Pheochromocytoma manifesting as cortical blindness secondary to PRES with associated TMA: a case report and literature review.

BACKGROUND: Pheochromocytomas are neoplasms originating from neuroectodermal chromaffin cells leading to excess catecholamine production. They are notorious for causing a triad of headaches, palpitations, and sweats. Though the Menard triad is one to be vigilant of, symptomatic presentation can vary immensely, hence the tumor earning the label "the great masquerader." CASE PRESENTATION: We report a case of pheochromocytoma initially presenting with cortical blindness secondary to posterior reversible encephalopathy syndrome and thrombotic microangiopathy from malignant hypertension. Our patient was seen in our facility less than a week prior to this manifestation and discharged after an unremarkable coronary ischemia work-up. In the outpatient setting, she had been prescribed multiple anti-hypertensives with remarkably elevated blood pressure throughout her hospitalization history. CONCLUSION: Pheochromocytoma presenting with malignant hypertension and hypertensive encephalopathy should be expected if left untreated; nonetheless, the precipitation of cortical blindness is rare in the literature. This case contributes an additional vignette to the growing literature revolving adrenal tumors and their symptomatic presentation along with complex management. It also serves to promote increased diagnostic suspicion among clinicians upon evaluating patients with refractory hypertension.

Does surgery or radiation therapy impact survival for patients with extrapulmonary small cell cancers?

BACKGROUND AND OBJECTIVES: Extrapulmonary small cell carcinomas (EPSCC) are rare tumors where therapy remains poorly defined. We sought to determine the impact of surgical extirpation and radiation therapy for outcomes of EPSCC. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried for patients with EPSCC which were further categorized by site and evaluated for survival by specific treatment strategy. RESULTS: We identified 94,173 patients with small cell carcinoma of which 88,605 (94.1%) and 5,568 (5.9%) had pulmonary small cell carcinoma and EPSCC, respectively. EPSCC patients were subdivided by site with the following proportions: genitourinary (24.1%), gastrointestinal (22.1%), head and neck (7.1%), breast (4%), and miscellaneous (42.7%). Overall EPSSC and specifically gastrointestinal disease had significantly improved median, 5- and 10-year survival with surgery and/or radiation for all stages and sizes. For all EPSCCs multivariate analysis revealed age (>50), gender (female), stage (regional, distant), radiation, and surgery to be independent predictors of survival. CONCLUSIONS: Although outcomes for EPSCC remains poor, both surgery and radiation is shown to significantly improve median, 5- and 10-year survival rates. EPSCC patients who are potential candidates for surgical resection or radiation therapy may benefit from these treatments.

Recurrent Hypoglycemia Due to a Massive Abdominal Tumor in a Nonagenarian: A Case Report and Review of the Literature.

BACKGROUND Whipple's triad is a rare condition that prompts urgent investigation. A rare cause of such a clinical presentation is excess production of insulin-like growth factor 2 (IGF-2) from a solitary fibrous tumor. CASE REPORT A 94-year-old man presented to the hospital following episodes of confusion, gait disturbance, and multiple falls secondary to hypoglycemia. His initial blood glucose was 45 mg/dL, with normalization to 144 mg/dL after administration of 1 ampule of glucose in the field. By the time the patient arrived at our facility, his blood glucose had fallen to 75 mg/dL, and then fell further to 38 mg/dL. He had no preceding history of hypoglycemia and led an active lifestyle. His medical history was relatively unremarkable with the exception of a large but asymptomatic solitary fibrous tumor previously diagnosed, being managed conservatively. A physical examination demonstrated a large, left-sided, nontender abdominal tumor. Computed tomography demonstrated a very large well-defined, complex mass in the left upper quadrant of the abdomen. Hypoglycemic episodes occurred frequently, and reliably ensued with fasting. Hypoglycemia proved refractory to conservative strategies, and surgical intervention was recommended. Despite challenges due to the tumor's characteristics, the mass was successfully resected and normoglycemia was achieved within 24 hours. CONCLUSIONS Solitary fibrous tumors may rarely present with hypoglycemia refractory to medical therapy. We present the first reported case of a nonagenarian patient with hypoglycemia secondary to ectopic production of IGF-2 from a solitary fibrous tumor managed with surgical resection.

