Outcomes of Extracorporeal Life Support (ECLS) in Acute Severe Asthma: A Narrative Review.

BACKGROUND: In this narrative review we aimed to explore outcomes of extracorporeal life support (extracorporeal membrane oxygenation (ECMO) and extracorporeal carbon dioxide removal (ECCO2R)) as rescue therapy in patients with status asthmaticus requiring mechanical ventilation. METHODS: Multiple databases were searched for studies fulfilling inclusion criteria. Articles reporting mortality and complications of ECMO and ECCO2R in mechanically ventilated patients with acute severe asthma (ASA) were included. Pooled estimates of mortality and complications were obtained by fitting Poisson's normal modeling. RESULTS: Six retrospective studies fulfilled inclusion criteria thus yielding a pooled mortality rate of 17% (13-20%), pooled risk of bleeding of 22% (7-37%), mechanical complications in 26% (21-31%), infection in 8% (0-21%) and pneumothorax rate 4% (2-6%). CONCLUSION: Our review identified a variation between institutions in the initiation of ECMO and ECCO2R in patients with status asthmaticus and discrepancy in the severity of illness at the time of cannulation. Despite that, mortality in these studies was relatively low with some studies reporting no mortality which could be attributed to selection bias. While ECMO and ECCO2R use in severe asthma patients is associated with complication risks, further studies exploring the use of ECMO and ECCO2R with mechanical ventilation are required to identify patients with favorable risk benefit ratio.

Light chain proximal tubulopathy with cast nephropathy in monoclonal gammopathy of renal significance.

Kidney tubular disorders due to monoclonal immunoglobulin light chains are common manifestations of B-cell neoplasm. Cast nephropathy (CN) is the most frequent type of these disorders and may present with acute kidney injury (AKI) due to the presence of excess light chains in the distal tubules. Light chain proximal tubulopathy (LCPT) is an uncommon form of renal disease and may present as Fanconi syndrome due to proximal tubular cell damage by intracellular deposition of light chains. The concomitant disorder of both CN and LCPT is rare given the inherent differences in the biochemical properties of the immunoglobulin light chains of each disorder. We report a 64-year-old man who presented with AKI and Fanconi syndrome who was discovered to have both CN and LCPT due to the underlying disorder of monoclonal gammopathy of renal significance and who has responded favourably with conventional chemotherapy. We also review the existing literature on this interesting subject.

Use of Radiation Therapy in Locally Advanced Pancreatic Cancer Improves Survival: A SEER Database Analysis.

OBJECTIVES: Although both radiation therapy and chemotherapy are frequently used to treat locally advanced pancreatic cancer (LAPC) patients, the role of radiation therapy remains controversial with data evaluating its efficacy mostly derived from small randomized trials. In this study, we evaluate the survival benefit of radiation therapy using SEER dataset in patients with LAPC. MATERIALS AND METHODS: The SEER Registry dataset from 2004 to 2011 was queried to identify LAPC (TNM stage III) patients. Patients with survival <2 months, unknown radiation status, or who received postoperative radiation were excluded. Multivariate analyses of prognostic factors related to survival were performed using a Cox proportional hazard-regression model. Propensity scores were estimated using probit regression. RESULTS: Our search identified 4460 patients; 59% who received radiation and 41% who did not. Radiation group patients were younger (below 65 y old: 49% vs. 38%), had smaller tumor size (largest dimension <4.5 cm: 80% vs. 75%), less lymph node involvement (33% vs. 36%), and lower rate of surgical resection (4% vs. 9%). Patients who received radiation therapy had better survival (HR=0.773; 95% CI, 0.687-0.782). The 12-month overall survival in the radiation group and nonradiation group was 43% versus 29%, respectively (P<0.001). On multivariate analyses, radiation was independently associated with improved outcomes. The survival benefit with radiation was observed in propensity score-matched cohort. CONCLUSIONS: Radiation therapy was associated with improved survival. Prospective randomized trials are needed to confirm these findings. The optimal schedule and radiation type remain undetermined.

Alzheimer's disease pathology and shunt surgery outcome in normal pressure hydrocephalus.

