Macrovascular Lesions Underlying Spontaneous Intracerebral Hemorrhage.

Spontaneous intracerebral hemorrhage (ICH) is a morbid disease with a high case fatality rate. Prognosis, rehemorrhage rates, and acute, clinical decision making are greatly affected by the underlying etiology of hemorrhage. This review focuses on the evaluation, diagnosis, and management of structural, macrovascular lesions presenting with ICH, including ruptured aneurysms, brain arteriovenous malformations, cranial dural arteriovenous fistulas, and cerebral cavernous malformations.

Radiological signs of the syndrome of the trephined.

INTRODUCTION: Syndrome of the trephined (ST) is a post-craniectomy complication. It is characterized by the appearance of new neurological symptoms following the craniectomy, which are relieved after cranioplasty. The purpose of our work was to identify radiological signs and imaging biomarkers of the ST. METHODS: CT images of 32 patients were retrospectively analyzed (ST = 13, controls = 19). While the shapes of craniectomy flap were qualitatively assessed, deviation of the midline structures, relative intracranial cerebrospinal fluid (CSF) volume, and the 3rd ventricle's volume were quantitatively measured. RESULTS: We did not find between-group differences in the mean age or number of post-craniectomy days. ST was diagnosed during the second post-craniectomy month. The occurrence of a sunken skin flap sign was similar in both groups (69.23 % in ST group, 57.89 % in control group). Occurrence of paradoxical herniation and deviation of the midline structures were not significantly different between groups. Mean relative intracranial CSF volume was significantly smaller in ST patients (ST = 5.59 %, controls = 8.12 %, p = 0.01). ST patients, compared to controls, had also significantly smaller mean 3rd ventricle volumes (ST = 1748 mm(3), controls = 2772.97 mm(3), p = 0.03). CONCLUSIONS: ST is an infrequent and delayed post-craniectomy complication. The most common radiological findings (paradoxical herniation, deviation of the midline structures, and sunken skin flap sign) might not be specific for ST. Significantly lower 3rd ventricle, and relative intracranial CSF volumes, suggest that altered biophysical CSF properties underlie ST pathophysiology. Therefore, volume measurements of 3rd ventricle could be useful for identification of patients who have higher probability of developing the ST.

Adverse radiation effects in volume-staged radiosurgery for large arteriovenous malformations: a multiinstitutional study.

OBJECTIVE: The optimal treatment paradigm for large arteriovenous malformations (AVMs) is controversial. One approach is volume-staged stereotactic radiosurgery (VS-SRS). The authors previously reported efficacy of VS-SRS for large AVMs in a multiinstitutional cohort; here they focus on risk of symptomatic adverse radiation effects (AREs). METHODS: This is a multicentered retrospective review of patients treated with a planned prospective volume staging approach to stereotactically treat the entire nidus of an AVM, with volume stages separated by intervals of 3-6 months. A total of 9 radiosurgical centers treated 257 patients with VS-SRS between 1991 and 2016. The authors evaluated permanent, transient, and total ARE events that were symptomatic. RESULTS: Patients received 2-4 total volume stages. The median age was 33 years at the time of the first SRS volume stage, and the median follow-up was 5.7 years after VS-SRS. The median total AVM nidus volume was 23.25 cm3 (range 7.7-94.4 cm3), with a median margin dose per stage of 17 Gy (range 12-20 Gy). A total of 64 patients (25%) experienced an ARE, of which 19 were permanent. Rather than volume, maximal linear dimension in the Z (craniocaudal) dimension was associated with toxicity; a threshold length of 3.28 cm was associated with an ARE, with a 72.5% sensitivity and a 58.3% specificity. In addition, parietal lobe involvement for superficial lesions and temporal lobe involvement for deep lesions were associated with an ARE. CONCLUSIONS: Size remains the dominant predictor of toxicity following SRS, but overall rates of AREs were lower than anticipated based on baseline features, suggesting that dose and size were relatively dissociated through volume staging. Further techniques need to be assessed to optimize outcomes.

Histological Tracking into the Third Dimension: Evolution of Early Tumorigenesis in VHL Kidney.

Using a novel three-dimensional (3D) approach, we tracked histological changes to elucidate the earliest stages of renal clear cell neoplasia in normal kidney tissue of patients with von Hippel-Lindau (VHL) disease. Tissue blocks of interest were procured, serially sectioned, and 3D reconstruction of the entirety of pathologic events was performed. The results reveal an abundance of foci with aberrant clear cell proliferation that initially develop along the tubular lining, but have the potential to aggregate within individual tubules. This stage is followed by the extension of clear cell aggregates beyond the tubular basement membrane, which allows for the recruitment of angiogenesis derived from interstitial vasculature. The results suggest that the most frequent pathologic event in VHL kidneys is the presence of isolated or aggregated clear cells within the tubular epithelium, potentially developing further into a protracted process of neoplasia. The abundance of independent pathologic events in VHL kidneys confirms developmental mechanisms to precede tumor initiation. To our knowledge, this is the first report demonstrating that tracking of histologic changes in the 3rd dimension enables the confirmation of the sequence of events from the earliest pathologic change in the VHL kidney to the neoplastic stage. This approach is not only useful for visualization and quantification of pathologic changes but also for targeted sampling allowing selective analysis of the earliest stages of clear cell carcinogenesis.

