Citations

Abstract Rationale Pulmonary complications contribute significantly to nonrelapse mortality following hematopoietic stem cell transplantation (HCT). Identifying patients at high risk can help enroll such patients into clinical studies to better understand, prevent, and treat posttransplantation respiratory failure syndromes. Objectives To develop and validate a prediction model to identify those at increased risk of acute respiratory failure after HCT. Methods Patients underwent HCT between January 1, 2019, and December 31, 2021, at one of three institutions. Those treated in Rochester, MN, formed the derivation cohort, and those treated in Scottsdale, AZ, or Jacksonville, FL, formed the validation cohort. The primary outcome was the development of acute respiratory distress syndrome (ARDS), with secondary outcomes including the need for invasive mechanical ventilation (IMV) and/or noninvasive ventilation (NIV). Predictors were based on prior case-control studies. Measurements and Main Results Of 2,450 patients undergoing stem cell transplantation, there were 1,718 hospitalizations (888 patients) in the training cohort and 1,005 hospitalizations (470 patients) in the test cohort. A 22-point model was developed, with 11 points from prehospital predictors and 11 points from posttransplantation or early (<24-h) in-hospital predictors. The model performed well in predicting ARDS (C-statistic, 0.905; 95% confidence interval [CI], 0.870–0.941) and the need for IMV and/or NIV (C-statistic, 0.863; 95% CI, 0.828–0.898). The test cohort differed markedly in demographic, medical, and hematologic characteristics. The model also performed well in this setting in predicting ARDS (C-statistic, 0.841; 95% CI, 0.782–0.900) and the need for IMV and/or NIV (C-statistic, 0.872; 95% CI, 0.831–0.914). Conclusions A novel prediction model incorporating data elements from the pretransplantation, posttransplantation, and early in-hospital domains can reliably predict the development of post-HCT acute respiratory failure.

AbstractSeveral environmental factors have been implicated in the pathogenesis of inflammatory bowel diseases (IBD); however, the evidence for alcohol is sparse, as is its implications on disease activity and overall management. Here, we examine the available evidence for the effect of alcohol on IBD, including its association with the development of IBD, role in exacerbations, and potential medication interactions. Several mechanisms have been demonstrated to mediate the effects of ethanol in the gastrointestinal tract. Alcohol has been shown to alter the gut microbiome, disrupt intestinal barrier, and increase intestinal permeability, directly and indirectly promoting immune activation. Conversely, specific alcoholic beverages, notably red wine, may have anti-inflammatory properties capable of assisting in disease control and affecting disease monitoring. Nonetheless, most alcohol-mediated effects seem to facilitate intestinal inflammation and consequently impact disease onset, recurrence, and symptom control. Furthermore, alcohol use interferes with the metabolism of several medications leading to increased side effect profiles or even loss of effect. Notably, mesalamine, azathioprine, methotrexate, and biologic medications can all be affected by concomitant alcohol intake via a variety of mechanisms.

Spontaneous intracerebral hemorrhage is an uncommon but recognized first presentation of both primary and metastatic brain tumors. The rate of tumor‐related intracranial hemorrhage is variably reported from less than 1% to 14.6%. Hemorrhage in primary gliomas occurs in 3.7 to 7.2% of cases, mainly in glioblastoma and oligodendroglioma with low‐grade astrocytomas accounting for less than 1%. Hemorrhage associated with pilocytic astrocytomas is only sporadically reported. A series of pilocytic astrocytomas presenting as a hemorrhages prompted us to examine the incidence of bleeding in these tumors. Cases with a confirmed tissue diagnosis of pilocytic astrocytoma from 1994–2005 were reviewed retrospectively. Only subjects with radiographic evidence of hemorrhage on CT and/or MRI occurring prior to biopsy or resection and in the absence of trauma or specific vascular pathologies were included. Of 138 patients with histological pilocytic astrocytoma, the average age at diagnosis was 23 years. Eleven (8%) patients (5M, 6F) had evidence of hemorrhage at presentation. Its occurrence was independent of tumor location. Hemorrhage in pilocytic astrocytoma is likely related to the frequently observed abnormal vasculature seen in these tumors, occurs with a greater frequency than previously thought, and should be considered in the differential diagnosis of spontaneous intracerebral hemorrhage.