Bilateral Lumbar Hernias Following Spine Surgery: A Case Report and Laparoscopic Transabdominal Repair.

Lumbar hernias are rare abdominal wall defects. Fewer than 400 cases have been reported in the literature and account for 2% of all abdominal wall hernias. Lumbar hernias are divided into Grynfelt-Lesshaft or Petit hernias. The former are hernia defects through the superior lumbar triangle, while the latter are defects of the inferior lumbar triangle. Primary lumbar hernias are further subdivided into congenital or acquired hernias and can further be classified as either primary or secondary. Secondary hernias occur after previous flank surgeries, iatrogenic muscular disruption, infection, or trauma. We review a rare presentation of metachronous symptomatic bilateral secondary acquired lumbar hernia following spine surgery. A successful laparoscopic transabdominal lumbar hernia repair with extraperitoneal mesh placement was performed, with resolution of the hernia symptoms. An extensive literature review regarding lumbar hernia and different types of repairs was performed.

Changes in relative glucose metabolic rate following cortisol administration in aging veterans with posttraumatic stress disorder: an FDG-PET neuroimaging study.

The authors aimed to examine central glucocorticoids effects by measuring relative glucose metabolic rate (rGMR) in the hippocampus, amygdala, and anterior cingulate cortex (ACC) and the relationship between amygdala and ACC activity. The participants were male combat veterans with and without PTSD, 52 to 81 years old. The authors utilized randomized, double-blind, placebo-controlled examinations of the rGMR response to 17.5 mg hydrocortisone (HCORT) using 2-Deoxy-2-[(18)F]fluorodeoxyglucose (FDG) Positron Emission Tomography (PET) neuroimaging. Group differences in hemispheric laterality of rGMR were observed following placebo administration, reflecting lower rGMR in the right hippocampus and ventral amygdala, and higher rGMR in the left ventral amygdala in the PTSD+ group compared to the PTSD- group. HCORT reduced these group differences in laterality. The net effect of HCORT was to restore a normal inverse association between the ACC and amygdala in the PTSD+ group, but disrupt this neural network in the PTSD- group. The magnitude of improvement in working memory correlated with greater hemispheric laterality in the dorsal amygdala following HCORT in both groups. The restorative effects of HCORT on metabolism and working memory provide a rationale for examining the therapeutic benefits of glucocorticoid manipulation in aging PTSD patients.

Parental posttraumatic stress disorder as a vulnerability factor for low cortisol trait in offspring of holocaust survivors.

CONTEXT: Lower cortisol levels in posttraumatic stress disorder (PTSD) may reflect a preexisting vulnerability associated with developing the disorder after trauma exposure. Because offspring of trauma survivors with PTSD have a greater prevalence of PTSD after their own life events than offspring of trauma survivors without PTSD and offspring of nonexposed persons, examination of patterns of basal cortisol secretion in such offspring provides an opportunity to test this hypothesis. OBJECTIVE: To characterize the patterns of basal cortisol secretion in offspring of Holocaust survivors with and without parental PTSD and children of nonexposed parents. DESIGN: Cortisol secretion was measured every 30 minutes for 24 hours. The raw hormonal data were subjected to a chronobiological analysis by applying single-oscillator and multioscillator cosinor analyses, a nonlinear least squares curve-fitting program, to determine circadian and ultradian regulatory dynamics. SETTING: The study was conducted under controlled conditions at the General Clinical Research Center at the Mount Sinai School of Medicine. PARTICIPANTS: Twenty-three Holocaust offspring with parental PTSD and 10 without parental PTSD were compared with 16 children of nonexposed parents. No participant had PTSD. MAIN OUTCOME MEASURES: Mean cortisol levels during the 24-hour cycle and other chronobiological parameters (amplitude, acrophase, circadian quotient, and goodness-of-fit coefficient) derived from single-oscillator and multioscillator models. RESULTS: Offspring with parental PTSD displayed lower mean cortisol levels, reflected by the circadian mesor and reduced cortisol amplitude, compared with offspring without parental PTSD and children of nonexposed parents. This effect seemed to be specifically related to the presence of maternal PTSD. CONCLUSIONS: Low cortisol levels and other chronobiological alterations in offspring are associated with the risk factor of maternal PTSD, raising the possibility that these alterations are acquired via glucocorticoid programming either from in utero exposures or in response to maternal behaviors early in life.