Clinical Outcomes between Stand-Alone Zero-Profile Spacers and Cervical Plate with Cage Fixation for Anterior Cervical Discectomy and Fusion: A Retrospective Analysis of 166 Patients.

Stand-alone (SA) zero-profile implants are an alternative to cervical plating (CP) in anterior cervical discectomy and fusion (ACDF). In this study, we investigate differences in surgical outcomes between SA and CP in ACDF. We conducted a retrospective analysis of 166 patients with myelopathy and/or radiculopathy who had ACDF with SA or CP from Jan 2013-Dec 2016. We measured surgical outcomes including Bazaz dysphagia score at 3 months, Nurick grade at last follow-up, and length of hospital stay. 166 patients (92F/74M) were reviewed. 92 presented with radiculopathy (55%), 37 with myelopathy (22%), and 37 with myeloradiculopathy (22%). The average operative time with CP was longer than SA (194 ± 69 vs. 126 ± 46 min) (p < 0.001), as was the average length of hospital stay (2.1 ± 2 vs. 1.5 ± 1 days) (p = 0.006). At 3 months, 82 patients (49.4%) had a follow-up for dysphagia, with 3 patients reporting mild dysphagia and none reporting moderate or severe dysphagia. Nurick grade at last follow-up for the myelopathy and myeloradiculopathy cohorts improved in 63 patients (85%). Prolonged length of stay was associated with reduced odds of having an optimal outcome by 0.50 (CI = 0.35-0.85, p = 0.003). Overall, we demonstrate that there is no significant difference in neurological outcome or rates of dysphagia between SA and CP, and that both lead to overall improvement of symptoms based on Nurick grading. However, we also show that the SA group has shorter length of hospital stay and operative time compared to CP.

Dose response and architecture in volume staged radiosurgery for large arteriovenous malformations: A multi-institutional study.

BACKGROUND: Optimal treatment paradigm for large arteriovenous malformations (AVMs) is controversial. Volume-staged stereotactic radiosurgery (VS-SRS) provides an effective option for these high-risk lesions, but optimizing treatment for these recalcitrant and rare lesions has proven difficult. METHODS: This is a multi-centered retrospective review of patients treated with a planned prospective volume staging approach to stereotactically treat the entire nidus of an AVM with volume stages separated by intervals of 3-6 months. A total of 9 radiosurgical centers treated 257 patients with VS-SRS between 1991 and 2016. We evaluated near complete response (nCR), obliteration, cure, and overall survival. RESULTS: With a median age of 33 years old at the time of first SRS volume stage, patients received 2-4 total volume stages and a median follow up of 5.7 years after VS-SRS. The median total AVM nidus volume was 23.25 cc (range: 7.7-94.4 cc) with a median margin dose per stage of 17 Gy (range: 12-20 Gy). Total AVM volume, margin dose per stage, compact nidus, lack of prior embolization, and lack of thalamic location involvement were all associated with improved outcomes. Dose >/= 17.5 Gy was strongly associated with improved rates of nCR, obliteration, and cure. With dose >/= 17.5 Gy, 5- and 10-year cure rates were 33.7% and 76.8% in evaluable patients compared to 23.7% and 34.7% of patients with 17 Gy and 6.4% and 20.6% with <17 Gy per volume-stage (p = 0.004). Obliteration rates in diffuse nidus architecture with <17 Gy were particularly poor with none achieving obliteration compared to 32.3% with doses >/= 17 Gy at 5 years (p = 0.007). Comparatively, lesions with a compact nidus architecture exhibited obliteration rates at 5 years were 10.7% vs 9.3% vs 26.6% for dose >17 Gy vs 17 Gy vs >/=17.5 Gy. CONCLUSION: VS-SRS is an option for upfront treatment of large AVMs. Higher dose was associated with improved rates of nCR, obliteration, and cure suggesting that larger volumetric responses may facilitate salvage therapy and optimize the chance for cure.

Characteristics and management of pain in patients with Klippel-Feil syndrome: analysis of a global patient-reported registry.