We aimed to determine whether presence of AD neuropathology predicted cognitive, gait and balance measures in patients with idiopathic normal pressure hydrocephalus (iNPH) after shunt surgery. This is a prospective study of gait and balance measured by Timed Up and Go (TUG) and Tinetti tests, and cognitive function measured by Mini Mental Status Exam (MMSE), before and after shunt surgery in participants 65 years and older with iNPH at the Johns Hopkins University. Random effects models were used and adjusted for confounders. 88 participants were included in the analysis with a median (IQR) time of 104 (57-213) days between surgery and follow-up. 23 (25%) participants had neuritic plaques present (NP+) and were significantly older [76.4 (6.0) years], but were otherwise similar in all demographics and outcome measures, when compared to the group without neuritic plaques (NP-). NP- and NP+ participants equally improved on measures of TUG (ß = -3.27, 95% CI -6.24, -0.30, p = 0.03; ß = -2.37, 95% CI -3.90, -0.86, p = 0.02, respectively), Tinetti-total (ß = 1.95, 95% CI 1.11, 2.78, p<0.001; ß = 1.72, 95% CI 0.90, 2.53, p<0.001, respectively), -balance (ß = 0.81, 95% CI 0.23, 1.38, p = 0.006; ß = 0.87, 95% CI 0.40, 1.34, p<0.001, respectively) and -gait (ß = 1.03, 95% CI 0.61, 1.45, p<0.001; ß = 0.84, 95% CI 0.16, 1.53, p = 0.02, respectively), while neither NP- nor NP+ showed significant improvement on MMSE (ß = 0.10, 95% CI -0.27, 0.46, p = 0.61, ß = 0.41, 95% CI -0.27, 1.09, p = 0.24, respectively). In summary, 26% of participants with iNPH had coexisting AD pathology, which does not significantly influence the clinical response to shunt surgery.

In-Hospital Mortality with Use of Percutaneous Endoscopic Gastrostomy in Traumatic Brain Injury Patients: Results of a Nationwide Population-Based Study.

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is a frequently performed invasive procedure that has been associated with high short-term mortality. Its use of special interest in traumatic brain injury (TBI) patients as nutrition support constitutes important issues in intensive care of this group. We used a national database to determine the incidence of, and factors associated with, in-hospital mortality among TBI patients undergoing PEG. METHODS: We conducted a retrospective study using the US nationwide inpatient sample to analyze data from all hospitalizations in 2008 with International Classification of Diseases, Ninth Revision, diagnostic and procedure codes identifying patients with TBI and hemorrhagic stroke who received PEG. Bivariate and multivariate logistic regression analyses were performed using demographic and clinical variables to identify predictors of in-hospital mortality in this patient population. Patients who did not undergo PEG were used as control. RESULTS: In-hospital mortality after PEG was 6% (95% CI, 0.05-0.76%) among the TBI population with 0.2% occurring in the first 7 days and 2% occurring in the first 14 days. These patients had a higher incidence of other trauma-related comorbidities and were classified as high-risk stratification based on SRRi score compared to the non-PEG group. Factors strongly predictive of in-hospital mortality were age >51 years, not receiving a PEG, and having a high comorbidity burden of >2. CONCLUSION: Understanding the rate of mortality associated with PEG in this patient population and identifying factors that increase and decrease the risk of death will improve patient selection for those most likely to benefit from this procedure.

Gastrointestinal follicular lymphoma: using primary site as a predictor of survival.

Gastrointestinal follicular lymphoma (GI-FL) is a rare extranodal variant of follicular lymphoma (FL) that has been increasingly reported in the literature. An especially indolent course is linked to the disease after a lack of observed patient death in past studies. However, overall survival (OS) and associated prognostic factors remain unclear. A large population-based database was utilized to identify demographic and clinicopathologic characteristics of GI-FL, along with survival differences among primary sites. The Surveillance, Epidemiology, and End Results Registry was used to identify GI-FL cases between the years of 1973 and 2012. Kaplan-Meier curves compared OS differences and Cox proportional hazard models analyzed prognostic factors. Final analysis included 1109 cases. Small intestinal cases, which included those with single-site and multi-segment involvement, were most common (63.6%) followed by gastric (18.2%) and colorectal cases (18.2%). Small intestinal GI-FL presented more frequently with grade I histology, and less often with grade III histology (P < 0.001 and P < 0.001, respectively). Small intestinal cases had better outcomes (5-year OS = 80.9%, P < 0.001) compared to cases involving the stomach (5-year OS = 52.7%) and colorectum (5-year OS = 71.5%). On multivariate analysis for predictors of mortality, small intestinal involvement predicted for better survival; hazard ratio (HR) 0.66 (95% CI: 0.51-0.85). Advanced age (≥66), grade (grade III), and stage (Ann Arbor Stage III/IV) predicted for mortality with HR 5.46 (95% CI: 3.80-7.84), 1.42 (95% CI: 1.10-1.83), 1.57 (95% CI: 1.15-2.16), respectively. GI-FL has poorer outcomes than previously suggested. Small intestinal involvement has a better prognosis. A possible biological basis for this will require further investigations in the future.

