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Elias Elias, Kimmo J Hatanpaa, Matthew MacAllister, Ali Daoud, Charbel Elias, and Zeina Nasser

BACKGROUND

Traumatic neuroma typically refers to a reactive process in the injured peripheral nerve, characterized by an excessive growth of axons, Schwann cells, and fibroblasts at the proximal end of the nerve after its interruption. The authors report a case of a traumatic neuroma in the cervical nerve root in a patient with no history of trauma.

OBSERVATIONS

The patient presented with sensation loss in the right-hand ulnar distribution, right flank around the T4–11 region, and right small toe along with motor power weakness over the right upper and lower extremity. Magnetic resonance imaging revealed an intradural extramedullary mass lesion with extension along the C7 nerve root. Histological examination showed traumatic neuroma. A total resection of the lesion along with the resolution of sensory and motor deficits was achieved directly after surgery.

LESSONS

Traumatic neuroma should always be kept in the armamentarium for diagnosis of an intradural nerve sheath tumor.

Open access

Brooke Elberson, Hayden Scott, Rohit Dhall, and Erika Petersen

BACKGROUND

Deep brain stimulation (DBS) is a well-established neurosurgical intervention for a growing number of neurological and psychiatric diseases. Patients who are affected by Parkinson’s disease may benefit from DBS of either the subthalamic nucleus or the globus pallidus internus. Patients who undergo DBS often notice a significant reduction in their clinical symptoms; however, the procedure is not without risks. Multicenter studies have reported postoperative complications such as hardware infection, intracranial hemorrhage, and perielectrode edema.

OBSERVATIONS

The authors report a case of a perielectrode cyst managed conservatively. Tracking the impedance trend was a novel approach to monitor for changes within the cyst and to herald a clinical change in the patient. Perielectrode cystic formation can be a transient process that resolves spontaneously or with conservative, nonoperative management, and all diagnostic information is valuable in making clinical decisions.

LESSONS

Impedance values have provided an appropriate estimation of this patient’s clinical picture. The authors suggest treatment of edema and a cyst after DBS lead implantation through conservative management and observation, avoiding the removal of hardware if a patient’s clinical picture is either stable or improving and forgoing additional clinical imaging if the impedance values are trending in an appropriate direction.

Open access

Irakliy Abramov, Charuta G Furey, Yuan Xu, Jennifer M Eschbacher, Kris A Smith, and Mark C Preul

BACKGROUND

Intraoperative frozen sections play a critical role in surgical strategy because of their ability to provide rapid histopathological information. In cases in which intraoperative biopsy carries a significant risk of bleeding, intraoperative confocal laser endomicroscopy (CLE) can assist in decision-making.

OBSERVATIONS

The authors present a rare case of a large sellar hemangioblastoma. Preoperative radiographic imaging and normal pituitary function suggested a differential diagnosis that included hemangioblastoma. The patient underwent partial preoperative embolization and a right-sided pterional craniotomy for resection of the lesion. Gross intraoperative examination revealed a highly vascular sellar lesion requiring circumferential dissection to minimize blood loss. The serious vascularity precluded intraoperative frozen section analysis, and CLE imaging was performed. CLE imaging provided excellent visualization of the remarkable vascular structure and characteristic histoarchitecture with microvasculature, intracytoplasmic vacuoles, and atypical cells consistent with hemangioblastoma. Resection and decompression of the chiasm was accomplished, and the patient was discharged with improved vision. The final histopathological diagnosis was hemangioblastoma.

LESSONS

When the benefits of obtaining intraoperative frozen sections greatly outweigh the associated risks, CLE imaging can aid in decision-making. CLE imaging offers real-time, on-the-fly evaluation of intraoperative tissue without the need to biopsy a vascular lesion.

Open access

Kenji Yagi

Open access

Jeyan Sathia Kumar, Nisha Dabhi, Daniel M. S Raper, Stepan Capek, R. Webster Crowley, M. Yashar Kalani, Ryan T Kellogg, and Min S Park

BACKGROUND

Flow diversion, specifically with the Pipeline embolization device (PED), represents a paradigm shift in the treatment of intracranial aneurysms. Several studies have demonstrated its efficacy and at times superiority to conventional treatment modalities for aneurysms with a fusiform morphology, giant size, or wide neck. However, there may be a nonsignificant risk of recurrence after flow diversion of these historically difficult-to-treat aneurysms, relative to aneurysms with a more favorable morphology and size (i.e., saccular, narrow necked). To date, only three papers in the literature have demonstrated the recurrence of a completely occluded aneurysm on follow-up.