INTRODUCTION: In younger men, in whom organic prostate disease is uncommon, the etiology of lower urinary tract symptoms (LUTS) is frequently unclear. Studies suggest that in this young cohort voiding phase dysfunction is more common than storage phase dysfunction or normal urodynamics. Abnormal pelvic floor muscle relaxation, which can result in constipation, could potentially result in voiding dysfunction also. To date, no studies have investigated the relationship between posterior pelvic floor dysfunction and LUTS in young men. METHODS: Men who had undergone both anorectal manometry and uroflowmetry (i.e. had lower gastrointestinal and urinary symptoms) between October 2012 and September 2018 were identified. Patients >40 years old were excluded to reduce the likelihood of prostate disease. Clinical data was extracted from the electronic health records. Descriptive and comparative analyses were performed. RESULTS: Of 47 identified patients, 13 were excluded due to a history of neurogenic bowel/bladder, urethral/anal surgery or significant neurological disease. The mean age and standard deviation (SD) of the 34 men analyzed was 30 ± 6 years. Twenty patients demonstrated an abnormal balloon expulsion test (BET). The median (and interquartile range [IQR]) number of LUTS was 1.5 (1-2). An abnormal BET was not associated with symptoms of nocturia, hesitancy, incomplete emptying or the total number of LUTS (2 vs 1, P = 0.6). An abnormal balloon expulsion test was associated with a lower maximum urinary flow rate (Qmax, 14 vs 29 mls/sec, P < 0.001) but not flow duration (34 vs. 27 seconds, P = 0.2), residual urine volume (55 vs. 43 mls, P = 0.7) or urine volume (300 vs. 301 mls, P = 0.5). No correlation was seen between anal pressures at rest or during simulated evacuation and maximum urinary flow rate. No correlation was found between functional constipation and Qmax, LUTS or abnormal uroflow. No relationship between rectal sensory thresholds and urinary symptoms was identified. CONCLUSION: In this study of young men undergoing both anorectal manometry and uroflow, pelvic floor dysfunction was not associated with specific LUTS but was associated with a clinically significant reduction in maximum urinary flow rate. However an objective diagnosis of functional constipation was not associated with urologic abnormalities. Further studies will be needed to prospectively characterize this cohort and to identify whether PFD-associated LUTS and reduced flow respond to biofeedback therapy.

Object Although nontraumatic spinal arteriovenous malformations and fistulas (AVMs and AVFs) restricted to the epidural space are rare, they can lead to significant neurological morbidity. Careful diagnostic imaging is essential to their detection and the delineation of the pathological anatomy. Aggressive endovascular and open operative treatment can provide arrest and reversal of neurological deficits. Methods The authors report on 6 cases of extradural AVMs/AVFs causing progressive myelopathy. Clinical findings, diagnostic evaluation, treatment, and outcome are discussed. Special consideration is given to the anatomy of the lesions and the operative techniques used to treat them. A review of the literature concerning extradural vascular malformations is also presented. Results All 6 cases of extradural AVMs had an extradural fistulous location with intradural medullary venous drainage. These cases illustrate progressive myelopathy through cord venous congestion (hypertension) that can be caused by an extradural nidus or fistula. In 4 cases, a large epidural lake was identified on angiography. At surgery, the epidural lake was obliterated and medullary drainage interrupted. All patients had stabilization of their neurological deficits and successful obliteration of the AVM/AVF was obtained. Conclusions Extradural AVMs and AVFs are a poorly described entity with published clinical experience limited to sporadic case reports and small series. Although these lesions have a purely extradural location of arteriovenous shunting and early venous drainage, they can be responsible for acute and progressive neurological symptoms similar to those caused by their dural-based intradural counterparts. With careful imaging recognition of the pathological anatomy, surgical and endovascular techniques can be used for the treatment of extradural AVMs affording effective and durable obliteration with stabilization or reversal of neurological symptoms. Venous drainage directly correlates the pathologic mechanisms of presentation. Specific attention must be paid intraoperatively to the epidural lake common to both variants so that recurrence is avoided.

Object Spontaneous intracerebral hemorrhage is an uncommon but recognized initial presenting sign of both primary and metastatic brain tumors. The rate of tumor-related intracranial hemorrhage is variably reported from < 1 to 14.6%. Hemorrhage in primary gliomas occurs in 3.7–7.2% of gliomas, mainly in glioblastoma muliforme and oligodendroglioma with low-grade astrocytomas accounting for < 1%. Hemorrhage associated with pilocytic astrocytomas (PAs) is only sporadically reported. The authors report on a series of patients in whom PAs presenting as hemorrhages prompted them to examine the incidence of bleeding in these tumors. Methods Cases involving a confirmed tissue diagnosis of PA from 1994–2005 were reviewed retrospectively. The authors included only patients with evidence of hemorrhage on computed tomography and/or magnetic resonance imaging seen prior to biopsy or resection and in the absence of trauma or other vascular pathological entities. Results In 138 patients with histologically proven PAs, the mean age at diagnosis was 23 years. In 11 patients (8%; 5 male and 6 female) there was evidence of hemorrhage at presentation. There were no locations more susceptible to hemorrhage than any other, although no bleeding occurred within the cerebellum. All but 1 patient was treated with a gross-total resection. Conclusions Hemorrhage in association with PAs likely results from the frequently observed abnormal vasculature in these tumors, occurs with a greater frequency than previously thought, and should be considered in the differential diagnosis of spontaneous intracerebral hemorrhage.