Increased Incidence of Second Primary Pancreatic Cancer in Patients with Prior Colorectal Cancer: A Population-Based US Study.

BACKGROUND: Identifying high-risk groups for pancreatic cancer (PC) may lead to earlier detection. We determined the risk of subsequent PC among survivors of sporadic colorectal cancer (CRC). METHODS: We evaluated data from the US Surveillance, Epidemiology, and End Results registry to identify individuals with primary CRC between the years 1973-2006. Standardized incidence ratios (SIRs) and 95 % confidence interval (95 % CI) were calculated to compare the risk of subsequent primary PC in the study cohort to that of the standard population. Analysis was stratified by age at diagnosis of CRC and sex. CRC characteristics were compared among CRC survivors with and without PC. Multivariate sub-hazard ratios were calculated to identify factors associated with subsequent primary PC, using death from non-PC causes as a competing event. RESULTS: Of the 273,144 patients with first primary CRC, 657 (0.24 %) developed subsequent PC. CRC survivors were more likely to develop PC (SIR 1.22; 95 % CI 1.09-1.35). Mean latency period (time between CRC and PC diagnosis) was 1, 3, and 5 years from index age of CRC 20-49, 50-64, and >65 years, respectively. Multivariate analysis showed CRC survivors >50 years had 3.5-fold, those with right-sided CRC had 1.2-fold, and those with localized and regional CRCs had sixfold and fivefold increased risk of PC, respectively. CONCLUSION: This study suggests that CRC survivors have an increased risk of developing subsequent PC within 1-5 years. CRC survivors age >50 with localized/regional stage, and right-sided CRC have higher predisposition to PC.

Functional gait outcomes for idiopathic normal pressure hydrocephalus after primary endoscopic third ventriculostomy.

We evaluated if patients with idiopathic normal pressure hydrocephalus (iNPH) showed functional improvement after primary endoscopic third ventriculostomy (ETV). The efficacy of ETV for iNPH remains controversial. We retrospectively reviewed 10 consecutive patients treated between 2009 and 2011 with ETV for iNPH. Seven patients with a median age of 73 years (range: 60-80) who underwent a primary ETV for iNPH were included for analysis. Median follow-up was 39 months (range: 26-46). Post-ETV stoma and aqueductal and cisternal flows were confirmed via high resolution, gradient echo and phase contrast MRI. Post-ETV timed up and go (TUG) and Tinetti performance oriented mobility assessment scores were compared to pre- and post-lumbar puncture (LP) values. A second LP was performed if ETV failed to sustain the observed improvement after initial LP. Patients who demonstrated ETV failure were subsequently shunted. Compared to pre-LP TUG and Tinetti values of 14.00 seconds (range: 12.00-23.00) and 22 (range: 16-24), post-LP scores improved to 11.00 seconds (range: 8.64-15.00; p=0.06) and 25 (range: 24-28; p=0.02), respectively. ETV failed to sustain this improvement with slight worsening between pre-LP and post-ETV TUG and Tinetti scores. Improvement from pre-LP assessment was regained after shunting and at last follow-up with TUG and Tinetti scores of 12.97 seconds (range: 9.00-18.00; p=0.250) and 25 (range: 18-27; p=0.07), and 11.87 seconds (range: 8.27-18.50; p=0.152) and 23 (range: 18-26; p=0.382), respectively. Despite stoma patency, ETV failed to sustain functional improvement seen after LP, however, improvement was regained after subsequent shunting suggesting that shunt placement remains the preferred treatment for iNPH.

Alveolar type II epithelial cell dysfunction in rat experimental hepatopulmonary syndrome (HPS).