OBSERVATIONS

The authors describe a patient with a giant middle cerebral artery fusiform aneurysm treated with multiple telescoping PEDs. On the 3-month follow-up angiogram, there was complete occlusion of the aneurysm. The patient was lost to follow-up and presented 4 years later with a recurrence of the aneurysm between PED segments, requiring retreatment. The patient represented 3 years posttreatment with the need for repeat treatment of the fusiform aneurysm due to separation of the existing PEDs along with stent reconstruction. At the 20-month follow-up after the third treatment, the initial aneurysm target was found to be occluded.

LESSONS

This case illustrates the need for long-term follow-up, specifically for patients with giant wide-necked or fusiform aneurysms treated with overlapping PEDs.

Restricted access

Jeffrey Campbell, Janet M. Legare, Joseph Piatt, Ethan Gough, Richard M. Pauli, S. Shahrukh Hashmi, David F. Rodriguez-Buritica, Peggy Modaff, Mary Ellen Little, Maria Elena Serna, Cory J. Smid, Lorena Dujmusic, Jacqueline T. Hecht, Julie E. Hoover-Fong, and Michael B. Bober

OBJECTIVE

The objective of this study was to describe the incidence and management of hydrocephalus in patients with achondroplasia over a 60-year period at four skeletal dysplasia centers.

METHODS

The Achondroplasia Natural History Study (CLARITY) is a registry for clinical data from achondroplasia patients receiving treatment at four skeletal dysplasia centers in the US from 1957 to 2017. Data were entered and stored in a REDCap database and included surgeries with indications and complications, medical diagnoses, and radiographic information.

RESULTS

A total of 1374 patients with achondroplasia were included in this study. Of these, 123 (9%) patients underwent treatment of hydrocephalus at a median age of 14.4 months. There was considerable variation in the percentage of patients treated for hydrocephalus by center and decade of birth, ranging from 0% to 28%, although in the most recent decade, all centers treated less than 6% of their patients, with an average of 2.9% across all centers. Undergoing a cervicomedullary decompression (CMD) was a strong predictor for treatment of hydrocephalus (OR 5.8, 95% CI 3.9–8.4), although that association has disappeared in those born since 2010 (OR 1.1, 95% CI 0.2–5.7). In patients born since 1990, treatment of hydrocephalus with endoscopic third ventriculostomy (ETV) has become more common; it was used as the first line of treatment in 38% of patients in the most recent decade. Kaplan-Meier analysis suggests that a single ETV will treat hydrocephalus in roughly half of these patients.

CONCLUSIONS

While many children with achondroplasia have features of hydrocephalus with enlarged intracranial CSF spaces and relative macrocephaly, treatment of hydrocephalus in achondroplasia patients has become relatively uncommon in the last 20 years. Historically, there was a significant association between symptomatic foramen magnum stenosis and treatment of hydrocephalus, although concurrent treatment of both has fallen out of favor with the recognition that CMD alone will treat hydrocephalus in some patients. Despite good experimental data demonstrating that hydrocephalus in achondroplasia is best understood as communicating in nature, ETV appears to be reasonably successful in certain patients and should be considered an option in selected patients.

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Walid Ibn Essayed, Joshua D. Bernstock, Jason A. Chen, Ari D. Kappel, Patrick R. Ng, and Rose Du

Cavernous-type malformations are venous lesions that occur in multiple locations throughout the body, and when present in the CNS, they have canonically been referred to as cavernomas, cavernous angiomas, and cerebral cavernous malformations. Herein all these lesions are referred to as "cavernous venous malformations" (CavVMs), which is congruent with the current International Society for the Study of Vascular Anomalies classification system. Even though histologically similar, depending on their location relative to the dura mater, these malformations can have different features. In Part 1 of this review, the authors discuss and review pertinent clinical knowledge with regard to CavVMs as influenced by anatomical location, starting with the dural and extradural malformations. They particularly emphasize dural CavVMs (including those in the cavernous sinus), orbital CavVMs, and spinal CavVMs. The genetic and histopathological features of CavVMs in these locations are reviewed, and commonalities in their presumed mechanisms of pathogenesis support the authors’ conceptualization of a spectrum of a single disease entity. Illustrative cases for each subtype are presented, and the pathophysiological and genetic features linking dural and extradural to intradural CavVMs are examined. A new classification is proposed to segregate CavVMs based on the location from which they arise, which guides their natural history and treatment.