Laparoscopic Hiatal Hernia Repair with Falciform Ligament Buttress.

BACKGROUND: Using synthetic mesh to buttress the crural repair during laparoscopic hiatal hernia repair may be associated with dysphagia and esophageal erosions, while a biologic mesh is expensive and does not decrease long-term recurrence rates. This study documents outcomes of laparoscopic paraesophageal hernia repairs using the falciform ligament to reinforce the crural repair. METHODS: This is a prospective study of laparoscopic paraesophageal hernia repairs with a falciform ligament buttress. Preoperatively and at 6 and 12 months postoperatively, medications, radiologic studies, and symptom severity and frequency scores were recorded. Patients with a hiatal defect greater than 5 cm were included, while patients with recurrent hiatal hernia repairs or prior gastric surgery were excluded. Symptom scores were compared pre- and postoperatively with a p < 0.05 considered significant. RESULTS: One hundred four patients were included with a mean age of 62.4 years, and 57 patients underwent an upper gastrointestinal series at least 12 months from the initial operation with a mean follow-up of 20.6 months. The mean symptom severity score decreased from 14.32 ± 0.93 to 4.75 ± 0.97 (p < 0.001), mean symptom frequency score decreased from 14.99 ± 0.97 to 5.25 ± 0.99 (p < 0.001), and mean total symptom score decreased from 29.31 ± 1.88 to 10.00 ± 1.95 (p < 0.001). Five patients developed recurrent hiatal hernias on upper gastrointestinal series, but only three required operative intervention. CONCLUSIONS: Laparoscopic paraesophageal hernia repair with a falciform ligament buttress is a viable option for a durable closure. Ongoing follow-up will continue to illuminate the value of this approach to decrease morbidity and recurrence rates for hiatal hernia repair.

Analysis of Minimally Invasive Esophagectomy at a Single Veterans Affairs Medical Center.

BACKGROUND: To date, there are no published studies focusing on the benefits of minimally invasive esophagectomy (MIE) versus open esophagectomy at a Veterans Affairs Medical Center (VAMC). Our primary outcome was the incidence of esophageal malignancy in the veteran population and the postoperative morbidity following traditional and MIE for malignancy. DESIGN: Retrospective analysis of the incidence of esophageal malignancy at a Veteran Integrated Service Network (VISN) 5 VAMC reported to the VAMC Esophageal Tumor Registry between 2003 and 2016 and outcomes of the veterans who received esophagectomy for malignancy. Patients were followed for 5 years following diagnosis of esophageal malignancy. RESULTS: The Washington DC VAMC Tumor Registry recorded over 130 individuals with a new diagnosis of esophageal cancer between 2003 and 2016; 18 patients underwent an open transhiatal or Ivor Lewis esophagectomy and nine underwent an Ivor Lewis MIE. Surgical candidates had an average stage less than two (T1-3, N0-1, M0) and nonsurgical candidates had an average stage greater than three. Age, body mass index, smoking status, or renal function at time of surgery was similar between the two surgical groups. Patients who underwent an MIE had less blood loss (222 cc versus 822 cc, P < .001), fewer transfusions (11% versus 56%, P = .027), and more nodes harvested (10.33 versus 2.72, P < .001) with no change in leak rate (11% versus 17%, P = .703) or postoperative mortality (0% versus 6%, P = .490) compared to traditional esophagectomy. CONCLUSIONS: This report supports the migration toward MIE for malignancy and reemphasizes that veterans present with advanced disease.