The hepatopulmonary syndrome (HPS) develops when pulmonary vasodilatation leads to abnormal gas exchange. However, in human HPS, restrictive ventilatory defects are also observed supporting that the alveolar epithelial compartment may also be affected. Alveolar type II epithelial cells (AT2) play a critical role in maintaining the alveolar compartment by producing four surfactant proteins (SPs, SP-A, SP-B, SP-C and SP-D) which also facilitate alveolar repair following injury. However, no studies have evaluated the alveolar epithelial compartment in experimental HPS. In this study, we evaluated the alveolar epithelial compartment and particularly AT2 cells in experimental HPS induced by common bile duct ligation (CBDL). We found a significant reduction in pulmonary SP production associated with increased apoptosis in AT2 cells after CBDL relative to controls. Lung morphology showed decreased mean alveolar chord length and lung volumes in CBDL animals that were not seen in control models supporting a selective reduction of alveolar airspace. Furthermore, we found that administration of TNF-α, the bile acid, chenodeoxycholic acid, and FXR nuclear receptor activation (GW4064) induced apoptosis and impaired SP-B and SP-C production in alveolar epithelial cells in vitro. These results imply that AT2 cell dysfunction occurs in experimental HPS and is associated with alterations in the alveolar epithelial compartment. Our findings support a novel contributing mechanism in experimental HPS that may be relevant to humans and a potential therapeutic target.

The impact of left ventricular hypertrophy on survival in candidates for liver transplantation.

Left ventricular hypertrophy (LVH) occurs in 12% to 30% of patients with cirrhosis; however, its prognostic significance is not well studied. We assessed the association of LVH with survival in patients undergoing a liver transplantation (LT) evaluation. We performed a multicenter cohort study of patients undergoing an evaluation for LT. LVH was defined with transthoracic echocardiography. The outcome of interest was all-cause mortality. LVH was present in 138 of 485 patients (28%). Patients with LVH were older, more likely to be male and African American, and were more likely to have hypertension. Three hundred forty-five patients did not undergo transplantation (212 declined, and 133 were waiting): 36 of 110 patients with LVH (33%) died, whereas 57 of 235 patients without LVH (24%) died (P = 0.23). After LT, 8 of 28 patients with LVH (29%) died over the course of 3 years, whereas 9 of 112 patients without LVH (8%) died (P = 0.007). This finding was independent of conventional risk factors for LVH, and all deaths for patients with LVH occurred within 9 months of LT. No clinical or demographic characteristics were associated with mortality among LVH patients. In conclusion, the presence of LVH is associated with an early increase in mortality after LT, and this is independent of conventional risk factors for LVH. Further studies are needed to confirm these findings and identify factors associated with mortality after transplantation to improve outcomes.

Invasive adenoma and pituitary carcinoma: a SEER database analysis.

Invasive pituitary adenomas and pituitary carcinomas are clinically indistinguishable until identification of metastases. Optimal management and survival outcomes for both are not clearly defined. The purpose of this study is to use the Surveillance, Epidemiology, and End Results (SEER) database to report patterns of care and compare survival outcomes in a large series of patients with invasive adenomas or pituitary carcinomas. One hundred seventeen patients diagnosed between 1973 and 2008 with pituitary adenomas/adenocarcinomas were included. Eighty-three invasive adenomas and seven pituitary carcinomas were analyzed for survival outcomes. Analyzed prognostic factors included age, sex, race, histology, tumor extent, and treatment. A significant decrease in survival was observed among carcinomas compared to invasive adenomas at 1, 2, and 5 years (p = 0.047, 0.001, and 0.009). Only non-white race, male gender, and age ≥65 were significant negative prognostic factors for invasive adenomas (p = 0.013, 0.033, and <0.001, respectively). There was no survival advantage to radiation therapy in treating adenomas at 5, 10, 20, or 30 years (p = 0.778, 0.960, 0.236, and 0.971). In conclusion, pituitary carcinoma patients exhibit worse overall survival than invasive adenoma patients. This highlights the need for improved diagnostic methods for the sellar phase to allow for potentially more aggressive treatment approaches.

Impact of the hepatopulmonary syndrome MELD exception policy on outcomes of patients after liver transplantation: an analysis of the UNOS database.