Object The authors reviewed the long-term outcome of focal resection in a large group of patients who had intractable partial nonlesional epilepsy, including mesial temporal lobe sclerosis (MTS), and who were treated consecutively at a single institution. The goal of this study was to evaluate the long-term efficacy of epilepsy surgery and the preoperative factors associated with seizure outcome. Methods This retrospective analysis included 399 consecutive patients who underwent epilepsy surgery at Mayo Clinic in Rochester, Minnesota, between 1988 and 1996. The mean age of the patients at surgery was 32 ± 12 years (range 3–69 years), and the mean age at seizure onset was 12 ± 11 years (range 0–55 years). There were 214 female (54%) and 185 male (46%) patients. The mean duration of epilepsy was 20 ±12 years (range 1–56 years). The preceding values are given as the mean ± standard deviation. Of the 399 patients, 237 (59%) had a history of complex partial seizures, 119 (30%) had generalized seizures, 26 (6%) had simple partial seizures, and 17 (4%) had experienced a combination of these. Preoperative evaluation included a routine and video-electroencephalography recordings, magnetic resonance imaging of the head according to the seizure protocol, neuropsychological testing, and a sodium amobarbital study. Patients with an undefined epileptogenic focus and discordant preoperative studies underwent an intracranial study. The mean duration of follow up was 6.2 ± 4.5 years (range 0.6–15.7 years). Seizure outcome was categorized based on the modified Engel classification. Time-to-event analysis was performed using Kaplan–Meier curves and Cox regression models to evaluate the risk factors associated with outcomes. Among these patients, 372 (93%) underwent temporal and 27 (7%) had extratemporal resection of their epileptogenic focus. Histopathological examination of the resected specimens revealed MTS in 113 patients (28%), gliosis in 237 (59%), and normal findings in 49 (12%). Based on the Kaplan–Meier analysis, the probability of an Engel Class I outcome (seizure free, auras, or seizures related only to medication withdrawal) for the overall patient group was 81% (95% confidence interval [CI] 77–85%) at 6 months, 78% (CI 74–82%) at 1 year, 76% (CI 72–80%) at 2 years, 74% (CI 69–78%) at 5 years, and 72% (CI 67–77%) at 10 years postoperatively. The rate of Class I outcomes remained 72% for 73 patients with more than 10 years of follow up. If a patient was in Class I at 1 year postoperatively, the probability of seizure remission at 10 years postoperatively was 92% (95% CI 89–96%); almost all seizures occurred during the 1st year after surgery. Factors predictive of poor outcome from surgery were normal pathological findings in resected tissue (p = 0.038), male sex (p = 0.035), previous surgery (p < 0.001), and an extratemporal origin of seizures (p < 0.001). Conclusions The response to epilepsy surgery during the 1st follow-up year is a reliable indicator of the long-term Engel Class I postoperative outcome. This finding may have important implications for patient counseling and postoperative discontinuation of anticonvulsant medications.

✓The authors present clinical, imaging, and histological features of two adult patients found to harbor a conventional schwannoma contiguous with a deep plexiform schwannoma. One patient had neurofibromatosis (NF) Type 2 (NF2), and both intracranial (bilateral oculomotor, trigeminal, acoustic, and hypoglossal schwannomas as well as meningiomas) and intraspinal (schwannomas and meningiomas) lesions. The proximal forearm lesions consisted of a conventional schwannoma and an underlying plexiform component. The second patient, who did not have NF2, presented with a similar enlarging mass in the distal arm; two contiguous lesions were resected. Both patients exhibited distinct, readily identifiable, magnetic resonance imaging features. Plexiform schwannomas are rare lesions that occur sporadically or, on occasion, in association with NF2 or meningiomas with or without multiple schwannomas. The authors believe that a more careful examination of patients with NF2 may show that these people have a higher incidence of plexiform schwannoma than previously thought. Pathological confirmation is crucial in distinguishing plexiform schwannomas from plexiform neurofibromas, because the latter are directly associated with NF1 and have a significant tendency to undergo malignant transformation. To the best of the authors’ knowledge, this is the first report of a conventional schwannoma contiguous with a deep plexiform schwannoma.