months, p = 0.63). As expected, GTR was associated with lower recurrence rates (4% vs 22%, p < 0.001). According to multivariate analysis, cervical location (OR 0.239, 95% CI 0.110–0.520) and presence of NF (OR 0.166, 95% CI 0.054–0.507) were associated with lower rates of GTR. In a separate model, only GTR (OR 0.141, 95% CI 0.046–0.429) was associated with tumor recurrence.

CONCLUSIONS

Resection is an effective treatment for spinal nerve sheath tumors. Neurofibromas were found more commonly in the cervical spine than in other regions of the spine and were associated with higher rates of recurrence and lower rates of GTR than other tumor types, particularly in patients with NF Types 1 or 2. According to multivariate analysis, both cervical location and presence of NF were associated with lower rates of GTR. According to a second multivariate model, the only variable associated with tumor recurrence was extent of resection. Maximal safe resection remains ideal for these lesions; however, patients with cervical tumors or NF should be counseled about their increased risk for recurrence.

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Avital Perry, Christopher S. Graffeo, Waleed Brinjikji, William R. Copeland III, Alejandro A. Rabinstein and Michael J. Link

Spontaneous intracranial hypotension (SIH) is an uncommon headache etiology, typically attributable to an unprovoked occult spinal CSF leak. Although frequently benign, serious complications may occur, including cerebral venous thrombosis (CVT). The objective of this study was to examine a highly complicated case of CVT attributable to SIH as a lens for understanding the heterogeneous literature on this rare complication, and to provide useful, evidence-based, preliminary clinical recommendations. A 43-year-old man presented with 1 week of headache, dizziness, and nausea, which precipitously evolved to hemiplegia. CT venography confirmed CVT, and therapeutic heparin was initiated. He suffered a generalized seizure due to left parietal hemorrhage, which subsequently expanded. He developed signs of mass effect and herniation, heparin was discontinued, and he was taken to the operating room for clot evacuation and external ventricular drain placement. Intraoperatively, the dura was deflated, suggesting underlying SIH. Ventral T-1 CSF leak was identified, which failed multiple epidural blood patches and required primary repair. The patient ultimately made a complete recovery. Systematic review identified 29 publications describing 36 cases of SIH-associated CVT. Among 31 patients for whom long-term neurological outcome was reported, 25 (81%) recovered completely. Underlying coagulopathy/risk factors were identified in 11 patients (31%). CVT is a rare and potentially lethal sequela occurring in 2% of SIH cases. Awareness of the condition is poor, risking morbid complications. Evaluation and treatment should be directed toward identification and treatment of occult CSF leaks. Encouragingly, good neurological outcomes can be achieved through vigilant multidisciplinary neurosurgical and neurocritical care.

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Michael M. Safaee, Vedat Deviren, Cecilia Dalle Ore, Justin K. Scheer, Darryl Lau, Joseph A. Osorio, Fred Nicholls and Christopher P. Ames

OBJECTIVE

Proximal junctional kyphosis (PJK) is a well-recognized, yet incompletely defined, complication of adult spinal deformity surgery. There is no standardized definition for PJK, but most studies describe PJK as an increase in the proximal junctional angle (PJA) of greater than 10°–20°. Ligament augmentation is a novel strategy for PJK reduction that provides strength to the upper instrumented vertebra (UIV) and adjacent segments while also reducing junctional stress at those levels.

METHODS

In this study, ligament augmentation was used in a consecutive series of adult spinal deformity patients at a single institution. Patient demographics, including age; sex; indication for surgery; revision surgery; surgical approach; and use of 3-column osteotomies, vertebroplasty, or hook fixation at the UIV, were collected. The PJA was measured preoperatively and at last follow-up using 36-inch radiographs. Data on change in PJA and need for revision surgery were collected. Univariate and multivariate analyses were performed to identify factors associated with change in PJA and proximal junctional failure (PJF), defined as PJK requiring surgical correction.

RESULTS

A total of 200 consecutive patients were included: 100 patients before implementation of ligament augmentation and 100 patients after implementation of this technique. The mean age of the ligament augmentation cohort was 66 years, and 67% of patients were women. Over half of these cases (51%) were revision surgeries, with 38% involving a combined anterior or lateral and posterior approach. The mean change in PJA was 6° in the ligament augmentation group compared with 14° in the control group (p < 0.001). Eighty-four patients had a change in PJA of less than 10°. In a multivariate linear regression model, age (p = 0.016), use of hook fixation at the UIV (p = 0.045), and use of ligament augmentation (p < 0.001) were associated with a change in PJA. In a separate model, only ligament augmentation (OR 0.193, p = 0.012) showed a significant association with PJF.

CONCLUSIONS

Ligament augmentation represents a novel technique for the prevention of PJK and PJF. Compared with a well-matched historical cohort, ligament augmentation is associated with a significant decrease in PJK and PJF. These data support the implementation of ligament augmentation in surgery for adult spinal deformity, particularly in patients with a high risk of developing PJK and PJF.