BACKGROUND & AIMS: Patients with hepatopulmonary syndrome (HPS) are prioritized for liver transplantation (given exception points) due to their high pre- and post-transplantation mortality. However, few studies have evaluated the outcomes of these patients. METHODS: We performed a retrospective cohort study using data submitted to the United Network for Organ Sharing in a study of the effects of room-air oxygenation on pre- and post-transplantation outcomes of patients with HPS. We identified thresholds associated with post-transplantation survival using cubic spline analysis and compared overall survival times of patients with and without HPS. RESULTS: From 2002 through 2012, nine hundred and seventy-three patients on the liver transplant waitlist received HPS exception points. There was no association between oxygenation and waitlist mortality among patients with HPS exception points. Transplant recipients with more severe hypoxemia had increased risk of death after liver transplantation. Rates of 3-year unadjusted post-transplantation survival were 84% for patients with PaO2 of 44.1-54.0 mm Hg vs 68% for those with PaO2 ≤ 44.0 mm Hg. In multivariable Cox models, transplant recipients with an initial room-air PaO2 ≤ 44.0 mm Hg had significant increases in post-transplantation mortality (hazard ratio = 1.58; 95% confidence interval [CI]: 1.15-2.18) compared with those with a PaO2 of 44.1-54.0 mm Hg. Overall mortality was significantly lower among waitlist candidates with HPS exception points than those without (hazard ratio = 0.82; 95% CI: 0.70-0.96), possibly because patients with HPS have a reduced risk of pre-transplantation mortality and similar rate of post-transplantation survival. CONCLUSIONS: Although there was no association between pre-transplantation oxygenation and waitlist survival in patients with HPS Model for End-Stage Liver Disease exception points, a pre-transplantation room-air PaO2 ≤ 44.0 mm Hg was associated with increased post-transplantation mortality. HPS Model for End-Stage Liver Disease exception patients had lower overall mortality compared with others awaiting liver transplantation, suggesting that the appropriateness of the HPS exception policy should be reassessed.

Role of argon plasma coagulation in management of bleeding GI tumors: evaluating outcomes and survival.

BACKGROUND/AIMS: Tumor related gastrointestinal (GI) bleeding is a challenging clinical problem in cancer patients. Argon plasma coagulation (APC) is preferred for the management of bleeding arterio-venous malformations. Our objective was to assess the role of APC in the management of bleeding GI tumors. MATERIALS AND METHODS: This is a retrospective review of endoscopies performed at the UT MD Anderson Cancer Center over 3 consecutive years (2009-2011). This study involved patients with primary or metastatic gastrointestinal cancer with suspected GI bleeding and interventions included were endoscopies with APC. Our main outcome measurements were immediate hemostasis rate, change in transfusion requirements, re-bleeding rate, and 30-day mortality. RESULTS: Immediate hemostasis was achieved in all 10 (100%) patients, with either APC performed alone (8 patients) or with adjuvant epinephrine (2 patients). There were no procedure related complications. The pooled transfusion requirements for all 10 patients 48 hours prior to the procedure were 26 packed red blood cells units, 11 platelet units and 6 fresh frozen plasma units, while the overall requirements in the 48 hours after the procedure were 5 packed red blood cells units, 6 platelet units and no fresh frozen plasma units. Re-bleeding occurred in 3 (30%) patients during follow up. Thirty day mortality rate was 0%. Total of 7 (70%) of patients were able to continue cancer specific therapy of either chemotherapy, radiation or both. CONCLUSION: APC is feasible and safe in routine practice to manage bleeding GI tumors. It is very effective in achieving initial hemostasis (100%) and allows majority of the patients (70%) to undergo cancer specific therapy.

Reduced incidence and mortality from colorectal cancer with flexible-sigmoidoscopy screening: a meta-analysis.