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Walid Ibn Essayed, Joshua D. Bernstock, Jason A. Chen, Patrick R. Ng, Ari D. Kappel, and Rose Du

Cavernous venous malformations (CavVMs) account for a spectrum of lesions with a shared pathogenesis. Their anatomical location dictates their clinical features and surgical treatment. Extradural and dura-based CavVMs were discussed in Part 1 of this review. In this part, intradural CavVMs are discussed, encompassing malformations growing within the intradural space without direct dural involvement. In addition to classic intra-axial CavVMs, cranial nerve CavVMs, intraventricular CavVMs, and intradural extramedullary spinal CavVMs are discussed in this group, given the similar natural history and specific management challenges. Herein the authors focus on critical clinical aspects of and surgical management of these malformations based on their location and discuss optimal surgical approaches at each of these anatomical locations with illustrative cases. The commonalities of the natural history and surgical management that are dictated by anatomical considerations lend to a new location-based taxonomy for classification of CavVMs.

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Peter G. Passias, Waleed Ahmad, Pooja Dave, Renaud Lafage, Virginie Lafage, Jamshaid Mir, Eric O. Klineberg, Khaled M. Kabeish, Jeffrey L. Gum, Breton G. Line, Robert Hart, Douglas Burton, Justin S. Smith, Christopher P. Ames, Christopher I. Shaffrey, Frank Schwab, Richard Hostin, Thomas Buell, D. Kojo Hamilton, and Shay Bess

OBJECTIVE

The purpose of this study was to investigate the cost utility of nonoperative treatment for adult spinal deformity (ASD).

METHODS

Nonoperatively and operatively treated patients who met database criteria for ASD and in whom complete radiographic and health-related quality of life data at baseline and at 2 years were available were included. A cost analysis was completed on the PearlDiver database assessing the average cost of nonoperative treatment prior to surgical intervention based on previously published treatments (NSAIDs, narcotics, muscle relaxants, epidural steroid injections, physical therapy, and chiropractor). Utility data were calculated using the Oswestry Disability Index (ODI) converted to SF-6D with published conversion methods. Quality-adjusted life years (QALYs) used a 3% discount rate to account for residual decline in life expectancy (78.7 years). Minor and major comorbidities and complications were assessed according to the CMS.gov manual’s definitions. Successful nonoperative treatment was defined as a gain in the minimum clinically importance difference (MCID) in both ODI and Scoliosis Research Society (SRS)–pain scores, and failure was defined as a loss in MCID or conversion to operative treatment. Patients with baseline ODI ≤ 20 and continued ODI of ≤ 20 at 2 years were considered nonoperative successful maintenance. The average utilization of nonoperative treatment and cost were applied to the ASD cohort.

RESULTS

A total of 824 patients were included (mean age 58.24 years, 81% female, mean body mass index 27.2 kg/m2). Overall, 75.5% of patients were in the operative and 24.5% were in the nonoperative cohort. At baseline patients in the operative cohort were significantly older, had a greater body mass index, increased pelvic tilt, and increased pelvic incidence–lumbar lordosis mismatch (all p < 0.05). With respect to deformity, patients in the operative group had higher rates of severe (i.e., ++) sagittal deformity according to SRS–Schwab modifiers for pelvic tilt, sagittal vertical axis, and pelvic incidence–lumbar lordosis mismatch (p < 0.05). At 2 years, patients in the operative cohort showed significantly increased rates of a gain in MCID for physical component summary of SF-36, ODI, and SRS-activity, SRS-pain, SRS-appearance, and SRS-mental scores. Cost analysis showed the average cost of nonoperative treatment 2 years prior to surgical intervention to be $2041. Overall, at 2 years patients in the nonoperative cohort had again in ODI of 0.36, did not show a gain in QALYs, and nonoperative treatment was determined to be cost-ineffective. However, a subset of patients in this cohort underwent successful maintenance treatment and had a decrease in ODI of 1.1 and a gain in utility of 0.006 at 2 years. If utility gained for this cohort was sustained to full life expectancy, patients’ cost per QALY was $18,934 compared to a cost per QALY gained of $70,690.79 for posterior-only and $48,273.49 for combined approach in patients in the operative cohort.

CONCLUSIONS

Patients with ASD undergoing operative treatment at baseline had greater sagittal deformity and greater improvement in health-related quality of life postoperatively compared to patients treated nonoperatively. Additionally, patients in the nonoperative cohort overall had an increase in ODI and did not show improvement in utility gained. Patients in the nonoperative cohort who had low disability and sagittal deformity underwent successful maintenance and cost-effective treatment.