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Michael M. McDowell, Jason E. Blatt, Christopher P. Deibert, Nathan T. Zwagerman, Zachary J. Tempel and Stephanie Greene

OBJECTIVE

Chiari malformation type II (CM-II) in myelomeningocele is associated with a significant rate of mortality and poor outcome. Death is frequently heralded by the onset or progression of neurological symptoms. The authors sought to identify predictors of poor outcome and mortality within the myelomeningocele population at Children’s Hospital of Pittsburgh.

METHODS

A retrospective chart and radiology review was performed on all infants who underwent primary closure of a myelomeningocele defect at Children’s Hospital of Pittsburgh between the years of 1995 and 2015. Preoperative symptoms and signs leading to CM-II decompression, as well as operative details and postoperative changes in these symptoms and signs, were investigated in detail and correlated to outcome. Poor outcome was defined as death, stridor, or ventilator dependence. Deceased patients were separately assessed within this subgroup.

RESULTS

Thirty-two (21%) of 153 patients were found to have symptomatic CM-II. Of the 32 patients meeting inclusion criteria, 12 (38%) had poor outcomes. Eight patients (25%) died since initial presentation; 5 of these patients (16% of the overall cohort) died within the 1st year of life and 3 (9%) died during adolescence. Seven (88%) of the 8 patients who died had central apnea on presentation (p = 0.001) and 7 (44%) of the 16 patients who developed symptoms in the first 3 months of life died, compared with 1 (6.3%) of 16 who developed symptoms later in childhood (p = 0.04). The median Apgar score at 1 minute was 4.5 for patients who died and 8 for surviving patients (p = 0.006). The median diameter of the myelomeningocele defect was 5.75 cm for patients who died and 5 for those who survived (p = 0.01). The anatomical level of defect trended toward higher levels in patients who died, with 4 patients in that group having an anatomical level at L-2 or higher compared with 5 of the surviving patients (p = 0.001). The median initial head circumference for the 5 patients dying in the 1st year of life was 41.5 cm, versus 34 cm for all other patients (p = 0.01).

CONCLUSIONS

CM-II in spina bifida is associated with a significant mortality rate even when surgical intervention is performed. Death is more frequent in symptomatic patients presenting prior to 1 year of age. Late deaths are associated with symptom progression despite aggressive surgical and medical intervention. In this patient cohort, death was more likely in patients with symptomatic presentation during the first 3 months of life, low Apgar scores, large myelomeningocele defects, early central apnea, and large head circumference at birth.

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Hirokazu Takami, Christoph M. Prummer, Christopher S. Graffeo, Maria Peris-Celda, Caterina Giannini, Colin L. Driscoll and Michael J. Link

Glioblastoma (GBM) of the internal auditory canal (IAC) is exceedingly rare, with only 3 prior cases reported in the literature. The authors present the fourth case of cerebellopontine angle (CPA) and IAC GBM, and the first in which the lesion mimicked a vestibular schwannoma (VS) early in its natural history. A 55-year-old man presented with tinnitus, hearing loss, and imbalance. MRI identified a left IAC/CPA lesion measuring 8 mm, most consistent with a benign VS. Over the subsequent 4 months he developed facial weakness. The tumor grew remarkably to 24 mm and surgery was recommended; the main preoperative diagnosis was malignant peripheral nerve sheath tumor (MPNST). Resection proceeded via a translabyrinthine approach with resection of cranial nerves VII and VIII, followed by facial-hypoglossal nerve anastomosis. Intraoperative frozen section suggested malignant spindle cell neoplasm, but final histopathological and molecular testing confirmed the lesion to be a GBM. The authors report the first case in which absence of any brainstem interface effectively excluded a primary parenchymal tumor, in particular GBM, from the differential diagnosis. Given the dramatic differences in treatment and prognoses between malignant glioma and MPNST, this case emphasizes the importance of surgical intervention on an aggressively growing lesion, which provides both the best probability of local control and the critical tissue diagnosis.

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Janice A. Miller, DeWitte T. Cross, Christopher J. Moran, Ralph G. Dacey Jr., Janice G. McFarland and Michael N. Diringer

✓ Selective intraarterial infusion of papaverine is used in the treatment of symptomatic cerebral vasospasm. The authors report two episodes of severe thrombocytopenia in a patient that were related to intraarterial administration of papaverine. A 70-year-old man with a right internal carotid artery aneurysm underwent craniotomy and aneurysm clipping. He became lethargic 8 days after the hemorrhage occurred. Cerebral angiography revealed moderate vasospasm. In addition to hypervolemic—hypertensive therapy, the patient was treated on two occasions with intraarterial administration of papaverine. Within 24 hours of both treatments he developed severe thrombocytopenia. On one occasion epistaxis requiring transfusion of blood products occurred. Laboratory data support the diagnosis of immune-mediated papaverine-induced thrombocytopenia. The authors conclude that intraarterial administration of papaverine for treatment of vasospasm can be associated with severe, rapidly reversible thrombocytopenia.