AIM: To conduct a systematic review and meta-analysis of published population-based randomized controlled trials (RCTs). METHODS: RCTs evaluating the difference in mortality and incidence of colorectal cancer (CRC) between a screening flexible sigmoidoscopy (FS) group and control group (not assigned to screening FS) with a minimum 5 years median follow-up were identified by a search of MEDLINE and EMBASE databases and the Cochrane Central Register for Controlled Trials through August 2013. Random effects model was used for meta-analysis. RESULTS: Four RCTs with a total of 165659 patients in the FS group and 249707 patients in the control group were included in meta-analysis. Intention-to-treat analysis showed that there was a 22% risk reduction in total incidence of CRC (RR = 0.78, 95%CI: 0.74-0.83), 31% in distal CRC incidence (RR = 0.69, 95%CI: 0.63-0.75), and 9% in proximal CRC incidence (RR = 0.91, 95%CI: 0.83-0.99). Those who underwent screening FS were 18% less likely to be diagnosed with advanced CRC (OR = 0.82, 95%CI: 0.71-0.94). There was a 28% risk reduction in overall CRC mortality (RR = 0.72, 95%CI: 0.65-0.80) and 43% in distal CRC mortality (RR = 0.57, 95%CI: 0.45-0.72). CONCLUSION: This meta-analysis suggests that screening FS can reduce the incidence of proximal and distal CRC and mortality from distal CRC along with reduction in diagnosis of advanced CRC.

Radionuclide shunt patency study for suspected ventriculoatrial shunt malfunction.

OBJECTIVE: Radionuclide shunt studies have been used for decades to evaluate intracranial shunt patency (SP); however, the methodology is neither standardized nor well validated. The purpose of this study was to determine the clinical utility of radionuclide ventriculoatrial (VA) SP study for diagnosis of suspected shunt malfunction. METHODS: A retrospective review was undertaken of all patients who had a VA radionuclide SP study between 2001 and 2009. All had a 20-minute gamma camera acquisition (1 min/frame) immediately following injection of 99mTc DTPA into the shunt reservoir. Time-activity curves were generated and a half-time (T½) of emptying quantified. The results were correlated with the final clinical diagnoses. RESULTS: Forty-nine studies in 40 adult patients with a minimum of 6 months' follow-up were analyzed. Thirteen shunt studies had a T½ of 3.9 to 8.0 minutes, had final diagnosis of normal functioning shunt, and did not need revision surgery for a mean follow-up of 15.1 months. Fourteen patient studies had a T½ of less than 3.9 minutes; 13 had final diagnosis of overdraining shunts, and 1 required revision surgery. Twenty-two had a T½ longer than 8 minutes: 13 had final diagnosis of shunt obstruction, 4 had overdrainage, and 5 had underdrainage. CONCLUSIONS: The radionuclide SP study is valuable for evaluation of VA SP. Results can be interpreted using a single variable (T½). T½ of 3.9 to 8 minutes indicates a patent shunt; T½ less than 3.9 minutes is consistent with overdrainage. T½ of >8 requires further evaluation to differentiate between obstruction and overdrainage/underdrainage.

Glycerol rhizotomy and radiofrequency thermocoagulation for trigeminal neuralgia in multiple sclerosis.

OBJECT: Patients with trigeminal neuralgia due to multiple sclerosis (TN-MS) and idiopathic TN (ITN) who underwent glycerol rhizotomy (GR) and radiofrequency thermocoagulation with glycerol rhizotomy (RFTC-GR) were compared to investigate the effectiveness of these percutaneous ablative procedures in the TN-MS population. METHODS: Between 1998 and 2010, 822 patients with typical TN were evaluated; 63 (8%) had TN-MS and 759 (92%) had ITN. Pain relief comparisons were made between 22 GR procedures in patients with TN-MS and 470 GR procedures in patients with ITN; 50 RFTC-GR procedures in patients with TN-MS and 287 RFTC-GR procedures in patients with ITN were compared. Analysis of time to recurrence included only procedures that achieved complete pain relief without medications. RESULTS: After 15 of the GR procedures (68%) in patients with TN-MS and 315 of the procedures (67%) in those with ITN, the patients were pain free without medications (p = 0.736). After 36 of the RFTC-GR procedures (72%) in patients with TN-MS and 210 of the procedures (73%) in those with ITN, the patients were pain free without medications (p = 0.657). The difference in pain relief between GR and RFTC-GR for patients with TN-MS was not significant (p = 0.447). The median time to failure of GR was 20 months in patients with TN-MS compared with 25 months in those with ITN (p = 0.403). The median time to failure of RFTC-GR was 26 months in the TN-MS population compared with 21 months in the ITN population (p = 0.449). Patients with TN-MS experienced similar times to recurrence whether they were treated with GR or RFTC-GR (p = 0.431). CONCLUSIONS: Pain relief and durability of relief outcomes of GR and RFTC-GR were similar in patients with TN-MS and ITN, reinforcing their use as preferred treatments of TN-MS. The GR and RFTC-GR achieved comparable outcomes in patients with TN-MS, suggesting that both can be used to good effect.