Effect of sertraline on glucocorticoid sensitivity of mononuclear leukocytes in post-traumatic stress disorder.

This study examined the effects of sertraline (SER) on glucocorticoid sensitivity in mononuclear leukocytes (MNL) from eight subjects with current post-traumatic stress disorder (PTSD) and nine comparison subjects. In all, 60 ml of blood was withdrawn by venipuncture at 0800, and MNL were isolated from blood and divided into two portions: the first contained live cells incubated with a series of concentrations of dexamethasone (DEX); the second contained cells incubated with similar concentrations of DEX+2 muM SER. Group difference in the concentrations of DEX required to inhibit lysozyme activity by 50% were evaluated under conditions of DEX-only and DEX+SER using analysis of covariance (ANCOVA). A significant Group x Condition interaction reflected that SER altered the lysozyme IC(50-DEX) in the direction of decreasing sensitivity to glucocorticoids in PTSD while having no uniform effect in cells from comparison subjects. The data provide support for the idea that glucocorticoid receptors might be more responsive to antidepressants in PTSD than in persons without PTSD. Insofar as increased sensitivity to glucocorticoids has been linked with PTSD, the actions of SER on the lysozyme IC(50-DEX) suggest that this medication may target a biologic alteration associated with PTSD pathophysiology.

24-week oral ganciclovir prophylaxis in kidney recipients is associated with reduced symptomatic cytomegalovirus disease compared to a 12-week course.

BACKGROUND: Cytomegalovirus (CMV) infection is associated with reduced graft and patient survival among kidney recipients. The highest risk of CMV infection occurs in CMV-naive recipients of kidneys from seropositive donors (D+/R-). Optimal CMV prophylaxis is not established. This prospective cohort study compared the safety and efficacy of prophylaxis with 12 versus 24 weeks of oral ganciclovir. METHODS: We prospectively administered 24 weeks ganciclovir to 31 D+/R- recipients. The control group comprised 39 patients transplanted in the immediately preceding era who received a 12-week course of prophylaxis. All patients received cytolytic therapy within the first month, as well as a tacrolimus-based maintenance regimen. A logistic regression model was fit to examine the relationship between 24 weeks ganciclovir prophylaxis and the odds of developing CMV infection by one year. RESULTS: Groups were matched, though the 12-week cohort had more delayed graft function than their 24-week counterparts (45% vs. 29%, P=0.04). CMV infection occurred in 31% and 7% patients in the 12-week and 24-week groups, respectively (P

Are adult offspring reliable informants about parental PTSD? A validation study.

We developed a short questionnaire--Parental PTSD Questionnaire--(PPQ), designed to assess the presence of posttraumatic stress disorder (PTSD) symptoms in parents. Fifty-eight adult offspring of Holocaust survivors (23 men and 35 women) completed the questionnaire about a parent who was independently evaluated by a trained clinician using the Clinician Administered PTSD Scale (CAPS). Only 5.2% of the offspring reported, "not knowing" if their parent had experienced 10 or fewer symptoms, while 56.9% provided estimates for all 17 items. There were no significant differences between lifetime frequencies of the individual symptoms as endorsed on the PPQ compared to the CAPS when subjects with completed PPQs were compared with CAPS. Interrater reliability between offspring and clinician was highly significant for each of the items when evaluated separately so as to include data for subjects who endorsed not knowing if a certain symptom had been present. Further studies are warranted to examine the psychometric properties of this measure.

Relationship between cortisol and age-related memory impairments in Holocaust survivors with PTSD.