OBJECTIVE: Klippel-Feil syndrome (KFS) is characterized by congenital fusion of the cervical vertebrae. Due to its rarity, minimal research has been done to assess the quality and management of pain associated with this disorder. Using a large global database, the authors report a detailed analysis of the type, location, and treatment of pain in patients with KFS. METHODS: Data were obtained from the Coordination of Rare Diseases at Stanford registry and Klippel-Feil Syndrome Freedom registry. The cervical fusions were categorized into Samartzis type I, II, or III. The independent-sample t-test, Wilcoxon rank-sum test, and Friedman test were conducted, with significance set at p < 0.05. RESULTS: Seventy-five patients (60 female, 14 male, and 1 unknown) were identified and classified as having the following types of Samartzis fusion: type I, n = 21 (28%); type II, n = 15 (20%); type III, n = 39 (52%). Seventy participants (93.3%) experienced pain associated with their KFS. The median age of patients at pain onset was 16.0 years (IQR 6.75-24.0 years), and the median age when pain worsened was 28.0 years (IQR 15.25-41.5 years). Muscle, joint, and nerve pain was primarily located in the shoulders/upper back (76%), neck (72%), and back of head (50.7%) and was characterized as tightness (73%), dull/aching (67%), and tingling/pins and needles (49%). Type III fusions were significantly associated with greater nerve pain (p = 0.02), headache/migraine pain (p = 0.02), and joint pain (p = 0.03) compared to other types of fusion. Patients with cervical fusions in the middle region (C2-6) tended to report greater muscle, joint, and nerve pain (p = 0.06). Participants rated the effectiveness of oral over-the-counter medications as 3 of 5 (IQR 1-3), oral prescribed medications as 3 of 5 (IQR 2-4), injections as 2 of 5 (IQR 1-4), and surgery as 3 of 5 (IQR 1-4), with 0 indicating the least pain relief and 5 the most pain relief. Participants who pursued surgical treatment reported significantly more comorbidities (p = 0.02) and neurological symptoms (p = 0.01) than nonsurgically treated participants and were significantly older when pain worsened (p = 0.03), but there was no difference in levels of muscle, joint, or nerve pain (p = 0.32); headache/migraine pain (p = 0.35); total number of cervical fusions (p = 0.77); location of fusions; or age at pain onset (p = 0.16). CONCLUSIONS: More than 90% of participants experienced pain. Participants with an increased number of overall cervical fusions or multilevel, contiguous fusions reported greater levels of muscle, joint, and nerve pain. Participants who pursued surgery had more comorbidities and neurological symptoms, such as balance and gait disturbances, but did not report more significant pain than nonsurgically treated participants.

Validating the Transformation of PROMIS-GH to EQ-5D in Adult Spine Patients.

Spinal disorders and associated interventions are costly in the United States, putting them in the limelight of economic analyses. The Patient-Reported Outcomes Measurement Information System Global Health Survey (PROMIS-GHS) requires mapping to other surveys for economic investigation. Previous studies have proposed transformations of PROMIS-GHS to EuroQol 5-Dimension (EQ-5D) health index scores. These models require validation in adult spine patients. In our study, PROMIS-GHS and EQ-5D were randomly administered to 121 adult spine patients. The actual health index scores were calculated from the EQ-5D instrument and estimated scores were calculated from the PROMIS-GHS responses with six models. Goodness-of-fit for each model was determined using the coefficient of determination (R2), mean squared error (MSE), and mean absolute error (MAE). Among the models, the model treating the eight PROMIS-GHS items as categorical variables (CATReg) was the optimal model with the highest R2 (0.59) and lowest MSE (0.02) and MAE (0.11) in our spine sample population. Subgroup analysis showed good predictions of the mean EQ-5D by gender, age groups, education levels, etc. The transformation from PROMIS-GHS to EQ-5D had a high accuracy of mean estimate on a group level, but not at the individual level.

The Subjective Experience of Patients Undergoing Shunt Surgery for Idiopathic Normal Pressure Hydrocephalus.

OBJECTIVE: The counseling of patients with idiopathic normal pressure hydrocephalus (iNPH) is difficult; there is variability in the diagnostic criteria, and a definitive diagnosis can be made only postoperatively. A patient's clinical response to shunting is also difficult to predict. This study examines the subjective experience of patients treated for iNPH, to identify the challenges patients face and to improve patient outcomes and satisfaction. METHODS: We reviewed a consecutive series of patients diagnosed with iNPH who underwent ventriculoperitoneal shunt surgery between January 2012 and March 2016 at our institution. Semistructured telephone interviews were conducted with 31 patients. Interviews were analyzed using the principles of grounded theory. RESULTS: Thirty-one patients who underwent shunt surgery for iNPH were interviewed to reach saturation of themes. Seven themes were identified: 1) long preoperative course causes morbidity; 2) the decision to have shunt surgery is easy to make; 3) patients primarily desire to gain independence; 4) patients show variable levels of anxiety; 5) comorbid conditions interfere with postoperative assessment; 6) patients stand by their decision to have shunt surgery; and 7) outside information is used before surgery. CONCLUSIONS: Patients often present to the neurosurgeon frustrated and desperate after a long preoperative course. It is important to acknowledge the uncertainty regarding diagnosis and response to shunting when counseling patients. Comorbid conditions interfere with the ability to assess progression of iNPH and the effectiveness of the shunt. Patient caregivers play a large role in decision making and clinical course and should be included when counseling patients.