Management of pediatric myxopapillary ependymoma: the role of adjuvant radiation.

INTRODUCTION: Myxopapillary ependymoma (MPE) is a rare tumor in children. The primary treatment is gross total resection (GTR), with no clearly defined role for adjuvant radiation therapy (RT). Published reports, however, suggest that children with MPE present with a more aggressive disease course. The goal of this study was to assess the role of adjuvant RT in pediatric patients with MPE. METHODS: Sixteen patients with MPE seen at Johns Hopkins Hospital (JHH) between November 1984 and December 2010 were retrospectively reviewed. Fifteen of the patients were evaluable with a mean age of 16.8 years (range, 12-21 years). Kaplan-Meier curves and descriptive statistics were used for analysis. RESULTS: All patients received surgery as the initial treatment modality. Surgery consisted of either a GTR or a subtotal resection (STR). The median dose of adjuvant RT was 50.4 Gy (range, 45-54 Gy). All patients receiving RT were treated at the involved site. After a median follow-up of 7.2 years (range, 0.75-26.4 years), all patients were alive with stable disease. Local control at 5 and 10 years was 62.5% and 30%, respectively, for surgery alone versus 100% at both time points for surgery and adjuvant RT. Fifty percent of the patients receiving surgery alone had local failure. All patients receiving STR alone had local failure compared to 33% of patients receiving GTR alone. One patient in the surgery and adjuvant RT group developed a distant site of recurrence 1 year from diagnosis. No late toxicity was reported at last follow-up, and neurologic symptoms either improved or remained stable following surgery with or without RT. CONCLUSIONS: Adjuvant RT improved local control compared to surgery alone and should be considered after surgical resection in pediatric patients with MPE.

Management of hemorrhage from cavernous malformations.

Cavernous Malformations (CMs) are immature vessels consisting of endothelium-lined sinusoids. Often diagnosed incidentally, they remain clinically silent in the vast majority of patients. Their natural history is now largely believed to follow a benign course that should be conservatively managed in the majority of cases. The exception is the treatment of deep lesions. Here there is not a consensus but the general inclination is towards radiosurgical treatment of inaccessible lesions. However, the results of radiosurgical or gross surgical resection have not been shown to be significantly better than many patients who were managed conservatively. In view of this, an understanding of the natural history of CM and the various outcomes from surgery, radiosurgery and conservative management are essential to define the goals for patients and to individualize treatment strategy.

Effectiveness of repeat glycerol rhizotomy in treating recurrent trigeminal neuralgia.

BACKGROUND: Percutaneous glycerol rhizotomy (GR) is used to treat trigeminal neuralgia (TN), with satisfactory pain relief lasting 2 to 3 years in most patients after the first intervention. The efficacy of subsequent GRs, however, has not been studied. OBJECTIVE: To compare the pain relief and durability achieved by the first GR with those obtained after subsequent GRs in a retrospective cohort of TN patients. METHODS: Between 1998 and 2010, 548 patients with TN underwent 708 GRs. After exclusions, 430 initial GRs (GR1) and 114 subsequent GRs (GR2+) were compared in terms of initial pain relief, durability, sensory change, and complications. Durability was assessed by determining median time to treatment failure for all GRs achieving complete pain relief without medications (n = 375: 264 failures, 111 censored). Predictors of initial pain relief were assessed by logistic regression, and predictors of failure were assessed by Cox regression analysis. RESULTS: After GR1, pain relief results were as follows: 285 patients (66%) were pain free without medications, 26 (6%) were pain free with medications, 66 (15%) improved, and 53 (12%) were unchanged. After GR2+, results were as follows: 90 patients (79%) were pain free without medications, 6 (5%) were pain free with medications, 7 (6%) improved, and 11 (10%) were unchanged (P = .03). Median time to treatment failure was 26 months after GR1 and 25 months after GR2+ (P = .34). On multivariate analysis, prior GR was a positive predictor of initial pain relief (odds ratio, 2.067; 95% confidence interval, 1.243-3.437; P = .005) and had no effect on durability. CONCLUSION: TN patients experienced greater pain relief and equivalent durability after GR2+ beyond the initial treatment.