RATIONALE: Holocaust survivors with PTSD appear to show an accelerated aging effect as evidenced by their performance on tests of explicit memory, and also show more exaggerated patterns on age-related alterations in cortisol release over the diurnal cycle than Holocaust survivors without PTSD and nonexposed subjects. To investigate the implications of age-related HPA axis alterations on cognition, we examined correlations between parameters reflecting circadian cortisol release and implicit and explicit memory performance. METHODS: Nineteen Holocaust survivors with PTSD (7 men, 12 women), 16 Holocaust survivors without PTSD (7 men, 9 women), and 28 non-exposed healthy comparison subjects (13 men, 15 women) collected salivary samples at six times over the diurnal cycle, and were tested with Paired Associates and Word Stem Completion Tests. RESULTS: Negative correlations were observed between several measures of salivary cortisol concentrations and explicit memory in Holocaust survivors with PTSD after adjusting for IQ, years of education and current age reflecting poorer performance in association with higher cortisol levels. This relationship was absent in Holocaust survivors without PTSD and in demographically-comparable subjects who were not exposed to the Holocaust or other extremely traumatic events. CONCLUSION: The significantly different relationship between cortisol and memory performance in these groups suggests that the neuropsychological impairments observed in Holocaust survivors with PTSD may reflect an interaction of PTSD and aging effects.

Comparison of open, laparoscopic, and hand-assisted approaches to live-donor nephrectomy.

BACKGROUND: Minimally invasive donor nephrectomy has become a favored procedure for the procurement of kidneys from live donors. The optimal minimally invasive surgical approach has not been determined. In the current work, we compared the outcome of kidneys procured using the traditional open approach with two minimally invasive techniques: the standard laparoscopic procedure and a hand-assist procedure. METHODS: The function of live-donor kidneys procured by open versus minimally invasive procedures was compared (procedures compared were the traditional open donor nephrectomy [ODN], the standard laparoscopic [LAP] approach, and the hand-assisted [HA] laparoscopic technique). The length of donor operation, donor length of stay in the hospital, surgical complications, and cost of hospitalization for three groups of patients were assessed in a series of 150 live-donor nephrectomies. RESULTS: We found that both minimally invasive procedures yielded kidney allografts with excellent early function and a minimum of complications in the donor. The open procedure was associated with a reduced operative time but increased donor length of stay in the hospital. Resource utilization analysis revealed that both minimally invasive techniques were associated with a slight increase in costs compared with the open procedure, despite a shorter hospital stay. CONCLUSIONS: Minimally invasive donor nephrectomy is safe and effective for procuring normally functioning organs for live-donor transplantation. Of the two minimally invasive approaches examined, the hand-assisted technique was found to afford a number of important advantages, including facilitating teaching of residents and students, that it is more readily mastered by transplant surgeons, and that it may provide an additional margin of safety for the donor.

Expanded-criteria donor kidneys: a single-center clinical and short-term financial analysis--cause for concern in retransplantation.

BACKGROUND: Expanded-criteria donor (ECD) kidneys are associated with a higher risk of posttransplant failure, but they remain a favorable alternative to dialysis. Now that a uniform definition of "expanded criteria" exists, it is more appropriate than ever to evaluate their utility compared with that seen with non-ECD kidneys. METHODS: The authors analyzed 202 cadaveric kidney-only recipients that underwent transplantation from January 1999 to September 2001, including 45 (22%) recipients whose donors met current ECD criteria. RESULTS: ECD and non-ECD kidney recipients had similar pretransplant characteristics except for older age and increased duration of renal failure in the ECD group. Patient, graft, and death-censored graft survival in both groups were similar in primary recipients but significantly worse in retransplant recipients of ECD kidneys. The relative risk of death-censored graft loss was 1.58 in the ECD group (P = 0.45). Overall inpatient charges (minus organ acquisition charge) for 1 year posttransplant were 76,962 US dollars (ECD) versus 71,026 US dollars (non-ECD) (P = 0.53); the same charges in retransplant recipients were 136,596 US dollars (ECD) versus 91,296 US dollars (non-ECD) (P = 0.25). ECD recipients, especially retransplant recipients, had consistently higher creatinine concentrations, although the average current value of all functioning ECD grafts remains less than 2 mg/dL. ECD recipients had a higher incidence of ureteral stricture (4.4% vs. 0%), but this never resulted in graft loss. CONCLUSIONS: Considering the widening disparity between renal allograft availability and need and the fact that ECD kidneys provide superior outcomes compared with dialysis, the authors' data encourage the continued use of ECD kidneys in primary recipients but justify caution in the retransplant setting.

Association of hepatitis C with posttransplant diabetes in renal transplant patients on tacrolimus.

Posttransplant diabetes mellitus (PTDM) remains a common complication of immunosuppression. Although multiple risk factors have been implicated, none have been clearly identified as predisposing to the increased PTDM frequency observed in patients on tacrolimus. Hepatitis C virus (HCV) has been associated with diabetes and is a significant renal transplant comorbidity. In this study, records of 427 kidney recipients who had no known diabetes before transplantation were retrospectively examined. A multivariate logistic regression model was fit with covariates that had unadjusted relationships with PTDM to examine the independent relationship of HCV and the odds of development of PTDM by 12 mo posttransplant. A potential interaction between HCV and the use of tacrolimus as maintenance therapy on the odds of the development of PTDM was examined. Overall, PTDM occurred more frequently in HCV(+) than HCV(-) patients (39.4% versus 9.8%; P = 0.0005). By multivariate logistic regression, HCV (adjusted odds ratio [OR], 5.58; 95% confidence interval [CI], 2.63 to 11.83; P = 0.0001), weight at transplantation (adjusted OR 1.028; 95% CI, 1.00 to 1.05; P = 0.001), and tacrolimus (adjusted OR, 2.85; 95% CI, 1.01 to 5.28; P = 0.047) were associated with PTDM. A significant interaction (P = 0.0001) was detected between HCV status and tacrolimus use for the odds of PTDM. Among the HCV(+) cohort, PTDM occurred more often in tacrolimus-treated than cyclosporine A-treated patients (57.8% versus 7.7%; P < 0.0001). PTDM rates in HCV(-) patients were similar between the two calcineurin inhibitors (10.0% versus 9.4%; P = 0.521, tacrolimus versus cyclosporine A). In conclusion, HCV is strongly associated with PTDM in renal transplant recipients and appears to account for the increased diabetogenicity observed with tacrolimus.

Use of EBV PCR for the diagnosis and monitoring of post-transplant lymphoproliferative disorder in adult solid organ transplant patients.

Epstein-Barr virus (EBV) is known to be involved in the majority of patients who develop post-transplant lymphoproliferative disorder after solid organ transplant. We conducted a retrospective study to determine the utility of qualitative and quantitative Epstein-Barr virus polymerase chain reaction (PCR) for the diagnosis and monitoring of post-transplant lymphoproliferative disorder in adult solid organ transplant patients. Peripheral blood leukocytes obtained from 35 adult solid organ transplant patients consecutively referred for evaluation of possible post-transplant lymphoproliferative disorder, were tested by EBV PCR at the time of initial evaluation and at time points thereafter. Eighteen of 35 (51%) patients were ultimately diagnosed with post-transplant lymphoproliferative disorder by tissue biopsy. Fifteen of 18 (83%) patients were found to have EBER-1 positive tumors by in situ hybridization. EBV PCR was positive in 7 of 15 patients, suggesting a sensitivity of 39%. Seventeen patients without post-transplant lymphoproliferative disorder and three with EBER-1 negative post-transplant lymphoproliferative disorder all had negative EBV PCR tests, suggesting a specificity of 100%. We observed that declines in EBV DNA load were associated with response to therapeutic interventions, such as reduction in immunosuppression, rituximab therapy and chemotherapy. We conclude that peripheral blood EBV PCR may have a role in the diagnosis and monitoring of post-transplant lymphoproliferative disorder in adult solid organ transplant patients.